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• •
Proctitis is an inflammation of the anus & the lining of the

rectum, affecting only the last 6 inches of the rectum. It causes discomfort, bleeding & occasionally a discharge of mucus or pus.

Causes

The cause of proctitis is not known. It is classified as an Inflammatory Bowel Disease (IBD) (such as Crohn’s disease & ulcerative colitis). It may also occur independently (idiopathic proctitis). Proctitis is also linked to stress & recent studies suggest it can result from intolerance to gluten. Non - sexually transmitted infections causing proctitis are seen less often than STD proctitis. The classical example of non - sexually transmitted infection occurs in children & is caused by the same bacteria that cause strep throat. Autoimmune proctitis is associated with diseases such as • ulcerative colitis or Crohn’s disease. Proctitis may also be caused by certain medications, • radiotherapy & inserting harmful substances into the rectum. Risk factors include - Autoimmune disorders
Causes, incidence & risk factors

can also be caused, showing symptoms such as pale skin, irritability, weakness, dizziness, brittle nails & shortness of breath. Bloody stools • Constipation • Rectal bleeding • Rectal discharge, pus • Rectal pain or discomfort • Tenesmus (pain with bowel movement) •
Signs & tests

• • • •

Examination of stool sample Proctoscopy Rectal culture Sigmoidoscopy

Treatment

There are many causes of proctitis, but they can be grouped in the following categories Autoimmune disease • Harmful substances • Non - sexually transmitted infection • Sexually transmitted disease (STD) •
Symptoms

Successful treatment of the underlying cause usually cures the problem. Proctitis caused by infection is treated with antibiotics. Corticosteroids or mesalamine suppositories may relieve symptoms of proctitis in those with Crohn’s disease or ulcerative colitis. Treatment for proctitis varies depending on severity & the cause. Enema & suppository applications are usually more effective, but some patients may require a combination of oral & rectal applications. Another treatment available is that of fiber supplements such as Metamucil. Taken daily these may restore regularity & reduce pain associated with proctitis.
Prognosis

The probable outcome is good with treatment.
Complications

A common symptom is a continual urge to have a bowel movement - the rectum could feel full or have constipation. Another is tenderness & mild irritation in the rectum & anal region. A serious symptom is pus & blood in the discharge, accompanied by cramps & pain during the bowel movement. If there is severe bleeding, anemia
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• • • •

Anal fistula Anemia Recto - vaginal fistula (women) Severe bleeding

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Sickle cell disease

• • •

Sickle cell disease is an inherited disorder of hemoglobin synthesis. Hemoglobin is a protein inside red blood cells that carries oxygen. Sickle cell anemia is caused by an abnormal type of hemoglobin called hemoglobin S (HbS). Hemoglobin S distorts the shape of red blood cells, especially when exposed to low oxygen levels. The distorted red blood cells are shaped like crescents or sickles. These fragile, sickle - shaped cells deliver less oxygen to the body’s tissues. They can also clog more easily in small blood vessels & break into pieces that disrupt healthy blood flow. Sickle cell anemia is inherited from both parents. It is much more common in people of African & Mediterranean descent. It is also seen in people from South & Central America, the Caribbean & the Middle East. Children who inherit the hemoglobin S gene from one parent & normal hemoglobin (A) from the other parent will have (AS) sickle cell trait. Individuals with sickle cell trait do not have the symptoms of true sickle cell anemia. Sickle cell disease results from a single amino acid substitution (valine for glutamate) in position 6 of the beta - globin chain of hemoglobin. In homozygotes (SS), this genetic alteration yields an unstable RBC with a shortened survival. Hypoxia or hemoconcentration causes the cells to become sickle shaped. The resulting abnormal hemoglobin causes a normocytic, hemolytic anemia with multiple diversely shaped RBCs that are susceptible to morphologically changing into a sickle shape. These sickled cells produce obstruction in small vessels, leading to ischemia & necrosis of distal tissue. Serious complications from visceral ischemia & impaired
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immunity are also common. The abnormal morphology & other cellular characteristics lead to sludging & thrombosis in small vessels with consequent tissue ischemia. Approximately 50% of patients survive beyond the fifth decade. Deaths during an acute crisis may occur in patients who are clinically free of organ failure. Infection is the leading cause of death in children. Strokes & trauma are the leading causes of death in patients aged 10 - 20 years. Children in this age range also die from acute chest syndrome, splenic sequestration crisis & aplastic crisis.

Mortality / Morbidity

• • • •

History

Patients with sickle cell disease are susceptible to different types of complications at different ages. The history differs according to the nature of the complication.
Acute pain

Pathophysiology

Acute pain due to vaso - occlusive crisis is the most frequent clinical symptom of sickle cell disease in children older than 2 years. The pain may be mild or extremely severe & can affect any area of the body. Severely painful symmetrical swelling of the hands & feet (dactylitis) caused by infarctions of the small bones may be the initial manifestation of sickle cell anemia.
Infection

Patients with SS disease are susceptible to infections because of functional asplenia secondary to auto infarction.
Acute chest crisis

• •

Chest crisis is a common cause of morbidity & mortality in patients with SS disease. Patients present with a new infiltrate on chest radiograph, often • with chest pain & fever. Tachycardia, tachypnea & hypoxemia are common. •

The pathophysiology is complex, and, in many cases, an inciting factor cannot be identified. Infection (viral or bacterial), pulmonary infarction & fat embolism are the most common. In younger children, infection is the most identified inciting factor.

Abdominal pain

Generalized abdominal pain may occur with diffuse tenderness, distension & muscular rigidity of the abdominal wall.
Splenic sequestration crisis

Vascular occlusion occurs in the splenic sinusoids, resulting in large volumes of blood trapped in the substance of the spleen.
Neurologic disease

Neurologic complications such as cerebral infarction, transient ischemic attack (TIA), intracerebral hemorrhage & spinal cord infarction are common. Presenting complaints depend on the nature of the event & • include gait disturbance, hemiparesis, paresthesias, aphasia, altered level of consciousness, or seizures.
Priapism

Heterozygotes (HS) with sickle cell trait typically have erythrocytes that contain only 30 - 40% HbS. Sickling does not occur under physiologic conditions & consequently sickle trait has a generally benign clinical course. In rare cases, hypoxia (such as when flying at high altitudes in • an unpressurized aircraft) may cause vaso - occlusive phenomena. Spontaneous hematuria, usually from the left kidney, also occurs • in patients with the sickle trait. The bleeding is often mild, but blood transfusion may rarely be needed. Patients with sickle cell disease & trait often have a high • incidence of enuresis because their kidneys cannot appropriately concentrate urine.
Physical Symptoms

• •

Priapism may occur at any age but is much more common after puberty. Priapism is defined as a sustained (more than 2 - 4 h) penile erection in the absence of sexual activity or desire. Infants & children are susceptible to aplastic crisis. Aplastic crisis is highly associated with parvovirus B19 infection. Human parvovirus B19 specifically invades proliferating erythroid progenitors. During episodes of crisis, anemia worsens. The patient appears acutely ill, tachycardic & pale.

Symptoms usually don’t occur until after age 4 months. Almost all patients with sickle cell anemia have painful episodes (crises), which can last from hours to days. These crises can affect the bones of the back, the long bones & the chest. Some patients have one episode every few years. Others have many episodes per year. The crises can be severe enough to require a hospital stay.
Common symptoms include

Aplastic crisis

• • •

Sickle cell trait
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• • • • • • • • • •

Attacks of abdominal pain Bone pain Breathlessness Delayed growth & puberty Fatigue Fever Paleness Rapid heart rate Ulcers on the lower legs (in adolescents & adults) Yellowing of the eyes & skin (jaundice)

Other symptoms include

Chest pain Excessive thirst Frequent urination Painful & prolonged erection (priapism - occurs in 10 - 40% of men with the disease) Poor eyesight/blindness • Strokes • Skin ulcers • Patients most often present with pain complaints; younger children who cannot communicate well must be observed for irritability, poor feeding, or fussiness. Vital signs should be evaluated for fever, tachycardia & • tachypnea. Pulse oximetry should be included when respiratory signs or symptoms are present. Patients usually have icteric sclera & pallor of the conjunctiva • & mucous membranes. Cardiac examination often reveals the presence of a murmur • due to anemia. Pulmonary auscultation may reveal rales or decreased breath • sounds. The presence of active bowel sounds, absence of emesis & • patient recognition of pain helps distinguish vaso - occlusive pain from other abdominal etiologies. Inspect the genitalia for priapism if the patient complains of • pain.
Causes

• • • •

More benign factors & environmental changes, such as fatigue, exposure to cold & psychosocial stress, can elicit the sickling process that prompts a crisis. Sepsis Hypovolemic Shock, Shock, Septic Stroke, Ischemic Subarachnoid Hemorrhage Withdrawal Syndromes

Differential diagnosis

Altitude Illness - Pulmonary Syndromes Chronic Anemia, Angioedema Rheumatoid Arthritis, Cholelithiasis Dehydration Pneumonia
Other problems to be considered

Thalassemia
Laboratory Studies

• •

• • • •

• •

The sickling process that frequently occurs with sickle cell anemia may be precipitated by multiple factors. A crisis may be induced by local tissue hypoxia, dehydration secondary to a viral illness, or nausea & vomiting, all of which lead to hypertonicity of the plasma. Any process that can lead to acidosis, such as infection or extreme dehydration, can cause sickling.
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Baseline abnormalities The patient with homozygous sickle cell disease (SS) typically has a hemoglobin level of 5 - 9 g/dL, with a hematocrit level decreased to 17 - 29%. The total leukocyte count is elevated to 12, 000 - 20, 000 cells/ mm3 (12 - 20 X 109/L), with a predominance of neutrophils. The platelet count is increased & the sedimentation rate is low. The reticulocyte count is usually elevated, but it may vary depending on the extent of baseline hemolysis. Peripheral blood smears demonstrate target cells, elongated cells & characteristic sickle erythrocytes.

Exams & Tests

Tests commonly performed to diagnose & monitor patients with sickle cell anemia include : Complete blood count (CBC) • Hemoglobin electrophoresis • Sickle cell test •
Other tests may include

though the condition often spontaneously resolves within 5 10 days. Severe anemia requires transfusion therapy. in most cases.generation .appearing patient presents with pain in the extremities that differs from his or her usual painful episodes. More severe pain usually requires narcotic analgesics. Provide oxygen & respiratory support as needed to relieve • hypoxemia. or are not receiving prophylactic penicillin. They should receive an intravenous third . More severe pain can often be controlled at home with oral narcotics.generation • cephalosporin & a macrolide. Acute splenic sequestration • • • Treatment Treatment is mainly directed towards prevention of complications & optimization of health. imaging of the affected extremity may be useful. Base analgesia on the severity of symptoms. have temperatures > 40 ºC. chest radiography is usually warranted. Pain should be treated.5 times the maintenance rate. transfusion is appropriate to increase the oxygen . Emergency Care Painful crisis • For the patient with acute splenic sequestration.1. CT is usually the preferred initial imaging test. therapy is primarily directed at restoring intravascular volume with appropriate isotonic fluids. administer intravenous isotonic sodium chloride solution at a rate of 1 . Hydration should be administered to replace ongoing losses & • meet maintenance needs. Therefore. Antibiotic therapy with drugs such as a third . thereby diminishing the tendency for intrapulmonary vaso .• • • • • • • • • • Bilirubin Blood oxygen CT scan or MRI Peripheral smear Serum creatinine Serum hemoglobin Serum potassium Urinary casts or blood in the urine White blood cell count Pulse oximetry is indicated when pulmonary signs or symptoms are present. When respiratory distress is moderate to severe.occlusion.carrying capacity & to decrease the percentage of sickle hemoglobin. Priapism In addition to other treatments. Evaluate any new neurologic changes by performing CNS imaging.risk patients appear toxic. Acute chest crisis Imaging Studies • • Patients presenting with variable signs of respiratory distress suggestive of acute chest syndrome should be given early & aggressive therapy. Fever • • 5 High . treat priapism with analgesia & hydration. If an ill . In patients with pulmonary symptoms. as well as to correct preexisting deficits. Aplastic crisis The mainstay of therapy for painful crises is hydration & analgesia. • Mild pain can sometimes be controlled at home by giving • Transfusions may be necessary for the patient with aplastic crisis. especially with • severe anemia. • ibuprofen or acetaminophen. Fluids should be given judiciously to avoid pulmonary edema.

rejection & graft . It is possible to diagnose sickle cell anemia during pregnancy. To reduce sickle cell crises. marriages.host disease. . including infection. gallbladder inflammation (cholecystitis). Also. • • • • • • • • • • • Preventing crises Parents should encourage children with sickle cell anemia to lead normal lives. parents & the child. Prevention Sickle cell anemia can only occur when two people who carry sickle cell trait have a child together. Genetic counseling is recommended for all carriers of sickle cell trait. painful erections (priapism) • Surgery for eye problems • Wound care. take the following precautions To prevent oxygen loss. bone infection (osteomyelitis) & urinary tract infection Joint destruction Leg sores (ulcers) Loss of function in the spleen Parvovirus B19 infection. However. lung) Drug (narcotic) abuse Erectile dysfunction (as a result of priapism) Gallstones Hemolytic crisis Infection. Therefore.cephalosporin & be admitted. hepatitis B & influenza. meningococcal. non pressurized • airplane flights) Smoking • Known sources of infection • 2) To make sure you’re getting enough fluids : Avoid too much exposure to the sun • Have fluids on hand. Preventing infections • • People with sickle cell anemia need to keep their immunizations up to date.threatening illnesses can have on siblings. both at home & away • 3) To avoid infection Have the child vaccinated as recommended • Share the above information with teachers & other caretakers. avoid Demanding physical activity (especially if the spleen is enlarged) Emotional stress • Environments with low oxygen (high altitudes. or surgery for leg ulcers • Bone marrow or stem cell transplants can cure sickle cell anemia. including Haemophilus influenza. transplants have many risks. they are currently not an option for most patients. zinc oxide. sickle cell anemia patients are often unable to find well .vs . including pneumonia. leading to low red blood cell production (aplastic crisis) Splenic sequestration syndrome Tissue death in the kidney Treatments for complications may include Dialysis or kidney transplant for kidney disease Drug rehabilitation & counseling for psychological complications Gallbladder removal • Hip replacement for avascular necrosis of the hip • Irrigation or surgery for persistent. life . Possible Complications 6 1) • • • • • • • • • Acute chest syndrome Anemia Blindness/vision impairment Brain & nervous system (neurologic) symptoms & stroke Death Disease of many body systems (kidney. pneumococcal. • when necessary Be aware of the effects that chronic.matched donors. Some patients may receive antibiotics to prevent infections. liver.

Mental retardation. long . Others experience severe. Note up . In 92 .95% of cases. bilateral epicanthal folds. but chromosomal material from 47 chromosomes is present because an extra chromosome 21 is attached (translocated) to another chromosome. open mouth with tendency of tongue protrusion & small ear 7 Down syndrome is the best . With a maternal age of 35 years.term. not to exceed 35 mg/kg/d 11 Down syndrome • • • • In 1866. open mouth with tendency for tongue protrusion & small ears with over folded helix.documented • risk factor for maternal meiotic nondisjunction. Down syndrome is by far the most common & best known chromosomal disorder in humans & the most common cause of intellectual disability. 3 – 5% cases of Down syndrome are caused by translocation. the risk is 1 in 106. today. Hydroxyurea Chemotherapy agent that allows sickled . patients can live into their 50s or beyond. brief. In 1959. Advanced maternal age remains the only well . • . Langdon Down described clinical characteristics of the syndrome that now bears his name. flat nasal bridge. • a single palmar crease (simian crease).slanted palpebral fissures (mongoloid slant) • a low nasal bridge with epicanthal folds. Pathophysiology In the past. Child with Down syndrome. the fetus will have an extra chromosome 21 in all cells. • With a maternal age of 40 years.shaped blood cells to assume the shape & characteristics of fetal hemoglobin by becoming larger & less adherent.20 mg/kg/d PO qd initially. in which the extra chromosome 21 in the sperm cell results from the nondisjunction in the meiotic stage. infrequent episodes. Causes of death include organ failure & infection. In another variant.21)). thus allowing travel through the blood vessels to occur easier. Infant with Down syndrome. 15 . sickle cell patients often died from organ failure between ages 20 & 40. Some people with the disease experience minor. When such a gamete becomes fertilized. understanding of the basic mechanism behind the maternal age effect is lacking. may increase by 5 mg/kg/d q12wk until maximum tolerated dose achieved. mosaicism. dysmorphic facial features & other distinctive phenotypic traits characterize the syndrome (see images below for examples).known example of a prenatal genetic disorder. The main features are upward . However. a small nose with flat nasal bridge.slanting palpebral fissures.slanting palpebral fissures. Down syndrome is caused by trisomy 21. • a small mouth & ears.Prognosis with over folded helix. Note up . Lejeune & Jacobs et al independently determined that trisomy 21 is the cause. the risk is 1 in 385. some cells have 47 chromosomes & others have 46 because of an error in one of the first cell divisions of the fertilized egg. bilateral epicanthal folds. where there are 46 chromosomes. Due to better understanding & management of the disease. frequent episodes with many complications. the risk is 1 in 30. usually chromosome 14 (designated as t (14. for a total of 47 chromosomes. • The characteristic phenotype of Down syndrome is basically the same in trisomy 21 & in translocation. • With a maternal age of 45 years.

cataracts. fainting episodes.15 :1) in newborns with Down syndrome. it often results in hypoxemia or hypercarbia.severe mental retardation occurs. a flat occiput. mobility or head tilt. early graying or loss of hair. Physical Growth : Short stature & obesity occurs during adolescence. the pediatrician should include the following : Parental concern about hearing.female ratio is increased (approximately 1. Down syndrome is diagnosed by recognizing dysmorphic features & the distinctive phenotype. gentleness. • respiratory infections & other problems Feeding history to ensure adequate caloric intake • Prenatal diagnosis of Down syndrome • Vomiting secondary to GI tract blockage by duodenal web or • atresia Absence of stools secondary to Hirschsprung disease • Delay in cognitive abilities. loss of motor skills. restlessness during • sleep. torticollis. neck pain. Hypotonia improves with age. large fontanels with late closure. hyperreflexia. or death. language • development (specifically expressive skills) & social competence Arrhythmia. change in bowel & bladder function. or chest pain • secondary to heart lesion Symptoms of sleep apnea. Shortly after birth. • CNS : Moderate . Premature aging : Decreased skin tone. limited neck 8 • • • • • an intelligence quotient (IQ) of 20 . A few patients exhibit anxiety & stubbornness. motor development. hypogonadism.clonic seizures are most common in older patients. chorionic villus sampling (CVS) & extraction of fetal cells from the maternal circulation.related increase in hypothyroidism. Age 2) 3) • • Down syndrome can be diagnosed prenatally with amniocentesis. palpitations. patience & tolerance are characteristics. clumsiness. quadriplegia. absent frontal & sphenoid sinuses & hypoplasia of the . a sloping forehead. degenerative vascular disease. a characteristic dermatoglyphic pattern. developmental delay. Seizure disorder (5 .body strength. In rare cases.• • • Sex a flat nasal bridge. genuine warmth. neoplasms. Behavior : Natural spontaneity. with • History When recording the history from the parents of a child with Down syndrome. the symptoms progress to paraplegia.10 %) : Infantile spasms are the most common seizures observed in infancy. difficulty walking. The male . age . spasticity. short & wide palms. whereas tonic . change in gait pattern. approximately 50).to . cheerful. incoordination. Skull : Brachycephaly. daytime somnolence. percutaneous umbilical blood sampling (PUBS).axial instability • 1) Symptoms include easy fatigability. Sleep apnea occurs when inspiratory airflow from the upper airway to the lungs is impeded for 10 seconds or longer. increased muscle tone in the legs & changes in sensation in the hands & feet. difficulty awaking. sensory deficits.85 (mean. This effect is restricted to free trisomy 21. vision. loss of adaptive abilities & increased risk of senile dementia of Alzheimer type are observed. extensor plantar reflex. a patent metopic suture. These symptoms often remain relatively stable for months or years. Articulatory problems are present. hemiplegia. loss of upper . including snoring.to . behavioral changes & school problems Symptoms of atlanto . abnormal neurologic reflexes. seizures. clonus. microcephaly. hearing loss.

imperforate anus & omphalocele are observed. tooth agenesis. Diabetes & decreased fertility can occur.50%) Abdomen . Meckel diverticulum.Xerosis.Congenital heart defects are common (40 . oppositional defiant disorder.fold increased risk of infectious diseases. Congenital heart defects . Laxity can cause backward displacement of the odontoid process. alopecia areata (< 10%). vitiligo. It can be successful in both prenatal diagnosis & diagnosis in the neonatal period. hyperextensible finger joints. • • • Eyes : Up . abscess formation & recurrent skin infections are observed. nonspecific disruptive disorder. • • Brushfield spots (speckled iris) Nose : Hypoplastic nasal bone & flat nasal bridge are typical characteristics. 2) • • • Ear of an infant with Down syndrome Note the characteristic small ear with over folded helix Neck . bilateral epicanthal folds. a pattern in hypothenar & interdigital III regions are observed. transverse palmar creases. Ears : The ears are small with an over folded helix Chronic otitis media & hearing loss are common.stimulating hormone (TSH) & thyroxine (T4) levels should be obtained at birth & annually thereafter. Genitourinary tract : Renal malformations. Hematologic system . Dermatoglyphics . hypospadias. Mouth & teeth : An open mouth with a tendency of tongue protrusion. malformed teeth.Diastasis recti & umbilical hernia occur. elastosis serpiginosa. CBC count with differential. Endocrine system Hashimoto thyroiditis that causes hypothyroidism is by far the 9 • most common acquired thyroid disorder in patients with Down syndrome. increased space between the great toe & the second toe & acquired hip dislocation (6%) are typical presentations. GI system (12%) . Skeleton : Short & broad hands.slanting palpebral fissures. ulnar loops (often 10). Immunodeficiency .maxillary sinuses occur.Atlantoaxial instability (14%) can result from laxity of • • • • • • • • • 1) transverse ligaments that ordinarily hold the odontoid process close to the anterior arch of the atlas.The disorders include attention deficit hyperactivity disorder. Hematologic tests (i. leading to spinal cord compression in about 2% of children with Down syndrome. delayed tooth eruption. a chapped lower lip. Neurobehavioral disorders . clinodactyly of the fifth fingers with a single flexion crease (20%).Patients have about a 12 . bone marrow examination) Laboratory Studies • • • • • • . angular cheilitis. a fissured & furrowed tongue. Hirschsprung disease (< 1 %). micropenis & cryptorchidism occur. Interphase fluorescence in situ hybridization (FISH) FISH may be used for rapid diagnosis.Duodenal atresia or stenosis. TE fistula. a single flexion crease in the fifth finger.Distal axial triradius in the palms. folliculitis. Chest : The inter nipple distance is decreased. mouth breathing with drooling. partial anodontia (50%). Thyroid function tests : Thyroid . Karyotyping is essential to determine the risk of recurrence. especially pneumonia. The incidence of Grave’s disease is also increased. localized hyperkeratotic lesions. including acute lymphoblastic leukemia & myeloid leukemia.e. because of impaired cellular immunity. autism spectrum disorders & stereotypical movement disorder in prepubertal children with Down syndrome The following studies may be indicated in Down syndrome : Cytogenetic studies : The clinical diagnosis should be confirmed with cytogenetic studies. Skin .Children with Down syndrome have an increased risk of developing leukemias.

Surgical Care Medical care & monitoring for the adolescent with Down syndrome • • . poor growth. eating problems. 10 • • • • • • • • • • Perform annual ophthalmologic evaluations for keratoconus or corneal opacities and/or cataracts. pregnancy & premenstrual syndrome. vitiligo & alopecia. as well as gross & fine motor. Screen for hypothyroidism & diabetes mellitus. particularly in terms of their independence.free diet. atopic dermatitis. Treat airway obstruction medically & surgically. Evaluate & treat behavioral problems. stereotypic behaviors. Perform annual audiologic evaluation.injurious behavior & Tourette syndrome. Swallowing difficulties may persist through the adolescent years & must be addressed. frequent baths. Administer anticonvulsants for tonic . seborrheic dermatitis. such as folliculitis. Timely surgery of cardiac anomalies. Early intervention programs are promising. may be necessary to prevent serious complications. fluoride treatments. elimination difficulties. self . Overall. or systemic antibiotic therapy.clonic seizures or for infantile spasms (treat with steroids). Early intervention techniques may improve the patient’s social quotient. application of antibiotic ointment.3 years are designed to comprehensively monitor & enrich their development by focusing on feeding. fungal infections of skin & nails. autism. Antibiotic prophylaxis during dental & surgical procedures in the presence of mitral valve prolapse Treatment options should include bone marrow transplantation if leukemia occurs. or weight loss) & treat the patient with a gluten . language. bloating. Programs for infants aged 0 . psychotherapy. Vaccination & medication • • • • • • • • • • • Usual immunizations & well childcare should be performed Thyroid hormone for hypothyroidism is needed to prevent intellectual deterioration & improve the individual’s overall function. If the couple has a child with trisomy 21. The risk does not appear to be increased in siblings of affected individuals. Treat skin disorders with weight reduction. proper hygiene. diarrhea. seizures & strokes. such as disruptive behavior disorders. hypotonia. academic achievement & vocational abilities. Prompt treatment of respiratory tract infections & otitis media is necessary. Manage neurologic problems. Provide pharmacologic agents. including mental retardation. community functioning & quality of life. positive developmental changes are observed in children with Down syndrome. Special attention to perioperative modalities because of atlantoaxial instability & problems with the respiratory system. xerosis. which are common during the first 6 months of life. and/or behavior therapies for psychiatric disorders. sexual abuse. Prevent dental caries & periodontal disease through appropriate dental hygiene. Continue speech & language therapy. Screen for celiac disease (symptoms such as constipation. Treat dermatologic issues.Medical Care Genetic counseling • • Trisomy 21 : Previous history of trisomy can increase a woman’s risk for a recurrence. personal & social development. with a focus on expressive language & intelligibility. Address concerns regarding menstrual hygiene. phobias. the risk of recurrence is about 1%. Prevent obesity by decreasing the patient’s caloric intake & increasing activity (social & leisure). good dietary habits & restorative care.

rheumatoid arthritis. azoospermia. gynecomastia & psychosocial problems. Adolescent male with Klinefelter syndrome who has female . pubic. In 1959. In general. Men with Klinefelter syndrome are at a higher risk of • autoimmune diseases. diabetes mellitus. decreased muscle mass & strength. small testes & an inability to produce sperm.• • • Prompt surgical repair is necessary for GI anomalies. decreased physical endurance. 11 . Klinefelter et al published a report on 9 men who had enlarged breasts. the extent of phenotypic abnormalities. is directly related to the number of supernumerary X chromosomes. Skeletal & cardiovascular abnormalities can become • increasingly severe. aganglionic megacolon & imperforate anus. hyalinization & fibrosis of the seminiferous tubules & elevated urinary gonadotropin levels. include hypogonadism. psychosocial problems. osteopenia & osteoporosis. In 1942. sparse facial & body hair. Androgen deficiency causes eunuchoid body proportions. such as tracheoesophageal (TE) fistula. systemic lupus erythematosus. resulting in seminiferous tubule dysgenesis & infertility. pyloric stenosis. Gonadal development is particularly susceptible to each additional X chromosome. The X chromosome carries genes that play roles in many body systems. Klinefelter syndrome is the most common chromosomal disorder associated with male hypogonadism & infertility. gynecomastia in late puberty. oligospermia). The addition of more than one extra X or Y chromosome to a male karyotype results in variable physical & cognitive abnormalities. as well as hypoplastic & malformed genitalia in polysomy X males. As the number of X chromosomes increases. Surgical intervention may be necessary to reduce atlantoaxial subluxation & to stabilize the upper segment of the cervical spine if neurologic deficits are clinically significant. feminine distribution of adipose tissue. XXY karyotype with variants that demonstrate additional X & Y chromosomes. • with elevated gonadotropin levels due to lack of feedback inhibition by the pituitary gland. Child with Klinefelter syndrome. Pathophysiology Klinefelter syndrome • • • • Images of physical characteristics seen in Klinefelter syndrome are shown below : Adolescent male with gynecomastia & Klinefelter syndrome. the phenotype is normal. these men with Klinefelter syndrome were discovered to have an extra sex chromosome (genotype XXY) instead of the usual male sex complement (genotype XY). diminished libido. small testes & penis. tumors (breast & germ cells). Adenotonsillectomy may be performed to manage obstructive sleep apnea. including testis function.type distribution of pubic hair & testicular dysgenesis. Klinefelter syndrome is a form of primary testicular failure. brain development & growth. The major consequences of the extra sex chromosome. somatic & cognitive development are more likely to be affected. including mental retardation. It is defined classically by a 47. or body hair. and Sjögren syndrome and historically have increased mortality. Other than a thin build & disproportionately long arms & legs. duodenal atresia. & osteoporosis. annular pancreas. The syndrome is characterized by hypogonadism (small testes. • sparse or absent facial. gynecomastia. usually • acquired through an error of nondisjunction during parental gametogenesis. axillary. leg ulcers.

many do not receive the diagnosis until they are adults.g. About 70% of patients have minor developmental & learning disabilities. weakness.g. Other symptoms include fatigue. hypospadias. micropenis. The 47. The most common indications for karyotyping are hypogonadism & infertility.20 times higher than in healthy individuals & the risk of deep vein thrombosis & pulmonary embolism is increased. . diminished short .esteem & behavioral problems. These may include academic difficulties. History distress.esteem & psychosocial development or a decreased ability to deal with stress.90% of all cases. XXY karyotype of Klinefelter syndrome spontaneously arises when paired X chromosomes fail to separate (nondisjunction in stage I or II of meiosis. which accounts for 80 . including 48. depression. This may be due to poor self . dyslexia & attention deficit disorder. epispadias. Most males with the 47. Subnormal intelligence or mental retardation may be associated with the presence of a higher number of X chromosomes. Klinefelter syndrome was found to be caused by a supernumerary X chromosome in a male. Dental Infertility & gynecomastia are the 2 most common symptoms that lead to diagnosis in patients with Klinefelter syndrome. subnormal libido. among males with known Klinefelter syndrome. this condition affects only males. Affected individuals also have disproportionately long arms & legs. the association is important to note because Klinefelter syndrome is one of the causes of genital abnormality or ambiguity. true hermaphroditism (e. Mosaicism (46. delayed speech & language acquisition. Patients may exhibit behavioral problems & psychological 12 About 40% of patients have taurodontism. weight & head circumference. About 25% have clinodactyly. XXY) is observed in about 10% of cases. language impairment. academic difficulty.40% of patients. Family background influences intelligence quotient (IQ) score. XY/47. The incidence rate is about 1% in healthy XY individuals. Psychiatric disorders involving anxiety. Other variant karyotypes. XXYY. during oogenesis or spermatogenesis). reading difficulties. Maternal & paternal meiotic nondisjunction each account for approximately 50% of Klinefelter syndrome cases The most common karyotype is 47. which is characterized by enlargement of the molar teeth by an extension of the pulp. ambiguous genitalia/under virilization (e. neurosis & psychosis are more common in this group than in the general population. decreased data . testicular feminization. Varicose veins occur in 20 . osteoporosis. Physical Growth • Infants & children achieve normal height.term memory. erectile dysfunction. The prevalence of venous ulcers is 10 . Causes • • In 1959. Age • Klinefelter syndrome goes undiagnosed in most affected males. XXY. The phenotype include complete sex reversal.retrieval skills. Although genital abnormalities are not commonly observed in • patients with Klinefelter syndrome. Height velocity increases by age 5 years & adults with Klinefelter syndrome are usually taller than adults who do not have the syndrome. ovotestes). XXY karyotype have normal intelligence.Sex Because the syndrome is caused by an additional X chromosome on an XY background. Cardiac & circulatory problems • • • CNS • Mitral valve prolapse occurs in 55% of patients. female external genitalia) & mild genital abnormalities. poor self .

Speech & behavioral therapy . microstomia. trisomy 18 is the second most common autosomal trisomy after trisomy 21. low set/malformed ears.Eunuchoid body habitus. Treatment should address 3 major facets of the disease : hypogonadism. XXXXY. malformed ears.Physical therapy should be • Trisomy 18 was independently described by Edwards et al & Smith et al in 1960. the 47. short sternum & abnormal clenched fingers. increase dosage annually in accord with the patient’s state of well . gynecomastia & psychosocial problems. Infant with Edward’s syndrome. micrognathia or retrognathia.related clinical signs that may prompt chromosomal evaluation. microcephaly. microphthalmia. are rare. Note the characteristic clenched hand with the index finger overriding the middle finger & the fifth finger overriding the fourth fingers. Note microphthalmia. & 49. or behavioral problems Older boys & adolescent males .Physicians should provide parents with information from unbiased follow . gynecomastia. academic difficulties & other psychosocial & behavioral problems.48. XXXYY.Language delay. or developmental delay Kindergarten (KG) . Testosterone enanthate • • • • • • Klinefelter syndrome may be diagnosed prenatally based on cytogenetic analysis of a fetus. The disorder is characterized by severe psychomotor & growth retardation.12 years : 50 mg IM qmo. degree of virilization. micrognathia / retrognathia. balance & coordination. Infants . Physical & occupational therapy .A multidisciplinary team approach • can assist in improving speech impairments. XXXY. Androgen Kallmann syndrome Infertility Laboratory studies Cytogenetic studies Exogenous androgen (testosterone) is the treatment of choice for many aspects of Klinefelter syndrome. Major therapeutic aims are to reduce serum gonadotropin concentrations to the upper limits of normal & to gradually induce virilization.aged boys & elementary school–aged boys . eventually reaching adult dose Edward’s syndrome (trisomy 18) • • Medical Care Early identification & anticipatory guidance are extremely helpful. although Klinefelter syndrome is rarely diagnosed in prepubertal males.90% of patients. 13 . Among liveborn children. distinctively clenched fingers & other congenital malformations.being. Androgen therapy . XXY karyotype may manifest as various subtle age . 49. cryptorchidism. Pediatric Beginning at age 11 . Differential diagnosis Fragile X Syndrome Hypogonadism Marfan Syndrome Other problems to be considered • recommended in boys with hypotonia or delayed gross motor skills that may affect muscle tone.Androgen therapy is the most important • aspect of treatment. growth & serum gonadotropin levels. If Klinefelter syndrome is not diagnosed prenatally. XXY variant is found in 80 . or small testes The 47. small phallus. Genetic counseling . learning disabilities.up studies of children with Klinefelter syndrome. microstomia.Hypospadias.

g. elongated skull.63) compared with fetuses with prenatal diagnoses (sex ratio. cerebellar hypoplasia. narrow pelvis & limited hip abduction Cardiac – More than 90% of infants with trisomy 18 have cardiac malformations. Arnold . camptodactyly. ocular hypertelorism. myelomeningocele) Cranial – Microcephaly. arthrogryposis. preauricular tags & low . corneal clouding.set & malformed ears (faunlike with flat pinnae & a pointed upper helix) Skeletal – Severe growth retardation. frontal lobe defect.bottom feet with prominent calcanei. Sex • • • • Age History • • o o o o o o o • o o o Prenatal history in trisomy 18 Maternal polyhydramnios possibly related to defective sucking & swallowing reflexes in utero Oligohydramnios secondary to renal defects Disproportionately small placenta Single umbilical artery Intrauterine growth retardation Weak fetal activity Fetal distress Clinical history in trisomy 18 Apneic episodes Poor feeding Marked failure to thrive • • • Physical Neurological • o Delayed psychomotor development & mental retardation (100 14 %) Neonatal hypotonia followed by hypertonia. defective falx cerebri. microcephaly. short sternum. In translocations that result in partial trisomy or in cases of mosaic trisomy 18. infrequent cleft lip & cleft palate. wide fontanels & prominent occiput Facial – Microphthalmia. 0. 0. short nose with upturned nares. microstomia. Pulmonary – Pulmonary hypoplasia & abnormal lobation of the lung GI – Omphalocele.e. prune belly anomaly. jitteriness. holoprosencephaly.Chiari malformation. Meckel diverticulum. apnea & seizures Malformations (e. dorsiflexed great toes. talipes equinovarus. iris coloboma. hypoplasia or aplasia of the corpus callosum.90) indicates a prenatal selection against males with trisomy 18 after the time of amniocentesis. absent . characteristic hand posture (i. meningoencephalocele. esophageal atresia with or without tracheoesophageal fistula. abnormal retinal pigmentation. hydrocephaly. syndactyly of the second & third digits. Trisomy 18 is detectable during the prenatal & newborn periods. short palpebral fissures. hypoplastic nails. rocker . common mesentery. anencephaly. diastasis recti. narrow bifrontal diameter. choanal atresia.Note a rocker . cataract. epicanthal folds. micrognathia or retrognathia. arachnoid cyst. radial hypoplasia or aplasia. clinical expression is less severe & survival is usually longer. Approximately 80% of cases occur in females. narrow palatal arch. diaphragmatic eventration. thumb aplasia. ileal atresia. clenched hands with the index finger overriding the middle finger & the fifth finger overriding the fourth finger). The preponderance of females with trisomy 18 among liveborn infants (sex ratio. Pathophysiology o o • • Trisomy 18 severely affects all organ systems. malrotation of the intestine.bottom foot with prominent calcaneus. short neck with excessive skin folds.

Abnormal erythrocyte values . which is one of the most common chromosomal abnormalities.This is the most common hematological abnormality detected.g. Treat infections as appropriate.This is the second most commonly detected abnormality. bronchitis. megalo ureters.e. Examples of manifestations of Genitourinary o o • • • Micro multicystic kidneys. These are usually secondary to otitis media. An extra chromosome 18 is responsible for the phenotype. inguinal. accessory spleens. upper respiratory tract infections (e. implications & possible outcomes. exstrophy of Cloaca. If a parent is a balanced carrier of a structural rearrangement. Orthopedic management of scoliosis may be needed secondary to hemivertebrae. Medical Care • • • • Causes • • o • o o Laboratory Studies Hematological studies in patients with trisomy 18 during the first week of life • • • Thrombocytopenia . 15 Turner syndrome • • Turner syndrome are shown in the images below. . the risk is substantially high. issues of diagnosis & survival are paramount. Full trisomy 18 is responsible for 95% of Edward’s syndrome cases. including its cause.average intelligence. pneumonia) & urinary tract infection. dermatoglyphics) – Increased number of simple arches on the fingertips. The incidence rate increases with advanced maternal age. Genetic counseling – Recurrence risk is 1% or less for full trisomy 18. Support services within the hospital & in the community should be made available to the family.This is the third most common hematological abnormality detected. pyloric stenosis. hydro ureters. Mosaicism & translocations cause few cases. In 1938. absent appendix. imperforate or malpositioned anus.e. The presence of a disabled child in any family is a source of stress & anxiety. increased atd angle & clinodactyly of the fifth fingers with a single flexion crease Intellectual capabilities range from profound mental retardation to above . double ureters. More than 95% of adult women with Turner syndrome exhibit short stature & infertility. occurring in 83% of those with trisomy 18 Neutropenia .gallbladder. hydronephrosis. bifid uterus. Cardiac management is primarily medical. Parents need information about the syndrome. transverse palmar crease. thyroid hypoplasia & adrenal hypoplasia Dermal (i. hypoplastic ovaries & clitoral hypertrophy in females Endocrine – Thymic hypoplasia. umbilical. Most of these children require a diuretic & digoxin for congestive heart failure. Psychosocial management During the neonatal period. horseshoe kidneys & unilateral renal agenesis Cryptorchidism. Henry Turner first described Turner syndrome. Provide nasogastric & gastrostomy supplementation for feeding problems. pilonidal sinus & hernias (i. diaphragmatic) Conventional cytogenetic studies • o • • • • • Full trisomy 18 (about 95% of cases) Trisomy 18 mosaicism (about 5% of cases) Translocation type trisomy 18 syndrome (very rare) Medical care in trisomy 18 is supportive. hypospadias & micropenis in males Hypoplasia of labia & ovaries.

Adrenarche. Short stature . Patients • may have dental crowding or malocclusion. X karyotype. In patients with 45.Many patients have hypoplastic or hyper convex nails.A patient with Turner syndrome. Nevi . Noonan syndrome. the beginning of pubic hair growth. Other anomalies include short fourth & fifth . Pubic hair . can occur in males or females. Children usually present with short stature. but pubic hair growth is normal. Dental . a karyotype is indicated in any girl with unexplained short stature.Excessive numbers of nevi. Pathophysiology development is absent when ovarian failure occurs before puberty. Cubitus valgus (increased carrying angle) .This is a common • skeletal anomaly in girls due to abnormal development of the trochlear head. but some girls younger than 11 years have heights within the normal range for girls without Turner syndrome. Sex Turner syndrome only occurs in females. Hyper convex nails in Turner syndrome. some girls have height & growth rates that are well within the normal range. • Nails . particularly when such individuals also are short in stature. Breast 16 Approximately 95% of individuals with Turner syndrome have both short stature & signs of ovarian failure upon physical examination. Lymphedema of the feet in an infant. when compared to other • family members. Diagnosis should be considered in individuals with primary or secondary amenorrhea & in adult women with unexplained infertility.Lymphedema in utero can cause a broad neck & • a low or indistinct hairline. about two thirds are missing the paternal X chromosome. Although the presence of other features may increase the index of suspicion.A high arched palate suggests the diagnosis.shaped cross section. Note the narrow hip development. Note U . short stature is due to both a slightly • slower growth rate in childhood & to an essentially absent adolescent growth spurt. It is an autosomal dominant genetic disorder & is not a chromosomal disorder. are common. occurs at a normal age & is not an indication that puberty will progress normally. Webbed neck . • Although these are not a clinical problem. History Older individuals may have a history of swollen hands & feet at birth. It is unrelated to Turner syndrome. The toes have the characteristic sausagelike appearance. Physical Turner syndrome is caused by the absence of one set of genes from the short arm of one X chromosome. but an increased risk of keloid formation is noted.Pubic hair development is normal.shaped chest. These may be removed if rubbed or irritated by clothing. sometimes inappropriately called male Turner syndrome. but heights are below the 50th percentile for girls without Turner syndrome. they are rarely seen in other patients. Ovarian failure . Before age 11 years. presenting symptoms usually involve issues of puberty & fertility as well as short stature. This posterior view shows a low hairline & a shield . In older adolescents & adults.Suspect ovarian failure in girls who have no • breast development by age 12 years or who have not started menses by age 14 years. Elevated levels of luteinizing hormone (LH) & FSH confirm ovarian failure.In adults.

Loose folds of skin. amblyopia & cataract s are more common in girls with Turner syndrome. The buccal smear for Barr bodies is obsolete. A male phenotype excludes the diagnosis. In patients with a single X chromosome. if needed. the chromosome is of • maternal origin in two thirds of cases. if not all. Turner syndrome. Turner syndrome may be prenatally diagnosed by amniocentesis or chorionic villous sampling. These can be cycled in a 3 . They should be evaluated clinically & referred for further treatment.This is usually due to intestinal vascular malformations.week on. but starting too early can compromise adult height. Hip dislocation . Taller adult heights occur with the longest treatment durations before the start of puberty. GI bleeding . yet some are susceptible to numerous chronic conditions. growth hormone therapy is standard to prevent short stature as an adult. In infants. it can be a clue to the presence of Turner syndrome.metacarpals & metatarsals. This is a result of resolving lymphedema & occasionally is observed after infancy.Serous otitis media is more common. particularly in the neck. In childhood.Although this finding is • • • • • • • of minimal clinical significance.Lymphedema may be present at any age & is one finding that can suggest Turner syndrome on fetal ultrasonography.15 years. Estrogen replacement therapy is usually required. Causes • Short fourth metacarpal or metatarsal . X cell line or a cell line with deletion of the short arm of the X chromosome (Xp deletion). Many of the features of Turner syndrome. such as the webbed neck & low posterior hairline. which is on the X chromosome. Prenatal signs The diagnosis of Turner syndrome requires the presence of typical phenotypic features & the complete or partial absence of a second sex chromosome.The chest appears to be broad with widely spaced nipples. The ideal age for initiating treatment has not been established.18 months. Most. Lymphedema . Medical Care • o o o Most concepti with a 45. are signs in newborns. . probably due to poor anatomic drainage of the middle ear.Ptosis. Ears . X karyotype spontaneously abort. progestin can be added later. Most people live long & healthy lives. strabismus. Shield chest . Diagnosis is confirmed by the presence of a 45. Laboratory Studies Diagnosis • • • Karyotyping is required for diagnosis of Turner syndrome. including the short • stature.arched palate. but the incidence of Crohn disease & ulcerative colitis is also increased. Advanced maternal age is not associated with an increased • incidence. 1 . Lymphedema is the cause of other anomalies.week off regimen after 6 . Cutis laxa . which may be associated with a high . Neonatal pedal edema suggests a diagnostic evaluation for 17 Turner syndrome is a lifelong condition. Eye . of those who survive to birth are suspected to have mosaicism for a normal cell line. Estrogen is usually started at age 12 . regardless of karyotype. This may be caused in part by a short sternum. the combination of dysplastic or hypoplastic nails & lymphedema gives a characteristic sausagelike appearance to the fingers & toes.Infants have a higher incidence of congenital hip dislocation. Treatment can be started with continuous low dose estrogens at 12 years. are due to the lack of a second SHOX gene.

an entity that von Jaksh described earlier. Because 2 types of chains α (& non . most thalassemias are not considered hemoglobinopathies because the globin chains are normal in structure & because the defect is limited to a decreased rate of production of these normal chains. β . Before long. pairing with ε chains ( α 2/ ε 2). normally produced globin chains. an excess of the normally produced type is present & accumulates in the cell as an unstable product. which he initially thought was erythroblastic anemia. is formed by α 2/ δ 2 chains. it produces the Hb molecule (heme plus globin equals Hb). ξ chains α (. which Whipple introduced.α chain. leading to the destruction of the cell. The type of thalassemia usually carries the name of the under . a Detroit pediatrician. Pathophysiology Globin chain production Background In 1925. which means “related to blood. This imbalance is the hallmark of all forms of thalassemia.12 Thalassemia A) of α 2/ β 2. In embryonic life. δ ) impedes Hb synthesis & creates an imbalance with the other. the molecular biology & genetics of the thalassemia syndromes have been described in detail. γ .1 ( ξ 2/ ε 2). Two different pairs of globin chains form a tetrameric structure with a heme moiety in the center. Fetal Hb is composed of α 2/ γ 2 & the primary Adult Hb (Hb 18 The thalassemias are inherited disorders of Hb synthesis that result from an alteration in the rate of globin chain production.binding characteristics. This Hb appears shortly after birth & remains at a low levels throughout life. Thomas Cooley. A third physiologic Hb. For this reason. Types of haemoglobins 1 To understand the genetic changes that result in thalassemia. All normal Hbs are formed from 2 α . The globin chain as a unit is a major building block for Hb : together with heme.2. Although Cooley was aware of the genetic nature of the disorder. Such Hbs exhibit different oxygen . Sickle cell anemia is a type of qualitative (structural) abnormality of hemoglobin whereas thalassemia is a type of quantitative (haemoglobin production) type of abnormality.like chains & 2 non . when α chains are produced. known as Hb A2. depending on the types of chains pairing together. described a severe type of anemia in children of Italian origin.” Their clinical severity widely varies. The word thalassemia is a Greek term derived from thalassa.like chains) combine with γ chains to produce Hb Portland ( ξ 2/ γ 2) & with å chains to produce Hb Gower . A decrease in the rate of production of a certain globin chain or chains ( α . ranging from asymptomatic forms to severe or even fatal entities. he failed to investigate the apparently healthy parents of the affected children. one should be familiar with the physiologic process of globin chain production in the healthy individual. • • • • The thalassemias are inherited disorders of hemoglobin (Hb) synthesis. they form Hb Gower .α ) pair with each other at a ratio close to 1 :1 to form normal Hbs. In recent years. which means “the sea” (referring to the Mediterranean) & emia. The name Mediterranean anemia. is misleading because the condition can be found in any part of the world. Cooley realized that erythroblastemia is neither specific nor essential in this disorder & that the term erythroblastic anemia was nothing but a diagnostic catchall. Subsequently. He noted abundant nucleated red blood cells (RBCs) in the peripheral blood. Various types of Hb are formed. revealing the wide range of mutations encountered in each type of thalassemia. normally related to the oxygen delivery requirement at different developmental stages in human life.

whereas β . The excessive chains in α thalassemia are γ chains earlier in life & β chains later in life. produce most of the manifestations encountered in all of the β thalassemia syndromes. α chains that accumulate in the RBC precursors are insoluble. although relatively unstable. the surviving cells that arrive in the peripheral blood with intracellular inclusion bodies (excess chains) are subject to hemolysis. but not all. This is due to the increased use of δ chains by the excessive free α chains. This is true in almost all anemias caused by impairment in production of either of the 2 main components of Hb : heme or globin. nevertheless remain viable & able to produce soluble Hb molecules such as Hb Bart (4 γ chains) & Hb H (4 β chains). this means that both hemolysis & ineffective erythropoiesis cause anemia in the person with β thalassemia. . interact with the membrane (causing significant damage) & interfere with cell division. The ability of some RBCs to maintain the production of γ chains. ineffective erythropoiesis). the thalassemia is termed β +. These basic differences in the 2 main types of thalassemia are responsible for the major differences in their clinical manifestations & severity. leading to hypochromasia.3% of the total Hb). For example. level of the three normal hemoglobin viz. which are capable of pairing with some of the excessive α chains to produce Hb F. A2 & F are reduced (alpha thalassemia). the consequences of accumulation of the excessive globin chains in the various types of thalassemia are different. The significant excess of free α chains caused by the deficiency of β chains causes destruction of the RBC precursors in the bone marrow (i. whereas the remaining α chains precipitate in the cells. precipitate in the cell. In β thalassemia. However. A. this does not occur in the silent carrier state. this is not the situation in α thalassemia. leading to the classic hypochromic & microcytic picture of thalassemia. In addition. the level of Hb A2 ( δ 2/ α 2) is usually elevated.e. of the excessive α chains are used to form Hb A2 with the δ chains. Some. In the most common type of β thalassemia trait. The degree of toxicity caused by the excessive chains varies according to the type of such chains (e.carrying ability. If the synthesis of alpha chain is suppressed.0 thalassemia indicates a complete absence of production of β chains from the involved allele. since both Hb level & RBC indices remain normal. Because such chains are relatively soluble. However. The consequences of impaired production of globin chains ultimately result in the deposition of less Hb into each RBC. The reduction varies from a slight decrease to a complete absence of production. unable to form Hb tetramers. Cellular pathophysiology The basic defect in all types of thalassemia is imbalanced globin chain synthesis.5 . by pairing with the α chains. leading to destruction of RBCs.produced chain or chains. is advantageous. The δ gene. reacting with cell membranes.g. in one way or another. This leads to excessive intramedullary destruction of the RBC precursors. The Hb deficiency causes RBCs to be smaller. the same pathophysiology applies with substantial exaggeration. On other hand if beta gene chains are suppressed because of mutations in beta gene then production of adult hemoglobin is suppressed (beta thalassemia). Binding some of the excess a chains undoubtedly reduces the symptoms of the disease & provides additional Hb with oxygen . precipitate in the RBC precursors and. when β chains are produced at a lower rate. 19 In the severe forms. the toxicity of α chains in β thalassemia is more prominent than the toxicity of β chains in α thalassemia). Delta thalassemia is due to suppression of both beta & delta chain synthesis (HbF is high) with moderate anemia. δ chains produce Hb A2 (approximately 2. they are able to form homotetramers that. excessive α chains. such as β thalassemia major or Cooley anemia. intervening with normal cell division & acting as foreign bodies. which results from a lack of adequate β chains with which to pair. is known to have a physiologic limitation in its ability to produce adequate δ chains. unlike β and α genes.

from macrophages & from hepatocytes. anemia of inflammation & chronic renal diseases. in response to severe anemia. Most nonheme iron in healthy individuals is bound tightly to its carrier protein. resulting in significant damage to these organs. the cellular iron exporter ferroportin. In patients with β thalassemia intermedia. Adding a second source of iron would theoretically result in more harm to the patient. results in massive splenomegaly. Iron status is another important factor that influences iron absorption. this is not the case in patients with severe β thalassemia because a putative plasma factor overrides such mechanisms & prevents the production of hepcidin. the transferrin becomes saturated & free iron is found in the plasma. the ineffective erythropoiesis is reversed & the hepcidin level is increased. The large numbers of abnormal RBCs processed by the spleen. Improvement & availability of hepcidin assays facilitates diagnosis of such conditions. the iron absorption decreases because of an increased hepcidin level. which produces a liver hormone called hepcidin. leading to manifestations of hypersplenism. despite a similar iron overload. thus. such as is seen in thalassemia. iron absorption continues despite the iron overload status. patients with β thalassemia intermedia who are not receiving blood transfusions have lower ferritin levels than those with β thalassemia major who are receiving regular transfusion regimens. leading to a low plasma iron concentration. the effect of hepcidin on iron recycling is carried through its receptor “ferroportin. Hepcidin regulates dietary iron absorption. In patients with iron overload (e. It acts by causing degradation of its receptor.” which exports iron from enterocytes & macrophages to the plasma & exports iron from the placenta to the fetus. adding more symptoms to the clinical & laboratory manifestations of the disease. such as the heart. hepcidin allows recycling of the iron from the macrophages. the severe expansion of the ineffective marrow is reversed. As a result. it decreases iron flow into the plasma from the gut. In severe hepcidin deficiency. in whom the macrophages are depleted despite iron overload. The elevated Hb F level increases oxygen affinity. When ferroportin is degraded. iron absorption is increased & macrophages are usually iron depleted. transferrin. Ferroportin is up regulated by iron stores & down regulated by hepcidin. which. hemochromatosis).Furthermore. releasing high amounts of ferritin.g. Thus. iron absorption is decreased & macrophages retain iron. together with its hematopoietic response to the anemia if untreated. such as is observed in patients with thalassemia intermedia. In the latter group. This iron is harmful since it provides the material for the production of hydroxyl radicals & additionally accumulates in various organs. resulting in a lower ferritin level. Malfunctions of the hepcidin . . adds another mechanism to protect the RBCs in persons with β thalassemia. The development of hepcidin agonists & antagonists may enhance 20 the treatment of such anemias. However. this is not the case because iron absorption is regulated by 2 major factors : ineffective erythropoiesis & iron status in the patient. increased production of Hb F. together with the profound anemia. This relationship may also explain why patients with β thalassemia who have similar iron loads have different ferritin levels based on whether or not they receive regular blood transfusions. Ineffective erythropoiesis results in increased absorption of iron because of down regulation of the HAMP gene. severe expansion of the ineffective erythroid mass leads to severe bone expansion & deformities. However. By administering blood transfusions. lower amounts of ferritin are released. In iron overload conditions. stimulates the production of erythropoietin. As mentioned above. leading to hypoxia. endocrine glands & liver. If the chronic anemia in these patients is corrected with regular blood transfusions. plasma iron concentration & tissue iron distribution & is the major regulator of iron. such as severe thalassemia. For example. Both iron absorption & metabolic rate increase.ferroportin axis contribute to the etiology of different anemias.

A CBC count in one parent that demonstrates hypochromia & microcytosis in the absence of any explanation 21 This condition. the diagnosis cannot be confirmed based on Hb electrophoresis results. one can appreciate that certain modifiers may result in the development of milder types of thalassemia.g. In this form. usually found by chance among various ethnic populations. Classification of thalassemia is frequently adequate evidence for the presence of thalassemia. • • • α thalassemia major . the Indian subcontinent & some parts of the Middle East. while the child is being evaluated for some other condition. giving α thalassemia the unique feature of gene duplication.This condition is the result of complete deletion of the a gene cluster on both copies of chromosome 16 (oo/oo). which results from the deletion or inactivation of 3 α globin genes (oo/ao). This condition is encountered mainly in Southeast Asia. icterus & abnormal RBC indices.Similar to patients who silently carry α thalassemia. or the presence of a milder thalassemia mutation all typically ameliorate the symptoms of thalassemia. except for occasional low RBC indices. which are unstable & precipitate in the RBC. the presence of higher Hb F level. which are usually normal in all α thalassemia traits. α (thalassemia intermedia) α thalassemia Several forms of α thalassemia are known in clinical practice. some of the more common forms are as follows : Silent carrier β thalassemia . represents α thalassemia intermedia. Supra vital stain in hemoglobin H disease that reveals Heinz bodies (golf ball appearance). splenomegaly. These inclusions represent b chain tetramers (Hb H).inheritance of α thalassemia. Patients are hematologically healthy. These inclusions are termed Heinz bodies. One may look for hematologic abnormalities in family members (e. unique inclusions in the RBCs are usually observed. Hb H disease A large number of thalassemic syndromes are currently known. particularly African American. parents) to support the diagnosis. The mutation that causes the . 2 α genes are located on each chromosome 16. leading to the severe form of homozygous α thalassemia.globin gene on chromosome 11. The ao/ao form is much more common in black populations because the doubly deleted (oo) form of chromosome 16 is rare in this ethnic group. In the silent carrier state. α thalassemia trait This trait is characterized by mild anemia & low RBC indices. co . which form the different Hbs normally found in RBCs. with mildly to moderately severe anemia. More sophisticated tests are necessary to confirm the diagnosis. When peripheral blood films stained with supravital stain or reticulocyte preparations are examined. This condition is typically caused by the deletion of 2 α (a) genes on one chromosome 16 (aa/oo) or one from each chromosome (ao/ao). several clinical forms of β thalassemia are recognized. The most important types in clinical practice are those that affect either α or β chain synthesis. β thalassemia . depicted below. giving it the appearance of a golf ball. these patients have no symptoms. Factors that may reduce the degree of globin chain imbalance are expected to modify the severity of the symptoms. The most common forms are as follows : Silent carrier α thalassemia • • • This is a fairly common type of subclinical thalassemia. which is usually incompatible with life & results in hydrops fetalis unless intrauterine blood transfusion is given. leaving only 3 of 4 genes (aa/ao). each involves decreased production of one globin chain or more. one of the α genes is usually absent. except for possible low RBC indices. As pointed out above.By understanding the etiology of the symptoms in thalassemia.Similar to α thalassemia. This duplication is in contrast to only one β .

which typically does not require regular blood transfusions.This condition is usually due to a • • • compound heterozygous state. target cells & faint basophilic stippling. they are encountered among all ethnic groups & in almost every country around the world. age at onset of symptoms varies significantly. thalassemic disorders. Sex Both sexes are equally affected with thalassemia. nucleated RBCs. Certain types of thalassemia are more common in specific parts of the world Mortality / Morbidity Peripheral blood film in thalassemia minor Thalassemia intermedia . disorders worldwide & people who carry thalassemia in India alone number approximately 30 million. Unexplained hypochromia & microcytosis in a neonate. depending on the severity of the condition. Heart failure due to severe anemia or iron overload is a common cause of death in affected persons. or the complications of splenectomy in others. or both. Age Peripheral blood film in Cooley anemia Frequency International Despite the inherited nature of thalassemia. marked anisocytosis. and. Patients with Hb H disease also require close monitoring. Some patients may require splenectomy. mortality & morbidity vary according to the severity of the disease & the quality of care provided. Reportedly. as shown below. Peripheral blood film examination usually reveals marked hypochromia & microcytosis (without the anisocytosis usually encountered in iron deficiency anemia).dependent anemia. The production of β chains from the abnormal allele varies from complete absence to variable degrees of deficiency. 80% of whom die within the first 5 years of life from complications of anemia. on occasion. In beta thalassemia. depicted below. Liver disease. massive splenomegaly.Patients have mild anemia. resulting in anemia of intermediate severity. Hb F. These facts confirm that thalassemias are among the most common genetic disorders in humans. polychromasia. Beta thalassemia major is a mortal disease & virtually all affected fetuses are born with hydrops fetalis as a result of severe anemia. Thalassemia major (Cooley anemia) . fulminating infection. are highly suggestive of the diagnosis. 15 million people have clinically apparent . hypochromic macrocytes. Peripheral blood film in hemoglobin H disease in a newborn 22 Worldwide. β thalassemia associated with β chain structural variants . massive splenomegaly in some patients. fragmented RBCs. In patients with various types of ? thalassemia.This condition is characterized by transfusion .• thalassemia is very mild & represents a β + thalassemia. or other complications precipitated by the disease or by its treatment are some of the causes of morbidity & mortality in the severe forms of thalassemia. complications of blood transfusions. abnormal RBC indices & abnormal Hb electrophoresis results with elevated levels of Hb A 2 .The most significant condition in this group of thalassemic syndromes is the Hb E/ β thalassemia. clinical abnormalities in patients with severe cases & hematologic findings in carriers are evident at birth. growth retardation & peculiar facies in untreated individuals. Morbidity is usually related to the anemia. which may vary in its clinical severity from as mild as thalassemia intermedia to as severe as β thalassemia major. They may require frequent or only occasional blood transfusions. as depicted below. β thalassemia trait . bone deformities. Examination of a peripheral blood preparation in such patients reveals severe hypochromia & microcytosis. immature leukocytes.

Bony changes may be severe. the requirement for iron to supply the overwhelming production of ineffective erythrocytes is tremendous. Iron overload is one of the major causes of morbidity in all patients with severe forms of thalassemia. this anomaly is due to the massive splenomegaly & the ineffective erythropoiesis that prevents the release of the cells from the bone marrow. despite the severe hemolysis. The diagnosis is usually suspected in children with an • unexplained hypochromic & microcytic picture. especially those who belong to one of the ethnic groups at risk. Manifestations of anemia include extreme pallor & enlarged • abdomen due to hepatosplenomegaly. no unusual signs or • symptoms are encountered. this complication is usually deferred until puberty (if the patient survives to that age). the • symptoms vary from extremely debilitating in patients who are not receiving transfusions to mild & almost asymptomatic in those receiving regular transfusion regimens & closely monitored chelation therapy. regardless of whether they are regularly transfused. causing significant increases in GI absorption of iron. o Patients’ typical reports may lead a physician who is not familiar with the condition to a first impression of acute leukemia. the elevated level of reticulocytes expected in all hemolytic anemias does not occur. physicians should always inquire about the patient’s ethnic background. but the reason behind this phenomenon is not clear. Many believe that. Increased iron absorption is the cause in nontransfused patients. The classic “hair on end” appearance on plain skull radiographs of a patient with Cooley anemia. In most patients with thalassemia traits. in nontransfused patients. high lactate dehydrogenase (LDH) level & low level of haptoglobin. o This impression is supported by the large spleen. leading to slight enlargement of the abdomen. family history of hematologic disorders & dietary history. such as beta thalassemia major.o History The history in patients with thalassemia widely varies. In more severe forms. For this reason. despite the iron overload state in these patients & the increased iron deposits in the bone marrow. depending on the severity of the condition & the age at the time of diagnosis. To support the impression of acute leukemia further. manifest some pallor & slight icteric discoloration of the sclerae with splenomegaly. especially those with somewhat more severe • forms of the disease. heavy iron turnover from transfused blood is usually the cause. An affected child’s parents or caregivers may report these symptoms. with elevated indirect bilirubin level. Some patients. which leads to thrombocytopenia & by the high WBC count & immature WBCs seen on the peripheral blood film due to the extreme 23 • activity of the marrow. • • • o o o The ineffective erythropoiesis also creates a state of hypermetabolism associated with fever & failure to thrive. Occasionally. In transfused patients. These changes are caused by massive expansion of the bone due to the ineffective erythroid production. . resulting in a characteristic radiologic picture (see Imaging Studies & the image below). Evidence of hemolysis is usually present. Bleeding tendency. Thalassemia should be considered in any child with • hypochromic microcytic anemia that does not respond to iron supplementation. gout due to hyperuricemia may be encountered. increased susceptibility to infection & organ dysfunction are all associated with iron overload.

Diabetes & thyroid or adrenal disorders have been described in these patients. The clinical expression & severity are subject to numerous factors that may either mask the condition or exaggerate the symptoms. this is due. thyroid disorder. Differential Diagnoses Anemia. depending on how well the disease is controlled. Mean corpuscular volume (MCV) & mean corpuscular Hb (MCH) are significantly low. prominent facial • bones & dental malocclusion. unless the spleen is markedly enlarged. the Hb level is not less than 9 . Chronic Hydrops Fetalis Pyruvate Kinase Deficiency Thalassemia Intermedia Laboratory Studies • o o o o o Laboratory studies in thalassemia include the following – The CBC count & peripheral blood film examination results are usually sufficient to suspect the diagnosis. heart murmur. even in patients whose disease is well controlled by chelation therapy. A shift to the left is also encountered. Leukocytosis is usually present. Complications of severe anemia are manifested as intolerance to exercise. in part. Causes Physical Patients with thalassemia minor rarely demonstrate any physical abnormalities. or other endocrinopathy. Findings include the following : Children who are not receiving transfusions have a physical • appearance so characteristic that an expert examiner can often make a spot diagnosis. Hemoglobin (Hb) evaluation confirms the diagnosis. or even signs of heart failure. In the severe forms of thalassemia. instead. Because the anemia is never severe and. such as frontal bossing. Acute Anemia. leading to a more severe disease. the stigmata of severe untreated • beta thalassemia major included the following : o Severe anemia.10 g/dL. even after excluding the nucleated RBCs. pallor & splenomegaly are rarely observed.7g/dL o Massive hepatosplenomegaly o Severe growth retardation o Bony deformities These stigmata are typically not observed. hypochromic macrocytes that . Severe pallor. unlike thalassemia trait. the findings upon physical examination widely vary. Growth retardation is a common finding. with an Hb level of 3 . in most instances. Patients may develop symptoms that suggest diabetes. slight to moderately severe jaundice & marked • hepatosplenomegaly are almost always present. patients look • healthy. In patients with severe forms of thalassemia. are usually striking. these are rarely the presenting reports.controlled disease as well as those with uncontrolled disease. reflecting the hemolytic process. overload may also demonstrate signs of endocrinopathy caused by iron deposits. Platelet count is usually normal. Any complication they develop is usually due to adverse effects of the treatment (transfusion or chelation). neuropathy or paralysis may result from compression of the spine or peripheral nerves by large extramedullary hematopoietic masses. Bony abnormalities. The WBC count is usually elevated in beta thalassemia major. the Hb level ranges from 2 . In Cooley’s original 4 patients. to miscounting the many nucleated RBCs as leukocytes. but.8 g/dL. reflecting the extreme anisocytosis. Peripheral blood film examination reveals marked hypochromasia & microcytosis. thalassemia major is associated with a markedly elevated RDW. Patients with signs of iron 24 Thalassemias are inherited disorders caused by various gene mutations.• • Poor growth in patients with thalassemia is due to multiple factors & affects patients with well . In patients with severe anemia who are not receiving transfusion therapy.

Complete RBC phenotype. thinning the cortex & causing osteoporosis. which result from the expanding marrow spaces. The liver is clear of iron loading much earlier than the heart. is also elevated. which results in maxillary overbite. MRI & CT scanning are usually used in diagnosing such complications. This is also true in measuring the response to chelation therapy in patients with iron overload. The striking expansion of the erythroid marrow widens the marrow spaces. A newer noninvasive procedure involves measuring the cardiac T2 with cardiac magnetic resonance (CMR). usually disappear when marrow activity is halted by regular transfusions.MRI in the liver & heart. skull & spine become more evident during the second decade of life. cardiac function tests obtained by echocardiography measurements & multiple gated acquisition scan (MUGA) findings were compared to the results of iron measurements on R2 . which is frequently used to monitor the status of iron overload. These changes contribute to the classic “chipmunk facies observed in patients with thalassemia major The classic “hair on end” appearance on plain skull radiographs of a patient with Cooley anemia. prominence of the upper incisors & separation of the orbit. which results from widening of the diploic spaces & observed on plain radiographs. which also suggests that deciding when to stop or reduce treatment based on liver iron levels is misleading. Chest radiography is used to evaluate cardiac size & shape. or echocardiography findings. such as ribs. folic acid level & human leukocyte antigen (HLA) typing are recommended before initiation of blood transfusion therapy. This suggests that cardiac iron overload cannot be estimated with these surrogate measurements. nucleated RBCs. However. MRI & CT scanning can be used as noninvasive means to evaluate the amount of iron in the liver in patients receiving chelation therapy. even in patients whose conditions are well . which could behave clinically like tumors.independent patient with thalassemia. Various iron overload patients were involved in the study that revealed that R2 MRI was strongly associated with HIC (weakly but significantly with ferritin level) & represents an excellent noninvasive method to 25 . the liver iron level. These changes. when the marrow in the peripheral bones becomes inactive while more activity occurs in the central bones. long bones & flat bones. Iron studies are as follows : • • • Serum iron level is elevated. more frequently occur during the second decade of life. Plain radiographs of the long bones may reveal a lacy trabecular pattern. Osteoporosis & osteopenia may cause fractures.represent the polychromatophilic cells. which usually correlates very well with the iron concentration in the liver measured using percutaneous liver biopsy samples & the serum ferritin level. an assessment using serum ferritin levels may underestimate the iron concentration in the liver of a transfusion . Compression fractures & paravertebral expansion of extramedullary masses. Hepatic iron content (HIC) obtained by liver biopsy. Imaging Studies Skeletal survey & other imaging studies reveal classic changes of the bones that are usually encountered in patients who are not regularly transfused. hepatitis screen. This procedure has shown decreased values in cardiac T2 due to iron deposit in the heart. with saturation reaching as high as 80%. may also be sites of major deformities. Unlike liver MRI. CMR does not correlate well with the ferritin level. basophilic stippling & occasional immature leukocytes. the maxilla may overgrow. Changes in the pelvis.controlled. In addition to the classic “hair on end” appearance of the skull. Other bony structures. shown below. The serum ferritin level.

Measurement of urinary excretion of iron after a challenge test of DFO is used to evaluate the need to initiate chelation therapy & reflects the amount of iron overload. Increased iron deposition is usually present in marrow.5 weeks is usually adequate to maintain the pretransfusion Hb level needed. Transfused blood should always be leukocyte poor. This regimen attempts to maintain a pretransfusion Hb level of 9 . Chelation therapy was considered after extensive research & many clinical trials.up care after the initial diagnosis is made.term regular blood transfusions.normal growth & development & extension of patients’ life spans. 10 . allowance of normal or near . as depicted in the image below. Medication). HLA typing is performed for patients for whom bone marrow transplantation is considered. Blood transfusion should be initiated at an early age when the child is symptomatic & after an initial period of observation to assess whether the child can maintain an acceptable level of Hb without transfusion. hepatitis B vaccination (if needed) & hepatitis workup. hearing tests. renal function tests & frequent blood counts are required to monitor the effects of deferoxamine (DFO) therapy & the administration of other chelating agents (see Treatment. especially when the family is at risk of a severe form of disease that may be prevented. Like all patients who require long . less demanding & more cost effective than any of the others. liver & other organs.15 mL/kg packed RBCs (PRBCs) at the rate of 5 mL/kg/h every 3 . liver.evaluate iron overload in the liver & heart & to monitor response to chelation therapy. Iron & folate levels should also be measured. heart & other tissues. elimination of ineffective erythropoiesis & its complications. This workup should include RBC phenotype. Accumulation of transfused iron & its consequences also needed to be monitored. Other Tests • • • The following tests may be indicated : ECG & echocardiography are performed to monitor cardiac function. one seems practical. Procedures Bone marrow aspiration is needed in certain patients at the time of the initial diagnosis to exclude other conditions that may manifest as thalassemia major. Iron therapy should not be used unless a definite deficiency is confirmed & should be discontinued as soon as the potential hemoglobin (Hb) level for that • Several blood transfusion regimens have been introduced. Histologic Findings individual is reached. In the untreated person with severe disease. extramedullary hematopoiesis in unusual anatomic sites is one of the known complications. 26 . Of these. patients with thalassemia require a pretransfusion workup.monitored chelation therapy has become the standard therapy & has drastically changed the outlook for this population of patients. Patients with severe thalassemia require medical treatment & a blood transfusion regimen was the first measure effective in prolonging life. Erythroid hyperplasia is observed in bone marrow specimens. including reversal of the complications of anemia. Blood transfusions All severe forms of thalassemia exhibit hyperactive marrow with erythroid hyperplasia & increased iron stores in marrow.9. Counseling is indicated in all persons with genetic disorders. Medical Care • • Patients with thalassemia traits do not require medical or follow . Eye examinations. it was found to provide patients with many benefits. Today. regular blood transfusion combined with well .5 g/dL at all times. Liver biopsy is used to assess iron deposition & the degree of hemochromatosis. In the process of experimenting with blood transfusion.

For this reason. intravenously. It means transfer of normal gene in stem cells to correct the underlying defect.• • Consider administration of acetaminophen & diphenhydramine hydrochloride before each transfusion to minimize febrile or allergic reactions. Why patients with excessive iron absorb large amounts of iron from the GI tract is not clear. the efficacy of which remains to be demonstrated. Some of the hemolysis in this population was attributed to peroxidation of the RBC membrane lipids by an iron . mainly because of the extreme demand associated with the severe expansion of the marrow. it must be administered parenterally. production of apoferritin is accelerated to provide means for storing iron in nontoxic forms as ferritin or hemosiderin. Because of its short half life. deferoxamine.Vitamin E deficiency has been reported in patients with severe thalassemia. subcutaneous infusion must be prolonged if it is to achieve the stated goal. Folic acid deficiency . As iron accumulates & exceeds body needs. for this reason it is administered orally once daily. the nontransferrin . patients nevertheless receive large amounts of iron with each blood transfusion. Several chelating agents have been tested. When administered in conjunction with blood transfusion regimens. vitamin E is expected to decrease cell toxicity. even prevent its occurrence.life. it targets the labile pool.This therapy is an attractive therapeutic modality. although many failed. whether intramuscularly. The introduction of chelating agents capable of removing excessive iron from the body has dramatically increased life expectancy. Several studies in the last few years have shown that this agent is as effective as its predecessor. in reducing ferritin level & tissue iron accumulation. Because the agent is not absorbed in the gut. DFO 27 • • Chelation therapy • • • • • is a complex hydroxylamine with high affinity for iron. Oral chelating agents have been in use in other countries for some time & newer ones are showing efficacy & some specificity for removing iron more efficiently from certain organs than DFO. patients with thalassemia major who received only transfusion therapy could not survive beyond adolescence.mediated free radical effect. Deferasirox is a relatively new oral chelating agent with a long half .40 mg/kg/d is infused over 8 . Surgical Care . and. chelation can delay the onset of cardiac disease and. largely because of cardiac complications caused by iron toxicity. Vitamin E deficiency . Iron overload • • • • Even though blood transfusion is supposed to decrease the excessive iron absorption in the GI tracts of patients with thalassemia. A total dose of 30 . Measuring the ferritin level in the first few years after the diagnosis of thalassemia is usually helpful in detecting iron overload status because ferritin correlates well with total body iron burden at this time. one particular agent was proven effective & safe. Until recently. • Complications of blood transfusion The major complications of blood transfusions are those related to transmission of infectious agents or the development of iron overload.12 hours during the child’s sleep for 5 d/wk by a mechanical pump. Gene therapy . As an antioxidant. Patients with documented transfusion reactions may benefit from having RBCs washed with saline or from receiving deglycerolized RBCs.bound iron (free pool) & the ferritin generated from reticuloendothelial iron. or subcutaneously.This deficiency is a common complication in patients with thalassemia. in some patients. folic acid (1 mg/d) has been recommended as a supplement for this patient population.

chelation therapy. Deferoxamine mesylate Required for DNA synthesis. Deficient in most patients with chronic hemolysis. therefore in great demand in these patients because of increased cellular turnover. heart failure. 3 mg/kg/d PO administered with SC deferoxamine infusion Alpha . Protects polyunsaturated fatty acids in membranes from attack by free radicals & protects RBCs against hemolysis. thereby protecting the rest of the body from this iron.e. The spleen acts as a store for nontoxic iron.40 mg/kg/d SC infusions through infusion pump over 8 12 h. After blood transfusion : 1 . The spleen also increases RBC destruction & iron distribution (i. 15 mg/kg/d PO (range 10 . small doses of ascorbic acid (vitamin C) & alpha . or Hb. scavenger function). 1 mg/d PO Hydroxyurea Chelates iron from ferritin or hemosiderin but not from transferrin. 20 . Diet Deferasirox Tab for PO susp.Not established • > 2 years .Administer as in adults • Ascorbic acid Delays conversion of transferrin to hemosiderin. PO iron chelation agent demonstrated to reduce liver iron concentration in adults & children who receive repeated RBC transfusions. Early removal of the spleen may be harmful (liver cirrhosis has occurred in such individuals). transfusion of about 100 mL/kg packed RBCs [about 20 U for patient weighing 40 kg] & serum ferritin level consistently > 1000 mcg/L). Iron should not be given & foods rich in iron should be avoided. Drinking coffee or tea has been shown to help decrease absorption of iron in the gut. with emphasis on the following supplements : folic acid.tocopherol A normal diet is recommended.mediated toxicity caused by peroxidation of cell membrane lipids. thus making iron more accessible to chelation.controlled disease are usually fully active.2 g IV at slow rate of 15 mg/kg/h 28 Inhibitor of deoxynucleotide synthesis.tocopherol (vitamin E). Treatment initiation recommended with evidence of chronic iron overload (i. cytochrome. In addition. Activity An antioxidant. 1 IU/kg/d PO Folic acid Patients with well . The spleen is known to contain a large amount of the labile nontoxic iron (i. Demonstrated to be deficient in patients with iron overload receiving chelation therapy.Splenectomy is the principal surgical procedure used for many patients with thalassemia. reducing extent of accompanying hemolysis. not to exceed 35 mg/kg/d Excessive chelation with deferoxamine may cause growth . Another surgical procedure in patients with severe thalassemia on transfusion therapy is the placement of a central line for the ease & convenience of administering blood transfusions. or massive hepatosplenomegaly are usually restricted according to their tolerances.e. Prevents iron . These facts should always be considered before the decision is made to proceed with splenectomy. or both. with recent reports of venous thromboembolic events (VTEs) after splenectomy. storage function) derived from sequestration of the released iron.20 mg/kg/d) initially. one should carefully consider the benefits & the risks before splenectomy is advocated. < 2 years . Patients with anemia. Binds iron with high affinity in a 2 :1 ratio. may increase by increments of 5 mg//kg/d q12wk.e. Approved to treat chronic iron overload due to multiple blood transfusions.

Type 1 DM may result due to autoimmune destruction of the beta cells. Environmental agents that have been believed to induce an attack & infect beta cell function include viruses (e. Somatropin • • • 1) 2) Treatment with insulin is necessary Increased tendency of DKA Family history present in 10% Genetic . Growth hormone may be effective in increasing growth rate in all thalassemic patient particularly the ones with growth hormone deficiency. Coxsackie B4). It is a chronic disease of carbohydrate.These factors may act as modifiers of disease rather than triggers. 0. hepatocytes.g. Exerts activity on specific cell receptors including insulinlike growth factor . Genetic predisposition ↑ . Dietary .18 .7 mg/kg/wk during puberty Depot : 1..retardation.Early introduction of cow milk may be associated Autoimmunity .5 mg/kg/month or 0. endocrinal disease with both biochemical & anatomical consequences. Elicits anabolic & anti . Pathogenesis Causes Human growth hormone produced by recombinant DNA technology (mouse C127 cell line). Autoimmunity is t – cell mediated.0. Phase 1 – phase of clinical symptoms This leads to progressive destruction of b cells. fat & protein metabolism caused by the lack of insulin. mumps. especially in those in their late 30s & early 40s. rubella. but can occur in adults.The etiology of type 1 DM has a strong genetic component. seasonal changes & cytotoxins. Caused due to autoimmune destruction of pancreatic islet cell Onset is acute Irreversible beta cell damage is present. there is low or absent endogenously produced insulin.3 mg/kg/wk SC divided into 6 . At onset of clinical diabetes 80 – 90 % of islet cells are destroyed. adipocytes. It includes 29 ↑ • In type 1 diabetes. toxic chemicals.7 injections. Environmental .75 mg/kg SC q2wk 3) 4) 13 Diabetes millitus • • Types 1) 2) Environmental insults (viral infections) Activation of immunological t & b lymphocytes Inflammatory reaction Auto antibodies against islet cells (autoimmune reaction) Destruction of islet/beta cells IDDM Type 1 (IDDM / juvenile diabetes) Type 2 (NIDDM) • • • • • Genetic predisposition & environmental factors lead to initiation of a autoimmune process against pancreatic islets. lymphocytes & hematopoietic cells. not to exceed 0. ↑ ↑ ↑ Diabetes millitus (DM) is a multisystem.1 (IGF 1). Occurs most commonly in childhood with average age 7 to 15 yrs.catabolic influence on various cells including : myocytes.

Most islet cell antibodies are directed against glutamic acid decarboxylase (GAD) within pancreatic B cells. Approximately 85% of patients have circulating islet cell antibodies & the majority also has detectable anti . polyphagia. nocturia. glucagon (glycogenolysis. those with type 1 DM generally are not obese & may present initially with diabetic ketoacidosis (DKA). Phase 3 – relapse This results from declining endogenous insulin secretion & insulin resistance. Phase 2 – honeymoon phase (remission phase) ketogenesis) Excessive lypolysis. amino acids Ketone body formation (acetone. gh (antagonize insulin action) Cortisol. prevent ketosis. plasma glucagon is elevated & the pancreatic beta cells fail to respond to all insulin . acetoacetic acid) (Metabolic acidosis) DKA Compensatory rapid deep breathing (hyperventilation) to excrete more co2 (kussmaul breathing) Fruity odor to breath (acetone) Ketonuria further dehydration. The pancreas shows lymphocytic infiltration & destruction of insulin . recent weight loss & fatigue. 30 • Type 1 DM is a catabolic disorder in which circulating insulin is very low or absent. Phase 4 – stage of total diabetes Complete beta cell destruction with no capacity for endogenous insulin synthesis or relapse. cerebral O2 Leads to impaired consciousness COMA Regeneration of new is lets. Transient decrease in insulin requirements associated with improved b cell function. gluconeogenesis. Pathophysiology Insulin (Insulin promotes glucose uptake from blood) • Utilization of glucose by muscles Post prandial hyperglycemia • • Glycosuria (when renal threshold exceeds > 180mg/dl) Osmotic diuresis Polyuria Dehydration (loss of water & electrolytes) polydipsia Physiologic (stress leads to release of stress hormones) • • • 1) 2) 3) Epinephrine (impair insulin secretion) Cortisol. causing insulin deficiency.secreting cells of the islets of Langerhans. It is a irreversible state.secretory stimuli. Unlike people with type 2 DM. lypolysis. It triggers the immune system in a genetically susceptible individual to develop an autoimmune response against altered pancreatic beta cell antigens or molecules in beta cells that .insulin antibodies before receiving insulin therapy. One theory regarding the etiology of type 1 DM is that it results from damage to pancreatic beta cells from an infectious or environmental agent. decrease hyperglucagonemia & normalize lipid & protein metabolism. Patients need exogenous insulin to reverse this catabolic condition.polyuria.

Gastrointestinal symptoms : Nausea. such as Graves’s disease. Approximately 95% of patients with type 1 DM have either human leukocyte antigen (HLA) . Acute fatty liver may lead to distention of the hepatic 31 • capsule. nausea & blurred vision. however. In type 2 dm patient does not require insulin for survival but for better glycemic control. which results from many factors. Physical In new cases of diabetes.• • • resemble a viral protein. Nocturnal enuresis : Severe enuresis secondary to polyuria can be an indication of onset of diabetes in young children. Symptoms at the time of the first clinical presentation can usually be traced back several days to several weeks.DR4. physical examination findings are usually normal. Prevalence is increased in patients with other autoimmune diseases. Hashimoto thyroiditis & Addison disease. It is bilateral. or even years. Currently. Polyuria : Polyuria is due to osmotic diuresis secondary to hyperglycemia. HLA DQs are considered specific markers of type 1 DM susceptibility. and. abdominal discomfort or pain & change in bowel movements may accompany acute DKA. beta cell destruction may have started months. except in DKA. Chronic gastrointestinal symptoms in the later stage of diabetes are due to visceral autonomic neuropathy. altering its normal focal length. altered mental status are present. Polyphagia with weight loss : The weight loss with a normal or increased appetite is due to depletion of water & calories due to a catabolic state with reduced glycogen. pancreatitis. autoimmunity is considered the major factor in the pathophysiology of type 1 DM. in a glove & stocking pattern. wherein signs of Kussmaul respiration. polyphagia. depending on the interval between the onset of the disease & initiation of treatment. dehydration. . lassitude. Fatigue & weakness : This may be due to muscle wasting from the catabolic state of insulin deficiency. • • • • • • • • • • Other signs are weight loss. hypotension.DR3 or HLA . causing right upper quadrant pain. hypovolemia & hypokalemia. Peripheral neuropathy : This presents as numbness & tingling in both hands & feet. They should have funduscopic examination for retinopathy & monofilament testing for peripheral neuropathy. Patients may maintain their normal weight or exhibit wasting. In established cases.g. • • • • Clinical manifestations Classical triad – polyuria. polydipsia. before the onset of clinical symptoms. Persistent abdominal pain may indicate another serious abdominal cause of DKA. proteins & triglycerides. including the accumulation of sorbitol in peripheral sensory nerves due to sustained hyperglycemia. low physical activity or hyper caloric diet Obese adults over 30 yrs of age Obesity is associated with insulin resistance but diabetes does not develop until some degrees of insulin secretion failure. e. Kussmaul breathing deep & rapid respiration Muscle cramps : This is due to electrolyte imbalance. 2) Type 2 (NIDDM) • • • • Polygenic disease aggravated by environmental factors. symmetric & ascending neuropathy. Blurred vision : This also is due to the effect of the hyperosmolar state on the lens & vitreous humor. patients should be examined every 3 months for macrovascular & microvascular complications. Glucose & its metabolites cause dilation of the lens. in some cases. Polydipsia Thirst is due to the hyperosmolar state & dehydration.

term steroid use). but is sometimes easier to treat. but this is classified as a diabetes due to a specific cause & not as type 2 diabetes.Genetics There is also a strong inheritable genetic connection in type 2 diabetes : having relatives (especially first degree) with type 2 increases risks of developing type 2 diabetes very substantially.Willi syndrome . Severe complications can result from improperly managed type 2 diabetes. blindness. most likely because adipose tissue (especially that in the abdomen around internal organs) is a (recently identified) source of several chemical signals to other tissues (hormones & cytokines).Thiazide diuretics. insulin resistance is generally “post . obesity.. This is a more complex problem than type 1. short stature & hypogonadism associated with diabetes mellitus (DM) Nondiabetic glycosuria Diagnosis • • • Other Problems to Be Considered • • • • • • • • • . trauma or surgery. glucocorticoids Chronic pancreatitis Cystic fibrosis Prader . Those with first . increasing with the number of those relatives. HTN. or the effects of drugs. phenytoin. Diabetes mellitus with a known etiology.degree relatives with type 2 have a much higher risk of developing type 2. PCOD) Maturity .g. long . acanthosis nigricans (genetic disorders with insulin resistance) Drugs . including coronary artery disease. Addison disease. erectile dysfunction. slow healing wounds (including surgical incisions) & arterial disease. meaning it is a problem with the cells that respond to insulin rather than a problem with the production of insulin. muscular hypotonia. catecholamines. Mode of NIDDM 1) 2) 3) Insulin resistance of skeletal muscles Increased hepatic glucose production Decreased glucose induced insulin production. About 55 percent of type 2 patients are obese at diagnosis.Endocrine tumor causing increased production of growth hormone. glucagon & somatostatin. a rare autosomal dominant condition found increasingly in adolescents. adipose tissue) Endocrine disorders . Insulin resistance means that body cells do not respond appropriately when insulin is present. Type 2 diabetes can causes obesity as an effect of the changes in metabolism & other deranged cell behavior attendant on insulin resistance. including renal failure. or certain types of medications (e. pancreatic insufficiencies. muscles. 32 However.Mental retardation.receptor”.onset diabetes of youth (MODY). The onset of type 2 has been most common in middle age & later life. such as secondary to other diseases. Graves disease. environmental factors (almost certainly diet & weight) play a large part in the development of Type 2 in addition to any genetic component There is a stronger inheritance pattern for type 2 diabetes. although it is being more frequently seen in adolescents & young adults due to an increase in child obesity & inactivity. Examples include diabetes mellitus such as MODY or those caused by hemochromatosis. glucocorticoids. Unlike type 1 diabetes mellitus. Secondary hyperglycemia Disorders of target tissues (liver. Hashimoto thyroiditis. Overweight child (BMI above 85th percentile) Family history in 1st or 2nd degree relative Signs of insulin resistance (acanthosis nigricans. Chronic obesity leads to increased insulin resistance that can develop into Type 2. especially in the early years when insulin is often still being produced internally. is more appropriately called secondary diabetes mellitus or diabetes due to a specific cause. known gene defects. dyslipedemia.

ketones & protein : Urine ketones are not reliable for diagnosing or monitoring DKA. Oral glucose tolerance test (OGTT) with insulin levels : Although this test is usually considered unnecessary to make the diagnosis in type 1 DM. the plasma acetone. or it may be due to increased glucose load on tubules by the elevated glucose filtration rate during pregnancy. Insulin therapy • • • • Insulin injected subcutaneously is the first . however. lispro & aspart insulins are the only types that can be administered intravenously. chronic renal failure).12 deficiency Blood glucose : random blood sugar = 200mg/dl on two separate occasions. Regular.acting insulins include regular insulin.g. At times.peptide level below 5 µU/mL.peptide is formed during conversion of proinsulin to insulin. The reference range for nondiabetic people is 6% in most laboratories. Fanconi syndrome. Islet cell antibodies Thyroxine (T4) & thyroid antibodies Other Tests Laboratory Studies • • • • • • Intravenous glucose test for possible early detection of subclinical diabetes HLA typing may be considered.2 y). specifically.4 principles should be followed – 1) Management of DKA 2) Metabolic control 3) Parenteral education 4) Control of intermittent & long term complications Type 1 diabetes mellitus (DM) patients require insulin therapy to control initial hyperglycemia & maintain serum electrolytes & hydration. intermediate & long . This remission is due to a partial return of endogenous insulin.line therapy in the treatment of type 1 diabetes. or 0. Fasting blood sugar = 126mg/dl Serum electrolytes Urinalysis for glucose.• • Renal glycosuria . Short. suggests type 1.hydroxybutyrate level. Rapid . This glucose may be due to an autosomal genetic disorder or dysfunction of the proximal renal tubule (e.Glucose appears in urine despite normal glucose concentration in blood. in which the disease remits & the patient requires little or no insulin.free period during which patients do not need treatment. Glycated hemoglobin predicts the progression of diabetic microvascular complications. 33 • • • • • C . and. The different types of insulin are based upon their times of onset & durations of action. is a reliable indicator of DKA. Human insulin is less antigenic than previously used animal • derived varieties. which may last for several weeks or months (and sometimes 1 . This honeymoon period follows the initial treatment. A high positive titre of glutamic acid decarboxylase antibodies also suggests type 1 DM. Rather. with the dramatic increase of type 2 diabetes in the young population. Peripheral neuropathy due to alcohol & vitamin B . • Medical Care . . lispro & aspart • insulin. the disease recurs & patients require insulin therapy. Ultimately. To determine whether the individual has type 1 rather than type 2 DM.6 ng/mL.acting insulins are available. an insulin and/or C . the beta . assessment of insulin secretion may become more important. the first incidence of ketoacidosis is followed by a symptom . White blood cell count & blood & urine cultures to rule out infection Glycosylated hemoglobin (Hb) or Hb A1c Hb A1c is the stable product of nonenzymatic irreversible glycosylation of the beta chain of Hb by plasma glucose & is formed at rates that increase with increasing plasma glucose levels.

Balanced diet should be advised Diet management includes education about the timing.acting insulins have slower onsets of action & longer durations of action & are usually administered in combination with faster .hour intervals.5 . Children with hyperglycemia & ketonuria but without acidosis 34 • • • • One of the first steps in managing type 1 DM is diet control.dose regimen).acting insulins to maximize benefits of a single injection. They stimulate proper utilization of glucose by the cells & reduce blood sugar levels. when blood glucose > 250 mg/dL). Its onset of action is 0.0. Therefore.acting insulin alone.operated infusion pump that administers a continuous subcutaneous infusion of rapid acting insulin. it peaks at 2.acting insulin that has no peak & produces a relatively stable level lasting more than 24 hours. The patients with significant proteinuria or a reduced creatinine • clearance should be referred to a nephrologist.to 6 . Lispro insulin is a form of regular insulin that is genetically engineered with the reversal of the amino acids lysine & proline in the B chain. Aspart insulin has aspartic acid substituted for proline in position 28 of the B chain.0. with additional doses of rapid . Diet recommendations should be made in view of the patient’s eating habits & lifestyle. . Diet • • Children with moderate hyperglycemia but without ketonuria or acidosis may be started with a single daily subcutaneous injection of 0.5 U/kg of intermediate . size. Intermediate .acting insulin before each meal (4 . This may increase the risks of hypoglycemia. a newer long . or dehydration may be started on 0.• • • • • Regular insulin is a preparation of zinc insulin crystals in solution.5 h & duration of action is 6 . Initiation of insulin therapy in children Consultations These patients should be referred to an endocrinologist for multidisciplinary management. Both of these insulins are absorbed more quickly & have a rapid onset (5 .1 U/kg of regular insulin at 4 . peak (1 h) & duration (4 h) of action. before meals. Intermediate .acting insulin at bedtime.acting insulins include ultralente insulin.5 . Continuous subcutaneous insulin infusion : This intensive insulin treatment uses a small battery . These patients should have a complete retinal examination by • an ophthalmologist at least once a year. Therefore.monitoring of plasma glucose levels.1 h.7 U/kg of intermediate .monitoring of glucoses before each meal & at bedtime. which contains 30% semilente insulin & 70% ultralente insulin in an acetate buffer.8 h. which contains a mixture of regular & protamine zinc insulin & lente insulin. short & intermediate acting insulins Multiple subcutaneous insulin injections are administered to control hyperglycemia after meals & to maintain normal plasma glucose levels throughout the day.day coverage.acting insulins include neutral protamine Hagedorn (NPH) insulin.acting insulins have the most rapid onsets of action & are used whenever quick glucose utilization is needed (e. containing large zinc insulin crystals in an acetate buffer & insulin glargine. they have the advantage that they may be administered shortly before eating. patients should be well educated about their disease & about self .hour insulin with a single daily injection. Long .10 min).5 . Insulin schedules • • Rapid.& short .acting insulin & subcutaneous injections of 0.3 . Both insulins can supply basal 24 . Rapid . Patients should adjust their daily dosage based on their self . These patients may need additional intermediate .g.or long acting insulin in the morning for all . About 25% of the total daily dose is administered as intermediate ..

e. The minimum protein requirement for good nutrition is 0. When the accumulated ketones exceed the body’s capacity of extracting them. ketonuria) If not treated promptly. Biochemically. Fat intake should be limited to 30% or less of the total calories & a low . Progressive rise of blood concentration of these acidic organic substances initially leads to a state of ketonemia. or composition of meals to avoid hypoglycemia or postprandial hyperglycemia.5 g/kg/d). fat & protein. but a reduced protein intake is indicated in cases of nephropathy. Pathophysiology • • • • • Activity • • • Exercise is an important aspect of diabetes management. DKA usually occurs as a consequence of absolute or relative insulin deficiency that is accompanied by an increase in counter .• • • • • frequency. This type of hormonal imbalance enhances hepatic gluconeogenesis. Respiratory compensation of this acidotic condition results in rapid shallow breathing (Kussmaul respiration). Ketones. but it is not uncommon in some patients with type 2 diabetes. a blood glucose level of greater than 250 mg/dL (although it is usually much higher). in particular beta hydroxybutyrate.9 g/ kg/d (range = 1 .2 & a bicarbonate level of 18 35 • • • • • Diabetic ketoacidosis (DKA) is a complex disordered metabolic state characterized by hyperglycemia. Hepatic gluconeogenesis. ketoacidosis). beta hydroxybutyrate & acetoacetate. Hepatic metabolism of free fatty acids as an alternative energy source (i. Patients should consume sucrose in moderation & increase their fiber intake.1. a recommended distribution consists of 20% of daily calories for breakfast. ketones. Ketones include acetone. midmorning & mid afternoon snacks are important to avoid hypoglycemia. This promotes glucose utilization & increases sensitivity of muscles to insulin.e. mEq/L or less. glycogenolysis secondary to insulin deficiency & counter . they overflow into urine (i.cholesterol diet is recommended. with a drop in pH & bicarbonate serum levels. ketogenesis) results in accumulation of acidic intermediate & end metabolites (i.regulatory hormone excess result in severe hyperglycemia. All patients on insulin should receive a comprehensive diet plan that includes a daily caloric intake prescription. major. Natural body buffers can buffer ketonemia in its early stages. DKA clinically is defined as an acute state of severe uncontrolled diabetes that requires emergency treatment with insulin & intravenous fluids. life threatening complication of diabetes. 30% for dinner & 15% for late evening snack. growth hormone & epinephrine).e. cortisol. Diabetic ketoacidosis • • • • Diabetic ketoacidosis (DKA) is an acute. In some cases. ketoacids). while lipolysis increases serum free fatty acids. more accumulation of organic acids leads to frank clinical metabolic acidosis (i. acetone produces the characteristic fruity breath odor of ketotic patients.e. acidosis & ketonuria.regulatory stress hormones (i. Hyperglycemia usually exceeds the renal threshold of glucose . DKA is defined as an increase in the serum concentration of ketones greater than 5 mEq/L. the caloric distribution is important. blood pH of less than 7. 35% for lunch. In these patients. recommendations for amounts of dietary carbohydrate.e. glycogenolysis & lipolysis. glucagon. & how to divide calories between meals & snacks. Moreover. DKA mainly occurs in patients with type 1 diabetes. induce nausea & vomiting that consequently aggravate fluid & electrolyte loss already existing in DKA. Patients should be encouraged to exercise regularly.

UTI) Medication (e. Symptoms of possible associated intercurrent infection may include fever.e. malaise & arthralgia. pneumonia. ketoacidosis (DKA). Causes 1) Patients with type 1 diabetes • • Among individuals with type 1 diabetes. vomiting) Medical.Characteristic acetone odor to breath Signs of acidosis . lactic acidosis. clozapine) Sepsis. diabetic ketoacidosis is much more common in young children & adolescents than it is in adults.Weak & rapid pulse.g. dysuria. osmotic diuresis. polydipsia) & urination (i. myocardial infarction. abdominal tenderness & disturbance of consciousness Although these signs are not usual in all cases of diabetic 36 • • • • • • 2) Diabetic ketoacidosis (DKA) present at diagnosis of type 1 diabetes due to acute insulin deficiency (occurs in 25% of patients) Poor compliance with insulin through the omission of insulin injections either due to lack of patient or guardian education or as a result of psychological stress. Salicylate Differential Diagnoses Physical • • • Alcoholic Ketoacidosis Hyperosmolar Coma Lactic Acidosis Metabolic Acidosis Pancreatitis. Hyperglycemia. particularly in adolescents Bacterial infection & intercurrent illness (e. possibly. water loss in the urine is increased due to osmotic diuresis induced by glycosuria. dry tongue & skin. hypotension & increased capillary refill time Patient odor . The most characteristic disturbance is total body potassium loss. Acute Other problems to be considered • • Bacteremia & sepsis Dehydration due to gastroenteritis Laboratory Studies Urine . Urine culture helps to identify any possible infecting organisms. UTI. thirst. serum hyperosmolarity & metabolic acidosis result in severe electrolyte disturbances.• • • Age absorption & results in significant glycosuria. . their presence signifies a severe form of DKA.e. Nausea & vomiting usually occur & may be associated with diffuse abdominal pain.This test is highly positive for glucose & ketones by • • dipstick testing. polyuria) are the most common early symptoms of diabetic ketoacidosis (DKA). corticosteroids. and. surgical. or emotional stress Brittle diabetes Idiopathic (no identifiable cause) Insulin infusion catheter blockage Mechanical failure of insulin infusion pump Patients with type 2 diabetes • • Intercurrent illness (e. among others. This incidence of increased water loss results in severe dehydration. coughing. Clinical • • • • • Insidious increased thirst (i. Bacterial Toxicity. Consequently. Signs of dehydration . Generalized weakness & fatigability may occur.Shallow rapid breathing or air hunger (Kussmaul or sighing respiration).g. pentamidine. prostatitis.g. tissue hypoperfusion. Altered consciousness in the form of mild disorientation or confusion is a possible symptom.

low bicarbonate & low pH (< 7. while peaked T wave is observed in hyperkalemia.7. T . Larger volumes of an insulin & isotonic sodium chloride solution mixture can be used. Arterial blood gases (ABG) frequently show typical manifestations of metabolic acidosis. the points that must be considered & closely monitored include correction of fluid loss with IV fluids.wave changes may produce the first warning sign of disturbed serum potassium levels. When treating DKA. Only short .15 U/kg q2h). When the patient becomes euvolemic. • 37 • • . Administer 1 liter over the first 30 minutes.acting insulin is used for correction of hyperglycemia. if available. using IV or IM routes is traditionally preferable.2).dose insulin regimen. The initial insulin dose is a continuous IV insulin infusion using an infusion pump. correction of acid . the physician may switch to half the isotonic sodium chloride solution. several points must be considered.48 hours is always advisable. depending on the degree of dehydration & central venous pressure (CVP) readings. then the rate of infusion decreases to 5 . providing that the infusion dose of insulin is similar.60 U of insulin in 500 mL of isotonic sodium chloride solution at a rate of 50 mL/h). unless the patient is monitored • Administer 1 liter every 4 hours. correction of hyperglycemia with insulin.3 U/h) until the ketoacidotic state abates. Blood culture findings may help to identify any possible infecting organisms.dose insulin regimen has the advantage of not inducing severe hypoglycemia or hypokalemia.• • • • Blood & plasma • • • • The blood glucose level usually is higher than 250 mg/dL. Do not allow the blood glucose level to fall below 200 mg/dL Insulin Other Tests • • • • • • • • • Management • Managing diabetic ketoacidosis (DKA) in an ICU during the first 24 . Fluids . & treatment of concurrent infection if present.base balance. The optimal rate of glucose decline is 100 mg/dL/h. Subcutaneous use of the fast .10% dextrose with half isotonic sodium chloride solution. Low T wave & apparent U wave always signify hypokalemia. Subcutaneous absorption of insulin is reduced in DKA because of dehydration. • Administer 1 liter over the second hour. BUN frequently is increased.Initial correction of fluid loss is either by isotonic sodium chloride solution or by lactated Ringer solution.5 mL/h (2 . particularly potassium loss.1 U/kg/h. particularly if hypernatremia exists. therefore. ECG should be performed every 6 hours during the first day. When blood sugar decreases to less than 180 mg/dL. • Administer 1 liter over the following 2 hours. Serum ketones are present. isotonic sodium chloride solution is replaced with 5 . A low . particularly in those with autonomic neuropathy. When insulin treatment is started. A mix of 24 units of regular insulin in 60 mL of isotonic sodium chloride solution usually is infused at a rate of 15 mL/h (6 U/h) until the blood sugar drops to less than 180 mg/dL. Larger volumes may be easier in the absence of an intravenous infusion pump (e.acting insulin analog (lispro) has been tried in pediatric DKA (0. as may be observed with a high . Electrocardiogram (ECG) This test may reveal signs of acute myocardial infarction that could be painless in patients with diabetes. at a rate of 0.g. correction of electrolyte disturbances.

administer proper antibiotics guided by the results of culture & sensitivity studies. Treatment of concurrent infection • • 14 Cerebral palsy Electrolyte correction Potassium • • • • • • • If the potassium level is greater than 6 mEq/L. birth injuries & stress to the fetus in the uterus are some of the known . Pathophysiology Correction of acid . The primary causes included birth trauma.• • during the first 4 . However there are certain risk factors which increase the chance of the child developing this condition. 100 . In the presence of infection.150 mL of 1. not to starting insulin therapy is advisable unless potassium replacement is underway in order to avoid potentially serious cardiac dysrhythmia that may result from hypokalemia. vision & intellect resulting from a defect or lesion of the developing brain. an orthopedic surgeon.4. It is a disorder of posture & movement often associated with abnormalities of speech. administer 10 mEq/h of potassium chloride. infections during pregnancy in the mother. If the potassium level is 4. administer 20 mEq/h of potassium chloride.5 hours of treatment. asphyxia & prematurity. This mistake usually results in a rebound ketosis derived by counter . This may be repeated every half hour if necessary. Cerebral palsy (CP) is a static encephalopathy. non . Monitoring of serum potassium must continue even after potassium infusion is stopped in the case of (expected) recurrence of hypokalemia.4% concentration is infused initially.5 mEq/L. The condition was first described almost 150yr ago by Little. The other hazard is that rapid correction of hyperglycemia & hyperosmolarity may shift water rapidly to the hyperosmolar intracellular space & may induce cerebral edema. Starting empiric antibiotics on suspicion of infection until culture results are available may be advisable. CP may present from extreme clumsiness (flaccidity) to extensive spasticity.regulatory hormones. It usually develops by 2 . Rebound ketosis requires a longer duration of treatment. Causes • • • • • CP is caused by damage to the developing brain.contagious neurological disease which causes physical disability. Hypoglycemia may develop rapidly with correction of ketoacidosis.base balance The pathophysiology of this condition is uncertain & generally no specific cause is found.6 mEq/L.5 . In severe hypokalemia. If the potassium level is 3 . 38 • • • • • Cerebral palsy is a term which covers a group of non progressive. A common mistake is to allow blood glucose to drop to hypoglycemic levels. • Rapid & early correction of acidosis with sodium bicarbonate may worsen hypokalemia & cause paradoxical cellular acidosis.3 years of age. severe jaundice in the infant. especially when associated with either sepsis or lactic acidosis. Sodium bicarbonate only is infused if decompensated acidosis starts to threaten the patient’s life. do not administer potassium supplement. If sodium bicarbonate is indicated. Monitor serum potassium levels hourly & the infusion must stop if the potassium level is greater than 5 mEq/L. Though most of the causes remain unknown.

000g at birth. It associated with spastic quadraplegia Seizures & epilepsy occurs in 21% Blindness occurs in 11% Medical complications are common : GI problems. metabolic Spastic PVL. foul . Primarily because of intracerebral hemorrhage & periventricular leukomalacia. cord compression. dental problems. Breech presentation 5) Post Natal causes . Convulsions shortly after birth. 7) Behavioral abnormalities. 3) Visual. 000 population 80% factors during antenatal period causing abnormal brain 39 Imaging • • • Epidemiology & etiology 1) 2) Topographic 4) Functional capacity Physiologic classification = the major motor abnormality. Babies born prematurely are at a particular risk. Maternal infections 4) Natal causes . Maternal toxemia.g.Intrauterine exposure to maternal infection (e. umbilical cord inflammation (funisitis). 10% . multicystic Infection. Risk Factors • • • • • • • • • • • Associated problems Mental retardation (IQ < 50) occurs in 30 .• factors.Kernicterus (bilirubin encephalopathy).50% but it should be • devlopment. 6) Cognitive. temperature greater than 38°C during labor & urinary tract infection) is associated with a significant increase in the risk of CP in normal birth weight infants. Prematurity (almost all cerebral palsy babies are premature) Congential infections Birth trauma Hypoxia Chemicals (alcohol. leukomalacia (PVL) infection endocrine. maternal sepsis. Trauma. ischemia.Anoxia. Motor syndrome CNS pathology Major causes Spastic diplegia Periventricular Prematurity. smoking etc. 5) Speech. Particularly those weighing less than 1. asphyxia Prevalence of 2/1. Any severe illness damaging the brain in the 1st year of life can result in CP. 2) 3) Clinical manifestations CP may be classified into • • • mentioned that cerebral palsy patients generally do not have problems with their intelligence. • CP is also associated with developmental disabilities 1) Mental retardation.natal causes . chorioamnionitis.intrapartum asphyxia.to detect structural malformations & vascular abnormalities MRI .Anoxia.. Ultrasound (in preterm babies) . Pre . genetic quadriplegia encephalomalacia Hemiplegia Stroke in utero Infection. inflammation of placental membranes. genetic 1) Physiologic 3) Etiologic categories . Difficult labor.smelling amniotic fluid. P/v bleeding.) Breech deliveries Birth defects Low birth weight babies Twin deliveries Abnormally small head (microcephaly).white matter lesions. Infections 6) Other . • Topographic taxonomy = the involved extremities.identifies risk of cerebral palsy CT . 2) Epilepsy. 7) High risk in low birth weight infants. Asphyxia. aspiration pneumonia. 4) Hearing. Sedatives given to mother.

The first indication of spastic diplegia is often noted when an affected infant begins to crawl. These children usually show toe walking & typical crossed (scissoring) gait. causing an equinovarus deformity of the foot. Severe spastic diplegia is characterized by disuse atrophy & impaired growth of the lower extremities & by disproportionate . CT or MRI at birth in infants with focal seizures often demonstrates the area of infarction. If the Spasticity is severe. in the leg & arm on the same side (hemiplegia). Spastic hemiplegia is more common than spastic diplegia in low birthweight infants. This is further classified into hemiplegic. The child uses the arms in a normal reciprocal fashion but tends to drag the legs behind more as a rudder (commando crawl) rather than using the normal four . Examination of the extremities may show growth arrest. contra lateral to the side of the affected extremities. because this area of the brain influences extremity growth. the feet are held in a position of equinovarus.limbed crawling movement. The arm is often more involved than the leg & difficulty in hand manipulation is Obvious by 1yr of age. 7) A CT scan or MRI may show an atrophic cerebral hemisphere with a dilated Lateral ventricle. especially if the contra lateral parietal lobe is abnormal.Extra pyramidal Bas basal ganglia. Hemiplegic . quadraplegic & diplegic spastic cerebral palsy based on which part of the body it affects. or in all four limbs (quadriplegia). Examination of the child reveals Spasticity in the legs with brisk reflexes. particularly 40 2) 3) 4) 5) Bilateral Spasticity of the legs. An affected child often walks on tiptoes because of the increased tone & the affected upper extremity assumes a dystonic posture when the child runs. a scissoring posture of the lower extremities is maintained. thalamus.arm & leg on same side • Diplegic . & the child walks on tiptoes. putamen Topographic classification Types of CP 1) Spastic Kernicterus.both legs but arms far less • Quadraplegic . particularly in the hand & thumbnail. 6) Ankle clonus & a Babinski sign may be present. Spasticity is apparent in the affected extremities. Walking is significantly delayed. application of a diaper is difficult owing to excessive adduction of the hips. The stiffness may occur in both legs (diplegia). About one third of patients with spastic hemiplegia have a seizure disorder that usually develops during the first year or two & approximately 25% have cognitive abnormalities including mental retardation. B) Spastic diplegia 1) 1) 2) 3) 4) 5) Decreased spontaneous movements on the affected side & show hand preference At a very early age. When the child is suspended by the axillae.whole body • A) Spastic hemiplegia the ankle. Walking is usually delayed until 18–24 mo & a circumductive gait is apparent. the deep tendon reflexes are increased & weakness of the hand & foot dorsiflexors is evident. The main symptom of spastic cerebral palsy is spasticity (muscle overactivity resulting in resistance to stretching). ankle clonus & a bilateral Babinski sign. infection It is the most common type in which the muscles are stiff & weak (due to uncontrolled contraction of the muscles). Intrauterine thromboembolism with focal cerebral infarction may be one cause.

6) C) Spastic quadriplegia 4) prominent. Speech is typically affected owing to involvement of the oropharyngeal muscles. Electroencephalogram (EEG) & CT scan . Management CP is a lifelong condition & cannot be cured. treatment may improve the quality of life by increasing independent 41 . Children with spastic quadriparesis often have evidence of athetosis & may be classified as having mixed CP. 3) Feeding may be difficult & tongue thrust & drooling may be 5) 6) 7) 8) History Physical examination Exclude progressive disorder of the CNS. causes hand tremors which affects reading & writing & can cause speech problems. These children eventually develop spasticity or athetosis. Flexion contractures of the knees & elbows are often present by late childhood.growth with normal development of the upper torso. Neurologic examination shows increased tone & spasticity in all extremities. seizures are uncommon & intellect is preserved in most patients. crawl or walk. This is characterised by involuntary movements & a progression from hypotonia to hypertonia. Generally.to determine the location & extent of structural lesions or associated congenital malformations. brisk reflexes & plantar extensor responses. However. spinal Cord tumor. staying in steady positions such as standing upright or sitting still can be difficult. Associated developmental disabilities. upper motor Neuron signs are not present. Multidisciplinary approach is most helpful in the assessment & treatment of such children. Due to these differences in muscle tone & the involuntary movements. Athetoid CP / extra pyramidal / Dyskinetic CP This generally affects balance.e. particularly in the area where fibers innervating the legs course through the internal capsule. including speech & visual abnormalities are particularly prevalent in this group of children. Sentences are slurred & voice modulation is impaired. or muscular dystrophy. 1) Rare. Ataxic 3) 1) 2) 3) 4) 5) 6) 2) The most severe form of CP because of marked motor impairment of all extremities & the high association with mental retardation & seizures. The child may also show an unusual posture or favor one side of their body. The most common neuro pathologic finding is periventricular leukomalacia. the baby may have delayed rolling over or the baby may be late to sit. Hypotonia (rather than spasticity) may also be present. Diagnosis 1) 2) 3) 4) Patients with this type of CP have bizarre twisting motions or unusual posturing. Tests of hearing & visual function. decreased spontaneous movements. 4) Hypotonic CP These children usually present with low muscle tone & increased floppiness. including degenerative diseases. Swallowing difficulties are common owing to supranuclear bulbar palsies & they often lead to aspiration pneumonia. 2) Affected infants are characteristically hypotonic & have poor head control & marked head lag. especially since the advent of aggressive management of hyperbilirubinemia & the prevention of kernicterus. The prognosis for normal intellectual development is excellent for these patients & the likelihood of seizures is minimal. Parents may first notice that their child is not developing motor milestones normally i.

Medical treatment Muscle relaxants are used to treat the spasticity. drugs 1) Parents should be taught how to handle their child in daily activities such as feeding. Soft tissue surgery 5. consideration should be given to performing surgical soft tissue procedures that reduce muscle spasm around the hip girdle. may increase the movement & strength of the muscles. Prognosis Dantrolene sodium. bathing & playing in ways that limit the effects of abnormal muscle tone. Physical therapy. 12. speech therapy is required. speech synthesizer may be required. including an adductor Tenotomy or Psoas transfer & release. care for the carriers is also important. A Rhizotomy procedure in which the roots of the spinal nerves are divided has produced considerable improvement in selected patients with severe spastic diplegia. help in lengthening a muscle. Patients with severe muscle contractures may require surgery to lengthen the muscle or some spinal surgery to reduce spasticity in the legs Medication may also be given to decrease the muscle spasticity. The treatment not only depends on the patient’s symptoms but also his age. Measures to be taken There are a variety of surgical procedures.mobility is one of the biggest issues in cerebral palsy & this is where the occupational therapist is at the most useful. botulinum toxin injections are available which when used with physical therapy. Spinal cord surgery 6. For children with speech problems. Equipments 4. Severity would include severe quadriplegia. In addition. 2.oesophageal reflux or pneumonias). 9. the Benzodiazepines (Diazepam) & Baclofen. mental Retardation. mental retardation & medical complications (gasto . A variety of mechanical aids like braces. 10. dressing. Patients with incapacitating athetosis occasionally respond to Levodopa & children with dystonia may benefit from Carbamazepine or Trihexyphenidyl. eye. 42 1. Exercise 3. In any chronic disabling condition. Baclofen pumps are also available to control spastically for a longer period of time. A tight heel cord in a child with spastic hemiplegia may be treated surgically by tenotomy of the Achilles tendon. behavior. epilepsy.mobility. carrying. Other interventions Mobility aids are very important . especially a tight Achilles tendon. the chances of reaching the age of 20 yrs drops to 40%. Finally. Handling. 8. including • • If a patient has marked spasticity of the lower extremities or evidence of hip dislocation. Occupational therapy may be required to develop skills to carry out day to day activities at home. the people looking after the patient are at a higher risk of developing psychological but also physical health problem. There is no proof that physical or occupational therapy prevents development of CP . There are also a variety of injections which can be given to relieve muscle spasticity in specific muscle groups (such as botulinum toxin or phenol). which consists of special exercises. 11. Some children with learning disabilities & mental retardation may require special learning programs. UTI. Communication. Motorized wheel 7. Intrathecal Baclofen – used in selected children with severe spasticity. Surgery This is incredibly variable. 2) They also need to be instructed in the supervision of a series of exercises designed to prevent the development of contractures. medications may be required to control the seizures. Now. Mild forms of the disease will mean that the patient probably has a normal life expectancy but at its most severe.

Classic vitamin K deficiency bleeding usually occurs after 24 hours & as late as the first week of life.tubercular drugs [e. modified typewriter & customized seating arrangements. such as walkers. Behavior problems interfere with the development of a child with cp.onset vitamin K deficiency bleeding usually occurs during first 24 hours after birth. Early . it can occur during first month 15 In the past. phenytoin. Communication skills may be enhanced by the use of bliss symbols. Parents should seek treatment for a baby who is jaundiced at birth or has prolonged jaundice within a few days after birth. however. The mechanisms by which anticonvulsant & anti . It is seen in infants born to mothers taking anticonvulsant or anti . the term hemorrhagic disease of the newborn was used to describe bleeding disorders in neonates associated with a traumatic birth or hemophilia.g. vitamin K antagonists [e. Usually the disease occurs from the second day of life to the end of the first week. anti . Numerous maternal medications and/or exposure to toxins during pregnancy are associated with vitamin K deficiency bleeding in neonates (e. especially if vitamin K supplementation was not provided during pregnancy. Serious hemorrhagic complications can occur in this type of hemorrhage. Classic vitamin K deficiency bleeding is observed in infants who have not received prophylactic vitamin K at birth. but limited studies suggest that vitamin K deficiency bleeding is a result of vitamin K deficiency & can be prevented by administration of vitamin K to the mother during the last 2 . phenprocoumon]). The proper diagnostic term that has been adopted is currently vitamin K deficiency bleeding because vitamin K deficiency is not the sole cause of hemorrhagic disorders in preterm & term infants.25 .g. vitamin K deficiency bleeding is usually classified by 3 distinct time periods after birth. anticonvulsants [e. carbamazepine]. Lower urinary tract dysfunction should receive prompt assessment & treatment. initial hemorrhage.4 weeks of pregnancy. special feeding devices. warfarin.g.in the initial assessment. isoniazid]. poles & standing frames. Vitamin K supplementation given after the birth for early onset vitamin K deficiency bleeding may be too late to prevent this disease. rifampin.onset vitamin K deficiency bleeding in the newborn • • • • • Prevention The pregnant mother should get regular antenatal care. barbiturates. but ample evidence shows that therapy optimizes the development of an abnormal child.1. Blood tests of mothers should be checked to detect Rh factor.tuberculosis medications cause vitamin K deficiency bleeding in neonates is not clearly understood. Although some controversy surrounds postnatal timing of the 43 Classic vitamin K deficiency bleeding in the newborn • • • • . as follows : Early . Children with spastic diplegia are treated with equipment.7 cases per 100 births. talking typewriters & specially adapted computers including artificial intelligence computers to augment motor & language function.tuberculosis medication.3) 6) 7) 8) 9) 10) 11) in infants at risk or that it corrects the neurologic deficit. The incidence of classic vitamin K deficiency bleeding ranges from 0. Learning & Attention deficit disorders & Mental retardation managed by a psychologist & educator. Strabismus.g. Nystagmus & optic atrophy –consult with ophthalmologist . assistance of a psychologist or psychiatrist. Quadriplegia is managed with motorized wheelchairs.

• •

& sometimes overlaps with late - onset vitamin K deficiency bleeding. Infants who have classic vitamin K deficiency bleeding are often ill, have delayed feeding, or both. Bleeding commonly occurs in the umbilicus, GI tract (i.e. melena), skin, nose, surgical sites (i.e. circumcision), and, uncommonly, in the brain. This usually occurs between age 2 - 12 weeks; however, late onset vitamin K deficiency bleeding can be seen as long as 6 months after birth. This disease is most common in breastfed infants who did not receive vitamin K prophylaxis at birth. Vitamin K content is low in mature human milk & ranges from 1 - 4 mcg/L. Industrial contaminants in breast milk have been implicated in promoting vitamin K deficiency bleeding. More than half of these infants present with acute intracranial hemorrhages.

Pathophysiology

Late - onset vitamin K deficiency bleeding in the newborn

• • • •

Currently, the following 3 forms of vitamin K are known

K1 : Phylloquinone is predominantly found in green leafy vegetables, vegetable oils & dairy products. Vitamin K given to neonates as a prophylactic agent is an aqueous, colloidal solution of vitamin K1. K2 : Menaquinone is synthesized by gut flora. • K3 : Menadione is a synthetic, water soluble form that is no longer used medically because of its ability to produce hemolytic anemia. Coagulation factors II, VII, IX & X & other Gla - proteins (e.g. protein C, protein S, protein Z) also depend on the presence of vitamin K for their activity. The role of Gla proteins is not completely understood. Vitamin K deficiency gives rise to abnormal prothrombin levels; thus, prothrombin does not effectively participate in blood clot formation.
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Newborn infants are at risk of developing vitamin K deficiency & this coagulation abnormality leads to serious bleeding. Transplacental transfer of vitamin K is very limited during pregnancy & the storage of vitamin K in neonatal liver is also limited. This makes the newborn infant vulnerable to hemorrhagic disorders unless exogenous vitamin K is given for prevention of bleeding immediately after birth. Once the infantile gut is colonized with bacterial flora, the microbial production of vitamin K results in a lower risk of infantile vitamin K deficiency bleeding. A gut - related microbial source of vitamin K is particularly important if dietary phylloquinone is restricted. The most common sites of hemorrhage or bleeding are the umbilicus, mucus membrane, GI tract, circumcision & venipuncture sites. Hematomas frequently occur at the sites of trauma (i.e. large cephalohematomas, scalp bruising related to instrumentation used at delivery, and, rarely, intracranial hemorrhage). Placental transfer of vitamin K is very limited & phylloquinone (vitamin K1) levels in umbilical cord blood is very low. The newborn infant’s intestinal tract is relatively sterile & takes some time to colonize with bacteria, which may have a role in synthesizing vitamin K2 (menaquinones). Because Bacteroides species are among the most common bacteria that inhabit the human intestinal tract & because strains such as Bacteroides fragilis synthesize vitamin K, Bacteroides species are more significant in producing human vitamin K in the intestine than Escherichia coli. Breast milk is a poor source of vitamin K (breast milk levels are 1 - 4 ì g/L). The recommended dietary intake of vitamin K is 1 ì g/kg/d. Exclusively breastfed infants have intestinal colonization with lactobacilli that do not synthesize vitamin K; thus, reduced production of menaquinones increases the neonatal risk of developing a hemorrhagic disorder if not supplemented with vitamin K. Formula fed infants have higher fecal concentrations of vitamin K1 because of

dietary intake & significant quantities of fecal menaquinones, reflecting the gut’s microflora. Preterm infants who are receiving total parenteral nutrition (TPN) are not at risk because they are receiving vitamin K via the multivitamin additive to the TPN. Special consideration is needed for very low birth weight infants whose intestinal tract bacterial flora is altered because of multiple courses of broad - spectrum antimicrobials. Once preterm infants are weaned off of TPN, they may develop vitamin K deficiency if they are exclusively fed breast milk.
Mortality / Morbidity

• •

Signs of intracranial hemorrhage include apnea with or without seizures & a shocklike syndrome. Internal hemorrhage of organs other than the brain may be difficult to detect; however, if they are suspected, careful physical monitoring & serial imaging after birth are indicated. Soft tissue hemorrhage is easier to recognize, but sequential measurements of the bleeding into soft tissues or muscle are mandatory. Vitamin K deficiency in the newborn can be present for various reasons Maternal medications that interfere with vitamin K stores or function (e.g. carbamazepine, phenytoin, barbiturates, some cephalosporins, rifampin, isoniazid, warfarin or warfarin like drugs) can result in vitamin K deficiency bleeding in the infant. In addition to breastfeeding, clinical states that are risk factors for late - onset vitamin K deficiency bleeding include : Diarrhea Hepatitis Cystic fibrosis Celiac disease Alpha1 - antitrypin deficiency Short bowel syndrome Intestinal bacterial overgrowth Chronic exposure to broad spectrum antimicrobials Consumption Coagulopathy Von Willebrand Disease

Causes

• •

Intracranial hemorrhage is uncommon in classic vitamin K deficiency bleeding but can be observed in more than 50% of infants with late - onset vitamin K deficiency bleeding. Intracranial hemorrhage is responsible for nearly all mortality & long - term sequelae due to vitamin K deficiency bleeding.
Age


o o o o o o o o

Vitamin K deficiency bleeding is mostly a disease of the newborn but such hemorrhage can occur beyond the neonatal period, especially if conditions such as short gut syndrome, intestinal bacterial overgrowth & certain genetic conditions are present.
History

The maternal history is very important when assessing vitamin K deficiency bleeding (VKDB), especially the medications used during pregnancy, the presence of medical conditions such as short gut syndrome & unusual dietary intakes. Better surveillance during pregnancy & careful medical evaluation of neonate after delivery are essential. Most newborn infants are healthy upon examination, even if early onset bleeding is present; however, intracranial hemorrhage can occur during the birthing process & can lead to severe complications.
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Differential diagnosis Other Problems to Be Considered

Physical

Maternal isoimmune thrombocytopenia Alloimmune thrombocytopenia Hepatobiliary disease Uncommon coagulopathies
Laboratory Studies

A prothrombin time (PT), activated partial thromboplastin time

o

o o

o

(aPTT), fibrinogen levels & a platelet count should be included in the initial workup for vitamin K deficiency bleeding (VKDB) in a newborn. A thrombin clotting time (TCT) is optional. A prolonged PT is usually the first laboratory test result to be abnormal in vitamin K deficiency bleeding; however, no laboratory test result can confirm the diagnosis of vitamin K deficiency bleeding. A direct blood measurement of vitamin K is not useful because levels normally are low in newborns. Levels of protein induced by vitamin K antagonism (PIVKA II) are increased in vitamin K deficiency bleeding, but this test is generally not available outside of research laboratories. Infants with vitamin K deficiency bleeding typically have a prolonged PT with platelet counts & fibrinogen levels within the normal range for newborns. Thrombocytopenia or a prolonged aPTT should prompt workup for other causes of bleeding during the neonatal period. The diagnosis of vitamin K deficiency bleeding is confirmed if administration of vitamin K halts the bleeding & reduces the PT value.

• • • •

coagulopathy. Intravenous (IV) administration of vitamin K has been associated with anaphylactoid like reactions. Fresh frozen plasma may be considered for moderate - to severe bleeding. Life - threatening bleeding may also be treated with prothrombin complex concentrates (PCC). Because the bleeding in classic vitamin K deficiency bleeding usually is not life threatening, a single dose of parenteral vitamin K is sufficient to stop the bleeding & return prothrombin time (PT) values to the reference range. Oral vitamin K has been studied as an alternative & can improve clotting studies & vitamin K levels, but it has not been studied in large randomized controlled trials to determine if this strategy effectively prevents early & late vitamin K deficiency bleeding. Breastfed infants should receive vitamin K supplementation; if mothers refuse prophylaxis, they should be counseled. Because breast milk is not a good source of vitamin K, infants of mothers who refuse prophylaxis & who exclusively breastfeed should have receive oral supplementation of vitamin K. A recent recommendation for oral vitamin K supplementation in term infants suggest weekly administration of 1 mg until age 12 weeks or 2 mg at birth repeated at age 1 week & age 4 weeks, but this recommendation emphasizes a lack of information related to dosing of oral vitamin K in preterm infants. An additional oral dose of 2 mg at age 8 weeks has also been suggested. The best sources are green leafy vegetables, legumes, soybean & olive oils.

Diet

Medical Care

Prevention of vitamin K deficiency bleeding (VKDB) with intramuscular vitamin K is of primary importance in the medical care of neonates. A single dose of intramuscular vitamin K after birth effectively prevents classic vitamin K deficiency bleeding. Conversely, oral vitamin K prophylaxis improves coagulation test results at 1 - 7 days, but vitamin K administered by this route has not been tested in randomized trials for its efficacy in preventing either classic or late vitamin K deficiency bleeding. Immediately administer vitamin K subcutaneously (hold pressure on the site) for any infant in whom vitamin K deficiency bleeding is suspected or who has serious, unexplained neonatal bleeding. IM administration can result in a hematoma because of the •
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Activity

During acute bleeding, the infant with vitamin K deficiency bleeding should be handled with caution until the coagulation profile

returns to normal after vitamin K supplementation. Vitamin K is the mainstay for prevention of & treatment of vitamin K deficiency bleeding (VKDB). Other coagulation factors are rarely needed. Severe bleeding may warrant the use of fresh frozen plasma. No other drugs or treatments are acceptable substitutes for prompt vitamin K dosing. Subcutaneous administration of vitamin K is preferred over the intramuscular (IM) route in symptomatic infants.
Vitamins

• • • • •

prolonged bleeding in a patient. Hemophilia is one of the oldest described genetic diseases
A person with hemophilia does not bleed faster, only bleeds longer

Vitamin K is required to correct the deficiency that defines vitamin K deficiency bleeding. Prophylaxis with IM vitamin K at birth is an effective means of preventing vitamin K deficiency bleeding in the newborn.
Phytonadione K1

Fat - soluble vitamin that promotes the hepatic synthesis of the following clotting factors : prothrombin (factor II), proconvertin (factor VII), plasma thromboplastin component (factor IX) & Stuart factor (factor X).
Pediatric

Prophylaxis at birth : > 32 wk gestation : 0.5 - 1 mg IM Preterm infants < 32 wk gestation : Birth weight > 1000 grams : 0.5 mg I M Birth weight < 1000 grams : 0.3 mg/kg SC/IM Healthy, term & exclusively breast fed infants : 2 mg PO with the first feeding & then at ages 1, 4 & 8

• •

16
Hemophilia

There are two types of hemophilia - Hemophilia A & Hemophilia B. The 2 disorders are considered together because of their similar clinical pictures & similar patterns of inheritance. Hemophilia A results from deficiency or abnormality of clotting factor in the blood called as “factor VIII”. Making up approximately 80% of hemophilia cases, hemophilia A (HA), considered the classic form of the disease, results from a congenital deficiency of factor VIII (FVIII). Hemophilia B results from deficiency or abnormality of another clotting factor called as “factor IX”. Hemophilia B (HB), also called Christmas disease, is a consequence of a congenital deficiency of factor IX (FIX). In 1952, Christmas disease was described & named after the surname of the first patient who was examined in detail. This disease was distinct from hemophilia because mixing plasma from a patient with “true hemophilia” & with plasma from a patient with Christmas disease corrected the clotting time; thus, hemophilia A & B were differentiated. These deficiencies result in an insufficient generation of thrombin by FIXa & FVIIIa complex through the intrinsic pathway of the coagulation cascade. Females are usually carriers (i.e. they have the abnormal gene that causes hemophilia but do not have clinical manifestations). The male children usually manifest/suffer the disease. Sometimes hemophilia occurs due to a fresh mutation in the gene sequence in the child.

Hemophilia is an inherited disorder in which there is inability to form an effective clot caused by deficiencies of clotting factor VIII (FVIII) & factor IX (FIX), respectively & hence causes
47

The following permutations - combinations can occur while inheriting hemophilia 1) Normal Mother + Father with hemophilia

During each pregnancy, the female offspring has 100% chance

activates factor IX in a calcium . 25% chance of a normal male & 25% chance of an affected male. FV III & FI X circulate in an inactive form. (Normal levels are between 50% & 150%). in turn. 25% chance of a carrier female. the resultant clot is stabilized by factor XIIIa & the cross . invasive procedure or surgery.dependent reaction. about once a month.2 to 5%) experience • more bleeds. 2) Carrier Mother + Normal Father spontaneous hemarthrosis & soft tissue bleeding in the absence of precipitating trauma. Severe hemophiliacs (factor level< 1%) bleed spontaneously • usually in joints. Coagulation system Each pregnancy has 25% chance of a female carrier. Activated factor XI. calcium ions & thrombin .linking of adjacent fibrin strands. 25% chance of a normal male & 25% chance of an affected male. the . Hence. 4) Mother with hemophilia + Father with hemophilia During each pregnancy there is 50% chance of an affected female & a 50% chance of an affected male (actual occurrence is rare). Then. factor XIIa converts prekallikrein (PK) to kallikrein & activates factor XI. Carriers of hemophilia are also sometimes found to have factor levels in the Mild range. Mild hemophiliacs (factor levels> 5% & < 50%) bleed only • after injury. factor V & factor XIII. Classification Hemophilia are divided into severe. Therefore. The activation of factor XII can also initiate the extrinsic pathway. sons of hemophiliac fathers & normal mothers are normal. Pathophysiology During each pregnancy. 3) Carrier Mother + Father with hemophilia The role of the coagulation system is to produce a stable fibrin clot at sites of injury. factor Xa (generated through the intrinsic or extrinsic pathways) forms a prothrombinase complex with phospholipids. cause fibrin monomers to form & then polymerize into a meshwork of fibrin. It is characterized by 48 The intrinsic system is initiated when factor XII is activated by contact with damaged endothelium.activated FVIII. moderate & mild diseases depending on the level of functional factor VIII or factor IX. Moderate hemophiliacs (factor levels . factor X is activated on the cell surface.weight kininogen (HMWK). a key enzyme that controls the conversion of fibrinogen to fibrin. 25% chance a female with hemophilia.activated factor Va.molecular . the results of the cleavage of peptides A & B by thrombin. muscles & soft tissues. In the extrinsic system. 5) Mother with Hemophilia + Normal Father During each pregnancy there is 50% chance of a carrier female & a 50% chance of an affected male though the actual occurrence is rare. the conversion of factor X to factor Xa involves tissue factor (TF). TF is released from the damaged cells. calcium ions & phospholipid. The clotting mechanism has 2 pathways : intrinsic & extrinsic. fibrinolysis. & calcium ions. Thrombin converts fibrinogen into fibrin & activates FVIII. thrombin . surgery or extreme exertion. there is 25% chance of a normal female. kinin generation & complement activation. usually after trauma. The complex cleaves prothrombin into thrombin & prothrombin fragments 1 & 2. In the common pathway.of being a carrier & the male offspring is always normal. Patients with mild hemophilia have more than 5% factor VIII (FVIII) activity & bleed only after significant trauma or surgery. Fibrinopeptides A & B. activation of factor X involves a complex (tenase complex) of factor IXa. In conjunction with high . these 2 factors cooperate to cleave & activate factor X. Factor IXa & factor XII fragments can also activate factor VII. They usually bleed in the muscles & joints. When activated. factor VII. or thromboplastin.

trauma. Regarding muscles. father with hemophilia & mother who is a carrier). Life . with minimal challenge. hemophilia A) & F IX (i. Look for jaundice. hemarthrosis Direct the examination to identify signs related to spontaneous • or.e. The absence of hemorrhagic manifestations at birth does not exclude hemophilia. Sex Ask about the patient’s family history & bleeding symptoms. In the GI tract. Intracranial hemorrhage is the second most common cause of • death & the most common cause of death related to hemorrhage. dental extraction & he or she may have spontaneous bleeding in soft tissues. Easy bruising may occur at the start of ambulation or primary dentition. and.to . Female patients may have clinical bleeding due to hemophilia if 1 of 3 conditions is present : 1) Extreme lionization (Lyonized females (i. those with unequal inactivation of FVIII or FIX alleles & with hemizygosity of all or part of the X chromosome) may be symptomatic) 2) Homozygosity for the hemophilia gene (e.g. other signs of liver failure (e. Age Male patients with severe hemophilia present at circumcision. result in clinical bleeding. especially the knees & • ankles. bleeding may complicate common GI disorders.tissue • hemorrhages that obstruct airways or damage the internal organs.lack of either of these factors may significantly alter clot formation and.g.e. the disease primarily affects male individuals. In the genitourinary tract. Other life . bleeding in the joints. • Examine the weight . • • • Both forms of hemophilia are sex . in general. Factor Activity % Cause of Hemorrhage Severity.bearing joints. • Intracranial hemorrhage is a life . muscles & other soft tissues.40 Major trauma / surgery Moderate 1-5 Mild . as a consequence. or 3) Turner syndrome (XO) associated with the affected hemophilia gene (on average. gross hematuria may occur.threatening hemorrhages include soft . weight .e. therefore.bearing joints & other joints are affected.linked recessive disorders. Observe the patient’s stature.linked coagulopathies because they are inherited as X . The principal sites of bleeding in patients with hemophilia are as follows : • • • • • For joints. hemophilia B) are located on the long arm of chromosome X. those most commonly affected are the flexor groups of the arms & gastrocnemius of the legs. Factor Activity & Hemorrhage Type Classification Significant deficiency in FVIII or FIX may be evident in the neonatal period & continue through the life of the affected individual.threatening hemorrhage with • a lifetime risk. The patient may have a history of hemarthroses & prolonged bleeding with surgical procedures. Clinical 49 Mild > 5 . only the X chromosome). Female individuals who carry the affected genes usually do not have bleeding manifestations. The genes for both F VIII (i. Iliopsoas bleeding is dangerous because of the large volumes of blood loss & because of compression of the femoral nerve. . Bleeding in the CNS is the leading cause of hemorrhagic death among patients with hemophilia.moderate trauma Severe <1 Spontaneous.threatening hemorrhage is also a significant problem. the large joints for deformities or ankylosis. cirrhosis • from viral infection) & signs of opportunistic infections in patients who are HIV seroconverted.

as well as chronic arthritic & muscular damage & deformity. Glanzmann thrombasthenia) • Deficiency of other coagulation factors. Any physical activity may trigger bleeding in soft tissues. and 5) The patient’s previous history of desmopressin acetate (DDAVP) use (e. Bleeding time is prolonged in patients with von Willebrand disease but normal in patients with hemophilia. Activity • Other congenital bleeding disorders must be excluded. 3) The patient’s previous treatments with blood products. Differentiation of hemophilia A from von Willebrand disease is possible by observing normal or elevated levels of von Willebrand factor antigen & ristocetin cofactor activity. . Usually. Hemophilia A & hemophilia B are a consequence of a • congenital deficit of FVIII & FIX. These may include the following : Von Willebrand disease (autosomal dominant transmission) • Platelet disorders (e. 4) The presence & possible titers of inhibitors.linked recessive pattern. exercise) can obscure the diagnosis of hemophilia A.e. stress. normal aPTT does not exclude mild or even moderate hemophilia because of the relative insensitivity of the test. • • Ehlers .Causes Both of these disorders are inherited in an X . age. but specific laboratory factor assays can help with the distinction. Numerous mutations are also described in the gene structure. The diagnosis of hemophilia B may be delayed by physiologically low levels of all vitamin K–dependent coagulation factors. respectively. FX. or they may even bleed spontaneously. The genes for FVIII & factor IX (FIX) are located on the long arm of the X chromosome in bands q28 & q27.Danlos Syndrome Factor V Factor VII Factor XI Deficiency Glanzmann Thrombasthenia Other problems to be considered Platelet Disorders von Willebrand Disease Laboratory confirmation of a FVIII or FIX inhibitor is clinically important when bleeding is not controlled after adequate amounts of factor concentrate are infused during a bleeding episode. Bleeding time. the activated partial thromboplastin time (aPTT) is prolonged. prothrombin time & platelet counts are normal.g.g. FVII. FXI. Patients with severe hemophilia can bleed from any anatomic site after negligible or minor trauma.g. FV. the following information should be obtained : 1) The type & severity of factor deficiency. respectively. Severe hemophilia is easily identified with a significantly prolonged aPTT. Differential diagnosis • • • severity of hemophilia. Laboratory Studies • • Medical care • The plasma concentration of FVIII or FIX determines the 50 Before a patient with hemophilia is treated. The defect results in the insufficient generation of thrombin by • the FIXa & FVIIIa complex by means of the intrinsic pathway of the coagulation cascade. however. Prophylactic factor replacement early in life may help prevent bleeding. in mild hemophilia A only) with the degree of response & clinical outcome. i. ABO blood type. Conditions that can increase FVIII levels (e. • or fibrinogen Acquired hemophilia • Differentiating between severe hemophilia A & hemophilia B is almost clinically impossible. 2) The nature of the hemorrhage or the planned procedure.

term control. . 51 • Sepsis is a common. Hospitalization is reserved for severe or life . • propoxyphene & pentazocine. The treatment of hemophilia may involve management of hemostasis.organ dysfunction such as altered mental status. severe sepsis & septic shock without a commonly understood definition. & related allied health professionals. use of factor replacement products & medications. Third . elevated creatinine.threatening bleeds. or immunosuppressive therapy with • cyclophosphamide & prednisone. The advantage of such products is the elimination of viral contamination. The clinical significance of meeting SIRS criteria in the absence of organ dysfunction or shock is still unclear.generation products without any exposure to animal proteins are now available to further decrease this risk. or dental work. such as large soft tissue bleeds. Pain medications are used for acute bleeding or chronic arthritis. Septic shock is defined as persistent hypotension despite adequate fluid resuscitation or tissue hypoperfusion manifested by a lactate greater than 4 mg/dL. Concerns regarding higher incidences of the presence of inhibitor appear to be unwarranted. Bacteremia is defined as the presence of viable bacteria within the liquid component of blood. IVIG. Sepsis is the presence of infection in association with meeting SIRS criteria. dentistry & surgery. oxycodone. • Safe analgesics include acetaminophen. They have lowered the risk of viral contamination. less morbidity & better overall outcome. Recombinant FVIII & FIX are now commercially available. Comprehensive care clinics are supported by evidence of better access to care. Current treatment of patients with hemophilia requires a comprehensive multidisciplinary approach. Management of hemostasis • Many recombinant FVIII concentrates are now available. deadly & often underappreciated disease process in emergency departments. nurses. orthopedics. more often. & hemorrhage related to head injury. have showed some success in achieving long . with specialists in hematology. Avoid all aspirin products.Various FVIII & FIX concentrates are now available to treat hemophilia A & hemophilia B. Clinicians often use the terms sepsis. surgery. management of bleeding episodes. The effectiveness of these products appears comparable to that of plasma .derived concentrates. treatment of patients with factor inhibitors & treatment & rehabilitation of patients with hemophilia synovitis. retroperitoneal hemorrhage. Severe sepsis is defined as evidence of end . • Plasmapheresis. Factor replacement products & medications • • • FVIII products Various products are available for replacement therapy. episode of hypotension. 17 18 Sepsis • • • • Hemostasis is achieved with replacement therapy aimed at correcting the coagulation factor deficiency. or evidence of disseminated intravascular coagulopathy. The basis of sepsis is the presence of infection associated with a systemic inflammatory response that results in physiologic alterations that occur at the capillary endothelial level. Fresh frozen plasma & cryoprecipitate are no longer used in hemophilia A & B because of the lack of safe viral elimination & concerns regarding volume overload. physiotherapists. Bacteremia may be primary (without an identifiable focus of infection) or. social workers.

Pathophysiology Cellular immunity The neonatal neutrophil or polymorphonuclear (PMN) cell. Onset is most rapid in premature neonates. bacteremia is not a necessary ingredient in the activation of the inflammatory response that results in severe sepsis. Although T cells are found in early gestation in fetal circulation & increase in number from birth to about age 6 months.• • • • • • • o o o • secondary (with an intravascular or extravascular focus of infection). GBS. Lastly. neutrophil reserves are easily depleted because of the diminished response of the bone marrow. Pseudomonas.90 days of life & is acquired from the care giving environment.negative staphylococci. The microorganisms most commonly associated with early onset infection include group B Streptococcus (GBS). E coli. respiratory tract. Meeting SIRS criteria is defined by the having at least 2 of the following 4 abnormalities : Temperature higher than 38°C or lower than 36°C Heart rate greater than 90 beats per minute Respiratory rate greater than 20 breaths per minute WBC count higher than 12. Organisms that have been implicated in causing late .onset 52 sepsis syndrome include coagulase . 000/mm3 or lower than 4. Premature & ill infants have an increased susceptibility to sepsis & subtle nonspecific initial presentations. Early onset sepsis syndrome is associated with acquisition of microorganisms from the mother. Candida. Vectors for such colonization may include vascular or urinary • catheters. Neonatal sepsis may be categorized as early or late onset.onset sepsis from invasive microorganisms. other indwelling lines. which is vital for effective killing of bacteria. 000/ mm3 or with more than 10% immature forms (bands) Of course. Serratia. a patient can have either severe sepsis or septic shock without meeting SIRS criteria & conversely. neonatal PMNs are less deformable. with acquisition of the microbe by passage through a colonized birth canal at delivery. SIRS criteria may be present in the setting of many other illnesses. therefore. The infant’s skin. whereas • meningitis & bacteremia are more common in late . or contact from caregivers with bacterial colonization. they are less able to move through the extracellular matrix of tissues to reach the site of inflammation & infection. Escherichia coli. Pneumonia is more common in early onset sepsis.onset sepsis syndrome occurs at 4 .onset sepsis. is deficient in chemotaxis & killing capacity. Also. Late . Acinetobacter & anaerobes. Haemophilus influenzae & Listeria monocytogenes. conjunctivae. Transplacental infection or an ascending infection from the cervix may be caused by organisms that colonize in the mother’s genitourinary tract. coagulase . A delay occurs in the formation of antigen specific memory function following primary infection & . GI tract & • umbilicus may become colonized from the environment. The limited ability of neonatal PMNs for phagocytosis & killing of bacteria is further impaired when the infant is clinically ill. The systemic inflammatory response syndrome (SIRS) as a group of vital signs & a laboratory value that if abnormal may indicate that sepsis physiology is occurring at the microvascular & cellular level. leading to the possibility of late . While sepsis is commonly associated with bacterial infection. especially in the premature infant. Enterobacter.negative Staphylococcus. they require much vigilance so that sepsis can be effectively identified & treated. Staphylococcus aureus. therefore. these cells represent an immature population. Klebsiella.

Neonates who are ill and/or premature are additionally at risk because of the invasive procedures that breach their physical barriers to infection.the cytotoxic function of neonatal T cells is 50 . nasal . IgM levels are generally low at birth. difficult delivery. poor prenatal care. however. low birth weight. Most of the IgG is acquired from the mother during late gestation. however.gamma upon primary stimulation than do adult NK cells. History The physical & chemical barriers to infection in the human 53 The most common risk factors associated with early onset neonatal sepsis include maternal group B Streptococcus (GBS) colonization (especially if untreated during labor). Most of this transfer occurs in late gestation. meconium staining & congenital anomalies. neonates are deficient in memory T cells. The neonate’s ability to generate immunoglobulin in response to antigenic stimulation is intact. Complement protein production can be detected as early as 6 weeks’ gestation. maternal substance abuse. Other factors associated with or predisposing to early onset neonatal sepsis include low Apgar score (< 6 at 1 or 5 min). premature rupture of membranes (PROM). These cells are also functionally immature in that they produce far lower levels of interferon . Although some infants have had complement levels comparable with those in adults. the magnitude of the response is initially decreased. prolonged rupture of membranes. primarily acquired through nonspecific placental transfer from the mother. Late onset sepsis is associated with the following risk factors : prematurity.15% of all neonatal deaths. The neonate may receive immunoglobulin A (IgA) from breastfeeding but does not secrete IgA until 2 . The neonate is also capable of synthesizing immunoglobulin M (IgM) in utero at 10 weeks’ gestation. maternal urinary tract infection. maternal urinary tract infection & chorioamnionitis. the number of these memory T cells increases. central venous catheterization (duration of > 10 d). deficiencies appear to be greater in the alternative pathway than in the classic pathway. rapidly rising with increasing postnatal age. recurrent abortion. In the preterm infant. inflammatory mediators associated with neonatal sepsis may contribute to brain injury & poor neurodevelopmental outcomes. Age Premature infants have an increased incidence of sepsis. Humoral immunity body are present in the newborn but are functionally deficient. however. Immunoglobulin G (IgG) & immunoglobulin E (IgE) may be synthesized in utero. contributing to 13 . the concentration of the various components of the complement system widely varies among individual neonates. Mortality / Morbidity The fetus has some preformed immunoglobulin present. poor maternal nutrition.5 weeks after birth. low socioeconomic status. T Barrier function The mortality rate in neonatal sepsis may be as high as 50% for infants who are not treated. Infection is a major cause of fatality during the first month of life. Natural killer (NK) cells are found in small numbers in the peripheral blood of neonates. thereby stimulating increased IgM production. unless the infant was exposed to an infectious agent during the pregnancy. Skin & mucus membranes are broken down easily in the premature infant. such that lower levels are found with increasing prematurity. maternal fever greater than 38°C. The risk for death or meningitis from sepsis is higher in infants with low birth weight than in full . birth asphyxia. As the neonate is exposed to antigenic stimuli. Response to bacterial polysaccharide antigen is diminished & remains so during the first 2 years of life.100% as effective as adult T cells. At birth. The incidence of sepsis is significantly higher in infants with very low birth weight (< 1000 g). preterm rupture of membranes.term neonates. prematurity.

Severe headache. dyspnea Abdominal & GI infections . edema. sore throat. Infants who are not infected may also demonstrate abnormal WBC counts related to the stress of delivery or several other factors. irregular respirations. sinus pain or tenderness. severe perinatal asphyxia & periventricular or intraventricular hemorrhage. Neurologic signs : Meningitis is the common manifestation of infection of the CNS.Abdominal pain. • diarrhea Pelvic & genitourinary infections . • pleuritic chest pain. The chest radiograph may depict bilateral consolidation or pleural effusions. Disseminated intravascular coagulation (DIC) can occur in infected infants. especially in the preterm infant.tissue infections . cervical or submandibular lymphadenopathy Chest & pulmonary infections .cannula or continuous positive airway pressure (CPAP) use. The infant has an increased glucose requirement because of sepsis. apnea. Neonates with intrauterine pneumonia may also be critically ill at birth & require high levels of ventilatory support. neck stiffness.proven sepsis.Cough (especially if productive). vasodilation & vascular mal distribution. Physical • Metabolic signs : Hypoglycemia. An inquiry should be made about fever onset (abrupt or gradual).Pelvic or flank pain. 54 • • o acidosis & jaundice all are metabolic signs that commonly accompany neonatal sepsis syndrome. duration & maximal temperature. moderate retracting. Normal WBC counts may be initially observed in as many as 50% of cases of culture . Fever is a common feature of sepsis. urgency Bone & soft . 000 may occur in neonatal sepsis in response to the cellular products of the microorganisms. The hallmark of severe sepsis & septic shock are changes that occur at the microvascular & cellular level with diffuse activation of inflammatory & coagulation cascades. vaginal • or urethral discharge. hyperglycemia. Hematologic signs o o • • Thrombocytopenia with counts less than 100.Focal pain or tenderness. cyanosis & grunting may be observed. Tachypnea. metabolic disorders. GI signs : The intestinal tract can be colonized by organisms in utero or at delivery by swallowing infected amniotic fluid. such as respiratory distress syndrome (RDS). The immunologic defenses of the intestinal tract are not mature. Cardiac signs : In overwhelming sepsis. frequency. maternal hypertension. metabolic The clinical signs of neonatal sepsis are nonspecific & are associated with characteristics of the causative organism & the body’s response to the invasion. These nonspecific clinical signs of early sepsis syndrome are also associated with other neonatal diseases. auscultation & palpation in that order to obtain the most information from the examination. altered • mental status. an initial early phase • characterized by pulmonary hypertension. vomiting. nausea. earache. The following localizing symptoms are some of the most useful clues to the etiology sepsis : Head & neck infections . H2 blocker/proton pump inhibitor use & gastrointestinal tract pathology. fluctuance A systematic physical assessment of the infant is best performed in series & should include observation. focal • erythema. Neutropenia is observed in sepsis. intracranial hemorrhage & a traumatic delivery. dysuria. decreased cardiac output & hypoxemia may occur. capillary endothelial leak & dysfunctional utilization of oxygen & nutrients at the cellular level. Tachycardia is a common feature of sepsis & indicative of a Physical • • • .

CRP levels rise secondary to macrophage. Tachycardia is the physiologic mechanism of increasing cardiac output & thus increasing oxygen delivery to tissues. T . tachycardia often persists in sepsis despite adequate fluid repletion. abnormal parenchymal echogenicities. It is considered a sign of organ dysfunction & is associated with increased mortality. an elevated protein level. A CBC count & differential may be ordered serially to determine changes associated with the infection The CSF findings in infectious neonatal meningitis are an elevated WBC count (predominately PMNs). It indicates hypovolemia & the need for intravascular fluid repletion. however. Attention should be paid to skin color & temperature.cell & adipocyte production of interleukin . The decrease in glucose is not reflective of serum hypoglycemia.24 hours of the growth.48 hours.6 (IL . Urine cultures are most appropriate when investigating late onset sepsis. especially if the infection has been present over a period of time. Pallor.reactive protein (CRP). As tissue hypoperfusion ensues. Imaging studies Head ultrasonography in neonates with meningitis may reveal evidence of ventriculitis.6). A lumbar puncture should be part of the evaluation of an infant with suspected sepsis. a depressed glucose level & positive culture results. Tachycardia may also be a result of fever itself. IgM concentration in serum may be helpful in determining the presence of an intrauterine infection. Skin is often warm in early septic shock as peripheral dilation & increased cardiac output occur (warm shock). grayish. The physical examination should first involve assessment of the patient’s general condition.• • • systemic response to stress. Blood & CSF cultures are appropriate for early onset & late onset sepsis. . Levels of C . extracellular fluid & chronic changes. cerebrospinal fluid (CSF) & urine cultures Bacterial culture results should generally reveal the organism of infection within 36 . or mottled skin are signs of poor tissue perfusion seen in septic shock. an acute phase protein associated with tissue injury. including an assessment of airway. subsequent initial identification of the organism occurs within 12 . the respiratory rate also increases in order to compensate for metabolic acidosis. Hypoglycemia Hypoplastic Left Heart Syndrome Meconium Aspiration Syndrome Laboratory Studies Urinary Tract Infection • • • • • • • Differential diagnosis Metabolic Acidosis Necrotizing Enterocolitis Bowel obstruction in the Newborn Osteomyelitis Coarctation of the Aorta Congenital Diaphragmatic Hernia Congenital Lung Malformations Persistent Pulmonary Hypertension Newborn Congenital Pneumonia Pulmonary Hypoplasia Congestive Heart Failure Pulmonary Sequestration Hemolytic Disease of Newborn Respiratory Distress Syndrome Hemorrhagic Disease of Newborn 55 • • Blood. Altered mental status is a common feature of sepsis syndromes. Stimulation of the medullary ventilatory center by endotoxins & other inflammatory mediators has been proposed as a cause. are elevated at some point in 50 90% of infants with systemic bacterial infections. breathing & circulation (ABCs) & mental status. Increased respiratory rate is a common & often underappreciated feature of sepsis.

vital signs. Trimethoprim . gentamicin. Feeding may be restarted via a nasogastric tube with (EBM) extracted breast milk for the infant with serious compromise.related toxicity & adequate serum & cerebrospinal fluid (CSF) concentration. These infants may also require an antimicrobial that has better penetration of the blood . parental contact is important. Activity The infant with temperature instability needs thermoregulatory support with a radiant warmer or incubator. feeding intolerance. breast milk is the enteral diet recommended by the AAP. erythromycin & piperacillin.brain barrier to achieve therapeutic drug concentrations in the CSF. The choice of antibiotic agents should be based on the specific organisms associated with . Begin antibiotics as soon as diagnostic tests are performed. resistance by gram .negative bacteria. Some of the antibiotics commonly used to treat neonatal sepsis syndrome include ampicillin. Ceftriaxone displaces bilirubin from serum albumin & should be used with caution in infants with significant hyperbilirubinemia. Once the infant is stable from a cardiopulmonary standpoint. This provides coverage for gram . Medical Care • When neonatal sepsis is suspected. especially group B Streptococcus (GBS) & gram . vancomycin. o Meningitis complicated by seizures or persistent positive cultures may require extended IV antimicrobial therapy. The need for blood product transfusion including packed RBCs (PRBCs). The specific antibiotics to be used are chosen on the basis of maternal history & prevalent trends of organism colonization & antibiotic susceptibility in individual nurseries. protein. however.spectrum penicillin antibiotic therapy.positive organisms.sulfamethoxazole has been shown to be effective in the treatment of highly resistant bacterial meningitis. IVIG infusion has been a possible therapy for neonatal sepsis to provide type . or poor feeding.negative organisms has occurred with their use.sulfamethoxazole should 56 • not be used if hyperbilirubinemia & kernicterus are of concern in the newborn. cefotaxime. Monitoring of blood pressure. minerals & electrolytes is adequate during this period. The current approach to treat early onset neonatal sepsis • syndrome includes combined intravenous (IV) aminoglycoside & expanded . Chloramphenicol or trimethoprim . The infant with sepsis may require treatment aimed at the overwhelming systemic effects of the disease. o Aminoglycosides & vancomycin both have the potential to produce ototoxicity & nephrotoxicity & should therefore be used with caution. hematocrit. platelets & fresh frozen plasma (FFP) is not uncommon. such as E coli. Diet The neonate may need to be given nothing by mouth (NPO) during the first days of treatment because of gastrointestinal symptoms. Cardiopulmonary support & intravenous nutrition may be required during the acute phase of the illness until the infant’s condition stabilizes. metronidazole. Consider parenteral nutrition to ensure that the patient’s intake of calories. o Cephalosporins are attractive in the treatment of nosocomial infection because of their lack of dose .Serially.specific antibodies to improve opsonization & phagocytosis of bacterial organisms & to improve complement activation & chemotaxis of neonatal neutrophils. o Infants with bacterial meningitis often require different dosages of antibiotics & longer courses of treatment. head ultrasonography can reveal the progression of complications. platelets & coagulation studies is vital. For most infants. treatment should be initiated immediately because of the neonate’s relative immunosuppression.

For these reasons. releasing iron & carbon monoxide. Bilirubin is produced in the reticuloendothelial system as the end product of heme catabolism & is formed through oxidation reduction reactions.soluble biliverdin is reduced to bilirubin. In the first oxidation step. In most infants. Approximately 75% of bilirubin is derived from hemoglobin. This free bilirubin is able to cross lipid containing membranes. Medical theses. Ligandin concentrations may be increased by the administration of pharmacologic agents such as phenobarbital. Z). which can be cause for concern because unconjugated bilirubin is neurotoxic & can cause death in newborns & lifelong neurologic sequelae in infants who survive (kernicterus). water . sensitivities of the bacterial agent & prevalent nosocomial infection trends in the nursery. Binding to other proteins & erythrocytes also occurs. where it binds to ligandin.limiting step in the process. the rate . biliverdin is formed from heme through the action of heme oxygenase. Pathophysiology Neonatal physiologic jaundice results from simultaneous occurrence of the following 2 phenomena : Bilirubin production is elevated because of increased • breakdown of fetal erythrocytes. the enzyme 57 responsible for binding bilirubin to glucuronic acid. However. Several of these texts also describe a lethal course in infants who probably had Rh isoimmunization. . Ligandin concentrations are low at birth but rapidly increase over the first few weeks of life. apparently by passive diffusion & excretion of bilirubin from the fetus occurs primarily through the maternal organism. leading to neurotoxicity. whereas carbon monoxide is excreted through the lungs & can be measured in the patient’s breath to quantify bilirubin production. essays & textbooks from the 18th & 19th centuries contain discussions about the causes & treatment of neonatal jaundice. in some infants.sepsis. such as certain drugs. the presence of neonatal jaundice frequently results in diagnostic evaluation. unconjugated bilirubin is transported in the plasma tightly bound to albumin. The yellow coloration of the skin & sclera in newborns with jaundice is the result of accumulation of unconjugated bilirubin. thus making bilirubin water soluble (conjugation). Viral infections such as herpes & fungal infections can masquerade as bacterial infections. The presence of endogenous & exogenous binding competitors. Neonatal jaundice may have first been described in a Chinese textbook 1000 years ago. also decreases the binding affinity of albumin for bilirubin. but degradation of myoglobin. Hepatic excretory capacity is low both because of low • concentrations of the binding protein ligandin in the hepatocytes & because of low activity of glucuronyl transferase. bilirubin is transported into liver cells. Uptake of bilirubin into hepatocytes increases with increasing ligandin concentrations. because of the intramolecular hydrogen bonds. serum bilirubin levels may excessively rise. Orth first described yellow staining of the brain. free bilirubin crosses the placenta. including the blood . Binding of bilirubin to albumin increases postnatally with age & is reduced in infants who are ill. In fetal life. is almost insoluble in water in its most common isomeric form (bilirubin IX α Z. Next. In 1875. but the physiologic role is probably limited. The iron is conserved for reuse. A minute fraction of unconjugated bilirubin in serum is not bound to albumin. in a pattern later referred to as kernicterus. which. 19 Background Jaundice is the most common condition that requires medical attention in newborns. unconjugated hyperbilirubinemia reflects a normal transitional phenomenon. Because of its hydrophobic nature. This is the result of the shortened lifespan of fetal erythrocytes & the higher erythrocyte mass in neonates. When it reaches the liver. cytochromes & catalase also contributes.brain barrier.

conjugation. Combination of the OATP . Rh. the observation of jaundice in some infants with hypertrophic pyloric stenosis may also be related to a Gilbert . Bilirubin conjugation is biologically critical because it transforms a water . can occasionally become more pronounced. Monoconjugates are formed first & predominate in the newborn. In mother . Such infants have an increased risk of developing jaundice through increased enterohepatic circulation. thus making it available for reabsorption.PD) deficiency.infant dyads who are experiencing difficulties with the establishment of breast feeding. Blood group incompatibilities (e.fold increased risk for developing marked neonatal jaundice. evidence suggests that supplementation with certain breast milk substitutes may reduce the degree of breast milk jaundice (see other therapies).transferase (ligandin) may contribute to higher levels of total serum bilirubin.fold. certain drugs (Phenobarbital.glucuronidases located in the brush border. bilirubin is eventually reduced to colorless tetrapyrroles by microbes in the colon. some interindividual variations in the course & severity of neonatal jaundice may be explained genetically.phosphatase dehydrogenase (G . deconjugation & reabsorption is termed ‘enterohepatic circulation’. This unconjugated bilirubin can be reabsorbed into the circulation. dexamethasone & clofibrate) can be administered to increase UDPGT activity.2 correlates with a 3 .Bilirubin is bound to glucuronic acid (conjugated) in the hepatocyte endoplasmic reticulum in a reaction catalyzed by uridine diphosphoglucuronyltransferase (UDPGT). Although this condition . Water .6 . The process may be extensive in the neonate. As the impact of these genetic variants is more fully understood.solubility allows conjugated bilirubin to be excreted into bile.8 weeks. Data suggest that the risk of breast milk jaundice is significantly increased in infants who have genetic polymorphisms in the coding sequences of the UDPGT1A1 or OATP2 genes. UDPGT activity is low at birth but increases to adult values by age 4 . Thus. This phenomenon is often referred to as breastfeeding jaundice & is different from the breast milk jaundice described below.soluble molecule. In addition.glucuronidase may play a role by uncoupling bilirubin from its binding to glucuronic acid. β . Further.2 gene polymorphism with a variant UDPGT1A1 gene further increases this risk to 22 . often resulting in the development of kernicterus. Interactions between the Gilbert genotype & hemolytic anemias such as glucose . 58 Once excreted into bile & transferred to the intestines. hereditary spherocytosis.S . partly because nutrient intake is limited in the first days of life.g. Although the mechanism that causes this phenomenon is not yet agreed on. Diconjugates appear to be formed at the cell membrane & may require the presence of the UDPGT tetramer. as described above.6 . Historically. some deconjugation occurs in the proximal small intestine through the action of B . increasing the total plasma bilirubin pool. Genetic polymorphism for the organic anion transporter protein OATP . or ABO hemolytic disease also appear to increase the risk of severe neonatal jaundice. Certain factors present in the breast milk of some mothers may also contribute to increased enterohepatic circulation of bilirubin (breast milk jaundice). This cycle of uptake. prolonging the intestinal transit time. Neonatal jaundice. ABO) may increase bilirubin production through increased hemolysis.type variant. although a normal transitional phenomenon in most infants. Rh isoimmunization was an important cause of severe jaundice. development of a genetic test panel for risk of severe and/or prolonged neonatal jaundice may become feasible. inadequate fluid & nutrient intake often leads to significant postnatal weight loss in the infant. excretion.insoluble bilirubin molecule into a water . Infants who have Gilbert syndrome or who are compound heterozygotes for the Gilbert promoter & structural mutations of the UDPGT1A1 coding region are at an increased risk of significant hyperbilirubinemia. However. Studies also suggest that polymorphisms in the gene for glutathione .

Rhesus incompatibility) may clearly contribute to the measurement. Nonimmune hemolytic disorders (spherocytosis. However. Age The risk of significant neonatal jaundice is inversely proportional to gestational age. The possible interaction between such conditions & genetic variants of the Gilbert & UDPGT1A1 genes. Previous sibling with jaundice in the neonatal period.2 weeks of life. Also. applying it to an actual neonate with jaundice is more difficult. An underpinning of physiologic processes & pathological process (e.4 days of life may also require closer scrutiny & monitoring. If a measurement of the height of the mountain is taken when standing on the summit. Death from kernicterus may occur. G . Rh isoimmunization remains common in developing countries. These discoveries also highlight the challenges involved in the common use of the terms physiologic jaundice & pathologic jaundice.has become relatively rare in industrialized countries following the use of Rh prophylaxis in Rh . Consider the following metaphor : Think of total serum bilirubin in neonatal jaundice as a mountain covered by a glacier.PD deficiency) may also cause increased jaundice & increased hemolysis appears to have been present in some of the infants reported to have developed kernicterus in the United States in the past 10 . presentation is on the second or third day of life. because genetic variants in bilirubin metabolism are only exceptionally pursued in the diagnostic work up of infants with jaundice. the amount of rock & the amount of ice that comprise this measurement is unclear. further family history should be explored (see below).g.6 . which are similar to those in female infants. History Presentation & duration of neonatal jaundice • • • • Typically. is discussed above. as well as genetic variants of several other proteins & enzymes involved in bilirubin metabolism.15 years. splenectomy. how much of the measured total value comes from each of these components is unclear. In infants with severe jaundice or jaundice that continues beyond the first 1 . the results of the newborn metabolic screen should be checked for galactosemia & congenital hypothyroidism. their possible contribution to the measured total serum bilirubin is usually unknown. or bile stones in family members or known heredity for hemolytic disorders Liver disease Maternal illness suggestive of viral or other infection Maternal drug intake Delayed cord clamping Family history • • • • • • • 59 Death from physiologic neonatal jaundice per se should not occur. The same is true for many total serum bilirubin values obtained in neonatal jaundice. Sex History of pregnancy & delivery . particularly in countries with less developed medical care systems. the infant’s weight curve should be evaluated. the mother’s impressions as far as adequacy of breastfeeding should be elicited & the stool color should be assessed. Infants who present with jaundice after 3 .negative women. Mortality / Morbidity Risk of developing significant neonatal jaundice is higher in male infants. particularly if the jaundice required treatment Other family members with jaundice or known family history of Gilbert syndrome Anemia. further evaluation is suggested. Jaundice that is visible during the first 24 hours of life is likely to be non physiologic. Although physiologic jaundice is a helpful concept from a didactic perspective. This does not appear to be related to bilirubin production rates.

Neonatal jaundice may be exacerbated in these situations. Brainstem auditory .g.• • • • • • • Birth trauma with bruising Loss of stool color Breastfeeding Greater than average weight loss Symptoms or signs of hypothyroidism Symptoms or signs of metabolic disease (e. or both. Hepatosplenomegaly. decreased excretory capacity secondary to low levels of ligandin in hepatocytes & low activity of the bilirubin .PD) deficiency & other hereditary hemolytic anemias. polycythemia & the presence of bruising or other extravasation of blood. breast milk jaundice & in several metabolic & endocrine disorders. Causes Physical Neonatal jaundice first becomes visible in the face & forehead. but both changes in bilirubin . yellow color is the only finding on physical examination. Incidence is also higher in infants with mutations/ polymorphisms in the genes that code for enzymes & proteins involved in bilirubin metabolism & in infants with homozygous or heterozygous glucose . In the presence of such symptoms or signs.6 .evoked potentials performed at this time may reveal prolongation of latencies. galactosemia) Exposure to total parental nutrition Postnatal history of phototherapy. either in the serum or noninvasively via transcutaneous bilirubinometry. Overt neurologic findings. even in 19th . petechiae & microcephaly may be associated with hemolytic anemia. Decreased clearance of bilirubin may play a role in breast feeding jaundice. sepsis & congenital infections & should trigger a diagnostic evaluation directed towards these diagnoses. Greeks living in Greece have a higher incidence than those living outside of Greece. This cephalocaudal progression is well described. More intense jaundice may be associated with drowsiness. Geography : Incidence is higher in populations living at high altitudes. Jaundice then gradually becomes visible on the trunk & extremities. Risk factors include Race : Incidence is higher in East Asians & American Indians • • • & is lower in African Americans. . Genetics & familial risk : Incidence is higher in infants with siblings who had significant neonatal jaundice & particularly in infants whose older siblings were treated for neonatal jaundice. since blanching reveals the underlying color. decreased amplitudes. or altered cry characteristics. In most infants.conjugating enzyme uridine diphosphoglucuronyltransferase (UDPGT).phosphatase dehydrogenase (G 6 . Combinations of such genetic variants appear to exacerbate neonatal jaundice. The explanation for this phenomenon is not well understood. independent of other factors. This phenomenon is clinically useful because.albumin binding related to pH & differences in skin temperature & blood flow have been proposed. Examples include immune or nonimmune hemolytic anemia. visible jaundice in the lower extremities strongly suggests the need to check the bilirubin level. Pathologic neonatal jaundice occurs when additional factors accompany the basic mechanisms described above. Jaundice disappears in the opposite direction. such as changes in muscle tone. in a significantly jaundiced infant are danger signs & require immediate attention to prevent kernicterus. Identification is aided by pressure on the skin.century medical texts. The potential need for exchange transfusion should not preclude the immediate initiation 60 Physiologic jaundice is caused by a combination of increased bilirubin production secondary to accelerated destruction of erythrocytes. seizures. effective phototherapy should commence immediately without waiting for the laboratory test results (see Laboratory Studies).

a baseline physiologic jaundice most likely occurs.Johnson Syndrome Duodenal Atresia Galactose . Additional studies may be indicated in the following situations : Infants who present with jaundice on the first or after the third day of life Infants who are anemic at birth Infants who otherwise appear ill Infants in whom serum bilirubin levels are elevated enough to trigger treatment Infants in whom significant jaundice persists beyond the first 2 weeks of life . or increased bilirubin production in hemolytic anemias. formulas containing protein hydrolysates have been shown to promote bilirubin excretion. a total serum bilirubin level test is the only one required in an infant with moderate jaundice who presents on the typical second or third day of life without a history & physical findings suggestive of a pathologic process. In these infants. Birthweight & gestational age : Incidence is higher in premature infants & in infants with low birthweight. Data suggest that the difference between breastfed & formula .1 . However. However. which is then exaggerated. increased enterohepatic circulation of bilirubin probably contributes to prolonged jaundice. Usually. when inadequate feeding volume is involved. Measurement of bilirubin fractions (conjugated vs unconjugated) in serum is not usually required in infants who present as described above.fed infants may be less pronounced with some modern formulas.physiologic jaundice. Such conditions include the following : Transcutaneous bilirubinometry can be performed using handheld devices that incorporate sophisticated optical algorithms. Use of some drugs may increase the incidence. both in breast milk & in infants’ serum. Recent data have shown that breast milk jaundice correlates with higher levels of epidermal growth factor.• Nutrition : Incidence is higher in infants who are breastfed or • • • who receive inadequate nutrition. whereas others decrease the incidence. by increased enterohepatic circulation in bowel atresia. for example. Use of such devices has been shown to reduce the need for blood sampling in infants with jaundice. However. bile stasis in choledochal cyst. they cannot be used to monitor the progress of phototherapy. The mechanism for this phenomenon may not be fully understood.Najjar syndrome Arias syndrome Gilbert syndrome Immune hemolytic anemia Nonimmune hemolytic anemia Congenital infections with cytomegalovirus or toxoplasmosis The following studies are indicated in neonatal jaundice : Laboratory Studies Bilirubin measurement may include the following : • Differential Diagnoses Biliary Atresia Breast Milk Jaundice Cholestasis Cytomegalovirus Infection Dubin . Congenital infection • • • • • • • • • • Bowel atresia Hypertrophic pyloric stenosis Choledochal cyst Conjugated hyperbilirubinemia Crigler . Maternal factors : Infants of mothers with diabetes have higher incidence.Phosphate Uridyltransferase Deficiency (Galactosemia) Hemolytic Disease of Newborn Hepatitis B Hypothyroidism • • o o o o o 61 Other problems to be considered Certain conditions may cause non .

it does not distinguish between intrahepatic & extrahepatic cholestasis. Tests for viral and/or parasitic infection : These may be indicated in infants with hepatosplenomegaly.oxidation was believed to be responsible for • the beneficial effect of phototherapy.iminodiacetic acid (HIDA) is indicated if extrahepatic biliary atresia is suspected. Liver function tests : Aspartate aminotransferase (ASAT or SGOT) & alanine aminotransferase (ALAT or SGPT) levels are elevated in hepatocellular disease. as follows : Initially. Peripheral blood film for erythrocyte morphology Reticulocyte count Conjugated bilirubin levels : Measuring bilirubin fractions may be indicated in the circumstances described above. Phototherapy Phototherapy is the primary treatment in neonates with unconjugated hyperbilirubinemia. Radionuclide scanning : A radionuclide liver scan for uptake of hepato . Blood gas measurements : The risk of bilirubin CNS toxicity is increased in acidosis. although bilirubin is bleached through the action of light.affinity binding site.4 days before performing the scan.o o • o o o o o o o o o Infants in whom family. are subject to significant inter laboratory & intra laboratory variation & are generally not a sensitive tool for diagnosing cholestasis unless repeated measurements confirm the presence of an elevated conjugated bilirubin. At the author’s institution. pregnancy. particularly if the rate of rise or the absolute bilirubin concentration is approaching the need for phototherapy : Blood type & Rh determination in mother & infant Direct antiglobulin test (DAT) in the infant (direct Coombs test) Hemoglobin & hematocrit values Serum albumin levels : This appears to be a useful adjunct in evaluating risk of toxicity levels because albumin binds bilirubin in a ratio of 1 : 1 at the primary high . Alkaline phosphatase & γ . petechiae.glutamyltransferase (GGT) levels are often elevated in cholestatic disease. However. Note that direct bilirubin measurements are often inaccurate. provided the infant has received sufficient quantities of milk. other suggested studies may include the following. 62 o o o thrombocytopenia. the process is slow & is now believed to contribute only minimally to the therapeutic effect of phototherapy. . A γ . maternal. This therapeutic principle was discovered rather serendipitously in England in the 1950s & is now arguably the most widespread therapy of any kind (excluding prophylactic treatments) used in newborns. patients are pretreated with phenobarbital 5 mg/kg/d for 3 . Reducing substance in urine : This is a useful screening test for galactosemia. particularly respiratory acidosis. or other evidence of hepatocellular disease. However.GT/ALAT ratio of more than 1 is strongly suggestive of biliary obstruction. Phototherapy is effective because 3 reactions can occur when bilirubin is exposed to light. Thyroid function tests Imaging Studies Ultrasonography : Ultrasonography of the liver & bile ducts is warranted in infants with laboratory or clinical signs of cholestatic disease. or case histories suggest the possibility of a pathologic process Infants in whom physical examination reveals findings not explained by simple physiologic hyperbilirubinemia In addition to total serum bilirubin levels. photo . Medical Care 1) 2) 3) 1) Phototherapy Intravenous immune globulin (IVIG) & Exchange transfusion are the most widely used therapeutic modalities in infants with neonatal jaundice.

isomers means that only 75 . newer phototherapy units. 15Z bilirubin isomers to water .brain barrier. During phototherapy. On the other hand. at equilibrium conditions. should not be able to cross the blood . the 4Z.25% of photo .isomers of bilirubin are excreted in bile and.490 nm) are probably most effective. This distance should not be greater than 50 cm (20 in) & can be less (down to 10 cm) provided the infant’s temperature is monitored. Although green light theoretically penetrates the skin better. light is used in the white. lamps with output predominantly in the blue region of the spectrum (460 . longer wavelengths penetrate better. by virtue of their water . Irradiating a large skin surface area is more efficient than irradiating a small area & the efficiency of phototherapy increases with serum bilirubin concentration.isomers. This process is enhanced by increasing the intensity of light. Fourth.soluble nature. Because green light makes babies look sick & is unpleasant to work in. Phototherapy can be administered in a number of ways. no experimental data support this speculation. some basic principles regarding wavelength & types of light are discussed below with comments & suggestions regarding each system. and. the efficiency of phototherapy depends on the amount of bilirubin that is irradiated. to some extent.6% of the total serum bilirubin concentration. Many older phototherapy units deliver much less energy.life of lumirubin in serum is much shorter than that in E isomers & lumirubin is the primary pigment found in bile during phototherapy. the presence of 20 . wavelength must be considered.response relationship may be observed between the amount of irradiation & reduction in serum bilirubin up to an irradiation level of 30 . This proportion is not significantly influenced by the intensity of light. lumirubin may constitute 2 . the isomer constitutes about 20 . the energy delivered to the infant’s skin decreases with increasing distance between the infant & the light source. it has not been shown unequivocally to be more efficient in clinical use than blue or white light. Large infants & infants who can move away from the circle’s center may receive less efficient phototherapy. Fifth. phototherapy may reduce the risk of bilirubin . turquoise & green wavelengths. Bear in mind when initiating phototherapy that lowering of the total serum bilirubin concentration may be only part of the therapeutic benefit.induced neurotoxicity as soon as the lights are turned on. the ability of light to penetrate skin is also important. • resulting in the formation of lumirubin.80% of the total bilirubin may be present in a form that can enter the brain. blue. which appears to be approximately 6 µW/cm2/nm. At any given total serum bilirubin concentration. Third. Bilirubin absorbs light 63 • primarily around 450 . a dose . The photo . the nature & character of the light source may affect energy delivery. The half . or E. However. Because photo . while ordinary blue fluorescent lamps are probably equivalent to standard . To understand the benefits & limitations of the various approaches. Second. Data have shown that formation of photo isomers is significant after as little as 15 minutes of phototherapy.soluble isomers in which one or both of the intramolecular bonds are opened (E. Z. some at or near the minimally effective level. First. green light has not gained widespread acceptance. Structural isomerization consists of intramolecular cyclization.spectrum blue lamps (special blue) appear to work best. Please note that although theoretically coherent. 15E isomer predominates. In human infants. In practice. Blue fluorescent tubes are widely used for phototherapy. Irradiation levels using quartz halide spotlights are maximal at the center of the circle of light & decrease sharply towards the perimeter of the circle. Thus. E).25% of circulating bilirubin after a few hours of phototherapy.Configurational isomerization is a very rapid process that changes some of the predominant 4Z.460 nm. Narrow .40 µW/cm2/nm. in urine. Z. when properly configured & with the use of reflecting blanket & curtains may deliver light energy above the 40 µW/cm2/nm suggested to be the saturation level. E.

which implies the concurrent use of 2 or 3 phototherapy units to treat the same patient. which may in fact be close to the apparent saturation level of bilirubin photoisomerization. a truly effective treatment was not available.yield phototherapy units. Until the 1940s.white daylight lamps. Drawbacks of fiberoptic phototherapy units include noise from the fan in the light source & decrease of delivered energy with aging and/or breakage of the optic fibers. Thus. Newer phototherapy units provide much higher levels of irradiance. has often been used in the treatment of infants with very high levels of serum bilirubin. Treatment was initiated at lower levels for these infants. autopsy findings & follow . however. Indications for phototherapy • • • The purpose of treating neonatal jaundice is to avoid neurotoxicity. When used as spotlights. At that time.output overhead phototherapy units but not to that of overhead units used with maximal output.up data suggested that immature infants were at risk of bilirubin encephalopathy at lower total serum bilirubin levels than mature infants. Efficiency may be comparable to that of conventional low . as discussed above.immunized infants with severe anemia. appeared to suggest that total serum bilirubin levels greater than 350 µmol/L (20 mg/dL) were associated with increased risk of neurotoxicity. They have a significant blue component in the light spectrum. These units deliver high energy levels. Quartz lamps are also used in single or double banks of 3 . Blue lights may cause discomfort in hospital staff members. As treatment of premature infants became more widespread & increasingly successful during the last half of the 20th century. White quartz lamps are an integral part of some radiant warmers & incubators. However. hyperbilirubinemia. Whether double or triple phototherapy also confers a benefit with the newer units has not been tested in systematic trials.4 bulbs attached to the overhead heat source of some radiant warmers. However. at least in full term infants. efficient phototherapy is accomplished with white lamps. with significantly less energy delivered at the perimeter. Fiberoptic light is also used in phototherapy units. the ability to increase energy delivery by moving lights closer to infants is limited. indications for treatment have been based on clinical studies of infants who developed kernicterus. exchange transfusion is not without risk for the infant & only with the discovery of phototherapy did neonatal jaundice start to become an indication for treatment on a wider scale. relatively low . or hydrops. The energy field delivered by these is much more homogeneous than that of spotlights & the energy output is reasonably high. Use of reflecting materials also helps. Advantages include the following Low risk of overheating the infant • No need for eye shields • Ability to deliver phototherapy with the infant in a bassinet • next to the mother’s bed Simple deployment for home phototherapy • The possibility of irradiating a large surface area when • combined with conventional overhead phototherapy units 64 (double/triple phototherapy) “Double” & “triple” phototherapy. White (daylight) fluorescent tubes are less efficient than special blue lamps. decreasing the distance between infants & lamps can compensate for the lower efficiency. because the lamps are fixed to the overhead heater unit. in developing countries where the cost of special blue lamps may be prohibitive. Once phototherapy was shown to be a rather innocuous . Historical data. but to a limited surface area. The studies that appeared to show a benefit with this approach were performed with old. much of which was derived from infants with hemolytic jaundice. which can be ameliorated by mixing blue & white tubes in the phototherapy unit. Thus. exchange transfusion was shown to be feasible & was subsequently used in the treatment of Rh . the energy field is strongly focused towards the center.

monitoring every 6 . Note that this does not apply to quartz lamps. . the higher the starting serum bilirubin concentration. the lower the serum bilirubin level. since photo energy drops off towards the circle’s perimeter. ensure that the infant is placed at the • center of the circle of light. Key points in the practical execution of phototherapy include maximizing energy delivery & the available surface area. Also consider the following : The infant should be naked except for diapers (use these only • if deemed absolutely necessary & cut them to minimum workable size) & the eyes should be covered to reduce risk of retinal damage.12 hours is probably adequate. Rather. Cover the inside of the bassinet with reflecting material. the infant is monitored for weight loss. At the author’s institution. In infants who are orally fed. Spotlights are probably more appropriate for small premature infants than for larger near .12 hours after discontinuation. Phototherapy probably serves no purpose in an infant who is not clinically jaundiced.up serum bilirubin testing must be individualized. In infants with more moderate elevations of serum bilirubin. In general.3 mg/dL) below the level that triggered the initiation of phototherapy. Indications for prophylactic phototherapy are debatable. therefore. In infants in whom serum bilirubin concentrations are still rising. In general. the more dramatic the initial rate of decline. routine fluid supplementation for infants under phototherapy is no longer recommended. the preferred 65 • • • • fluid is milk because it serves as a vehicle to transport bilirubin out of the gut. phototherapy should result in measurable reductions in serum bilirubin levels within a few hours. fluid loss is not significantly increased by phototherapy. Expectations regarding efficacy of phototherapy must be tailored to the circumstances. Hang a white curtain around the phototherapy unit & bassinet.5 .20 cm if temperature homeostasis is monitored to reduce the risk of overheating. the distance should be no greater than 50 cm (20 in). Observe the infant closely to ensure that the infant doesn’t move away from the high . Fluid intake is adjusted accordingly. Discontinuation of phototherapy is a matter of judgment & individual circumstances must be taken into consideration.treatment. When using spotlights. white • linen works well. lights were turned on at lower serum bilirubin values than those that had triggered exchange transfusion.50 µmol/L (1. Newer data suggest that if temperature homeostasis is maintained. • With fluorescent lamps. Timing of follow . In infants admitted with extreme serum bilirubin values (> 500 µmol/L or 30 mg/dL). urine output & urine specific gravity. monitoring should occur every hour or every other hour. Older data suggested that phototherapy was associated with • increased insensible water loss. the less efficient the phototherapy. In practice. It seems more rational to apply truly effective phototherapy once serum (and skin) bilirubin has reached levels at which photons may do some good. Reductions in serum bilirubin values of 85 µmol/L/h (5 mg/dL/h) have been documented under such circumstances. In infants in whom serum bilirubin concentrations are close to their peak. many clinicians have routinely added a certain percentage to the infant’s estimated basic fluid requirements. a significant reduction of the rate of increase may be satisfactory. Check the distance between the infant’s skin & the light source. phototherapy is discontinued when serum bilirubin levels fall 25 . These simple expedients can multiply energy delivery by several folds. Serum bilirubin levels may rebound after treatment has been discontinued & follow up tests should be obtained within 6 . This distance may be reduced down to 10 .energy area.term infants.

as measured through evaluation of weight curves. a device for measuring the irradiance delivered by the equipment used should be readily at hand. however.up to deliver optimal efficiency. Shield total parenteral nutrition solutions from light as much as possible. but this effect is less pronounced in modern servo controlled incubators. or other blood group incompatibilities that cause significant neonatal jaundice. An increased incidence of patent ductus arteriosus (PDA) has been reported in these circumstances. ABO. elevated cord bilirubin level (> 70 µmol/L or 4. but data suggest that this issue is not as important as previously believed. In addition. Skin blood flow is increased during phototherapy. every time phototherapy is initiated & use this as a tool to focus staff attention on maximizing energy delivery. In an NICU environment.5 mg/dL/h) in the presence of moderate anemia (11 . the following adverse effects & complications have been noted : Insensible water loss may occur. Some recommend this routinely. Generally. Phototherapy may be associated with loose stools. The appropriate treatment of PDA has been reviewed. even in the absence of high serum bilirubin levels. phototherapy is very safe & may have no serious long . the author recommends fluid supplementation tailored to the infant’s individual needs.5 mg/dL). hydrops.strand breakage & other effects on cellular genetic material.term effects in neonates. or both. Intravenous immune globulin 2) In recent years. Care must be taken lest the patches slip & leave the eyes uncovered or occlude one or both nares.• • • • • • Wherever phototherapy is offered as a therapeutic modality. This has been suggested to be mediated by altered melatonin metabolism. In vitro & animal data have not demonstrated any implication for treatment of human neonates. Hypocalcemia appears to be more common in premature infants under phototherapy lights. Therefore. Regular maintenance of the equipment is required because accidents have been reported. The combination of hyperbilirubinemia & phototherapy can produce DNA . intensive phototherapy should . Increased fecal water loss may create a need for fluid supplementation. Early exchange transfusion has usually been performed because of anemia (cord hemoglobin < 11 g/dL). infants exposed to higher levels of ambient light were found to have an increased risk of retinopathy.down) diapers during phototherapy. including burns resulting from a failure to replace UV filters. as was a more moderate rate of increase (> 8 . Concentrations of certain amino acids in total parenteral nutrition solutions subjected to phototherapy may deteriorate. urine specific gravity & fecal water loss. However. the issue of gonad shielding may be moot. because most hospitals use (cut . In fact. the procedure may be indicated in infants with erythroblastosis who present with severe anemia. 3) Exchange transfusion Exchange transfusion is indicated for avoiding bilirubin neurotoxicity when other therapeutic modalities have failed or are not sufficient. IVIG has been used for numerous immunologically mediated conditions. In the presence of Rh.20 µmol/L /h or 1 mg/dL/h) was an indication for exchange transfusion. This assists in configuring the phototherapy set . Retinal damage has been observed in some animal models during intense phototherapy.10 µmol/L/h or 0. redistribution of blood flow may occur in small premature infants.13 g/dL). 66 • • However. Rather than instituting blanket increases of fluid supplements to all infants receiving phototherapy. urine output. covering the eyes of infants undergoing phototherapy with eye patches is routine. Intensive phototherapy is strongly recommended in preparation for an exchange transfusion. or both. A rapid rate of increase in the serum bilirubin level (> 15 . IVIG has been shown to significantly reduce the need for exchange transfusions.

This approach may virtually eliminate neonatal jaundice as a clinical problem.term safety of the drugs must be answered. thus. in certain instances. The baby’s eyes are contaminated during passage through the birth canal from a mother infected with either Neisseria gonorrhoeae or Chlamydia trachomatis. Because this latter intervention causes less interference with the establishment of the breastfeeding dyad. If phototherapy does not significantly lower serum bilirubin levels. Surgical care is not indicated in infants with physiologic neonatal jaundice. The most common procedure used is push pull method through the umbilical vein. Apparently. Phototherapy has minimal side effects. 67 is a form of bacterial conjunctivitis contracted by newborns during delivery. its use has not gained wide popularity. interruption of breastfeeding for 24 . exchange transfusion should be performed. Medical treatment The commonly used type of blood should be withdrawn within 72 hrs. Eye drops containing .hemolytic disease. Evidence suggests that the simple expedient of supplementing feeds of breast milk with 5 mL of a breast milk substitute reduces the level & duration of jaundice in breast milk–fed infants. This can be achieved through the use of metal mesoporphyrins & protoporphyrins.48 hours & feeding with breast milk substitutes often helps to reduce the bilirubin level. also known as ophthalmia neonatorum.be performed on an emergency basis in any infant admitted for pronounced jaundice. do not await laboratory test results in these cases. which act by binding bilirubin in the gut. Prophylactic treatment of Rh . inhibition of heme oxygenase does not result in accumulation of unprocessed heme. However. before the treatment can be applied on a wide scale. important questions regarding the long . 20 Neonatal conjunctivitis Classification & external resources A newborn with gonococcal ophthalmia neonatorum. Intravenous immunoglobulin (IVIG) at 500 mg/kg has been shown to significantly reduce the need for exchange transfusions in infants with isoimmune hemolytic disease. whereas the waiting period for laboratory test results & blood for exchange can take hours & could constitute the difference between intact survival & survival with kernicterus. However. The mechanism is unknown but may be related to the way the immune system handles red cells that have been coated by antibodies. A new therapy currently under development consists of inhibition of bilirubin production through blockage of heme oxygenase. however. Oral bilirubin oxidase can reduce serum bilirubin levels. 160 ml/kg blood is usually needed for one exchange transfusion. the author prefers to use this approach rather than complete interruption of breast feeding in most cases. an inducer of hepatic bilirubin metabolism. heme can be directly excreted through the bile. Neonatal conjunctivitis. Other therapies In infants with breast milk jaundice. has been used to enhance bilirubin metabolism. The same may be said for agar or charcoal feeds. phenobarbital. Surgical Care Medications are not usually administered in infants with physiologic neonatal jaundice. but administration of immunoglobulin does not appear to be likely associated with greater risks for the infant than an exchange transfusion. Published experience is still somewhat limited. presumably by reducing enterohepatic circulation. Frozen RBC reconstituted in saline or plasma may also be used.negative women with Rh immunoglobulin has significantly decreased the incidence & severity of Rh . Surgical therapy is indicated in infants in whom jaundice is caused by bowel or external bile duct atresia.

The newborn baby’s closed lids should be thoroughly cleansed & dried. natal & post natal care. Complications Untreated cases may develop corneal ulceration. Infants with chlamydia pneumonitis should be treated with oral erythromycin for 10–14 days. silver nitrate is no longer in common use. The two most common causes are N. but may occur up to 2 weeks after delivery. Streptococcus pneumoniae. Deliveries should be conducted under hygienic conditions taking all aseptic measures. Antenatal measures include thorough care of mother & treatment of genital infections when suspected. Thus. conjunctival cytology samples & culture sensitivity swabs should be taken before starting treatment Chemical ophthalmia neonatorum is a self . Corneal involvement (rare) may occur in herpes simplex opthalmia neonatorum. Cause Non infectious Conjunctiva shows hyperaemia & chemosis. Definition • • Neonatal conjunctivitis by definition presents during the first month of life. However in cases with proved penicillin susceptibility. Gonococcal ophthalmia neonatorum needs prompt treatment to prevent complications.5% erythromycin ointment into the eyes of babies immediately after birth Single injection of ceftriaxone 50 mg/kg IM or IV should be given to infants born to mothers with untreated gonococcal infection. Curative treatment as a rule. Staphylococcus aureus. usually lasting 2–4 days. which may perforate resulting in corneal opacification & Staphyloma formation. Signs & symptoms 3) • • B) • • Prophylaxis needs antenatal. Streptococcus haemolyticus. In contrast. Saline wash hourly till the discharge is eliminated Bacitracin eye ointment four times per day (Because of resistant strains topical penicillin therapy is not reliable. Infectious A) 1) 2) Many different bacteria & viruses can cause conjunctivitis in the neonate. Eye lids are usually swollen. gonorrhoeae & Chlamydia acquired from the birth canal during delivery. In most instances neomycin & chloramphenicol eye drops are used instead. Treatment Chemical irritants such as silver nitrate can cause chemical conjunctivitis. Other agents causing Opthalmia neonatorum include Herpes simplex virus (HSV 2). Postnatal measures include : Use of 1% tetracycline ointment or 0. 68 1) 2) .erythromycin are typically used to prevent the condition. The discharge is usually more watery in nature (mucopurulent) & less inflamed. Natal measures are of most importance as mostly infection occurs during childbirth. It may be infectious or non infectious. conjunctivitis secondary to infection with chlamydia (Chlamydia trachomatis) produces conjunctivitis after day 3 of life.limiting condition & does not require any treatment. If left untreated it can cause blindness. Babies infected with chlamydia may develop pneumonitis at a later stage (range 2 weeks – 19 weeks after delivery). penicillin drops 5000 to 10000 units per ml should be instilled every minute for half Topical therapy should include • • Pain & tenderness in eyeball Conjunctival discharge : purulent. mucoid or mucopurulent depending on the cause. Ophthalmia neonatorum due to gonococci (Neisseria gonorrhoeae) typically manifests in the first 5 days of life & is associated with marked bilateral purulent discharge & local inflammation.

The extent of the shortage will decide the severity of the condition.calorie (energy) malnutrition to include both entities.” Edema is characteristic of kwashiorkor but is absent in marasmus. Patients with protein . Jelliffe suggested the term protein . increased protein & energy requirements. essential fatty acids & trace elements. Marasmus involves 69 • • inadequate intake of protein & calories & is characterized by emaciation. every five minutes for next half an hour & then half hourly till infection is controlled) If the cornea is involved then atropine sulphate ointment should be applied. Preschool . The term Marasmus is derived from the Greek word marasmus. 000 units (for full . Primary malnutrition results from inadequate food intake & secondary malnutrition results from defective absorption or increased nutrient loss Protein . Malnutrition includes both overnutrition & undernutrition but PEM is mainly related to under nutrition. associated with infections. but is deficient in total protein & essential amino acids. If there is a shortage of energy requirements from protein or non .energy malnutrition (PEM) compromises a group of related disorders that include marasmus. which means withering or wasting. especially infantile gastroenteritis may then develop & worsen the malnutrition.protein sources.feeding. For this reason. the child may develop kwashiorkor. It is associated with the early abandonment or failure of breast . 000 units (for premature or low weight babies) IM twice daily for three days (if penicillin is susceptible) Herpes simplex conjunctivitis is usually a self . Topical antiviral drugs control the infection more effectively & may prevent recurrence. QID Cefotaxime 100 – 150 mg/kg/day IV / IM. If a child’s diet has excessive non . Studies suggest that marasmus represents an adaptive response to starvation.3) an hour. immature immune systems causing a greater susceptibility to infection Etiology of PEM • • .term normal weight babies) or 20.energy malnutrition may also have deficiencies of vitamins. The term Kwashiorkor is taken from the Ga language of Ghana & means “the sickness of the weaning.protein calories from starch or sugar. Children may present with a mixed picture of marasmus & kwashiorkor & children may present with milder forms of malnutrition. especially in children. the most common cause of malnutrition is inadequate food intake. it may result in Protein Energy Malnutrition (PEM). Severe inadequacy of energy & nutrients causes total exhaustion from malnutrition. occurring most frequently in infants & young children. • • • • 21 PEM • • • • • • • It includes a range of pathological conditions arising from coincident lack of proteins & calories in varying proportions. • Systemic therapy Neonates with gonococcal ophthalmia neonatorum should be treated for seven days with one of the following regimens Ceftriaxone 75 – 100 mg/kg/day IV / IM.limiting disease. 12 hourly Ciprofloxacin 10 – 20 mg/kg/day / Norfloxacin 10 mg/kg/day Crystalline benzyl penicillin G 50.aged children in developing countries are often at risk for malnutrition because of their dependence on others for food. Worldwide.Kwashiorkor. whereas kwashiorkor represents a maladaptive response to starvation. Infections. kwashiorkor & intermediate states of Marasmic . leading to marasmus.

resulting in emaciation. Serum levels of zinc correlate closely with the presence of edema. tuberculosis leads to anorexia. In kwashiorkor.) & the impaired B . the body draws on its own stores. Marasmus is mainly due to undernutrition of energy.energy malnutrition. when the access to health care is inadequate. Low intake of calories or an inability to absorb calories is the 70 key factor in the development of kwashiorkor. etc. culture & dietetics.e. anxiety & attention deficit may indicate protein . whooping cough. This period is characterized by increased energy requirements & increased susceptibility to viral & bacterial infections. It can be ineffective when the foods introduced provide inadequate nutrients. Causes of low food intake • • • • • • • • • • 2) • • Poverty Ignorance Inappropriate feeding . Pathologic differentiation Adaptation in marasmus 1) Diet Age Kwashiorkor is mainly due to protein malnutrition with relative excess of energy.risk period. adequate carbohydrate consumption & decreased protein intake leads to decreased synthesis of visceral proteins. • slowing of linear growth. Marasmus most commonly occurs in children younger than 5 years. fish Prolonged breast feeding above 2 yrs of age without weaning Breast feeding stopped early i. fever (negative nitrogen balance) . In children. measles. Signs include diarrhea & psychomotor changes. In particular. Impaired synthesis of B . Protein . stunting of growth & severe wasting. decreased social responsiveness. Infections Social factors History • Like pneumonia. Pathophysiology • • • • • • In general. such as irritability.lipoprotein produces a fatty liver. Over diluted top feeds Anorexia due to mental causes & infections.lipoprotein synthesis produces fatty liver. the dermatosis of zinc deficiency. apathy. Women & girl children eat last & the least Restriction of feeds during certain illness. This may signify a catabolic process that requires nutritional intervention. public health. hygiene. Patients with protein . the child is apathetic when undisturbed but irritable when picked up. the findings of poor weight gain or weight loss. Weaning is affected by geography. The resulting decrease in albumin causes dependent edema (swelling of the feet.energy malnutrition can also present • with nonhealing wounds. before 6 mt. The classic mosaic skin & flaky pain dermatosis of kwashiorkor bears considerable resemblance to the skin changes of acrodermatitis enteropathica. Kwashiorkor characteristically affects children who are being • weaned.certain foods are not accepted culturally—meat.& exposure to non hygienic conditions. malaria. and/or when the patient cannot access or purchase proper nourishment. marasmus is an insufficient energy intake to match the body’s requirements. when the food & water are contaminated. As a result. Weaning (the gradual deprivation of breast milk & the commencement of nourishment with other food) occurs during this high . economy. diarrhea. & behavioral changes.energy malnutrition also involves an inadequate intake of many essential nutrients.

Return of social smile is early sign of recovery. CHD congenital heart diseases.Markedly retarded growth Psychomotor changes Oedema Muscles of upper limb are wasted but lower limbs appear swollen Wasting is masked by well preserved subcutaneous tissue & edema. • Metabolically active brown fat is wasted first.term & IUGR These babies tend to be malnourished if proper care is not taken. Secondary to illness Inborn errors of metabolism. Regression of milestones may occur. hepatitis. However. oedema is clinical feature of increased extracellular water volume Hypoalbuminemia Retention of water & sodium due to increased capillary permeability due to infections & hyperkalemia. Occasionally. Delayed milestones may be present. • Buccal fat is last to be wasted. Presence of skin folds over buttocks & thighs. Sunken eyes. gross loss of weight. Diarrhea & dysentery cause malabsorption. thin limbs & large head. Mental changes are due to brain edema. Spectrum of PEM 1) Mild to moderate inconsistent cutaneous findings include fine. electrolyte imbalance. growth retardation & wasting of subcutaneous fat & muscle. • Failure to gain weight & height. Monkey facies /old man/wise man look secondary to a loss of buccal fat pads is characteristic of this disorder.oesophageal fistula. Characterized by emaciation. As nutritional deficiency prolongs for longer period with infections. Marasmus may have no clinical dermatosis.e. bronchiectasis. The skin is xerotic. If ascites is present abdominal T. TOF –tracheo . Free radical induced damage to cell membranes. flabby & hypotonic muscles With marked loss of subcutaneous fat & muscle wasting. marasmus & kwashiorkor. wrinkled & loose. 71 Causes 1) 2) 3) • • • • • .energy malnutrition. B & liver dysfunction must be ruled out Mental changes – previously irritable child becomes lethargic. growth hormone deficiency. neurotransmitter imbalance. brittle hair. 4) 5) Pre . & fissuring of the nails. • Child is generally alert but irritable & has voracious appetite. alopecia. marked growth of lanugo hair is noted. impaired growth. liver cirrhosis. malignancies.• 3) parasitic infections. 2) Kwashiorkor (oedematous PEM) • • • These children are adapted to fulfill the deficit to some extent. Congenital defects Like cleft lip/palate. • In protein . It is the commonest type of PEM. diabetes mellitus. • • • • • • • • • • Marasmic infants show hunger. a reverse of normal. PS pulmonary stenosis. as in anorexia nervosa. marked stunting & no edema. more hairs are in the telogen (resting) phase than in the anagen (active) phase. asthma. Energy is conserved by decreasing the physical activity The weight is reduced & looks disproportionate with long body. the child leads to severe forms of PEM i. Marasmus (non oedematous PEM) • • • • • 2) Worst form of PEM 3 essential features .

72 Old man look. Due to excess lipolysis & mobilization of fat into the liver along with deficiency of b lipoproteins to transport fat out of liver. diarrhea) in advanced cases Hepatomegaly is constant finding in Kwashiorkor.term effects of malnutrition in childhood are not fully understood. It is reversible & does not lead to cirrhosis. infection. Protein . The degree of mental impairment is related to the duration of malnutrition & age of onset. In contrast to pellagra. revealing pale areas between the cracks. but is restored when the child’s condition improves. upper limb wasted. The eyelashes can undergo the same change. likely secondary to pressure when the child lies down.exposed areas. Behavioral development may be markedly retarded in the severely malnourished child. Skin changes are characteristic & progress over a few days. xerophthalmia & cheilosis can occur. lusterless. or heart failure. generalized wasting Commonly infants < 60% ++ Common Nil Nil/mild Age Weight Growth retardation Prevalence Oedema Apathy Moon face. Flag sign – due to periods of poor nutrition & optimum nutrition. including the back. fibrosis & thrombosis. Prognosis Mortality varies between 15 & 40%. oedema on dependant parts 1 .5 yrs 60 . a small vessel vasculopathy involving mural calcification with intimal proliferation. Also. Atrophy of the papillae on the tongue. angular stomatitis. dermatitis. An infant with marasmus is affected most severely than a child with kwashiorkor. • • The dermatitis manifests in sun . alternating bands of pale & dark hair may occur. A mild degree of retardation may be present until the school going age. Comparison between marasmus & Kwashiorkor Marasmus Appearance Kwashiorkor Nail plates are thin & soft & may be fissured or ridged. prominent bony parts. dry & then splits open when stretched. This feature is seen especially over extensor surfaces. Long . Immunity is poor during the acute phase of the disease. Niacin deficiency clinically manifests as pellagra (i. having a so . With adequate treatment the liver probably recovers fully without subsequent damage. Marbalisation – soft & shiny skin with marble like feeling Depigmentation of hair causes it to be reddish yellow to white.• • • • • • • • • • • • • • • • • Moon facies Child typically presents with a failure to thrive Swollen abdomen (potbelly). these changes seldom occur on sun exposed skin. Temporal recession & hair loss from the back of the head occur. neck (Casal necklace). and A fatty liver. Curly hair becomes straightened. sparse & brittle.energy malnutrition is also associated with an increased likelihood of calciphylaxis.80% + Rare ++ ++ . hairs become dry. Flaky paint dermatosis – peeling of skin like old paint flaking off the wall is pathognomic of kwashiorkor. face & dorsum of the hands (gauntlet of pellagra) initially as painful erythema & itching. Crazy pavement dermatosis . In some cases. Death in the first days of treatment is usually due to electrolyte imbalance.e. loss of hair can be extreme.The skin becomes dark. they can be pulled out easily. hypothermia (severe decrease in body temperature). Hair can also become softer & finer & appear unruly.called Broomstick appearance. dementia.

Grades loss of fat from Buttocks Axilla / groin Abdomen.12 yrs. diet during diseased state & present diet should be taken in consideration. chest. for age • • • I) II) III) IV) 2) I) II) III) 3) I) II) III) IV) 71 .90 % 70 .70 % 51 .80 % 61 . (kg) normal 1st degree wasting 2nd degree wasting 3rd degree wasting Age in mts+9 infant Ht.6 yrs 7 . albumin Anabolism Catabolism Prognosis Alert Voracious Nil/mild Nil/mild + + Low / normal + ++ Good Irritable Poor + + ++ ++ Very low Very low + Poor/guarded • 2) 3) a) feeding. 2 . height & MAC are compared with reference standards. back Buccal pad of fat.12 yrs Age in yrs × 2 + 8 Age in yrs × 7 – 5 • An overall assessment starting right from breast feeding.Mental status Appetite Hair changes Skin changes Fatty liver Infections Sr. Classification of stunting (waterlow) Height for age grade > 95 % normal 90 .80 % < 70 % Wt. weaning.Grades % of standard wt. The 50 th percentile is taken as the expected weight or 100%. Weight for age.90 % 61 . This overall assessment is then compared with RDA (recommended dietary allowances) Clinical features Clinically observed symptoms & signs are seen. initial . Anthropometry Most reliable & practical method.90 % 2 degree < 85 % 3 Classification of wasting (waterlow) Weight for height interpretation Assessment of nutritional status 1) 3) 5) 7) 1) > 90 % 80 . protein. Height It is measured with stadiometer in children & with infantometer for age < 2 yrs. K is added to the grade.60 % ≤ 50 % If oedema is present. Gomez classification . height for age & weight for height are interpreted. (cm) 73 1 . Following parameters are used Dietary factors 2) Clinical features Anthropometry 4) Biochemical parameters Morphological features 6) Radiological features Vital statics Dietary factors Weight.75 % < 60 % Udani’s classification . for age 76 .95 % I degree stunting 85 .Grades % of standard wt. Age dependant anthropometry Grading of PEM 1) IAP classification .

lipoprotein) & hypoglycemia.) Electrolytes Significant findings in kwashiorkor include hypoalbuminemia (10 . Electrolytes. In children older than 2 years. patients should be fed ad libitum. body mass index (BMI) & a complete physical examination are indicated. Malnutrition also causes immunosuppression.for .25 g/L). intake rates should approach 175 kcal/kg & 4 g/kg of protein for children & 60 kcal/kg & 2 g/kg of protein for adults. Milk .energy malnutrition • (which may be delayed 24 . All but the most severely ill respond to a diet based on milk. 74 Detailed dietary history. Macronutrient repletion should be started within 48 hours under the supervision of nutrition specialists. Small.negative tuberculin skin test results & the subsequent failure to accurately assess for tuberculosis. which may result in false . especially potassium & magnesium. iron deficiency anemia & metabolic acidosis are present. the first step in the treatment of protein . Urinary excretion of hydroxyproline is diminished. Ketonuria occurs & protein .48 h in children) is to supply macronutrients by dietary therapy. Levels of some enzymes (including lactase) are decreased & circulating lipid levels (especially cholesterol) are low. After 1 week.for . Sensitive measures of nutritional deficiency in children include height . or greater than 2 standard deviations below the mean for age.age or weight . A daily multivitamin should also be added. iron & folic acid supplements. are depleted. The most common electrolyte abnormalities are hypokalemia. Indications for hospitalization . Unless urgent. In both kwashiorkor & marasmus. Increased urinary 3 methylhistidine is a reflection of muscle breakdown & can be seen in marasmus. Plasma cortisol & growth hormone levels are high. At the beginning of dietary treatment. frequent feedings round the clock are tolerated best in the early stages of recovery.based formulas are the treatment of choice. treatment of malaria or other parasitic infections will be postponed until the patient is clinically improved. but insulin secretion & insulinlike growth factor levels are decreased. hypophosphatemia & hypomagnesemia.energy • malnutrition (PEM) is to correct fluid & electrolyte abnormalities & to treat any infections. hypoproteinemia (transferrin. Medical Care Treatment Other Tests A doctor’s aim will be to restore & maintain fluid & electrolyte balance.height measurements less than 95% & 90% of expected. Antibiotics may be prescribed. essential amino acids. reflecting impaired growth & wound healing. growth measurements. • hypocalcemia.Laboratory Studies • o o o o o o o o • • • • • • • • • • • The WHO recommends the following laboratory tests : Blood glucose Examination of blood smears by microscopy or direct detection testing Hemoglobin Urine examination & culture Stool examination by microscopy for ova & parasites Serum albumin HIV test (This test must be accompanied by counseling of the child’s parents & strict confidentiality should be maintained. The percentage of body water & extracellular water is increased. Anemia is usually mild & responds to oral protein. Supplementary vitamins may be advisable. growth of less than 5 cm/y may also be an indication of deficiency. respectively. In children. The second step in the treatment of protein .energy malnutrition may cause a decrease in the urinary excretion of urea because of decreased protein intake.

with : Severe dehydration. Hypothermia (rectal < 35. but is neither thirsty nor restless Developed septic shock : dilated rather than constricted superficial veins. dehydration septic shock vitamin deficiency Severe anemia & heart failure. tachypnea (> 50 In 2 . Initial treatment (IV). give 5 ml/kg of body weight of sterile 10% glucose intravenously 75 Incipient septic shock. infections Stabilization to start to feed the child To correct micronutrient deficiency Rehabilitation . If losing consciousness. Follow up : One packet + 6 cc KCl + + 100 cc glucose 5 % + 300 cc water : 70 . IV fluids The only indication is circulatory collapse caused by severe dehydration or septic shock. repeat 15 ml/kg over another hour & then shift to ReSolMal by NGT.0 °C) : Kangaroo technique Clothe the child well (including the head). Treatment of dehydration A) Oral Management of severe malnutrition ReSolMal Amount Duration Route B) A) • • • • • • B) • • II) III) Emergency treatment of life threatening complications hypoglycemia & hypothermia. hypothermia. Signs of systemic. apathetic & profoundly anorexic. Substantial weight deficit. but without a history of watery diarrhoea Hypothermia or hypoglycemia .To increase feeding to recover lost weight. Purpura. give 50 ml of 10% glucose or sucrose. Jaundice. grunting. Persistent diarrhea & vomiting. Extreme pallor.5 °C or the axillary < 35. Type : Ringer lactate with 5% glucose or 0. Treatment of septic shock Emergency Management Hypoglycemia (< 54 mg/dl or 3 mmol/l) Start treatment on suspicion. Duration : One hour. Give broad spectrum antibiotics.12 months & > 40 in 1 . : 12 hrs. GI bleeding Signs of dehydration.100 ml/kg. < 70 % of standard weight for height Or < 60 % of standard weight for age. Persistant loss of appetite. cover with a warmed blanket Place a lamp over Treat for hypoglycemia & for serious systemic infection. Amount : 15 ml/kg.g. If not improved. : oral / NGT. respiratory tract or other localized infection. The child is usually weak. If conscious or can be aroused & is able to drink. Severe anemia (Hb < 5 g dl). followed by 50 ml of 10% glucose or sucrose by nasogastric (NG) tube. e. consider septic shock & treat accordingly. If improved.1) 2) 3) • • • • • • • • I) Oeedema (Kwashiorkor or marasmic kwashiorkor).5 yrs).45% NaCl with 5% glucose. cannot be aroused or has convulsions. clinical evidence of shock.

infective treatment Start feeding : Formula : (F 75) : Each 100 ml : 75 Kcal. give diuretics.2 mg/ kg / d. give 2 ml of a 50% solution of magnesium sulfate IM.7mg Cyanocobalamin (vitamin B12) 1?g Folic acid 0. Stabilization Very severe anaemia haemoglobin concentration is < 4 g/dl or the PCV < 12%.5 mg/kg every 8 hours for 7 days (Oral : 1 cc / kg / d) 76 Vit E : 2. or giving a diet with high sodium content The first sign of heart failure is fast breathing 1) Stop all oral intake & IV fluids. Complications or proved infection : Ampicillin.1mg Retinol (vitamin A) 1. is removed when takes ¾ feed orally. No fluid should be given until the heart failure is improved. Frequency : 2 hours (11 cc / Kg * 12 feeds).vitamin preparation containing : (for 7 kg BW) Thiamine (vitamin B1) 0. If heart failure develops. add chloramphenicol.3 gm lactose Amount : 130 ml / Kg. 1. Vitamins 1) 2) No apparent signs of infection & no complications : Cotrimoxazole (25 mg of sulfamethoxazole + 5 mg of trimethoprim/kg) orally twice daily for 5 days (0. 5 mg of folic acid orally on day 1 & then 1mg orally per day thereafter. if oral intake < 80 Kcal/ kg/ d. 25mg/kg IM or IV every 8 hours (or every 6 hours if meningitis is suspected) for 5 days. 7.35mg Ascorbic acid (vitamin C) 100mg Pantothenic acid (vitamin B5) 3mg Biotin 0.0mg Nicotinic acid 10mg Pyridoxine (vitamin B6) 0.6 cc / kg/12 hrs). If no improvement after the 1st hour give blood transfusion or plasma (10 ml/kg over at least 3 hours).5mg (5000 IU) Calciferol (vitamin D) 30?g (1200 IU) Vitamin K 40?g Minerals . very severe anaemia. Cup & spoon. ] 3) Do not give digitalis unless the diagnosis of heart failure is unequivocal (jugular venous pressure is elevated) & the plasma potassium level is normal (digitalis is given at 0. Muti . Vitamins : A large oral dose of vit on day 1 : 50 000 IU (< 6 months). 2) Give a diuretic IV [furosemide (1 mg/kg). Steroids. If develops abdominal distension during NG feeding.005mg/kg) Anti . NGT.7mg Riboflavin (vitamin B2) 2. IV fluid therapy.9 – 1. 0. Epinephrine are of no value & should not be used If the child fails to improve within 48 hours. Congestive heart failure This is usually a complication of overhydration. Give 10 ml of packed red cells or whole blood per kg of body weight slowly over 3 hours.Management includes 3) 1) 2) 3) 4) 5) Broad spectrum antibiotics. 7.5 mg/kg IM or IV once daily for 7 days.1 gm. Some institutions routinely give malnourished children metronidazole. Route : Oral. 100 000 IU (6 – 12 months) & 200000 IU (> 12 months). 50mg/kg IM or IV every 6 hours & gentamicin. blood or plasma transfusion. even if this takes 24–48 hours. better to avoid bottle.

5 mg Zn) Mg : 16 . Frequency : 3 hourly or 4 hourly.50mg/kg/d. crawls. whereas others are hungry from the start Rehabilitation Formula : (F 100) : Each 100 ml : 100 Kcal. selenium (4. stands or walks (depending on age) Normal temperature (36. 2. if more than 100 kcal/kg/d amount of feed should be reduced.6 weeks until 90% of the expected weight for height is attained (. the child is failing to respond Rehabilitation usually takes 2 .1 SD).9 gm protein. responds to stimuli. (1 cc zinc sulphate 2% = 4. Amount : Starting with 130 ml / kg / d (16 ml/ kg/ feed). Recording food intake If the daily intake < 80 kcal/kg/d the amount should be increased. End of initial phase Assessing progress The usual weight gain is about 10–15 g/kg /d.7 mmol/kg/d (up to 7. If < 5 gm/ kg/ d for 3 days = failure to respond Criteria for transfer to a nutrition rehabilitation centre • • • • • • • Eating well Mental state has improved : smiles.5 °C) No vomiting or diarrhoea No oedema Gaining weight : > 5 g/kg of body weight per day for 3 successive days Failure to respond When the child’s appetite improves & becomes hungry Usually 2 – 7 days.based feeds Acidic faeces (pH < 5.220 Kcal/kg/d.3 mg/ kg /d). Complications may take longer. If < 130 Kcal /kg/d. When this occurs appetite diminishes. increased by 10 ml each feed until infant refuses to finish the feed. Sodium : Should be restricted to less than 2 mmol/kg/d Potassium : 4. Calcium : 50 . Caloric intake : 150 .5–37.Iron supplementation : Postponed. interested in surroundings Sits. Lactose intolerance : should be diagnosed only if : Watery diarrhoea promptly after milk .2 gm Criteria for failure to respond to treatment Criteria Time after admission Primary failure to respond : Failure to regain appetite Day 4 Failure to start to lose oedema Day 4 Oedema still present Day 10 Failure to gain at least 5 g/kg of body weight per day 10 Secondary failure to respond : Failure to gain at least 5 g/kg of body weight per day During rehabilitation for 3 successive days Frequent causes of failure to respond Problems with the treatment facility lactose. 77 • • • • • • Poor environment for malnourished children Insufficient or inadequately trained staff Inaccurate weighing machines Food prepared or given incorrectly Insufficient food given Vitamin or mineral deficiency Problems of individual children .0) Presence of reducing substances in the faeces.7 mmol / kd /d) Zinc : 2 mg/kg/d.80 mg/kg/d.7?g /kg /d).4. Phosphorus : 60 mg/kg/d Trace elements including : copper (0. iodine (12 ?g / kg / d).

where it undergoes hydroxylation to the active metabolite calcitriol (1. The increase of calcium & phosphorus in extracellular fluid. intestinal helminthiasis & HIV/AIDS especially those who receive no oral supplementation & those who have darkly pigmented skin. which circulates in the plasma as the most abundant of the vitamin D metabolites & is thought to be a good indicator of overall vitamin D status. leads to the calcification of osteoid. Calcitriol may also directly facilitate calcification. 78 Cholecalciferol (i. Vitamin D . hypocalcemia develops. further reducing deposition of calcium in the bone. Calcitriol promotes absorption of calcium & phosphorus from the intestine. Failure of osteoid to calcify in adults is called osteomalacia. which blocks penetration of ultraviolet light. primarily at the metaphyseal growing ends of bones but also throughout all osteoid in the skeleton. The first hydroxylation occurs at position 25 in the liver. The second hydroxylation step occurs in the kidney at the 1 position. pneumonia.3) is formed in the skin from 5 . malaria. This cholecalciferol is not technically a vitamin but a hormone. 25 . a dietary deficiency of calcium or phosphorus may also produce rickets.• • • Malabsorption of nutrients Rumination Infections.up growth Other . vitamin D . In the vitamin D deficiency state.7) Initial stabilization 8) Catch . urinary tract infection. Calcitriol acts at 3 known sites to tightly regulate calcium metabolism.dihydroxycholecalciferol).dihydrotachysterol.hydroxycholecalciferol). though phosphorus levels remain low. Less commonly. which stimulates excess parathyroid hormone. especially diarrhoea.9) Provide loving care & stimulation 10) Prepare for follow . Therefore. These actions increase the concentrations of calcium & phosphorus in extracellular fluid. This steroid undergoes hydroxylation in 2 steps.hydroxylation step in vitamin D metabolism. Pathophysiology WHO guidelines 10 steps for routine management of children with severe malnutrition Prevent & treat .e.up after discharge 22 Rickets • • • • • • Rickets is a disease of growing bone that is unique to children. producing calcidiol (25 . Parathyroid hormone facilitates the 1 . After the parathyroid response. tuberculosis. in turn. infants who are breastfed are at risk for rickets. otitis media. It is caused by failure of osteoid to calcify in growing children. dysentery. Early in the course of rickets. Rickets is observed only in growing children. the calcium concentration usually returns to the reference range. Vitamin D deficiency rickets occurs when the metabolites of vitamin D are deficient.B light. which stimulates renal phosphorus loss. Excess parathyroid hormone also produces changes in the bone similar to those occurring in hyperparathyroidism. although the effects may be observed later in life. the calcium concentration in the serum decreases. Alkaline . increases reabsorption of phosphate in the kidney & acts on bone to release calcium & phosphate.1) Hypoglycaemia 2) Hypothermia 3) Dehydration 4) Electrolyte imbalance 5) Infection 6) Micronutrient deficiencies Provide special feeds for .3 (cholecalciferol) is formed in the skin from a derivative of cholesterol under the stimulus of ultraviolet .

In the long bones.phosphatase. severe phosphorus deficiency that occurs • when human milk is used without mineral fortification presents with rickets. however it is often within the reference range at the time of diagnosis as parathyroid hormone levels increase. Weight bearing produces deformities such as bowlegs & knock . Anticonvulsant drugs (e. In the chest. they may fracture one side of the cortex (i. At the ankle. Because the softened long bones may bend. palpation of the tibial malleolus gives the impression of a double epiphysis (Marfan sign). however. Laboratory Studies • • The following findings may be noted in patients with rickets – Early on in the course of rickets. Mortality / Morbidity • along the costochondral junctions. the calcium (ionized fraction) is low. This produces frontal bossing & delays the closure of the anterior fontanelle. such as hypophosphatemic vitamin D–resistant rickets. phenobarbital. In premature infants. producing knobby deformity. Jansen syndrome is a rare autosomal dominant form of short • limbed dwarfism in which infants present with metaphyseal chondroplasia.e. Calcidiol (25 . If rickets occurs at a later age. leaks to the extracellular fluids so that its concentration rises to anywhere from moderate elevation to very high levels. phenytoin) accelerate metabolism of calcidiol. In more severe instances in children older than 2 years. Calcium & vitamin D intakes are low in infants who are fed vegan diets. The ends of the long bones demonstrate that same knobby thickening. determining calcidiol . Craniotabes manifests early in infants with vitamin D deficiency. Severe rickets has been associated with respiratory failure in children & resulting pelvic distortion in females may lead to problems with vaginal delivery later in life. particularly lacto vegans & monitoring of their vitamin D status is essential. including hypophosphatasia have • been confused with rickets in infancy. Severe calcium deficiency can also cause a syndrome that is • confused with vitamin D deficiency rickets.knees. greenstick fracture). which is visualized on radiography as cupping & flaring of the metaphyses. Other problems to be considered Skeletal deformity & short stature may occur. laying down of uncalcified osteoid at the metaphases leads to spreading of those areas. vertebral softening leads to kyphoscoliosis.hydroxy vitamin D) levels are low & parathyroid hormone levels are elevated.breast deformity. particularly in children who are kept indoors in institutions. The sternum may be pulled into a pigeon . which is known as Harrison groove. which is produced by overactive osteoblast cells. Intestinal malabsorption of fat & diseases of the liver or kidney may produce the clinical & secondary biochemical picture of nutritional rickets. History • • • • • The history in patients with rickets may include – Generalized muscular hypotonia of an unknown mechanism is observed in most patients with clinical (as opposed to biochemical & radiographic) signs of rickets. although this feature may be normal in infants. especially for those born prematurely.g. knobby deformities results in the rachitic rosary 79 The following should also be considered in patients with suspected rickets : Rare metabolic bone diseases. which may lead to insufficiency & rickets. Hereditary disorders of vitamin D metabolism have also been • described. The weakened ribs pulled by muscles also produce flaring over the diaphragm. thickening of the skull develops.

old girl with hypophosphatemia depict severe fraying of the metaphysis. alkaline phosphatase. Because both calcitriol & calcidiol have short half .glass osteopenia of scurvy.6 . A generalized aminoaciduria occurs from the parathyroid activity.7 days.• • • • & parathyroid hormone levels is typically not necessary. 25 dihydroxyvitamin D.hydroxy vitamin D & 1. The metaphyses exhibit widening & cupping because of their exaggerated normal concavity & irregular calcification. 000 U) of vitamin D. Other Tests Imaging Studies Serum measurements include calcium. Treatment for rickets may be gradually administered over several months or in a single . Treatment • • • • Anteroposterior & lateral radiographs of the wrist of an 8 . If the vitamin D dose is administered in a single day. Along the shaft.old boy with rickets demonstrates cupping & fraying of the metaphyseal region. phosphorus. If severe deformities have occurred. success depends on compliance. orthopedic correction may be required after healing. Most of the deformities correct with growth. they are unsuitable. 000 U) is given daily for 2 . The best single radiographic view for infants & children younger than 3 years is an anterior view of the knee that reveals the metaphyseal end & epiphysis of the femur & tibia. Because this method requires daily treatment. An intramuscular injection is also available.year .250 mcg (5000 .10. Medical Care Human milk contains little vitamin D & contains too little 80 . Calcitriol levels maybe normal or elevated because of increased parathyroid activity. Radiography is indicated in patients with rickets. Neither happens with FHR. aminoaciduria does not occur in familial hypophosphatemia rickets (FHR). 125 .day dose of 15. they would bypass the natural physiologic controls of vitamin D synthesis. Radiographs of the knee of a 3. The phosphorus level is invariably low for age unless recent partial treatment or recent exposure to sunlight has occurred. This site is best because growth is most rapid in this location. with visible coarsening of trabeculae in contrast to the ground . 000 mcg (600. Because calcified osteoid is abundant. Generalized osteomalacia occurs (observed as osteopenia). the phosphorous level rises in 96 hours & radiographic healing is visible in 6 . parathyroid hormone. Alkaline phosphatase levels are elevated. the provisional calcification zone of the metaphysis is much more distant from the calcification center of the epiphysis than is normal for age.lives.3 months until healing is well established & the alkaline phosphatase concentration is approaching the reference range. 25 . Vitamin D is well stored in the body & is gradually released over many weeks. thus the changes are accentuated. it is usually divided into 4 or 6 oral doses.year . Examples of radiographic findings are shown in the images below. Radiograph in a 4 . In nutritional rickets. the uncalcified osteoid causes the periosteum to appear separated from the diaphysis.old girl with rickets depicts bowing of the legs caused by loading.day therapy avoids problems with compliance & may be helpful in differentiating nutritional rickets from familial hypophosphatemia rickets (FHR). If the gradual method is chosen. The single .year .

Icteric phase Dark urine appears first (bilirubinuria).to . a direct correlation between increasing age & likelihood of adverse events (i. hepatitis A virus infection is often asymptomatic & subclinical.e.soluble vitamin used to treat vitamin D deficiency or for prophylaxis of deficiency. morbidity & mortality) is present. Humans appear to be the only reservoir for the hepatitis A virus. The hepatitis A virus was isolated by Purcell in 1973. The degree of icterus also increases with age. Pathophysiology • • • • The hepatitis A virus is a single .stranded.6 weeks & the time to onset of symptoms may be dose related. 000 mcg (600.3.3 mt. linear RNA enterovirus & a member of the Picornaviridae family. Mortality / Morbidity Vitamin D . therefore.250 mcg (5000 . Recommending a vitamin D supplement from the first week of life for susceptible infants who are breastfed is safe & effective and. malaise. phosphorus) if breast milk is their primary dietary source. 000 U) qid for 2 . The period of greatest shedding of the hepatitis A virus is during the anicteric prodrome (14 . Single . although less common than jaundice. The hepatitis A virus can remain viable for many years. myalgia & mild headache.sense. Minimal cellular morphologic changes result from hepatocyte 81 • o • o o o o Not every patient with fever. Boiling water is an effective means of destroying it & chlorine & iodine are similarly effective. Some of the important differential diagnoses for acute hepatitis warrant early & specific management.day dose method 15. nausea & vomiting. The single most important determinant of illness severity is age. Vitamin D • infection.Time course of infection Fat . In humans. Infants weighing less than 1500 g need special supplementation (i. Person . Abdominal pain occurs in approximately 40% of patients. is . hepatomegaly & jaundice has hepatitis A virus infection. 23 Hepatitis A Globally.10. 000 U) PO qd divided in 4 . fatigue. The presence of disease manifestations & the severity of symptoms following hepatitis A virus infection directly correlate with patient age. Prodrome Patients may have mild flulike symptoms of anorexia.e.oral transmission). History One of the more common causes of acute hepatitis is hepatitis A virus (HAV).e.1 mg provides 40000IU vitamin D activity. Itching (pruritus).21 d) of infection & corresponds to the time when transmission is highest. Hepatitis A . positive . Jaundice is less likely in children & is uncommon in infants. should be considered. Cholecalciferol The incubation period usually lasts 2 . vitamin D. viral replication depends on hepatocyte uptake & synthesis & assembly occurs exclusively in liver cells. fecal .6 doses for 1 d Gradual method 125 .person contact is the most common means of transmission & is generally limited to close contacts.phosphorus for babies who weigh less than 1500 g. Other populations with increased likelihood of adverse sequelae caused by acute hepatitis A virus infection are those with significant comorbidities or concurrent chronic liver disease. Acquisition results almost exclusively from ingestion (i. calcium.

000 mIU/mL.hepatitis A virus IgM are positive at the time of onset of symptoms & usually accompany the first rise in Alanine aminotransferase (ALT) level. Test results for anti . therapy is generally supportive. with ALT levels generally greater than AST levels. Arthralgia & skin rash. This test is sensitive & specific. persistence beyond 3 months indicates cholestatic hepatitis A virus infection. Prothrombin time Prothrombin time usually remains within or near the reference range In the presence of encephalopathy. fulminant hepatic failure). Initial therapy often consists of bed rest. CBC count Mild lymphocytosis is not uncommon. Patients may have a fever with temperatures of up to 40°C. Nausea & vomiting are treated with antiemetic. with no specific treatment. an elevated prothrombin time has ominous implications (e. Rash more often occurs on the lower limbs. Medical Care For acute cases of hepatitis A virus infection. Jaundice or scleral icterus may occur. IgM persists in patients with relapsing hepatitis for the duration of this pattern of disease.induced liver injury Acute HIV infection Drug . Both direct & indirect fractions increase because of hemolysis. • . Levels may be high & can remain elevated for several months.g. o o The diagnosis of acute hepatitis A virus infection is based on serologic testing for IgM antibody to the hepatitis A virus. Slight fall in serum albumin level may accompany the illness. Hepatomegaly is common. 82 Imaging Studies An ultrasound scan may be required when alternative diagnoses warrant exclusion & should assess vessel patency & evaluate any evidence supporting unsuspected underlying chronic liver disease. Rises in alkaline phosphatase accompany the acute disease & may progress during the cholestatic phase of the illness following the rises in transaminase levels. although associated.Chiari Syndrome Cytomegalovirus Other Viral Hepatitis Acute drug . Hepatic synthetic function Bilirubin level rises soon after the onset of bilirubinuria & follows rises in ALT & AST levels.g. Levels may exceed values of 10. Risk factors for acquisition of hepatitis A include the following – Personal contacts • Institutionalization • Occupation (e. Pure red cell aplasia & pancytopenia may rarely accompany infection. are less frequent than the above symptoms. Levels usually return to reference ranges over 5 20 weeks.induced hypersensitivity reactions Laboratory Studies • • o o • o Liver enzymes Rise of levels in ALT & Aspartate aminotransferase (AST) assays are sensitive for this disease. which often occurs in acute hepatitis A virus infection.o generally accompanied by jaundice. daycare) • Foreign travel • Male homosexuality • Illicit parenteral drug use • Differential diagnosis • o o Budd . • o Physical • • • o Causes Most patients have no defined risk factors for hepatitis A.

Restricting transmission is important. Mortality / Morbidity • • • • Pertussis. . • 24 25 Pertussis • respiratory epithelium. pulmonary. In 1906. pulmonary. Bacteria spread via aerosolized droplets from coughing of infected individuals. Young infants. B pertussis attaches to & damages ciliated 83 • • • • History • • Pathophysiology • The 3 stages of disease progression are as follows : Stage 1 : The initial (catarrhal) phase is indistinguishable from • common upper respiratory infections with nasal congestion. Reported cases of pertussis decreased by more than 99% after the introduction of pertussis vaccine combined with diphtheria & tetanus toxoids in the 1940s. with as many as 80% of susceptible household contacts becoming infected after exposure. infants younger than 6 months with pertussis are more likely to have severe disease. B pertussis is a gram . Typically. Bordet isolated the most common causative organism. or neurologic disease are at high risk for contracting the disease & for complications Infants born prematurely & patients with underlying cardiac. especially in the early phases of the illness. encephalopathy & death). It was first identified in the 16th century. hospitalization is unnecessary. adolescents & adults often have mild or atypical illness. or respiratory secretions from an infected source. Bordetella pertussis. each lasting from 1 . Older children. or through contact with oral. although data to support this practice are lacking. Most children have minimal symptoms. • Activity • Bed rest during the acute illness may be important. sharing a confined space. Humans are the sole reservoir for the organisms. The goals of pharmacotherapy are to reduce morbidity & to prevent complications. is a respiratory tract infection characterized by a paroxysmal cough. pertussis was a major cause of morbidity & mortality among infants & children.• • Dehydration may require hospital admission & intravenous fluids.negative pleomorphic bacillus that spreads via aerosolized droplets from coughing of infected individuals. or neurologic disease are at high risk for complications of pertussis (e.12 days. Pertussis is highly contagious. neuromuscular. paroxysmal & convalescent stages. pneumonia.face contact.2 weeks. Bordetella parapertussis has also been associated with whooping cough in humans.week disease divided into catarrhal. commonly known as whooping cough or cough of 100 days. Approximately one half of adolescents with pertussis cough for 10 weeks or longer.g. seizures. nasal. Compared with older children & adults. Humans are the sole reservoir for B pertussis & B parapertussis. especially those born prematurely & patients with underlying cardiac. neuromuscular. In most instances. Pertussis is a 6 . Before the advent of vaccinations. Transmission can occur through direct face . the incubation period of pertussis ranges from 3 .to . to develop complications & to require hospitalization.

grade fever. 000 10 3/µL) with absolute • lymphocytosis occurs during the late catarrhal & paroxysmal phases. color changes. or air in the soft tissues may be seen. or inspiratory whoop) o A cough that lasts at least 14 days in an outbreak setting A confirmed case is defined as one of the following : • o Any cough illness in which B pertussis is isolated & cultured o A case consistent with the clinical case definition confirmed by polymerase chain reaction (PCR) findings or epidemiologic linkage to a laboratory . Cough bout followed by vomiting episode & flushing of face are common in affected children. observing the severity of cough. Imaging Studies · B pertussis & B parapertussis are the causative organisms for pertussis infection in humans. A clinical case of pertussis is defined as one of the following : • o An acute coughing illness that lasts at least 14 days with at least one characteristic pertussis symptom (i. clarithromycin & azithromycin. rest & recovery. which may last for weeks.e. In all patients with pertussis. Occasionally.50. clinicians need to make the diagnosis of pertussis presumptively in patients with a history of intense paroxysmal coughing with or without whooping. pneumothorax. physical examination findings contribute little to the diagnosis. paroxysmal cough. macrolide antibiotics such • as erythromycin. Differential diagnosis Chest radiography may reveal perihilar infiltrates or edema with variable degrees of atelectasis.• • rhinorrhea & sneezing. Consolidation is indicative of secondary bacterial infection or. pertussis pneumonia. The goals of therapy include limiting the number of paroxysms. 84 The mainstay of therapy in patients with active pertussis infections is supportive.confirmed case Leukocytosis (15. but pertussis may remain communicable for 3 or more weeks after the onset of cough. For patients aged 1 month or older. rarely. 000 . Erythromycin & clarithromycin are not recommended in infants younger than 1 month because their . incomplete or absent pertussis vaccination & finding of lymphocytosis on laboratory examination. tearing & conjunctival congestion. Conjunctival hemorrhages & facial petechiae are common & result from intense coughing. Infants younger than 6 months do not have the characteristic whoop but may have apneic episodes & are at risk for exhaustion. Stage 3 : Patients in the third (convalescent) stage have a chronic cough. It is a nonspecific finding but correlates with severity of the disease. pneumomediastinum. Physical In patients with uncomplicated pertussis. the paroxysms of coughing occasionally are followed by a loud whoop as inspired air goes through a still partially closed airway. Causes Therefore. are the preferred agents. Stage 2 : Patients in the second (paroxysmal) phase present with paroxysms of intense coughing lasting up to several minutes. Pertussis is most infectious when patients are in the catarrhal phase. In older infants & toddlers. fever is typically absent. variably accompanied by low . posttussive vomiting. Medical Care Afebrile Pneumonia Syndrome Bronchiolitis Chlamydial Infections Mycoplasma Infections Respiratory Syncytial Virus Infection Laboratory Studies Laboratory confirmation of pertussis is difficult & delayed. providing assistance when necessary & maximizing nutrition. posttussive vomiting. Most patients do not have signs of lower respiratory tract disease.

epidemic poliomyelitis was more frequently observed. or neuromuscular disease. with the following dosage : trimethoprim 8 mg/kg/d & sulfamethoxazole 40 mg/kg/d in 2 divided doses. Polioviruses are RNA enteroviruses within the Picornaviridae family. 1958 : First general use of Sabin (live attenuated vaccine) by mouth.6 months. poliomyelitis occurred sporadically. o Antimicrobial agents given during the catarrhal phase may ameliorate the disease. These viruses are resistant to ether & chloroform but can be inactivated by formaldehyde. not to exceed 1 g/d for 5 . especially in relation to coughing paroxysms. premature young infants. Following is a timeline of the recorded history of this disease : 1789 : First known description of poliomyelitis by Underwood. feeding. Before the 19th century.7 days. The evidence for this is in the withered & deformed limbs of certain Egyptian mummies. vomiting & weight changes should be recorded. abortive disease. 1951 : Three types of polio virus isolated & identified. respiratory rate & oxygen saturation of hospitalized patients continuously. The use of corticosteroids. unless observed paroxysms are not severe. 1954 : First large . & infants or children with underlying pulmonary. Poliomyelitis is an enteroviral infection that can manifest in 4 different forms : inapparent infection. The typical contractures of post polio residual paralysis . reaching its peak in the mid 1950s. albuterol & other beta2 . Hospitalization should be strongly considered for patients at • risk of severe disease & complications.scale trial of Salk (dead vaccine) by injection. o Patients who are severely ill may require treatment in an ICU. Azithromycin is the recommended agent in the youngest patients. The worldwide prevalence of this infection has decreased significantly since then because of aggressive immunization programs.sulfamethoxazole is another antibiotic option. nonparalytic poliomyelitis & paralytic disease. o Pay attention to the young infant’s hydration & nutritional status.fecal route or by ingestion of contaminated water. infants aged 3 .20 mg/kg/d PO in 2 divided doses. 1949 : Growth of the virus on tissue culture. Once cough is established. Poliovirus is transmitted through the oral . 1855 : First description by Duchenne of the pathologic process in poliomyelitis involving the anterior horn cells of the spinal cord. They multiply in the GI tract but are particularly neurotropic.12 mg/ kg/d PO in 1 dose for a total of 5 days. antimicrobial agents may not alter the course of the illness but are still recommended to limit the spread of disease. cardiac. 1834 : First epidemic of poliomyelitis on the island of St. Helena. 1908 : Transmission of poliomyelitis to a monkey by Landsteiner. 1909 : Passage of the virus through a monkey by Flexner. During the 19th & 20th centuries. Trimethoprim .adrenergic agents for the treatment of pertussis is not advised. • • 26 Poliomyelitis 85 • Poliomyelitis first occurred nearly 6000 years ago in the time of the ancient Egyptians.use has been associated with increased risk for infantile hypertrophic pyloric stenosis (IHPS). o Monitor heart rate. Coughing. Antibiotics • • • • • • • • • • • • • Azithromycin is typically administered in doses of 10 . including infants younger than 3 months. Clarithromycin is administered at 15 .

with subsequent hematologic spread.40 years after infection with poliovirus. a history of the following is found with normal neurologic examination findings o Anorexia o Vomiting o Abdominal pain o Duration of illness usually less than 5 days 86 The spectrum of disease varies from inapparent infection to paralytic disease.95%) are inapparent. Paralytic poliomyelitis involves systemic manifestation. the following nonspecific signs & symptoms are • observed & usually resolve within a few days : o Fever o Headache o Nausea o Vomiting o Abdominal pain o Oropharyngeal hyperemia Nonparalytic poliomyelitis is characterized by the symptoms • described above in addition to the following : o Nuchal rigidity o More severe headache o Back & lower extremity pain o Meningitis with lymphocytic pleocytosis Paralytic poliomyelitis occurs in fewer than 5% of affected • patients & is characterized by the following : o Compromise of the motor neurons may be localized or widespread. symptoms are usually those observed in abortive disease in addition to meningeal irritation. 5 . however. which may be bulbar or spinal in distribution. Although most cases of poliomyelitis (90 . Physical • The destruction of motor neurons leads to the development of flaccid paralysis. In cases of abortive poliomyelitis (5 . This post polio syndrome may develop 20 . Mortality / Morbidity Mortality is more frequently observed in cases of paralytic poliomyelitis & is associated with complications such as respiratory failure.35 days. • • When nonparalytic poliomyelitis develops.Pathophysiology • • • • • • The poliovirus is shed in oral secretions for several weeks & in the feces for several months. After a period of viremia. Patients who have recovered from poliomyelitis occasionally develop a post poliomyelitis syndrome.10%). The viral particles initially replicate in the nasopharynx & GI tract & then invade lymphoid tissues. Three antigenically distinct strains are known. However. The incubation period for poliovirus is 5 . in which recurrences of weakness or fatigue are observed & which usually involve groups of muscles that were initially affected. Infection with one type does not protect from the other types. individuals of any age (especially those who are immunocompromised) may also develop the disease. with type I accounting for 85% of cases of paralytic illnesses.10% of patients who acquire this infection develop symptoms. History • • Most patients infected with poliovirus develop inapparent infections & are frequently asymptomatic. the virus becomes neurotropic & produces destruction of the motor neurons in the anterior horn cells in the spinal cord & brainstem. Age Poliomyelitis is mainly the disease of children. In mild cases. in addition to symptoms observed in nonparalytic poliomyelitis. . such as respiratory failure. immunity to each of the 3 strains is lifelong.

autonomic dysfunction (including hyperhidrosis or hypohidrosis) & decreased limb temperature may occur due to involvement of vasomotor centre. urinary retention. Head drop sign – when the examiners hand is placed under the shoulders of the child & the trunk is raised. hyperasthesis. Bulbar form • Neck rigidity Tripod sign – when the child is asked to sit without any support. Infections with polioviruses can be predisposed by factors such as exercise.o o o More frequently. • sensory symptoms & signs. asymmetric loss of muscle function is observed with involvement of major muscle groups. pharynx & vocal cords. shallow & irregular. Respiratory insufficiency may occur due to paralysis of the diaphragm & intercoastal muscles. This stage lasts usually for 2–3 weeks but may extend for up to 2 months. the presence of any tenderness in the muscles is evidence that the acute stage is not over. a transverse myelitis with paraparesis. or severe combined immunodeficiency. Spinal form • In this form paralysis of the spinal muscles occurs suddenly. patients who are immunocompromised. Additionally. abdominal cramps & pain & diarrhea. flexes the knees & puts his hands behind him for sitting. Minor illnesses The minor associated illnesses occur 1–3 days before the onset of paralysis. fever. such as those with human immunodeficiency virus (HIV) infection. He cannot do so due to stiffness of the spine & may suddenly draw up his knees. bulbar polio & paralytic poliomyelitis. Kiss the knee test – In sitting position the child is asked to kiss his knees. with gastrointestinal complaints such as nausea & vomiting. he moves from side to side. but permanent weakness results from necrosis of anterior horn cells Rarely. are particularly at high risk of developing poliomyelitis when exposed to both wild - Causes • • In this type the paralysis of the vagus nerve causes weakness of the soft palate. Poliovirus infections can be divided into minor & major forms. The virus is shed in oral secretions for several weeks & in the feces for several months. When respiratory centre is involved respiration becomes 87 . or absent. such as sore throat. polio encephalitis. the head lags behind. neck stiffness (nuchal rigidity) & a pleocytosis in the • CSF Profound asymmetrical muscle weakness • The initial phase is typically followed by some recovery of • muscle strength. Recovery may be complete. malaise & headache. fatigue & tonsillectomy. There are also systemic manifestations. immunoglobulin A (IgA) deficiency. Muscle atrophy is generally observed several weeks after the beginning of symptoms. B . Signs in poliomyelitis • • The major associated illnesses include all forms of central nervous system (CNS) disease caused by poliovirus. Clinical findings Acute stage Poliovirus is primarily spread by fecal–hand–oral transmission from one host to another. tremors & diminished deep tendon reflexes follow the flaccid paralysis. alone or in combination. Major illnesses The clinical findings associated with an attack of polio are as follows : Fever.cell dysfunction. partial. including aseptic meningitis (or nonparalytic polio). Myalgia. Bulbar polio is not always associated with spinal muscles paralysis.

Residual .paralysis stage • • • • No antiviral agents are effective against poliovirus.attenuated viruses present in the oral poliovirus vaccine. In neglected cases. Surgical Care • • Obtain specimens from the cerebrospinal fluid (CSF). General supportive treatment for the pyrexia & irritation. Post . Recovery stage Diet Because patients with poliomyelitis are prone to develop constipation. Treatment In this stage. Physical therapy is indicated in cases of paralytic disease. for the prevention of secondary respiratory infection. Children with extensive paralysis & gross deformities have to crawl on all fours to move from place to place. 88 The period beyond 2 years after the onset of the disease is called the residual . provide frequent mobilization to avoid development of chronic decubitus ulcerations. Mechanical ventilation is often needed in patients with bulbar paralysis.type polioviruses & vaccine . Total hip arthroplasty is a surgical therapeutic option for patients with paralytic sequelae of poliomyelitis who develop of hip dysplasia & degenerative disease.immunoglobulin M (IgM) titer during the acute stage is diagnostic.polio syndrome Post . This stage lasts for up to 2 years after the onset of the disease.fold increase in the immunoglobulin G (IgG) antibody titers or a positive anti . There is also disuse atrophy of muscles & shortening of the leg due to interference with growth.term ventilatory support. also known as the convalescent stage. there is gradual recovery of the muscles. No recovery of muscle power occurs in this stage. In paralytic disease. It has been estimated that 25–60% of the patients who had acute polio may experience . The knee joint is held at 5° of flexion & the foot is supported in a 90° position.Barre Syndrome (infective polyneuritis) Spinal Muscular Atrophy Myotonic dystrophy Laboratory Studies Rabies Tetanus Aseptic meningitis Severe hypokalemia Transverse myelitis • Tracheostomy care is often needed in patients who require long . Splinting relieves pain & spasm & prevents the development of deformities. and for any respiratory paralysis are the main aspects of treatment. Fecal impaction is frequent in cases of paralytic disease & can be treated with laxatives as soon as it develops. the recovery is rapid in the first 6 months but is slower during the subsequent months. the acute symptoms & muscle tenderness disappear & the paralyzed muscles begin to recover. gross fixed deformities of the hip. Analgesia is indicated in cases of myalgias or headache. Deformities are liable to occur due to imbalance of muscle power & poor posture. Active & passive motion exercises are indicated during the convalescent stage. During this entire period. A 4 .polio syndrome is the term used for the newly occurring late manifestations of poliomyelitis that develop in patients 30 to 40 years after the occurrence of the acute illness. The paralyzed legs are supported by plaster splints or pillows & sandbags to keep the hip joints in 5° of flexion & in neutral rotation. stool & throat for viral cultures in patients with suspected poliomyelitis infection to differentiate with septic meningitis. knee & foot occur with severe wasting of muscles. Differential Diagnoses • • o o Botulism Enteroviral Infections Guillain .paralysis stage. a diet rich in fiber is usually indicated. The treatment of poliomyelitis is mainly supportive.

Joint replacement surgery In patients with post .these late effects of the disease. soft tissue injury & shortening of the foot. knee pain. Treatment of residual paralysis (27) Rubella • • • • The final aim should be for patients to return home & be accepted & integrated into their communities. Limb lengthening Often. canes & crutches or may use new. degenerative arthritis. joint replacement can be indicated. contractures. Common issues include genu recurvatum. poliomyelitis is unilateral. lighter orthotic devices to treat new symptoms.tract problems. such as the increased risk of neurovascular injury. Rubella & congenital rubella syndrome are caused by rubella virus. the role of slowly progressive.polio residual deformities. Pin . Many patients require revision of orthotic devices such as braces. which occasionally requires limb lengthening. communicable exanthematous disease.extremity orthoses. Management of post .length inequality. hydrotherapy & electrical stimulation of muscles are essential in the management of paralytic polio. Arthrodesis (surgical immobilization of a joint by fusion of the bones) 89 • The name rubella is derived from a Latin term meaning “little red. weight . Ilizarov techniques Efficient physiotherapy Efficient physiotherapy is the mainstay of the management of poliomyelitis. infection in younger children is characterized by mild . Since overuse weakness is frequently present in these patients. Surgery for scoliosis or fractures may also be necessary to treat new conditions. or arthralgia. exercise therapy. Recurrence of instability & progressive functional deterioration are possible in all knees affected by poliomyelitis that have undergone total knee replacement. back pain. It is caused by rubella virus. An alternative approach that can eliminate these problems is the Ilizarov method.polio syndrome There are many drawbacks to using conventional approaches for the treatment of complex foot deformities.” Rubella is generally a benign. causing limb . Generalized fatigue may be treated with energy conservation. non fatiguing exercise in their rehabilitation is emphasized. Clinical manifestations & severity of illness vary with age. which is a member of the Rubivirus genus of the family Togaviridae. residual deformity & recurrence of deformity can complicate the Ilizarov method. For instance. Only one antigenic type of rubella virus is available & humans are the only natural hosts. relieve pain in arthritic joints & reduce the number of joints a weak muscle is acting across.loss programs & lower . Medical Therapy • • • • o o o • • • • Release of joint contractures Reestablish muscle balance around the joint to prevent deformities Muscle transplantation to replace a paralyzed muscle Stabilization of relaxed or flail joint by the following : Tenodesis Fixation of ligaments Construction of artificial check ligaments Arthrodesis Limb lengthening Ilizarov techniques Joint replacement surgery Arthrodesis is used to correct a deformity. but they appear to occur more commonly in more severely affected knees.

21 days after exposure to a person with rubella. History Pathophysiology Postnatal rubella The usual portal of entry of rubella virus is the respiratory epithelium of the nasopharynx. An infected individual begins to shed the virus from the nasopharynx 3 . The virus is transmitted via the aerosolized particles from the respiratory tract secretions of infected individuals. The virus can be transmitted to the fetus through the placenta & is capable of causing serious congenital defects. Mortality / Morbidity In contrast to postnatal rubella. synovial fluid & lungs. Regardless of the mechanism. The fetal defects observed in congenital rubella syndrome are likely secondary to vasculitis resulting in tissue necrosis without inflammation. Congenital rubella syndrome • o o o o § § § § § § § § § § 90 Fetal infection occurs transplacentally during the maternal viremic phase. urine. conversely. This may be the result of Postnatal rubella Exposure : Rubella virus is transmitted from person to person via the aerosolized particles from the respiratory tract. The following signs & symptoms usually appear 1 . Children may acquire the infection from a completely asymptomatic patient or from an individual shedding the virus during the incubation period. conjunctival sac. It then spreads hematogenously (primary viremia) to regional & distant lymphatics & replicates in the reticuloendothelial system. Incubation period : The incubation is usually 14 . abortions & stillbirths.• constitutional symptoms. The major complication of rubella is its teratogenic effects on fetus when pregnant women contract the disease. A history of exposure may not be present.14 days after onset of the rash. Another possible mechanism is direct viral damage of infected cells. which is not a debilitating disease. breast milk. The virus attaches to & invades the respiratory epithelium. rubella may be complicated by arthralgia. endocrinologic & neurologic abnormalities. adolescents & adults. It is demonstrated that cells infected with rubella in the early fetal period have reduced mitotic activity. cerebrospinal fluid (CSF). especially in the early weeks of gestation. mental retardation. During this viremic phase.5 days before the onset of rash : Eye pain on lateral & upward eye movement (a particularly troublesome complaint) Conjunctivitis Sore throat Headache General body aches Low . rubella virus can be recovered from different body sites including lymph nodes. congenital heart disease & eye. but the mechanisms by which rubella virus causes fetal damage are poorly understood. Viremia peaks just before the onset of rash & disappears shortly thereafter.8 days after exposure for 6 . learning disability. Prodromal phase : Prodromal symptoms are unusual in young children. chromosomal breakage or due to production of a protein that inhibits mitosis. any injury affecting the fetus in the first trimester (during the phase of organogenesis) results in congenital organ defects. hearing loss.grade fever Chills Anorexia Nausea Tender lymphadenopathy (particularly posterior auricular & suboccipital lymph nodes) .20 days after infection. arthritis & thrombocytopenic purpura. congenital rubella infection may result in growth delay. This is followed by a secondary viremia that occurs 6 . in older children. rash & suboccipital adenopathy.

blotchy. Hearing impairment may be bilateral or unilateral & may not be apparent until the second year of life.) Maternal history of immunization or medical history of rubella Evidence of intrauterine growth retardation during pregnancy Manifestations suggestive of congenital rubella syndrome in a child § § o § § Physical Postnatal rubella o § Rash The exanthem of rubella consists of a discrete rose .& . including late manifestations in congenital rubella syndrome usually occurring in the second or third decade of life (e. Mouth : The Forchheimer sign may still be present on the soft palate.5°C (101.pink maculopapular rash ranging from 1 . irregular pigmentation.old girl with a 4 . Lymph nodes : Enlarged posterior auricular & suboccipital lymph nodes are usually found on physical examination.pepper pigmentary change or a mottled. Image in a 4 . thyroid abnormalities. behavioral disorders. prematurity. meningoencephalitis & microcephaly Hepatosplenomegaly Jaundice Hepatitis Skin manifestations.day history of low . including blueberry muffin spots that represent dermal erythropoiesis & dermatoglyphics abnormalities Bone lesions.g.day measles” derives from the typical course of rubella exanthem that starts initially on the face & neck & spreads centrifugally to the trunk & extremities within 24 hours.year . Other findings in congenital rubella syndrome include the following : Intrauterine growth retardation. symptoms of an upper respiratory tract infection & rash.§ Forchheimer sign (an enanthem. such as radiographic lucencies Endocrine disorders. Rubella retinopathy consists of a salt . Congenital heart disease including patent ductus arteriosus (PDA) & pulmonary artery. hypotonia. usually with the greatest density in the macula. including mental retardation. diabetes mellitus) Hematologic disorders. § § § § § o o o • o § The synonym “3 . stillbirth & abortion CNS abnormalities. such as anemia & thrombocytopenic purpura Mycoplasma Infections Parvovirus B19 Infection Syphilis Toxoplasmosis Differential Diagnoses Contact Dermatitis Cytomegalovirus Infection Enteroviral Infections Herpesvirus 6 Infection Measles . Temperature : Fever is usually not higher than 38.4 mm. Ocular abnormalities including cataract.5°F). Congenital rubella syndrome The classic triad presentation of congenital rubella syndrome consists of the following : Sensorineural hearing loss is the most common manifestation 91 § § § of congenital rubella syndrome. It then begins to fade on the face on the second day & disappears throughout the body by the end of the third day.grade fever. encephalographic abnormalities. infantile glaucoma& retinopathy. consists of pinpoint or larger • o o o o petechiae that usually occur on the soft palate) Congenital rubella history focuses on the following : The number of weeks of pregnancy when maternal exposure to rubella occurred (The risk of congenital rubella syndrome is higher if maternal exposure occurs during the first trimester.

The laboratory diagnosis of rubella can be made either though serologic testing or by viral culture. Liver function tests. Imaging Studies • • o • • • A clinical diagnosis of rubella may be difficult to make because many exanthematic diseases may mimic rubella infection.specific immunoglobulin M (IgM) antibody in a single serum sample or observation of a significant (> 4 . aspartate aminotransferase. CBC count may reveal leukopenia & thrombocytopenia.glutamyl transpeptidase levels may reveal hepatic injury due to disseminated rubella infection.fold) rise in rubella specific immunoglobulin G (IgG) antibody titer between the acute & convalescent serum specimens drawn 2 . Among all the serologic tests available.specific IgG antibody levels are observed & do not decline at the rate expected from 92 o o o o Congenital rubella • Postnatal rubella : Imaging studies are usually not performed in postnatal rubella. as many as 50% of rubella infections may be subclinical. Echocardiography is important for patients with congenital heart defects to help diagnose the type of heart anomaly & evaluate the severity of the heart defect so that appropriate surgical plans can be made.related interstitial pneumonitis or pulmonary edema that may result from congestive heart failure in children with severe or complicated congenital heart anomalies. . Radiography of the long bones may reveal radiolucencies in the metaphyses of long bones. head retraction & arching of the back. irritability. rubella antigen detection by monoclonal antibody & polymerase chain reaction (PCR). lethargy. such as total & direct bilirubin.3 weeks apart. Congenital rubella in infants & children is diagnosed by viral isolation or by serologic testing. such as bulging anterior fontanel. especially in neonates. therefore. MRI of the head may reveal cortical atrophy & white matter changes in patients with late . laboratory studies are important to confirm the diagnosis of acute rubella infection. ELISA is the most widely used because it is relatively inexpensive. Congenital rubella syndrome can also be diagnosed using placental biopsy. urine.e. viral isolation is the preferred technique in congenital rubella syndrome because rubella serology may be difficult to interpret in view of transplacental passage of rubella . It is used to monitor the course of thrombocytopenia.onset progressive panencephalitis. Congenital rubella syndrome Chest radiography is indicated for infants who develop respiratory distress or other respiratory symptoms to exclude rubella . Congenital rubella syndrome should be strongly suspected in infants older than 3 months if rubella . cerebrospinal fluid & buffy coat of the blood. alanine aminotransferase. rapid & very sensitive. CT scanning of the head may reveal intracranial calcifications & enlargement of the ventricles.Mononucleosis & Epstein . Specimens used for viral isolation in congenital rubella include nasopharyngeal swab.Barr Virus Infection Laboratory Studies Postnatal rubella passive transfer of maternal antibody (i. seizures. hypotonia. technically easy to perform. equivalent of a 2 fold decline in HI titer per mo) in a compatible clinical situation.specific maternal IgG antibody. In addition. The serologic diagnosis consists of demonstrating the presence of rubella . Other Tests • • • Procedures • Lumbar puncture is indicated to evaluate for possible causes in children who develop signs & symptoms of meningoencephalitis. In contrast to postnatal infection. alkaline phosphatase & gamma .

atrial septal defect (ASD) & pulmonary artery stenosis. Surgical Care • • o o Congenital rubella syndrome Activity • • • o Activity in rubella can be maintained as tolerated. 28 Tetanus o o o • • o o Tetanus is an intoxication characterized by increased muscle tone & spasms caused by the release of the neurotoxin tetanospasmin by Clostridium tetani following inoculation into a human host. including generalized. Treatment of symptomatic newborns is as follows : Provide careful evaluation of the eyes & ophthalmology referral for babies with corneal clouding. Provide vision screening & hearing screening for asymptomatic newborns. if severe. rest is advised for patients who develop arthralgia or arthritis.inflammatory drugs (NSAIDs) may be helpful. Echocardiography may be essential for diagnosis of heart defects. IVIG may be considered in infants who develop severe thrombocytopenia. Babies with congenital rubella syndrome who develop respiratory distress may require supportive treatment in the ICU. consider intravenous immunoglobulin (IVIG).Medical Care Postnatal rubella • • • o o o Treatment is supportive. Tetanus occurs in several clinical forms. cephalic. encourage rest. cataract & retinal neovascularization. Postnatal rubella : Surgical care is not indicated. provide supportive care with adequate fluid & electrolyte maintenance. treatment is as follows : For severe arthritis affecting weight . Medication Drug therapy is currently not a component of the standard of care for rubella. Infants who have a rubella . localized & neonatal disease. Congenital rubella syndrome Surgical treatment may be required for congenital heart anomalies. Treatment is supportive. Starch baths & antihistamines may be useful for adult patients with uncomplicated rubella & troublesome itching. Coarctation of aorta. but corticosteroids are not indicated. No specific antiviral agent for rubella is currently available. Splenectomy is not indicated. For complicated cases. Most cases of tetanus are caused by direct contamination of Pathophysiology . Surgical treatment may be required for eye defects such as glaucoma. No intervention is required.bearing joints. cataract & retinopathy. Hepatosplenomegaly is monitored clinically. ventricular septal defect (VSD). however. Nonsteroidal anti . Corticosteroids are not indicated. including patent ductus arteriosus (PDA). For patients with encephalitis. Contact isolation is required for patients with congenital rubella during hospitalizations because babies are infected at birth & are usually contagious until older than 1 year unless viral cultures have produced negative results.related heart abnormality should 93 • be carefully observed for signs of congestive heart failure. Patients with hyperbilirubinemia may require phototherapy or exchange transfusions if jaundice is severe to prevent kernicterus. Corneal clouding may indicate infantile glaucoma. Thrombocytopenia is usually self . Corticosteroids have not demonstrated any specific benefit.limited but.

Finally. a foreign body. septic abortion.40% mortality rate. Wounds with low oxidation . This diminished inhibition results in an increase in the resting firing rate of the motor neuron. and. enters the axon.infection with other organisms develops). causing weakness & paralysis. surgical wounds.1 indicates mild severity with less than a 10% mortality rate. largely depending on . Prevalence Mortality / Morbidity A rating scale for the severity & the prognosis of tetanus is described below. The organism is found worldwide. via retrograde intra neuronal transport. Tetanospasmin. Localized tetanus develops when only the nerves supplying the affected muscle are involved. The blood .3 indicates moderate severity with a 10 . on inanimate objects. and. Score of 2 . Score one point for each of the following : • • • • • • • Incubation period less than 7 days Period of onset less than 48 hours Acquired from burns. The disease is common in areas where soil is cultivated. umbilical stump. is an anaerobic.6 indicates very severe tetanus with greater than a 50% mortality rate. C tetani. 94 • Generalized tetanus Generalized tetanus is the most commonly found form of tetanus. Neonatal tetanus is always very severe. in human feces.wounds with clostridial spores. or active infection. Infection by C tetani results in a benign appearance at the portal of entry because of its inability to evoke an inflammatory reaction (unless co . The etiologic agent of tetanus. Total score indicates the severity & the prognosis as follows : • • • • • • • History C tetani is found worldwide in soil. in animal feces. gram . colorless. The incubation period is 7 .21 days. such as those with dead or devitalized tissue.20% mortality rate. compound fractures.aminobutyric acid (GABA) by cleaving proteins crucial for the proper functioning of the synaptic vesicle release apparatus. The toxin migrates across the synapse to presynaptic terminals where it blocks the release of the inhibitory neurotransmitters glycine & gamma . One of these important proteins is synaptobrevin. tetanospasmin can block neurotransmitter release at the neuromuscular junction. Generalized tetanus develops when the toxin released at the wound spreads through the lymphatics & blood to multiple nerve terminals.brain barrier prevents direct entry of toxin to the CNS. is released in the wound & binds to the peripheral motor neuron terminal. tetanus predominantly develops in neonates & young children. reaches the nerve cell body in the brainstem & spinal cord. or intramuscular injection Narcotic addiction Generalized tetanus Temperature greater than 104°F (40°C) Tachycardia greater than 120 beats per minute (> 150 beats per minute in neonates) Score of 0 . In countries without a comprehensive immunization program. in rural areas. in warm climates.reduction potential. The spores may survive for years in some environments & are resistant to disinfectants & to boiling for 20 minutes. Score of 5 . during summer months & among males. motile. Score of 4 indicates severe tetanus with a 20 . are ideal for germination of the spores & release of toxin. Cephalic tetanus is always severe or very severe. which is responsible for the observed muscle rigidity.positive rod that forms an oval. a zinc metalloprotease. occasionally. terminal spore & assumes a shape that resembles a tennis racket or a drumstick.

rigidity. Cephalic tetanus • profound diaphoresis. These early manifestations reflect involvement of bulbar & paraspinal muscles.axonal antitoxin transport.abdominal process (due to rigid abdomen) Laboratory Studies • Generalized tetanus : Sustained trismus may result in the characteristic sardonic smile (risus sardonicus) & persistent spasm of the back musculature may cause opisthotonus. possibly because they are innervated by the shortest axons. It may remain localized or progress to generalized tetanus.2 days. Bacterial Rabies Other Problems to Be Considered Strychnine poisoning Dystonic drug reactions (e. Cephalic tetanus : Cranial nerve findings & rapid progression are typical.the distance of the injury site from the CNS. Physical Arthrogryposis Meningitis. This form may remain localized or progress to generalized tetanus. The mortality rate exceeds 70%. which is why it is sometimes referred to as the disease of the seventh day. The usual incubation period after birth is 3 . Trismus is the most common presenting symptom.10 days. Other early features include irritability. Aseptic Meningitis. • • 95 The diagnosis of tetanus is based entirely on clinical findings. hyperthermia. The usual cause is the use of contaminated materials to sever or dress the umbilical cord in newborns of unimmunized mothers. This is an unusual form of tetanus with an incubation period of 1 . metoclopramide) Hypocalcemic tetany Encephalitis Acute intra . Tetanus neonatorum Causes of tetanus include under immunization & the use of contaminated materials in newborn care Differential Diagnoses This is generalized tetanus that results from infection of a neonate. This is an unusual form of tetanus & the prognosis for survival is excellent. diaphoresis & dysphagia with hydrophobia. The newborn usually exhibits irritability. Localized tetanus • • Localized tetanus involves an extremity with a contaminated wound & widely varies in severity. In more severe cases.g. patients may have weakness of the involved extremity. Involvement of the autonomic nervous system may result in severe arrhythmias. Tetanus neonatorum : Physical examination findings are similar to generalized tetanus findings. Symptoms consist of isolated or combined dysfunction of the cranial motor nerves (most frequently the seventh cranial nerve). intense painful spasms occur & usually progress to generalized tetanus. Noise or tactile stimuli may precipitate spasms & generalized convulsions. The prognosis for survival is usually poor. presumably due to partial immunity. rhabdomyolysis. C tetani can be cultured from wounds of patients without tetanus . drooling & spasm of the back muscles. The condition may progress for 2 weeks despite antitoxin therapy because of the time needed for intra . facial grimacing & severe spasms with touch. Tetanus is unlikely if a reliable history indicates the completion of a primary vaccination series & the receipt of required booster doses. poor feeding. laryngeal spasm & urinary retention Localized tetanus : In mild cases. phenothazines. Wounds should be cultured in suspected cases. Causes Cephalic tetanus generally follows head injury or develops with infection of the middle ear. oscillation of the blood pressure. However. restlessness.

controlling disease manifestations & managing complications. 96 erythromycin. Wounds should be explored. Stopping toxin production : Antimicrobials are used to decrease • the number of vegetative forms (source of toxin) of C tetani in the wound. the patient should be sedated.15 mg/h is suitable). If ventilation is compromised. decrease rigidity & control spasms (midazolam administered intravenously at 5 . The current antimicrobial drug of choice is metronidazole. • o o Other Tests • • • Medical Care • The goals of treatment in patients with tetanus include initiating supportive therapy. intubated & provided with a soft nasal feeding tube. Nutrition should be intravenously supported. Clinical tetanus does not induce immunity against future attacks. Electromyography may show continuous discharge of motor subunits & shortening or absence of the silent interval normally observed after an action potential. Activity The patient should be on bedrest in a room that can be kept dark & quiet. creatine kinase. all patients should be fully immunized with tetanus toxoid during the convalescent period. Diet Patients should not be given any food by mouth because of the risk of aspiration. Managing complications : Specific therapy for autonomic system complications & control of spasms should be initiated. stopping the production of toxin within the wound. therefore. Controlling disease manifestations : A benzodiazepine should be used to produce sedation. Hypotension requires fluid replacement & dopamine or norepinephrine administration. Sympathetic hyperactivity is treated with combined alpha & beta blockade or morphine.6000 U is recommended for children & adults. Cerebrospinal fluid examination yields normal results. 29 .term neuromuscular blockade is required. neutralizing unbound toxin. Even the slightest physical stimulus can cause a cycle of spasms. If the spasms are not controlled with benzodiazepines. if necessary.01 or higher are considered protective & make tetanus unlikely. long . Consultation with a nutritionist is helpful. Epidural blockade with local anesthetics may be needed. although rarely cases have been reported despite the presence of protective antitoxin levels.g. Nonspecific changes may be evident on electrocardiography. A single total dose of 3000 . A tracheostomy may be needed. 000 100. aldolase) may be elevated. tetracycline & vancomycin. Neutralizing unbound toxin Human tetanus immunoglobulin (TIG) is recommended for treatment. Serum antitoxin levels of 0. The most susceptible wounds are grossly contaminated or caused by blunt trauma or bites. carefully cleansed & properly debrided. with penicillin as an alternative treatment.• • • • & frequently cannot be cultured from wounds of patients with tetanus. Serum muscle enzyme levels (e. debriding the wound to eradicate spores & alter conditions for germination. Tetanus antitoxin is administered as a single dose of 50. 000 U after appropriate testing for sensitivity & desensitization. The leukocyte count may be high. Initial supportive therapy : The patient should be placed in a • quiet room in an ICU. Other antimicrobials that have been used include clindamycin. Doses as low as 500 U are effective in infants with tetanus neonatorum. Wound debridement & care : The possibility of developing • tetanus directly correlates with the characteristics of the wound.

Transmission of M. It can lie dormant. usually by airborne Chance of transmission increases when the patient has an acid . By natural passages -a. The word “tuberculosis” refers to disease.M. 2) Ingestion – leads to abdominal tuberculosis. The presence of M. poor air circulation. Through swallowing (intestinal TB) e. growth can be detected in 1–3wk in selective liquid medium using radio labeled nutrients (the BACTEC radiometric system) & drug susceptibilities can be determined in an additional 3–5 days. adults do not transmit the organism within 2wk after beginning adequate chemotherapy. tuberculosis. Epidemiology 97 of open case. 4. Tuberculosis. Local spread – through macrophages 2. Tuberculosis disease occurs when signs & symptoms or radiographic changes become apparent. CD4 cells are sensitized by M.fast smear of sputum. no matter how mild the infection. • Mycobacterium bovis. Transbronchial spread c. tuberculosis is person to person. The agents of tuberculosis. Mold like appearance in culture –hence the name. The cell wall is rich in lipid. • Mycobacterium africanum.Tuberculosis Tuberculosis (TB) is the most common cause of infectious disease–related mortality worldwide. tuberculosis. Mode of Transmission 1) Inhalation – organisms present in fresh sputum or dried sputum • Mycobacterium tuberculosis. Delayed Type Hyper (DTH) sensitivity causes caseous necrosis. Species . M bovis – latter is resistant to PZI. children with tuberculosis rarely infect other children Spread of TB 1. When stained cell wall resists decolonization by acid. Haematogenous spread – spread through blood causing military TB. Cell mediated immunity protects host. A reactive tuberculin skin test & the absence of clinical & radiographic manifestations are the hallmark of this stage (latent tuberculosis infection LTBI). Lymphatic spread – primarily it is infection of lymphoid tissue. The majority of primary TB infections are so mild that they • may be completely unrecognized clinically. From lungs to pleura b. Renal to bladder Immunology Cell mediated immunity is most important. yet progression to frank disease is always possible at any stage in the life span. A lipid . It is a typical granulomatous inflammation reaction seen in • humans. The dangers of progression are greatest in the first two years of • life & for this reason treatment of every young child is desirable. – It produces cytokine which activates macrophages. 3. Childhood tuberculosis is a primary type of TB as infection • occurs for the first time with tubercle bacilli. The tubercle bacilli are strict aerobes. tuberculosis in clinical specimens can be detected within hours using PCR. • Tuberculosis infection occurs after the inhalation of infective droplet containing M.rich cell wall accounts for resistance to the bactericidal actions of antibody & complement. • Mycobacterium microti First described by Robert Koch in 1882. DTH has no relation . 3) Inoculation – occurs through skin 4) Tran placental – congenital TB in fetus from infected mother. Tuberculosis disease in infancy & childhood differs • considerably from that of adults. Into peritoneal cavity (intestinal TB) d. Liver is the primary site. Mycobacteria grow slowly.

3) Lymphadenitis – Enlarged hilar lymph nodes & trachea bronchial 98 Occurs in individuals who are previously infected. 2) Infected. TB is transmitted by airborne droplets. T lymphocytes secrete cytokines such as interferon gamma. Secondary tuberculosis 2) Primary infection occurs in lungs. Primary TB / Ghons complex / childhood TB Poor immunity & unfavorable environment Increased inflammation process (progressive primary TB) Consolidation (primary focus & regional lymph nodes merge) 3) Liquefaction . They may become dormant & remain asymptomatic. B lymphocytes & fibroblasts are among the cells that aggregate to form a granuloma.metastasis 7) Caseous node erode blood vessels . no disease. by secreting perforin & granulysin. or They may proliferate after a latency period (reactivation disease). Reactivation TB may occur following either (2) or (3) above. (Puhl’s lesion) Regional lymph nodes are not involved in chronic pulmonary . Pathophysiology lymph nodes. Upon encountering the bacilli.obstructive emphysema (incomplete) . Within the granuloma. with lymphocytes surrounding the infected macrophages. Common site of lesion is apex. but in whom there is no other evidence of TB. The bacilli have 4 potential fates 1) Primary focus + lymphatic drainage +lymph nodes Primary complex Good community & favorable environment Fibrosis Calcification 1) 2) 3) 4) • • They may be killed by the immune system. Macrophages. Tuberculosis in childhood constitutes a spectrum of 3 broad categories : 1) These are children who have been in contact with an open case of TB.atelectasis (complete obstruction) 5) Nodes erode bronchus . When inhaled. T lymphocytes. macrophages ingest & transport the bacteria to regional lymph nodes. They may multiply & cause primary TB.TB bronchitis . Cytotoxic T cells can also directly kill infected cells. TB exposure occurs by sharing common airspace with an individual who is in the infectious stage of TB. 4) Nodes compress bronchus . They are otherwise well.bronchogenic dissemination. Children above 7 yrs especially girls. May be endogenous or exogenous spread.with immunity.ray.cavity. 3) Clinically active – Here there has been progression of the primary complex & disease is clinically apparent.Simon focus in apex . Primary complex consists of 3 components 1) Primary focus – Lesion in the lung tissue at the entry of bacilli. lymph nodes. They show no evidence of infection but may well harbor Mycobacteria at the earliest stage. tuberculin negative & with a normal chest X . skin or GIT. 6) Haematogenous spread .spitted out .TB meningitis. Asymptomatic tuberculin reactors. which activates macrophages to destroy the bacteria with which they are infected. 2) Lymphangitis – The lymphatics which drain the lesion contain phagocytes with bacilli. These are children under 5 years who are tuberculin positive. The granuloma functions not only to prevent dissemination of the mycobacteria. Fate of primary complex Humans are the only known reservoir for Mycobacterium tuberculosis.miliary TB . droplet nuclei are deposited within the terminal airspaces of the lung. but also provides a local environment for communication of cells of the immune system.

Fever may be low .grade or absent. & pain. the hips & knees are affected most commonly. Patients with pulmonary TB occasionally present with hemoptysis or chest pain.The most common site of skeletal TB involvement is the spine (Pott disease). Symptoms of gastrointestinal TB are according to the site infected. 99 Physical examination findings associated with TB depend on the organs involved. Gastrointestinal TB . Another option is fiberoptic bronchoscopy with transbronchial biopsy & bronchial washings. The most common findings of postnatal TB include adenopathy & a lung infiltrate.Any site along the gastrointestinal tract may become infected. liver.morning gastric aspirate may also produce a good specimen in children. or blood cultures is occasionally necessary & may be helpful. In women. Cutaneous TB . difficulty swallowing with esophageal disease. Clinical Pulmonary tuberculosis .3 weeks. neurologic deficit. diarrhea. including the following : nonhealing ulcers of the mouth or anus. Tuberculous meningitis . In men. genital TB may mimic pelvic inflammatory disease.TB. Sputum is not readily obtainable from children & gastric • washing or other techniques must be used in order to obtain material.usually involves only one joint. only sporadically. the chest radiographic findings may be normal in infants with disseminated disease. or not at all. fatigue & night sweats. Biopsy of bone marrow. Signs may include confusion.Typical symptoms of pulmonary TB • Physical • • • • • • • include a productive cough. especially over the upper lobes or areas involved. Tuberculous lymphadenitis (scrofula) . wrist & shoulder. It is usually unilateral & causes little or no pain. abdominal pain mimicking peptic ulcer disease with stomach or duodenal infection. Other systemic symptoms include anorexia. Mycobacteria are excreted in a child’s sputum in small number. Tuberculous arthritis .Reported symptoms of genitourinary TB include flank pain. dysuria & frequency. Signs of extrapulmonary TB differ depending on the tissues involved. Early . Symptoms include back pain or stiffness. False positives are frequent on direct smear of gastric washings • & results of culture may only be available after some weeks. elbow.The most common site of tuberculous lymphadenitis is in the neck along the sternocleidomastoid muscle. Laboratory Studies In contrast to adults. Genitourinary TB . fever & weight loss. or hematochezia with infection of the colon. Newer technologies. allow identification within 24 hours. Other laboratory tests • • CBC count Alanine aminotransferase (ALT) or aspartate aminotransferase (AST) . Patients with pulmonary TB have abnormal breath sounds.Direct inoculation may result in an ulcer or wartlike lesion. lymphadenopathy & cutaneous lesions Postnatal TB is contracted via the airborne route. Skeletal TB . followed by the ankle. chorioretinitis. Sputum should be collected in the early morning on 3 consecutive days.Patients with tuberculous meningitis may present with a headache that is either intermittent or persistent for 2 . Traditional mycobacterial cultures require weeks for growth & identification. malabsorption with infection of the small intestine. genital TB may manifest as epididymitis or a scrotal mass. Although any joint may be involved. However. including ribosomal RNA probes or DNA polymerase chain reaction. coma.

silicosis.1ml of PPD) of purified protein derivative. is diagnostic. the criteria for each cutoff point are listed below. The size of induration. False negative test – Poor technique of administration Biased / misreading of results PEM Infections Drugs like steroids Immunosuppressed state. 2) Tuberculin skin testing (Mantoux test) False + 6 – 9 mm + 10 – 14 mm ++ 15 – 20 mm +++ 21 – 30 mm ++++ > 30 mm or necrosis. the best diagnostic test is the examination of the placenta for pathology. a ‘proper’ intradermal tuberculin test should be done. local vasodilation & edema. gastrectomy) Recent converter . If the result is doubtful. In reaction to this T cells which were sensitized by prior infection accumulate at the site & release lymphokines which produce induration. Mycobacterial blood cultures of the newborn may also be helpful. a) Larger than or equal to 5 mm • • • • • • Grade It is the most widely available test for diagnosing tuberculous infection in the absence of active disease (latent infection). The response is measured as the amount of induration at 48 72 hours. malnutrition. Treatment may be necessary until placental culture results are negative Other Tests 1) Tine / Monotest • • • • • A positive Tine is one where 2 or more tine papules have run together to form a larger papule or blister. age & patient risk factors. but not active tuberculosis. hematologic malignancies.mm increase in skin test in past 2 years (regardless of age) Recent immigrants (within 5 y) from a high . The tuberculin skin test involves an intradermal injection of 5 tuberculin units (0. Positive montoux test indicates prior infection by TB bacilli. A Tine is only a screening test.g.Alkaline phosphatase Total bilirubin Uric acid Creatinine HIV serology in all patients with TB. diabetes mellitus. 3 cutoff points of clinical significance exist.stage renal disease. Atypical mycobacterial infection. intravenous drug use [known to be HIV negative]. histology & culture.prevalence country . jejunoileal bypass. size of induration • • • • b) Close contacts to persons with newly diagnosed TB Persons with HIV infection Patients with organ transplant or patients who are taking the equivalent of more than 15 mg/d of prednisone for one month or more Patients with fibrotic lesions on chest radiography (not granulomas) Larger than or equal to 10 mm • Interpretation • • 100 < 5mm Patients with medical conditions that increase the risk of TB (e. False positive test – Previous BCG vaccination. rather than erythema. carcinoma of the head & neck. Interpretation of skin testing depends on the size of induration. The tuberculin skin test is not a sensitive test for active TB. end .At least 10 . For congenital TB. All reactions less than this are ‘negative’ or ‘doubtful’.

c) Tuberculosis Persons with none of the above TB may be discovered incidentally in association with other chronic disorders such as HIV infection & malaria. homeless shelters & mental institutions Larger than or equal to 15 mm • different observers. Grades – Negative . History of contact : A history of possible contact with TB is • . should be done. Grade 2 . Various patterns may be seen. Hence detection of bacilli by AFB staining & culture by gastric aspirate. TB may be found in any part of the lung. Intradermal shots given & reading is taken between 3 – 7 days. There is a wide “grey zone” of X . The lordotic view may better demonstrate apical abnormalities.. Tuberculosis • • • PA view showing collapse – consolidation of right upper lobe.3 wks. Any patient with HIV should be suspected of having TB & an intensive search made for clinical & laboratory evidence. o Miliary TB is characterized by the appearance of numerous small nodular lesions that resemble millet seeds on chest radiography. CT scanning of the chest may help to better define abnormalities in patients with vague findings on chest radiography. Grade 3 . 3) Heaf test .• • Children younger than 4 years exposed to adults at high risk for TB Residents & employees of facilities for long .Useful for mass testing.popular induration at least 4 sites. B. Poor weight gain or weight loss : very common in many • communities for other reasons.ray • • This radiograph shows a patient with typical radiographic findings of tuberculosis. including correctional institutions.acquired pneumonia on chest radiography. are virtually diagnostic of TB. either may be associated with pleural effusion or cavitation. The following are the factors to be considered : symptoms : cough. • Signs : painless lymph node enlargement. a miliary pattern. 5) BACTEC radiometric system – Mycobacterial growth can be detected in 1 .ring formation of at least 6 sites.ray changes which may or may not represent TB & are often interpreted differently by 101 This radiograph shows a patient with typical Primary TB is more likely to mimic the appearance of routine • community .lobe involvement is most common. such as large hilar glands. in contrast to reactivation T. but upper . A lateral film is essential to assess hilar nodes. Chest radiographs may show a patchy or nodular infiltrate (as seen in the image below). tiredness etc. o Noncalcified round infiltrates may be confused with lung carcinoma. or narrowed airways. 6) PCR – Using DNA or RNA probes. nursing homes.term care. hilar lymphadenopathy Changes. wheezing & other • chest signs may or may not be present. as follows : • o Cavity formation indicates advanced infection & is associated with a high bacterial load. have many other causes. fever.vesicle formation or ulceration. urine etc.ertyhema without induration Grade 1 .20 mm) are tuberculomas & represent old infection rather than active disease. 7) Chest X . Multiple puncture test using spring loaded gun technique. o Homogeneously calcified nodules (usually 5 . M tuberculi can be detected in hours. 4) AFB sputum test – In children sputum production is nil or minimal.plaque formation Grade 4 . with or without infiltrates in the lungs.

Ampicillin Bactericidal – rifampicin (R). also known as enteric fever. an ethereal smoke or cloud that was believed to cause disease & madness. the patient’s level of consciousness is truly clouded. or if no supervision is possible. with no disease should receive INH & RIF under direct observation for 3 months. Ethambutol Second line drugs Cycloserine. typhoid fever is a serious illness that may progress to delirium. or at least 5 days a week. Streptomycin.6 months. A mildly positive reaction in a malnourished or immuno • compromised child should always be viewed with suspicion.ethionamide. crowding & social chaos. is a potentially fatal multisystem illness caused primarily by Salmonella typhi. PAS. ethambutol. If untreated. bowel perforation & death. pyrazinamide (P). malaise. But it does not necessarily mean that active disease is present. • • contained in tablets which may be chewed or dissolved in 5 ml of water. relative bradycardia. In the advanced stages of typhoid fever. These are now easy to administer as special formulations are available for children. obtundation. Short Course . Marked nodal enlargement or consolidation • Bronchopneumonic spread • Bone/joint • Abdominal & glandular • 6 months of treatment is required. A 4th drug . Ethionamide. diffuse abdominal pain & constipation. paratyphi B & S paratyphi C can cause paratyphoid fever in humans.e. S typhi has been a major human pathogen for thousands of years. . isoniazid (INH). Rifamycin. thriving in conditions of poor sanitation.treatment or Suspected resistance Finally children at risk & those infected. The classic presentation includes step ladder type fever. streptomycin (S) Bacteriostatic Drugs First line drugs - • • Ethambutol (E).DOTS) Thrice weekly regimens requiring higher drug dosages may be used where the more desirable daily schedules are not possible. PAS All children diagnosed with probable TB should receive INH. This is achieved by extending the period on INH & RIF to 4 MONTHS.elicited very frequently. Another source of confusion is the almost universal • administration of BCG vaccination which induces variable skin reactivity for at least two years. rose spots. INH only for 3 . or streptomycin is added for part or all of the 6 month period in the case of – Miliary disease. i. Treatment is given daily. 30 Typhoid fever • • • Treatment Effective with minimal toxicity Isoniazid. intestinal hemorrhage. The name S typhi is derived from the ancient Greek typhos. The drugs are 102 • Typhoid fever. Amikacin. S. Imipenem. Rifampicin. In extensive or disseminated disease. S.These are used in drug resistant case Quinolones. Re . Kanamycin Others . paratyphi A. A reactive tuberculin test : A positive reaction indicates that infection with tubercle bacilli has occurred. But a negative reaction certainly does not rule out the presence of active tuberculous disease. under supervision (Directly Observed Therapy. Pyrazinamide. Capreomycin. leucopenia. Meningitis. RIF & PZA for the initial 2 months. This is followed by INH & RIF for a further 4 MONTHS as recommended by WHO but may not be essential if the child has no localizing signs of TB & responds well to the 2 months of treatment.

aged children & young adults.2 receptor antagonists (H2 blockers). The fever pattern is stepwise. less commonly.contaminated water or shellfish • Pathophysiology • extension of S typhi –infected bile. In contrast to the nontyphoidal salmonellae. S typhi enters the host’s system primarily through the distal ilium. drinking unpurified water & living in a household that does not have a toilet. characterized by a rising temperature over the course of each day that drops by the subsequent morning. However. ranging from a mild febrile illness to severe convulsions & the S typhi infection may go unrecognized.Etiology S typhi has no nonhuman vectors. It co . . the main relay point for macrophages traveling from the gut into the lymphatic system. Afterward.opts the macrophages’ cellular machinery for their own reproduction as it is carried through the mesenteric lymph nodes to the thoracic duct & the lymphatics & then through to the reticuloendothelial tissues of the liver.5. S typhi are able to survive a stomach pH as low as 1.enters the gastrointestinal tract in the bile & reinfects Peyer patches. All pathogenic Salmonella species are engulfed by phagocytic cells. The bacteria then induce their host macrophages to attract more macrophages. gastrectomy & achlorhydria decrease stomach acidity & facilitate S typhi infection. Classic typhoid fever syndrome • Typhoid fever begins 7 .14 days after ingestion of S typhi. The gallbladder is then infected via either bacteremia or direct 103 • Age • • • History A severe nonspecific febrile illness in a patient who has been exposed to S typhi should always raise the diagnostic possibility of typhoid fever (enteric fever). The following are modes of transmission : Oral transmission via food or beverages handled by an • individual who chronically sheds the bacteria through stool or. Nontyphoidal salmonellae are phagocytized throughout the distal ilium & colon. the true incidence among very young children & infants is thought to be higher.mouth transmission after using a contaminated toilet • & neglecting hand hygiene Oral transmission via sewage . The presentations in these age groups may be atypical. bone marrow & lymph nodes. spleen. breaking out into the bloodstream to invade the rest of the body.to . living in the same household with someone who has new case of typhoid fever. urine Hand . which then pass them through the mucosa & present them to the macrophages in the lamina propria. the bacteria induce macrophage apoptosis. the S typhi bacteria pause & continue to multiply until some critical density is reached. S typhi has specialized fimbriae that adhere to the epithelium over clusters of lymphoid tissue in the ilium (Peyer patches). The result is that the organism re . Once there. proton pump inhibitors. sharing food from the same plate. histamine . Risk factors • • • • • • • • The incubation period is 14 days with a range limit of 3 to 60 days. Environmental & behavioral risk factors that are independently associated with typhoid fever include eating food from street vendors. Most documented typhoid fever cases involve school . washing the hands inadequately. Bacteria that do not reinfect the host are typically shed in the stool & are then available to infect other hosts. Antacids.

especially in India & Africa. liquid diarrhea (pea soup diarrhea). mental 104 state & abdominal distension slowly improve over a few days. green . In addition. the still febrile individual grows more toxic & anorexic with significant weight loss. In most contemporary presentations of typhoid fever.colored. Young children. the notorious gastrointestinal manifestations of the disease develop. the fever plateaus at 103 . acute lobar pneumonia. orchitis. isolated arthralgias. These are bacterial emboli to the dermis & occasionally develop in persons with shigellosis or nontyphoidal salmonellosis. race factors & the infecting bacterial strain. fierce colicky right upper quadrant pain. Weight loss & debilitating weakness last months. osteomyelitis & abscesses anywhere on the body. present primarily with neurologic manifestations such as delirium or. the fever has a steady insidious onset. urinary symptoms. these generally resolve within 2 . or fever alone. which is characterized by apathy. The abdomen becomes distended & soft splenomegaly is common.• • • • • • • • • Over the course of the first week of illness. The stepladder fever pattern that was once the hallmark of typhoid fever now occurs in as few as 12% of cases. in some localities. myocarditis. Some patients. the second beat weaker than the first) may develop.4 cm wide & fewer than 5 in number. the disease begins to remit after about 2 days & the patient’s condition markedly improves within 4 .5 days. The individual may descend into the typhoid state.40°C). Atypical manifestations of typhoid fever include isolated severe headaches that may mimic meningitis. Various presentations of typhoid fever • • • • • The clinical course of a given individual with typhoid fever may deviate from the above description of classic disease. Treated typhoid fever If appropriate treatment is initiated within the first few days of full . The individual then develops a dry cough. This complication is often unheralded & may be masked by corticosteroids. causing constipation that lasts the duration of the illness. dull frontal headache. These include diffuse abdominal pain & tenderness and. Intestinal & neurologic complications may still occur in surviving untreated individuals. Relative bradycardia & dicrotic pulse (double beat.Barré syndrome.104°F (39 . The patient develops rose spots. or intestinal hemorrhage may cause death. The timing of the symptoms & host response may vary based on geographic region. individuals with AIDS & one third of immunocompetent adults who develop typhoid fever develop diarrhea rather than constipation.blown illness.yellow. Some patients experience foul. delirium & an increasingly stuporous malaise At approximately the end of the first week of illness. in some cases. The tongue is often coated in the centre (coated tongue)and is clear at the margins. in extremely rare cases. truncal. Abdominal distension is severe. Parkinsonian symptoms or Guillain . typhoid fever is generally more apt to cause diarrhea than constipation. Some survivors become asymptomatic S typhi carriers & have the potential to transmit the bacteria indefinitely. blanching. At this point. overwhelming toxemia. the fever. severe jaundice. If the individual survives to the fourth week. which are salmon . Monocytic infiltration inflames Peyer patches & narrows the bowel lumen. The conjunctivae are infected & the patient is tachypneic with a thready pulse & crackles over the lung bases. Necrotic Peyer patches may cause bowel perforation & peritonitis. Other unusual complications include pancreatitis. In the third week. confusion & even psychosis. maculopapules usually 1 .5 days. meningitis. the signs & symptoms listed above progress. Any . During the second week of illness.

The Widal test was the mainstay of typhoid fever diagnosis for • decades. Delirium. o Mild hyponatremia & hypokalemia are common. Feces & urine must be disposed . ureters & bladder (KUB) is useful if bowel perforation (symptomatic or asymptomatic) is suspected. It is used to measure agglutinating antibodies against H & O antigens of S typhi. while ratio of less than 9 :1 supports typhoid hepatitis. among other sites. Medical Care Treatment should not be delayed for confirmatory tests since prompt treatment drastically reduces the risk of complications & fatalities.30% later in the disease course. Neither sensitive nor specific. or shock demands a particularly aggressive approach. stool culture may be positive for S typhi several days after ingestion of the bacteria secondary to inflammation of the intraluminal dendritic cells. Compliant patients with uncomplicated disease may be treated on an outpatient basis. marked neuropsychiatric symptoms. They must be advised to use strict handwashing techniques & to avoid preparing food for others during the illness course. stool culture results are positive because of bacteria shed through the gallbladder. Hospitalized patients should be placed in contact isolation during the acute phase of the infection. They decline to 20% . stupor. serious abdominal discomfort.90% of patients with typhoid fever who present within the first week of onset. Complicated typhoid fever is characterized by melena. A ratio of greater than 9 :1 supports a diagnosis of acute viral hepatitis. thrombocytopenia & relative lymphopenia. Cultures are widely considered 100% specific. or other severe manifestations. In particular. Depending on the adequacy of diagnosis & treatment. the Widal test is no longer an acceptable clinical method. intestinal secretions (vomitus or duodenal aspirate) & stool culture results are positive for S typhi in approximately 85% . Culture • o The criterion standard for diagnosis of typhoid fever has long been culture isolation of the organism.delay in treatment increases the likelihood of complications & recovery time. have an elevated erythrocyte sedimentation rate (ESR). o Blood. Other nonspecific laboratory studies • o Most patients with typhoid fever are moderately anemic. Polymerase chain reaction (PCR) : PCR has been used for the • diagnosis of typhoid fever with varying success. coma. 105 A serum alanine amino transferase (ALT)–to–lactate dehydrogenase (LDH) ratio of more than 9 :1 appears to be helpful in distinguishing typhoid from viral hepatitis. Antibiotic therapy should be narrowed once more information is available. CT scanning & MRI : These studies may be warranted to investigate for abscesses in the liver or bones. Laboratory Studies o The diagnosis of typhoid fever (enteric fever) is primarily clinical. obtundation. o Liver transaminase & serum bilirubin values usually rise to twice the reference range. Imaging Studies • • Procedures Bone marrow aspiration : The most sensitive method of isolating S typhi is BMA culture. Radiography : Radiography of the kidneys. o Most also have a slightly elevated prothrombin time (PT) & activated partial thromboplastin time (aPTT) & decreased fibrinogen levels. Staging The proper treatment approach to typhoid fever depends on whether the illness is complicated or uncomplicated. complicated disease may develop in up to 10% of treated patients. intestinal perforation. Later in the illness.

20 mg/kg/d PO.line agents for complicated cases.of safely.50 mg/kg/d PO divided q8h for 14 d 106 . in the late 1980s. as a second . as is true of all herpes viruses. 20 . < 2 months : Do not administer > 2 months : 15 . For complicated typhoid fever. Ampicillin & trimethoprim . the IAP recommends cefixime and. Chloramphenicol Arrests bacterial cell wall synthesis. The varicella .75 mg/kg/d PO/IV divided q6h Amoxicillin 31 32 33 Chicken pox • Chickenpox is caused by the varicella .zoster virus is a member of the human herpesvirus subfamily Alphaherpesvirinae and. However.sulfamethoxazole (TMP . Aztreonam & imipenem are second .generation cephalosporin with broad . not to exceed 500 mg/d Days 2 .zoster virus. resistance to first .75 mg/kg/d IV/IM divided q12h. resulting in bactericidal activity against susceptible bacteria. 50 . tid/qid for 14 d Cefotaxime Surgery is usually indicated in cases of intestinal perforation. not to exceed 125 mg/d Infants & children : 50 . Fluoroquinolones are now the most recommended antibiotics for the treatment of typhoid fever. not to exceed 250 mg/d Ceftriaxone Third . the gallbladder should be resected. They are highly effective against susceptible organisms.generation fluoroquinolones is widespread in many parts of Asia. For empiric treatment of uncomplicated typhoid fever. Unfortunately. not to exceed 2 g/d Binds to 50S bacterial . based on TMP.50 mg/kg/d IV/IM. < 6 months : Not established > 6 months Day 1 : 10 mg/kg PO once. they recommend ceftriaxone. is a Interferes with synthesis of cell wall mucopeptides during active multiplication. yielding a better cure rate than cephalosporins.180 mg/kg/d IV/IM divided q4 .SMZ) then became treatments of choice.ribosomal subunits & inhibits bacterial growth by inhibiting protein synthesis.spectrum gram negative activity against gram .positive organisms.mediated resistance to all three of these agents. 200 mg/kg/d IV in divided doses for 14 d Infants & children : 50 . when widespread resistance occurred. > 7 days : 25 . which inhibits bacterial growth. Surgical Care Trimethoprim & sulfamethoxazole Inhibits bacterial growth by inhibiting synthesis of dihydrofolic acid. azithromycin. some S typhi & S paratyphi strains (multidrug resistant [MDR] S typhi or S paratyphi) developed simultaneous plasmid .6h > 12 years : Administer as in adults Azithromycin Treats mild to moderate microbial infections. Antibiotics History of antibiotic resistance Chloramphenicol was used universally to treat typhoid fever from 1948 until the 1970s. If antibiotic treatment fails to eradicate the hepatobiliary carriage.line agent.5 : 5 mg/kg PO qid.

self . Papules & vesicles. have high populations of the virus. Transmission • • • • 107 An example of varicella is shown in the image below. The disease is generally regarded as a mild. Varicella is highly contagious. Varicella is associated with humoral & cell .exposure & subclinical infections may serve to boost the immunity acquired after an episode of chicken pox.6 days later. Direct person . secondary attack rates range from 80 . The disease can be serious in neonates. The pleomorphic rash characteristic of varicella Papules. This viremia also spreads the virus to respiratory sites & is responsible for the contagion of varicella before the appearance of the rash. After a week. Persons older than 14 years account for 10% of varicella cases. Second cases within the household are often more severe.person contact with lesions also spreads the virus.90% for household contacts. usually 5 days later.mediated immune responses. making the disease highly contagious even before the rash appears. pneumonia. The virus initially replicates in the regional lymph nodes.limiting viral illness with occasional complications. 4 . The mortality rate in children who are immunocompromised is much higher.to . a primary viremia spreads the virus to reticuloendothelial cells in the spleen. Mortality / Morbidity Most deaths are from associated encephalitis. The disease presents with fever & blistered rash. In children who have recovered from chickenpox. Repeat subclinical infection can occur in these persons. eliciting the typical skin lesions. Infection of the CNS or liver also occurs at this time. a secondary viremia disseminates the virus to the viscera & skin. but not the crusts. depending on the timing of infection in the mother. Herpes Zoster (Shingles) is a painful localized rash caused by the varicella virus. the virus remains inactive (dormant) in certain nerve roots & can be activated spontaneously after several months or years.6 years. Shingles usually afflicts adults’ especially old people. vesicles & pustules are concurrently present. Transmission involves the following : Transmission occurs mainly through respiratory droplets that contain the virus. It may occur on the trunks or limbs & characteristically involves only one side of the trunk. The rash is similar to the rash of chicken pox (fluid filled blisters) but is more crowded & extremely painful. Age The maximum incidence of varicella in unvaccinated populations is in children aged 1 . secondary bacterial infection & Reye syndrome. . Maximum transmission occurs during late winter & spring. Re . but second attacks of chickenpox are extremely rare in immunocompetent persons. These responses induce long .zoster virus enters through the respiratory system & colonizes the upper respiratory tract.• • • • • • • • • DNA virus. Household transmission rates are 80 . The infectious period begins 2 days before skin lesions appear & ends when the lesions crust.lasting immunity. liver & elsewhere.90%. Pathophysiology • • • • The varicella . Children who are susceptible rarely acquire the disease by contact with adults with zoster. The virus then remains latent or dormant in the body. Among the most serious complications are varicella pneumonia & encephalitis.

The disease is infectious a day before the rash appears. Redness or swelling around a lesion should lead to suspicion of bacterial superinfection. Risk factors Neonatal period • o o o o o o o o o Prodrome • • A neonate’s first month of life is a susceptible period for severe varicella.2 days Some children complain of abdominal pain Rash. Delivery before 28 weeks’ gestation also makes a baby susceptible because transplacental transfer of immunoglobulin G (IgG) antibodies occurs after this time.34 d). malignancy. Incubation : The incubation period is typically 10 .e. or school. especially if the mother is seronegative.14 days. Most patients have a history of exposure in the home. pustule & crust.500 lesions but may have as few as 10 or as many as 1500. Immunocompromised state (e.grade fever preceding skin manifestations by 1 . anti malignancy drugs. Malignancy : All children with cancer have an increased risk for severe varicella.free virus particles derived from skin lesions or the respiratory tract. An otherwise healthy child usually has 250 . Some lesions may appear in the oropharynx. usually starting on the head & trunk & spreading to the rest of the body Typically. daycare center.• Maternal varicella with viremia can transplacentally spread to the fetus. Even short . The characteristic rash appears in crops. complaints of intense pruritus Headache Malaise Anorexia Cough & coryza Sore throat Rash Physical • o o o o o o o o o o o • • History • o Exposure o • Varicella spreads primarily by airborne droplets.12 d) & completely heal by 16 days (range 7 . Each lesion starts as a red macule & passes through stages of papule. Steroid therapy : High doses (i.102°F) but may be as high as 106°F. Fever • o 108 Fever is usually low grade (100 . doses equivalent to 1 . Eye lesions are rare. The infectious particles are cell . The hallmark is the simultaneous presence of different stages of the rash. .g. Lesions usually crust by 6 days (range 2 . The risk is highest for children with leukemia.2 mg/ kg/d of prednisolone) for 2 weeks or more are definite risk factors for severe disease.term therapy at these doses immediately preceding or during the incubation period of varicella can cause severe or fatal varicella. This leads to neonatal varicella. human immunodeficiency virus [HIV]. The vesicle on a lesion’s erythematous base leads to its description as a pearl / dewdrop on a rose petal. vesicle. other congenital or acquired immunodeficient conditions) : Defects of cellular but not humoral immunodeficiency make a person susceptible to severe varicella.5 days. The following may be noted with varicella : Adolescence & adulthood • Low . New lesions continue to erupt for 3 . Prolonged eruption of new lesions or delayed crusting & healing can occur with impaired cellular immunity. although it may extend to 21 days.

Most children with varicella have leucopenia in the first 3 days. Radiography may also detect focal infiltrates suggestive of secondary bacterial pneumonia. The cause is maternal varicella infection after the 20 weeks’ gestation. Imaging Studies • • • Chest radiography may be indicated. cataracts. This syndrome manifests as intrauterine growth retardation. limb hypoplasia. Infantile zoster Contact Dermatitis Enteroviral Infections Herpes Simplex Virus Infection Impetigo Urticaria Measles Drug reactions Insect bites Laboratory Studies o § § § o Infantile zoster usually manifests within the first year of life.zoster immune globulin (VZIG) & acyclovir. • followed by leucocytosis. the mother develops antibodies against the varicella virus. Differential Diagnoses § § Congenital varicella syndrome occurs in 2% of children born to women who develop varicella during the first or second trimester of pregnancy. Onset of maternal varicella more than 5 days antepartum provides the mother sufficient time to manufacture & pass on 109 Laboratory studies are unnecessary for diagnosis because varicella is clinically obvious. Prophylaxis or treatment is required with varicella . cortical atrophy. Chest radiographic findings may be normal or may show diffuse bilateral nodular infiltrates in primary varicella pneumonia. microphthalmia. Marked leucocytosis may indicate a secondary bacterial • infection but is not a dependable sign. Children with high temperatures & respiratory signs should have chest radiography to confirm or exclude pneumonia. Immunohistochemical staining of skin lesion scrapings can • confirm varicella. Neonatal varicella § § § § Neonatal varicella can be a serious illness. Infantile zoster commonly involves the thoracic dermatomes. depending on the timing of maternal varicella & delivery. Congenital varicella syndrome antibodies along with the virus. chorioretinitis & cutaneous scarring. In utero infection : Outcomes of in utero varicella infections vary based on the timing of the infection. The severity of the disease in the newborn depends on whether transplacental passage includes only the virus or includes both the virus & the antibodies. After the initial viremia. Full . . Prolonged fever should prompt suspicion of complication or immunodeficiency. the baby is exposed to the secondary viremia of the mother. If the mother develops varicella within 5 days before or 2 days after delivery. Polymerase chain reaction (PCR) examination of skin scrapings • is quick & sensitive. microcephaly. Most children with significant secondary bacterial infections do not have leucocytosis.term neonates of these women usually have mild varicella because of the attenuating effect of the transplacentally acquired antibodies. fever typically subsides within 4 days.o o • o In otherwise healthy children. The baby transplacentally acquires the virus but acquires no protective antibodies because of insufficient time for antibodies to develop in the mother.

Nonsteroidal anti .threatening varicella complications may require hospitalization in an ICU.positive bacillus. Trimming the child’s fingernails & having the child wear mittens while sleeping may reduce scratching. The 3 isolated strains of C diphtheria include gravis. Discourage scratching to avoid scarring. It is always used for complications of varicella (e. Activity Antihistamine has a sedating effect & is effective for pruritus 5 mg/kg/d PO divided tid/qid. localized cutaneous infections & rarely systemic infection. not to exceed 3200 mg/d Fever is usually low grade but may be elevated. Children who are most susceptible to infection are those who are not completely immunized or have low antitoxin antibody levels & have been exposed to a carrier or diseased individual. Diet encephalitis. nonmotile. Acetaminophen is probably the safest drug to use for this purpose. C diphtheria is a nonencapsulated. Corynebacterium diphtheria. Medical Care Manage pruritus in patients with varicella with cool compresses & regular bathing. pneumonia) & for immunocompromised individuals with varicella. 80 mg/kg/d PO divided in 4 .Other Tests – Lumbar puncture Children with neurological signs should have their cerebrospinal fluid (CSF) examined. Children who develop severe & life . Acyclovir Antiviral that acts by inhibiting herpes virus DNA polymerase & terminating viral replication. It reduces the number of lesions & duration of fever if started within 24 h of appearance of rash. It does not affect incidence of pruritus. complications. 110 • Diphtheria manifests as either an upper respiratory tract or cutaneous infection & is caused by the aerobic gram . intermedius & mitis. It is communicable for 2 . Adequate hydration is especially important if the child is receiving acyclovir because the drug can crystallize in the renal tubules if administered to dehydrated individuals. Salicylate usage for varicella is associated with Reye syndrome. A carrier is someone whose cultures are positive for the diphtheria species but does not exhibit signs & symptoms.5 doses for 5 d. Other groups that require specific treatment are children who are immunocompromised.6 weeks without antibiotic treatment. those who are otherwise at risk for severe disease & those who already have severe disease. Pathogenic strains can result in severe localized upper respiratory infection.positive bacteria. .inflammatory drugs (NSAIDs) have been suspected of suppressing immune function & promoting infection progress in patients infected with invasive group A streptococci. or secondary transmission. Some children with varicella have reduced appetite & should be encouraged to take sufficient fluids to maintain hydration. therefore. gram . never prescribe these agents.g. The infection usually occurs in the spring or winter months. Acyclovir is the drug of choice for these situations. Medication Antiviral • • • • • Chicken pox is usually a benign disease in children & almost all children recover uneventfully. Diphenhydramine Advise parents to provide a full & unrestricted diet to the child. not to exceed 300 mg/d 34 Diphtheria No activity restrictions are needed for young children with uncomplicated varicella.

4 secondary to asphyxia with a pharyngeal membrane or due to myocarditis. fibrin. Local tissue destruction enables the toxin to be carried lymphatically & hematologically to other parts of the body. C diphtheria adheres to mucosal epithelial cells where the exotoxin.grade fever (rarely > 103°F)and chills Malaise. Removal of the membrane reveals a bleeding. The pseudo membrane is characterized by the formation of a • dense. weakness. affecting populations younger than 12 years. The distribution of the membrane varies from local (e.g. RBCs. kidneys & nervous system. Infants become susceptible to the disease at age 6 . nonhealing ulcers covered with a gray membrane. released by endosomes. 1 . Age Onset of symptoms of respiratory diphtheria typically follows an incubation period of 2 . A combination of cervical adenopathy & swollen mucosa • imparts a bull’s neck appearance to many of the infected patients The most frequent cause of death is airway obstruction or • suffocation following aspiration of the pseudo membrane. Most deaths occur on day’s 3 . gray debris layer composed of a mixture of dead cells. contact with wound exudates may result in the transmission of the disease to the skin as well the respiratory tract. Respiratory involvement typically begins with sore throat & mild pharyngeal inflammation. edematous mucosa. Elaboration of the diphtheria toxin may affect distant organs such as the myocardium. Symptoms initially are general & nonspecific. substandard living conditions. WBCs & organisms.10 days). tonsillar. Development of a localized or coalescing pseudo membrane • can occur in any portion of the respiratory tract. Immunization patterns have the most influence on mortality patterns. nasopharyngeal secretions & rarely fomites. prostration Sore throat . Human carriers are the main reservoir of infection.Pathophysiology History Overcrowding.5 days (range. The ulcers are often co . It may reach higher than 20% in children younger than 5 years & adults older than 40 years. The lesions of cutaneous diphtheria are infectious & bacteria from cutaneous lesions have been found to cause pharyngeal infections & thus serve as a reservoir for infection. causes a localized inflammatory reaction followed by tissue destruction & necrosis.infected with Staphylococcus aureus & group A streptococci. Immunity from exposure or vaccination wanes over time. even if adequately immunized previously. incomplete immunization & immunocompromised states facilitate susceptibility to diphtheria & are risk factors associated with transmission of this disease. Inadequate boosting of previously vaccinated individuals may result in increased risk of acquiring the disease from a carrier. diphtheria has been primarily a disease of childhood. In the case of cutaneous disease.12 months after their transplacentally derived immunity wanes Patients with diphtheria may present with the following complaints Low . The membrane is intensely infectious & droplet & contact precautions must be followed when examining or caring for infected patients. Infected patients & asymptomatic carriers can transmit C diphtheria via respiratory droplets. poor health. pharyngeal) to widely covering the entire tracheobronchial tree. Cutaneous diphtheria is a disease characterized by indolent. Clinical • • o o o 111 Historically. often resembling • a typical viral upper respiratory infection (URI). Mortality / Morbidity The most widely quoted diphtheria mortality rate is 5 .10%.

Pharyngeal diphtheria • Patients may present with general symptoms of fever. Neck : Extensive anterior & submandibular cervical • lymphadenopathy imparts a bull’s neck appearance. thus older people who have not received booster vaccination are more susceptible to contract the disease from carriers.o o o o o • • Headache Cervical lymphadenopathy & respiratory tract pseudo membrane formation Serosanguineous or seropurulent nasal discharge. which is especially important in the adult population & as well the pediatric population in underdeveloped countries. • • • • Physical General Patient has a low . circulatory collapse. The following factors may predispose to diphtheria infection : Incomplete or absent immunization. gray. soft palate. cardiac chamber dilatation & weakness. may predispose to infection. Low herd immunity. cough Respiratory diphtheria may quickly progress to respiratory failure due to airway obstruction or aspiration of pseudo membrane into the tracheobronchial tree.2 weeks of illness following improvement in the pharyngeal phase of the disease. or laryngeal nervous dysfunction. Cardiac toxicity typically occurs after 1 . tachycardia & anxiety. • accessory muscle use & retractions. respiratory stridor. Cutaneous diphtheria begins as a painful lesion resembling an erythematous pustule. wheezing. diminished heart sounds. disease states including HIV. facial & pharyngeal. a stocking & glove peripheral sensory neuropathy pattern can be observed. dysphagia Dyspnea. cyanosis. Respiratory distress manifesting as stridor. oropharynx. thick. • leathery membrane variably covers the tonsils. It can occasionally also be associated with dysphonia. Antitoxin titers decrease over time & immunity wanes. immunity does not prevent infection but lessens the severity of the disease. In some cases.Due to pharmacologic immune suppression. Occasionally. It follows an indolent course. possibly leading to increasing prevalence of diphtheria infections Immunocompromised states . Peripheral neuritis develops anywhere from 10 days to 3 months after the onset of pharyngeal disease. Occasionally. Most patients with severe disease develop neuropathy. typically lasting weeks to months. or more subtly with progressive dyspnea. Tonsils & pharynx : Pharyngeal erythema & edema. It may 112 • manifest as follows : Myocarditis is seen in as many as 60% of patients (especially if previously unimmunized) & can present acutely with congestive heart failure (CHF). nasopharynx & uvula. Cutaneous diphtheria often develops at a site of previous trauma or a primary dermatologic disease. The patient may hold his or her head in extension. Attempts at scraping the pseudo membrane causes bleeding of the underlying mucosa. wheezing.grade fever but is toxic in appearance & also may have a swollen neck. it may cause respiratory diphtheria. It manifests initially as a motor defect of the proximal muscle groups in the extremities extending distally. ciliary paralysis. halitosis. Neurologic toxicity is proportional to the severity of the pharyngeal infection. white nasal membrane Hoarseness. which breaks down to form an ulcer covered with a gray membrane. Deficits include the following : Cranial nerve deficits including oculomotor. or relative Causes • • • • .

Initiate electrical pacing for clinically significant conduction disturbance & provide pharmacologic intervention for arrhythmias or for heart failure. • provide invasive monitoring & aggressive resuscitation for patients with septicemia. Isolate all cases promptly & use universal & droplet precautions • to limit the number of possible contacts.T wave changes. Maintain close monitoring of cardiac activity for early detection • of rhythm abnormalities. Emergency Care To establish the diagnosis of C diphtheriae. it is vital to both isolate C diphtheriae in culture media & to identify the presence of toxin production. Secure definite airway for patients with impending respiratory • compromise or the presence of laryngeal membrane. • Diphtheria antitoxin is a horse . the Centers • for Disease Control & Prevention (CDC) should be contacted & further testing may be requested. ECG may show ST .• compromise such as from diabetes or alcoholism Low socioeconomic status Pharyngitis Peritonsillar Abscess Pharyngitis Retropharyngeal Abscess Rheumatic Fever Shock. bronchoscopy as indicated in intubated patients Electrical pacing for high .derived hyper immune 113 . Urinalysis (UA) may demonstrate transient proteinuria. Other laboratory studies • • • CBC may show moderate leukocytosis. Serum troponin I levels seem to correlate with the severity of myocarditis. Septic Tonsillitis Septic arthritis Sepsis Procedures Differential diagnosis Angioedema Endocarditis Mononucleosis Myocarditis Oropharyngeal / esophageal candidiasis Epiglottitis Peripheral nerve palsies Acute renal failure Laboratory Studies • • • • Endotracheal intubation Surgical airway . Consider involving ENT or operating room personnel for intubation & securing of airway if there is suspicion for loss of the airway or respiratory failure. Early airway management allows access for mechanical removal of tracheobronchial membranes & prevents the risk of sudden asphyxia through aspiration.bore IVs for patients with a toxic appearance.Cricothyroidotomy or tracheostomy Laryngoscopy. Provide 2 large . Patients should be transported to the nearest hospital. Chest radiograph & soft tissue neck radiography/CT or ultrasonography may show prevertebral soft tissue swelling.grade conduction disturbances Pre hospital Care Careful assessment of airway patency & cardiovascular stability. Once diphtheria infection has been established. Polymerase chain reaction (PCR) assays for detection of DNA • sequence encoding the A subunit of tox+ strain are both rapid & sensitive. Neutralize the toxin as soon as diphtheria is suspected. enlarged epiglottis & narrowing of the subglottic region. Antibiotics hasten recovery & prevent the spread of the disease to other individuals. thus limiting the amount of toxin production. Initiate prompt antibiotic coverage (erythromycin or penicillin) • for eradication of organisms. variable heart block & dysrhythmia. Imaging Studies • • Treatment of diphtheria should be initiated even before confirmatory tests are completed due to the high potential for mortality & morbidity.

000 U for extensive disease with duration of 3 or more days or edema of the neck (bull’s neck) Administer IM for less severe disease Test all patients with a 1 :10 . macrolide therapy has been associated with an increase in pyloric stenosis in children younger than 6 months. 000 U IV over 60 min for laryngeal or pharyngeal disease of < 48 h duration 40. which results in bactericidal activity. Obtain throat & nasal swabs from persons in close contact with the suspected diphtheria victim. Throat cultures should be repeated in 2 weeks after treatment. 000 . as it is most efficacious early during the course of the disease.60. alternately. thus. 000 U/d IM (penicillin G benzathine) for 10 d Diphtheria antitoxin is derived from a horse serum. Antitoxins Neutralizes toxin before it enters cells. as the antitoxin does not influence immunity. It is also known as rubeola & is marked by prodromal fever. desensitize subjects with increasing doses of diluted DAT Erythromycin & penicillin are both recommended for the treatment of diphtheria. However. Additionally. close contacts. initiation or completion of immunization with diphtheria toxoid is necessary. An increased incidence of pyloric stenosis is associated with administration of erythromycin to infants younger than 6 months.appropriate diphtheria booster.50 mg/kg/d IV divided bid. Treatment is most effective in the early stages of disease & decreases the transmissibility & improves the course of diphtheria. 000 U/d IM (penicillin G benzathine) for 10 d 30 . administer age . not to exceed 2 g/d. Diphtheria disease does not confer immunity. administer epinephrine. Penicillin is recommended in household contacts who may not comply with the duration of erythromycin treatment.50 mg/kg/d PO divided qid for 14 d Penicillin G benzathine Interferes with synthesis of cell wall mucopeptides during active multiplication. It prevents the progression of symptoms. 000 . if an immediate reaction occurs. . 300.60 lb : 125 mg PO (penicillin V) qid for 10 d. The CDC has approved macrolide such as erythromycin as first . must receive chemoprophylaxis regardless of immunization status or age. it neutralizes unbound exotoxin.line agents for patients older than 6 months of age.• • • antiserum that neutralizes circulating toxin prior to its entry into the cells. The treatment of endocarditis requires the addition of an amino glycoside. Dose given depends on site of infection & length of time patient is symptomatic 20. It is to be administered as soon as diphtheria suspected. especially treatment with erythromycin. < 30 lb : 60 mg PO (penicillin V) qid for 10 d.1 :100 dilution of DAT SC. Initiate antibiotic therapy with erythromycin or penicillin for chemoprophylaxis in a patient with suspected exposure. such as family members. Medication Patients with active disease as well as all close contacts should be treated with antibiotics. 40 . 000 U for nasopharyngeal lesions 80. 600. 000 . Administer immunization toxoid booster. Diphtheria antitoxin 114 35 Measles • • Measles is a highly communicable acute disease. Erythromycin 20 . household contacts & potential carriers.120. Effective treatment for systemic diphtheria. hypersensitivity to horse serum is not a contraindication to antitoxin injection.40. Intramuscular penicillin is recommended for patients who will be noncompliant or intolerant to an erythromycin course. The horse serum antitoxin is given to anyone suspected to have diphtheria & can be administered without confirmation from cultures.

particularly bronchopneumonia.type hypersensitivity. Age Unvaccinated young children are at the highest risk. the peak incidence of infection occurs during late winter & spring. Vitamin A supplementation during acute measles significantly reduces risks of morbidity & mortality.related mortality among younger children.8 years). Infection confers life . measles virus causes progressive & often fatal giant cell pneumonia.attenuated measles vaccination.1% of cases. Maternal antibodies play a significant role in protection against infection in infants younger than 1 year & may interfere with live . Measles is still a leading cause of death among children. 115 • • • • Following the amplification of measles virus in regional lymph nodes.specific lymphoproliferative responses that persist for weeks to months after the acute infection. bronchopneumonia. croup). a predominantly cell . interleukin 12 production & antigen .e. Transient hepatitis may occur during an acute infection.associated viremia disseminates the virus to various organs prior to the appearance of rash. Subacute sclerosing panencephalitis (SSPE) is a rare chronic degenerative disease that occurs several years after measles infection. is a member of the Morbillivirus genus in the Paramyxoviridae family. A generalized immunosuppression that follows acute measles frequently predisposes patients to bacterial otitis media & bronchopneumonia. can result from a persistent measles infection. Incubation period is 8 – 12 days. measles causes acute encephalitis. conjunctivitis & pathognomonic enanthem i. laryngotracheobronchitis (i. In temperate areas. and sub acute sclerosing panencephalitis (SSPE). In individuals with deficiency in cellular immunity. a major cause of measles . Measles virus a negative . Rare complications include hemorrhagic measles. school . a 5% susceptible population is sufficient to sustain periodic outbreaks in otherwise highly vaccinated populations. Immunosuppression may predispose individuals to severe bacterial infection. interstitial pneumonitis. . SSPE is characterized by the onset of behavioral & intellectual deterioration & seizures years after an acute infection (the mean incubation period for SSPE is approximately 10. purpura fulminans. coryza. less commonly. encephalomyelitis & diarrhea. perhaps carried by pulmonary macrophages.• • • • • • • cough. Measles virus infection causes an immunosuppression marked by decreases in delayed . a degenerative CNS disease. depending on local immunization practices & incidence of the disease. A single dose of measles vaccine administered to a child older than 12 months induces protective immunity in 95% of recipients. exacerbation of tuberculosis.e. Koplik spots. transient loss of hypersensitivity reaction to tuberculin skin test. Age specific attack rates may be highest in susceptible infants younger than 12 months. Because measles virus is highly contagious. In approximately 0.sense enveloped RNA virus. by airborne transmission. measles virus infects local lymphatic tissues. After 2 .long immunity. Initial infection & viral replication occur locally in tracheal & bronchial epithelial cells. disseminated intravascular coagulation (DIC). followed by an erythematous maculopapular rash on the third to seventh day. despite the availability of an effective vaccine. Mortality / Morbidity Pathophysiology • • • • Common infectious complications include otitis media.4 days. or young adults.aged children. hepatitis. Measles virus is spread by direct contact via respiratory droplets or. SSPE.

• • • • • Enanthem . laryngotracheobronchitis (i. may occur after one week. Risk factors Risk factors for infection • • • Children with immunodeficiency due to HIV or acquired immunodeficiency syndrome (AIDS). bluish .A temperature often exceeding 104°F (40°C) begins • with the prodrome & persists 7 . mild hepatomegaly & appendicitis may occur because of generalized involvement of lymphoid tissue.10 days.e.Complications such as otitis media.gray specks or “grains of Differential Diagnoses sand” on a red base) appear on the buccal mucosa opposite the second molars near the end of the prodrome. Lymphoid involvement .e.Generalized lymphadenopathy. immunocompromised individuals can be contagious during the duration of the illness. The prodromal phase is marked by malaise. bronchopneumonia. 14 days after exposure & starts on the face & upper neck & spreads to extremities The entire course of uncomplicated measles. anorexia. Although this is the pathognomonic enanthem of measles. It is generally seen 2 days prior to the appearance of the rash & lasts until 2 days after the rash appears. from late prodrome to resolution of fever & rash. Desquamation & brown staining. regardless of immunization status Travel to areas where measles is endemic or contact with travelers to endemic areas Infants who lose passive antibody prior to the age of routine immunization Malnutrition Underlying immunodeficiency Pregnancy Vitamin A deficiency Parvovirus B19 Infection Roseola Infantum Scarlet Fever Rubella Toxic Shock Syndrome Risk factors for severe measles & its complications Physical Fever . Laboratories can confirm measles by demonstrating more than • a 4 . History • • • • • • • The first sign of measles is usually a high fever that usually last 4 . and conjunctivitis.2 days before onset of symptoms. or corticosteroid therapy. Cough may be the final symptom to appear.Koplik spots (i. extremities. Koplik spots in measles Rash .fold rise in immunoglobulin G (IgG) antibodies between acute & convalescent sera. The rash may be absent in patients with underlying deficiencies in cellular immunity. Patients are contagious from 1 . which spares the palms & soles.5 days before the appearance of the rash to 4 days after the onset of rash. On the other hand. . 116 Diagnosis of measles is usually determined from the classic clinical picture. alkylating agents. fever.7 days. Rash usually occurs. it lasts for about 5 days. on average. Healthy children are also contagious during the period from 3 . its absence does not exclude diagnosis. This enanthem begins to slough as the rash appears. cough & coryza (the “3 Cs”). leukemia.10 days. is 7 .An erythematous & maculopapular rash that becomes • Dengue Drug Eruptions Enteroviral Infections Infectious Mononucleosis Kawasaki Disease Meningococcemia Laboratory Studies confluent begins on the face & then proceeds to the trunk. palms & soles. croup) & diarrhea are more common in young children. Patients appear most ill during the first or second day of the rash.

The first dose is given to healthy individuals at age 15 mts.25 mL/kg IM (0. The earliest confirmation of measles using IgG antibodies takes about 3 weeks from the onset of illness. Ig is recommended in these patients.24 months who are hospitalized for measles . Elevated hepatic transaminase levels may be detected in patients with measles virus hepatitis. The morbidity rate is high in children younger than 1 year.• • • • • Patients with subacute sclerosing panencephalitis (SSPE) have unusually high titers of measles antibody in their serum & cerebrospinal fluid.related mortality rate exceeds 1%. 0. therefore. Cerebrospinal fluid examination reveals the following : Increased protein Normal glucose Mild pleocytosis with a predominance of lymphocytes Treatment of measles is essentially supportive. Two doses of vitamin A given 24 hours apart are recommended. usually at age 4 6 years. not to exceed .rubella (MMR) in two doses. Vitamin A o o o o < 6 months : Not established 6 months to 1 year : 100. Vitamin A supplements is associated with reduction in morbidity & mortality & is recommended in the following children : Those who live in developing countries or in areas of impoverishment in developed countries Children aged 6 . o o • Other diagnostic tests • o o Imaging Studies Chest radiography is performed if bacterial pneumonia is suspected. intramuscular Transient source of IgG. Measles virus can be isolated from nasopharyngeal swabs. The second dose is given upon school entry. repeat dose the next day & at 4 wk for ophthalmologic evidence of vitamin A deficiency > 1 year : 200. Ig is given to the following individuals : Those with immunocompromise Infants aged 6 months to 1 year Infants younger than 6 months who are born to mother without measles immunity Pregnant women Vitamins Medical Care The World Health Organization (WHO) recommends vitamin A administration to all children with measles in communities where vitamin A deficiency is a recognized problem & where the measles virus (MV) . consider administering measles virus (MV) vaccine or human immunoglobulin (Ig). a delay too long to permit implementation of effective control measures.5 mL/kg for patients with HIV). The Measles virus vaccine is routinely administered as measles . A leukopenia with a relative lymphocytosis occurs in the late stages of viremia. Other Tests • o o o o • • o o o • • Lumbar puncture if encephalitis is suspected.related complications Those with immunodeficiency Those with evidence of an ophthalmologic complication due to vitamin A deficiency 117 Children with malnutrition Children with impaired intestinal absorption To prevent or modify measles in exposed susceptible individuals. 000 IU PO as a single dose.mumps . repeat dose the next day & at 4 wk for ophthalmologic evidence of vitamin A deficiency Immune globulin. 000 IU PO as a single dose.

a cumulative dose of 15 mL; if dose exceeds 10 mL, divide dose into several muscle sites to reduce local pain

36
• •
Meningitis is a clinical syndrome characterized by inflammation of the meninges. Encephalitis is defined as inflammation of the brain along with dysfunction of the brain. Encephalopathy only implies the dysfunction of the brain. Clinically, this medical condition manifests with meningeal symptoms (e.g. headache, nuchal rigidity & photophobia) & an increased number of white blood cells in the cerebrospinal fluid (CSF; pleocytosis). Depending on the duration of symptoms, meningitis may be classified as acute or chronic. Acute meningitis denotes the evolution of symptoms within hours to several days, while chronic meningitis has an onset & duration of weeks to months. The duration of symptoms of chronic meningitis is characteristically at least 4 weeks.

• •

Causes

There are numerous infectious & noninfectious causes of meningitis. Noninfectious causes include medications (e.g. nonsteroidal anti - inflammatory drugs, antibiotics) & carcinomatosis. Depending on the specific bacterial cause, the syndrome may be called, for example, Streptococcus pneumoniae meningitis, meningococcal meningitis, or Haemophilus influenzae meningitis. Fungal & parasitic causes of meningitis are also termed according to their specific etiologic agent, such as cryptococcal meningitis, Histoplasma meningitis & amebic meningoencephalitis. Aseptic meningitis is defined as clinical & laboratory evidence of inflammation of the meninges (e.g., CSF pleocytosis & increased protein) without evidence of bacterial infection on Gram stain or
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culture. More than 80% of cases are caused by enteroviruses (i.e., coxsackievirus, enterovirus, echovirus, and, rarely, poliovirus). Aseptic meningitis is a broad term that denotes a non - pyogenic • cellular response, which may be caused by many different etiologic agents. In many cases, a cause is not apparent after initial evaluation. Patients characteristically have an acute onset of meningeal symptoms, fever & cerebrospinal pleocytosis that is usually prominently lymphocytic. After an extensive workup, many of these cases are found to have a viral etiology & can then be reclassified as acute viral meningitis (e.g. enterovirus meningitis, herpes simplex virus [HSV] meningitis). While viruses cause most cases of aseptic meningitis, it can also be caused by bacterial, fungal, mycobacterial & parasitic agents. In general, the findings among neonates & young infants with • meningitis are minimal & often subtle. Temperature instability (fever or hypothermia) occurs in approximately 60% of infected infants; increasing irritability is present in about 60%, poor feeding or vomiting in roughly 50% & seizures in about 40%. Lethargy, respiratory distress & diarrhea are frequent • nonspecific manifestations of meningitis in this patient group. On physical examination, approximately 25% of newborns & • young infants have a bulging fontanel & only 13% have nuchal rigidity. The diagnosis of meningitis cannot be excluded on the basis of the absence of these physical findings in infants.
Etiology of Meningitis Common - Enteroviruses

Less Common

Neisseria meningitidis Strep. Pneumoniae Group B streptococci Escherichia coli Haemophilus influenzae Listeria monocytogenes - Herpes simplex virus Klebsiella spp. (and other Enterobacteriaceae)

Mycobacterium tuberculosis Pseudomonas aeruginosa Staphylococcus aureus Rare - Cysticercosis Cryptococcus neoformans Lymphocytic choriomeningitis Mumps Syphilis Amoebae Any other cause of brain irritation is called as encephalopathy & can be seen with diseases such as hypertension (high BP), kidney failure (uremic encephalopathy), jaundice (hepatic encephalopathy), poisoning & heavy mental intoxication.
Diagnosis

Pathophysiology

Diagnosis of encephalitis is made by detecting cells in the cerebrospinal fluid. (Cerebrospinal fluid is the fluid present in the brain cavity). This cerebrospinal fluid also helps to detect the organisms causing the infection. MRI brain helps to determine which area of the brain is involved & look for complications such as brain abscess & hydrocephalus (increase in size of brain cavity due to increase pressure in the cerebrospinal fluid). EEG can help to determine Herpes virus infection. Other tests done are either CT scan or MRI of the brain. These are done to look for complications such as brain abscess, pus in the brain or look for increase in size of ventricles (hydrocephalus).
Complications

Rapidly progressive encephalitis can lead to more brain damage & death. Untreated bacterial, TB or fungal meningitis can lead to increase coma & death. Viral meningitis most often improves on its own. Meningitis can often be complicated with brain abscess or increase ventricle size. Common long term sequelae can occur in 30 percent of patients & include mental retardation, hearing loss, vision impairment, paralysis & epilepsy.
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Three major pathways exist by which an infectious agent (i.e. bacteria, virus, fungus & parasite) gains access to the central nervous system (CNS) & causes meningeal disease. Initially, the infectious agent colonizes or establishes a localized • infection in the host. This may be in the form of colonization or infection of the skin, nasopharynx, respiratory tract, gastrointestinal tract, or genitourinary tract. From this site, the organism invades the submucosa by • circumventing host defenses (e.g. physical barriers, local immunity, phagocytes/macrophages) & gains access to the CNS by 1) Invasion of the bloodstream (i.e. bacteremia, viremia, fungemia, parasitemia) & subsequent hematogenous seeding of the CNS, which is the most common mode of spread for most agents 2) A retrograde neuronal (i.e. olfactory & peripheral nerves) pathway or 3) Direct contiguous spread (i.e. sinusitis, otitis media, congenital malformations, trauma & direct inoculation during intracranial manipulation). Once inside the bloodstream, the infectious agent must escape immune surveillance (e.g. antibodies, complement - mediated bacterial killing & neutrophil phagocytosis). The ensuing cerebral edema (i.e. vasogenic, cytotoxic, • interstitial) significantly contributes to intracranial hypertension & a consequent decrease in cerebral blood flow. Anaerobic metabolism ensues, which contributes to increased • lactate concentration. Eventually, if this uncontrolled process is not modulated by effective treatment, transient neuronal dysfunction or permanent neuronal injury results. Another important component or complication of meningitis • is the development of increased intracranial pressure (ICP). The pathophysiology of this complication is complex & may involve many proinflammatory molecules as well as mechanical

elements. Interstitial edema (secondary to obstruction of CSF flow, as in hydrocephalus), cytotoxic edema (swelling of cellular elements of the brain through the release of toxic factors from the bacteria & neutrophils) & vasogenic edema (increased BBB permeability) are all thought to play a role in the development of increased ICP.
Mortality / Morbidity

Animal contacts should be elicited. Patients with rabies could present atypically with aseptic meningitis & rabies should be suspected in a patient with a history of animal bite (e.g. dog, fox & bat). The presence of a ventriculoperitoneal shunt & a history of recent cranial surgery should be elicited. Signs of cerebral dysfunction are common, including confusion, irritability, delirium & coma. These are usually accompanied by fever & photophobia. Signs of meningeal irritation are observed in only approximately 50% of patients with bacterial meningitis & their absence certainly does not rule out meningitis. Kernig sign : In a supine patient, flex the hip to 90° while the knee is flexed at 90°. An attempt to further extend the knee produces pain in the hamstrings & resistance to further extension. Brudzinski sign : Passively flex the neck while the patient is in a supine position with extremities extended. This maneuver produces flexion of the hips in patients with meningeal irritation. Nuchal rigidity : Resistance to passive flexion of the neck is also a sign. Exacerbation of existing headache by repeated horizontal movement of the head, at a rate of 2 - 3 times per second, may also suggest meningeal irritation. Cranial nerve palsies may be observed as a result of increased ICP or the presence of exudates encasing the nerve roots. Focal neurologic signs may develop as a result of ischemia from vascular inflammation & thrombosis. Seizures occur in approximately 30% of patients. Papilledema & other signs of increased ICP may be present. Coma, increased blood pressure with bradycardia & cranial nerve III palsy may be present.

Physical

Mortality from meningitis varies with the specific etiologic agent. The mortality rate for viral meningitis (without encephalitis) • is less than 1%. In patients with deficient humoral immunity (e.g. agammaglobulinemia), enterovirus meningitis may have a fatal outcome. Bacterial meningitis was uniformly fatal before the • antimicrobial era. With the advent of antimicrobial therapy, the overall mortality rate from bacterial meningitis has decreased but remains alarmingly high.
Age

o

o

Meningitis occurs in people of all age groups, but infants & young children & elderly individuals (> 60 y) are more predisposed to the infection.
History

o o

• •

The classic presentation of meningitis includes fever, headache, neck stiffness, photophobia, nausea, vomiting & signs of cerebral dysfunction (e.g. lethargy, confusion & coma). These symptoms may develop later in the course of illness in some patients who may initially present with atypical symptoms such as leg pain & cold hands & feet. The triad of fever, nuchal rigidity & change in mental status is found in only two thirds of patients. The classic presentation of acute meningitis is the onset of symptoms within hours to a few days, compared to weeks for chronic meningitis.
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• • • • o

o o

o o o

The presence of papilledema also suggests a possible alternate diagnosis (e.g. brain abscess). Hepatosplenomegaly & lymphadenopathy suggest a systemic disease, including viral (e.g. mononucleosis like syndrome in EBV, CMV & HIV) & fungal (e.g. disseminated histoplasmosis) disease. The presence of a murmur suggests infective endocarditis with secondary bacterial seeding of the meninges. Evidence of parotitis is observed in some cases of mumps meningitis. The presence of a ventriculoperitoneal shunt or a cochlear implant may suggest a bacterial cause of meningitis. In contrast to bacterial meningitis, patients with aseptic meningitis syndrome usually appear clinically nontoxic with no vascular instability.

of the translocation of gut microflora with the Strongyloides stercoralis larva during hyperinfection syndrome).
Aseptic meningitis syndrome

Aseptic meningitis is the most common infectious syndrome affecting the CNS. Most episodes are caused by a viral pathogen, but they can also be caused by bacteria, fungi, or parasites.
Acute viral meningitis

Causes

S pneumoniae H influenzae Group A streptococci CSF shunts Coagulase - negative staphylococci S aureus Aerobic gram - negative bacilli Propionibacterium acnes Patients with hyposplenism, hypogammaglobulinemia, multiple • myeloma, glucocorticoid treatment, defective complement (C1 - C4), diabetes mellitus, renal insufficiency, alcoholism, malnutrition & chronic liver disease are at increased risk. Other predisposing risk factors include • 1) Neurosurgical procedures or intracranial manipulation; 2) Old age; 3) Immunosuppression; 4) High - grade gram - negative bacillary bacteremia; & 5) Disseminated strongyloidiasis, which has been reported as a classic cause of gram - negative bacillary bacteremia (as a result
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Basilar skull fracture

Viral meningitis comprises most aseptic meningitis syndromes. The viral agents for aseptic meningitis include the following : Enterovirus • The non polio enteroviruses (NPEV) • Herpes virus • Varicella zoster virus (VZV), or HHV - 3 & CMV are causes • of meningitis in immunocompromised hosts, especially patients with AIDS & transplant recipients. Human immunodeficiency virus • o Aseptic meningitis syndrome may be the presenting symptom in a patient with acute HIV infection.
Chronic meningitis

Chronic meningitis is a constellation of signs & symptoms of meningeal irritation associated with CSF pleocytosis that persists for longer than 4 weeks. Chronic bacterial meningitis • Tuberculous meningitis • o M tuberculosis is an acid - fast bacillus that causes a broad range of clinical illnesses that can affect virtually any organ of the body. o Always consider tuberculous meningitis in the differential diagnoses of patients with aseptic meningitis or chronic meningitis syndromes. o Involvement of the CNS with tuberculous meningitis is usually caused by rupture of a tubercle into the subarachnoid space. o The presentation may be acute, but the classic presentation is subacute & spans weeks. Patients generally have a prodrome

30%).o of fever of varying degrees. The opening pressure (reference range is 80 .90% of patients with bacterial meningitis. • • Differential Diagnosis Brain Abscess Noninfectious meningitis. These groups include those with newly onset seizures. Some patients may have elevated blood sugar levels as a result of underlying diabetes mellitus & the predictive value of the CSF & blood sugar ratio may not be accurate in these circumstances. The CSF picture of acute viral meningitis is different from the CSF picture of bacterial meningitis. Low CSF WBC count (< 20 cells/µL) in the presence of a high bacterial load suggests a poor prognosis. VI & VII). chemistry (i. signs suspicious for space occupying lesions (such as papilledema & focal neurologic signs) & moderate . usually in the hundreds. HSV. V.200 mm H2O) may be elevated.1000 cells/µL) with a predominance of lymphocytes. Examination of the CSF in patients with acute bacterial meningitis reveals the characteristic neutrophilic pleocytosis (usually hundreds to a few thousand. The CSF glucose (reference range is 40 . In general. The opening pressure is usually within the reference range.70 mg/dL) is less than 40 mg/dL in 60% of patients. Gram stain & cultures). The CSF cell count is usually in the few hundreds (100 . Some cases of echovirus. In some cases of L monocytogenes meningitis (25 . Measure the opening pressure & send the fluid for cell count (and differential count).severe impairment in consciousness. whenever the diagnosis of meningitis is strongly considered. including medication . Obtain a simultaneous blood glucose determination for comparison purposes. but some cases of LCM.induced meningeal inflammation Meningeal carcinomatosis CNS vasculitis Stroke Encephalitis Laboratory Studies • • lymphocytic predominance may occur.e. Stage 3 refers to an individual with stupor & lethargy. CSF glucose & protein) & microbiology (i. Virus isolation from the CSF can be performed. Patients often develop central nerve palsies (III. CT scan of the brain may be performed prior to lumbar puncture in some patient groups with a higher risk of herniation. IV. an immunocompromised state. .50 mg/dL) is usually elevated. Stage 1 shows no change in mental function with no deficits & no hydrocephalus. malaise & intermittent headaches. The CSF glucose level is usually within the reference range. Stage 2 refers to a patient with confusion & evidence of neurologic deficit. suggesting basilar meningeal involvement. The CSF protein (reference range is 20 . Clinical staging of meningeal tuberculosis is based on neurologic status. promptly perform a lumbar puncture. with > 80% PMN cells). CSF protein levels may be within the reference range but are usually elevated. Tuberculosis meningitis Acute viral meningitis • • • The cornerstone in the diagnosis of meningitis is examination of the CSF. CSF Gram stain permits rapid identification of the bacterial cause in 60 .to . mumps & polio may cause low CSF glucose levels. mumps & HSV meningitis may produce a neutrophilic picture early in the course of disease.e. suggesting some form of increased ICP from cerebral edema. a 122 • • • • • • • Bacterial meningitis • The CSF of patients with tuberculosis meningitis is characterized by a predominantly lymphocytic pleocytosis.

The penicillins. A characteristic hypoglycorrhagia (glucose < 40 mg/dL) is present & the protein level is usually elevated.14 Ceftriaxone / cefotaxime Ceftriaxone / cefotaxime Ceftriaxone / cefotaxime plus vancomycin or rifampin Ampicillin 7 Ceftriaxone / cefotaxime Penicillin G / ampicillin 7 . Recommended Empiric Antibiotics According to Predisposing Factors for patients with Suspected Bacterial Meningitis Predisposing Feature Antibiotic (s) • Age 0 . fluoroquinolones & rifampin provide high CSF levels. third .g. Therefore.generation cephalosporins). brain abscess. The dose of the chosen antimicrobial agent should always be adjusted based on the renal & hepatic function of the patient. Neuroimaging is indicated in patients with prolonged fever. These studies are helpful in the detection of CNS complications of bacterial meningitis. such as H influenzae. subdural empyema & venous sinus thrombosis. they should be administered prior to or during the administration of antimicrobial therapy. drug toxicity. Kinyoun stain) in the CSF is difficult & usually requires a large volume of CSF. The use of steroids has been shown to improve the overall outcome of patients with certain types of bacterial meningitis.e. especially if a CSF block is present. Medical Care Bacterial meningitis is a neurological emergency that is associated with significant morbidity & mortality. focal neurologic symptoms & signs.3 months Age 3 months to 50 years Older than 50 years Procedures Perform lumbar puncture promptly in all patients whenever a strong clinical suspicion for meningitis exists. One of the major contributors to the morbidity associated with bacterial meningitis is the severity of inflammation. The chosen antibiotic should attain adequate levels in the CSF. The initiation of empiric antibacterial therapy is therefore essential for better outcome. Achieving this usually depends on the drug’s lipid solubility. the carbapenems.• • • The opening CSF pressure is usually elevated. evidence of increased ICP & suspected basilar fracture. with auramine . hearing defects) & its treatment (e.rhodamine stain. seizures. or CSF shunt Specific Antibiotics & Duration of Therapy for Patients with Acute Bacterial Meningitis Bacteria Antibiotic (s) Ampicillin plus cefotaxime / an aminoglycoside Ampicillin plus cefotaxime plus vancomycin Ceftriaxone / cefotaxime plus vancomycin Ampicillin plus ceftriaxone / cefotaxime plus vancomycin Ampicillin plus ceftazidime plus vancomycin Vancomycin plus ceftazidime Duration (Days) S pneumoniae H influenzae N meningitidis Penicillin G 10 .Neelsen stain. Imaging Studies • adjunctive treatment of bacterial meningitis. Strongly consider the use of steroids as 123 Impaired cellular immunity Neurosurgery.g. certain cephalosporins (i. The demonstration of the acid . tuberculous & pneumococcal meningitis. hydrocephalus.4 weeks Age 1 .& fourth . its molecular size. Ziehl .g. pharmacologic interventions to reduce the degree of inflammation may improve outcome.binding capability & the state of inflammation at the meninges. its protein . head trauma. Monitor for the occurrence of complications from the disease (e. It is also indicated for evaluation of the paranasal sinuses. cerebral infarct. If steroids are given..fast bacilli (e. such as hydrocephalus. hypersensitivity).

if warranted Enterobacteriaceae Ceftriaxone / cefotaxime plus an aminoglycoside P aeruginosa Ceftazidime plus an aminoglycoside Tuberculous meningitis L monocytogenes • 14 . which can cause disease after months or years.21 • 21 21 Complications may develop very rapidly. After 7 – 20 days. Z) should be included in the regime. the danger of malaria has worsened & the disease is now a major global problem. In Plasmodium vivax & Plasmodium ovale infection. • 37 Malaria • • • • Malaria (mal . The following 4 species can infect humans : P vivax (benign tertian) P falciparum (malignant tertian) P malariae (quartan malariae) P ovale (ovale malariae) Causes • • • • • The treatment of tuberculosis meningitis should include antitubercular therapy for at least 12 months. Malaria is caused by Plasmodium species. Pathophysiology The female anopheles mosquito is infected with gametocytes of • • Diet No strict dietary restriction is necessary. Because of plasmodial & mosquito resistance to drugs & insecticides. At least 4 antitubercular drugs (INH. which are also asexual forms. known as erythrocytic schizogony.16 days & results in the host cell bursting & releasing thousands of merozoites into the blood. 124 • • plasmodium when it first sucks the infected blood from human body. the sporozoites. Sporozoites enter the hepatocytes & form schizonts. The symptoms often differ from those in adults & in any febrile illness malaria should be suspected if the child has been exposed. R. Children are the worst affected. Consultations with neurosurgical service may be needed when a skull fracture is suspected or an abscess formation is demonstrated. especially children aged 6 months to 5 years. aria . sporozoites are formed in the mosquito. which are protozoal blood parasites.21 14 . some sporozoites convert to dormant forms called hypnozoites. In parts of the world where malaria is endemic. The bite of an infected mosquito introduces this asexual form of the parasite. Merozoites enter the erythrocytes & initiate another asexual reproductive cycle.foul. Schizonts undergo a process of maturation & multiplication known as pre erythrocytic or hepatic schizogony. Surgical Care • • In certain cases of increased ICP. E. nothing by mouth is recommended for patients with altered levels of consciousness. Male anopheles does not suck blood but feeds on plant juices. repeated lumbar puncture or the insertion of a ventricular drain may be necessary to relieve the effects of increased ICP. The parasite successively passes through the stages of .Ampicillin / penicillin G plus an aminoglycoside S agalactiae Penicillin G plus an aminoglycoside. To diminish the risks of aspiration. it may cause as many as 10% of all deaths in children. into the bloodstream of a healthy individual.air) is an ancient disease causing suffering to humanity. Pre erythrocytic schizogony takes 6 .

A mosquito that sucks this blood from a patient with gametocytemia acquires these sexual forms & plays host to the sexual stage of the plasmodial life cycle. headache & nausea.2 days. History • • • Cerebral malaria Plasmodium falciparum malaria. presence of plasmodia in the bloodstream without clinical manifestations of malaria). The periodicity of malarial fever depends on the time required for the erythrocytic cycle & is definite for each species. with or without treatment. with periodic fever. an acute illness that is mostly observed • in children aged 6 months to 3 years. Malaria can mimic any febrile illness & should be suspected in any febrile child who has recently been in a malarious area. frequent convulsions. depending on the species. The other 3 species each take 48 hours for one cycle & cause fever on alternate days (tertian malaria). Malarial anemia can be quite severe. older children & adults often have asymptomatic parasitemia (i. Young children manifest this disease in many different ways. Older children may report aching body. some merozoites differentiate into the sexual forms : the male & female gametocytes. After a few cycles of this erythrocytic schizogony. with mild cough & cold. History of blood transfusion. The degree of anemia is related to destruction of erythrocytes which are infected by the parasites. Plasmodium malariae needs 72 hours for each cycle. Many children have only flulike respiratory symptoms at presentation. Age • • • Children of all ages living in non malarious areas are equally susceptible to malaria. sometimes causing death. Repeated frequent seizures 125 • . is not observed. which causes the paroxysms of malarial fever. Anemia • · Anemia is so common in malaria that it is considered almost a part of the disease. but the classic picture of malaria. Thus.18 days. Older children may manifest the classic periodicity of fever with chills & shivering. drowsy. a child can experience prolonged. shivering & sweating. In endemic areas. periodicity is not observed. Early diagnosis & prompt treatment with a drug to which the parasite is susceptible is important to save the life of the child. children are asymptomatic while the parasites complete the liver cycle & one erythrocytic cycle.e. After the mosquito bite. Rupture of a large number of erythrocytes at the same time releases a large amount of pyrogens. leading to the name quartan malaria. These merozoites again infect new erythrocytes. Children then become restless. this periodicity requires all the parasites to be developing & releasing simultaneously. Upon maturation of these merozoites. apathetic & anorexic. organ transplantation & (for newborns) malaria in the mother should be inquired. which can lead to prostration & death. Most of the mortality of malaria is due to this complication of Even without cerebral malaria. if this synchronization is absent. ultimately giving rise to several merozoites . The survivors develop partial immunity. Fever is usually continuous & may be very high (40°C) from the first day. These symptoms abate in 1 . Most deaths resulting from malaria occur in children younger than 5 years. which takes 8 . children younger than 5 years have repeated & often serious attacks of malaria. releasing the merozoites & multiple antigenic & pyrogenic substances into the bloodstream. the • • • • erythrocyte ruptures. Vomiting is very common in children with malaria & may make oral therapy ineffective.trophozoite & schizont. However.

Seizures are common & may occur at the onset of the disease. a malarious area No previous exposure to malaria (hence no immunity) No chemoprophylaxis or improper chemoprophylaxis Pneumonia Salmonella Infection Sinusitis Tetanus Toxic Shock Syndrome Urinary Tract Infection Viral Hemorrhagic Fevers Yellow Fever Enteric fever Encephalitis Viral pneumonia Differential diagnosis • • • • Dengue Bacterial Endocarditis. with resultant delay in initiating therapy. These children often develop only a low . jaundice & hepatosplenomegaly. Classic periodicity may be established after some days. Bacterial Meningitis.grade fever.• • • • • Mild diarrhea is often observed. Because the liver stage is not present. restlessness. This jaundice subsides with the treatment of malaria. High fever. profuse diarrhea with dehydration & circulatory failure is observed. Fever can be very high from the first day. cough & diarrhea are other symptoms that may occur. poor oral intake & vomiting all contribute to • dehydration. refuse feeds & often develop anemia. Complications include cerebral malaria. Prolonged malaria can cause anemia & malarial anemia causes • significant mortality. hypoglycemia & metabolic acidosis. especially in the first attack in nonimmune children. poor appetite & malaise.spectrum antibiotics should be given to children with severe malaria.scale destruction of erythrocytes. 126 Temperatures of 40°C & higher are often observed. Babies have fever. Gastroenteritis Giardiasis Aseptic Meningitis. severe anaemia or hepatitis. which do not enter the liver cells. Parasitemia in neonates within 7 days of birth implies transplacental transmission. Occasionally. Apart from other causes of fever. fever occurs every 72 hrs or 4th day. The liver may be slightly tender. Renal failure & respiratory distress syndrome are rare in young children. Malaria can be transmitted through blood transfusion. are irritable. Fever is usually continuous or irregular. Secondary bacterial sepsis is common & broad . huge splenomegaly sometimes occurs. mild jaundice may occur. Children living in an area where malaria is endemic have repeated frequent infections & develop & maintain partial immunity. with dark green mucoid stools. This congenital malaria is usually associated with placental parasitemia. Risk factors include • Physical • • • • • • • • Residence in. anaemia. In children from an endemic area. severe jaundice. Agitation & respiratory distress as a result of metabolic acidosis are ominous signs. With heavy parasitemia & large . which sometimes persists even after adequate treatment with antimalarial drugs. anemia. The malaria transmitted is by the merozoites. Tiredness. or travel through. curing the acute attack results in complete cure. even before high fever has set in. Otitis Media Pharyngitis Plague Lobar pneumonia Laboratory Studies . In benign tertian malaria fever recurs every 48 hrs or on the 3rd day In malignant tertian malaria fever occurs on alternate days In quartan malaria. Splenomegaly takes many • days. malaria is often misdiagnosed as meningitis.

These films may have to be taken repeatedly for diagnosis. The inside of the house. Children with malaria who are fully conscious. Children should be protected against mosquito bites at all times & mosquito nets used to cover bedding. If the child has hyperpyrexia. impregnated with insecticide. DNA & RNA probes & polymerase chain reaction (PCR) have good sensitivity & specificity but require sophisticated expensive equipment. can be used. Activity • • Children should be allowed to decide their own activity levels. Many children with malaria develop anemia. who have low .to . Both thick & thin films are essential. If repeated vomiting has led to dehydration. A negative finding on examination does not rule out malaria. Lower blood glucose levels are associated with higher mortality rates. Lumbar puncture is indicated to rule out meningitis in cerebral malaria & febrile seizures with malaria. especially the bedrooms. even on repeated examination. Ancillary treatment in severe malaria • • • Watch for hypoglycemia Rehydration is important. • • Anticonvulsants may be needed to terminate seizures Infants & children under 5 years are at especially high risk of severe malaria & should not be taken into malarial areas unless this is essential. A positive smear for malaria parasite does not always confirm a diagnosis of malaria in these children.moderate fever & who are maintaining their nutrition & hydration orally can be treated on an outpatient basis. If the fever is intermittent.containing fluids help counter the hypoglycemia that sometimes accompanies severe malaria. Asymptomatic parasitemia is common in children from an endemic area who have partial immunity to the disease. as required. Thick smears are used for detection of the parasite & thin smear are used for identification of the individual type of parasite. Skin repellents containing 20% DEET are effective against mosquito bites but are reported to be dangerous for use in children. Severe P falciparum malaria is often associated with hypoglycemia. Earlobe or finger prick is used for older children. but restricts fluids if unconscious 127 . tepid sponging can rapidly bring the temperature down. Glucose . Oral paracetamol is safe & effective for fever & should be used in doses of 10 mg/kg.6 times a day.• • • • • Demonstration of the parasite in a smear of the blood definitely establishes the presence of malaria. Standard hematinic therapy is effective. the child needs appropriate parenteral fluids to correct it. should be sprayed at dusk with an aerosol insecticide after closing the windows. Because onset is gradual. This dose can be repeated 3 . the child withstands a low level of hemoglobin quite well & blood transfusions are rarely needed. many children feel quite well between paroxysms of fever & come to no harm through activity. It is advisable to keep babies under mosquito nets as much as possible between dusk & dawn. the great toe is used in infants. Prevention • • • • • Other Tests • • Treatment • Procedures • Obtaining thick & thin blood films at the bedside is important. Only 50% of children with malaria have positive smear findings. Special insecticide mats.

line drugs for the treatment of malaria. quinidine. Vomiting stops when the malaria is cured. Such a child should be treated as an emergency. In fully breast . • Overdoses of chloroquine can have serious effects & all antimalarial drugs should be stored out of the reach of children in childproof containers. but not for P falciparum. 10 mg/kg initial dose (not to exceed 600 mg). hypoglycemia & jaundice are risk factors for death. may cause sudden death 5 mg/ kg IV diluted in IV fluid & infused over 6 h (0. signifying renal involvement Shock Bleeding diathesis In a child with malaria. While chloroquine acts rapidly. Chloroquine & proguanil can safely be given to babies & young children. It is available as tabs containing 260 mg & as injections containing 300 mg/mL Treatment 8. Indications for immediate hospitalization Intractable vomiting Dehydration Seizures Altered consciousness or coma Repeated convulsions Difficulty in breathing or acidotic breathing Severe pallor (indicating severe anemia) Hypoglycemia Oliguria or anuria. Antimalarials Blood schizonticides are the first .3 mg as base/kg PO tid for 7 d (equivalent to 10 mg as salt/ kg) Do not administer IM except as lifesaving measure in severe .8 mg/kg/h). quinine. followed by a 14 .line drugs for the treatment of malaria & must be started as soon as the diagnosis is made or even suspected. An antiemetic such as domperidone can be used & antimalarials should be continued.fed infants give half the recommended pediatric dose of chloroquine. Chloroquine This drug is effective against the erythrocytic forms of the parasite & is the drug of choice for P vivax. can be administered q8h to complete course after 5 infusions or until patient can take PO medication to complete course Quinine A blood schizonticidal drug & still the DOC for severe & complicated malaria. P vivax & P ovale have dormant stages (hypnozoites) in the liver & the treatment of an episode of malaria requires eradication of these forms also. followed by 3 doses of 5 mg/kg after 6. They act on the asexual forms in the erythrocytes & interrupt clinical attacks. which has a poorer 128 • • • • • • • • • • • • prognosis than uncomplicated malaria. resistance is widespread & an accurate travel history should be obtained before choosing the antimalarial drug. Delay in treatment of P falciparum malaria can lead to development of severe malaria.feeding infant. halofantrine & artemisinin compounds are the rapidly acting drugs that can terminate an acute malaria attack. Although chloroquine is secreted into the breast milk. against which it is gametocidal as well. 24 & 48 h IM injections never used in children. impaired consciousness. Blood schizonticides are the first .day course of chloroquine. Chloroquine. Treatment 25 mg/kg (as base) PO over 3 d. Doxycycline is contraindicated in children less than 8 years.• Vomiting is common in malaria. P malariae & P ovale malaria. Mefloquine is contraindicated in children weighing less than • 15 kg. the levels are not high enough to protect the breast . mefloquine.day course of primaquine. respiratory distress. It is gametocidal for P vivax & P malariae. The classic treatment is a 3 .

followed by 10 mg/kg/dose q8 . Primarily used for treatment of acute attacks of malaria caused by 129 Indicated for treatment of acute.3 mg/kg/d PO primaquine base for 14 d To render patient noninfectious : 0. Primaquine is also an effective & fairly safe drug for chemoprophylaxis For radical cure of P vivax & P ovale malarias : 0. uncomplicated P falciparum malaria. reduce dose to 5 mg/kg q8h.5 mg/kg/dose q8 .resistant P falciparum malaria. It has been found to be very effective in combination with quinine. switch to PO therapy as soon as possible Quinidine multidrug . 15 .6 mg/kg) Clindamycin An antibiotic that acts against P falciparum.resistant P falciparum < 40 kg : 8 mg/kg PO q6h for 3 doses.25 mg as salt/kg PO as single dose Artemether Antifolate drug combination that is a slow .12h if continuation beyond 48 h is needed.6 mg/kg IV qid for 4 d (total 9.5 mg/kg (based on pyrimethamine component) PO pyrimethamine as a single dose Primaquine Effective against erythrocytic forms of all 4 human plasmodia but is used for multidrug . it is as effective as quinine against blood schizonts but has significantly more cardiac toxicity. not to exceed 500 mg/dose.acting drug against erythrocytic forms of malaria. then 1. It has no action on hepatic forms or gametocytes. < 2 months : Contraindicated > 2 months : 1. 4 mg/kg PO on day 1.malaria while awaiting transport to hospital with requisite facilities 15 .30 mg as base/kg/d PO in divided doses for 7 d 15 mg/kg diluted in IV infusion fluid over at least 4h followed by 7.resistant P falciparum malaria.3 mg/kg/d PO primaquine base for 5 d for P falciparum infection Halofantrine Effective against blood forms of all 4 types of human malaria parasites. 5 .d course Pyrimethamine sulphadoxine It is useful for the treatment of multidrug . 25 . 3.5 d Alternatively. followed by 2 mg/kg/d for 3 . .10 mg/kg PO q12h for 3 . may be divided bid. Contains fixed ratio of 20 mg of artemether & 120 mg of lumefantrine (1 : 6 parts).resistant P falciparum infections. It is also gametocidal against all 4 species of human plasmodia & is used to render patients noninfectious.7 d Arthemether & lumefantrine A rapid .resistant P falciparum It has been found to be the fastest acting drug against blood forms of the malaria parasite & so the IV form is especially valuable in the management of severe & complicated malaria. 4 mg/kg/d PO divided bid IM : Administer as in adults Artesunate The only drug in clinical use that destroys hypnozoites of both P vivax & P ovale & so is used for the radical cure of the relapsing malarias. infuse over at least 4 h. repeat after 1 wk > 40 kg : Administer as in adults Administer on empty stomach at least 1 h ac or 2 h pc Mefloquine A blood schizonticidal drug. Special usefulness is its action against multidrug . It is effective against blood schizonts but has no activity against hypnozoites & gametocytes. the most dangerous form of malaria.12h.2 mg/kg IV on day 1. effective in some cases of chloroquine . switch to PO therapy as soon as possible & complete 7 . even against malaria acquired in areas where drug resistance is common.acting blood schizonticide. It is available as tabs of quinidine sulphate containing 200 mg.20 mg/kg IV diluted in IV infusion fluid to 1 mg/mL.

Oophoritis is quite rare & is usually a benign inflammation of the ovaries. After the prodromal period.grade fever. viral replication occurs. which could be confused with bacterial meningitis. 70 . which may require as long as 10 days. anorexia & abdominal pain. Occasionally. Orchitis occurs in 10 .20% of patients.stranded RNA virus & a member of the family Paramyxoviridae. nephritis. Unilateral swelling usually occurs first. • • Pathophysiology Symptoms in the history mostly consist of fever. Systemic symptoms include low . The virus may be isolated from saliva. thrombocytopenia purpura. or polyneuritis is rare. Unilateral hearing loss is associated with mumps infection but is also rare.containing foods may aggravate discomfort of the parotid gland. parotiditis). but in patients with mumps. mumps is no longer the most common cause of parotitis. Within 24 hours. parotitis. Other salivary glands may be involved. pain with pressure & obscured angle of the mandible.Number of tab per dose by body weight • • 38 Mumps • • Mumps is a benign viral infection of the salivary glands with systemic manifestations & complications.21 days & mumps is communicable from 6 days before to 9 days after facial swelling is apparent. Mortality / Morbidity Physical • • • • • • • • Approximately 10% of all infected patients develop a mild form of meningitis. subsequent sterility is rare. These usually resolve within 2 . Mumps virus is a single . the parotid gland is not palpable. patients report an ear pain localized near the lobe of the ear & aggravated by a chewing movement of the jaw. headache & malaise. blood. urine & cerebrospinal fluid (CSF). Parotitis : The classic illness of mumps consists of swelling of the parotid gland (i.e. pancreatitis. one or both parotid glands begin to enlarge. it rapidly progresses to maximum swelling over several days. well before the salivary gland edema disappears. mastitis & pneumonia. It usually follows parotitis but may Mumps virus produces a generalized infection. arthritis.3 weeks without sequelae.80% of cases are bilateral. However. Older children may describe a sensation of swelling at the angle of the jaw in the early stage. transient myelitis. After entry into the oropharynx. Acid . The fever usually subsides after a variable period of as long as 1 week. causing subsequent viremia & involving glands or nervous tissue. genus Paramyxovirus. Ordinarily. Affected glands show edema & lymphocyte infiltration. Encephalitis. thyroiditis. headache. Orchitis : Approximately one third of postpubertal male patients develop unilateral orchitis. simultaneous involvement of both parotid glands occurs. malaise. • • • • • History • • 130 . The incubation period is 14 . Fever subsides within 1 week & disappears before swelling of the parotid gland resolves. Other rare complications include myocarditis. followed by bilateral parotid involvement. including both submaxillary glands & sublingual glands & orifices of the ducts may be erythematous & edematous. Edema over the parotid gland typically occurs with non discrete borders. especially with a sour taste.

Viruria is common even in uncomplicated mumps. Avoiding acid . Use of plenty of fluids is essential because adequate hydration & alimentation of patients is important. Laboratory Studies Conservative therapy is indicated in patients with mumps. 131 • • It refers to a persistent & pervasive disorder that initially manifests in childhood & is characterized by hyperactivity. which is a self limited disease. Procedures Lumbar puncture may be needed to obtain cell profile & culture information if meningoencephalitis is the prominent presentation. emotional & social functioning.• • • precede parotitis or occur in the absence of parotitis. ataxia & vomiting is followed by permanent deafness. Sudden onset of tinnitus. In orchitis. Serum amylase is elevated in mumps parotitis & pancreatitis. tomato. Prescribe analgesics for severe headaches or discomfort due to parotitis. fever. vomiting & meningismus are common. and/or inattention. Meningoencephalitis . orange juice) are beneficial to reduce pain. ADHD is usually noticed in children below 7 years of age. No antiviral agent is indicated for mumps. vinegar containing food additives) & liquids (e. myocarditis & hematologic complications.containing foods (e.g. . this maneuver produces no tenderness with adenitis. It may precede parotitis or appear in the absence of parotitis but usually occurs in the first week after parotitis. A light diet with generous fluid intake is recommended. Headache. Other complications : Less common complications include arthritis. Medical Care • • • • • Diet Differential diagnosis Human Immunodeficiency Virus Infection Coxsackievirus parotitis Influenza virus parotitis Parainfluenza virus parotitis Suppurative parotitis commonly caused by Staphylococcus aureus or other bacteria Adenitis Recurrent parotitis Calculus of Stensen duct Tumors of the parotid gland Upward pressure applied to the angle of the mandible produces tenderness with mumps (Hatchcock’s sign). Deafness : Neuritis of the auditory nerve may result in deafness. • • Activity Bed rest is recommended for a faster recovery & is needed for patients with complicated cases. impulsivity. 39 Attention Deficit Hyperactivity disorder (ADHD) • • • Diagnosis of mumps is clinical & laboratory tests are unnecessary. stronger analgesics may be needed. Other neurologic complications include facial nerve neuritis & myelitis. Marked changes in sensorium & convulsions are not usual.g. Serum lipase is elevated in pancreatitis.CNS involvement with mumps is not uncommon & it occurs more often as meningitis rather than true encephalitis. Foods & liquids that contain acid may cause swallowing difficulty as well as gastric irritation. nausea. • • CBC count reveals a normal or elevated WBC count with lymphocyte predominance. These symptoms can lead to difficulty in academic.

cupboards etc) They will be continuously talking. The child often avoids. pencils. school assignments. Therapy may consider the use of medication. rather than too little attention. behavioral therapy & adjustments in day . The child may walk away from class or from situations where the child is expected to remain seated. The child cannot follow instructions. The child often has difficulty sustaining attention in tasks or play activities. (e. The child often has difficulty awaiting his/her turn. The child often has difficulty playing or engaging in leisure activities quietly.day lifestyle activities. The child often fidgets with his/her hands or feet or squirms in his/her seat. The child is often forgetful in daily activities. or other activities. The child often leaves his/her seat in the classroom or in other situations in which remaining seated is expected. The diagnosis is established by satisfying specific criteria & may be associated with other neurological.to . leading them to have little focus. The child cannot organize work. Hyperactivity • • • • • • • • • The child may make repeated mistakes at school. The following features are usually seen in children with ADHD The child often fails to give close attention to details or makes careless mistakes in schoolwork. and/or focusing too intensely on specific stimuli to the exclusion of what is relevant. The child often does not follow through on instructions & fails to finish schoolwork. actually have difficulty regulating their attention.• • • • This behavior makes it difficult for the child to adjust & adapt to the social environment in spite of having normal intelligence The term attention deficit is misleading. hyperactivity & impulsivity must have persisted for at least six months to a degree that is maladaptive & inconsistent with the developmental level of the child. The child often talks excessively.g. The child is forgetful in daily activities & tends to lose things. The child is often easily distracted by extraneous stimuli. dislikes. or tools). chores. inhibiting their attention to non relevant stimuli. or is reluctant to engage in tasks that require sustained mental effort (such as schoolwork or homework). work. many persons with ADHD (ADD) pay too much attention to too many things. In one sense. The child may not be able to play the same game for a long time. They tend to interrupt & disturb others. The child often runs about or climbs excessively in situations in which it is inappropriate. Impulsivity . attention deficit disorder (ADD). The child is very fidgety. books. and/or developmental/learning disabilities. climb tables. The child often does not seem to listen when spoken to directly. or duties in the workplace (not due to oppositional behavior or failure to understand instructions). The child often blurts out answers before questions have been completed. The child often loses things necessary for tasks or activities (toys. The child often has difficulty organizing tasks & activities. Three basic forms of ADHD (ADD) are 1) Attentional Inattention 2) hyperactive/impulsive 3) Combined • • • • • • Diagnostic Criteria • • • • • • • • • • 132 All of the symptoms of inattention. In general. the current predominating theories suggest that persons with attention deficit hyperactivity disorder (ADHD). significant behavioral.

Executive functions are major tasks of the frontal lobes. desipramine. anxiety etc.• • • • The child often interrupts or intrudes on others (for example.angle glaucoma (stimulants. desipramine) Heart disease (clonidine. delayed sleep phase syndrome) Consider screening patients for the following : • • • Sex • • • • • • Screen for the following medications or supplements • • • • • • • • • • • • . impulsive. The frontal cortex & the circuits linking them to the basal ganglia are critical for executive function and. imipramine) Urinary retention or hesitancy (atomoxetine. butts into conversations or games). ADHD (ADD) is more frequently diagnosed in boys than in girls. panic disorder. Most estimates of the male .to . expressive) Conduct disorder (oppositional defiant disorder in children) Depression Dissociative disorders Eating disorder Enuresis/encopresis Learning disability Pervasive developmental disorder including Asperger syndrome Posttraumatic stress disorder (PTSD) Psychotic disorders Sleep disorder (sleep apnea. Past medical history Anticonvulsant agents Antihypertensive agents Caffeine . guanfacine. Criteria for diagnosis of ADHD requires that some hyperactive. social phobia) Bipolar disorder Communication disorder (receptive. stimulants) Heart palpitations (stimulants) Hepatic disease (atomoxetine) Hypertension (stimulants.containing drugs Pseudoephedrine 133 • Ephedra Screening for medical concerns that may have negative interactions with ADHD (ADD) medications (Drugs of concern are shown in parentheses. bupropion) Orthostasis (atomoxetine.compulsive disorder [OCD]. psychosis. desipramine. Dreaming is a part of growing up & is considered normal. restless leg syndrome. clonidine) Seizure disorder (bupropion. Before labeling a child as ADHD. MRI in individuals with ADHD (ADD) also strongly supports weakened activity in the right inferior prefrontal cortex & left caudate during a task that involves timing of a motor response to a sensory cue. imipramine. atomoxetine.female ratio range between 3 :1 & 4 :1 in clinic populations. stimulants) Anxiety disorders (generalized anxiety disorder [GAD]. therefore. it is imperative to rule out physical causes of restlessness in children (commonest being worms). depression. Many children with learning disabilities may suffer from short attention. obsessive . or inattention symptoms that cause present difficulties were present before 7 years of age & are present in two or more settings (at school [or work] or at home). to attention & exercising inhibition. Also rule out epilepsy (an EEG may be recommended). bupropion. stimulants) Pregnancy (all) Renal disease (bupropion. Abnormal day dreaming is when the dreams are so intense that they interfere with day to day activity. bupropion. as they may be depressed as they are unable to comprehend regular studies.) • • • • • • • • • • • Pathophysiology • Major arterial disease (stimulants) Narrow . imipramine.

related disorders Thought disorder Tourette syndrome or other tic disorders Somatic comorbidity (No somatic comorbidities are significantly associated with ADHD [ADD]. toxin exposure.) Family history : Inquire about a family history of ADHD Social history : Inquire about the following : Anger or rage reactions are prevalent. marital or family . High activity level is noted. Report cards Reprimands or notes sent home Homework completion and/or turning homework in on time Extracurricular activities Drug abuse. snuff) Physical Although a child or adolescent with ADHD (ADD) may exhibit few symptoms in a clinical setting. alcohol abuse. Completion of multistep directions is difficult.g. A genetic predisposition has been demonstrated in (identical) twin & sibling studies. impulse control. at time of birth or even in the neonatal age group. Environmental causes • • • Alcohol Caffeine Other illicit drugs 134 No environmental causes have been clearly identified. However. planning & mental flexibility. heavy marijuana use beginning in early adolescence. problems with pregnancy (including cigarette smoking during pregnancy) and/or delivery. Vital signs Home & family interactions consistent with ADHD • • • • Height Weight Blood pressure Pulse General appearance o Fidgeting o Impulse control o State of arousal Mental status examination School performance • • • • Affect Cognition Speech patterns Thought patterns Family dysfunction Causes Abuse of substances by patient or his or her friends (if the patient is an adolescent) The exact cause of ADHD is still not known. Homework organization & completion are often a problem. Losing or forgetting material or conversations is observed. Animal studies have demonstrated differences in the chemistry of brain transmitters involved with judgment. head injuries.• • • • • • • • • • • • • • • • • • • • • • • • Substance . The child usually seems most awake in the late evening. careful observation of behavior is important. cigarettes. it is postulated that these children suffer from minimal brain damage either prenatal. or both Parent (s) with ADHD Physical abuse Sexual abuse Recent death of loved one or friend Severe chronic illness Severe financial problems • Tobacco (e. alertness. Awakening the child for school causes major problems. chewing tobacco.

Differential diagnosis • Anxiety Disorder : Generalized Anxiety Anxiety Disorder : Obsessive Compulsive Disorder Conduct Disorder Eating Disorder : Anorexia Eating Disorder : Bulimia Social phobia Psychotic disorders Post traumatic stress disorder (PTSD) Laboratory Studies Mood Disorder : Bipolar Disorder Mood Disorder : Depression • Teachers have an important function.g. narrative descriptions & telephone follow . Medications Workup in attention deficit hyperactivity disorder (ADHD) includes the following : Liver function tests • Determination of CBC counts • Drug screening • Imaging Studies The 2 major components in the medical care of children with attention deficit Categories of medications Psychostimulants are effective in patients with ADHD. “Hyper . they have been available for many decades. Psychometric & educational testing is often important for the diagnosis of ADHD (ADD). A seizure is hypersynchronous neuronal activity.up is generally an indispensable component of ongoing care. involuntary. In addition. tics. allowing for a strong appreciation of their lack of major adverse effects when used at therapeutic doses. Implementation of academic accommodations & adaptations is often necessary Behavioral modification & family therapy are usually necessary for optimal care. 40 Seizures / convulsions Medical Care The 2 major components in the medical care of children with attention deficit hyperactivity disorder (ADHD) are behavioral & pharmaceutical therapies. Their periodic feedback about the child’s school performance through the use of standardized scales. Central alpha agonists can be helpful in treating hyperactivity. paroxysmal. physical activity is often an important component of therapy. or delayed sleep onset.dysfunction & low social class have all been associated with ADHD.related disorders • • Activity Regular physical activity is important in patients with some of the common coexistent conditions (e. episodic clinical events associated with abnormal electrical activity from the neurons. Refer parent (s) for evaluation of ADHD. depression. Psychotherapeutics Anxiety Disorder : Separation Anxiety & School Refusal Pervasive Developmental Disorder : Asperger Syndrome Sleep Disorder : Night Terrors Sleep Disorder : Nightmares Sleep Disorder : Problems Associated With Substance . School or education interventions 135 • • Seizures are defined as an attack of recurrent. if suspected. anxiety) & helps improve concentration. Central alpha agonists • • Evidence suggests that MRI & positron emission tomography (PET) may be useful as future diagnostic methods. Therefore.

synchronous”, means “unsynchronized.” Seizures occur when the brain becomes irritable & abnormal electrical firings cause alterations in normal brain function. Usually, the patient becomes temporarily unresponsive & the • exact location of the electrical short circuits will determine what abnormal physical findings are witnessed. Based on the type of behavior & brain activity, seizures are • divided into 2 broad categories : 1) Generalized - Generalized seizures are produced by electrical impulses from throughout the entire brain 2) Partial (also called local or focal) - Partial seizures are produced (at least initially) by electrical impulses in a relatively small part of the brain. The part of the brain generating the seizures is sometimes called the focus. Absence seizures are a form of seizure in which a patient has a brief spell of staring blankly & becoming unresponsive for a few seconds to minutes.

1)

2)

3) 4)

The most common types of seizures are listed below Generalized Seizures Symptoms

(Produced by the entire brain)
1. “Grand Mal” / Generalized tonic - clonic 2. Absence (petit mal) 3. Myoclonic 4. Clonic 5. Tonic 6. Atonic Generalized seizures 1) Grand - mal seizure

Unconsciousness, convulsions, muscle rigidity Brief loss of consciousness Sporadic (isolated), jerking movements Repetitive, jerking movements Muscle stiffness, rigidity Loss of muscle tone

2)

In this type of seizure, the patient loses consciousness & usually collapses. Classically 4 stages are present namely 1) Aura 2) Tonic phase 3) Clonic phase 4) Post ictal phase
136

3)

Aura - These are a type of warning symptoms the child experiences before the onset of convulsion. This typically include epigastric discomfort or pain, feeling of fear Tonic phase - In this phase there is loss of consciousness followed by generalized body stiffening (hyper tonicity) for 30 to 60 seconds. Skeletal muscles go into continuous spasm. Some children may emit a shrill cry due to spasm of laryngeal muscles, eyes may roll back & child may rapidly become cyanotic with apnea. There may be frothing from mouth. Urine or stool may be passed involuntarily. Clonic phase - There is violent jerking for 30 to 60 seconds, along with rhythmic clonic contractions & relaxations. Post ictal phase - After this the child goes into a deep sleep (after - seizure phase). It is a semicomatose condition which may remain for 30 min. to 2 hrs of duration. During grand mal seizures, injuries & accidents may occur, such as tongue biting & urinary incontinence. There may be truncal ataxia, hyperactive deep tendon reflexes, clonus & Babinski sign may be positive. The child has no or little recollection of the episode. Absence seizures (petit mal epilepsy) - In this type of seizures there is a short loss of consciousness (just a few seconds) with few or no symptoms. Sudden stopping of motor activity or speech may be observed. There is a blank facial expression with flickering of eyelids or a staring look. These seizures begin & end abruptly & may occur several times a day. Patients are usually not aware that they are having a seizure, except that they may be aware of “losing time.” This type of seizure is never associated with aura, loss of posture, no incontinence of urine or stool & no breathing difficulty. Most common age of occurrence is 6 - 8 yrs. Myoclonic seizures - It consists of repetitive jerks, usually on both sides of the body. Patients sometimes describe the jerks as brief electrical shocks. When violent, these seizures may

4) 5)

6)

result in loss of body tone with dropping/slumping forward or involuntarily throwing objects. Clonic seizures - These are repetitive, rhythmic jerks that involve both sides of the body at the same time. Tonic seizures - Are characterized by stiffening of the muscles. This causes all of muscles to suddenly contract, so the child falls down. Atonic seizures - It consists of a sudden & general loss of muscle tone, particularly in the arms & legs, which often results in a fall.
Symptoms

one part of the body & then involves entire body due to spread of electrical discharge to other parts of brain is called as partial seizure becoming secondarily generalized.
Pathophysiology

1) 2) 3) 4)
1)

Hereditary/genetic theory Biochemical theory Electro physical theory Neuro pathological theory
Hereditary theory

Partial Seizures (Produced by a small area of the brain)

Epilepsy can be familial There may be multifactorial/polygenic inheritance
2) Biochemical theory

Simple (awareness is retained)

Complex (Impairment of awareness)

Partial seizure with secondary generalization

a) Jerking, muscle rigidity, spasms, head - turning b) Unusual sensations affecting either the vision, hearing, smell taste / touch c) Memory / emotional disturbances Automatisms such as lip smacking, chewing, fidgeting, walking & other repetitive, involuntary but coordinated movements Symptoms that are initially associated with a preservation of consciousness that then evolves into a loss of consciousness & convulsions.

a) NA+ – K+ ATPase theory – NA+ – K+ pump – in resting phase the nerve cells are polarized due to differences in concentrations of ions across the cell membrane. This is called polarized state or RMP. At rest the charge on outside is + & inside is –. When stimulated permeability of membrane to K & NA ion is changed. NA floods inside the neuron. This is called depolarization creating nerve impulse or action potential. In repolarization K ions flood out of the neurons & movement of these ions returns the membrane potential to its resting state. As the neuron returns to its original resting state the action of sodium potassium pump (Na - K ATPase) expels NA from the cell in exchange for K. In epileptic cortices (brain) levels of Na - K ATPase (enzyme)are low Na leaks into the neuronal cell &K remains outside RMP altered Depolarization
137

Partial seizures

It often consists of change in one small thing, like a single motor disturbance, personality change & strange feelings. These are the only non generalized seizures. The electrical discharge begins in one part of the brain leading to involvement of only one side of body. They are also called focal seizures. If the patient remains conscious during the episode it is known as simple partial seizure & if becomes unconscious, it is known as complex partial seizure. A fit that starts in

Electrical discharge (until depolarization continues)
b) Neurotransmitter theory

Imbalance in the neurotransmitter concentrations is a major cause of seizures.2 main types of neurotransmitters are present excitatory (acetylcholine) & inhibitory (gamma amino butyric acid)
Formation of GABA

• • • • • • •

Glucose + protein = glutamine – GABA (Cofactor pyridoxine B6) In newborns vit B6 deficiency is present. Hypoglycemia can lead to seizures.
c) Status epilepticus induced biochemical changes

Epilepsy. Head injury. Fever, causing febrile convulsions. Infection - especially meningitis & encephalitis. Metabolic problems such as hypoglycemia, hypoxia & hyponatremia, or thyroid storm. Increased ICP, commonly from brain haemorrhage or brain tumor. Drug abuse or medication withdrawal.

Investigations

In status epilepticus, there is continuous firing by the neurons which leads to increased motor action. This induces some biochemical changes like Hypoxia (due to high utilization & increased demand of O2) Hypoglycemia (due to high utilization & increased demand of glucose) Increased heat production due to increased motor activity (Vicious cycle) BMR Increased O2 & glucose demand
3) Electro physical theory

If a child suddenly has a seizure with no previous history, screening for intracranial causes with a head CT, ABG in order to rule out the metabolic causes. Next step is an EEG to scan the brain’s electrical patterns. It’s most effective during a seizure, but some children with epilepsy will have abnormal patterns even when they are not in the middle of a seizure.
Management of acute episode

41

It is characterized by recurrent, episodic, paroxysmal, involuntary clinical events in which clusters of nerve cells, or neurons, in the brain sometimes signal abnormally. In epilepsy, the normal pattern of neuronal activity becomes disturbed, causing strange sensations, emotions & behavior, or sometimes convulsions, muscle spasms & loss of consciousness. During a seizure, neurons may fire as many as 500 times a second, much faster than normal. For about 80 percent of those diagnosed with epilepsy, seizures can be controlled with modern medicines & surgical techniques. However, about 25 to 30 percent of people with epilepsy will continue to experience seizures even with the best available treatment. This is called as intractable epilepsy.

RMP altered neuronal cells excited neuronal firing Stimulus (decreased threshold) Causes seizures.
4) Neuro pathological theory

• •

Pathological lesions of the brain like tumors, scars, infections, inflammation & vascular block can lead to convulsions.
Differential Diagnosis
138

A single episode of seizure does not necessarily mean that a child has epilepsy. Only when a child experiences two or more seizures; he is considered to have epilepsy. Epilepsy is not contagious & is not caused by mental illness or mental retardation. Some children with mental retardation may experience seizures, but seizures do not necessarily mean the child has or will develop mental impairment. Many children with epilepsy have normal or above - average intelligence. Famous people who are known or rumored to have had epilepsy include the Russian writer Dostoyevsky, the philosopher Socrates, the military general Napoleon & the inventor of dynamite, Alfred Nobel, who established the Nobel Prize. Several Olympic medalists & other athletes also have had epilepsy. Prolonged refractory seizures can lead to brain damage. Some patients may develop poor memory due to epilepsy or it treatment. Excessive stress, worry & anxiety can also precipitate seizure. Sometimes epilepsy may be associated with a metabolic disorder in which case certain food items may have to be omitted. Lack of sleep may also precipitate seizures. Video games due to its flickering light & changing patterns of images can precipitate seizures. Certain drugs such as antidepressants, antibiotics, theophylline may provoke a seizure. Most children with well - controlled seizures & no additional handicap can attend a normal school. Extra curricular activity such as swimming, climbing & cycling on road should be avoided. Informing the schoolteacher about the child’s disorder will aid in helping them to know what to expect when a child has a seizure & decide what to do. EEG may be abnormal in only about 60% of patients with epilepsy. Also, a small proportion of general population may have epileptiform abnormalities in EEG. EEG is useful in identifying type of epilepsy, detecting a structural brain abnormality; differentiate between true seizures
139

& pseudoseizures.
Why do some patients continue to have seizures despite treatment? There could be several reasons

• • • • • •

Inappropriate drug for a particular kind of seizure Incorrect dose of drug Irregular treatment Underlying condition is getting worse Patient does not have epilepsy at all Patient has true drug resistant seizure.

What are the side effects of anticonvulsant drugs?

Certain drugs such as phenobarbitone or phenytoin can cause memory disturbance. Some drugs may cause cosmetic side effects such as increase in gums or weight gain. 20% of patients have refractory seizures in spite of being on appropriate anti - seizure medication. These patients can be considered for epilepsy surgery. Some patients may respond to diet modification. Ketogenic diet (high fat diet) is used in small group of children with resistant seizures.
The following factors have a poor outcome

• • • • • • • • •

Epilepsy difficult to control More than one anti - seizure drug is required to control seizures Epilepsy is associated with structural brain damage Repeated episodes of prolonged seizures Associated mental retardation or cerebral palsy Seizures that start early (before 2 years of age). Epilepsy that starts between 5 & 13 years of age Epilepsy which is inherited Seizures that are easily controlled & require only drug to control it.

Following factors are associated with a good outcome

Measures to be taken at the time of seizure

Once a patient has seizures, clear the mouth to prevent difficulty in breathing. Put nothing in the mouth as it will not prevent biting but

including trotting jumping. beginning in childhood.Terrestrial locomotion Travel Locomotion may refer to specific types of motion : Gait analysis walking running. Many of the classification systems for mental retardation have been based on the timing of the insult to the CNS.may obstruct breathing. The Diagnostic & Statistical Manual of Mental Disorders. or prematurity Hereditary abnormalities. It may be defined as a condition where a child is unable to learn & perform normally due to low intelligence.pitched voice & is caused by a deletion in chromosome 5p3. home living.chat syndrome.care. Physical causes are evident in most cases of moderate . self . It may refer to Motion (physics) Animal locomotion . such as inborn errors of metabolism • or chromosomal aberrations Medical conditions of infancy or childhood.du . health & safety Onset before age 18 years 42 • • • • • • • • • • • The term locomotion means movement / travel. A disadvantaged environment is more likely in mild retardation.direction. trauma.to profound retardation. Do not try to hold the tongue. The successive classification systems developed by the American Association on Mental Retardation also followed the timing approach. • resources. Text Revision (DSM .An intelligence quotient (IQ) of approximately 70 or below Concurrent deficits or impairments in adaptive functioning in at least 2 of the following areas : communication. infection. social/interpersonal skills. such as • autism Problems of pregnancy & the perinatal period. such as central • nervous system (CNS) infection or trauma. The best known example is cri . hypoxia. use of community 140 The causes of mental retardation can be grouped from most to least common as follows : Alterations in embryonic development. self . leisure.Genetic Disorders • • Prenatal genetic disorders are characterized by changes in the genetic material. that results in significant limitation of intellect or cognition & poor adaptation to the demands of everyday life.TR) defines mental retardation as follows : Significantly subaverage intellectual functioning . such as fetal • malnutrition. functional academic skills. Call for a doctor & ambulance if seizures last more than 10 minutes. Turn the patient on the side so that tongue does not fall back & obstruct the airway.IV . Prenatal Causes . including leaping gaits crawling climbing swimming flying Causes 43 • Mental retardation is a state of developmental deficit. Chromosomal aberrations • • Down syndrome Deletions A loss of part of a chromosome is called a deletion. which may or may not have been inherited from the parents. Fourth Edition. work. such as those caused • by chromosomal abnormalities or fetal exposure to drugs or toxins Environmental deprivation & other mental disorders. or lead poisoning. which is characterized by a high . • • .

which originate in the period of organogenesis. psychomotor retardation. with dominant inheritance & the penetrance is lower in females. When used heavily during pregnancy. and 3) CNS dysfunction.linked mental retardation Fragile X syndrome is the most common inherited form of mental retardation and. Disorders with autosomal . alcohol causes abnormalities in 3 main categories: 1) Dysmorphic features. delay in motor development. the most important infection. the more severe their effect 141 During the neonatal period. Disorders with autosomal .3. which leads to a high serum phenylalanine level. are delayed. which might be associated with mental retardation.g. They include an oblong face. affecting. Congenital cytomegalovirus infection may result in microcephaly. which is the cause of fetal alcohol syndrome (FAS). 000 live births. Phenylketonuria (PKU) is the best known & most common of the metabolic disorders. The enzymatic defect is diminished activity of phenylalanine hydroxylase. which. as are eczema & psychotic manifestations.• • Malformation syndromes due to micro deletions Prader . hydrocephalus. They are caused by single mutated genes that disturb the metabolism by deficient enzyme activity. leading to obesity. myelination of the CNS. it may remain undiagnosed & such individuals might even be referred for psychiatric treatment because of an eating disorder. including mild . The severity of the symptoms is related to the amount of alcohol ingested. as exemplified by congenital rubella. ending in insult to the CNS. Infections Viral infections in the mother can interfere with organogenesis & the earlier in pregnancy they occur. after Down syndrome. is herpes simplex type 2. microcephaly. from the point of view of its developmental sequelae. it appears as if the chromosome is fragile & a part of it is breaking off. Neonatal bacterial infections might result in sepsis & meningitis. may cause hydrocephalus. It is X linked. X . the most important being maternal toxemia with its consequences. in turn.Willi syndrome has an excessive appetite & indiscriminate eating habits. including microcephaly. Because of a constriction at the location Xq27. Maternal infections The most important of the teratogenic substances is ethanol. Seizures & tremors are common. among other things. Rubella infection during the first month of pregnancy affects the organogenesis of 50% of embryos. vision & hearing impairment) & in milder developmental problems later in life. the characteristic phenotypical features may appear. the most common genetic form.recessive inheritance will be. hyperactivity & attention deficit. Toxemia of pregnancy & placental insufficiency Intrauterine growth retardation has many causes. with a prevalence of approximately 1 in 10.dominant inheritance Tuberous sclerosis is an example of the disorders in this group. prominent ears & jaw & macroorchidism. Toxic substances Most metabolic disorders belong to this category.to .moderate mental retardation. especially speech development. Because this syndrome has no clear pathognomonic features. Their developmental milestones. 2) Prenatal & postnatal growth retardation. sensorineural hearing loss & psychomotor retardation Congenital toxoplasmosis may result in significant problems in approximately 20% of infected infants (e. After puberty. Perinatal Causes This period refers to 1 week before birth to 4 weeks after birth. Delivery problems .

Toxic substances Lead poisoning is still an important cause of mental retardation. 70 . Children with borderline mental retardation are usually low • . Psychosocial problems 142 Mental retardation can be diagnosed by assessing the intelligent quotient (IQ) of the child. causes various symptoms of cerebral palsy (CP) & seizure disorder. malnutrition.80% are gliomas. Some are benign & treatable.51 is mild mental retardation & IQ between 50 . Other perinatal problems Retinopathy of prematurity is seen frequently when the use of 100% oxygen in neonates is common.90 is said to have borderline mental retardation. in addition to affecting intelligence. drowning & other traumas are the most common causes of death during childhood. resulting in various neuropsychiatric symptoms depending on their location & extent. Poverty predisposes the child to many developmental risks. brain tumors are second in frequency after leukemias. depending on the location of the pathological condition. mental retardation & other developmental problems. Traffic accidents. Other postnatal causes Among childhood malignancies. deafness. symptoms of which depend mostly on location. It is often associated with other CNS damage.based paint). seizures & other defects. Assessment Bacterial & viral infections of the brain during childhood may cause meningitis & encephalitis & result in permanent damage. speak & • carry out daily routine activities. which. Severe maternal mental illness is another risk factor. no cause can be identified in approximately 30% of cases of severe mental retardation & in 50% of cases of mild mental retardation. They are fully dependent on others for care & survival. It leads to cell death. Even greater is the number of children who become disabled. Postnatal Causes Infections The developmental level of a growing individual depends on the integrity of the CNS & on environmental & psychological factors. Unknown causes Despite detailed assessment. resulting in blindness. Children with mild mental retardation are able to talk. Infants with extremely low birth weight are at risk for intracranial hemorrhage & hypoglycemia resulting from a lack of hepatic glycogen storage. but most have deleterious effects. Children with severe & profound mental retardation have a • significant amount of brain damage & may have associated blindness. Children of mothers who have schizophrenia are at risk for the development of cognitive deficits. Children with moderate mental retardation can become partially • independent with special training aimed at self . old. abuse.e. The importance of environmental stimulation for child development has been appreciated since research on children in institutions showed that development was severely affected in a depriving environment.36 is moderate mental retardation. although these may not be secondary to maternal illness but may represent a genetically determined predisposition to schizophrenia. lead . ingestion of flaking. such as teenage pregnancies. IQ between 70 . They can be educated to a certain extent & can lead an independent life. poor medical care & deprivation. Premature infants & those with intrauterine growth retardation are at special risk for damage to the cortex or thalamus. A child with IQ between 70 .35 is severely retarded & those with IQ less than 20 have profound mental retardation. The most frequent source of lead is pica (i. A child with IQ between 21 .During delivery.help skills. even if adequate physical care was provided. asphyxia is the most important factor causing an insult to the CNS. Of these. which might be demonstrated with neuroimaging techniques as leukomalacia.

including adverse effects of medications. Deafness. Modulation of physical discomfort : Medical conditions.• • • • • achievers in school & by proper training can lead an independent normal life. such as a precipitous move to a new setting or change in care provider. are quite common in persons with mental retardation. Modulation of emotional discomfort : Problem behaviors can occur as a state . discomfort.injurious behavior. may trigger a depressive episode. Anxiety disorders Verbal persons with mild mental retardation can report on subjective feelings of anxiety. for example. Depression also may manifest as aggressive behavior. However. Environmental events. Adverse effects of medications should be considered. hoarding objects. flicking lights on & off & tidying & arranging.compulsive disorder (OCD) may be difficult in nonverbal persons who cannot report on obsessional thoughts underlying their compulsions. all have been suggested as indications of OCD in persons with mental retardation. 143 Anorexia & bulimia nervosa are relatively rare in the context of mental retardation. particularly moderate to severe mental retardation. leading to aggression or self . manic phase.A child with a normal intelligence may appear to be mentally retarded due to other problems that prevent him/her to perform adequately. needs). ADHD should be differentiated from situation . such as at school if the academic expectations are too high & adverse medication effects. Some repetitive behaviors. Attention deficit hyperactivity disorder The diagnosis of obsessive . emotional problems. Eating disorders The diagnostic criteria for attention deficit hyperactivity disorder (ADHD) are based on observable behavior as reported by multiple informants & thus can be applied to nonverbal children. removing a person from an unpleasant situation in response to such behavior will increase the probability that the person will react similarly in the future).specific inattentiveness.blockers or agitation associated with akathisia from a neuroleptic drug. Pseudo mental retardation . In nonverbal persons. especially depressive disorders. as follows : Socio environmental control : Aggression & self . appropriate to their developmental level. blindness. symptoms such as avoidance behaviors & agitation might suggest the diagnosis.injurious behavior can be reinforced (i. but mental retardation is a predisposing factor for other eating disorders such as pica & rumination. attention. for example.dependent function of disorders such as major depression or bipolar disorder. depression resulting from beta . speech problems. Lowry & Sovner describe 4 functions of problem behavior that should be considered in any evaluation. cerebral palsy & psychiatric problems may be associated factors leading to inadequate performing skills. The tendency toward anxiety & social avoidance also is a part of the behavioral phenotype of fragile X syndrome.compulsive disorder Specific Diagnosis of Common Comorbid Mental Disorders Pervasive developmental disorders Most children with pervasive developmental disorders also have mental retardation.g. children with mental retardation alone do not have significant impairments in reciprocal social interaction & can engage in social communication.e. Communication : Problem behaviors can be a nonverbal means of communicating a variety of messages (e. verbal or nonverbal (such as gestures & eye contact). can cause physical discomfort. Obsessive . Posttraumatic stress disorder Posttraumatic stress disorder in persons with mental retardation might be quite frequent & should be routinely considered in the differential diagnosis. Mood disorders Mood disorders. .

Development landmarks (e. Management of mental retardation in a child requires a multi pronged approach.g. contingent restraint o Behavioral parent & teacher/staff training to help them function as co . proper screening & treatment for vision & hearing problems & intervention by child psychologist. Seizures Stigmatization Taunts. such as contingent reward for specific behaviors that are incompatible with problem behavior o Behavior . temperature. roommate. Serious acute illness. or rejection Breakup of romantic attachment. disorganization. extinction. intellectual problems Frustration Due to inability to communicate needs & wishes. hearing & speech & communication & a higher occurrence of seizure disorders. Being fired from a job or suspended from school Environmental Overcrowding.g. School or work stress Parenting & social Lack of support from family.accelerating procedures. or partner. overcorrection. Reduced privacy in congregate housing. achieving majority) Interpersonal loss Loss of parent. Due to lack of choices about residence. time out. altered route to work. pediatrician.term stressors that may be triggering or exacerbating psychiatric disorders or behavioral problems in children with mental retardation.Treatment Planning & Stressors That May Trigger Problems A complete evaluation & an individualized treatment plan require attention to the possible short . Stressors That May Trigger Behavioral Problems Types of Stressor Examples Transitional phases Change of residence. or letters.Reducing problem behaviors by • rearranging physical and/or social conditions that seem to provoke them. the incidence • of concomitant sensory & motor deficits increases. going into puberty. Difficulty with ambulation. Neglect. A greater degree of mental retardation suggests a greater amount • 144 of brain damage. crowding) and/or enrichment of environment through social or sensory stimulation Client and/or family education . new school or work place. caregiver. work situation. Hostility. phone calls. Each child needs individualized therapy & needs special education facilities. diet. excessive noise.term & long . Lack of satisfactory stimulation.decelerating techniques. response cost. support problems Destabilizing visits. physiotherapist/occupational therapist & speech therapist to help him/ her achieve development to the best of his/her ability. reflected in a higher occurrence of impaired vision. Sensory defects. Physical or sexual abuse Illness / disability Chronic medical or psychiatric illness. examples include changes in activities (e. The 3 behavioral interventions recommended by the expert consensus panel for most situations are as follows : Applied behavior analysis . during which the problem behavior did not occur. Because of realization of deficits With increasing severity of mental retardation. friend.Techniques that are based on the • principles & methods of behavior analysis & are intended to build appropriate functional skills & reduce problem behavior include the following : o Behavior . such as contingent reward for specified time periods. noise. teasing. exclusion.Helping clients and/or families • understand more about the behavioral psychiatric problems that may accompany mental retardation & how to manage them Psychopharmacological Treatment . lighting. because of physical/ being bullied / exploited. friends.therapists and/or to avoid incidental reinforcement of the problem behavior Managing the environment .

after upper respiratory infection (URI). despite frequent use of systemic antibiotics to treat the illness & its complications. Chronic suppurative OM (CSOM) is a chronic inflammation of the middle ear that persists at least 6 weeks & is associated with otorrhea through a perforated TM. an immature immune system & frequent infections of the upper respiratory mucosa all play major roles in AOM development. • duration. otalgia. In ET dysfunction (ETD). irritability). developmental alterations of the ET. behavioral problems respond to psychosocial interventions. causing OM. the mucosa at the pharyngeal end of the ET is part of the mucociliary system of the middle ear. formerly termed serous OM or secretory OM is MEE of any duration that lacks the associated signs & symptoms of infection (e. OM is any inflammation of the middle ear without reference to • etiology or pathogenesis. symptomatology & physical findings. Acute OM (AOM) implies rapid onset of disease associated • with one or more of the following symptoms : Otalgia • Fever • Otorrhea • Recent onset of anorexia • Irritability • Vomiting • Diarrhea • These symptoms are accompanied by abnormal Otoscopic findings of the tympanic membrane (TM). Other clinical conditions require psychopharmacologic treatment at the outset. However. The host immune & inflammatory response to bacterial invasion of the middle ear produces fluid in the middle ear & the signs & symptoms of AOM. Intra temporal complications • • • • • • Hearing loss (conductive & sensorineural) TM perforation (acute & chronic) Chronic suppurative OM (with or without cholesteatoma) Cholesteatoma Tympanosclerosis Mastoiditis . situations exist in which psychotropic medications can enhance a patient’s response to psychosocial treatment. Certain viral infections cause abnormal host immune & inflammatory responses in the ET mucosa & subsequent microbial invasion of the middle ear. tumor. an indwelling tympanostomy tube (TT). Pathophysiology 44 Otitis media (OM) is the second most common disease of childhood. OME usually follows an episode of AOM. Interference with this mucosa by edema. Mortality / Morbidity Morbidity from this disease remains significant. In children. 145 • The most important factor in middle ear disease is eustachian tube (ET) dysfunction. or a surgical myringotomy.In many individuals with mental retardation. Esophageal contents regurgitated into the nasopharynx & middle ear through the ET can create a direct mechanical disturbance of the middle ear mucosa & cause middle ear inflammation. which may include the following : Opacity • Bulging • Erythema • Middle ear effusion (MEE) • Decreased mobility with pneumatic otoscopy • OM with effusion (OME). or negative intratympanic pressure facilitates direct extension of infectious processes from the nasopharynx to the middle ear. OM can be classified into many variants on the basis of etiology.g. fever. Intra temporal & intracranial complications of OM are the 2 major types.

sinus congestion) 146 Pneumatic otoscopy remains the standard examination technique for patients with suspected OM. cough.g. rhinorrhea. with or without effusion.18 months. General • • • Intracranial complications History of fever. through a preexisting TT. Children who are diagnosed with AOM during the first year of life are much more likely to develop recurrent OM & chronic OME than children in whom the first middle ear infection occurs after age 1 year. GI tract Exposure to environmental risk factors is another important aspect of the history & includes the following • • • • • • Passive exposure to tobacco smoke Group daycare attendance Seasonality : AOM prevalence is much higher in winter & early spring than in summer & early fall. Host factors • Immune system : The immature immune systems of infants or the impaired immune systems of patients with congenital immune deficiencies. Otorrhea : Discharge may come from the middle ear through a recently perforated TM. or diabetes may be involved in the development of OM. Symptoms include anorexia. allergic & environmental factors contribute to OM development. Irritability may be the sole early symptom in a young infant or toddler. A history of lethargy. nausea. Tympanometry. Proper pneumatic otoscopy technique is crucial to distinguish AOM from OME because recommended therapies for these entities are significantly different. vomiting & diarrhea. or through another perforation.e. acoustic reflectometry & audiometry are important adjunctive techniques with which to evaluate patients with MEE.• • • • • • • • • • • Age Petrositis Labyrinthitis Facial paralysis Cholesterol granuloma Infectious eczematoid dermatitis Meningitis Subdural empyema Brain abscess Extradural abscess Lateral sinus thrombosis Otitic hydrocephalus Peak prevalence of OM in both sexes occurs in children aged 6 . HIV infection. is a sensitive marker for sick children & should not be dismissed. Causes A multitude of host. in children with a history of the following symptoms : Head & neck • • • • Otalgia : Young children may exhibit signs of otalgia by pulling on the affected ear or ears or pulling on the hair. although fevers greater than 40°C are uncommon & may represent bacteremia or other complications. excluding a basilar skull fracture with associated cerebrospinal fluid (CSF) otorrhea is important. infectious. although nonspecific. . Headache Concurrent or recent URI symptoms (e. Supine bottle feeding (i. bottle propping) Physical History Suspect acute otitis media (AOM). For trauma patients.

Klebsiella species & Pseudomonas aeruginosa) play a much larger role in AOM. In infants younger than 6 weeks. Physiologic dysfunction : Abnormalities in the physiologic function of the ET mucosa. Infant feeding methods The protective effects of breastfeeding for the first 3 .• • • • • • Familial (genetic) predisposition : Although familial clustering of OM has been demonstrated. Many patients with OM have positive results to skin testing or radioallergosorbent testing (RAST). asthma). Down syndrome & Treacher Collins syndrome. Environmental factors • • Infectious factors Bacterial pathogens • • The most common bacterial pathogen in AOM is Streptococcus pneumoniae. The middle ear mucin gene expression is unique compared with the nasopharynx. allergic rhinitis. influenza viruses. Obesity has been linked to an increased incidence of OM. possibly because delaying onset of the first OM episode reduces recurrence of OM in these children. OM is most common in the winter & early spring. yet most major allergens (e. 147 • • • . Specific anomalies that correlate with high prevalence of OM include cleft palate. Human parechovirus 1 (HPeV1) infection is associated with OM & cough in pediatric patients. Brief list of evidence for & against the etiologic role of allergy in OM • • • Many patients with OM have concomitant allergic respiratory disease (e. These 3 organisms are responsible for more than 95% of all AOM cases with a bacterial etiology. followed by nontypeable Haemophilus influenzae & Moraxella catarrhalis. Mucins : The role of mucins in OME has been described. which increases the risks of respiratory infection.12 months after breastfeeding ceases.g.g. the immune system is still developing & allergies are unlikely to play a role in recurrent AOM in this age group. especially the tensor veli palantini. Speculations include alteration of intrinsic cytokine profile. especially upregulation of MUC5B in the ear. tree & grass pollens) peak in the late spring & early fall. including ciliary dysfunction & edema.negative bacilli (e.g. parainfluenza viruses. Abnormalities of this gene expression. Mucins are responsible for gel . no specific genes have been linked to OM susceptibility. Crouzon syndrome or Apert syndrome. increase the risk of bacterial invasion of the middle ear & the resultant OME. and/ or fat accumulation. Escherichia coli. exhibit marked ETD & have higher risk for OM. gram . S pneumoniae & H influenzae are also the most common pathogens in this age group. Anatomic abnormality : Children with anatomic abnormalities of the palate & associated musculature. In children younger than 4 years. Vitamin A deficiency is associated with pediatric upper respiratory infections & AOM. Viral pathogens • • The viruses most commonly associated with AOM are respiratory syncytial virus (RSV). rhinovirus & adenovirus. although the causal factor is unknown. may have a predominant role in OME. all of these have been linked with an increased incidence of OM. Passive smoke exposure Group daycare attendance Daycare centers create close contact among many children. Factors related to allergies The relationship between allergies & OM remains unclear. increased gastroesophageal reflux with alterations of the oral flora.6 months persist 4 .like properties of mucus secretions.

AOM may be the first presenting illness in some of these diseases. OM is associated with multiple systemic diseases & congenital syndromes. age. In patients in whom an OM complication is suspected.ear inflammation. Acetaminophen & ibuprofen are first . The mean duration of the effusions is 23 .feeding. the imaging study of choice is a contrast . MRI is usually performed following CT if further information is needed for definitive diagnosis. identify signs of MEE & evaluate for the presence of signs & symptoms of middle . crowded group daycare. Tympanometry may help with diagnosis in patients with OME. Differential Diagnoses • Imaging studies are not indicated in patients with OM unless intratemporal or intracranial complications are suspected. If pain is present. order appropriate laboratory studies to confirm or exclude possible systemic or congenital diseases. 148 Medical therapy Most cases of OME occur after an episode of AOM & 67% of patients develop an MEE. especially small middle ear collections. Some practitioners also use acoustic reflectometry to evaluate for MEE in patients with OM. OM is one of the most common disorders in children & concern regarding antimicrobial resistance due to aggressive antibiotic use is growing.nasopharyngeal colonization with pathogenic microbes & OM. illness severity & assurance of follow . the clinician should recommend treatment to reduce pain. the clinician should confirm a history of acute onset. In general. Socioeconomic factors Imaging Studies • • Socioeconomic status encompasses many independent factors that affect both the risk of OM & the likelihood that OM will be diagnosed.line drugs for pain reduction. lower socioeconomic status confers higher risk for environmental exposure to parental smoking. MRI is more helpful in depicting fluid collections. therefore. bottle .up. Bacterial Nasal Polyps Nasopharyngeal Cancer Otitis Externa Otosclerosis Parainfluenza Virus Infections Passive Smoking & Lung Disease Pharyngitis Pneumococcal Infections Primary Ciliary Dyskinesia Respiratory Syncytial Virus Infection Rhinovirus Infection Sinusitis Other Tests • • Medical Care Guidelines for medical management of AOM • • • • To diagnose AOM. Allergic Rhinitis Apert Syndrome Bacteremia Cholesteatoma Cleft Lip & Palate Colic Diarrhea Down Syndrome Fever in the Toddler Fever in the Young Infant Fever Without a Focus Gastroenteritis Gastroesophageal Reflux Haemophilus Influenzae Infection Head Trauma Hearing Impairment Human Immunodeficiency Virus Infection Laboratory Studies Mastoiditis Meningitis.enhanced CT scan of the temporal bones. The management of AOM should include an assessment of pain. crowded living conditions & viruses & bacterial pathogens. Observation without use of antibacterial agents in a child with uncomplicated AOM is an option for selected children based on diagnostic certainty.

decongestants • Intranasal & systemic steroids • Nonsteroidal anti . or who appear toxic Unsatisfactory response to antimicrobial therapy OM in a newborn. Short stature. is recognized by comparing an individual child’s height with that of a large population of a similar genetic background and. genetically relevant population. Turner’s syndrome . such as those with cleft palate.parental target height.down syndrome.days. GF is a pathologic state of abnormally low growth rate over time. Most cases of OME spontaneously resolve. Growth failure (GF) is often confused with short stature. frequently recurrent tonsillitis.nourished. may require early surgical intervention to prevent OM. Skeletal maturation is typically determined by the bone age. Studies of the natural history of this disease report the following : The following are among the many strategies advocated for medical treatment in patients with OME : Antimicrobials • Antihistamine . tonsillectomy may be performed concurrently with surgery for OM if indications are present (e. more particularly.If upper limbs do not reach mid pelvis in infancy & upper thigh after infancy. Down syndrome. Short stature can be promptly recognized only with accurate measurements of growth & critical analysis of growth data. Failure of physical. any of whom may harbor an unusual organism Initial surgery : Myringotomy & TT placement are the initial surgical techniques (withhold adenoidectomy unless the patient has a nasal obstruction). whereas short stature is often a normal variant. or patient who is immunologically deficient. Indications for tympanocentesis o o o • • OM in patients who have severe otalgia. An adult male with height < 130 cm & female < 122 cm are called dwarf. short stature is defined as a standing height more than 2 standard deviations (SDs) below the mean (or below the 2.inflammatory drugs (NSAIDs) • Mucolytics • Aggressive management of allergic symptoms • Surgical Care • • • • • • Certain special patient populations. which is assessed using anteroposterior radiography of the left hand & wrist. Regardless of the genetic background. mental & sexual development is referred as infantilism.5 percentile) for sex. • • Short stature is defined as length/height below 3rd percentile for age. Tonsillectomy : Although it is not indicated for treatment of OM because it has not been shown to benefit ET function. optimally defined relative to the genetic endowment of the individual. sick neonate. using the mid .g. Compared with a well . pharyngeal obstruction). Nutritional deprivation IUGR Endocrinal disorders Genetic disorders . By definition. short stature may be a sign of a wide variety of pathologic conditions or inherited disorders. Proportionate short stature Causes A) 45 Short stature 149 Features . who are seriously ill. but many persist much longer. or other craniofacial abnormalities.

normal growth velocity & predicted adult height appropriate to the familial pattern. • malnutrition remains the leading cause of short stature worldwide. The hallmarks of familial short stature include bone age appropriate for chronologic age.Turner syndrome (45. menarche reached when older than 15 y. With short limbs Achondroplasia Chondrodysplasia Hypochondroplasia Diastrophic dysplasia b. B) Disproportionate short stature (skeletal/bone dysplasias/genetic causes) Features – short neck.Weill dyschondrosteosis (SHOX gene) Differential Diagnoses Pathophysiology 150 Achondrogenesis Hypercalcemia Achondroplasia Hypercalciuria Acidosis Respiratory & Metabolic Hypogonadism Hyponatremia Adrenal Hypoplasia Hypopituitarism Adrenal Insufficiency Hypothyroidism . pathophysiology depends on the underlying cause. History & examination is normal.degree relative with constitutional growth delay (e. Endocrine diseases are rare causes of short stature. Causes 1) 2) 3) • • • • Chronic disease (including under nutrition genetic disorders). small chest. protuberant abdomen. Familial (genetic) short stature Parents or close relatives are short statured. Therefore. constitutional growth delay is characterized by delayed bone age. By contrast. Short stature can also be the sign of a wide variety of pathologic conditions or inherited disorders when it results from GF or premature closure of the epiphysial growth plates. With short trunk Spondyloepiphyseal dysplasia Mucopolysaccharidosis Caries spine Hemi vertebrae The causes of short stature can be divided into 3 broad categories Short stature may be normal. XO) Lerí . Genetic causes of short stature • • • Down syndrome (trisomy 21) Ullrich . Adolescent growth spurt & sexual maturation is delayed. Patients with constitutional growth delay typically have a first . Chronic diseases of childhood : Among the chronic conditions. Birth length near 3rd percentile. Age is normal.degree or second . Obtaining the family history of growth patterns & direct measurement of the parents is crucial to determine the genetic potential for growth in the child. The non endocrine causes of short stature can be divided into 3 major categories. After that growth decreases. as follows : Constitutional delay of growth & sexual development • Birth length is normal & infants grow normally for few months.Chronic diseases Emotional deprivation. adult height attained in male relatives when older than 18 y).g. normal growth velocity & predicted adult height appropriate to the familial pattern. Familial (genetic) short stature & Constitutional delay of growth & development. • • a. Finally achieved height is normal. The hallmark of endocrine disease is linear GF that occurs to a greater degree than weight loss.

Boys do appear more likely to have idiopathic GHD or constitutional delay of growth & development. & the final (or current) heights & weights of parents. The evaluation of a short female. Whenever possible. or a female with primary amenorrhea.Russell syndrome Shwachman . prior growth pattern.Russell Syndrome Down Syndrome Sprue Eating Disorder : Thyroiditis Anorexia & bulimia Turner Syndrome Failure to Thrive Ulcerative Colitis Glycogen .Storage Disease Type I Williams Syndrome Growth Failure Growth Hormone Deficiency Human Immunodeficiency Virus Infection Causes of normal variant short stature (also called familial short stature) Genetic (known defect) • • • • • • • • • • • Renal Insulin receptor gene mutations (leprechaunism) Silver . siblings & grandparents.Russell syndrome Hypochondroplasia Turner syndrome Growth hormone (GH) receptor gene mutations (Laron syndrome) GHRH gene mutations GH gene mutations 151 Boys who are short are more likely to come to medical attention than girls who are short.e. obtain the original birth records to • . Chronic Late Effects of Childhood Cancer & Treatment Bartter Syndrome Malabsorption Syndromes Bone Marrow Transplantation Marasmus Congenital Adrenal Hyperplasia Constitutional Growth Delay Microphallus Noonan Syndrome Crohn Disease Panhypopituitarism Cryptosporidiosis Pellagra Cystic Fibrosis Rickets Diarrhea Short Bowel Syndrome Disorders of Bone Mineralization Silver . History Key data to obtain for the evaluation of short stature include the child’s weight & length at birth. mandates a karyotype to exclude this disorder.Alkalosis Metabolic & Respiratory Irritable Bowel Syndrome Laron Syndrome Anemia. TS) affects only females. Ullrich Turner syndrome (i.Diamond syndrome CF Severe asthma Chronic obstructive pulmonary disease Restrictive lung disease Hypoxemia Congestive heart failure Low cardiac output states Precocious puberty Chronic renal insufficiency Renal failure Renal tubular acidosis Chronic neglect Starvation Genetic (unknown defect) Pulmonic Cardiac • • • • • Sex Psychosocial dwarfism • • • • • • • Down syndrome (trisomy 21) Silver .

Social Participation in sports that require weight control (e. among which the author prefers the following : Target height in cm for a girl = [mother’s height in cm + (father’s height in cm . flatulence. Other chronic diseases that may result in short stature include severe asthma associated with chronic steroid use & cystic fibrosis (CF). Cardiac disease : Signs include peripheral edema.1) 2) 3) High velocity < 4cm/yr between 5 . Mid – parental height • Rough estimation of projected adult ht. In particular. early morning nausea. according to one of several formulas. dysgerminoma). measure each parent’s height in the clinic for optimal calculation of the mid . weight & fronto . discomforting. Vomiting. Consider dietary intake & composition. + (mothers ht +13cm)/ 2 . or even audible peristalsis) suggest malabsorption. Neurologic Visual field deficits often herald pituitary neoplasms. or coaches. Renal Polyuria & polydipsia are important symptoms of hypothalamic & pituitary disorders.degree relatives.• • • • • • • o o o o • • document length. or polydipsia is often associated with masses of the CNS. juices & other casual intake. polyuria. crew & gymnastics) may be associated with anorexia nervosa or bulimia induced by the patient. GI Diarrhea. men over report their height & women underreport their weight. Boys = fathers ht.child pair studies of puberty. Chronic renal disease is a common cause of growth failure (GF). Chronic infections : Poor wound healing & opportunistic 152 • o o • o o • o o • o o infections are signs of potential immune deficiency.12 yrs indicates pathological short stature. Most parents can usually recall these 2 milestones. Review of symptoms by organ system provides additional clues to the etiology underlying short stature.birth 1.parental target height.g. which have proven reliable predictors of pubertal timing & tempo in parent . murmurs & cyanosis. Pain or abdominal discomfort suggests inflammatory bowel diseases. Assessing the heights of both parents is absolutely essential. or borborygmi (frequent. Ideally. Physical Clinical evaluation 1) US : LS . Vomiting can suggest an eating disorder or a CNS disorder (e.3 : 1 7yrs 1 : 1 Then LS increases slightly US (1 : 1. Generally. ask about intake of carbonated beverages.occipital circumference at birth. The growth pattern with adequate nutrition in a loving environment over time is critical to distinguish pathologic GF from normal variant short stature in such patients.7 : 1 3yrs 1. wrestling. At a minimum. peers.g. determine the age at onset of menarche for the child’s mother & the age of adult height attainment for the father. Pulmonary Sleep apnea can be a cryptic cause of short stature.13)]/2 Target height in cm for a boy = [(mother’s height in cm + 13) + father’s height in cm)]/2 Document pubertal timing in first .

ray for bone age • • • 5) • o o • • o o • • • o At birth & neonates – X . Documenting growth velocity over time complements the initial height assessment.wrists (carpal bones appearance & their number) Later X .3. Calculate growth velocity as the change in standing height over at least 6 months (in children) or in length over at least 4 months (in infants). determine the fronto . Arm span also reveals a decrement in growth. Ullrich . Newborns typically display a ratio of 1. Inspect mucous membranes for ulcerative stomatitis. a bifid uvula suggests the possibility of a .ray elbows etc. In infants. A single. those with contractures). the lower segment can be determined by 153 o o o • • • • o o • • o o measuring from the superior midline brim of the symphysis pubis to the floor. or crown to pelvis. Carefully examine the midface. Inspect fourth metacarpals.mounted stadiometer (or on the floor with a wall mounted stadiometer). Pretibial ulcerations are also observed in persons with Crohn disease & ulcerative colitis. The upper . Measure standing height in triplicate using a calibrated wall mounted stadiometer. as it is in individuals with hypochondroplasia or achondroplasia. The mean value of the triplicate data serves as the true measurement. arm span provides a reliable alternative for longitudinal assessment of long bone growth. which gradually drops to approximately 1 during prepubertal growth & remains close to 1 in adulthood. SMR – SMR should be assessed using tanners method. Weigh all patients.limb dwarfism usually have an US/LS ratio that remains above 1.occipital circumference. which is otherwise indiscernible in a child with spinal deformation (e.Turner syndrome & Albright hereditary osteodystrophy.g. which can be associated with Hashimoto thyroiditis. as the child sits upright on a platform . In infants. the sitting height can be obtained by measuring the upper body segment. myelomeningocele).• 4) Girls = mothers ht. central.to . genetic composition & chronologic age. Alternatively.g. Similarly. those with spina bifida. Rectal tags & clubbing are also typical in individuals with Crohn disease. initially by gross confrontation. The ratio is more than 1 in children with shortened limbs. maxillary incisor reflects a defect in midline facial development. with the child standing (feet placed together).lower segment ratio (US/LS) should be close to 1.7. In patients in whom short . which are shortened in persons with pseudohypoparathyroidism. Palpate for thyroid enlargement & firmness. Poor linear growth is defined as linear growth velocity more than 2 SDs below the mean for gender. typical of Crohn disease & various trace mineral & vitamin deficiencies. Patients with short . Test visual fields for signs of pituitary & hypothalamic tumors. length is determined in triplicate using a tabletop recumbent infantometer. Both the arm span & US/LS ratio can be informative regarding the cause of short stature. the most common cause of acquired hypothyroidism.limb dwarfism is suspected. + (fathers ht – 13cm)/2 X .ray knees (for epiphysis at lower end of femur & upper of tibia) Up to 8 yrs – X . In children who cannot completely stand or recline (e.

e. skeletal dysplasias. such as the pituitary gland. Other useful tests include the following • • CBC count for hematologic disease Wintrobe sedimentation rate for inflammatory bowel disease . Therefore. Types damaged child. measuring a single random serum GH value is of no use in the evaluation of the short child. a random low serum GH concentration does not confirm the diagnosis of GHD. Disproportionate – US : LS is abnormal for given age.natal onset rickets. formerly named somatomedin C & IGF binding protein . Growth hormone deficiency (GHD) or panhypopituitarism should be considered as a cause of short stature in such patients.95% 85 .I). Measuring serum levels of GH • Beyond the first months of life. which may be palpable. except in pubertal patients & those with history of a brain tumor. These intermittent peaks are greatest after exercise. yet not visible on inspection.I • (IGF . after meals (as blood glucose levels decrease) & during deep sleep.3) These are useful tests for growth hormone deficiency (GHD).90% < 85% Laboratory Studies grade 1st degree 2nd degree 3rd degree > 95% Laboratory studies used to assess the major causes of short stature in children include the following : Measurement of serum levels of insulin like growth factor .3 (IGFBP . – hypothyroidism. are common in patients with major midline facial anomalies. IUGR Chromosomal disorders Dysmorphic disorders Malnutrition GI disorders Celiac disease Chronic diarrhea Cardiac diseases CCF Cyanotic heart diseases Respiratory diseases asthma cystic fibrosis Renal disorders CRF RTA Chronic anaemia haemolytic anaemia Chronic infections chronic TB Endocrinal disorders hypopitutarism. – hypopitutarism.g. Although a random serum GH value of more than 10 mg/dL generally excludes GHD. Waterlow classification of stunting Short stature Normal variant Pathological Familial short stature Constitutional delay in growth Proportionate short stature disproportionate short stature Pre . hypogonadism Psychosocial short stature maternal deprivation.natal Post . brain 154 Height for age normal 90 . e. all skeletal dysplasias. endogenous GH is secreted in a pulsatile fashion. Beyond the neonatal period. Associated anomalies of midline structures. values obtained during the daytime are unlikely to be detectable. DM Cushing syndrome.o o submandibular cleft palate.g. Proportionate dwarf — upper segment : lower segment is normal for given age. hypothyroidism.

Nine inconclusive clinical studies that focused on this particular issue have been published to date.Laron IGFD : 0.out rates from treatment • regimens (presumably due.g.) Serum total thyroxine (total T4) & thyrotropin (TSH) levels to test for hypothyroidism Sweat chloride testing to exclude cystic fibrosis (CF) : Consider this test in patients who are short & have a history of meconium ileus or pulmonary symptoms. Recombinant human growth hormone (rhGH) administration has not been proven to remarkably improve final adult height in children with normal variant short stature. Obtain psychologic or psychiatric consultation for patients with eating disorders. Brain tumors that cause hyposomatotropism may require neurosurgical intervention. hypothyroidism.1U/kg/day At pubertal age administration of sex steroids. Treatment of malnutrition – vitamins. to the parents’ or health care provider’s dissatisfaction with results of therapy in these 155 These agents improve symptoms associated with growth hormone deficiency (GHD). Published studies were flawed because of the following : Selection bias due to high drop . randomization) Inadequate follow . transglutaminase IgG & antigliadin IgG titers for sprue (gluten enteropathy) (Antiendomysial IgA titers are more sensitive & IgG titers are more specific. Activity 1) 2) 3) 4) Treatment of cause – infections. placebo controls.05 mg/kg/d SC hs for prepubertal . Treatment Optimize nutrition in patients with GI disease.up study to final adult height Surgical Care Surgical care depends on the underlying cause of short stature. in part. Serum transferrin & prealbumin concentrations for under nutrition • • individuals) Lack of key design elements for a proper clinical trial (e. M. The most commonly associated anomalies include horseshoe kidney & bicuspid aortic valve. Do not restrict activity in children with normal variant short stature.Weill dyschondrosteosis. double blinding. widely available since 1985.Turner syndrome. haematinics. Chondrodysplasia of the distal radial epiphysis (Madelung deformity) suggests Lerí .• • • • Antiendomysial immunoglobulin A (IgA) & immunoglobulin G (IgG). Perform renal & cardiac ultrasonography in all patients with Ullrich . Somatropin hGH produced via recombinant DNA technology in Escherichia coli. Limb . depending on the tumor type & location (see Hyposomatotropism). deworming In case of GH deficiency – 0. Growth Hormone Medical Care Medical care depends on the etiology of the short stature. Medication Medication administered depends on the etiology of the short stature. Forced energy intake in children with normal variant short stature has not been demonstrated to improve short . D.lengthening procedures have been performed but carry enormous morbidity & mortality risks & are not recommended. Diet Imaging Studies Perform anteroposterior radiography of left hand & wrist to assess bone age.term growth or final adult height. Regimens vary according to product & the indication The following doses of recombinant human somatotropin analogs have proven effective Non .

fatty acids & amino acids into tissues.08 mg/kg SC bid initially with meal or snack.children.05 mg/kg/d SC Mecasermin (0. The infant who fails to breathe spontaneously at birth should be placed under a radiant warmer. Vitamin K injection 156 All equipments must be set up & checked before delivery. < 2 years : Not established > 2 years : 0. chest compressions must be started by an assistant.thick meconium is present in the airway. The infant born with primary apnea is most likely to respond to the stimulation of drying & gentle tapping of the soles of the feet.term treatment of GF in children with severe primary IGFD (primary IGFD defined as basal serum IGF .I is the principal hormone for linear growth & directly mediates GH actions. clean cord clamp. clean razor. intubation & medications are necessary. Epinephrine can be administered via the endotracheal tube. Adequate ventilation is assessed by looking for chest wall excursions & listening for air exchange.04 mg/kg/ dose.12 mg/kg bid Individualize dose & adjust downward if hypoglycemia occurs 2) 3) 4) 46 – 55 56 Neonatal resuscitation Care of baby at time of delivery As soon as the baby is born & is breathing effectively. first from the nose. The base & tip of the cut umbilical cord should be cleaned with spirit or alcohol. Ensure breathing has been established adequately. the baby should be dried & covered in pre .1 mg) is to be given intramuscularly to all babies.feeding should be given within half an hour in babies born vaginally & within 4 hours of a caesarean section. cartilage & organs by stimulating uptake of glucose. dried & positioned to open the airway. 1. Breast . clean cord stump. IGF . may increase by 0.3. If the infant fails to respond to these measures.to .Willi syndrome : 0. surface. 0. The heart rate should be assessed while positive pressure ventilation is being applied. The umbilical cord should be tied by a disposable umbilical clamp with the clamp applied at least 2 to 3 cm away from the base of the cord. The mouth & nares should be suctioned & gentle stimulation should be provided.08 mg/kg/d SC h s Prader . Mouth should be cleaned with sterile gauze or suctioned with suction bulb or mucous trap.warmed clothes & placed in a warmer. 0.04 . 5 & 10 min following delivery. if tolerated after 1 wk.1 (rhIGF .I level & height SD scores < .1) indicated for long . Primary IGFD is characterized by absent IGF I production despite normal or elevated GH release. The eyes should be cleaned with sterile saline (use fresh cotton swab for each eye) & if required eye drops can be installed to prevent conjunctivitis. Routine delivery room care of the newborn 1) 2) 3) 4) .0. not to exceed 0. Delivery room care 1) Recombinant human insulin like growth factor . The infant who fails to respond rapidly to these measures is experiencing secondary apnea & requires positive pressure bag ventilation with oxygen.2 mg/kg/d SC for pubertal males TS or chronic renal insufficiency : 0. Prolonged or overly vigorous suctioning may lead to bradycardia & should be avoided unless moderate . If the heart rate does not increase rapidly after ventilation. Apgar scores are used to assess the status of the infant at 0. The mouth should be suctioned first to prevent aspiration into the lungs. IGF .1 mg/kg/d SC for pubertal females.I is essential for normal growth of children’s bones. normal or elevated serum GH level). Head low position Cleaning – clean hands.

. Ocular prophylaxis • • core. kidney & nape of the neck. Umbilical cord care Umbilical cord should be inspected for number of arteries & veins. neck. Skin care & cord care Bath should be given with lukewarm water & undedicated soap.0. Blood flowing from brown fat becomes warm & with • circulation transfers heat to other parts of body. Umbilical cord should be clamped preferably with a cord clamp. Maintenance of body temperature Newborn babies are prone to getting low body temperature & hence bath is usually given after a day or 2 after delivery to prevent hypothermia. Brown fat is site for heat production. interscapular & axillary regions.5 – 37 c Normal temp. It is located around adrenal • glands. The quantity of brown fat is directly related to weight of baby. Stomach wash with NS is indicated in following conditions Babies born by LSCS. – cloths. Ethyl Alcohol should be applied at the tip & around the base of the umbilical stump everyday to prevent colonization. The umbilical cord should be checked after 2 to 4 hours of clamping. then consult your doctor. The cord should be kept dry.1 ml intramuscular in the anterolateral aspect of the thigh. armpits & diaper area should be cleaned properly. Infants body heat falls by app. Brown fat is important source of heat production in newborns. Exposure of the baby to direct sunlight during hot summer months should be avoided.g. The room in which the baby is to be kept should be warm & baby should be effectively covered with caps & socks to prevent hypothermia.5 to 36 c Cold stress Moderate hypothermia → < 36 to 32 c → < 32 c Severe hypothermia LBW baby has decreased thermal insulation due to reduced • subcutaneous fat. Vitamin K injection is given in the dose of 1 mg =0. → 36. This is known as non shivering thermogenesis. Erythromycin ophthalmic ointment should be installed for prevention against gonorrheal & chlamydial infection & is administered after birth. of skin & 0. Severely asphyxiated babies Polyhydramnios Meconium stained liquor SFD babies Single umbilical artery Maintenance of body temperature – Body temperature in newborn is unstable Body surface area is 3 times more than adult. Oil massage provides insulation against heat loss & is useful in maintaining body temperature. Any discharge or redness from the cord may suggest infection & a doctor should be consulted.• • • • • • 5) Mucus trap is called De Lee catheter.1 c/min of Hence newborns are more prone to develop hypothermia. Dip baths should be avoided till the cord has fallen. The cord usually falls off in 5 to 10 days. Scalp.3 c/min. If the cord has not fallen off even after 14 days. → < 36. Ways of heat loss in newborns – 1) Evaporation – Evaporation of amniotic fluid from skin 2) Conduction – Contact with cold objects e. • 157 Clean eyes with saline water & cotton immediately after the birth of the baby. trays 3) Convection – hot air surrounding the baby is replaced by cold air 4) Radiation – heat is radiated from the body of the baby Vitamin K is given to the infant by intramuscular injection to prevent hemorrhagic disease of the newborn. Meconium stained cord is one of the sign of (MAS) meconium aspiration syndrome.

Local application of triple dye or bacitracin ointment should be applied.40 30 to 40 70/45 70 . or a chromosomal abnormality. Auscultation of breath & heart sounds.gestational age infant is at risk for shoulder dystocia. 500 g.gestational age infant is at greater risk for cold stress. birth trauma & hypoglycemia. The large . A low . Pallor. The small . cyanosis. Height. The normal heart rate 158 Examination of organ systems & regions . Factors that may result in an infant who is small for gestational age include chromosomal & other dysmorphic syndromes.50 3) 4) Chest wall excursions should be observed & the respiratory rate b) determined. maternal hypertension & smoking.for . 2) Premature infants If the mother is hepatitis B surface antigen . hypocalcemia & polycythemia.The gestational age of the newborn BCG vaccine should be given to the newborn before discharge from the hospital along with zero dose of OPV. uterine anomalies & multiple gestations. Assessment of the adequacy of fetal growth 1) Gestational age assessment . Preterm infants may develop respiratory distress syndrome. congenital infections. Measurements & growth charts 1) The normal temperature of the newborn is 36. head circumference. The normal neonatal respiratory rate is 40 to 60 breaths per minute. a congenital anomaly of the larynx.for . weight.positive (HBsAg). Vaccination during the first week of life may range from 94 to 175 beats per minute.glass appearance. an elevated oxygen requirement & a roentgenographic picture of poor inflation & a fine homogeneous ground . Height. The differential diagnosis for the large .birth weight infant is defined as any neonate with a birth weight < 2.0 degrees vital sign/age Temp. Look for any generalized edema which may be due to prematurity. the infant should be given an IM injection of hepatitis B immune globulin & a course of three injections of hepatitis B vaccine (before hospital discharge & at 1 & 6 months of age). retractions. Pulse Respiratory rate Blood pressure 2) newborn 1mth to 1 year a) 36. hypothyroidism. An unusual cry may indicate sepsis. weight & head circumference should be plotted as a function of gestational age on an intrauterine growth chart.for . bradycardia & retinopathy of prematurity.5 to 37. hydrops fetalis Following observations should be assessed – Temperature. apnea. Respiratory distress syndrome is recognized by tachypnea. or if she has active hepatitis B. Hepatitis B prophylaxis infant is assessed with the help of Ballard score of neuromuscular & physical maturity. Respiratory distress.75 / 45 .5 to 130 to 37 0C 150 110 to 130 appro. Vital signs A preterm infant is defined as an infant of less than 37 weeks’ gestation & a post term infant is defined as being of greater than 42 weeks’ gestation. hypoglycemia. Physical examination of the newborn General examination a) b) The examiner should assess whether the infant appears to be sick or well. chest circumference & mid arm circumference should be measured. hypoprotenemia.gestational age infant includes maternal diabetes & maternal obesity. grunting.

d) The lips. perforation. foot deformities.10 days. cystic hygroma & sternocleidomastoid tumor. In case of male babies examine the genitalias for undescended testis. b) Dysmorphic facial features that suggest a chromosomal anomaly include mid facial hypoplasia. Generally liver is 2 cm palpable below the right coastal margin. meconium ileus. or glaucoma. face & neck The head circumference is measured & plotted & the scalp.e. kernicterus). Nares patency can be documented by closing the mouth & occluding one nostril at a time while observing air flow through the opposite nostril. The absence of a clear red reflex is indicative of a retinoblastoma. infants who are small for gestational age.to .2 . which requires immediate endocrinologic & urologic consultation. Down syndrome & breech presentation. congenital 159 About 60 to 70 percent of newborn babies develop jaundice on 2nd or 3rd day of life which disappears within 7 . infants of diabetic mothers & infants who have experienced perinatal asphyxia. b) fontanelles & sutures are examined. Hypoglycemia is defined as a blood glucose of < 40 . Initial workup for neonatal hyperbilirubinemia includes measurements of total & direct bilirubin levels. High levels of bilirubin can cause an acute encephalopathy (i. Bruising & hematomas of the scalp should be noted. Fracture of the clavicle occurs in 0. Epstein pearls are present on the hard palate 2) Abdomen & gastrointestinal system torticollis. Abdominal palpation for masses. mouth & palate are inspected & palpated for clefts. or renal masses is completed & the anus should be visually inspected. caput succedaneum. 3) Genitourinary system C) 1) 2) The genitalia are examined for ambiguous genitalia.5% of vaginal deliveries. retractile testis. Jaundice congenital dislocation of the hips (CDH). Hypoglycemic infants require early feedings or IV glucose.maternal hemorrhage. Physical findings include local swelling & crepitations & an asymmetric Moro reflex. The female to male ratio is 7 :1. overriding of the parietal bones over the frontal & occipital bones may be present. Anemia during the newborn period may be caused by hemolytic & congenital anemias. Risk factors for hip dysplasia include a family history. Coombs test & testing of urine for reducing substances to exclude galactosemia. hepatosplenomegaly. fetal . small eyes.45 mg/ dL. This is a normal physiological process & is known as physiological jaundice & no treatment is required.1) a) Head. 4) a) Musculoskeletal system Hip examination may detect developmental dysplasia & Hyperbilirubinemia occurs frequently in the normal newborn because of increased production & decreased elimination of this breakdown product of heme. If the jaundice starts within first 24 hours of life or is very deep or it persists beyond 2 weeks of life. hematocrit. Moulding i. or low . Treatment consists of making a sling by pinning the shirt sleeve of the involved side to the opposite side of the shirt. then other . Distended abdomen may be observed in obstruction.e. placental abruption & occult hemorrhage.3. Bilirubin metabolism Visual inspection of the abdomen should be done to assess symmetry & distension. Cephalohematoma. c) The eyes should be examined with an ophthalmoscope to document a red reflex. vacuum caput should be looked for. cataract. Hypoglycemia Common neonatal problems A) 1) 2) B) Hypoglycemia is common in premature infants. Ultrasonography is used to evaluate suspected hip dysplasia.set ears. Neck should be examined to rule out goiter.

This can be a normal pattern. When the baby is being breastfed & is not receiving any other top feeding. Hence burping the baby by holding upright on the shoulder for 5 to 10 minutes after the feed relieves the gas & regurgitation. nose block & some trivial trauma may be reasons for cry. An infant with colic may have gaseous distension of abdomen & usually feels comfortable when placed in prone position which facilitates expulsion of gas.pitched cry may suggest infection & should be referred to the doctor immediately. It is a normal phenomenon. a urinary tract problem should be ruled out. Most babies pass 4 to 8 stools in a day (some babies pass stools after each feed) whereas some babies pass stools once in 2 . Feeding problems Regurgitation of milk During the summer months. Urinary problems Most newborns pass urine within 48 hours of birth. it is unlikely to develop diarrhea due to infection.term newborns pass a meconium stool before 24 hours of age. a bladder or kidney problem should be ruled out.nine percent of normal full . most babies keep their eyes closed & go to sleep after taking only a few sucks at the breast. meconium ileus (cystic fibrosis). the baby passes black. The child is however active & keen to feed. A baby swallows air while sucking (aerophagy) & as air is burped out part of the milk also comes out along with the air. Excessive crying in newborns Most newborns bring up some amount of milk soon after feeding. the baby is active & playful. Bilious vomiting in the newborn is always abnormal & usually is caused by an intestinal obstruction. A delayed or absent passage of meconium may be caused by meconium plug syndrome. Ensuring proper feeds & hydration leads to disappearance of the fever.3 days. Babies usually cry on passing urine due to wet nappies. Sleep variation During the first two to three days. or imperforate anus. If urination has not occurred within 24 hours. This is probably because in the mother’s womb. This is known as Dehydration fever & is a transient condition & due to excessive environment temperature.term infants will urinate by 24 hours.6 weeks. or there is excessive straining on passing urine or there is dribbling at the end. some babies may develop transitory fever during the second or third day of life. If the baby has not passed urine within 48 hours. Also most newborns sleep during the day & are awake & playful during the night. Vomiting in the newborn also may be caused by inborn errors of metabolism & congenital adrenal hyperplasia. Presence of inconsolable crying or a high .causes of neonatal jaundice should be promptly investigated D) Gastrointestinal problems Urine output 1) 2) Ninety . Hirschsprung disease. A normal baby passes about 5 . . If the urine stream in narrow. Fever E) Ninety . Sometimes insect bites. renal ultrasonography should be done & an intravenous fluid challenge may be given. Babies tend to fall into a set sleep pattern in 4 .six percent of full . tarry stools (meconium) which are followed by greenish stools (transitional stools) for next 1 or 2 days & then regular semisolid sticky golden yellow stools. during the daytime.10 times urine per day. the baby is rocked in the amniotic water & sleeps & when the mother is resting at night. Stools Most babies cry when they are either hungry or having discomfort such as wet nappies or soiled stools. 160 During first few days of life.

The less saturated blood from the right ventricle passes into the pulmonary arteries. thus raising left atrial pressure. it mixes with blood returning from the coronary sinus & superior vena cava & flows into the right ventricle. (Its oxygen requirement is fulfilled through mother’s blood via the placenta). The more highly oxygenated blood that crosses the foramen ovale mixes with the small amount of pulmonary venous return & then crosses the mitral valve into the left ventricle. which has bypassed the liver. Blood flow in the umbilical vein divides at the porta hepatis. creating the standard circulation. head & upper torso. Since small amount of blood comes back from the lungs. links the right atrium to the left. Ductus venosus is the continuation of umbilical vein which 4) 5) connects directly to the inferior vena cava thus most of the blood bypasses the liver which is non functional in the foetus. streams into the inferior vena cava to pass preferentially through the patent foramen ovale into the left atrium. Ductus arteriosus connects the pulmonary artery to the descending aorta thus shunting the blood in systemic circulation as foetal lungs are nonfunctional. pressure in the left atrium is low & is high in the right atrium. The output from the left ventricle passes into the ascending aorta to the heart. In utero Because the lungs are inactive in the foetus the heart does not pump much blood towards it. ductus venosus & umbilical arteries are collapsed. which causes the constriction of the ductus arteriosus. This causes the flap over foramen ovale to shut down & prevent the blood flow between atria. with the remainder entering the umbilical artery where it is returned to the placenta for reoxygenation. Within minutes of delivery. In the right atrium. For this reason the foramen ovale. the ductus arteriosis closes up too. abdomen & lower extremities. Pulmonary blood flow & then passes into the inferior vena cava. the lungs inflate which increases the pulmonary blood flow. Because the pulmonary vessels are constricted & highly resistant to flow. This creates the same heart circulation as is seen in adults. 2) 3) 1 umbilical vein carries oxygenated blood from placenta to the foetus.57 Modifications present in fetal circulation 1) 2 umbilical arteries carry deoxygenated blood from the foetus to the placenta. This portal blood flow perfuses the liver 161 As soon as the baby takes its first breaths. Over the next few hours/days. The remainder of the blood takes the path of least resistance through the patent ductus arteriosus into the descending aorta. Foramen ovale is a valve like opening which allows the blood flow from right to left atrium thus allowing very small amount of blood to flow towards the fetal lungs. The desaturated blood returning from the liver & lower body streams into the inferior vena cava to the right atrium. with 50 . This results in more blood coming back from the lungs. . allowing blood to pump through from right to left. Fetal pulmonary vascular resistance (PVR) is high & the fetal systemic vascular resistance (SVR) is low. Changes after birth The umbilical vein carries the oxygenated blood from the placenta to the fetus. Approximately one third of this blood is carried to the trunk. This high pressure keeps the valve adherent so that it can cover the open hole. Cutting of the cord stops the placental circulation & thus the umbilical vein. The more highly oxygenated blood. brain. the newborn’s pulmonary vascular resistance decreases. only about 12% of this blood enters the lungs.60% of the blood passing directly to the inferior vena cava via the ductus venosus & the remainder of the blood passing into the portal circulation. Relatively little mixing of the blood occurs in the inferior vena cava from these 2 sites. As the pulmonary circulation starts the blood oxygen levels are increased.

pink with abundant lanugo & little vernix caseosa. There is also high possibility of congestive heart failure if the patent duct is not closed surgically. This is more serious however. the ductus arteriosis can fail to close. or Tetralogy of Fallot. Subcutaneous fat is deficient Breast nodule is not palpable In males scrotum is pinkish with less rugae. growth & development as a child. Whereas the usual definition of preterm birth is birth before 37 weeks gestation.to . a significant overlap exists between preterm birth & prematurity : generally. Head circumference is usually less than 33cm Fontanels are large & sutures are widely separated. preterm babies are premature & term babies are mature. Face – face is small Ears – cartilage is soft & the ear recoil is poor Scalp hairs are wooly Skin is thin.When it goes wrong Again. LBW 162 Signs & symptoms Anthropometry Classification • • • • • • • • • • • • Length is less than 47 cm. since the higher pressure in the aorta than the pulmonary artery means that there is a left . Preterm birth is by far the most common cause of prematurity & is the major cause of neonatal mortality in developed countries. The lungs are one of the last organs to develop in the womb. Genitalia . • • 58 • • Preterm birth refers to the birth of a baby with less than 37 • weeks from the last day of the menstrual period. Prematurity can be reduced to a small extent by using drugs to accelerate maturation of the fetus & to a greater extent by preventing preterm birth. premature babies typically spend the first days/weeks of their life on a ventilator. In some congenital conditions such as Transposition of the Great Arteries. commonly used as a synonym for preterm birth. the baby suddenly has great difficulties. including disabilities & obstruction in growth & mental development. • • refers to the birth of a baby before its organs mature enough to allow normal postnatal survival.right shunt & the child can suffer from labored breathing & failure to thrive. because of this. a premature infant is one that has not yet reached the level of fetal development that generally allows life outside the womb. several organ systems mature between 34 & 37 weeks & the fetus reaches adequate maturity by the end of this period. Premature birth. the abnormalities are such that if the normal closure of these ducts takes place. One of the main organs greatly affected by premature birth is the lungs. In these cases it is referred to as a duct dependant circulation. In females labia minora is not covered by libia majora. Testes may be absent in the scrotum. Preterm IUGR Symmetrical Asymmetrical Mixed In the normal human fetus. if the ducts close. the blood no longer circulates to all parts of the body adequately. Therefore. Premature infants are at greater risk for short & long term complications. or dies.

Respiratory problems are common. even months. Obstetric abnormalities like incompetent cervix. thyroid disease & heart disease. Marital status is associated with risk for preterm birth. hypocalcaemia. short cervical length. Anxiety & depression have been linked to preterm birth. Babies with birth defects are at higher risk of being born preterm.Specific risks for the preterm neonate however. The frequency of infection in preterm birth is inversely related Infection • . • metabolic acidosis. and 4) Intrauterine inflammation/infection. an association does not establish causality. such conditions include high blood pressure.eclampsia. hypoxic • ischemic encephalopathy (HIE). chronic lung disease BPD (bronchopulmonary dysplasia)congenital pneumonia. specifically the respiratory • distress syndrome (RDS) or HMD (hyaline membrane disease). Multiple pregnancies (twins. Activation of one or more of these pathways may happen gradually over weeks. excessive alcohol during pregnancy also increases the chance of preterm delivery. too much (polyhydramnios) or too little (oligohydramnios) are also at risk. 2) Uterine over distension. Metabolic problems arise from hypoglycemia. pre . asthma. Genetic make . As a result they are at risk for numerous medical problems affecting different organ systems. Abnormal amounts of amniotic fluid. 3) Decidual bleeding. retinopathy of prematurity (ROP). pulmonary haemorrhage. bicornuate uterus the capacity of the uterus to hold the growing pregnancy may be limited & preterm labor ensues. Maternal medical conditions increase the risk of preterm birth. Infection. Cardiovascular complications may arise from the failure of the • ductus arteriosus to close after birth : patent ductus arteriosus (PDA). pneumonia & urinary tract infection • Causes • • • • • • • • • • • • • Labor is a complex process involving many factors. Hematologic complications include anemia of prematurity. apnoea. it is associated with diabetes & hypertension which are risk factors by themselves Women with a previous preterm birth are at higher risk for a recurrence at a rate of 15–50%. maternal diabetes. • thrombocytopenia. uterine malformations like tumors. including sepsis. Four different pathways have been identified that can result in preterm birth & have considerable evidence : 1) Precocious fetal endocrine activation. cerebral palsy & intraventricular hemorrhage. Neurological problems include apnea of prematurity. Maternal background Preterm infants usually show physical signs of prematurity in reverse proportion to the gestational age. triplets.the use of tobacco.up is a factor that can cause preterm birth. however. The mental status of the women is of significance. developmental disability. Gastrointestinal poor gastric motility & necrotizing enterocolitis • (NEC). From a practical point a number of factors have been identified that are associated with preterm birth. etc. placenta previa. Addiction .) The use of fertility medication that stimulates the ovary has been implicated as an important factor in preterm birth. vitamin K deficiency & hyperbilirubinemia (jaundice) that can lead to kernicterus. 163 • • • (BOH) bad obstetric history Low socio – economic status Maternal age under 16 & above 35 years Women with a low BMI & poor nutritional status Obesity does not directly lead to preterm birth. cocaine.

Post term In developed countries premature infants are usually cared for in a neonatal intensive care unit (NICU). Phototherapy may be used to treat newborn jaundice (hyperbilirubinemia). then the baby is at the risk of developing meconium aspiration syndrome. as they do not produce their own surfactant. Typical glucocorticoids that are used for the above purpose are betamethasone or dexamethasone. post maturity. infants may be underweight for other reasons than a preterm delivery.dates pregnancy all refer to post mature birth. which supplies the baby with nutrients & oxygen from the mother. Almost all neonates in the latter two group are born preterm. . Post . namely intraventricular haemorrhage. simple measures such as (KMC) kangaroo mother care (skin to skin warming). In the NICU. oxygenation & brain activity. Treatments may include fluids & nutrition through intravenous catheters. As survival has improved. 1) Low birth weight (LBW) have a birth weight of less than 2500 g (5 lb 8 oz) & are mostly but not exclusively preterm babies as they also include small for gestational age (SGA) babies. respiration. in the neonate. For this reason pregnant mothers with threatened premature delivery prior to 34 weeks are often administered at least one course of glucocorticoids. Weight generally correlates to gestational age. Neonatal care Most children even if born very preterm adjust very well during childhood & adolescence. encouraging breastfeeding & basic infection control measures can significantly reduce preterm morbidity & mortality. however. The pediatricians who are specialized in the care of very sick or premature babies are known as neonatologists. If the fetus passes fecal matter. mental retardation. a second “rescue” course of steroids can be administered 12 to 24 hours before the anticipated birth. however. cardiac function.mature births do not have any harmful effects on the mother. Prevention Glucocorticosteroids developing countries where advanced equipment & even electricity may not be available or reliable. The risks of medical & social disabilities extend into adulthood & are higher with decreasing gestational age at birth & include cerebral palsy. disorders of psychological development. necrotising enterocolitis & patent ductus arteriosus. often when the fetus has reached viability at 23 weeks. prolonged pregnancy & post . can begin to suffer from malnutrition. This directly leads to respiratory distress syndrome. the focus of interventions directed at the newborn has shifted to reduce long term disabilities. Modern neonatal intensive care involves sophisticated measurement of temperature. starts aging & will eventually fail. mechanical ventilation support & medications. Prognosis Severely premature infants may have underdeveloped lungs. oxygen supplementation. other neonatal complications are reduced by the use of glucocorticosteroids. particularly those related to brain injury.term. The steroid crosses the placental barrier & stimulates the production of surfactant in the lungs of the fetus. also called hyaline membrane disease. Besides reducing respiratory distress. Post . After the 42nd week of gestation. disabilities of vision & hearing & epilepsy. which are bassinets (?????) enclosed in plastic with climate control equipment designed to keep them warm & limit their exposure to germs. A number of maternal bacterial infections are associated with preterm birth including pyelonephritis. premature babies are kept under radiant warmers or in incubators (also called isolettes). the placenta. In 164 Postmaturity is when a baby has not yet been born after 42 weeks of gestation. behavior & emotion. but the fetus. asymptomatic bacteriuria. 2) Very Low Birth Weight (VLBW) which is less than 1500 g & 3) Extremely Low Birth Weight (ELBW) which is less than 1000 g. which is not typical until after birth & the child breathes it in. pneumonia & appendicitis.• to the gestational age. In cases where premature birth is unavoidable.

becomes an increased risk. contractions occur more frequently than a natural occurring birth. But post . thus increasing the length of labor. This limits the blood flow through the placenta & ultimately leads to placental insufficiency & the baby is no longer properly nourished. green. Pitocin . Many women report a feeling similar to urination. creases on the baby’s palms & soles of their feet.mature birth. Breaking the water . pulsation of umbilical cord or definite movements of voluntary muscles irrespective of the attachment of placenta to the cord. – Baby born with birth weight less than 1500 gm (up to 1499 gm) . Difficulty in delivering the shoulders.mature babies are larger than average forceps or vacuum delivery may be used to resolve the difficulties at the delivery time. Pitocin doesn’t have the natural effects of stress relief that the naturally occurring hormone. is used.When the synthetic hormone. a lot of hair on their head & either a brown. 3) 4) 5) the duration of the pregnancy & which is not expelled or extracted from the mother. they are also more intense. Post .Having one’s water broken feels like a slight tug & then a warm flow of liquid. Fetal death – Death prior to complete expulsion or extraction from its mother of a product of conception irrespective of duration of pregnancy & death indicated by absence of any signs of life. Birth weight – The first weight recorded of a live or still bon baby preferably taken within first hour of life. What it feels like Stripping the membranes . This is called cephalopelvic disproportion. Complications 2) • • • • Towards the end of pregnancy calcium is deposited on the walls of blood vessels & proteins are deposited on the surface of the placenta. Still birth – A product of conception that after expulsion or separation from mother does not show any evidence of life at gestational age of 20 wks or more or weighing more than 500 gm.term birth are unknown. The labor is increased because the baby’s head is too big to pass through the mother’s pelvis.Causes • • • • The causes of post . or yellow discoloration of their skin. overgrown nails. Post . When there is a miscalculation. (up to 2499 gm) irrespective of gestational period. shoulder dystocia. which changes the placenta. 59 Definitions 1) Fetus – It is defined as a product of conception.Stripping the membranes only takes • Birth weight groups LBW – Baby born with birth weight less than 2500 gm VLBW 165 a few minutes & causes a few intense cramps. does. irrespective of Symptoms Different babies will show different symptoms of post maturity. minimal fat. pitocin. irrespective of the duration of the pregnancy & which after separation.term babies may be larger than an average baby. oxytocin. Due dates are easily miscalculated when the mother is unsure of her last menstrual period. Live birth – It is defined as complete expulsion or extraction from the mother of a product of conception. the baby could be delivered before or after the expected due date.mature births are more likely when the mother has experienced a previous post . breathes or shows any other evidence of life such as beating of heart. When post .mature births can also be attributed to irregular menstrual cycles. The most commons symptoms are dry skin.

Following birth. Fetal pulmonary vascular resistance (PVR) is high & the fetal systemic vascular resistance (SVR) is low. Post term – Baby born with a gestational age of 42 wks (294 or more) irrespective of birth weight.ELBW – Baby born with birth weight less than 1000 gm • (up to 999 gm) Gestational age It is calculated from the first day of LMP till the date of birth & is always expressed in completed weeks. pulmonary blood flow must increase & spontaneous respiration must be established. the placenta has a multi villous circulation that allows for maximum surface area for the exchange of oxygen & carbon dioxide between the mother & fetus. First. Two major characteristics of placental circulation enable the placenta to maintain adequate oxygenation of the fetus. distended by lung fluid secreted by the pulmonary epithelium. the pulmonary capillary bed & pulmonary blood vessels remain constricted. Term – Baby born with a gestational age between 37 – 41 wks (259 – 293 days) irrespective of birth weight. This lung fluid maintains lung volume at about functional residual capacity & determines normal lung growth. it requires excellent assessment skills & a thorough understanding of fetal & neonatal physiology. At birth. most of the blood flow is shunted away from the lungs & directed to the placenta where fetoplacental gas exchange occurs. as well as other changes to independent organ system functions. or cyanosis & hypoxia rapidly develop. • In utero. the lungs must transition (change) rapidly to become the site for gas exchange. which results in increased transfer of oxygen from the maternal Fetal pulmonary physiology 60 Neonatal resuscitation • • Resuscitation (rise again) involves knowing much more than an ordered list of skills & having a resuscitation team. alveoli & terminal saccules are open & stable at normal fetal lung volumes. The fetal lung is filled with approximately 20 mL fluid at term. the newborn’s pulmonary vascular resistance decreases. causing a corresponding increase in neonatal pulmonary blood flow. Within minutes of delivery. for the lungs to operate as a functional respiratory unit providing adequate gas exchange. A thorough understanding of normal transitional physiology leads to a better understanding of the needs of the infant who is experiencing difficulties and. several factors result in the lowering of maternal pH & increasing of fetal pH. 166 • • • Respiratory adaptation • . the airways & the alveoli must be cleared of fetal lung fluid. should result in a more effective resuscitative effort. The placenta provides the respiratory function for the fetus. Fetal airways. A constant flow of this fluid is secreted into the alveolar spaces throughout development. therefore. The physiology of transaction • • • Neonatal transition requires spontaneous breathing & successful cardiopulmonary changes. Gestational age groups— 1) Pre term – Baby born with a gestational age of less than 37 2) 3) • wks (< 259 days) irrespective of birth weight. Second. which contributes to the fetal amniotic fluid. Pulmonary & bronchial circulation also develops as the alveoli appear. High vascular resistance & low pulmonary blood flow results. Because of the compressive effect of the fetal lung fluid & the low partial pressure alveolar oxygen (paO2) in utero.

With subsequent lung aeration.releasing hormone. located at the basilar layer of the pulmonary epithelial cells.90% of FRC is established within the first hour .• • • • • to the fetal hemoglobin or RBCs.dissociation curve to the left allows the fetal hemoglobin to bind more oxygen. The first breath must overcome the viscosity of the lung fluid & the intra . which results in a lower affinity of the hemoglobin for oxygen & the release of additional oxygen to the fetal hemoglobin. the fetus is not able to regulate this response until approximately 24 weeks of gestation. These findings could account for the increased incidence of transient tachypnea of the newborn following birth by cesarean delivery without labor. the intraparenchymal structures stretch & gasses enter the alveoli. In addition. Thoracic recoil allows for passive inspiration of air into the larger bronchioles.alveolar surface tension. The increased paO 2 & pH result in pulmonary vasodilatation & constriction of the ductus arteriosus. Lung expansion & aeration is also a stimulus for surfactant release with the resultant establishment of an air .33% of the fluid may be expressed from the oropharynx & upper airways. Active sodium transport by energy . During the thoracic squeeze. After birth. Fetal “breathing. Exposure to an air interface along with high concentrations of glucocorticoids & cyclic nucleotides reverses the direction of ion & water movement in the alveoli leading to highly selective sodium channels. active ion transport. Only a small portion of this fetal lung fluid is removed physically during delivery. drive liquid from the lung lumen into the pulmonary interstitium where it is absorbed by the pulmonary circulation & lymphatics. Maternal blood. with the onset of labor. The corresponding shift in the fetal oxygen . These areas sense both pH & partial pressure of carbon dioxide (pCO2). begins at approximately 11 weeks’ gestation & increases in strength & frequency throughout gestation. 80 . the fetus produces adrenaline & the mother produces thyrotropin . lung fluid is removed by several mechanisms. which stimulates the pulmonary epithelial cells to begin reabsorption of fluid.fluid interface & development of functional residual capacity (FRC). Fetal “breathing” is controlled by chemoreceptor located in the aorta & at the bifurcation of the common carotid. passive movement from Starling forces & lymphatic drainage. This changes the fetal lung epithelial cells from a pattern of chloride secretion to one of sodium reabsorption accelerates reabsorption of fetal lung fluid. Normally. however. resulting in increased paO2 & pH. releases oxygen to the fetal circulation & accepts both carbon dioxide & various byproducts of metabolism from the fetal circulation. Effective transition requires that any remaining liquid be quickly absorbed to allow effective gas exchange. carrying oxygen on adult hemoglobin. These transfers result in a decrease in the maternal placental blood pH & a corresponding shift of the maternal oxygen dissociation curve to the right. the fetal airways & alveoli are filled with lung fluid that needs to be removed before respiration. including evaporation. 25 .requiring sodium transporters. although this amount may be markedly less. A reflex response to altered pH & pCO 2 is present at approximately 18 weeks’ gestation.” or chest wall & diaphragmatic movement. As discussed above. This first breath must also generate high transpulmonary pressure. which helps drive the alveoli fluid across the alveolar epithelium. Labor is also associated with an increase in catecholamine levels 167 • • • • • Neonatal pulmonary physiology • • • • • • that stimulate lymphatic drainage of the lung fluid.

therefore. the neonate may demonstrate no response to the inflation attempt. an initial increase in ventilation occurs. Clamping of the cord removes the low resistance placenta. Certain evidence also suggests that the increased arterial paO2 following the initial breaths may be responsible for the development of continuous breathing via hormonal or chemical mediators that are still undefined. The carotid bodies & peripheral chemoreceptor located at the bifurcation of the common carotids are stimulated during hypoxia to increase minute ventilation.g.” in which the neonate responds to positive pressure lung inflation with a positive intraesophageal pressure to resist the inflation. When the newborn lungs fill with air. the paO2 should rise gradually. such as prostacyclin. but also may cause high transient inflation pressures. Soon after birth. Cardiovascular Adaptation Fetal circulation . tactile & thermal changes. This response acts to not only reduce lung inflation. The second response is “Head’s paradoxical response” in which the neonate responds to positive pressure lung inflation with an inspiratory effort. That is to say. The pulmonary vascularity is stimulated to dilate by chemical mediators. lungs & airways the infant must generate a negative pressure so that air moves from an area of higher pressure to one of lower pressure. In term infants with a persistent hypoxia. Stimuli for the first breath may be multifactorial. This effect is even more profound in premature infants whose CNS is not as mature. because of extreme prematurity or sedation). with the resultant negative. The first response is the “rejection response.• • • • • • • of birth in the term neonate with spontaneous respirations. increased noise & light) activate a number of sensory receptors that may help initiate & maintain breathing. Prostacyclin acts on the pulmonary vascular smooth muscle bed to induce pulmonary vasodilatation.g. Two major physiologic responses have been described for the initial lung inflation in the neonate. despite constant delivery of inflation pressure. This inspiratory effort. These physiologic responses to positive pressure inflation in the delivery room may cause large variability in the tidal volume & intrapulmonary pressures. To overcome the viscosity & resistance of fluid filled lungs & recoil & resistance of the chest wall. In asphyxiated infants who cannot increase minute ventilation (e. Prostacyclin has a short half . not generating any change in intraesophageal pressure during the positive pressure inflation & passive inflation subsequently results. fetal respiratory activity must transition to normal spontaneous breathing. Premature & critically ill infants with surfactant deficiency or dysfunction may have limited ability to clear lung fluid & establish a functional residual capacity. causing a negative intraesophageal pressure.life in the bloodstream and. causing an increase in systemic vascular resistance & consequently causing an increase in both systemic blood pressure & pulmonary blood flow. does not affect the systemic circulation. 168 • • • • • • • • Of course. The environmental changes that occur with birth (e. profound bradycardia may result. nitric oxide & prostaglandins. pressure produces a fall in inflation pressures but results in a transient increase in tidal volume. followed by a decrease in ventilation occurs. the infant actively resists attempts to inflate the lungs by generating an active exhalation. The formation of certain prostaglandins. is induced by the presence of increased oxygen tension. Nitric oxide is released when pulmonary blood flow & oxygenation increases.

With the decrease in right atrial pressure & the increase in left atrial pressure. with increased flow to the heart. Clamping of the umbilical cord removes the low resistance placental vascular circuit & causes a resultant increase total systemic vascular resistance with a resultant increase in left ventricular & aortic pressures. brain & adrenal gland & decreased flow to the rest of the body. Primary & secondary apnea cannot be clinically distinguished. Infants who are undergoing asphyxia have an altered respiratory pattern. Therefore. However.left shunting to left . this causes a decrease in blood pressure & tissue perfusion. Permanent closure of the ductus venosus may be delayed in preterm infants or infants with persistent pulmonary hypertension. combined with the decreased pulmonary vascular resistance. During primary apnea. Infants who experience secondary apnea do not respond to tactile or noxious stimulation & require positive . the myocardium fails & bradycardia occurs. reverses the shunt through the ductus arteriosus (from right . Hypoxia & acidosis leads to pulmonary arteriolar vasoconstriction.pressure ventilation (PPV) to restore ventilation.valve” foramen ovale is pushed closed against the atrial septum. The contraction of this tissue is dependent on 169 both the increase in arterial oxygen related to the onset of spontaneous respirations & a fall in circulating prostaglandin E2 (PGE2). Systemic cardiac output is redistributed.Neonatal circulation • • • • • • • The aeration of the lung results in an increase in arterial oxygenation & pH. PPV should be instituted as outlined in the Neonatal .right shunting) until the ductus completely closes. The decrease in pulmonary vascular resistance leads to an increase in blood flow to the lungs & in pulmonary venous return. the infant responds to stimulation with reinstitution of breathing. which terminates umbilical venous return.to . Early in the course of asphyxia. which slowly decrease in frequency & eventually cease (termed secondary apnea).to . if the asphyxia continues. with ongoing hypoxia & acidosis. These respiratory efforts eventually cease with continued asphyxia (termed primary apnea). The constriction & closure of the ductus arteriosus is accomplished by contractile tissue within the walls of this blood vessel.way “flap . A corresponding decrease in right ventricular & pulmonary artery pressures is also noted. Pulmonary vascular resistance increases. Anatomical closure subsequently occurs at approximately 1 2 weeks. Initially. Response to Asphyxia • • • • • • • • The fetus or newborn that is subjected to asphyxia begins a “diving” reflex (so termed because of certain similarities to the physiology of diving seals) in an attempt to maintain perfusion & oxygen delivery to vital organs. The ductus venosus closes because of the clamping of the umbilical cord. This functional closure at birth is followed by anatomical closure that usually occurs at several months of age. with a resulting dilation of the pulmonary vessels. the infant then begins irregular gasping efforts. leading to a decreased pulmonary blood flow & increased blood flow directly to the left atrium. Functional mechanical closure of the ductus venosus is accomplished by the collapse of the thin . However. they have rapid respirations. leading to eventual tissue ischemia & hypoxia.walled vessels. All of these peripartum events result in closure of the other fetal shunts. Decompression of the capillary lung bed further decreases the pulmonary vascular resistance. the one . if an infant does not readily respond to stimulation. The increased systemic vascular resistance. systemic blood pressure increases.

warmed • blankets. the longer the onset of spontaneous respirations is delayed following the initiation of effective ventilation through the use of PPV. dry.o Bulb syringe o Regulated mechanical suction o Suction catheters (6F. The equipment should include a radiant warmer. the stimulation of the ventilatory efforts causes the infant to resume breathing. trays equipped for emergency procedures & drugs that may be useful in resuscitation. These infants should remain with their mothers during & after routine care.5 . Equipment Preparation for Resuscitation Rapid Assessment • • • • Newborn infants who need extensive resuscitation should be rapidly identified. Anticipation • • Causes of Depression & Asphyxia 170 The delivery room should be equipped with all the necessary tools to successfully resuscitate a newborn of any size or gestational age. position. 8F. drying the infant & assessing the color. stimulate & reposition). clearing of airway (if needed). If the infant is in secondary apnea. catheter tipped. These goals may be attained more readily when risk factors are identified early. adequate cardiac output & tissue perfusion & normal core temperature & serum glucose. A large number of ante partum & intrapartum maternal conditions carry an increased risk for intrapartum asphyxia. The goals of resuscitation are to assist with the initiation & maintenance of adequate ventilation & oxygenation. Infants who do not meet criteria for routine care need additional steps in their resuscitation. PPV is required for a longer period.o Intravenous catheters (22 g) • . instruments & supplies for establishing intravenous access. If an infant is experiencing primary apnea. 10F) o Suction tubing o Suction canister o Feeding tube (8F catheter) o Syringe. a source of oxygen. adequate respiratory effort & good muscle tone should receive routine care.4) o Tape & scissors o Laryngoscope (0 & 1 sized blades) o Extra bulbs & batteries o CO2 detectors o Stylettes for endotracheal tubes (optional) o Laryngeal mask Airway (optional) Suction . clear the airway. This includes the initial stabilization (provide warmth. The longer the infant is asphyxiated. neonatal problems are anticipated. a source of regulated suction. equipment is available.o Oxygen supply o Assorted masks o Neonatal AMBU bag & tubing to connect to an oxygen source o Manometer o Endotracheal tubes (2. personnel are qualified & available & a care plan is formulated.• • Resuscitation Program guidelines. It may also include ventilation. Routine care includes provision of warmth. Term infants with clear amniotic fluid. chest compressions & medications. 20 mL o Meconium aspirator Fluids . instruments for visualizing & establishing an airway. The minimum equipment necessary includes the following : • Respiration .

The newborn infant has limited energy stores. 000) .Drugs Procedures Tape & sterile dressing material Dextrose 10% in water (D10W) Isotonic saline solution T . premature.20 mL) Epinephrine (1 :10. When brown fat is metabolized.retarded infants.5F.e. with no use of calories or oxygen by the fetus. medication administration & emergency procedures. Neonates have a very limited capacity for metabolic heat production. • • • Resuscitating Neonates Thermoregulation • • • • Preventing heat loss during the resuscitation is essential. critically ill & depressed infants are unable to accomplish flexed positioning. use of bag mask ventilation & performance of chest compressions. The main source of heat production in the newborn is nonshivering thermogenesis. Intrauterine thermoregulation is passive. If the delivery is identified as high risk. Additionally. Heat is produced as a byproduct of the increased metabolic rate & oxygen consumption. however. at least one person should be present who is skilled in neonatal resuscitation & has responsibility for only the infant. including intubation. Animals ordinarily attempt to decrease heat loss by decreasing exposed surface area (i. Additionally. hemostats. forceps) o o o o o • • • Trained Personnel • • • For all deliveries. “curling up”). triglycerides are hydrolyzed to fatty acids & glycerol. infants do not shiver effectively. which increases heat & fluid evaporative loss. Stimulation by cold leads to norepinephrine production & thyroid hormone release causing brown fat to be metabolized. leading to further heat loss.g. Several factors lead to increased heat losses in the newborn infant. This paucity of fat stores is more pronounced in premature & growth . glycolysis is initiated & glycogen stores are used. Brown fat storage begins during the third trimester. 2 or more skilled individuals should be assigned for the infant at delivery. 171 • • • The fluid loss from the skin (not due to sweating but caused by direct transdermal water loss) results in massive heat loss. assorted (1 .o Umbilical catheters (2. Additional personnel should be immediately available to assist in tasks that may be required as part of the resuscitation. if needed. Additionally. This intrauterine thermoregulation therefore allows for maximal intrauterine growth without fetal energy expenditure for thermal homeostasis. the newborn infant (especially if premature) has a limited capacity to change body position for heat conservation. Brown fat is highly vascularized & stored in pockets around the neonate’s body. Infants who experience heat loss have an increased metabolic rate & use more oxygen. Brown fat may be used for heat production in the newborn period. Increased oxygen consumption can be dangerous in infants who are experiencing respiratory compromise. Thermo receptors in the face have a marked sensitivity to heat & cold. This person must be skilled in initiation of resuscitation. largely because of decreased subcutaneous fat & brown fat stores. both processes resulting in glucose production. The neonate has a large skin surface area–to–body weight ratio. which is a major source of heat production in the adult. This reduction in exposed surface area is accomplished by assuming a flexed position. 5F) o Chest tube (10F catheter) o Sterile procedure trays (e. scalpels.connectors Syringes. The addition of cold stress in infants who are poorly oxygenated . The thin skin with blood vessels that are near the surface provides poor insulation.

Inspired gasses that are sent to the lungs are subsequently heated & humidified by the infant. This stabilization area should be kept as warm as possible. Weights should be obtained on radiant warmer bed scales. This change in metabolism may lead to tissue hypoxia & acidosis because of the buildup of metabolic byproducts such as lactate. thus resulting in massive heat exchange due to evaporative heat losses & insensible water loss. Alternatively. Adequately warming the transport incubator is essential.mask device. whenever possible. the intubated & ventilated infant should be placed on a heated ventilator circuit as soon as is feasible. Infants with asphyxia have thermoregulatory instability & hypothermia delays recovery from acidosis. as opposed to later rewarming a cold infant. Airway management • • Once the infant is placed in a heated environment.warmed heat source. Infants have a vagal reflex response to sensory stimulation of the larynx.risk infant with the comfort of the adult staff in that area. cold stress can lead to both metabolic acidosis & hypoglycemia.valve . Open bed warmers.• • • • • • • potentially can lead to a change from aerobic to anaerobic metabolism. are used in most delivery rooms. the infant uses up glucose & glycogen reserves rapidly & still produces only a limited amount of energy for heat production. Following delivery. a stabilization room) should be separate from the operating room or labor room. balancing the requirements of the high . even when drying & a radiant heat source are used. which may induce apnea. Therefore. the thermal environment is precisely regulated by the mother’s core temperature & heat losses are nonexistent. encourages evaporative heat losses. which use radiant heat. can & should be performed with the plastic wrap in place.15 minutes thereafter until continuous temperature monitoring has been established. Ideally.warmed blankets or towels 172 & placed on a pre . Another common source of heat loss in the newborn infant undergoing resuscitation is the use of unheated non . instead. The primary goal in neonatal thermoregulation is to prevent heat loss. including line placement. The infant’s temperature should be documented as soon as possible after birth & every 10 . Heat losses are related to the differences both in water concentrations between the skin & the air as well as the absolute temperature gradient. the delivery room temperature should be increased. Because of the inefficiency of anaerobic metabolism. Low delivery room temperatures can predispose to hypothermia & NRP guidelines recommend that if a preterm delivery is anticipated.g. this allows special attention to the unusual thermal & environmental needs of the newborn high . bradycardia. infants continue to lose large amounts of heat. Therefore. warmed & humidified gasses should be provided in the resuscitation area.humidified oxygen sources for the bag .risk infant. They provide warmth during resuscitation & for any subsequent invasive procedures. As a fetus. an area (e. the act of suctioning the posterior oral airway or the trachea with a catheter because of extremely . Therefore. hypotension & laryngospasm. A bulb syringe should be used for the initial suctioning. Premature infants (< 1500 g) should be covered in plastic wrap (polyethylene) to prevent excessive heat loss. Continuous monitoring of temperature should occur as soon as possible after the delivery. A full resuscitation. A woolen head cap should be used. the infant should be positioned to open the airway & the mouth & nose should be suctioned. A potential approach to delivery room temperatures is to have a dedicated room in which ambient temperature can be well controlled Newborns should be dried with pre . The environmental temperature is also important in controlling heat loss in the newborn. It is important for the practitioner to keep in mind that this source of heat does not protect the infant from evaporative heat loss but.

then heated humidified oxygen should be supplied via an oxy hood. Wall suction should be set so that pressures do not exceed 100 mm Hg. For a number of reasons (discussed above). when necessary. if more vigorous stimulation is necessary. Vigorous suctioning of the nares with a catheter can lead to edema of the nasal tissues with resulting respiratory distress after the infant leaves the delivery room. The addition of positive pressure aids in the development of functional residual capacity & is needed more commonly in premature infants. Most infants do not require further intervention. deep suctioning of the trachea should be limited to infants who have thick mucous that cannot be removed by bulb syringe or used for the aspiration of aspirate stomach contents. This is considered routine care for most term infants with clear amniotic fluid who are actively breathing & crying & have good muscle tone.by oxygen via oxygen tubing or a mask. Therefore. At this point. The instillation of saline into the trachea also has been shown to stimulate the afferent sensory neurons leading to these sequelae & has no place in the immediate resuscitation period. If a blender is not available.stained fluids may cause profound central apnea.92% in the preterm infant. Infants who have a sustained heart rate of more than 100 beats per minute (BPM) & adequate respiratory effort but who remain cyanotic should receive blow . 173 • • • • Stimulation • Most infants need observational care. however. Mechanical lung inflation is also important to reverse persistent bradycardia in an apneic asphyxiated infant. retracting.pressure ventilation • • • Supplemental oxygen • • • . the infant’s respiratory rate. it can be difficult for the infant to clear fluid from the airways & establish air filled lungs. with the FiO2 adjusted to result in pulse . slapping the soles of the feet or rubbing the back may be effective. Further resuscitative efforts should be guided by simultaneous assessment of respirations. it may be assumed the infant is in secondary apnea & PPV should be initiated. Neonatal transition occurs over time. bradycardia & laryngospasm. with a fraction of inspired oxygen (FiO2) of 1 at a flow rate of 8 . Drying & suctioning often is enough stimulation to initiate breathing. The back should be visualized briefly for any obvious defect in the spine before beginning these maneuvers. or color need further intervention.96% in the term infant & 88 . Infants with adequate respirations who are having respiratory distress manifested by tachypnea. Supplemental oxygen should be initially provided.• • • thick or meconium . grunting. Term infants may also be resuscitated with a FiO2 of less than 1. Lung inflation has been shown to reverse the effects of vagal stimulation. Post resuscitative care is the term used for infants who require a more extensive resuscitation. • • Positive . This reflex bradycardia may be profound. heart rate & color should be evaluated. but it should be increased if the infant does not improve within 90 seconds. If supplemental oxygen is to be provided for a prolonged period. Initial respiratory efforts may need to be augmented by the addition of either continuous positive airway pressure (CPAP) or PPV. an FiO2 of 1 should be used & has not been shown to be detrimental to premature infants for a brief duration. heart rate & color. oxygen should be on a blender & blended up or down to keep the infants saturations around 90%. tone. Infants who do not meet the criteria for routine care or have difficulties with respiratory effort. flaring. If there is no response to stimulation.10 L/ min.oximetry saturations of 92 . In premature infants.

PPV should be initiated immediately. oxygen should be weaned. In term infants. To provide adequate distending pressure. If an FiO2 of less than 1 is used. Sufficient.limited T .piece have been shown to be equally effective. a self inflating bag or a pressure . If the infant is apneic. non aerated status to a neonatal status. A rise & fall in the chest wall movement is not always adequately assessed. It allows for a more precise delivery of inflation pressure & inspiratory times. but not excessive.40 cm H2 O during the first breaths. Ventilatory rates of 40 . Self . Infants who have continued central cyanosis despite supplemental oxygen should also receive PPV.inflating bags remain a standard of care. If the oxyhemoglobin concentration rises to about 95%. It has been shown to be just as effective as flow .60 BPM should be provided initially.24 cm H2 O in preterm infants with an increase in pressures if the chest does not rise or the heart rate does not rapidly increase.inflating & flow . Flow . either the airway is blocked or insufficient pressure is being generated by the squeezing of the bag. This may need to be reduced to 20 . others need continued ventilatory support. A T . with proportionally fewer assisted breaths provided if the infant’s spontaneous respiratory efforts increase. CPAP. the oxygen should be increased to 1 if the infant does not respond within 90 seconds. Any infant who does not respond to PPV with a heart rate of about 100 BPM should be placed on an FiO2 of 1 & have the mask repositioned. or a heart rate of less than 100 BPM. initial slow ventilation with more prolonged inspiratory times may be useful to assist in this task. have inadequate respiratory efforts (gasping). These mechanical devices control flow & limit pressure & have been shown to be more consistent than bags.controlled. Laryngeal mask airways have been shown to be effective .limited mechanical devices are acceptable for delivering PPV. Therefore. the neonatal lung is converting from a fetal. If supplemental oxygen is not available. a FiO2 of 1 should be used when PPV is started. Premature infants (< 32 wk) who require PPV should begin with oxygen concentrations between room air & 100% to maintain an oxyhemoglobin concentration of around 90% based on pulse oximetry. The ideal bag is equipped to deliver positive end . room air should be used to deliver PPV. A pressure manometer should always be used with a pressure release valve.expiratory pressure & the appropriately sized mask should be applied firmly to the face.inflating & self . The primary measure of adequate initial ventilation when providing assisted breaths is a rapid increase in heart rate. If no chest rise occurs. pressure . or both. The neonatal lung has a requirement for gas exchange & this requires the development of a functional residual capacity with the resorption of lung fluid & the resolution of most of the atelectasis. the infant must be properly positioned & the upper airway must be cleared of secretions. limiting the positive pressure to 30 .inflating bag. Some infants respond to brief mechanical ventilation & subsequently begin independent ventilation.• • • • • • • • • • or persistent central cyanosis may benefit from positive end expiratory pressure (PEEP).piece resuscitator device gives a measured pressure through a mask with a thumb occlusion & provides PEEP. initial pressure must be used to 174 • • • • • • • adequately inflate the lungs. At the moment of delivery & first breath.inflating bags. balanced with the avoidance of inappropriate inspiratory pressures. or bradycardia & apnea will persist. Ventilation provided with a flow . the mask must be the correct size & form a tight seal on the face.

congenital diaphragmatic hernia is suspected.15 seconds of intubation • is an indication of adequate ventilation & correct tube placement. Upon insertion of the endotracheal tube. CPAP may be beneficial in premature infants once they are breathing spontaneously. Before securing the endotracheal tube. Premature infants are at high risk for lung injury from large volume inflation. When CO2 detectors are used in infants with poor pulmonary blood flow with an inability to deliver sufficient CO2 to the lungs. An appropriate blade (Miller size 0 or 1) should be chosen depending on the size of the infant. Initial inflation pressures of 20 . effective bag . The use of CO2 detectors is the only technique that has been • evaluated for confirmation of endotracheal tubes in infants & should be used to confirm endotracheal intubation. All of these signs should be assessed within 30 seconds of PPV administration. The endotracheal tube should then be secured & cut to an • appropriate length to minimize dead space & flow resistance.25 cm H2 O is usually adequate. the tube should be advanced until the vocal cord guide mark near the distal tip of the tube is visualized to be slightly past the vocal cords. Premature infants may be more easily intubated with a size 0 blade & term infants require a size 1 blade. Another estimate of correct placement of the endotracheal tube • is to use the weight of the infant in kilograms plus 6 to arrive at the number of centimeters at which the tube should be secured at the lip. spontaneous breathing & improvement in muscle tone.flow oxygen should be provided throughout the procedure & effective ventilation should be provided via the bag or ventilator after the infant is intubated Endotracheal Tube Size & Measurement at Lip According to Infant Weight . CO2 detectors use a colorimetric change to indicate exhalation of CO2 gas. or a prolonged need for assisted ventilation exists. the infant should be • assessed for equal bilateral breath sounds with maintenance of oxygenation. Measurement of exhaled CO2 detector provides a secondary confirmation of endotracheal tube placement after intubation. endotracheal tube position is confirmed with chest • radiography. A CO2 detector can help confirm that the endotracheal tube is in the trachea & not the esophagus. Infants may require tracheal intubation if direct tracheal suctioning is required. An increase in the heart rate within 5 . This guide mark is positioned a variable distance from the distal tip (depending on the endotracheal tube size) & is designed to result in the placement of the tube tip between the vocal cords 175 Intubation • • • & the carina at the bifurcation of the right & left main stem bronchi.mask ventilation cannot be provided. The effectiveness of assisted ventilation should be evaluated by observing an increase in heart rate. Free .• • • • for assisted ventilation when bag mask ventilation & intubation are unsuccessful. An immediate increase in the heart rate is an excellent indicator of appropriate endotracheal tube placement. Other signs that should be monitored include improvement in color. a false negative result may occur leading to an unnecessary extubation. chest compressions are performed. Higher pressures may be needed if no improvement in heart rate or chest movement is noted. An appropriate size of endotracheal tube should be chosen based on the weight of the infant. endotracheal administration of medications is desired. Ultimately. Monitoring the pressure used in these patients & providing consistent inflations without high pressures is essential.

finger technique may be used when access to the umbilicus is required.5 . The current recommended dose for epinephrine is 0.3 endotracheal tube 3 . The administration of epinephrine may be followed with 0. Chest compressions should be initiated following only 30 seconds of effective PPVs if the heart rate remains less than 60 BPM.3. In contrast.Weight Endotracheal Tube Size Endotracheal Tube measurement at Lip • < 1000 g 1000 . Chest compressions should be discontinued as soon as the heart rate is higher than 60 BPM. Evaluate the heart rate & color every 30 seconds. If vascular access cannot be obtained epinephrine may be given via the endotracheal tube but the dose should be increased to 3 times that of the intravenous dose. 000 solutions) intravenously administered. Infants who fail to respond may not be receiving effective ventilatory support. The 2 . as well as the emergency room.1 .5 .0.5 endotracheal tube 2. Pressure should be applied to the lower portion of the sternum. Ensure that the small volume is not deposited on the endotracheal tube connector or in the lumen of the tube. Epinephrine use should be considered only when the heart rate is less than 60 BPM & ventilation has been established & provided for at least 30 seconds. Neonatal resuscitation drugs should be stocked in any area in which neonates are resuscitated. depressing it to a depth approximately one third of the anterior . The thumb technique is preferred because of improved depth control during compressions. This technique may also generate higher peak systolic & coronary perfusion.01 . further support is not effective.vascular volume expansion agent.3 mL of the 1 :10. Higher intravenous doses are not recommended & the post .0. Chest compressions should be discontinued when the heart rate is 60 bpm or higher. Intravenous administration is the preferred route. Medications • • • • • . dosages & routes of administration. including each delivery & stabilization area. Personnel should be familiar with neonatal medications.5 endotracheal tube 3. allowing for 30 breaths per minute.resuscitation hypertension could put premature infants at risk for intraventricular hemorrhage.9%) as an intra .2000 g 2000 . 1 ventilation should be interposed after every 3 chest compressions. constantly evaluating ventilation is imperative. but the rescuer’s thumbs should not remain in place. 000) & isotonic sodium chloride solution (0. The chest should fully reexpand during relaxation. Drugs currently recommended include epinephrine (1 :10.03 mg/kg (0.4 endotracheal tube 7 cm at the lip 8 cm at the lip 9 cm at the lip 10 cm at the lip • Cardiovascular support & chest compressions • • • • • • • • • Most infants who present at delivery with a heart rate less than 100 BPM respond to effective ventilatory assistance with a rapid increase in heart rate to normal rates. An assessment of the heart rate can be obtained by palpating the umbilical stump at the level of insertion of the infant’s abdomen or by direct auscultation of the precordium. 176 The recommended chest compressions/ventilations ratio is 3 :1 (120 events/min).3000 g > 3000 g 2. concentrations. if an effective airway & effective ventilation is not established. The only exception to this rule may be in infants who are born without a detectable pulse or heart rate. Chest compressions may be performed either by circling the chest with both hands & using a thumb to compress the sternum or by supporting the infant’s back with one hand & using the tips of the middle & index finger to compress the sternum.posterior diameter at a rate of 90 inches per minute. thus.5 1 mL of saline to ensure that the drug is delivered to the lung.

The currently recommended dosage for volume expansion is 10 mL/kg intravenously over 5 . A dose of 2 mEq/kg may be intravenously administered. More often than not. it is essential to use the lowest support possible to allow for adequate oxygenation & ventilation.resuscitation Period Maintenance of Airway & Ventilation • • • The goal of delivery room management is to stabilize the airway & assure effective oxygenation & ventilation. If an umbilical venous catheter is used for medication administration.negative blood that has been cross matched with the mother. Systolic blood 177 pressure also may be elevated falsely with pain. Sodium bicarbonate. In an editor ’s note commenting on an article entitled Cardiopulmonary Resuscitation in the Delivery Room. Volume expansion may be used in neonates with evidence of acute blood loss or with evidence of shock of any etiology. In general. may be useful in prolonged arrest after adequate ventilation is established. avoiding overdistention is essential.9% saline provides better cardiac & blood pressure support to correct both the acidosis & the underlying etiology of the metabolic acidosis..” • • • • • • • Sodium bicarbonate had previously been recommended in the delivery room to reverse the effects of metabolic acidosis related to hypoxia & asphyxia. The current recommendations for volume expansion during resuscitation include isotonic sodium chloride solution or lactated ringers.negative packed RBCs. the catheter should be inserted only until blood flow is obtained. you & the infant can then take deep breaths & you can beat your own chest instead of the infant’s. Once the appropriate functional residual capacity is obtained. If sodium bicarbonate is used in the face of a persistent respiratory acidosis & elevated pCO2. Breaths delivered by bag . the need for emergent placement of umbilical venous catheters has been reduced markedly in the delivery room. Because the dosing recommendations for epinephrine have included the endotracheal route of administration. ready availability & the lack of evidence of the superiority of other agents.carrying capacity is essential. However. Catherine DeAngelis writes.. providing adequate positive end . .• • • where it is absorbed & delivered to the heart. Restoring the critical oxygen . 5% albumin. usually 3 . the neonatal heart responds well to the increase in preload at the atrial level caused by the volume expansion. while at the same time preventing overinflation. The Immediate Post . the acidosis is not corrected. “. low cost. Hypovolemia may be masked in a newborn infant because of the significant peripheral vasoconstriction caused by the elevated catecholamines following delivery. Use of sodium bicarbonate in the delivery room has been associated with an increased incidence of intraventricular hemorrhage in very low birthweight infants. isotonic sodium chloride solution is the most frequently used agent for volume expansion. consider use of O . Therefore. However.10 minutes & it may be infused more cautiously in extremely preterm infants.mask ventilation may be difficult to control & may result in overdistention & consequent pneumothorax or pneumomediastinum.5 cm. mechanical ventilation should be initiated as soon as possible once the infant is stabilized. Once initial lung recruitment is obtained. recent studies show that 0.. Plasmanate. Although the ideal mode of assisted ventilation is controversial. Additionally. resulting in hypothermia. When blood loss is known. however.expiratory pressure to prevent atelectasis. check the airway (optimize respiratory support) one more time before compressing the chest.humidified oxygen can quickly cool the infant via the large surface area of the lungs. because of the advantages of long shelf life. the unheated non . or O .

hypoglycemia may occur rapidly in critically ill or premature infants. as discussed in previous sections. Taking the infant’s environment into account when calculating fluid requirements is very important. Additionally. With the clamping of the cord. lactate & amino acids to store fuels that are used during transition. Preparation for Transport • • • • • • Special problems during resuscitation This section is devoted to congenital & other neonatal conditions that may present in the delivery room & that may alter the . These basics of care may be neglected in the rush to prepare the infant for transport. Fluid rates may be started at 60 . requiring adequate intravascular volume to maintain adequate cardiac output. nutrients are provided in their basic form. care must be taken to secure all lines. the greatest amount of glucose. Blood glucose determinations should be performed as soon as possible & a continuous infusion of glucose should be started at 4 . Neonates must develop a homeostatic balance between energy requirements & the supply of substrate as they move from the constant glucose supply of fetal life to the normal intermittent variations in the availability of glucose & other fuels. Dextrose boluses should be limited to symptomatic infants because they may result in transient hyperosmolarity & rebound hypoglycemia.60 mg/dL. the maternal glucose supply is cut off.96% range for the term infant & 88 .6 178 • • Fluid & Electrolyte Management • • • • • mg/kg/min for those infants who are not able to tolerate enteral feedings. First. Electrolytes. Rapid & complete documentation of the resuscitation & subsequent therapies also is required for future caretakers. tubes. urine output & serum sodium concentrations closely because inappropriate fluid overload or restriction can lead to increased mortality & morbidity. should not be added initially because the fluid shifts from other body compartments allow for adequate electrolyte supply until adequate renal function is documented.g. The neonatal cardiovascular system is very sensitive to preload. In utero.92% in the preterm infant after the initial stabilization. The immediate goal of fluid & electrolyte support following resuscitation is to maintain an appropriate intravascular volume & to provide glucose homeostasis & electrolyte balance. Monitoring in the transport environment is only possible with functioning leads in place.6 hours of life occurs in healthy newborns. whereas fluid rates may be much higher for the infant in a dry radiant warmer environment. Saturations should be maintained in the 90 . The liver. Following resuscitation. catheters & leads for transport. The blood glucose usually reaches a nadir & stabilizes at 50 . with potentially disastrous results. potassium & chloride. Fetal glucose uptake parallels maternal blood glucose concentration.80 mL/kg/d for the infant in a humidified incubator.• • Oxygen saturations should be monitored continually & arterial blood gas analyses performed as needed during the initial stabilization period. completing all the routine care that is required of newborn infants is essential. A fall in blood glucose during the first 2 . therefore. isotonic sodium chloride solution) often is considered in the neonate with inadequate blood pressure or perfusion. such as sodium. Preparation of the infant for transfer to a remote nursery for subsequent care requires several considerations. Glucose is the major energy substrate of the fetus. which is frequently difficult. expansion of intravascular volume with appropriate solutions (e. The practitioner should monitor the weight. The fetus uses glucose. Therefore. heart & brain receive the greatest cardiac output and. clinical hydration status.

especially because of organisms that colonize the skin surface (e. staphylococcal species). Insensible water loss in the premature infant is increased secondary to the infant’s poorly cornified epidermis & an immature stratum corneum. Volume expansion should only be administered in the face of true hypotension.resuscitation.containing fluids. This thin skin barrier also places the extremely immature infant at risk for toxicities from topically applied substances. Placing infants in a humidified environment decreases transepidermal water loss. Measures to decrease insensible water loss should be initiated at delivery. The skin barrier dysfunction increases the risk for infection. Differences in skin maturity. insensible water losses. which should be limited 179 • • • • • • in premature infants. hypoglycemia & intraventricular hemorrhages. Because of high evaporative loss with the accompanying heat loss. With increased parenteral fluid administration using dextrose . Knowledge of normal blood pressure values for infants of various gestational ages is essential. infants born before 28 weeks’ gestation continue to have increased TEWL for 4 .g. Ventricular hemorrhage & periventricular leukomalacia often lead to serious permanent neurodevelopmental disabilities. The skin is the most important route for water depletion after delivery of the extremely immature infant. This population of infants is at increased risk for respiratory failure. Despite declines in TEWL with advancing age. the ability to achieve & maintain thermoregulation is compromised further. The presentation of the disease & the immediate resuscitative efforts are discussed. The stratum corneum is not functionally mature until 32 . Additionally. Prevention or reduction of the severity of these disorders may begin in the delivery room.5 weeks following birth. Volume expansion in the face of normal blood pressure increases the risk of intraventricular hemorrhage. when administering hyperosmolar medications (e. care should be taken to ensure that the barrier does not block the transmission of the radiant heat source. Additionally. Extreme Prematurity • • • • • • • Premature infants have special needs that must be considered during the critical period immediately following delivery if mortality & morbidity are to be decreased in this group.34 weeks’ gestation. sodium bicarbonate). Because radiant warmers are used routinely at deliveries because of a need for maximal patient access. the dextrose needs to be monitored closely to ensure euglycemia. improves the maintenance of body temperature & does not delay skin maturation. . Premature infants are at risk for intraventricular hemorrhages & periventricular leukomalacia (PVL) secondary to their immature cerebral vascular regulation & the persistence of the germinal matrix.g. However. compared to infants born at term. which presents little barrier to evaporative heat loss. Premature infants need increased fluid administration rates initially if they are on radiant warmers for a prolonged period. Mechanical ventilation & fluid administration must be managed cautiously in this group of infants. skin integrity is disrupted easily by the use of adhesives. ventilation requirements & environmental conditions all may influence the magnitude of insensible water loss that occurs following birth. slow administration is important. Transepidermal water loss (TEWL) is highest at birth in infants who are born before 28 weeks’ gestation & decreases slowly with advancing gestational age. prenatal nutritional status. infants less than 1000 g should have a plastic blanket or other barrier applied to decrease evaporative water loss until they can be placed in a humidified environment.

Wheezing or stridor may be audible with inspiration & collapse of the small airways with vigorous inspiratory effort can occur. Prematurity with respiratory distress syndrome (RDS) is not associated with mucous production in the first 24 hours of life. Following the institution of mechanical ventilation. At rest. causes nasal passage swelling in any infant with the subsequent iatrogenic occlusion mimicking the congenital condition. Pierre Robin syndrome • 180 . intraventricular hemorrhage & periventricular leukomalacia. which may occlude the upper airway. especially by inexperienced personnel. often leads to inconsistent tidal volumes & pressures. Following the instillation of artificial surfactant. intubation is necessary. its administration may lead to hyperventilation & overdistention when not administered by experienced attentive personnel. these infants usually manifest severe apnea. Intubation relieves the obstruction so that minimal ventilation (if any) is required. Airway Problems Choanal atresia • • • Choanal atresia is caused by a failure of embryologic regression of nasal airway tissue. thereby resulting in a partial or complete occlusion of the nasal airway.8 weeks of life. Supplemental oxygen should be administered to infants with choanal atresia & an oral airway may be of assistance. The infant in respiratory distress should be stimulated to cry & an artificial oral airway may be used to avoid intubation. Respiratory distress & cyanosis are caused by the obstruction of the upper airway. rapid reaction to changes in pulmonary compliance to prevent the onset of hypocarbia & alkalosis is essential. Although artificial surfactant administration is associated with a reduction of adverse sequelae in infants. especially with repeated attempts. retractions & respiratory distress that may be relieved with crying. Premature infants are also at high risk for volutrauma caused by poor lung compliance & overventilation following the administration of exogenous surfactants if changes in lung compliance are not monitored carefully. Stabilization of the infant using the lowest possible peak inspiratory pressure required to oxygenate & ventilate adequately is essential. If the infant remains in significant respiratory distress. Rapid alterations in pCO2 & pH result in acute fluctuations in the cerebral blood flow of the premature infant with immature cerebral vascular autoregulation. thus suctioning protocols should be altered to provide minimal suctioning during this time. However. Use of a mechanical ventilator designed for infants offers the advantages of more consistent tidal volumes & a reduction of the heat losses because of the use of unheated non . Hand ventilation of an intubated infant. A central cleft of the soft palate is usually present. especially when pCO2 & pH are rapidly altered.humidified air with hand bagging. This syndrome presents with micrognathia & with a resultant displacement of the tongue into the posterior pharynx. The clinical diagnosis is achieved by the inability to pass a small caliber catheter through the nasal passages. These choanal defects may be bony or membranous. An alternative noninvasive method of excluding the diagnosis of complete atresia is to place a glass slide under the nasal orifices & look for fogging with expiration. care should be taken with airway suctioning because vigorous or frequent airway suctioning is associated with hypoxia. the act of passing catheters.• • • • • • • Mechanical ventilation may lead to harmful fluctuations in cerebral blood flow. Complete bilateral stenosis usually results in a neonatal respiratory emergency at birth because infants generally are obligate nasal breathers during the first 6 . Overventilation with excessive tidal volumes & hypocarbia are associated with chronic lung disease. with most having a bony component.

Infants with isolated esophageal atresia usually do not demonstrate respiratory distress immediately in the delivery room but may have excess secretions. Tracheal webbing • • • • • • The pathogenesis of tracheal webbing originates in the tenth week of gestation when an arrest in the development of the larynx near the vocal cords results in a residual web of tissue persisting in the airway. with the development of distress later when activity increases & the need for airway flow increases. the infant should be given supplemental oxygen & placed in a prone position in an attempt to have the tongue move forward in a dependent fashion from the posterior pharynx. but this is rare. Type V (Esophageal atresia with a double fistula) : This type is rare.type esophageal atresia) : An isolated fistula connects the esophagus & trachea. controlled surgical placement of a gastrostomy tube is . Air is absent from the lower GI tract. relieving the airway obstruction. The most common clinical symptoms of esophageal atresia with or without an esophageal . This distending pressure cannot be relieved by esophageal reflux through the atretic esophagus. however. In rare emergency situations. An upper esophageal segment is present with a fistula to the trachea & a blind lower esophageal segment. which then results in abdominal distention. but an air . Any positive pressure applied to the airway results in inflation of the fistula. placing these infants at high risk for aspiration. If the infant is manifesting severe distress. If at all possible. Air is absent from the lower GI tract.threatening emergency. Approximately 75% of tracheal webs occur at the level of the vocal cords. These lesions are critical if more than 50% of the airway diameter is occluded.tracheal fistula include coughing. however. a large bore needle or catheter may be placed into the trachea to allow for gas exchange while arranging for emergency treatment. These disorders may be relatively asymptomatic at birth. choking & cyanosis. early diagnosis is essential to prevent further complication. An upper esophageal segment is present with a fistula to the trachea & a second fistula connects the distal esophagus & trachea. Type III (H . If the infant continues to have persistent respiratory distress. The continued application of PPV also may lead to massive gastric distention & possible rupture. Air is present in the stomach. Air is present in the stomach. The types of esophageal atresia are as follows : Type I (Esophageal atresia with a distal fistula) : This is the most common type (85%). 181 • • • Esophageal atresia with or without tracheoesophageal fistula • • • Type II (Esophageal atresia only) : A blind upper & lower esophageal segment is present.• • In the delivery room. the contents of the esophageal pouch readily reflux. Alternatively. Because secretions or oral feedings are not capable of passage into the stomach. percutaneous gastrotomy may be required to decompress the stomach. Caution must be used because inexperienced personnel may confuse this rare disorder with simple inability to visualize the vocal cords. A blind end upper esophageal segment is present with the distal segment of the esophagus connected to the trachea via a fistula. stomach & bowel. an oral airway may be placed. Relief of the distending pressure occurs with reflux of gastric contents into the lungs via the fistula.filled blind upper pouch may be observed. an appropriately sized endotracheal tube may be passed through the nose into the hypopharynx. PPV should be avoided in these infants. usually occurring at the upper portion of the trachea & esophagus. Type IV (Esophageal atresia with a proximal fistula) : This type is rare. This condition rarely is considered a life .

the infant often presents with respiratory distress.valve . Air that dissects into the hilum results in a pneumomediastinum. Following the initial rupture of a small airway or an alveolus. Respiratory distress in the delivery room may be caused by either a pulmonary hypoplasia or may be secondary to an expansion of the bowel caused by swallowed air. The expansion of the bowel results in compression of the lung. These infants may present with significant respiratory distress & require immediate intubation with deep positioning of the endotracheal tube to relieve the obstruction by stinting open the airway. including extracorporeal bypass. resulting in a migration of the abdominal viscera into the chest during development.” Infants in acute distress should have a needle aspiration . In the delivery room. The occurrence of a pneumothorax often is associated with PPV. air may enter the perivascular & peribronchial spaces & track along the lymphatic channels.mask ventilation. Lymph accumulates & may compress the airway. Pneumothorax • • This condition is the result of a congenital deformity of the lymphatic channels. Infants with a pneumomediastinum should be observed. mode of ventilation & expertise of delivery room personnel. Cystic hygromas of crying or bag . Spontaneous rupture of the lung directly into the pleural space is thought to occur rarely but may be caused iatrogenically with the percutaneous insertion of a chest tube. • • Pulmonary Compression Congenital diaphragmatic hernia • • • • • The pathogenesis of this disorder is caused by the incomplete formation of the diaphragm in the fetus. These infants usually are asymptomatic or minimally symptomatic because air in the mediastinum is capable of escaping to the tissues of the neck. which allows the management to be transferred to a perinatal referral center where pediatric surgery & appropriate medical support are available. Pneumomediastinum frequently is an isolated disorder that occurs spontaneously in infants with minimal pulmonary disease. Physical signs may include a scaphoid abdomen & a shift in heart sounds to the right hemithorax.preferable. Intrathoracic tension is relieved & circulation is not compromised. If the defect is large & the abdominal viscera have caused long standing compression of the developing lungs. Intervention usually is unnecessary. The diagnosis of diaphragmatic hernia is established frequently by prenatal ultrasonography. Approximately 80% of these lymphatic cystic accumulations occur in the neck & may compress the trachea. Intubation prevents distention of the stomach & bowel contents because 182 • • • Air leak syndromes are disorders produced when a rupture of pulmonary tissue occurs with the resultant escape of air into spaces in which air would not be present normally. but it also may occur in infants who are not receiving assisted ventilation. Air that tracks into the pleural space manifests as a pneumothorax. need for assisted ventilation. The incidence of pneumothorax varies with gestational age. Pneumothorax may occur immediately in the delivery room or later when significant pulmonary disease has developed. Delivery room management includes immediate intubation & passage of a large catheter for gastric decompression. This is termed a “tension pneumothorax. Following the initial air leak. depending on the size & location of the lymph accumulation. the subsequent expansion of intrathoracic spaces often rapidly results in an increase of intrathoracic pressure such that there is an inability to ventilate the lungs & an inability to return venous blood to the heart. pulmonary hypoplasia may develop. severity of pulmonary disease.

Because of the excess fluid in the lungs. If the infant is diagnosed with an omphalocele. Miscellaneous Multiple gestations be available in the delivery room. if the condition is recognized prenatally. These procedures may need to be performed immediately if the fluid accumulation is causing difficulties in ventilation. if required. amniocentesis for chromosomal analysis should be offered to the family. Symptomatic pneumothorax is managed with the insertion of a chest tube until the pulmonary leak is resolved. Omphalocele & gastroschisis • • • The delivery & subsequent resuscitation of multiple infants presents a considerable challenge to the labor & delivery team. Supplemental oxygen (FiO2 = 1) often is administered for 6 . Therefore. Evaluation & recommendations for changes in the current standards is an ongoing . the blood glucose should be assessed because this defect may be associated with Beckwith .Wiedemann syndrome. such as a paracentesis or thoracentesis. with the bowel covered by a thin membrane. In contrast. Congenital anomalies • Hydrops • • • Severe malformations observed in the delivery room should not change the resuscitative management unless skilled & experienced care providers are able to determine that the condition is incompatible with life. Preoperatively. Controversies in Resuscitation • Neonatal resuscitation has been standardized with the development of a certification program. a sufficient number of skilled personnel must be in the delivery room to ensure that the multiple needs of this significantly compromised neonate can be met. Trisomy 18 is associated with this anomaly. often using high pressures & oxygen are necessary initially. A minimum of 2 experienced personnel should be available for each infant. The first consideration to be addressed with the initial prenatal diagnosis of multiple gestations is to ensure that the care of such a pregnancy is at an institution capable of providing such support for the mother & multiple infants at delivery.performed to evacuate the extrapulmonary air while preparation is made to place a chest tube. When preparing for the resuscitation of a hydropic infant. to maintain thermoregulation & to prevent bowel ischemia.12 hours to hasten reabsorption of the trapped intrapleural air. preparation to ensure the presence of appropriate personnel & equipment must be planned well in advance of the delivery. Infants with severe malformations should be resuscitated & stabilized until an accurate diagnosis can be made. for higher order gestation involving triplets or more. an omphalocele involves the bowel herniating through the umbilical opening. The family should be involved in any decision in which no resuscitation is to occur. blood for transfusion should 183 • • Gastroschisis is an abdominal wall defect lateral to the umbilicus that does not have a sac or membrane covering the bowel. For both omphalocele & gastroschisis. Therefore. as multiple gestation infants are often born prematurely (especially with higher order gestation) & more personnel may be required for each infant. these infants have increased fluid requirements unless the bowel is appropriately wrapped with an airtight material. If the hydrops is caused by anemia. unless the membrane has been ruptured intrapartum. maintain adequate intravascular fluid volume. Equipment should be prepared before the delivery & all personnel in the room should be assigned specific procedures. A chest tube may not be required if the pneumothorax is small & does not involve an infant who is not receiving PPV.

Once an infant has been stabilized. A meconium aspirator should be applied directly to the endotracheal tube & continuous pressure should be applied using 120 . overdistention of the alveoli. pneumothorax. Surfactant deficiency. extremely low birth weight (< 400 g) & chromosomal anomalies that are inconsistent with life (e. With the second intubation. Currently. This may include extreme prematurity (< 23 weeks’ gestation). The obstetric & neonatal team. Free . trisomy 13). as well as the parents. it is essential to evaluate the quality of the studies & make changes in practice based on evidence. birth weight & congenital anomalies are associated with certain death should not be resuscitated. Depressed infants should be placed on a radiant heat source & no drying or stimulation provided until they are intubated & direct tracheal suctioning is performed.15% of all deliveries & rarely is seen before 34 weeks of gestation. surfactant deficiency & persistent pulmonary hypertension. intubation & suctioning can be performed again. Room Air Versus 100% Oxygen • • • • Oxygen is a drug with the potential for serious adverse effects that must be considered. As new research is published. it is necessary to evaluate the heart rate before a second intubation is performed. Of newborns born with meconium stained fluid. should decide when to withhold or discontinue resuscitative.150 mm Hg as the tube is removed. In other situations in which the prognosis is uncertain but the associated morbidity rate is high. Of these infants that require stabilization and/or resuscitation. The use of lower oxygen concentrations when resuscitating the neonate may decrease the number of oxygen free radicals & their damaging adverse effects. This section outlines some of the current controversies & concerns in resuscitation. 184 • • Meconium aspiration in a newborn can lead to atelectasis. is the most common cause for persistent & progressive respiratory distress in premature infants.flowing oxygen should also be used in infants with central cyanosis. Discontinuing resuscitation may be justified in infants who have not responded to continuous & appropriate resuscitation for a full 10 minutes & who have no heart rate or respiratory effort (no signs of life). Infants whose gestational age. 60% require stabilization and/or resuscitation. In one study. Meconium staining of amniotic fluid occurs in 10 . resuscitation with room air was shown to be as effective as 100% oxygen at lowering pulmonary vascular resistance. supplemental oxygen should be provided whenever PPV is required during resuscitation. 3 . parenteral desires should be considered.process. Oxygen free radicals are capable of tissue injury & have been implicated in several disease states in the neonate. If meconium is obtained. pneumonitis. the practitioner may want to consider providing PPV through the endotracheal tube after suctioning is performed. Prophylactic dosing of artificial surfactant is performed in the delivery room before the first breath or within 15 minutes following birth.g. the primary factor in the development of RDS. Withholding & Discontinuing Resuscitation • • Timing of Artificial Surfactant Administration • • • Intubation & Suctioning for Meconium • • 61 . Clinicians may begin resuscitation with an oxygen concentration of less than 100% & may even consider starting with room air as new research data become available.4% are diagnosed with meconium aspiration syndrome.

mongoloid slant.5 Average 50 cm Average 35 cm Average 32 cm 2. the process through which the mammary gland develops the capacity to secrete milk (lactogenesis). The development of the mammary gland (mammogenesis). cleft lip or plate. Icterus seen within 24 hrs after birth Blood loss from any opening Lethargy Imbalance in body temperature Danger signs in a newborn – – – – • • • Acrocyanosis is the cyanosis of extremities which is normal & is present due to cold Erythema toxicum is small.Examination of newborn Anthropometry of newborn Anthropometry Pre term Full term Post term • • 2. Head Face • • • Neck Face should be observed for any signs of dysmorphism like low set ears. perforation. exomphalus. average 35 cm average 32 cm (3 cm less than head circumference) Weight Length Head circumference Chest circumference Weight Length Head circumference Chest circumference Skin • Look for any masses or lump present Abdominal distension may be present due to obstruction. umbilical hernia Excessive crying Excessive drooling of saliva from mouth Central cyanosis Seizures Refusal to feed Difficulty in feeding Vomiting/diarrhea No passage of meconium within 24 hrs & urine within 48 hrs. trunk & extremities. Abdomen 185 The breast begins to develop in utero. webbing etc. 1) 2) 3) 4) 5) 6) 7) 8) 9) 10) 11) 12) 62 • Breast milk is thought to be the best form of nutrition for neonates & infants.5 – 3. These disappear within first year of life. bladder extrophy. vesiculopustular papules present after 2 . the process of milk production (lactation) & the specific properties of human milk those make it unique & appropriate for human infants. laxity. immune & guts development. depressed nasal bridge. Hypertelorism. This dynamic fluid provides a diverse array of bioactive substances to the developing infant during critical periods of brain. undergoing the first of many developmental changes necessary for proper breastfeeding to . • Pathophysiology Mammogenesis Neck should be examined for presence of any swelling or tumor. It may be seen on face. epicanthal folds etc.4 days of birth. back & other body parts. Ears should be examined for any preauricular tags Mouth should be examined for presence of natal teeth. Mongolian spots are greenish blue areas of pigmentation commonly seen over buttocks.5 kg average 50 cm. Look for abdominal wall defects as in trisomy 18. meconium ileus etc.

Oxytocin reflex The other important hormone involved in the milk ejection or letdown reflex is oxytocin. traveling to the mammary . Lactogenesis includes all processes necessary to transform the mammary gland from its undifferentiated state in early pregnancy to its fully differentiated state sometime after pregnancy. A bulb . are then relayed to the central nervous system. the breast becomes capable of milk production.occur. their combined actions are essential for successful lactation. Endocrine control switches to autocrine (supply . Breasts are full & warm. The suckling infant stimulates the touch receptors that are densely located around the nipple & areola. When the neonate is placed at the breast & begins suckling. The stimulation of the nuclei causes the release of oxytocin down the pituitary stalk & into the posterior pituitary gland. where oxytocin is stored. prolactin (PRL) & oxytocin. allowing milk to flow to the recipient infant.19 weeks’ gestation. The basic unit of the mammary gland is the alveolus or acinus cell that connects to a ductule. Stage 2 (day 2 or 3 to day 8 after birth) : The tight junction in the alveolar cell closes. The stages of lactation can be summarized as – • o • • o o • Mammogenesis : Mammary (breast) growth occurs. Autocrine system control continues.demand) control. The infant’s suckling creates afferent impulses that stimulate the posterior pituitary gland. This cyclical process of milk synthesis & secretion is termed lactation. The secretion of PRL appears to be both positively & negatively regulated. Copious milk secretion begins. Lactation occurs with the help of 2 hormones. which are in direct response to stimulation of the nipple & areola (mammae). This releases oxytocin in a pulsatile fashion to adjacent capillaries.shaped mammary bud can be discerned in the fetus at 18 . Prolactin reflex Milk synthesis occurs in the mammary gland epithelial cells in response to PRL. These lactiferous sinuses drain to 15 25 openings in the nipple. Lactogenesis Stage 1 (late pregnancy) : Alveolar cells are differentiated from secretory cells. Although PRL & oxytocin act independently on different cellular receptors. PRL stimulates mammary glandular ductal growth & epithelial cell proliferation & induces milk protein synthesis. oxytocin is released. the mammary gland develops the capacity to secrete milk. These signals. o o Lactation • o Production & secretion of milk is dependant on maternal hormonal reflexes & reflexes in the baby. Factors enhancing prolactin reflex frequent emptying of breast frequent suckling expression of milk Inhibiting factors Bottle feeding Incorrect feeding & positioning Painful breast conditions. Maternal reflexes – Prolactin reflexes (milk secretion reflex) • Oxytocin reflexes (milk ejection or letdown reflex) Neonatal reflexes – Rooting reflex • Sucking reflex Swallowing reflex 186 o Two essential hormones (prolactin & oxytocin) During the second stage of lactogenesis. Lactogenesis • o In lactogenesis. Galactopoiesis (later than 9 d after birth to beginning of involution) : Established secretion is maintained. The size & weight of the breast increase.

Milk secretion directly correlates with synthesis The regulation of milk synthesis is quite efficient. Thus. bottles. Duration & frequency of feeding Breastfeeding should be given baby is hungry without any restriction of time. You should be able to give nipple & maximum possible areola (dark portion behind the nipple) in baby’s mouth for optimal breastfeeding. Oxytocin causes the contraction of the myoepithelial cells that line the ducts of the breast. This may also prevent successful establishment of breastfeeding because a baby with a full stomach may not suckle at the breast. Factors that lessen milk production Neonatal reflexes helpful in breast feeding – 1) Rooting reflex – When the mother holds the baby for breast 1) 2) 3) 2) • • • 3) • o feeding the nipple touches the lips. 187 Dummies. in turn. 4) Certain medications like OCP. when stimulated. Also learn to express breast milk & storage . formula feed either as pre – lacteals or anytime. Emotional stress. Methergine. Milk synthesis remains remarkably constant at approximately 800 mL/d. The suck – swallow – breath cycle lasts for about 1 sec. pacifiers Making baby wait for feeds Giving feeds like sugar. Feeding should be given even during the night. the rate of milk synthesis is related to the degree of breast emptiness or fullness. learn to hold & position the baby properly & comfortably while breastfeeding. Relaxation is key for successful lactation. Factors enhancing oxytocin reflex Sight of baby. Do not give glucose/ jaggery/ sugar/ plain water or honey before the first breastfeed. Worry. This may cause infection in the baby’s digestive system. honey. Milk production is responsive to maternal states of well . The mechanism for this effect is the down . gripe water.o o myoepithelial cell receptors that. shift to the second breast. water. The swallowing reflex develops early in foetal life but sucking reflex develops by 32 – 34 wks of gestation. Squeezing movements of tongue draws the milk from lactiferous sinuses. a local factor secreted into the milk. Swallowing reflex – When the mouth is filled with milk. 5) Painful breast conditions like sore/cracked nipples & congested breast. the actual volume of milk secreted is adjusted to the requirement of the infant by feedback inhibitor of lactation. Feed at one breast at a time till it is empty because the initial milk (foremilk) is watery (contains sugar & proteins) & quenches the baby’s thirst while the milk that is secreted later (hindmilk) is rich in fats & satisfies baby’s hunger. These smooth muscle– like cells. therefore. After the first breast is empty. Confident mother Factors inhibiting oxytocin reflex Anxiety.being. expel milk from alveoli into ducts & subareolar sinuses that empty through a nipple pore. or both. stimulate the cells to contract. norepinephrine. Pain o The emptier breast produces milk faster than the fuller one. It consists of Drawing the nipple & areola into the mouth to form an elongated teat Nipple & areola is pressed between jaws & tongue against the palate. Before discharge from the maternity home Before discharge from the maternity home. Sucking reflex – After accepting the nipple baby starts to suck.regulation of milk synthesis with increased levels of dopamine. the baby swallows it & then breathes. However. cheek or side of the mouth the baby turns its head towards the stimulus & searches the nipple. which inhibit PRL synthesis. stress & fatigue adversely affect a woman’s milk supply. Sound of baby.

Magnesium. Lactose. Water. For example.lactalbumin. Mother’s milk contains enough water. minerals. Secretory IgA. Lactic acid 188 • o • o In addition to the changes from colostrum to mature milk that mirror the needs of the developing neonate. Balkadu.lactalbumin. triglycerides. Specific enzymes to aid neonatal digestion Human milk contains various enzymes. Polymorphs. Proteins.3 LCP fatty acids are deposited in the developing brain . Oligosaccharides. complex nutritive fluid with immunologic & growth . The fats are composed of cholesterol. Growth factors.lactalbumin & whey). Composition of breast milk o o Human milk is a unique.chain polyunsaturated (LCP) fatty acids. honey. Caseins. Hence even in summer. fatty acid synthetase. Copper.dimensional structure of human milk Under a microscope. short . Plasma cells (B cells) 8) Probiotic substances – Lactobacilli. Iron. protein & total lipid concentrations in human milk o Give only breast milk till the baby is 6 months old. Start breastfeeding as soon as the baby shows readiness to suckle. thio esterase). species . Sodium 6) Trace elements – Chromium. the appearance of human milk is truly amazing.to 22 .promoting properties. Large amounts of omega . Triglycerides 4) Water soluble vitamins – Biotin. Three . This ensures that breastfeeding can continue for a sufficiently long period of time Exclusive breastfeeding This unique fluid actually evolves to meet the changing needs of the baby during growth & maturation. fats & carbohydrates that facilitate the infant’s ability to break down food & to absorb human milk. This hind milk is thought to facilitate satiety in the infant. Folate. 1) Proteins – α . This relationship reverses as the infant matures. The LCP fatty acids (18 . the diurnal variations in breast milk reflect maternal diet & daily hormonal fluctuations. Neutrophils. 2) Carbohydrates – Lactose. Fatty acids. some are specific for the biosynthesis of milk in the mammary gland (e. Hence these items should not be given. an infant does not require water. Vit. lactose synthetase. fruit juices or any other item should not be given during this period.6 & omega . Glycopeptides. early milk or colostrum has lower concentrations of fat than mature milk but higher concentrations of protein & minerals. Niacin.chain fatty acids & long . Potassium. Bifidus factor 3) Lipids – Fat soluble vitamins. Selenium. Riboflavin 5) Minerals – Calcium. Cobalt. Iodine. Vit. whereas others are specific for the digestion of proteins. almonds. Finally. carbohydrates (lactose). vitamins & fats for the growing term infant. Babies are very alert in the first hour after delivery & tend to suckle easily & vigorously. human milk has substantial structure in the form of compartmentation.g. then it is easier for the mother to establish regular breastfeeding. Phospholipids.The first contact & feed Biochemistry of human milk Hold your baby in close skin – to – skin contact within half of delivery. Zinc 7) Cells – Macrophages. vitamins. Although it is a fluid. top milk.carbon length) are needed for brain & retinal development. carbohydrates & designer fats for optimal brain development Human milk provides appropriate amounts of proteins (primarily alpha . B12 & 6. Epithelial cells. tonics for teething or any other similar preparations are unnecessary & can be dangerous. C.specific. β . These will interfere with breastfeeding & may introduce infection. Gripe water. Leukocytes. It has been proved that if the baby is breastfed earlier. variation exists within a given breastfeeding session.

Interferon & fibronectin have antiviral activities & enhance lytic properties of milk leukocytes. E). various immunologic & bioactive milk components act synergistically to provide a passive immunologic support system from the mother to her infant in the first days to months after birth. 189 • o Mucins adhere to bacteria & viruses & help eliminate them from the body. insulin like growth factors (IGFs) & interleukins. o The antigenic specificity of the mother’s sIgA in her milk is directed against the same antigens in the neonate. which are present on the milk . Transforming growth factor (TGF)–alpha. with the transition from colostrum to mature milk. Immunologic properties of human milk Human milk immunoglobulins Human milk contains all of the different antibodies (M. nerve growth factor (NGF). with the remainder of cells primarily consisting of lymphocytes & polymorphonucleocytes. By 7 . Types of breast milk . may have a limited ability to synthesize optimal levels of AA & DHA from linoleic & linolenic acid. TGF . Milk leukocytes can tolerate extremes in pH.beta & granulocyte colony .o o & retina during prenatal & early postnatal growth. Passive immunity from mother to recipient breastfeeding infant • o While awaiting endogenous maturation of the baby’s own immunologic systems. Milk lipids. which binds to iron. o The immunologic components include Lactoferrin. which damage membranes of enveloped viruses. such as epidermal growth factor (EGF). Therefore. the percentage of macrophages then increases to 80 .90% at a concentration of 104 . which intercept bacteria & form harmless compounds that the baby excretes. Milk . Certain bioactive substances & live cells in milk appear to influence neonatal gut maturation & growth through their transfer of developmental information to the newborn.derived sIgA is a significant source of passively acquired immunity for the infant during the weeks before the endogenous production of sIgA occurs. An infant.fat globule membrane. human milk has numerous factors that can affect the intestinal microflora of the baby. Bioactive properties of human milk Human milk also contains growth modulators. particularly a preterm infant. and Mucins.60% of the cells in colostrum.negative organisms.105 human milk macrophages per milliliter of milk. Human milk leukocytes • o Macrophages comprise 40 . these 2 fatty acids may be considered essential fatty acids. D. A. These factors enhance the colonization of some bacteria while inhibiting the colonization by others. which enhances sIgA bactericidal activity against gram . The unique blend of fatty acids in the breast milk has been linked to the development of innate & adaptive immune regulation. thus making it unavailable to pathogenic bacteria. Ingested milk passively immunizes the neonate. but secretory immunoglobulin A (sIgA) is the most abundant. Lysozyme. G.stimulating factor (G .CSF) are also identified in human milk. Rather than producing better vision or greater intelligence. temperature & osmolality.10 days postpartum. breast milk may protects the developing neonatal brain from injury or less optimal development by providing necessary building materials & growth factors that act synergistically rather than in isolation. Oligosaccharides. Other immunologic properties of human milk • o In addition to antibodies.

Low fat. Breast fed babies have better visual development. Fat & sugar content increases. 190 Natural contraception due to lactational amenorrhoea. high lactose. Low acidic pH Hygienic as passes straight into infant’s mouth. probably from heart diseases. proteins & sodium as needed by the pre term baby. High mineral content. right temp. GIT diseases. Satisfies thirst of the baby. ADEK Low vit B. 2) Transitional milk • • • • • 2) Secreted 2 wks after delivery. ovarian cancer & osteoporosis is decreased. Incidence of breast cancer. According to phase of feeding a) Fore milk Secreted at start of the feed. Benefits of breast feeding 1) To baby • • • • • • Higher protein content – (IgA coats immature) Low carbohydrate & fat content. To society • • Higher child survival decreases Healthcare load by preventing illness & allergies. Changes in composition & quantity. 4) Pre – term milk It is a viscous. high concentration of fat. obesity) Prevent allergies Develops emotional bonding & security Free fatty acids promote brain growth & source of energy. protein. HTN in adulthood. High concentration of vit. It also changes during feeds to meet the thirst & hunger of the baby. vitamins. provides energy Satisfies hunger of the baby. • • • • • Advantages Antibodies & cells help in immune mechanism. yellow colored secretion that is secreted for about 3 – 5 days after birth. Composition Milk produced by pre maturely delivered mother More calories. Cells like polymorphonuclears.The content of nutrients within human milk changes over time as the recipient infant matures. Protects against illnesses (otitis media. macrophages & T & B lymphocytes in high concentration. Decreases mother workload by saving time & energy. vit C & potassium. Protein & immuno globin content decreases. Burning off unwanted fat & thus useful for cosmetic purpose. respiratory. mineral & water content. Decrease the chance of infant botulinium. Hence should be never discarded. Easily digestible & well absorbed. 1) Colostrum b) Hind milk – Secreted towards the end of feed Rich in fat. . cognition & IQ score in later life. Thinner & watery. To mother • • • • • 3) It follows transitional milk. 3) Mature milk Complete food Available in right quality & right time. Helps in uterine involution as oxytocin produced for milk production also contracts the uterus & prevents PPH. Laxative action due to large fat globules helps in passage of meconium & reduces the risk of jaundice.

being of the breastfeeding infant. Genetic factors A) 63 Growth & development Growth . while in under . Maternal medications – thalidomide leads to phocomelia Maternal irradiation during pregnancy causes severe fetal damage & anomalies. etc. environment & nutrition. promote the growth & well . Placental & uterine disorders like insufficiency.nourished babies development can be delayed. in concert. commercial formula clearly cannot replicate all of the valuable properties that are inherent in human milk. Development . This can be assessed from just watching the child & getting them to do stuff. Maternal endocrinal diseases like DM leads to LGA babies & other congenital anomalies. in the art of compounding a nutritious fluid for infants. With each of these measurements. Multiple pregnancies is associated with increased morbidity & mortality.” With the ever . polyhydramnios. in addition to its numerous nutrients that make it an ideal food source for the growing term infant. – galactosemia. • Conclusion Human milk. Maternal infections like TORCHS. as a child grows it will have a height. It is dependent upon maturation & myelination of the nervous system. chicken pox.expanding knowledge resulting from current research.It is increase in size or mass of tissues which occurs due to • • • • • • • • B) 1) multiplication of cells leading to quantitative or physical maturation. There is no reason why an infant getting positive emotional support & protective environment should not develop normally. Good nutrition also helps in proper development of brain & its functional aspect. . mucopolysaccharoidoses) Environmental factors Prenatal causes Basically. Physiological development • • • • • • • • 191 The key is to check that the child has reached the appropriate milestones for its age. Oliver Wendell Holmes said it best when he stated. Maternal stress adversely affects the foetus.• Helps in population control by natural contraception. Factors affecting growth & development Maternal malnutrition is related to IUGR &LBW babies.g. a weight.It is maturation of functions or qualitative growth leading to mental maturation. Good nutrition & healthy environment helps in normal development. syphilis & HIV. “A pair of substantial mammary glands has the advantage over the two hemispheres of the most learned professor’s brain. Physical Development (growth) Genetic makeup (genotype) Physical expression (phenotype) parenteral characteristics Race Sex Biorhythm & maturation Genetic disorders (anomalies like trisomy21. This bioactive fluid contains numerous factors & live cells that. • Infant’s development is influenced by genetic factors. it is referred to as failure to thrive. APH can adversely affect the foetus. If a child is under growing. turner syndrome) Genetic mutations like metabolic defects (e. trisomy 13. is a bioactive fluid that evolves from colostrum to mature milk as the infant matures. head circumference. there is a normal range of growth & then an abnormal deviance from this.

walk etc. Stages of growth 1) 2) 3) It is a continuous & orderly process. breast feeding & good weaning practices. Second spurt occurs in adolescence. 6) Certain primitive reflexes like grasp reflex & walking reflexes must be lost before achievement of corresponding voluntary movements. osteogenesis imperfecta. In each individual it has a unique pattern. supplementary feeding. • Lymphoid growth – Especially tonsils. ???? ???????. 8) Delay in achieving single milestone is called isolated delay & it is of not much significance. • Gonadal growth – Noted around puberty.3 yrs of life & then slows. Almost 90% is completed at two years of age. 7) Generalized mass activity of infants is replaced by specific responses e.e. 10) In case of preterm babies corrected age (conceptional age) = chronological age – period of prematurity. ?? 6/12 ???? ??? – growth occurs as time passes. – first 4 wks (28) days . ???? ???????? ??? – (factors enhancing body elements) ???? – ???? ??? : ?????? ???????? :???? ????????. Age of father – as paternal age advances it is associated with achondropoasia. should be considered up to infancy. General pattern is cephalocaudal i. craniostenosis. – on seeing a toy an infant shows interest by moving arms. ?? ?????? ??? – ???? – ???? ?????? ???? ????. crawl. Physical factors like climate & pollution Biological factors – infections lower the growth velocity. lymph nodes growth is prominent at mid .childhood (4 – 8yrs) as they act as organs of immunity in children. anxiety & insecurity affect growth. stand. SGA babies have grave prognosis than preterm. ???? ?????? ???? ?. 1) • • • 2) 3) • 192 Prenatal Ovum Embryo Foetus Perinatal Postnatal Newborn – – – – 0 – 14 days 14 days to 9 wks 9 wks until birth. mother child interaction. ???? ???????? – nutritional factors like maternal nutrition. ? Laws of growth & development 4) Different tissues grow at different rates. Exercise Accidents Emotional factors – mother infant bonding. – reflexes like asymmetric tonic neck reflex should be lost before voluntary movements like rolling over can be achieved. sit.2) Postnatal causes • • • • • • • • • • Neonatal hypoxia leads to brain damage. legs & trunk. 5) Development is related to maturation of nervous system. 28 wks of gestation to 7 days after birth. – It is maximum in later pregnancy & in first • Brain growth two years of life. ?????? ?????? : ??? ?????????????????. ?????? – physical & biological factors.g. • Somatic growth – very fast in first 2 . while older child merely smiles & opts for the object. ????????????!! ?. 9) Global delay or gross development delay is highly pathological. Availability of good nutrition. breast feeding Age of mother – as maternal age advances there is greater incidence of anomalies like Down’s syndrome. head control is achieved first which is followed by ability to grasp. e. ?????? ???????? – natural instinct of growing/genetic coding for growth.g.

In neonates & infants – detectometer (beam type) In older children – spring machines or bathroom scales. 400gm/mth up to 1 year of age. then 1cm/mth for next 3mts. hearing defects. . Regaining of wt. birth trauma like fractures.• • • • • First 7 days – early neonate Infancy – first year of life Toddler – 1 – 3 yrs Pre . dislocations. congenital anomalies like dislocations etc.5 kg age * 2 + 8 age × 3 or age × 7/2 – 5 age in mts. neurological disorders. early diagnosis can be made of moderate to severe mental disorders. Electronic scales or dial weight machines. + 9/2 • • • • • • • Length for babies up to 2 yrs with infantometer Above 2 yrs – height is measured by stadiometer. in first few days of life due to loss of edema & fluid.5 – 3.5 1 to 6 yrs > To 12 yrs Up to 1 yr b) Height – – – – 3. weight. starts by 10th day of life. body measurements 2) Skeletal maturation 3) Growth charts 4) Percentile charts 5) Growth velocity 6) Predicted ht. Importance of study of growth It is an indicator of general indicator of general health & nutrition 2) Screening in public health 3) Clinically. in child based on mid parenteral height (MPH) 1) Anthropometry – it includes height. head circumference. then 0.25 – 30 gm/day for 1st 3 mts. At birth – 50 cm 1 yr – 75 cm 2 yr – 87 cm 3 yr – 95 cm 4 yr – 100 cm 12 yr – 150 cm Head circumference • • • It should be recorded with child unclothed. Assessment of growth – it can be assessed by following means.14 yrs (girls) 14 – 16 yrs (boys) Post pubescent – 14 – 18 yrs (girls) 16 – 20 yrs (boys) 2 yr – quadriples the BW (12) 3 yr – five times the BW (15) 5 yr – BW * 6 (18) 10 yrs – BW *10 (30) Newborn loses about 10% of birth wt. At birth – 2. Formulae for calculating height – After 2 yrs (2 – 12) yrs = age × + 77 Birth length doubles by 4 yrs (100) Birth length triples by 12 yrs (149) Thereafter 5cm/yr up to 10 yrs.school – 3 – 6 yrs School age – 6 to 10 yrs (girls) 6 to 12 yrs (boys) Adolescence Prepubescent – 10 – 12 yrs (girls) 12 – 14 yrs (boys) Pubescent – 12 . 1) Anthropometry i.e. It is measured with help of non stretchable. plastic tape encircling the prominent areas of forehead & occiput. then.. Normal at birth – 33 – 35 cm. Then increases 2 cm/month for first 3 mts. Formula for calculating wt.5 kg 5 mts – double of birthweight (6) 1 yr – triple of birthweight (9) 193 c) • • • • It is a very important measurement in infants & children.5 cm/month for next 6 mts. cerebral palsy. chest circumference & mid arm circumference (MAC) a) Weight 1) At birth 2.

It is constant between 1 – 4 yrs of age (about 13 to 17 cm) in healthy children. These are also known as ‘road to health’ charts or ‘passport to child health When the anthropometric measurements of a large number of child populations of normal health are arranged in ascending order. birth date. length in cm /2 + 9. hypothyroidism. capitates & hamate at 5 . cuboid appear. Head of humerus is present by 1 month. severe malnutrition & constitutional delay It is advanced in precocious puberty. Then it exceeds the head circumference. H/O sickness etc. Skeletal maturation 1) 2) 3) 4) 5) 4) • • • • • • • 3) In full term newborn babies. Planning & policy making – by grading malnutrition it is possible to make a plan in relation to child health Other information – identification. They are useful for growth monitoring. measurements of a child are plotted on the growth chart & any deviation from the normal pattern can be visualized & interpreted. . midway between the olecranon process & the acromion.2. we will get a bell shaped curve. Mid arm circumference Types of growth charts Uses of growth charts e) • • 2) It is measured with help of non stretchable plastic tape.6 mt. It is lower than head circumference at birth. Definition – it is a visible graphical display of child’s physical growth & development useful to monitor growth.3% (app. of India & IAP charts 4) UNICEF rainbow charts • • It is measured at the level of nipples midway between inspiration & expiration.5cm) • • Chest circumference • care’. Equals with head circumference at 1 yr of age. 1) 3) WHO charts ICDS charts 2) Govt. The wt. five ossification centers namely – lower end of femur. calcaneus. malnutrition. Carpal bones viz. A flat curve & downward curve is not desirable. upper end of tibia & three tarsal bones talus. Percentile charts • • • • • • • 194 • These were first designed by David Morley. Growth charts Growth monitoring Diagnostic tool to identify ‘high risk’ children. The ossification centers appear first on left side of the body & then on the right side of the body. immunization history. This is called the ‘Gaussian Distribution’ or bell shaped curve. An upward curve in ‘road to health’ is desirable.Birth 1yr 2yr 5yrs 10 yrs 12 yrs Up to 1 yr d) – – – – – – – 35cm 45cm 48cm 49cm 50cm 52cm. 68. Education tool – with the help of visual/color charts even mother can be educated to monitor growth. The curve will be symmetrical on both sides & most of the observations will fall around the centre of the curve. are available. weight.2/3) of observations lie within 1 SD.5 (+/ . Bone age is delayed in hypopituitarism. Median indicates that 50% of observations lie above & 50% lie below this point. Head of femur by 4 – 6 mts. Median is 50% value (average/mean) (standard value) SD is the degree of dispersion of the observations away from the mean.

95. 2) Denver development screening test – (DDST) gross motor.4% of observations lie within 2 SD around mean. raises head 45 degrees 6 . sit & stand against gravity. Development after the baby is born is another captivating journey that all children traverse to become an adult.Limbs flexed .5 kg (or more) is a very fascinating one. Newborn . It signifies the control of body. + maternal ht. The story of transformation of a single cell in the womb (few micrometers) to a newborn that is 50 centimeters long.Sits without support. Milestones are changes in specific physical & mental abilities (such as walking & understanding language) that mark the end of one developmental period & the beginning of another.2 SD.)/2 . 1 SD = observations between 16th (1 SD below mean) & 84th (1 • SD above mean) percentile.5 cm in second year 6 cm/yr till puberty.8 weeks . MPH • • • 6) • • MPH (cm) for boys = (paternal ht. fine motor & gross motor areas. parents & everything in immediate environment & then he has to learn fine functions like holding. vision. 1 kg/yr in pre . However.13 Assessment of development Largely based on works by Arnold Gessel. language. It is all very challenging since the child has to learn to move. 99.8 months . milestones indicate a stage transition.born • • • It is the weight gain or height gain over a unit period of time. social & linguistic 3) The Woodside system refers to social. velocity – 6 kg in first year. For stage theories. It is better indicator of growth. This development is not only physical but also includes growth of these elements – fine motor. cognitive & social. + maternal ht. there is considerable variation in the achievement of milestones. Height velocity – 25 cm in first year 12. language.)/2 +13 MPH (cm) for girls = (paternal ht. 1) Nancey Baley scale of development – it measures motor & mental maturation. He has to accustom his eyes to look & recognize objects. In premature babies corrected age should be used. weighing around 2.Whilst lying on stomach. writing & eating etc. Values beyond 2 SD are unusual. even between children with developmental trajectories within the normal range.Head lag on pulling the child up to the sitting position 6 . Developmental assessment can be done by any of the following set of tests – 1) The Denver developmental screening test (DDST) 2) Bayley scale of infant development 3) Gessel development schedule (GDS) 4) Baroda developmental screening test (BDST) 195 • Gross motor • • • .school child 2 kg/yr up to puberty. with a round back at 6 months & with a straight back at 8 months Physical development of newly .7% fall beyond 3SD Allowable normal range of variation in observations is • conventionally taken between 3rd & 97th percentile curve or mean +/ .Symmetrical posture . fine motor. 5) Growth velocity • • • 5) 6) Trivandrum developmental screening test (TDSC) Developmental observation card (DOC) Developmental quotient (DQ) = developmental age/chronological age × 100 • • • Developmental age is the average of the motor & mental performances. Wt.

4 word sentences.base gait 15 months .Copies a double lined cross 9 yrs – copies a cylinder 11 yrs – copies a cube.Crawling 10 months . responds to no.Steps made from building blocks (after demonstration) .Tower of 6 blocks.grips object in palm 10 months .L .Follows face in midline (but does not turn head) 6 weeks . • • • • • • • • • • • • • • • • • • • • • • • • • • • • • Newborn . toys 6 months . 10 months – speaks one word with meaning.copies a hexagon/diamond 7 yrs . Able to draw a circle. 6 years – knows right from left. hand – eye coordination.follows moving object by turning head 12 wks – grasp reflex disappears. puts food in mouth. Speech.Uses two or more words to make a simple phrase. imitation. 12 months .Train with four bricks .Palmar grasp .Separation anxiety. 2½ .g.Able to draw a cross/rectangle 196 1 month – responds to sound of bell/rattle 3 months . 2 years .knows age & sex.Transfers objects from one to the other .Can point to four parts of the body 20 . 4 wks – looks eagerly at face of mother or examiner 8 weeks – social smile.• • • • • • • • • 8 .Able to draw a triangle or multiplication sign 6 years . broad . 7 months .Turns to soft sounds out of sight 9 months – responds to name.2 to 3 words other than “dada”/”mama” 15 to 18 months – child jargon. cooing (“aah .Walks steadily 18 months – creeps upstairs & downstairs 2 yrs – climbing stairs. running walks backwards 3 yrs – rides tricycle 4 yrs – goes downstairs.24 months . 8 months . Social • • • • • .Able to draw a square 5 years . 2½ years .3 years . skips on one foot 6 yrs – skips on both feet • • • • • • • 4½ years .shape with the long side on the floor 3 years – can build a tower of 9 blocks.10 words . watch movement of own hands (hand regard) 4 months .Pincer grip .Vocalizes alone.Reaches for objects e.aah”) 6 months – babbling da –da — ma – ma. 4 years – can tell a story.copies a kite 8 yrs .Startles at loud noises • Fine motor & vision This includes eye coordination.Tower of eight building blocks OR . Smiles responsively 3 months – recognizes mother 6 months – smiles at mirror image.Fix & follow . Names atleast 4 colors. 5 years – can speak atleast 10 word sentence. can draw horizontal or vertical lines Handedness is established at around 2 yrs. when spoken too.Walks around with support from furniture 12 months .can grip objects between a single finger & thumb 13 months – can turn 2 pages of book 14 months . Talks constantly in 3 .Tower of 3 cubic building blocks.Scribbles with a pencil on a piece of paper 15 months – can drink with a cup 18 months . 18 months . laughs. hand mouth coordination & hand skills.9 months . language & hearing Newborn . can dress & undress himself 4 years . can tell experience of recent events.Walks unsteadily.

dresses without supervision. The child is asked to draw a man. plays peek .Waves bye . IQ above 85 is considered normal. Intelligence test can be employed in children above 3 yrs of age. 12 months – pulls clothes to attract mother 18 months . learn how to learn. 5) 6) Good enough draw a man test for Indian children Piaget’s theory of cognitive function IQ test is generally done in dyslexia or suspected MR. understand & reason properly. 4 years – plays with other children 5 years – domestic plays.. 2 years . memory. receptive capacity & ability of expression through language.boo. mimics others. The basic age of three years is required for this test Draw a man test • • Following important milestones should be achieved at age limits indicated below • • • • • • • • Social smile Head holding Sitting with support Sitting without support Standing Walking – – – – – – 2 ½ months 3 ½ – 4 months 7 – 8 months 9 months 1 year 1 ½ year. imaginative plays with doll. Toilet training begins. These tests include several brain functions including hearing. Intelligence tests commonly used are Stanford Binet intelligence scale Binet Kamat test Wechsler intelligence scale for children (WISC) Malin intelligence scale for children (MISC) 197 Intelligence quotient (IQ) – mental age/chronological age × 100 • • 1) 2) 3) 4) . Assessment of intelligence • • It is the ability to know.Holds a spoon & gets food safely to mouth Symbolic play.• • • • • • • 10 months . Intelligence learning is not teaching or reading at early period but is motivating the child to find pleasure in learning. 3 years .Takes turns.Dry by day – wears simple garments/shoes/socks.Interactive play .a .bye. vision.

then collapses with a bump Drops toys & Cooperates with watches where they go dressing. Sits unsupported.Overview of motor. to sound 5 months 198 6 months Double syllable Localizes sound sounds such as 45 cm lateral to ‘mumum’ & ‘dada’ either ear May show ‘stranger shyness’ Transfers objects from one hand to the other. Turns head round Discriminates smile. No grasp reflex Holds head steady. waves goodbye. appropriately. Babbles 2 or 3 Stands alone for a words repeatedly second or two. speech. Pulls self up to sit & sits erect with supports. Picks up objects with pincer grasp 1 year Babbles tunefully Looks for toys dropped Apprehensive about strangers Stands holding furniture. Objects taken to mouth Enjoys vocal play Makes vowel noises Hand regard : following Serves to practice the hand with the eyes emerging visual skills Also observed in blind children. Palmar grasp of cube 9–10 months Wiggles & crawls. Goes for objects & gets them. vision & hearing development Developmental Milestones Age Motor Speech Vision & hearing Additional Notes 4–6 weeks Smiles at parent 6–8 weeks Vocalizes 12–20 weeks 3 months Prone : head held up for prolonged periods. Rolls over prone to supine. Follows dangling toy Squeals with delight from side to side. understands simple commands .

pulse/ min.18 months Can walk alone.3 cm (0. Attends to own toilet needs 6 years Questioning at its height.foot per step & downstairs 2 feet per step. Imitates gate with cubes. toy without falling over. Knows right from left & number of fingers Dresses & undresses with assistance.5°C 25 to 50 2. 2 years 3 years Able to run.51 in) per month . skips on one foot. Feeds self with a spoon. Can build a tower of 3 or 4 cubes & throw a ball Joins 2–3 words in sentences Parallel play. Begins to jump with both feet. temp. Speaks in sentences. Many Gets up/down stairs intelligible words holding onto rail. Undresses with assistance.8–18 lb) (doubling birth weight) 100–200 g per week 500 g per month 30 to 40 35. Walks up & down stairs 2 feet per step. Builds tower of 6 cubes Goes up stairs 1 . Most children with autism are diagnosed at this age. Copies circle. Builds tower of 9 cubes Constantly asks questions. imitates cross & draws man on request. copies a cross Copies a diamond.98 in) per month 1. Drinks from a cup with both hands. Cooperative play.5 cm (0.7– 37. Many infantile substitutions in speech Fluent speech Physical specifications Age Average Length/height (cm) 1–4 months 4–8 months Length growth Average weight Weight gain Respiration Normal Heart Rate body rate per min. Picks up ‘Jargon’. Dry by day Demands constant mothering. Visual acuity (Snellen chart) 50–70 cm (20–28 in) 70–75 cm (28–30 in) 4–8 kg (8. Imaginary companions 199 4 years Goes down stairs one foot per step.

by 1st birthday 9–13 kg (20–29 lb) 130–250 g per month 80 to 110 20/60 3 years 35– 37 °C 90 to 110 20/40 4 years 95–100 cm 5–8 cm (37–39 in) (2.0–3.5 cm (2.0–3.1 in) per year 12–15 kg (26–33 lb) about 4 times birth weight 13–17 kg (29–37 lb) 1.5–17 kg (32–37 lb) 1.4°C 110 20/30 200 5 years 105–115 cm (41–45 in) 17–21 kg (37–46 lb) 1.7– 37.6– 90 to 37.5 cm (40.6 in) per year 5–7 cm (2.4–2.8–2.8 in) per year 17–22 kg (37–49 lb) 2 kg per year .0–2.0–2.1 in) Nearly double per year birth length 101.6–114 cm 5–6.8–12 months Approx.5 °C (96– 100 °F) 20/100 12-24 months 2 years 80–90 cm (31–35 in) 85–95 cm (33–37 in) 1 kg per year 20 to 35 5–8 cm (2.6 in) per year 14.0–2.3 kg per year 20–30 90 to 110 20/20 > 5 years 105–120 cm (41–47 in) 5–6.0–45 in) (2.5 times birth length by 1st birthday 22 to 40 500 g per month 20 to 45 35.3 kg per year 20–30 36.3 kg per year 20 to 30 9.1.1 in) per year 7–13 cm (2.8–5.6 kg (21 lb) Nearly triple the birth wt.8–2.

Picks up objects using finger & thumb (pincer grip). Head circumference increases approximately 1 cm per month until six to seven months. Rooting & sucking reflexes are well developed.3 cm. Reflexive behaviors are changing : Blinking reflex is well established Sucking reflex becomes voluntary Moro reflex disappears When lowered suddenly. Holds hands in an open or semi . Posterior fontanel closes by the second month.5 cm per month until four months.open position. when baby is held in a prone (face down) position. Grasp reflex gradually disappears. with upper & lower incisors coming in first. biting & mouthing of objects. respiration rate depending on activity. then increases 1. grasps object using entire hand (palmar grasp). Teeth may begin to appear. later reaches with one hand or the other. bowing gradually disappears as infant grows older. Gums may become red & swollen. Reaches for objects with both arms simultaneously. shakes & pounds objects. early movements are large & jerky. Swallowing reflex appears & allows infant to move solid foods from front of mouth to the back for swallowing. Motor development • • • • • • . Muscle strength & control improving. rate & patterns vary from infant to infant.5 cm per month. Turns head side to side when in a supine (face up) position. 201 • • • • • • • • • • • • • • • • • • Motor development Head & chest circumferences are basically equal. Grasps with entire hand. Transfers objects from one hand to the other. the head is held upright & legs are fully extended. Legs may appear bowed. Landau reflex appears near the middle of this period. True eye color is established. Handles. indicating healthy. Skin remains sensitive & easily irritated. chewing. Increases are an important indication of continued brain growth. reaches for objects. head circumference should continue to increase steadily. 4–8 months Physical • • • • • • • • • • • Head & chest circumference are nearly equal to the part of the abdomen. Eyes begin moving together in unison (binocular vision). Able to hold bottle. Swallowing reflex & tongue movements are still immature. Legs may appear slightly bowed. gradually become smoother & more purposeful.Specifications sorted by reached age 1–4 months Physical • Upper body parts are more active : clasps hands above face. Breathing is abdominal. infant throws out arms as a protective measure. Head circumference increases approximately 2 cm per month until two months. puts everything in mouth. then 0. Continues to breathe using abdominal muscles. near the end of this period can hold head up & in line with the body. Anterior fontanel closes to approximately 1. continued drooling & inability to move food to the back of the mouth. ongoing brain growth. accompanied by increased drooling. Cries with tears. upper arms & neck. Raises head & upper body on arms when in a prone position. waves arms about. strength insufficient to hold items. Fat rolls (“Baby Fat”) appear on thighs.

activity & clothing still affect variations in body temperature. or 13 to 20 ft. moves around obstacles by side . toys & finger foods. holding head erect. therefore.• • • • • • • • Sits alone without support. but generally does not move forward. Creeps on hands & knees. weather. More teeth appear. transferring them from one hand to the other. objects & activities in the immediate environment. Reaches for toys that are out of reach but visible Recognizes objects in reverse Drops thing intentionally & repeats & watches object Imitates activities like playing drum Toddlers (12–24 months) Physical Motor development 202 • • Weight is now approximately 3 times the child’s birth weight. may be difficult to test informally. Legs may continue to appear bowed. Walks with adult support. also places objects inside one another. Shows awareness of distant objects (4 to 6 mt. Beginning to stand alone. Responds to hearing tests (voice localization). Arm & hands are more developed than feet & legs (cephalocaudal development). or 13 to 20 ft. Respiration rate varies with emotional state & activity. Uses deliberate pincer grasp to pick up small objects. rocks back & forth. . Releases objects or toys by dropping or throwing. • • • • • Reaches with one hand leading to grasp an offered object or toy.a . back straightened & arms propped forward for support Pulls self into a crawling position by raising up on arms & drawing knees up beneath the body. May accidentally begin scooting backwards when placed on stomach. Watches people. Follows simple instructions. Can see distant objects (4 to 6 mt. may begin to walk alone. Continues to use abdominal muscles for breathing. away) by pointing at them. leaning on furniture for support. Feet appear flat as arch has not yet fully developed. crawls up & down stairs. soon will begin to crawl forward. Manipulates objects. Head & chest circumference remain equal. can shift positions without falling.boo’ games Cannot understand “no” or “danger” • • • • • • • • • • • • • • 8–12 Months Physical • • • • • • • • • • • • Respiration rates vary with activity Environmental conditions. Explores new objects by poking with one finger. Stacks objects. often in the order of 2 lower incisors then 2 upper incisors followed by four more incisors & two lower molars but some babies may still be waiting for their first. away) & points at them. Looks for fallen objects by 7 months Plays ‘peek . Can roll over from back or stomach position. cannot intentionally put an object down. Has good balance when sitting. hands appear large in proportion to other body parts. however. holding onto adult’s hand. Beginning to pull self to a standing position. upper arms & neck. Anterior fontanel begins to close. Both eyes work in unison (true binocular coordination). Lifts head when placed on back.stepping. “Baby Fat” continues to appear on thighs. loses interest quickly and.

Tries to make mechanical objects work after watching someone else do so. Enjoys looking at picture books.like appearance. Manages three to four objects by setting an object aside (on lap or floor) when presented with a new toy. Crawls up stairs on all fours. Carries toys from place to place. places three geometric shapes in large form board or puzzle.heavy. places teacup on saucer & sips from cup. grows approximately 1. collapses backwards into a sitting position or falls forward on hands & then sits. frequent spills should be expected. tries to make doll stand up. anterior fontanel is nearly closed at eighteen months as bones of the skull thicken. Places several small items (blocks. Produces considerable “jargon” : puts words & sounds together into speech . Legs may still appear bowed. Rapid eruption of teeth. Later. the child always searches in the same location for a hidden object (if the child has watched the hiding of an object). Shows increasing understanding of spatial & form discrimination : puts all pegs in a pegboard.like (inflected) patterns. still appears top .hiding activities Early in this period. six to ten new teeth will appear. but cannot truly imitate facial expression. not always able to maneuver around obstacles.3 cm every six months. Uses furniture to lower self to floor. has difficulty stopping & usually just drops to the floor. Most children with autism are diagnosed at this age. Demonstrates understanding of functional relationships (objects that belong together) : Puts spoon in bowl & then uses spoon as if eating. not always accurate in getting utensils into mouth. Sits in a small chair. Responds with some facial movement. Body shape changes. takes on more adult . the child will search in several locations. Names many everyday objects. 203 • • • • Helps turn pages in book.an important neurological development). Repeatedly picks up objects & throws them. such as furniture or toys. Enjoys pushing or pulling toys while walking. back is swayed. Enjoys object . Chest circumference is larger than head circumference. Holophrastic speech : uses one word to convey an entire Cognitive development • • • • • Motor development • • • • • • • • • • • • • • • • • • • • • • Language . goes down stairs in same position. clothespins. Puts toys in mouth less often. Attempts to run. Shows or offers toy to another person to look at. abdomen protrudes. direction becomes more deliberate. cereal pieces) in a container or bottle & then dumps them out. Toddler will begin to lose the “Baby Fat” once he/she begins walking. Most children walk unassisted near the end of this period. Gets to feet unaided. Stacks two to six objects per day.arm movement. legs stiffened & arms extended for support. falls often. Helps feed self. Passes toy to other hand when offered a second object (referred to as “crossing the midline” . uses whole .• • • • • • • Rate of growth slows Head size increases slowly. enjoys holding spoon (often upside down) & drinking from a glass or cup. Enjoys crayons & markers for scribbling. Stands alone with feet spread apart. Crawls skillfully & quickly.

“Give Daddy the cup. wants to try doing things without help. produces two . Motor development . abdomen still large & protruding.g. Another important advancement is active social play with adults including mirroring & repeating. Psychological • • 2 year old Physical • • • • • • • • Social • • • • • • • • Posture is more erect. Shame & Doubt (will) Psychosocial stimulation is vital during the toddler years. ear).taking in other kinds of vocal exchanges. Enjoys adult attention. Helps pick up & put away toys. Brain reaches about 80 percent of its adult size. May have a tantrum when things go wrong or if overly tired or frustrated. little teapot.word phrases to express a complete thought (telegraphic speech) : “More cookie. Often imitates adult actions in play.” When asked. some turn .” Follows simple directions. Beginning to assert independence.” “cookie”.bye. Exceedingly curious about people & surroundings. often refuses to cooperate with daily routines that once were enjoyable. animals & toys. incy wincy spider. Identifies three body parts if someone names them : “Show me your nose (toe. Plays by themselves Enjoys being held & read to. tries to join in.) are a great way to encourage & stimulate this area of development. Songs. Enjoys the companionship of other children. Climbs stairs unassisted (but not with alternating feet). Autonomy vs. Acquires & uses five to fifty words. resists getting dressed. Toddlers begin to learn & exhibit independence. Respirations are slow & regular Body temperature continues to fluctuate with activity. such as making & imitating sounds. back swayed. Seems aware of reciprocal (back & forth) aspects of conversational exchanges. Recognizes self in mirror. Later. putting on shoes. verbal request is often accompanied by an insistent gesture. toddlers need to be watched carefully to prevent them from getting into unsafe situations. Enjoys rhymes & songs. taking a bath. etc. meaning depends on the inflection (“me” may be used to request more cookies or a desire to feed self). Less wary of strangers. Speech is 25 to 50 percent intelligible during this period. food & toys. Responds to simple questions with “yes” or “no” & appropriate head movement. eating.” “Daddy bye . emotional state & environment.• • • • • • • • • • • thought. to direct adult attention. rhymes & finger plays (e.” Indicates a few desired objects & activities by name : “Bye bye. Play begins to become interactive. will point to familiar persons. Uses gestures. gives hugs & kisses. because abdominal muscles are not yet fully developed. such as pointing or pulling. but ironically they enjoy sharing this discovery with others. but does not play 204 • • • cooperatively. typically these are words that refer to animals. likes to know that an adult is near. 15 baby teeth almost finished growing out Can walk around obstacles & walk more erect Squats for long periods while playing. Locates familiar objects on request (if child knows location of objects).

may pretend to pick something off the page & taste or smell it. Uses feet to propel wheeled riding toys. Stacks four to six objects on top of one another. most two . Opens doors by turning doorknobs. Begins to use objects for purposes other than intended (may push a block around as a boat). Enjoys pouring & filling activities . scribbles enthusiastically on large paper.” Repeatedly asks. where the dog has gone.year . notes their absence. Does simple classification tasks based on one dimension (separates toy dinosaurs from toy cars). the child will indicate readiness for toilet training. in other words.year olds understand significantly more than they can talk about. Throws large ball underhand without losing balance. seems fascinated by. Recognizes & expresses pain & its location. figuring out a situation : where the tennis ball has rolled. take them apart.selected activities for longer periods of time. can put objects together. Is able to verbalize needs. Discovering cause & effect : squeezing the cat makes her scratch. Uses fifty to three hundred different words. water. (This is what Piaget termed object permanence. such as believing that a toy bear is a real bear. Eye–hand movements better coordinated. turns around & sits down. “What’s that?” Uses some plurals.” Expresses negative statements by tacking on a negative word such as “no” or “not” : “Not more milk. turning pages. finds a hidden object by looking in last hiding place first. much of a two . Knows where familiar persons should be.bye”. Holds cup or glass (be sure it is unbreakable) in one hand. has no trouble verbalizing “mine. which usually occurs during the sensorimotor stage of Piaget’s childhood theory of 205 • • • cognitive development) Names objects in picture books. Speech is as much as 65 to 70 percent intelligible. Climbs up on chair.• • • • • • • • • • • • • Balances on one foot (for a few moments). vocabulary continuously increasing. unzips large zippers. Some stammering & other dysfluencies are common. Has broken the linguistic code. Realizes that language is effective for getting others to respond to needs & preferences. but may fall. uses conventional word order to form more complete sentences. fit large pegs into pegboard. Enjoys being read to if allowed to participate by pointing. Receptive language is more developed than expressive language. Refers to self as “me” or sometimes “I” rather than by name : “Me go bye . Stares for long moments. Often achieves toilet training during this year (depending on child’s physical & neurological development) although accidents should still be expected. tells about objects & events not immediately present (this is both a cognitive & linguistic advance). appears to sometimes be overly affectionate in offering hugs & kisses to children Language • • • • • Cognitive • • • • • • • • • • • Social & emotional . jumps up & down. styrofoam peanuts.old’s talk has meaning to him or her. Unbuttons large buttons. Grasps large crayon with fist. Shows signs of empathy & caring : comforts another child if hurt or frightened.& four . Utters three .word statements. making relevant noises.sand. or engrossed in. what has caused a particular noise. Is expected to use magical thinking. Attends to self .

Needs minimal assistance eating. not in a fist as earlier. Circumference of head & chest is equal. Catches a large bounced ball with both arms extended. Physical aggression usually lessens as verbal skills improve. but seldom interacts directly. Builds a tower of eight or more blocks.kneed. Manipulates large buttons & zippers on clothing. Enjoys playing with clay. wants it both ways. routines carried out exactly as before. “Bossy” with parents & caregivers.shaped objects. using alternating feet. may jump from bottom step. Can walk on one foot. uses vertical.• • • • • • • • • • Continues to use physical aggression if frustrated or angry (for some children. May begin to show hand dominance. Listens attentively to age . pours liquid from pitcher into another container. Jumps on the spot. finds it difficult to wait or take turns. 300 J (1.appropriate stories. abdomen no longer protrudes. plays near others. belongings placed “where they belong. wants everything “just so”. can jump from low step can stand up & walk around on tiptoes 206 • • • • • • Cognitive development . rolls & squeezes it. 57%. still tends to hoard toys. Can kick big ball . Legs grow faster than arms. brushes own teeth. Holds crayon or marker between first two fingers & thumb (tripod grasp). Enjoys swinging on a swing (not too high or too fast). this is more exaggerated than for others). Watches & imitates the play of other children. Posture is more erect. Enjoys “helping” with household chores. expects immediate compliance from adults. Can turn pages of a book one at a time Enjoys building with blocks. Making choices is difficult. Ritualistic. imitates everyday activities : may try to toilet a stuffed animal. makes demands. cannot be reasoned with while tantrum is in progress. shouting “no” becomes automatic. Shows improved control of crayons or markers. Impatient. Temper tantrums likely to peak during this year. “Baby fat” disappears as neck appears.” • • “baby” teeth stage over. Usually achieves complete bladder control during this time. 500 calories) daily. orders them around. Often defiant. Carries a container of liquid. Offers toys to other children. feed a doll. Slightly knock . but not thoroughly. landing on both feet. often choosing similar toys & activities (parallel play). Throws a ball overhand. Walks up & down stairs unassisted. Needs to consume approximately 6. but is usually possessive of playthings. head size is in better proportion to the body. horizontal & circular strokes. balance momentarily. Pedals a small tricycle. Washes & dries hands. Adult height can be predicted from measurements of height at three years of age. males are approximately 53% of their adult height & females. especially those that relate to home & family events. Makes relevant comments during stories. without much spilling. aim & distance are limited. Likes to look at books & may pretend to “read” to others or Motor development • • • • • • • • • • • • • • • • • 3 year old Physical • • • • • • • • • Growth is steady though slower than in first two years. solitary play is often simple & repetitive. pounds. such as a cup of milk or bowl of water.

Hops on one foot. distance & aim improving. Understands the concepts of “tallest. Enjoys stories with riddles.• • • • • • explain pictures. Jumps over objects 12 to 15 cm (5 to 6 in) high.” “in. Becomes more accurate at hitting nails & pegs with hammer. Throws a ball overhand. 207 Motor Development • • • • • • • • • • • • • • • • • • Language • • • • . Walks a straight line (tape or chalk line on the floor).” & “under.” “baby’s.” “biggest.” Answers “Whose?”. selects the picture that has the “most houses” or the “biggest dogs.” Rote counts to 20 or more. such as alphabet books with only a few words per page & many pictures. Hearing acuity can be assessed by child’s correct usage of sounds & Language also.” “same. Can run in a circle Can recognize that certain words sound similar Names eighteen to twenty uppercase letters. starts. Delights in wordplay. avoids obstacles & oncoming “traffic. Indicates negatives by inserting “no” or “not” before a simple noun or verb phrase : “Not baby. “What is this?” & “Where?” questions dealing with familiar objects & events. often puts “ . turns corners. 700 calories daily.” Uses the prepositions “on. “Why?” & “How many?” Produces elaborate sentence structures : “The cat ran under Cognitive 4 year old Physical Development • • • Head circumference is usually not measured after age three. but often has problems implementing it so calls the creation something else. stops & moves around obstacles with ease.” Speech is understandable most of the time.” Answers “What are you doing?”. Builds a tower with ten or more blocks. Understands the sequence of daily events : “When we get up in the morning.” When looking at pictures can recognize & identify missing puzzle parts (of person. Forms shapes & objects out of clay : cookies.” & “more”. brown dog. may have an idea in mind. Likes stories about how things grow & how things operate. brush our teeth & go to school. Writes several letters & sometimes their name. uses “ . Runs.s” to indicate more than one. trees. Holds a crayon or marker using a tripod grasp. lands with both feet together. A few children are beginning to read simple books.” Uses possessives consistently : “hers. have breakfast. playground equipment. Produces expanded noun phrases : “big. creating silly Language. Requires approximately 1. snakes. • • • • • • • • • • Paints & draws with purpose. “Who?”. by the child’s appropriate responses to questions & instructions. Very good storytellers. but not always in order follows two to three step directions given individually or in a group May put the “ed” on the end of words such as “I goed outside & I played. guessing & “suspense. simple animals. Reproduces some shapes & letters. Threads small wooden beads on a string. Counts 1 to 7 objects out loud.” Climbs ladders.” Produces verbs with “ing” endings.s” on already pluralized forms : geeses. Pedals & steers a wheeled toy with confidence. car & animal). we get dressed.” “theirs. mices.

” Refers to activities. Builds three . Walks a balance beam.” “Daddy went to work. “Did the baby drink all of his milk?” States first & last name. not always able to take turns or to understand taking turns under some conditions. Walks unassisted up & down stairs. usually with training wheels. H. paper airplane that will not fold right.3 ft) away. Imaginary playmates or companions are common. overly enthusiastic at times. tattles on other children. Changes tone of voice & sentence structure to adapt to listener’s level of under . objects & people that are not present. Requires approximately 7. triangle. I. events. Walks backwards. 5 year old Physical • • • • • • • • • • • • • Head size is approximately that of an adult’s. Balances on either foot with good control for ten seconds. gender. tests the limits with “bathroom” talk. Jumps or hops forward ten times in a row without falling. laughing one minute. holds conversations & shares strong emotions with this invisible friend. friendly. toe to heel. but may get so frustrated as to verge on tantrums when problems arise : paint that drips.standing : To baby brother. Rides a tricycle or wheeled toy with speed & skillful steering. L. Enjoys role . 500 J (1. crying the next. Body is adult . or hungry. some children learning to ride bicycles. Answers appropriately when asked what to do if tired. Begins to correctly use the past tense of verbs : “Mommy closed the door. May begin to lose “baby” (deciduous) teeth. T. Cooperates with others. alternating feet. threatens : “You can’t come to my birthday party” 208 • • Name .dimensional structures with small cubes by copying from a picture or model. A. may throw tantrum over minor frustrations (a block structure that will not balance). cold.like in proportion. U. Establishes close relationships with playmates.believe activities.up stories or claims of boldness. Can touch toes without flexing knees. sulk over being left out. Cuts on the line with scissors (not perfectly).playing & make . May learn to turn somersaults (should be taught the right way in order to avoid injury). may yell angrily rather than hit to make a point. Learns to skip using alternative feet. C. Outgoing. Insists on trying to do things independently. may begin to color within the lines. “Milk gone?” To Mother. Relies (most of the time) on verbal rather than Physical aggression.calling & taunting are often used as ways of excluding other children. Reproduces many shapes & letters : square. Often appears selfish. O. Boasts exaggerates & “bends” the truth with made .• • • • • • the house before I could see what color it was. Demonstrates fair control of pencil or marker. Shows pride in accomplishments.” Speech is almost entirely intelligible. Recites & sings simple songs & rhymes. participates in group activities. beginning to have “best” friends. 800 calories) daily Visual tracking & binocular vision are well developed. Catches a ball thrown from 1 m (3. Hand dominance is fairly well established. siblings’ names & sometimes own telephone number. Moods change rapidly & unpredictably. Social development Motor development • • • • • • • • • • • • • • • • • . seeks frequent adult approval.

” Relates clock time to daily schedule : “Time to turn on TV when the little hand points to 5. second.” “swam. Asks innumerable questions : Why? What? Where? When? Eager to learn new things. places objects in order from shortest to tallest.” “washed. many children count to 100. last. same size. smallest to largest. shares toys. Recognizes numerals from 1 to 10. Identifies objects with specified serial position : first.tense inflection (. beginning to count & save money. Rote counts to 20 & above. Participates in group play & shared activities with other children. 209 • • • • • • • • • • • Tells a familiar story while looking at pictures in a book. Builds steps with set of small blocks. Boasts about accomplishments. Answers telephone appropriately. much longer sentences are not unusual. light & early : “I got up early. Produces sentences with five to seven words.& lowercase letters. Sorts objects on the basis of two dimensions. before anyone else. a bed is to sleep in. States the name of own city or town. takes turns. It was still dark.ed) appropriately to mark regular verbs : “jumped. Identifies & names four to eight colors. Understands concept of same shape. two o’clock. Knows what a calendar is for. Needs comfort & reassurance from adults but is less open to comfort. Uses “would” & “could” appropriately.” Uses past . calls person to phone or takes a brief message Speech is almost entirely intelligible. Many children know the alphabet & names of upper . is generous.” “rained.” Enjoys & often has one or two focus friendships. can say how many pieces an object has when it’s been cut in half.Cognitive • • • • • • • • • • • • • • • • • • • • Forms rectangle from two triangular cuts. birthday & parents’ names. suggests imaginative & elaborate play ideas. Sorts a variety of objects so that all things in the group have a single common feature (classification skill : all are food items or boats or animals). Uses past tense of irregular verbs consistently : “went. Vocabulary of 1.control over swings of emotions. Defines simple words by function : a ball is to bounce.” “caught. Understands the concept of half. Social development • • • • • • • • • Language development 6 year old Physical . Recognizes & identifies coins. such as color & form. Understands the concepts of less than : “Which bowl has less water?” Understands the terms dark.” Some children can tell time on the hour : five o’clock. Understands the concepts of smallest & shortest. Plays cooperatively (can lapse). makes up jokes & riddles. Likes entertaining people & making them laugh. Shows affection & caring towards others especially those “below” them or in pain Generally subservient to parent or caregiver requests. Recognizes the humor in simple jokes. 500 words plus. Has better self .

pencil mazes & games that involve matching letters & words with pictures. 210 Language • • • • • • • • • • • • • • • Motor development Can identify right & left hands fairly consistently.• • • • • • • • • • • • • • • • Weight gains reflect significant increases in muscle mass. expresses fear that parents may die.perceived failure can make the child easily disappointed & frustrated. counting & sorting activities. Recognizes some words by sight.& . Enjoys vigorous running. Can Tie Laces. Enthusiastic & inquisitive about surroundings & everyday events. may complain excessively about minor hurts to gain more attention. Making things is enjoyed. Often can’t view the world from another’s point of view Self . 700 J to 7. 000. t/f. The most common vision problem during middle childhood is myopia. Uses language rather than tantrums or physical aggression to express displeasure : “That’s mine! Give it back.solving situations (though the “logic” may be unclear to adults). movements are more precise & deliberate. reassurance & praise. though Can concentrate effort but not always consistently. (Berk. often. or nearsightedness. Understands time (today. Experiments with slang & profanity & finds it funny. word order & sentence structure. needs & seeks adult approval. attempts to sound out words In some cases the child may be reading well. Reverses or confuse certain letters : b/d. vocabulary of 10. yesterday) & simple motion (things go faster than others). Holds onto positive beliefs involving the unexplainable (magic or fantasy) Arrives at some understanding about death & dying. Span of attention increases. puzzles & mazes Enjoys the challenge of puzzles. or kick a ball. climbing & throwing est. Loves telling jokes & riddles. works at tasks for longer periods of time. Learns 5 to 10 words a day. if below 20/40 should see a professional. you dummy. Heart rate & respiratory rates are close to adults. g/q. p/g. Uses appropriate verb tenses. 700 calories) a day. 100 J (1. Folds & cuts paper into simple shapes. the humor is far from subtle. 000–14. Talks a lot. Has fun with problem solving & sorting activities like stacking. string (like shoes). Recognizes seasons & major activities done in the times. though some clumsiness persists. Has mood swings towards primary caregiver depending on the day Friendship with parent is less depended on but still needs closeness & nurturing. Able to trace objects. paper . Body may appear lanky as through period of rapid growth. Baby teeth beginning to be replaced by permanent ones. Gains greater control over large & fine motor skills.” Talks self through steps required in simple problem . 2007). values are based on others enforced values. tomorrow. swing a bat. Can’t handle things not going their own way Does not understand ethical behavior or moral standards especially when doing things that have not been given rules Understands when he or she has been thought to be “bad”. starting with the two upper front teeth 20/20 eyesight. jumping. Functioning which facilitates learning to ride a bicycle. 600 to 1. Uses 6. Social & emotional • • • • • • • • • • • • • . swim. Able to carry on adult .like conversations. asks many questions. Anxious to please. Has trouble staying still.

Vitamin C is important for the immune system & growth. for this is the crucial • time when the foundations for future good health are laid.the requirements • for energy & nutrients are high. The first few years are of vital importance.meat sources (non heme iron).• May be increasingly fearful of the unknown like things in the dark. especially iron from non .Vitamin A is needed for healthy skin & cell development & can be often be lacking in diets of this age group. Vitamin intakes are often low in children who eat little. • are high but dietary intake is often low. muscles & healthy tissues. 64 All parents wish their children to be healthy & there’s a great deal you can do to give your child the best start in life as well as good health in later years. Food & nutrients are the building blocks in a good diet. especially if little or no meat is eaten. Vitamin A. Vitamin D is essential for calcium metabolism (breakdown of 211 . Calcium .Iron deficiency is common in this age group as iron requirements calcium) & can be synthesized through the action of sunlight on the skin. • Diet to be “Nutrient & energy Dense” . A child’s diet needs special care & planning . which • help them to form strong teeth & bones. Particularly important nutrient include Iron .providing plenty of • nutrients in small volume of food. The diet therefore needs to be : Made up of small frequent meals.This is vital for the growth of bones & teeth. make sure that you include dietary sources of vitamin D. It also helps in the absorption of iron. but appetite is small & eating habits are likely to be finicky. D . Requirements will be met as long as the child consumes enough milk & dairy products. Foods rich in Vitamin C such as orange or tomato with evening meal eaten at the same time help maximize iron absorption. C. along with vitamin D in tablet or liquid form. A good diet can also help to protect against illness. In winter & if your child is always covered. noises & animals.

Key nutrients & dietary sources Nutrient Iron Important for Dietary sources Needed to make red blood cells & carry oxygen to the body as part of hemoglobin the blood.eggs. yam (suran). chapatti & rotlas. methi. rice. Dairy based products-milk. pulses & green leafy vegetables (cabbage. needed for bone & teeth growth & help us see in the dark Vitamin C Essential for structure of bones. other cereals & potatoes • • • • • • • • • • • • • Ragi porridge/cornflakes with milk Vermicelli cooked in milk Rice pudding. Plant (non heme) sources fortified cereals like cornflakes. tomatoes mango & apricots). blood clotting & nerve cells. Milk Cheese Bread. Calcium Essential for strong bones & teeth. Thepla. meat. Poha Pancakes (Made from rice flour & Gram flour (Chana Atta) Palak Puri. dark leafy vegetables & dairy products Helps the immune system & the absorption of iron Citrus fruits. Bombay duck & dairy products. custard Rawa or Sago kheer Bread pudding Cheese on Bread Cubes of Cheese Cheese on Vegetables Soup with grated Cheese Paneer in curries Add fresh fruit to Yoghurt Yoghurt with Sugar or Honey Yoghurt made into Lassi drink / Fruit Lassi • To serve as “Kadhi” Starchy staples form an important part of anyone’s diet. liver & kidney. 212 Tasty snacks Milk & dairy foods : 4 Major Food Groups Yoghurt (dahi) • • • • • Cornflakes Popcorn Toast. One can choose from variety of breads. spring onions. yoghurt (dahi) cheese. lamb. palak. Suitable foods are divided into Four Major Groups & a fifth Minor . dried fruits. Vitamin D Aids in bone & tooth formation & helps the heart & nervous system Yellow & orange fruits & vegetables (peppers. If child’s intake includes these groups it certain that all the important nutrients are provided. beans. bread. jowar. paneer Vitamin A Formation & maintenance of skin. Fishes like mackerel. bajra.Dairy based – Fish with soft edible bones. hair & membranes.or ‘occasional’ group. Meat based (heme) sources . Potato pattice Upma. They are also called as fillers. Bun. dark leafy vegetables. cornflakes. green beans) Non . maize. berries & vegetables (make sure you don’t cook too soft) potatoes & fruit juice. cartilages & muscles. sesame seeds (Til). It will provide energy for growth & calcium for strong bones & teeth. More substantial meals Aim for 500-600 ml of milk each day. They provide energy. various nutrients & fibre. potatoes. .

3 small nutritious snacks Offer variety of foods with some favorites • • Offer small portions & praise your child for eating even a little • Do not force feed • Family mealtimes • Happy. chocolates. they will decay. Peas & Soya Granules are good sources of proteins. Apple slices. If you are vegetarian. Dal or lentil curry with chapatti or rice. Eggs. chocolate & such foods as reward. wafers. give at least 2 portions of vegetable servings to ensure enough proteins & iron in the diet. Therefore avoid using foods like cakes. Or meat soup VEG. custard.It consists of – • • They contain lot of vitamins. • Use fruits in puddings 4) Meat. biscuits. porridge. Nut. Try & introduce lots of different types from early age. fish & veg. relaxed environment • Do not offer sweets or favorite foods as reward instead reward them with a trip to park. Omelet with bread or sandwiches. Refuses • Try mixing vegetables in other foods to eat • Include small amount of fruits or vegetables at . biscuits. Tomato Slices. ghee.containing foods like kheer. Mix chopped or mashed vegetables with rice or Dal. Beans. Fish. Meat & fish • • • • • • • • • • • Boiled Egg. homemade ice cream & lassi. omelet Sandwich made with shredded chicken Egg on toast Lentil soup Sprouts boiled & made into chat. sweets. zoo or taking out to museum Refuses to • Offer in small fun cup or through a colored straw drink milk • Add milk or cream (malai) to foods • Include milk . ALTERNATIVES Dal khichri Lentil soup 213 • Structured meal pattern3 meals & 2 . . minerals & fibre & also add variety to meals by adding coloring textures & flavors. Banana slices. Omelet with bread or sandwiches.• • • • • 3) Chapatti or Stuffed Paratha with Paneer or Dal or Potato Pulav or Khichri with Yoghurt Noodles or rice mixed with shredded omelet & vegetables Chapattis or Rotlas with vegetables or dal. cakes. • Try flavored milk or milkshakes. Children between this age group require around 25 gm of fat per day. Stuffed parathas with vegetables Spinach cutlets or Vegetable cutlets. alternatives Sugar : A small amount of sugar as mild sweetening is fine with foods in MILK Group. Fatty & sugary foods : This group includes butter. When teeth are in constant contact with sugary foods & drinks. oil sugar. Pulses. Cucumber • Slices. Lentils. soft drinks & other sugary drinks. • Theplas (Wheat flour mixed with Methi or Spinach) • Give Carrot sticks. Too much will impair the intake of more nutritious foods from the other food groups & compromise the quality of diet. Possible Solutions Some Common problems Problems Food refusal Protein is needed by young children to grow & develop. Meat.These foods should not be given too often & when they are. Fish curry with rice Mince meat cutlets. Fruits & vegetable group 5th group – the occasional foods group . only in small amounts. or Pineapple Slices as snacks. ice cream.

gum hardening & tooth eruption takes place which is an indication for introducing semisolid food. 8) Amylase enzyme appears in the gut which is necessary for digestion of starch. kanji) can be given. carrot. • Blend fruit drinks or milk shakes added fruit • To prepare kheer or porridge with added fruits Excessive • Limit milk intake to 500 .9 months. breast milk production is on its peak after which it starts to decrease in quantity. 9) Breast milk & cow’s milk is unable to provide enough energy & other nutrients to maintain growth velocity in infants after 4 mt. Cereal based gruel (khichidi. Weight of the baby doubles at about 5 mt. cauliflower. of age From about 4 . Weaning is a transition from breast milk or formula milk to solid foods. The baby is taken away gradually from the from the habit of sucking at the breast which is the second most step towards independent existence. of age. • 6) 7) Initial period : From 4 . By 6 mt. During 6 .6 months old. Hand & mouth coordination is established at about 5 . Weaning the baby from the breast or the bottle starts from about 4 . • Non fibrous vegetables e. of age hence most babies grow well on breast milk. potato. Vegetables & fruits : soft cooked vegetables & fruits are suitable • e.6 mt.complex. lauki • (bottle gourd. of age. • 9 . Pulses provide protein.g. banana.7 mt. Starchy foods : Initially cereal is used such as baby rice or sago • (sabudana). baby needs more iron & other nutrients like Vitamin D & Vitamin C that milk alone cannot 214 give. 10) Late weaning leads to growth failure & malnutrition. It is defined as “the systematic introduction of suitable food at right time with addiction to mother’s milk in order to provide nourishment to the infant. iron & B .fruits & each mealtime to allow opportunity to try vegetables • Children learn by example .12 months. C & provitamin A. It is divided into the following stages : Initial period . • Mashed. The first step is ‘cutting of the umbilical cord’. Head control is achieved at about 4 mt.g. mango & chickoo. : apple.600 ml per day milk • Give milk after meals or at snacks time drinking • Use cup not bottle· Offer milk in small cups 65 • • The word ‘weaning’ is derived from the word ‘wenian’ which means ‘to accustom’.Babies are usually ready to start on solid foods • between 4 . sweet potato.6 months for following reasons Breast milk provides essential nourishment up to 6 mt. pear.” It is a gradual process of replacement of breast feeding by introduction to a wide range of ‘non milk’ suitable food mainly semisolid foods.6 months. Fruit juices & mashed seasonal fruits can be given to provide • vit. dudhi) Remember 1) The aim is to get the baby used to taking food from a spoon Start with teaspoonful (quantities will be small) & milk will . 6 . yam (suran) can • be given.6 months • • • • 1) 2) 3) 4) 5) Baby should be still having 600ml of breast or infant formula milk daily. pureed spinach. Hence baby can be fed in upright position.if parents & other family members eat fruits & vegetable then they are more likely to do so.

wait & try again another day. mashed boiled sweet potato with carrots with cauliflower.2) 3) 4) still be major sources of nutrients) Foods should not be salted or sweetened.If the food doesn’t seem to be wanted.khichri (gruel of rice & pulses) – sooji halwa / porridge – nachni mixed with milk (porridge). Allow plenty of time for feeding which helps the infant & his bowel to get used. soft banana & pear.12 months Problems during weaning Toddlers (1 . Food items should be culturally accepted. potato. Soft cooked strips of carrot. Gradually increase amount of food to give either before or after milk feed. Porridge of rice or suji (rava) or nachni or dahlia or mashed banana. Energy dense food items like egg.feeding should be encouraged. In toddlers ‘physiological anorexia’ can lead to refusal of child to accept food. At this age growth velocity is decreased. Spinach & tomato soup. Dairy products : Cow’s milk may be used to mix solids. oil.3 yrs) 66 . Other cooked vegetables & fruits may now be coarser in texture. Introduce wheat based cereals . chewable foods should be introduced. Hard cheese used as finger foods. ghee should be used. exploring & interacting with environment. Cheese may be given as finger foods. aerophagy or abdominal colic. Whole cow’s milk may be now used as a drink. One type of food should be introduced at one time for about 7 days & then another type of food item should be introduced. banana. Rumination due to psychological disorder. It should be cheap & easily available. 215 • • • • From 6 . It should be started slowly with small quantities of food increasing gradually.9 months • • • • • For this reason feeding should be made interesting by combining it with playing. It should be easy to prepare at home. In infants lack of burping can lead to regurgitation. cooked green beans. To continue to use cow’s milk to mix with solids. To try the food after a milk feed or in the middle of one By this age the baby should be fed with most of the food cooked in the house. Food should not be salted or sweetened Solid. Baked fish can be given. Encourage lightly cooked or raw foods. Apple stew. Meat soup can be introduced. Methods of weaning • • • • • • • • • • • • • • • Type of weaning food • • • • • • • • • From 9 . Don’t force food on the baby . Weaning is bridge between liquid & solid diet. Introduce new foods mixed with familiar foods Time of the day when you both are relaxed. Self . Physiological anorexia may be seen due to loss of interest in eating & more interest in playing. Hence weaning should be introduced properly to avoid malnutrition. Over consciousness of parents can lead to overfeeding. Child may now take almost all that is cooked for regular meals at home. Unhygienic preparations can lead to infections. It should provide all necessary nutrients & energy.

It usually takes 14 days to one month for the child to develop immunity after the vaccination. Pneumococcal Polio (Salk). Herd immunity theory proposes that.e. it is more difficult to maintain a chain of infection when large numbers of a population are immune. Yellow fever Cholera Pertusis. Tetanus. – pulse polio vaccination). Vaccines Live Killed • F/b exposure to microbe. Both the terms mean process of giving vaccines to children so that they develop immunity or resistance against that particular disease. But. Diphtheria. Most of the vaccines give 80 . healthier is the community for that particular disease. Rabies. Antigen recognition by b & t cells. ?? 26/81 Natural Artificial Natural (active (passive immunization) immunization) Artificial Immunization is the process of making the body resistant to certain prevalent diseases & it is achieved by means of vaccination. leads to antibody secretion by plasma cells (B) & (T) CD4 cells &B&T memory cells. (active) Immunization . This is why incidence of measles. Higher the number of vaccinations. Vaccination or immunizations are the terms used • interchangeably. Hepatitis b. (Smallpox.It is a process of inducing immunity artificially or either by vaccination (active) or administration of antibody (passive). Any child acquiring infection during this period will not be protected. Japanese encephalitis • Natural acquired • Species Racial Individual Active Passive Natural Artificial Natural Artificial • 216 The success rate of vaccination program depends on the vaccination status of a community.g. the . antibodies & antitoxins Herd immunity (or community immunity) • • Bacterial Viral Types BCG.67 68 Vaccination ?????????????? ??? ?????????????????? ????????????? ???????????????? – ???????? ??????? ???????????????? ????????? ??????? ?????????????????? – ?. The higher the proportion of individuals who are immune. Vaccination . Typhoid Polio. Diphtheria. Pertussis & Poliomyelitis has been minimized to a great extent. MMR. TB) Antigens From mother introduced through by vaccination placenta stimulate cell IgG – from mediated & breast milk IgA antibody mediated response Intravenous immuno globulins i. in a community if all children are vaccinated the risk of transmission is reduced & thus incidence of disease becomes very low (e. in diseases passed from individual to individual.It is administration of any vaccine or toxoid for prevention of disease.100% protection. It describes a type of immunity that occurs when the vaccination of a portion of the population (or herd) provides protection to unprotected individuals.

then they are given immunoglobulin. Whenever a disease causing body strikes a vaccinated child. It does not apply to diseases such as tetanus (which is infectious. . Vaccination acts as a sort of or firewall in the spread of the disease. the efficacy of the vaccine & the contact parameter for the population. As of 2009. Complications arise when widespread vaccination is not possible or when vaccines are rejected by a part of the population. It is the general aim of those involved in public health to establish herd immunity in most populations. when young children are exposed to dangerous diseases for which they are not vaccinated. Unvaccinated individuals are indirectly protected by vaccinated individuals. herd immunity is compromised in some areas for some vaccine . No vaccine offers complete protection. It provides temporary protection through administration of exogenously produced antibody such as immunoglobulin & antibody It also occurs transplacentally – usually prior to infection. Passive • • • • • • . to help protect the fetus before & shortly after birth. it is considered best left for those who cannot safely receive vaccines because of a medical condition such as an immune disorder or for organ transplant recipients. as the latter will not contract & transmit the disease between infected & susceptible individuals. slowing or preventing further transmission of the disease to others. Sometimes. but is not contagious).preventable diseases. The proportion of immune individuals in a population above which a disease may no longer persist is the herd immunity threshold. Vaccines are antigens that provoke a response in the body to produce antibodies that fight infection & reserve it in the body. This type of immunity is called Active Immunity.made antibodies are given to a person. Since only a small fraction of the population (or herd) can be left unvaccinated for this method to be effective. 217 • Herd immunity should not be confused with contact immunity. but the spread of disease from person to person is much higher in those who remain unvaccinated. unvaccinated subgroup. Immunization Active • • • It includes stimulation of the immune system to produce antibodies (B cells) & cellular (T cells) immune response that protect against the infectious agents. the immune system (from that reserved stimuli) has the ability to protect body & hence does not allow infection to grow & cause disease. Passive immunization is where pre . Hence. a public health policy of herd immunity may be used to reduce spread of an illness & provide a level of protection to a vulnerable. where the vaccine protects only the vaccinated person from disease. Immunoglobulin are readymade antibodies given to protect the baby for sometime till they are vaccinated & their active immunity starts to work. including pertussis & measles & mumps. This is artificial passive immunization & is normally given by injection as a emergency treatment option.usually prior to infection These Antibodies developed by false infection then fights when real infection occurs. in part because of parental refusal of vaccination. Passive immunization is naturally acquired when antibodies are being transferred from mother to fetus during pregnancy. hence avoiding illness. Its value varies with the virulence of the disease.• • • • • • • lower the likelihood that a susceptible person will come into contact with an infectious individual. a related concept wherein a vaccinated individual can ‘pass on’ the vaccine to another individual through contact. Herd immunity only applies to diseases that are contagious.

Whenever disease causing bacteria/virus strikes us for the first time, we fall sick because our body is not prepared to deal with such infection. But slowly body learns to fight this infection by producing specific antibodies. These antibodies are the main defense - line of our body combating external forces. Therefore second time when such infection occurs, the body behaves smartly & uses antibodies (outcome of pervious infection) to fight it. Hence we don’t fall ill the second time.

Active immunization

Active immunization entails the introduction of a foreign molecule into the body, which causes the body itself to generate immunity against the target. This immunity comes from the T cells & the B cells with their antibodies. Active immunization can occur naturally when a person comes in contact with, for example, a microbe. If the person has not yet come into contact with the microbe & has no pre - made antibodies for defense (like in passive immunization), the person becomes immunized. The immune system will eventually create antibodies & other defenses against the microbe. The next time, the immune response against this microbe can be very efficient; this is the case in many of the childhood infections that a person only contracts once, but then is immune. Artificial active immunization is where the microbe, or parts of it, is injected into the person before they are able to take it in naturally. If whole microbes are used, they are pre - treated, attenuated vaccine.
Passive immunization

Passive immunization occurs physiologically, when antibodies are transferred from mother to fetus during pregnancy, to protect the fetus before & shortly after birth. Artificial passive immunization is normally administered by injection & is used if there has been a recent outbreak of a particular disease or as an emergency treatment for toxicity (for example, for tetanus). The antibodies can be produced in animals (“serum therapy”) although there is a high chance of anaphylactic shock because of immunity against animal serum itself. Thus, humanized antibodies produced in vitro by cell culture are used instead if available.
Autoimmune diseases

Passive immunization is where pre - synthesized elements of the immune system are transferred to a person so that the body does not need to produce these elements itself. Currently, antibodies can be used for passive immunization. This method of immunization begins to work very quickly, but it is short lasting, because the antibodies are naturally broken down & if there are no B cells to produce more antibodies, they will disappear.
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To function properly, t cells must have two characters Self recognition - They must be able to recognize own MHC (Major histocompatibility) antigens. 2) Self tolerance - They must lack reactivity to peptide fragments from your own proteins. Loss of self tolerance leads to autoimmune diseases. Vaccines are agents derived from bacteria or viruses either as part or as a whole such that they lose the ability to produce disease but retain the ability to induce antibodies in response. The purpose of vaccines is to boost immunity of the body to fight infection. Normally in many diseases the disease - forming germ enters the body, produces the disease & subsequently the body mounts immune response or fighting power against the germ & lastly one recovers from the disease. Here immunity against disease is produced after one has suffered from the disease. A vaccine is nothing else but whole or part of the disease germ, which has been processed or modified in such a way than it has lost its capacity to produce disease but it can still induce immunity or fighting power by body when administered in the body. Hence by vaccination one develops immunity without suffering from the disease. 1)
Types of vaccines

Vaccines can be oral vaccines like oral polio vaccine or oral typhoid vaccine or it can be injectable vaccine like DPT vaccine

or hepatitis B vaccine. Vaccines can be single vaccine like the measles vaccine or combination of more than one vaccine like the MMR vaccine (which acts against measles, mumps & rubella) or the DPT vaccine (which acts against diphtheria, pertussis & tetanus). Injectable vaccines can be given subcutaneously i.e. below the skin like the measles vaccine or given intramuscularly i.e. in the muscle like DPT vaccine. Lastly vaccines are usually given prophylactically i.e. before the exposure to the disease germs like most of the vaccines e.g. polio vaccine, DPT, vaccine etc. Some vaccines work when given even after the exposure to the disease germ like the rabies vaccine, which is given after the dog bite.
Live attenuated Killed or inactivated detoxified agents. Live attenuated

Age Birth - 15 days

Vaccine

6 weeks - 8 weeks

10 weeks - 12 weeks 14 weeks - 16 weeks 6 months - 9 months

Active 1) 2) 1)

15 months - 18 months

5 years 10 years 15 – 16 years Vaccine

• • •

Organisms multiply in the recipient until desired immune response occurs, similar to that with natural infection. Thus live attenuated viral vaccines are likely to confer lifelong protection with single dose. They contain live bacteria/virus that has been altered so it can’t cause disease. e.g. BCG, Polio, Mumps, Measles. Exception— OPV – oral feeding does not always result in infection.
Killed agents

BCG, OPV – zero dose, Hepatitis B Vaccine – 1st dose. DPT – 1st dose, OPV – 1st dose, Hepatitis B Vaccine – 2nd dose. DPT – 2nd dose, OPV – 2nd dose. DPT – 3rd dose, OPV – 3rd dose. OPV – 4th dose Hepatitis B Vaccine – 3rd dose Measles vaccine. MMR (Measles, Mumps, Rubella) DPT – 1st Booster dose, OPV – 1st Booster dose. DPT – 2nd Booster, OPV – 2nd Booster. TT (Tetanus Toxoid)–3rd Booster dose, Hepatitis B Vaccine – Booster dose. TT – 4th Booster dose.
Disease Prevented

Diseases prevented by vaccines

2)

Do not multiply in the recipient. Many killed vaccines which have a lesser antigenic mass require booster vaccinations. They contain bacteria Killed with chemicals. E.g. Typhoid, Cholera, Hepatitis A, Plague & injectable poliomyelitis. The schedule recommended by the Indian Academy of Pediatrics is as follows :
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• •

BCG OPV (Oral Polio Vaccine) DPT (Triple Vaccine)

Hepatitis – B Vaccine Measles Vaccine MMR Vaccine

Tuberculosis Poliomyelitis. D : Diphtheria, P : Pertussis (Whooping Cough), T : Tetanus. Hepatitis – B Measles M : Mumps, M : Measles, R : Rubella (German Measles).

Precautions

It specifies a condition in which a vaccine may be indicated if the benefit to an individual outweighs the risk & consequence of adverse event. OPV dose can be given during diarrhea but should not be • counted & an extra dose in the schedule should be given One can feed the child including breast feeds before & after • any vaccination.
Contraindication

hyperpyrexia (fever > 1050C) with DPT, which must be reported immediately to the doctor.
Other considerations

• • • • • •

• • • • • • •

It indicates that the vaccine should not be administered Anaphylactic reaction to previous dose of same vaccine. Live vaccines are generally contraindicated in immunocompromised persons. Immunosuppressive therapy Corticosteroid therapy Encephalopathy following DPT vaccination. Severe egg allergy in measles vaccine prepared in chick fibroblast cells. Minor illness like mild cough, cold, loose stools, vomiting or even mild fever is not a contraindication for vaccination Common – fever, temporary swelling, redness, pain, induration at site. Mild – pruritis, urticaria & angioedema. Serious – broncospasm, laryngeal edema, shock, CVS collapse. Patient can get pain, redness, swelling at the injection site. Rarely a nodule may form which remains for few weeks especially following DPT. Rarely child can develop fussiness, irritability, anorexia, vomiting, diarrhea, excessive crying etc. especially after DTP injection Lastly there could develop severe reaction like convulsion, depressed responsiveness, altered consciousness, shock,
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Cold chain is important to maintain vaccine potency. Minimum interval between two doses should be at least 1 month. Antibodies appear after 1 - 2 wks of immunization. 2 - 3 i.e. BCG +OPV+DPT vaccines can be given at same time. Different vaccines should not be mixed together. If child misses one dose, whole schedule is not to be repeated.

Minimum gap between 2 vaccines

any number of vaccines can be given on same day at separate sites but if not given on same day there should be gap of 4 weeks between two vaccines in general. One should not give one vaccine today, second 7 day & 3rd vaccine after another 7 days. Extra doses of OPV like in pulse immunization or ring immunization is notable exemption to this rule. Some vaccines like rabies vaccine have schedule where 5 doses are given in 1 month’s time. Again rabies vaccine or tetanus toxoid when indicated should be started as soon as possible irrespective of vaccines received in recent past
Precautions to be taken after vaccination

Side effects

• • • • • •

Keep firm pressure for few minutes at the injection site with a spirit swab. Do not massage or rub at the injection site. Wait in the consulting room for another 30 minutes should any reaction develop following vaccination. Note down carefully when to come back for next vaccination. Inform the doctor immediately if the child develops any reaction or abnormal behavior or habits following vaccination in the next few days.
Immunizing agents Active

1)

Vaccines - A suspension of proteins, polysaccharides or nucleic agents of live attenuated or killed pathogens that induce specific responses that inactivate, destroy or suppress the pathogen.

2)

Toxoid - A purified or modified bacterial toxin that has been made non toxic but retains capacity to stimulate the formation of antitoxin. They contain Toxins/poisons produced by germs which are made harmless. e.g. Diphtheria & Tetanus.

Vaccine carriers

Passive

3)

Immunoglobulin - pre - formed antibodies derived from human blood & primarily used for maintenance of immunity in immunodeficient persons or for passive immunization available in I. M. & I. V. preparations. 4) Anti - toxin - pre - formed antibodies derived from serum of animals after stimulation with specific antigens. Used for passive immunization. Adjutants - agents used to increase immune response e.g. – aluminum salts in toxoids.
Cold chain

• • • • • • •
1) 2) 3) 4) 5)

The equipment & the system of keeping the vaccines cold at right temperature from the manufacturer at the centre to the beneficiary in the periphery is called as ‘cold chain’. All vaccines must be stored at +20 to 8o c. If not stored properly vaccine loses its potency. Heat, sunlight & prolonged power failure can damage vaccines. t –series vaccines damaged by freezing. These are lined with pipes of ice. Act as buffer in case of power failure. No freezer compartment in ILR. In ordinary refrigerators Freezer - storage of ice packs. Top compartment – OPV, measles, MMR. Middle compartment - BCG, T - series & diluents. Lower compartment - IPV, bottles of water Door compartment – no vaccines stored.
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Used to bring vaccines for a days use at peripheral centre. • Contains frozen ice packs. • Can carry 8 – 10 vials of vaccine for a 6 - 8 hrs session. Reverse cold chain – it is the system for sending vaccines cold at right temperature from the periphery to the laboratory for potency checking. Unimmunized child of age 1 - 5 yrs Primary immunization - should receive 3 doses of OPV & DPT with BCG at first visit. Measles at 3 rd visit. Age > 5yrs DT replaces DPT • only 2 doses of OPV & DT • BCG if montoux is negative. • Minimum doses to be given in age < 5yrs 2 doses of TT to pregnant women • 1 dose of BCG • 5 doses of OPV • 4 doses of DPT • 1 dose of DT • 1 dose of MEASLES. •
BCG - Bacilli Calmette Guerine (live)

ILR (ice lined refrigerator)

• •

BCG vaccine is a live bacterial vaccine given for protection against tuberculosis, mainly severe forms of childhood tuberculosis. BCG should be given as early as possible in life, before child comes in contact with tuberculosis. It can be given up to 5 years of age. If it is given beyond 6 months it is preferable to do a prior Mantoux test to see if the patient is already sensitized to tuberculosis. If patient is already sensitized as shown by positive MT, BCG is not necessary India - India introduced BCG mass immunization in 1948, the

4 million bacilli. which looks. B.1mg) =0. It grows in size & forms a nodule.1 ml (o. The ulcer should be kept dry & cleaned with soap & water. measles.first non . — each ampoule is 20 dose & should be prepared by diluting with 2 ml normal saline. consult your doctor. Types . Hence BCG should only be given over left arm & nowhere else. one should give repeat BCG 222 Immediately after the BCG vaccine there is a small swelling at the injection site. If negative.3 times. At the insertion of deltoid muscle in left arm.8 weeks a swelling reappears. It is nothing to be worried about. loss of potency. —below 4 wks of age – 0. A wheal or swelling of 6 mm is raised above the surface. immunodeficient states. Local care at injection site BCG can be given with other vaccines except Measles & MMR. BCG should not be given along with measles or MMR Reaction . Sometimes this process of ulceration & healing recurs 2 . rounded scar of 4 .1925. No spirit or antiseptic should be applied over the site before injection. However it is the only simple & convenient way of determining success of BCG vaccine. The ulcer heals by forming a scar. Ultimately the typical puckered scar is formed which remains for lifetime.8 hours. There may be an associated swelling in the armpit or in some cases on the neck. Infact. Initially given orally – 1921 . No ointment/cream is required. like a mosquito bite. bath with soap & water should suffice even when it has ulcerated.European country to do so. Given only in left arm for universal identification By convention BCG scars are looked for over the left arm & hence it is easier to recognize for the doctor when parents do not remember whether BCG was given in the past or not. If no reaction occurs it may be due to faulty administration. After that the swelling disappears & the injection site looks normal. It should not be fomented. BCG significantly decreases the risk of tuberculous meningitis (Brain TB) & other widespread forms of tuberculosis. Nothing needs to be applied locally.1) Liquid fresh vaccine 2) Freeze dried vaccine Composition – freeze dried vaccine is packed in dark colored ampoules & wrapped in black paper. disseminated T. In fact. WHO BCG policy WHO recommends that BCG be given to all children born in countries highly endemic for TB because it protects against miliary TB & TB meningitis.05ml Route – intradermal. BCG is given along with zero doses of OPV & 1st Dose of Hepatitis B vaccine at birth. Dosage – 0.given to induce benign. which persists for 6 .12 wks) Heals with permanent. Hence search for another cause & treat accordingly. Formation of scar is neither necessary nor is the only indication of success of BCG vaccine. . The whole process takes 2 . BCG does not lead to fever. . If no scar is visible at all after 6 months one needs to do Mantoux test.5 weeks.8 mm diameter. intradermally given since 1927. If ulceration occurs within 7 days of injection. After 6 .ID BCG given Development of papule at site (indication of multiplication of bacteria) (5 wks) Size increases to 5 – 8 mm (6 wks) Ulcer formation (8 . it may be a sign of tuberculosis in the child. which breaks open & discharges some fluid & forms an ulcer. artificial primary infection of tuberculosis. It may take 3 .6 months for the scar to form. but if the swelling increases more than 1 cm in diameter or becomes red or soft or attached to the overlying skin.1 to 0. Normal reaction to BCG vaccine Injection site should not be pressed or rubbed.

swelling & pain at the injection site & fever up to 72 hours is common. However.5 years of age. DPT (diphtheria. Such a child when comes in contact with a patient with tuberculosis can still catch the wild germ & develop primary TB. If BCG is accidentally given to an immunocompromised patient (e. . but the spread will be mostly prevented by previous BCG immunity.5 ML Route Site – intramuscular (deep) – anterolateral aspect of thigh After receiving the DPT injection.8 weeks of age along with the 3 primary doses of oral polio vaccine. BCG immunization causes pain & scarring at the site of injection. The documented incidence of this happening is less than 1 per million immunizations given. with an unparalleled safety record. Such children when they grow as adults can catch tuberculosis again & develop adult form of tuberculosis which is a different type of tuberculosis altogether. The main adverse effects are keloid . children are infected early in life & develop primary TB. The scar of BCG immunization must be distinguished from that of small pox vaccination which it may resemble. a tuberculin skin test should always be done before administering BCG. BCG is one of the most widely used vaccines in the world. A reactive tuberculin skin test is a contraindication to BCG. Applying ice wrapped in handkerchief over the injection site helps to reduce swelling & pain to some extent Age – 6—8 wks after birth. Types Composition Dosage – 0.g. BCG immunization leaves a characteristic raised scar that is often used as proof of prior immunization. It is a common misconception that tuberculin reactors are not offered BCG because “they are already immune” & therefore do not need BCG. it is important to note that an abscess is not always associated with incorrect administration & it is one of the more common complications that can occur with the vaccination. If BCG is accidentally given subcutaneously.threatening infection. it can cause disseminated or life . In children younger than 3 . Efficacy of BCG vaccine 223 In a country like ours where tuberculosis is endemic.3 primary doses of DPT vaccine are given at 4 weeks interval starting at 6 . Schedule – 3 doses for primary immunization at4—8 wks interval between each dose. The insertion of deltoid is most frequently used because the local complication rate is smallest when that site is used. Thus BCG does not prevent adult type of tuberculosis. there is a high risk of severe local inflammation & scarring. this can spread & lead to severe & serious forms of childhood tuberculosis.large.Accelerated BCG response occurs within 4 days. Except in neonates. Hence such children will not develop serious forms of childhood tuberculosis. It occurs if child is previously exposed to mycobacterium tuberculi infection or disease. DT protects against diphtheria & Tetanus & TT protects against tetanus. If someone with a positive tuberculin reaction is given BCG. If given subcutaneously. pertusis. People found to have reactive tuberculin skin tests should be screened for active tuberculosis. BCG being live vaccine itself induces a benign primary infection. It is recommended routinely for each & every child. an infant with SCID). which leads to some immunity. then a local abscess may form (a BCG . BCG causes a local skin infection that may spread to the regional lymph nodes causing a suppurative lymphadenitis. Tetanus & Pertussis (whooping cough). raised scars. Paracetamol is given for 48 – 72 hours after DPT to reduce the severity of the effects. tetanus) (killed DPT is a combination vaccine to protect against Diphtheria.oma) that may ulcerate & often requires treatment with antibiotics.

It persists for 24 .Booster 15 . swelling. It may soften & form a sterile abscess. Acellular pertussis Vaccines contain one or more Bordetella pertussis proteins that serve as immunogens. irritability. First booster dose of DPT is given at the age of 15 . Hence IAP recommends OPV & DPT & not only DT as the 2nd booster at 4 . Such reactions should be immediately reported to doctor. If a patient develops any of those adverse reactions it is a contraindication to further use of DPT & instead only DT should be used. The efficacy of DPT vaccine in walking. excessive crying etc. seen in 30 – 40 % of patients. lasts for 2 .6 years whereas Indian Academy of Pediatrics recommends DPT & OPV as the 2nd booster at 4 . Govt. • vomiting. Side effects Local side effects : It includes pain.3 days for the symptoms to disappear. hyperpyrexia. & 5 yrs. In case abscess has formed.3 days & responds to paracetamol Sometimes. it can be drained. Systemic side effects : It includes fever. as it does not lead to any other symptoms. treat with antibiotics & drainage.18 mt. encephalopathy. Fever is usually mild to moderate. An acellular pertussis vaccine that does not have whole cell wall of pertussis has been developed that can be routinely used for immunization. It is seen in 30 . which are not required for efficacy of vaccine.wall components that result in a high incidence of adverse effects. the severe adverse side effects seen with DPT are due to pertussis component. anaphylactic reaction. It takes 2 . difficulty in walking & fever. redness.oligosaccharide & other cell . Persistent nodule should be left alone. redness & difficulty • The recommendations differ from authority to authority regarding 2nd booster dose Indian Academy of Pediatrics feels that the immunity against pertussis & poliomyelitis will wear off by 5 years as will happen for diphtheria & tetanus. DT vaccine . Sometimes a nodule forms at the injection site. convulsions Contraindications – progressive neurological symptoms uncontrolled seizures successive doses of DPT not given if convulsions. It was realized that the reactions were due to components of cell wall of pertussis organism. This will lead to increased risk of whooping cough & poliomyelitis in adolescents & adults. The pertussis component is a weak candidate & the protective efficacy is seen in 70 . These vaccines contain lipo . including filamentous hemagglutinin. Pertussis component of DPT is responsible for fever more than 1050 F. Whole . lassitude.term protection after primary & booster doses of DPT.cell pertussis Paracetamol or ibuprofen can be used to treat pain. The protection is seen in nearly 100% of vaccinees with good long . fimbrial proteins & pertactin. Treatment of side effects following DPT Efficacy of diphtheria toxoid & tetanus toxoid is excellent. excessive crying & screaming spells lasting for more than 4 hours & convulsions. swelling. The acellular vaccines are associated with a much lower incidence of side effects. persistent & high pitched cry for more than 3 hrs.6 years. Acellular pertussis DPT vaccine As seen before. anorexia. Complications – encephalopathy.90% of cases. which may persist for several days to weeks.40% of vaccinees.72 hours & responds to paracetamol.6 years of age. If it shows fluctuation.18 months along with the 1st booster dose of OPV. 224 Vaccines consist of whole bacteria that have been inactivated & are nonviable. It does not merit any treatment except analgesics. of India under EPI recommends only DT as the second booster at the age of 4 . All acellular pertussis vaccines contain at least detoxified pertussis toxin & most contain other antigens as well.

Route – It is given subcutaneous (in the fat just below the skin) over the thigh or the arm.immunize the patient. If he then develops any injury or requires any surgery there is no need to take anymore TT as he is protected in between the doses. However. it will hyper . which acts as a booster. TT administration in an injured child TT is routinely used for children > 10 years of age as they do not need both Diphtheria & pertussis components. After this a booster of TT is given at 10years. . One needs to give booster of diphtheria at 10 years & maybe every 10 years thereafter to maintain protective titres. TT vaccination desirable or safe to give TT for each & every injury every now & then in such a protected person. If he is 15 . DT is useful in such cases as a booster at 10 years (instead of TT). Immunization in a child already suffered from diphtheria. Effect of frequent TT administration If TT is given frequently. Measles – Measles vaccine is given for protection against measles. Such a child is now protected till 10 years of age. Hence it is not desirable to give frequent injections of TT in an otherwise immunized patient.immunized should receive 3 doses of TT during pregnancy at 1 . With lack of natural boosting due to mass vaccination. diphtheria can occur beyond 10 . It is given as a booster dose at 10 years & 16 years to children who have received their primary doses/boosters of DPT/DT before. If full dose of diphtheria toxoid is given as present in DT/ DPT in children above 10 years of age. it can lead to serious side effects like heart toxicity. These symptoms do not need any treatment except paracetamol to control the fever. serum sickness like reactions etc. fever. In between such doses there is no need to give TT for injury. a person who has recovered from such diseases should receive 3 primary doses & boosters of DPT/DT/TT as appropriate for his age. cough & cold are commonly seen post vaccination. tetanus or pertussis disease leads to strong immunity. the typical fever with rash of childhood so well known to most of the people. Such a child is protected till 4 years & does not need a TT till that age. he can be given one dose of TT.tetanus immunity too.18 months of age he should receive his first 1st booster of OPV + DPT which will also boost up anti .6 years he should receive his 2nd booster of DT or OPV + DPT which will boost up his anti . The last dose should be at least 30 days prior to the expected date of delivery so that there is enough time for good antibody titres to develop in mother & for it to be passed on to the fetus to prevent tetanus in newborn. 2 doses of TT are given at 4 weeks interval. Such a patient can develop arthus like phenomenon with development of fever.5 years in past. If such an adult has taken the last TT beyond 4 . tetanus & pertusis A lady who is previously un . joint pain. Again the last dose is given at least 30 days before delivery. TT to an adult with injury An adult who has never received or has received incomplete course of TT before in life should be given 3 doses of TT at 4 weeks interval followed by a booster after 1 year & then every 5 years.10 years to maintain protection lifelong TT to pregnant women A child who has received 3 primary doses of DPT/DT is protected till 15 months of age & does not need TT. rash.month interval starting the first dose at 28 weeks. If he is between 4 . It is neither required nor 225 Neither of diphtheria. joint swelling etc. It can be then taken every 5 .15 years of age in vaccinees.tetanus immunity. During repeat pregnancy. DT vaccine has 1/10th dose of diphtheria toxoid than present in DT/DPT.It is used in patients where pertussis component is contraindicated & as 2nd booster. measles like rash. Hence. Measles vaccine is usually without any side effects. 16 years & every 5 years thereafter.

In case with mild egg allergy. red eyes & rash over skin. mumps & rubella. Age . as is practiced by USA. At such time a booster of MMR at 5 years (school entry) or 15 years (school exit) is recommended. Side effects – mild fever & rash. Rubella or German measles though a benign disease can lead to congenital rubella syndrome if it occurs during pregnancy. mumps & rubella (German measles).18 mt.Age – It is given at the age of completed 9 months (270+ days of life). During epidemics of measles the vaccine can be given as early as 6 months. but this should be followed by one more measles vaccine at 9 months. heart disease. it is recommended to give measles vaccine at A booster in form of MMR vaccine is given at 15 . Such a child will have cataract. which will act best against mumps & rubella & will act as a booster for measles.sub cutaneous Site . Even if a patient who is vaccinated develops measles. Sometimes a patient can develop measles like infection with cough. It is seen in less than 10% of patients. A time may come in coming years when the routine coverage of MMR will go up which will lead to drastic reduction in natural infections & hence decrease natural boosting against measles.18 months of age. Side effects of measles vaccine Measles vaccine is a very safe vaccine. measles & MMR vaccines are contraindicated. Booster dose of MMR vaccine In patients. Measles vaccine contraindications 9 months. This is because the routine coverage by measles & MMR vaccine is poor. Mumps is a common cause of morbidity in children with occasional chances of complications like encephalitis & pancreatitis. Immunization in a child suffered from measles A child who suffers from measles develops life long immunity. which will protect early against measles followed by MMR vaccine at 15 months of age. cold. small head. Hence MMR vaccine is essential. mumps. induration or fever. measles or MMR vaccines can be given but with strict medical supervision & all the measures of resuscitation & drugs kept ready should the patient develop reaction.0. . it is usually modified & mild with quick recovery & without serious complications. It can occur from the day of injection till 5 .7 days. Schedule – single dose. it leads to mild pain. Again it lasts for 2 .15 . swelling. Hence such a child does not need measles vaccine. The efficacy of measles vaccine Measles vaccine is an excellent vaccine with more than 90% efficacy with a booster given in form of MMR. This has been the experience in western countries. is transient & mild. mental retardation & many other complications. When in doubt it is better to give measles vaccine as no harm is done even if patient has suffered from measles in past. deafness. 226 As of today there is no need to give booster dose of MMR vaccine in India. Booster dose of measles vaccine MMR (measles. with mild egg allergy. Hence we advise measles vaccine at 9 months followed by MMR at 15 months. Measles though a mild disease in children can occasionally lead to pneumonia & brain damage (SSPE). mumps & rubella are common which act like natural booster in vaccinees.5 day & is self limiting. If at all.5 ML Route .upper arm or anterolateral aspect of thigh. measles vaccine can lead to allergic reactions. In cases of severe egg allergy. Therefore naturally occurring subclinical measles. lasts for < 24 hours & responds well to paracetamol. Composition Dosage . rubella) (live) MMR vaccine is given for protection against 3 common childhood viral diseases namely measles.

In case with mild egg allergy. Hence. cold. state. measles or MMR vaccines can be given but with strict medical supervision & all the measures of resuscitation & drugs kept ready should the patient develop reaction. red eyes & rash over skin. Contraindications of MMR vaccine OPV far outweigh the rare risk of paralysis. Child comes late for polio vaccine OPV given at the time of birth is called zero polio. It occurs in 1 in million doses. In the sewage water it can survive only for 48 – 72 hrs & hence it has to find shelter into another susceptible host for it to survive & to continue cycle of transmission. The dose is 2 drops per dose. Most of the children are immune by 5 years naturally due to subclinical infections. redness & tiredness in 10 – 20 % of vaccinees.limiting. Contraindications of polio vaccine In patients. Sometimes a patient can develop measles like infection with cough.7 days.5 ml of vaccine. Availability Paralytic poliomyelitis can occur till 15 years of age. though. Over & above that ‘pulses polio’ doses are administered at least twice a year (usually in winter) to all children below 5 years of age. Wild poliovirus can survive either in the intestines of a susceptible host (usually children < 5 years of age) or in the sewage water. swelling. Such a child should receive 3 primary doses followed by 1st booster 1 year after 3rd primary dose & a second booster 3 years – 4 years after the 1st booster dose provided the child is still less than 5 years old then. If at all. IPV is available as a single dose vial containing 0. OPV OPV is contraindicated in patients with low immunity especially if IPV is available. Again it lasts for 2 . In west. It can lead to fever. measles & MMR vaccines are contraindicated.5 days & is self . swelling. it leads to mild pain. city) at a time on a given day. it can become a medico –legal problem. When you give pulse OPV to all . It is also available as combination vaccines containing IPV+DPT or IPV+DPT +Hepatitis B or IPV + DPT + Hib or IPV + DPT + Hepatitis B + Hib. IPV can lead to local side effects like pain. Extra doses (pulses) of OPV are given to all children below 5 years of age in an area (like country. irrespective of number & time of previous polio doses. It is seen in less than 10% of patients are transient & mild. The most important but extremely rare side effect with OPV is vaccine . which is mild & lasts for 24 – 48 hours. Massive benefits of 227 It is a strategy of mass immunization by which one can eradicate poliomyelitis. which will lead to local gut immunity without the risk of vaccine induced paralysis due to the partial immunity conferred by prior IPV. In cases of severe egg allergy. Such pulses are repeated every year. The aim is to achieve 100% coverage. induration or fever. OPV can be given to a patient with diarrhea. Pulse polio immunization OPV is available as vial containing multi doses. Total 5 polio doses are given in the first year of life. IPV is contraindicated if patient has developed severe adverse effect to its use in past. It can lead to GI upset like diarrhea. but that dose should not be counted & should be followed by an extra dose. with mild egg allergy MMR vaccine can cause allergic reactions. Hence in west. It does not lead to fever. Most of the side effects seen with OPV +DPT are due to the DPT vaccine.associated paralysis (VAP). people use first IPV to induce systemic antibodies & then use OPV. OPV is given to any child who presents till 5 years of age. vomiting. Side effects of polio vaccines OPV has minimum side effects. It can occur from the day of injection till 5 .Side effects of MMR vaccine MMR vaccine is a very safe vaccine. lasts for < 24 hours & responds well to paracetamol.

induces both types of immunity. steroid therapy. vaccine induced polio & vaccine failure may occur. not reinfection Almost no contraindication Live attenuated 6. No local immunity prevents paralysis. malignancy. cheap. Additional doses of polio may be given in a particular area if a case of polio is found in that area. As wild poliovirus cannot survive for more than 48 .immunocompromised child. leukemia. the intestines of these children are flooded by vaccine virus & hence wild poliovirus cannot get entry into it. Due to temperature & prolonged storage the color of square changes from pink &becomes darker.4 . its circulation will drastically fall. • • • Dose Route Immunity Repeat OPV administration if child spits Avoid hot liquids. difficult to manufacture.5 – 10% develop chronic carrier state. pregnancy Vaccine induced polio (1/5 million cases) Easy administration. IgA in serum. IgG. antibody production slower. 10. 10. pregnancy Hepatitis B (HBV) is a type of virus that leads to jaundice & infective hepatitis.the children of < 5 years of age. steroid therapy. Hepatitis B Complications Advantages Safe in immunocompromised steroid therapy. food & breast feeding ½ hour before &after vaccination. 228 No local immunity.5 wks 0. It is placed on outer colored circle printed on label of OPV vial. diarrhea. 14 wks Zero dose at birth 6. pregnancy. It is compared with the color of outer circle to check potency. trained persons required. Disadvantages Polio vaccines General considerations antibody production even with single dose. VVM – vaccine vial monitor Contraindications It is a small square made up of heat sensitive material. Again of these carriers. 30% will develop chronic liver disease & some will develop liver cancer. prevents paralysis & intestinal infection useful in epidemics Acute infection.5 ml IM/SC Produce humoral IgM.1 – 2% of patients with HBV infection develop fulminant hepatitis which carries > 80% mortality. dysentery. When such pulse is repeated after 6 weeks it still further reduces transmission. costlier. > 50 yrs. HBV leads to acute hepatitis & most of the patients recover. Intramuscular injections & tonsillectomy should be avoided during epidemic of polio. no trained persons required. 14 wks 1st booster 18 mts 2nd 5 yrs 2 drops Oral Both humoral& local immunity. Type Characteristic Composition Age Schedule IPV (SALK) OPV (SABIN) Inactivated/killed 8 wks initiation 3 doses at 6 wks interval 1st booster 18 mts 2nd . 1st vacation. useful in epidemics. Cannot be used in immunocompromised. more than 1 dose required to induce immunity Contraindications . Modes of transmission of HBV Vertical transmission occurs from Hepatitis B positive pregnant . Such pulses done every year will ultimately eradicate the wild poliovirus from nature.72 hrs in environment.

Oral ty21a vaccine is in form of capsules & the capsules need to be swallowed intact. a patient of HBV infection develops protective antibodies to surface antigen (anti – HBs) on recovery. 6 weeks along with OPV/DPT & 3rd dose can be given at 14 weeks. In a child more than 6 years old. Local reactions including redness. it would be worthwhile to administer this vaccine. Boosters are necessary every 3 years. Oral ty21a vaccine is a course of 3 capsules given orally on alternate days. Instead just complete the remaining doses as per original schedule. patients on high dose long . It can also spread via blood products. but it is a total waste of such an expensive vaccine. which occurs when one partner is infected. This is given as a single capsule every alternate day for 3 doses. pain & swelling at injection site. The capsules should be swallowed intact & not opened or chewed. Last is the sexual route of transmission. IV drug abuse. Types of typhoid vaccine Vi antigen vaccine : It is an inactivated vaccine available as Oral ty21a vaccine Availability injectable form. Typhoid fever can occur at any age. But sachet form is not available in India. Newer vaccines now available are administered as a single dose injection after two years of age. There is no need to restart the course.10 years. It is given as a single dose. the pack contains 4 capsules & such 4 capsules are given on alternate days.5 ml containing mono dose prefilled syringes.term steroids & patients on hemodialysis have poor seroconversion following conventional schedule. Hence earlier the vaccine is given better it is. Immuno – compromised children Children with leukemia. ear piercing etc. 229 Vi antigen vaccine is given in the dose of 0.10% of cases occur in infants < 1 year of age. If the child comes late for subsequent doses Considering the high incidence of typhoid in our country. A booster is not required for 5 . There is no risk associated with vaccination. The ty21a oral vaccine is available as a pack of 3 capsules. Ideally it should be given to every individual. : This is an oral live vaccine.5 ml intramuscularly either on thigh or arm. an oral (capsule) preparation is available. In fact. However such delays are not desirable as the child remains unprotected till the course of 3 doses is completed.5 ml of vaccine or as multi dose bulbs containing 4 – 10 doses (2 – 5 ml) depending on brand used. Transmission may also occur due to close contact e. Even children with Down’s syndrome show poor seroconversion & titres & should be given double the normal doses should Hepatitis B vaccine be given to a carrier or to a patient who has recovered from HBV infection? Neither the carrier nor the patient who has recovered needs Hepatitis B vaccine. The most common group to be affected is school going children & adolescents. What is the schedule of Hepatitis B immunization? Schedule consists of giving 3 doses at birth. Schedule The side effects of the vaccines are very few if any. Hepatitis B vaccine Typhoid Hepatitis B vaccine protects against infection by HBV.g. acupuncture needles. Side effects of Hepatitis B vaccine The Vi antigen vaccine is available as single dose ready to use vials containing 0. surgical instruments. Though the vaccine is effective after 2 . Some brands are available as 0. The Vi antigen vaccine can be given only after completing 2 years of age. amongst family members or at day care centers. The oral vaccine also needs to be taken every 3 years for continued protection.women to their babies during the perinatal period. contaminated needles. Hence it is recommended to double the dose of vaccine. In most. multi – transfused thalassemics. Liquid form of oral ty21a vaccine in sachet form is more effective than the capsule form. tattooing. on chemotherapy.

The currently available vaccines are safe & efficacious.limiting. If it occurs during pregnancy. any child who has shown severe reactions to previous typhoid vaccine should not be given the same vaccine again. the child has chances of developing herpes zoster at an early age. college students. Typhoid is an endemic disease in India with estimated incidence of 5 million cases per annum. tenderness etc. Those at high risk of exposure to chickenpox like teachers of young children. Care should be taken to stop chemotherapy/ steroids for at least 2 weeks before & after vaccination or at least for the first dose of vaccination. pneumonia.limiting disease & occasionally leads to fever. If it occurs in first half of 230 pregnancy it will lead to fetal varicella syndrome characterized by scarred skin. If it occurs within 21 days of delivery. diarrhea & occasionally mild fever.years of age. Schedule It is to be given subcutaneous over left arm or thigh.g. Hence such children should be immunized irrespective of age. The complications are rare & mortality even more rare.14 days. Systemic side effects are rarely seen & include mild fever lasting for 24 hours. practically a child above 4—6 years of age can swallow capsules. those who eat outside open food frequently. family members of a child with leukemia.pregnant women of childbearing age. liver damage & brain infection.care nursery workers.March every year. redness. vomiting.15 months of age.8 .9 years leading to rash. Oral ty21a vaccine : It leads to mild GI discomfort like pain in abdomen. If it occurs in 2nd half of pregnancy. Chicken pox can be very severe in immunocompromised • patients e. But there are occasional cases of complications like encephalitis. More important. It is given at 12 . last for 24 hours & respond to paracetamol. the reactions are less common. Non . it is given 4 weeks after complete recovery from typhoid fever. pneumonia & even death in normal children. Side effects of typhoid vaccines Vi antigen vaccine : It can lead to local side effect like pain. Again. swelling. 2 doses are given at 6 . Varicella vaccine is definitely indicated in certain high . in patients with cancer & can even lead to death. A normal child who develops chickenpox suffers for 1 . Hence it is recommended after the age of 4—6 years. limb defects & eye defects. In such cases. there are 25% chances of • transmission of the virus to fetus. only one dose is required. It must be given to all food handlers. One needs to give 2 doses of injections.risk groups : Immuno . Even the parents will have to take leave & lose wages for 7 . which responds well to paracetamol.2 weeks of disease. Contraindications to typhoid vaccine • • i) ii) iii) Oral ty21a vaccine is contraindicated in immune compromised host. there are 25% chances of newborn developing chicken pox. Health personnel. during & after vaccination with oral ty21a vaccine. travelers to endemic areas. The side effects are very mild & are seen in < 10% of vaccinees. Lastly. day . it leads to social problems like the child missing school or even final exams as it usually occurs in months of Jan . military personnel etc. as it is a live vaccine. It should also be given to those who have suffered typhoid fever as typhoid does not lead to protective immunity & relapses are known to occur. It is a self . Adults in contact with children who are at high risk of complications of chicken pox e. Varicella zoster iv) • Varicella or chickenpox is a highly contagious disease with peak age at 5 . In children > 13 years & adults. In children < 13 years.compromised host : Varicella can be very severe in immunocompromised host as seen before. Antibiotics that act against typhoid should not be given 5 days before.g. mild & self .

which are very mild with rapid recovery.8 weeks 10 weeks . they are recommended as routine vaccines for all children. Secondly.12 weeks 14 weeks .7% of vaccines can develop varicella like rash. Herpes zoster.50 years.patients with neomycin hypersensitivity as the vaccine contains traces of neomycin.16 weeks 6 months 9 months (completed) 15 months . this can lead to permanent damage to vision. chemotherapy should be withheld for at least 2 weeks before & after the first dose of vaccine. it can lead to complications like pneumonia & brain damage. It is contraindicated during pregnancy & in fact pregnancy should be avoided for at least 6 . Systemic side effects like fever are rare. he develops chickenpox. herpes zoster if 231 These include typhoid vaccine & Hib vaccine. EPI schedules as recommended by Govt. For patients with leukemia in remission. Local side effects seen include pain. Common childhood diseases like hepatitis A & chicken pox lead to more suffering but very less chance of death. it could also involve the eyes & unless one takes proper precaution. These diseases are usually mild & self . varicella virus. though usually a benign disease lasting for 7 10 days can lead to complications.8 weeks after vaccination. maceration.term steroids should be given the vaccine when steroids are temporarily withheld for at least 2 weeks before & after vaccine. Side effects it occurs in immuno .18 months 4 years . When a person comes in contact with the virus for the first time. prolonged contact with urine & feces. the virus remains latent in the nerves for years.15 days 6 weeks .compromised patients need 2 doses irrespective of age.3 . Contraindications Firstly it is not indicated in those who have definite history of chickenpox in the past.5 years thereafter. the virus is able to spread albeit locally along the nerve root causing herpes zoster. it can lead to pain. Firstly. of India It is a very safe vaccine.weeks interval. 69 Diaper dermatitis • It is caused by over hydration of the skin.limiting in children & hence option of routine vaccination of children is left to parents. The currently available typhoid vaccines given are after 2 years of age & repeated every 3 . Immuno . These are costly vaccines. It is contraindicated in patients with immunodeficiency including symptomatic HIV & in children with acute leukemia on chemotherapy.e. Similarly patients on high dose of long . if it involves the face.20% of them. Even when he recovers.6 years 10 years 16 years BCG + OPV (ZERO DOSE) + Hep B1 OPV1 + DPT1 + Hep B2 OPV2 + DPT2 OPV3 + DPT3 Hep B3 Measles vaccine 1st booster of OPV/ DPT DT vaccine Tetanus Toxoid Tetanus Toxoid Vaccination schedule recommended by Indian Academy of Pediatrics (IAP) IAP Time Schedule of routine vaccination Optional vaccines Varicella & herpes zoster are manifestations of the same virus i. Lastly. Chickenpox & Hepatitis A vaccine can be given after 1 year of age. This makes their use optional as per wishes of parents. swelling in < 5% of vaccines.compromised patients like HIV. retained diaper soaps & topical preparations & is a prototypical example of irritant contact . which can go on for months to years. In 15 . It is contraindicated in . Herpes zoster Birth . But for the cost. This occurs due to natural fading of immunity & hence occurs after the age of 40 . It can occur within 6 weeks of vaccination. redness.

adding to the effect. may result in a triad of hair loss. Nutritional history may also be an important factor to consider in diaper dermatitis. Diaper rash affects the areas within the confines of the diaper. Lipases & proteases in feces mix with urine on non intact skin & cause an alkaline surface pH. chemical & enzymatic mechanisms.binding ligands in the intestine. but is usually not necessary in patients with diaper dermatitis. Children with a previous medical history of eczema or atopic dermatitis may be more susceptible to diaper dermatitis. but often involved in primary candidal dermatitis. such as occurs with elemental formula alone.poor diet. Increased wetness in the diaper area makes the skin more susceptible to damage by physical. affecting the abdomen from the umbilicus down to the thighs & encompassing the genitalia. with peak incidence occurring when the individual is aged 9 . (Feces in breastfed infants have a lower pH & breastfed infants are less susceptible to diaper dermatitis) The bile salts in the stools enhance the activity of fecal enzymes. A biotin .• dermatitis. Lack of zinc . fissures & erosions. perineum & buttocks. dermatitis & diarrhea. developmental delay. Wet skin increases the penetration of irritant substances. Urease has a mild irritant effect on non intact skin. Children with diaper dermatitis have marked discomfort from intense inflammation. . The eruption may be patchy or confluent. Causes • • Age • • • • • • • The following causes have been noted : Over hydration of the skin Maceration Prolonged contact with urine & feces Retained diaper soaps Topical preparations More than 3 diarrheal stools per day Side effects of oral antibiotics Contact Dermatitis Herpes Simplex Virus Infection Histiocytosis Scabies Syphilis Varicella Differential diagnosis • • • Acrodermatitis Enteropathica Atopic Dermatitis Biotin Deficiency Candidiasis Child Abuse & Neglect : Physical Abuse& Sexual Abuse Laboratory Studies • Diagnosis of candidal dermatitis can be established by potassium hydroxide (KOH) preparation or culture. may result in perioral erythema. Genitocrural folds are spared in irritant dermatitis. Signs & symptoms are restricted in most individuals to the area covered by diapers. 232 Physical Pathophysiology • • Patients with diaper dermatitis present with an erythematous scaly diaper area often with papulovesicular or bullous lesions. Candida albicans has been identified as another contributing factor to diaper dermatitis Diaper dermatitis commonly affects infants. adding to the irritation. Treatment • Provide education regarding diaper dermatitis to patient. such as in the autosomal recessive disorder acrodermatitis enteropathica.12 months. Super hydration urease enzyme found in the stratum corneum liberates ammonia from cutaneous bacteria. loss of hair & hypotony (in addition to diaper dermatitis).

Pediatric .• o o o • o parents. which is composed principally of white petrolatum.Have antiseptic & astringent properties. mineral oil & lanolin. Pediatric .counter (OTC) emollient available for newborns is pure white petroleum ointment. zinc oxide.Apply to diaper area after every diaper change Zinc oxide . Another safe alternative is Aquaphor ointment. Pediatric . that is both a skin protectant & has a drying effect on vesicular or wet dermatoses. It is more expensive than pure white petrolatum ointment. Pediatric .Apply to diaper area after every diaper change Petrolatum.line therapy for diaper dermatitis is zinc oxide ointment. first .potency steroids. Protective topical agents Ideally.Fungicidal & fungistatic antibiotic obtained from Streptomyces noursei. topical low .the . Pediatric . mineral oil & wood wax alcohol.Apply to the affected skin & surrounding areas q12 .An adrenocorticosteroid derivative suitable for application to skin or external mucous membranes. Pediatric .Effective in cutaneous infections. which acts by trapping water beneath the epidermis.line therapy for individuals with diaper dermatitis is zinc oxide ointment. the first .Combination product 233 .Apply to diaper area after every diaper change Nystatin . Petrolatum .4 wk Hydrocortisone. and. The safest over .Apply sparingly to diaper area bid Activity The diaper area may be left open to air or covered with a topical emollient.24h for 2 . aluminum acetate solution . topical . and/or caregivers Ideally. Medical therapy for diaper dermatitis includes the use of protective topical agents. Plays significant role in wound healing with low risk for allergic or contact dermatitis. It has mineralocorticoid & glucocorticoid effects resulting in anti inflammatory activity. topical anticandidal agents. Another safe alternative is Aquaphor ointment.Apply sparingly over affected area bid Econazole . which is principally composed of white petrolatum. Zinc oxide is an inexpensive treatment with the following properties : Antiseptic & astringent Significant role in wound healing Low risk for allergic or contact dermatitis The safest OTC emollient available for newborns is pure white petroleum ointment.Traps water beneath the epidermis. possibly. It is more expensive than pure white petrolatum ointment.

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