Pediatric Disorders: Module

THE ILL AND HOSPITALIZED CHILD STRESSORS and FEARS of HOSPITALIZATION 1. Separation Stages 2. Loss of Control 3. Body Injury 4. Pain 5. Immobility 6. Punishment and Rejection PREPARATION for HOSPITALIZATION 1. Parents eagerly seek guidance from nurses on what and how much to tell their children about an anticipated admission. The preparation a parent makes for a child obviously varies according to the child's age and individual experience. No matter what the child's age, however, parents should be encouraged to convey a positive attitude. 2. Children between 2 and 7 years of age should be told about a scheduled ambulatory or inpatient hospitalization as many days before the procedure as the child's age in years. 3. On the day of admission, it is important for you to discuss the preparation the child has received to ensure that the child and family accurately understand the child's condition and upcoming procedures. ASSESSMENT ON ADMISSION 1. Assess each child's level of preparation for a hospitalization on admission to the facility. Be aware of not only what the child describes orally but also what facial expressions or nervous manifestations may be indicating. 2. Interview parents on hospital admission for a nursing history to obtain the information needed to plan nursing care. 3. Make a note of any medication or food allergy on the child's plan of care 4. Take and record the child's temperature, pulse, and respirations. Measure height and weight to determine overall growth and to allow for determination of surface area, the measurement on which medication dosage is calculated. PAIN ASSESSMENT For children, pain is not only a hurting sensation, but it can also be a confusing one because a child did not anticipate the pain, cannot explain its presence, and cannot always understand its cause. Because children may have difficulty describing pain in a manner adults can understand, it is difficult to assess the extent of their discomfort. Both helping children describe the type and extent of pain they are feeling and performing active interventions to relieve pain are important nursing roles.

METHODS OF PAIN ASSESSMENT Pain Experience Inventory - A tool consisting of eight questions for children and eight questions for the child's parents. It is designed to elicit the terms a child uses to denote pain and what actions the child thinks will best alleviate the pain. Cries Neonatal Postoperative Pain Measurement Scale - 10-point scale on which five physiologic and behavioral variables frequently associated with neonatal pain can be assessed and rated:  Amount and type of crying  Need for oxygen administration  Increased vital signs  Facial expression  Sleeplessness Comfort Behavior Scale - A pain rating scale devised by nurses to rate pain in very young infants. On the first part of the scale, six different categories (alertness, calmness/agitation, crying, physical movement, muscle tone, and facial expression) are rated from 1 to 5. Six is the lowest score (no pain), and 30 is the highest (a great deal of pain). In addition to rating physical parameters, nurses then observe the infant for 2 minutes and rate their evaluation of the baby's pain on an analogue (1-to-10) visual scale. FLACC Pain Assessment - A scale by which health care providers can rate a child's pain when a child cannot give input, such as during circumcision. It incorporates five types of behaviors that can be used to rate pain: facial expression, leg movement, activity, cry. FACES Pain Rating Scale - This scale consists of six cartoon-like faces ranging from smiling to tearful. Explain to the child that each face from left to right corresponds to a person who has no hurt up to a lot of hurt the words under each face to describe the amount of pain the face represents. Numerical or Visual Analog Scale - It uses a line with end points marked ³0 = no pain´ on the left and ³10 = worst pain´ on the right. Divisions along the line are marked in units from 1 to 9. Explain to children that the left end of the line (the 0) means a person feels no pain. At the other end is a 10, which means a person feels the worst pain possible. The numbers 1 to 9 in the middle are for ³a little pain´ to ³a lot of pain.´ Ask children to choose a number that best describes their pain.

Adolescent Pediatric Pain Tool - It combines a visual activity and a numerical scale. On one half of the form is an outline figure showing the anterior and posterior view of a child. To use the tool, a child is asked to color in the figure drawing where he or she feels pain. In addition, on the right side of the form, the child rates the pain in reference to ³no pain,´ ³little pain,´ ³medium pain,´ ³large pain,´ and ³worst possible pain.´ For a third activity, children are asked to point to or circle as many words as possible on the form that describe their pain (words such as horrible, pounding, cutting, and stinging) - This is a useful tool for involving parents to talk with their child about his or her pain. Reading the words together helps the child examine the type, location, and level of pain he or she is experiencing. It also helps parents to better understand what their child is experiencing. PAIN MANAGEMENT Non-Pharmacologic 1. Distraction - It aims at shifting a child's focus from pain to another activity or interest. 2. Substitution of meaning - is a distraction technique to help a child place another on a painful procedure. Children are often more adept at imagery than adults because their imagination is less inhibited 3. Thought Stopping - a technique in which children are taught to stop anxious thoughts by substituting a positive or relaxing thought. As with imagery, this technique requires a great deal of practice before it is used in a painful situation. For this technique, help the child to think of a set of positive things about the approaching feared procedure. 4. Hypnosis - is not a common pain management technique with children but can be very effective when a child is properly trained in the technique. 5. Magnet Therapy - is based on the belief that magnets can control or shift body energy lines to restore health or relieve pain. 6. Music Therapy ±the use of music for calming or improving well-being and can be effective. 7. Yoga and Meditation - It offers a significant variety of proven health benefits, such as increasing the efficiency of the heart, slowing the respiratory rate, improving fitness, lowering blood pressure, promoting relaxation, reducing stress, and allaying anxiety. 8. Acupuncture - involves the insertion of needles into critical positions in the body to achieve pain relief. Although acupuncture is almost painless, children can be very afraid of it at first because of the sight of the needles. 9. Transcutaneous Electrical Nerve Stimulation - involves applying small electrodes to the dermatomes that supply the body portion where pain is experienced.

Pharmacologic 1. Topical Anesthetic Cream - to reduce the pain of procedures such as venipuncture, lumbar puncture, and bone marrow aspiration, a local anesthetic cream or a solution of lidocaine and epinephrine is available 2. Oral Anlgesia ± relatively easy to administer 3. Intramuscular Injection 4. Intravenous Administration - most rapid-acting route and the method of choice in emergency situations. 5. Conscious Sedation - refers to a state of depressed consciousness usually obtained through IV analgesia therapy. The technique allows a child to be both pain-free and sedated for a procedure. Unlike with the use of general anesthesia, protective reflexes are left intact and a child can respond to instructions during the procedure. 6. Intranasal Administration 7. Local Anesthesia Injection 8. Epidural Analgesia - injection of an analgesic agent into the epidural space just outside the spinal canal, it can be used to provide analgesia to the lower body for 12 to 24 hours. LEADING CAUSES of accidents/ injuries in children Infancy:  Aspiration  Suffocation  Fall Toddler:  Fall  Drowning  Poisoning  Burn Pre-schooler  Drowning  Motor accident  Burn

DEVIATIONS FROM NORMAL IN THE NEWBORN Pre-term or Low Birth Weight Infant BACKGROUND OF THE STUDY A preterm infant is usually defined as a live-born infant born before the end of week 37 of gestation; another criterion used is a weight of less than 2,500 g (5 lb 8 oz) at birth. About 7% of all pregnancies end in preterm birth, and all such infants need neonatal intensive care from the moment of birth to give them their best chance of survival without neurologic after-effects (Petrou, 2003).

When a preterm infant is recognized by a gestational age assessment, watch for the specific problems of prematurity, such as respiratory distress syndrome, hypoglycemia, and intracranial hemorrhage.

Differences Between Small-for-Gestational-Age and Preterm Infants Characteristic Gestational age Birthweight Congenital malformations Pulmonary problems Hyperbilirubinemia Hypoglycemia Intracranial hemorrhage Apnea episodes Feeding problems Weight gain in nursery Future restricted growth Small-for-Gestational-Age Infant 24±44 wk Under 10th percentile Strong possibility Meconium aspiration, pulmonary hemorrhage, pneumothorax Possibility Very strong possibility Strong possibility Possibility Most likely due to accompanying problem such as hypoglycemia Rapid Possibly always be under 10th percentile due to poor organ development Preterm Infant Younger than 37 wk Normal for age Possibility Respiratory distress syndrome Very strong possibility Possibility Possibility Very strong possibility Small stomach capacity; immature sucking reflex Slow Not likely to be restricted in growth as ³catch-up´ growth occurs

NEWBORN PRIORITIES IN FIRST DAYS OF LIFE

All newborns have eight priority needs in the first few days of life:

W - aste elimination establishment A -dequate nourishment T-emperature Control E - xtrauterine circulation establishment R- espiration initiation and maintenance I- nfection prevention D - evelopmental care / care that balances physiologic needs and stimulation for best development E - stablishment of an infant±parent relationship

These are also the priority needs of high-risk newborns. Because of small size or immaturity or illness, fulfilling these needs, however, may require special equipment or care measures. Not all newborns will be able to achieve full wellness because of extreme insults to their health at birth or difficulty adjusting to extrauterine life.

ETIOLOGY - The exact cause of premature labor and early birth is rarely known. - There is a high correlation between low socioeconomic level and early termination of pregnancy. - The major influencing factor in these instances appears to be inadequate nutrition before and during pregnancy, as a result of either lack of money for or lack of knowledge about good nutrition.

Factors Associated with Preterm Birth

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Low socioeconomic level Poor nutritional status Lack of prenatal care Multiple pregnancy Previous early birth Race (nonwhites have a higher incidence of prematurity than whites) Cigarette smoking Age of the mother (highest incidence is in mothers younger than age 20) Order of birth (early termination is highest in first pregnancies and in those beyond the fourth pregnancy) Closely spaced pregnancies Abnormalities of the mother's reproductive system, such as intrauterine septum Infections (especially urinary tract infection) Obstetric complications, such as premature rupture of membranes or premature separation of the placenta Early induction of labor Elective cesarean birt

ASSESSMENT:

- Preterm infant appears small and underdeveloped. - Head is disproportionately large (3 cm ormore greater than chest size). - Skin is generally unusually ruddy because the infant has little subcutaneous fat beneath it; veins are easily noticeable, and a high degree of acrocyanosis may be present. - The preterm neonate, 24 to 36 weeks, typically is covered with vernixcaseosa. However, in very preterm newborns (less than 25 weeks' gestation), vernix is absent because it is not formed this early in pregnancy. - Lanugo is usually extensive, covering the back, forearms, forehead, and sides of the face, because this amount is present until late in pregnancy. - Both anterior and posterior fontanelles are small. - There are few or no creases on the soles of the feet. - The eyes of most preterm infants appear small. - Although difficult to elicit, pupillary reaction is present. - A preterm infant has varying degrees of myopia (near-sightedness) because of lack of eye globe depth. - The cartilage of the ear is immature and allows the pinna to fall forward. The ears appear large in relation to the head. - If tested, reflexes such as sucking and swallowing will be absent if an infant's age is below 33 weeks; deep tendon reflexes such as the achilles tendon reflex are also markedly diminished. - During an examination, a preterm infant is much less active than a mature infant and rarely cries. - If the infant does cry, the cry is weak and high-pitched.

POTENTIAL COMPLICATIONS Because of immaturity, preterm infants are prone to a number of specific conditions.

1. Anemia of Prematurity - Many preterm infants develop a normochromic,

normocytic anemia (normal cells, just few in number). The reticulocyte count is low because the bone marrow does not increase its production until approximately 32 weeks. 2. Kernicterus - a destruction of brain cells by invasion of indirect bilirubin. Preterm infants are more prone to the condition than term infants because with the acidosis that occurs from poor respiratory exchange, brain cells are more susceptible to the effect of indirect bilirubin than normally. 3. Persistent Patent DuctusArteriosus - Because preterm infants lack surfactant, their lungs are noncompliant, so it is more difficult for them to move blood from the pulmonary artery into the lungs. This condition leads to pulmonary artery hypertension, which may interfere with closure of the ductusarteriosus. 4. Periventricular/Intraventricular Hemorrhage - Preterm infants are prone to periventricular hemorrhage (bleeding into the tissue surrounding the ventricles) or intraventricular hemorrhage (bleeding into the ventricles); these conditions occur in as many as 50% of infants of very low birthweight. This occurs because preterm infants have both fragile capillaries and immature cerebral vascular development.

NURSING DIAGNOSIS Because a preterm infant has few body resources, both physiologic and psychological stress must be reduced as much as possible and interventions initiated gently to prevent depletion of resources. Close observation and analysis of findings are essential to managing problems quickly.

1. Nursing Diagnosis: Impaired gas exchange related to immature pulmonary functioning

Outcome Evaluation: Many preterm babies, particularly those under 32 weeks of age, have an irregular respiratory pattern (a few quick breaths, a period of 5 to 10 seconds without respiratory effort, a few quick breaths again, and so on). There is no bradycardia with this irregular pattern (sometimes termed periodic respirations).

2. Nursing Diagnosis: Risk for imbalanced nutrition, less than body requirements related to additional nutrients needed for maintenance of rapid growth, possible sucking difficulty, and small stomach. Outcome Evaluation: Infant's weight follows percentile growth curve; skin turgor is good; specific gravity of urine is maintained between 1.003 and 1.030; infant has no more than 15% weight loss in first 3 days of life and continues to gain weight after this point.

3. Nursing Diagnosis: Risk for infection related to immature immune defenses in preterm infant Outcome Evaluation Temperature is maintained at 97.6°F (36.5°C) axillary; further signs and symptoms of infection such as poor growth or a reduced temperature are absent

CONGENITAL HEART DEFECTS Congenital heart disease refers to a problem with the heart's structure and function due to abnormal heart development before birth. Congenital means present at birth. ACYANOTIC HEART DISEASE Acyanotic heart disease is a broad term for any congenital heart defect in which all of the blood returning to the right side of the heart (shunt that moves blood from the arterial to the venous system or left-to-right shunts), passes through the lungs and pulmonary vasculature in the normal fashion. The common forms of acyanotic congenital heart defects are those where there is a defect in one of the walls separating the chambers of the heart, or obstruction to one valve or artery. TYPES OF ACYANOTIC HEART DISEASE 1.) ATRIAL SEPTAL DEFECT

DEFINITION -Is a form of congenital heart defect that enables blood flow between the left and right atria via the interatrial septum. -The interatrial septum is the tissue that divides the right and left atria. Without this septum, or if there is a defect in this septum, it is possible for blood to travel from the left side of the heart to the right side of the heart, or vice versa.

-This results in the mixing of arterial and venous blood, which may or may not be clinically significant. ETIOLOGY The heart is forming during the first 8 weeks of fetal development. It begins as a hollow tube, then partitions within the tube develop that eventually become the septa (or walls) dividing the right side of the heart from the left. Atrial septal defects occur when the partitioning process does not occur completely, leaving an opening in the atrial septum. Some congenital heart defects may have a genetic link, either occurring due to a defect in a gene, a chromosome abnormality, or environmental exposure, causing heart problems to occur more often in certain families. Most atrial septal defects occur sporadically (by chance), with no clear reason for their development. SIGNS AND SYMPTOMS  Child tires easily playing.  Fatigue.  Sweating.

when 

Rapid breathing.  Shortness of breath.  Poor

growth.

DIAGNOSIS  Physical exam auscultation of the heart- there is a loud harsh systolic murmur in the left sternal border at the 3rd-4th interspaces  Echocardiography- an atrial septal defect may be seen on color flow imaging as a jet of blood from the left atrium to the right atrium.  Transcranial Doppler (TCD) Bubble study- This method reveals the cerebral impact of the ASD or PFO.  Electrocardiogram- Individuals with atrial septal defects may have a prolonged PR interval (a first degree heart block).  Chest x-ray - a diagnostic test which uses invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs onto film. With an ASD, the heart may be enlarged because the right atrium and ventricle have to handle larger amounts of blood flow than normal. NURSING DIAGNOSIS  Activity intolerance  Decreased cardiac output  Deficient knowledge (diagnosis and treatment) 

Fatigue  Impaired gas exchange  Risk for infection

EXPECTED OUTCOMES FOR NURSING CARE PLAN  The patient will carry out activities of daily living without weakness or fatigue.  The patient will maintain hemodynamic stability, and cardiac output will remain adequate. 

The patient or her parents will verbalize understanding of the atrial septal

defect and plans for treatment. 
The patient will report that she has more energy.  The patient will maintain adequate ventilation and oxygenation.  The patient will remain free from signs and symptoms of infection.

TREATMENT/MANAGEMENT  Surgery- to close the defect for children 1-3 years of age. This is to prevent risk for infectious endocaditis and eventual heart failure.  Cardiac catheterization- technique if the defect is small wherein the edge of the opening of the septum is sutured.  Open heart surgery and cardiopulmonary bypass- for large defects. POST-OPERATIVE CARE 
Ventilator - a machine that helps your child breathe while he/she is under    

  

anesthesia during the operation. Intravenous (IV) catheters - small, plastic tubes inserted through the skin into blood vessels to provide IV fluids and important medications that help your child recover from the operation. Arterial Nasogastric (NG) tube - a small, flexible tube that keeps the stomach drained of acid and gas bubbles that may build up during surgery. Heart monitor - a machine that constantly displays a picture of your child's heart rhythm, and monitors heart rate, arterial blood pressure, and other values. Closely monitor vital signs, central venous and intra-arterial pressures, and intake and output. Watch for atrial arrhythmias. Give an antibiotic and an analgesic, as ordered. Provide range-of-motion exercises and coughing and deep-breathing exercises.

PROGNOSIS With a small to moderate atrial septal defect, a person may live a normal life span without symptoms. Larger defects may cause disability by middle age because of increased blood flow and shunting of blood back into the pulmonary circulation. Some patients with ASD may have other congenital heart conditions, such as a leaky valve.

2.) VENTRICULAR SEPTAL DEFECT

DEFINITION A ventricular septal defect is an abnormal opening in the wall (septum) that divides the two lower chambers of the heart (ventricles). A Ventricular septal defect closure is a procedure performed to correct this defect. ETIOLOGY The cause of VSD (ventricular septal defect) includes the incomplete looping of the heart during days 24-28 of development. Faults with NKX2.5 gene can cause this. Congenital VSDs are frequently associated with other congenital conditions, such as Down syndrome DIAGNOSIS Cardiac auscultation- VSD causes a pathognomonic holo- or pansystolic murmur. Auscultation is generally considered sufficient for detecting a significant VSD. Ultrasound (echocardiography) CLINICAL MANIFESTATION  Tachypnea is typically the first presenting symptom.  Dyspnea results in poor nursing and frequent rest during feedings  Hepatomegaly may be present.  The murmur of VSD is due to left-to-right shunting at the ventricular level. Small ventricular septal defects are typically louder than larger ones. The murmur of a VSD is heard best at the left lower sternal border.  Right-to-left shunting at the VSD are not audible due to a small amount of pressure difference between the right and left ventricles.  A loud third heart sound or diastolic rumble is heard with large left-to-right shunting due to increased flow across the mitral valve.  A thrill is felt in many cases, particularly beyond infancy.

NURSING DIAGNOSIS  Alteration in tissue perfusion  Risk for infection  Fatigue 

Weakness.  Ineffective breathing pattern

SIGNS AND SYMPTOMS  Pansystolic (Holosystolic)  Heart sounds are normal. murmur (depending upon the  sweaty and tachypnoiec size of the defect) (breathe faster) with feeds  +/- Palpable thrill (palpable  easy fatigue turbulence of blood flow). TREATMENT/MANAGEMENT  If the opening is SMALL- 85% closes spontaneously.  MODERATE- cardiac catheterization, Cardiopulmonary bypass, the edges of the septal opening is sutured.  LARGE- (over 3 mm) open heart surgery, Silastic or Dacron patch is sutured to occlude the space. PRE-OPERATIVE TEACHING:  If at all possible, it is important that the patient be free of infection prior to going to surgery.  If the patient is due for immunizations within a week of surgery, contact the Congenital Heart Surgery Clinic and ask to speak to the clinic nurse  Patients undergoing cardiac surgery frequently need blood products  Provide emotional support to the family.  Signing of consent. POST-OPERATIVE TEACHING  Watch out for the following: redness, swelling, or oozing/bleeding from incision, fever, altered mental status, excessive fatigue, feeding/eating problems, prolonged or worsening pain  Avoid activities or movement for 4-6 weeks. PROGNOSIS This is excellent for most patients. The vast majority are able to live a normal and unrestricted life. Re-operations for residual VSDs are now uncommon.

3.) PATENT DUCTUS ARTERIOSUS

DEFINITION Is a congenital disorder in heart wherein a neonate's ductus arteriosus fails to close after birth. The condition leads to abnormal blood flow between the aorta and pulmonary artery, two major blood vessels surrounding the heart. The ductus arteriosus (DA) is the vascular connection between the pulmonary artery and the aortic arch. ETIOLOGY Before birth, the ductus arteriosus allows blood to bypass the baby's lungs by connecting the pulmonary arteries (which supply blood to the lungs) with the aorta (which supplies blood to the body). Soon after the infant is born and the lungs fill with air, this blood vessel is no longer needed. It will usually close within a couple of days. If the ductus arteriosus does not close, there will be abnormal blood circulation between the heart and lungs. RISK FACTOR PDA is rare. It affects girls more often than boys. The condition is more common in premature infants and those with neonatal respiratory distress syndrome. Infants with genetic disorders, such as Down syndrome, and whose mothers had German measles (rubella) during pregnancy are at higher risk for PDA. SIGNS AND SYMPTOMS  Bounding pulse  Fast breathing  Poor feeding habits  Shortness of breath  Sweating while feeding  Tiring very easily  Poor growth 

Wide pulse pressure  Murmur can be heard in upper left sternal border or under the left clavicle

(older children)  Short grade II and III harsh systolic sound (newborn)  Ventricle enlargement DIAGNOSIS  AUSCULTATION- murmur  Echocardiogram. An echocardiogram uses sound waves to produce a video image of the heart. This image can help doctors see the heart chambers and evaluate how well the heart is pumping. This test also checks the heart valves and looks for any other heart defects.  Chest X-ray. An X-ray image helps the doctor see the condition of your baby's heart and lungs and the amount of blood in the lungs  Electrocardiogram (ECG). This test records the electrical activity of the heart. This test helps diagnose heart defects or rhythm problems.  Cardiac catheterization. This test isn't usually necessary for diagnosing a PDA alone, but may be done to examine other congenital heart defects found during an echocardiogram  Cardiac computerized tomography (CT) or magnetic resonance imaging (MRI). TREATMENT  PD is open because of stimulation of prostaglandins (PGE1) from the placenta and decrease O2 of fetal blood. If PGE1 decreases and O2 increases PD is stimulated to close.  If PD doesn¶t close spontaneously IV INDOMETHACIN and ibuprofen, prostaglandin inhibitors are given.Side effects: Decrease glomelular filtration, impaired platelet aggregation, and diminished G.I. and Cerebral blood flow.  Dacron-coated staimless steel coils by interventional cardiac catheterization (6 mos-1 yr).  LARGE DUCTAL LIGATION  TRANSCATHETER DEVICE CLOSURE

(Cardiomegaly and Pulmonary edema). 22 days of life, before the surgery.

Following surgical ligation of PDA, there is improving edema and less cardiomegaly. PROGNOSIS Adults and children can survive with a small opening remaining in the ductus arteriosus. Treatment, including surgery, of a larger PDA is usually successful and frequently occurs without complications. Proper treatment allows children and adults to lead normal lives.

4.) COARCTATION OF AORTA

DEFINITION Is a narrowing of the aorta, the large blood vessel that branches off your heart and delivers oxygen-rich blood to your body. When this occurs, your heart must pump harder to force blood through the narrow part of your aorta. Coarctation of the aorta usually occurs beyond the blood vessels that branch off to your upper body and before the blood vessels that lead to your lower body. This often means you'll have high blood pressure in your arms, but low blood pressure in your legs and ankles. ETIOLOGY AND RISK FACTOR The aorta carries blood from the heart to the vessels that supply the body with blood and nutrients. If part of the aorta is narrowed, it is hard for blood to pass through the artery. Aortic coarctation is more common in persons with certain genetic disorders, such as Turner syndrome. However, it can also be due to birth defects of the aortic valves. Aortic coarctation is one of the more common heart conditions that are present at birth (congenital heart conditions). It is usually diagnosed in children or adults under age 40. SIGNS AND SYMPTOMS BABIES WITH SEVERE COARCTATION  Pale skin  Irritability OLDER CHILDREN  High blood pressure 

Heavy sweating  Difficulty breathing  Shortness

of breath, especially during exercise 

Headache  Muscle weakness  Leg cramps or cold feet 

Nosebleeds

DIAGNOSIS/DIAGNOSTIC PROCEDURE  HISTORY and PHYSICAL ASSESSMENT  The pulse in the femoral (groin) area or feet will be weaker than the pulse in the arms or the carotid (neck). Sometimes, the femoral pulse may not be felt at all.  The blood pressure in your legs is usually weaker than in the arms. Blood pressure is usually higher in the arms after infancy. BP in arms is 20mmhg higher than in the leg 

Echocardiography  ECG-show that you

might have a thickened heart muscle (ventricular hypertrophy).  X-ray may show an enlarged heart or a narrowing in the aorta at the site of the coarctation. Left-sided heart enlargement.  MRI- reveals the location of the coarctation of the aorta.  MRI or MR angiography of the chest may be needed in older children

ASSESSMENT  Slight Coarctation- absent of palpable femoral pulse  Obstruction is proximal- absent of brachial pulse  As infant grow older- leg pain on exertion due to diminish blood supply to lower extremities TREATMENT/MANAGEMENT  Surgical resection of the narrow segment if there is arterial hypertension.  angioplasty NURSING DIAGNOSIS  Fatigue  Activity intolerance  Ineffective breathing pattern secondary to pulmonary hypertension 

Cardiac

catheterization and aortography  DACRON graft  Balloon Angioplasty 
sleep deprivation secondary

to discomfort and irritability

PROGNOSIS Coarctation of the aorta can be cured with surgery. Symptoms quickly get better after surgery. However, there is an increased risk for death due to heart problems among those who have had their aorta repaired. Without treatment, most people die before age 40. For this reason, doctors usually recommend that the patient has surgery before age 10. Most of the time, surgery to fix the coarctation is done during infancy. Narrowing or coarctation of the artery can

return after surgery. This is more likely in persons who had surgery as a newborn. CYANOTIC HEART DEFECT Definition: y y y Blood is shunted from the venous to the arterial system as a result of abnormal communication between the two Deoxygenated blood to oxygenated blood Right to left shunt

Types: y y Transposition of great artery Tetralogy of fallot

TRANSPOSITION OF GREAT ARTERY Definition: y y y y y y Defect with mixed blood flow Aorta arises from right ventricle instead of left Pulmonary artery arises from left ventricle instead of right Atrial and ventricular septal defects occur in connection with transposition making the entire heart one mixed circulatory system Large newborn(9-10lbs), more on boys 5% of congenital anomalies

Etiology/Pathophysiology: The pulmonary and systemic circulations function in parallel, rather than in series. Oxygenated pulmonary venous blood returns to the left atrium and left ventricle but is re-circulated to the pulmonary vascular bed via the abnormal pulmonary arterial connection to the left ventricle. Deoxygenated systemic venous blood returns to the right atrium and right ventricle where it is subsequently pumped to the systemic circulation, effectively bypassing the lungs. This parallel circulatory arrangement results in a deficient oxygen supply to the tissues and an excessive right and left ventricular workload. It is incompatible with prolonged survival unless mixing of oxygenated and deoxygenated blood occurs at some anatomic level.

Signs and Symptoms: y y Cyanotic from birth No murmurs/ various murmurs

Laboratory/Diagnostic Findings: y y y Echocardiography reveals enlarged heart ECG may or may not reveal enlarged heart Decreased oxygen saturation in cardiac catetherization

Management: y y y y y Patent ductus arteriosus PGE administration Balloon atrial septal pull through operation Cardiac catheterization Arterial switch procedure (1week to 3 mos, 95%)

Nursing diagnosis: 1. Ineffective cardiopulmonary tissue perfusion related to impaired cardiac function and increased cardiac workload. 2. Excess fluid volume related to impaired cardiac contractility and venous congestion 3. Activity intolerance related to effects of heart failure. 4. Risks for injury related to congenital heart defect and surgery. 5. Risks for infection related to immature immune system and neonatal age. 6. Risks for imbalanced nutrition, less than body requirements. Implementation 1. Administer supplemental oxygen. 2. Elevate head of the bed 30 to 60 degrees or have child sit upright. 3. Assess vital signs including heart rate, pulse and respirations. Auscultate heart and lung sounds. 4. Obtain baseline weight and monitor at least daily. 5. Monitor intake and output and urine specific gravity. 6. Provide a balance of activity and rest periods. 7. Provide small and frequent meals. 8. Assesses infant's skin integrity; sensory impairments, and immune status. 9. Implements protective measures to prevent injury caused by electrical, thermal, chemical, or physical sources, including

* verifying allergies, * applying safety devices, * ensuring prep electrosurgical grounding pad, solution does not run under

* performing required counts, and * using supplies and equipment within safe parameters. Prognosis: The child's symptoms will improve after surgery to correct the defect. Most infants who undergo arterial switch do not have symptoms after surgery and live normal lives. If corrective surgery is not performed, the life expectancy is months.

TETRALOGY OF FALLOT Definition: y y y Defect with decreased pulmonary blood flow 10% of children with congenital disease Four anomalies: pulmonary stenosis VSD Dextraposition of aorta Hypertrophy of right ventricle 15% of children with this disorder show deletion abnormality of chromosome22

y

Etiology/Pathophysiology:  Pulmonary stenosis A narrowing of the right ventricular outflow tract and can occur at the pulmonary valve (valvular stenosis) or just below the pulmonary valve (infundibular stenosis). Infundibular pulmonic stenosis is mostly caused by overgrowth of the heart muscle wall (hypertrophy of the septoparietal trabeculae), however the events leading to the formation of the overriding aorta are also believed to be a cause. The pulmonic stenosis is the major cause of the malformations, with the other associated malformations acting as compensatory mechanisms to the pulmonic stenosis. The degree of stenosis varies between individuals with TOF, and is the primary determinant of symptoms and severity. This malformation is infrequently described as sub-pulmonary stenosis or subpulmonary obstruction. 

Overriding aorta An aortic valve with biventricular connection, that is, it is situated above the ventricular septal defect and connected to both the right and the left ventricle. The degree to which the aorta is attached to the right ventricle is referred to as its degree of "override." The aortic root can be displaced toward the front (anteriorly) or directly above the septal defect, but it is always abnormally located to the right of the root of the pulmonary artery. The degree of override is quite variable, with 5-95% of the valve being connected to the right ventricle.  Right ventricle hypertrophy The right ventricle is more muscular than normal, causing a characteristic bootshaped (coeur-en-sabot) appearance as seen by chest X-ray. Due to the misarrangement of the external ventricular septum, the right ventricular wall increases in size to deal with the increased obstruction to the right outflow tract. This feature is now generally agreed to be a secondary anomaly, as the level of hypertrophy generally increases with age.  Ventricular septal defect A hole between the two bottom chambers (ventricles) of the heart. The defect is centered around the most superior aspect of the ventricular septum (the outlet septum), and in the majority of cases is single and large. In some cases thickening of the septum (septal hypertrophy) can narrow the margins of the defect. Signs and Symptoms: y y y y y y y y y May not exhibit high degree of cyanosis immediately Bluish tint Polycythemia Severe dyspnea Growth restriction Clubbing of fingers Fainting Hypoxic episode (tet spell) Cognitive challenge

Laboratory/Diagnostic Findings: y y Increased Hg, Hct Echocardiography shows enlarged chamber of right heart

y y y y y

Echocardiography shows decrease size in pulmonary artery and reduced blood flow through lungs. ECG shows enlarged chamber of right heart Cardiac catheterization and angiography evaluate extent of defect Polycythemia Reduced oxygen saturation

Management: y y y y y Propranolol Administer oxygen Knee chest position Blalock taussig Brock procedure

Nursing diagnosis: 1. Decreased cardiac out put related to structural defect. 2. Activity intolerance related to imbalance between oxygen supply and demand. 3. Altered growth and development related to inadequate oxygen, nutrients to tissue and social isolation. 4. High risk for infection related to debilitated physical status. 5. Altered family process related to having a child with a heart condition. 6. High risk for injury (complications) related to cardiac condition and therapies. Implementation 1. Administer supplemental oxygen. 2. Elevate head of the bed 30 to 60 degrees or have child sit upright. 3. Assess vital signs including heart rate, pulse and respirations. Auscultate heart and lung sounds. 4. Allow time for frequent of rest. 5. Help child to select activities appropriate to age, condition and capabilities. 6. Avoid extremes of environmental temperature. 7. Provide well balanced highly nutritive diet. 8. Avoid contact with infected persons. 9. Discuss with parents their fears regarding child symptoms. 10. Encourage family to participate in care of child while hospitalized. 11. Encourage family to include others in child¶s care to prevent their own exhaustion.

Prognosis: Most cases can be corrected with surgery. Babies who have surgery usually do well. Ninety percent survive to adulthood and live active, healthy, and productive lives. Without surgery, death usually occurs by the time the person reaches age 20. Patients who have continued, severe leakiness of the pulmonary valve may need to have the valve replaced. Regular follow-up with a cardiologist to monitor for life-threatening arrhythmias (irregular heart rhythms) is recommended TRANSPOSITION OF GREAT ARTERIES

TETRALOGY OF FALLOT

OVERRIDING OF AORTA

Acute Rheumatic Fever Background of Case - Autoimmune disease that occurs as a reaction to a group A betahemolytic streptococcal infection - Follows attack of pharyngitis, tonsillitis, scarlet fever, ³strep throat´, or impetigo - Inflammation from immune system will lead to fibrin deposits on endocardium and valves , and body joints Etiology - group A beta-hemolytic streptococcal infection (GABS) Laboratory findings 1. High ESR 2. Anemia, leucocytosis 3. Elevated C-reactive protein 4. ASO titre >200 Todd units.(Peak value attained at 3 weeks,then comes down to normal by 6 weeks) 5. Anti-DNAse B test 6. Throat culture-GABHstreptococci 7. ECG- prolonged PR interval, 2nd or 3rd degree blocks, ST-depression, Tinversion 8. 2D Echo cardiography- valve edema,mitral regurgitation, LA & LV dilatation, pericardial effusion, decreased contractility Signs and Symptoms  Major: 1. Subcutaneous nodules 2. Pancarditis 3. Arthritis 4. Chorea 5. Erythema marginatum  Minor 1. Fever 2. Arthralgia 3. Previous rheumatic fever attacks 4. All that is stated in the laboratory findings (Using Jones Criteria)*the presence of 2 MAJOR criteria or of 1 MAJOR and 2 MINOR criteria indicates a high probability of acute rheumatic fever, if supported by evidence of Group A streptococcal infection

NANDA Problems 1. Nursing Diagnosis: Risk for non-adherence to drug therapy related to knowledge deficit about importance of long-term therapy Outcome Evaluation: child takes oral penicillin daily; absence of symptoms of throat infection; vital signs are within age-acceptable parameters. 2. Nursing Diagnosis: Situational low-esteem related to chorea movements secondary to rheumatic fever Outcome Evaluation: child expresses frustration with inability to control movements; continues to feed and dress self with help as needed. Children may have difficulty feeding themselves because of chorea. They may be also be emotionally unstable and cry easily. Emphasize the transitory nature of the chorea; stress that is frustrating to have to be fed and to be unable to use your hands meaningfully, but that is lack of coordination will pass without permanent effects. Provide toys and games that do not require fine coordination, because it may be frustrating to try to do something such as move checkers or chessmen on a board (a typical low activity game). Children with chorea who are on bed rest may need to have bedrails padded so they do not injure themselves with thrashing movements. Nursing and Medical Management 1. Bed Rest 2. Monitor vital signs 3. Penicillin Therapy/ single intramuscular injection of benzathine penicillin 4. Oral ibuprofen 5. Corticosteroids: SE- Hirsutism, Cushing¶s Syndrome 6. Phenobarbital and Diazepam Prognosis - Rheumatic fever can recur whenever the individual experience new GABH streptococcal infection, if not on prophylactic medicines - Good prognosis for older age group & if no pancarditis during the initial attack - Bad prognosis for younger children & those with pancarditis with valvar lesions

KAWASKI DISEASE (Mucocutaneous lymph node syndrome) Definition: y The incidence is higher in late y A febrile disease winter and spring y Multisystem disorder almost y Unknown cause exclusively in children before the age of puberty. y Develops in genetically y The peak incidence is in boys predisposed clients under 4 years old Etiology/Pathophysiology: y Unknown Etiology y Pathophysiology 1. After the infection (upper respiratory infection) 2. Altered immune function occurs 3. Increase in the antibody production 4. Creates circulating immune complexes that bind 5. to the endothelium and cause inflammation Signs and Symptoms: ‡ High fever( 102 to 104 F [39 to 40 C]) Does Not Respond toAntipyretic ‡ Child acts lethargic/irritable ‡ May have reddened and swollen hands & feet ‡ Bulbar mucous membrane of the eyes become inflamed (Conjunctivitis) Laboratory/Diagnostic Findings: y WBC and ESR are both elevated Progression of the Disease: 10 days after onset(subacute phase) ‡ The skin desquamates (palms and soles) ‡ Platelet count rises (increased clotting necrosis of distal body cells, particularly in the fingertips)

6. The inflammation of the blood vessels (Vasculitis) leads to: y Aneurysms y Platelet accumulation y Formation of Thrombi y Obstruction in the heart and blood vessels

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Strawberry tongue and red, cracked lips Rashes occur(diaper area) Cervical lymph node become enlarged Internal lymph nodes swell May develop abdominal pain, anorexia and diarrhea Joints may swell and redden(simulating arthritic process)

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Aneurysms may form in coronary arteries(CHA) Sudden death from accumulating thrombi or rupture of an aneurysm (MOST DANGEROUS PHASE)

25 days after onset (Convalescent Phase) ‡ Begins @ about the 25th day and last until 40 days STAGE III last from 40 days until ESR returns to normal Management: y Administration of acetysalicylic acid or ibuprofen (dec I & PA) y Abciximab ± platelet receptor inhibitor specific for KD y IV immune globulin can also be administed (reduce immune response) y Coronary artery bypass surgery (CAD from stenosis of the Coronary Arteries) NOTE: *Steriods CONTRAINDICATED (increase aneurysm formation)* Nursing diagnosis: 1. Conjunctivitis: "L & R eye redness and yellow drainage" (Doesn't need evidence. This IS the evidence) 2.Gingivitis: "Gum irritation M/B redness and swelling at upper and lower gums" 3.Rash: "Impaired Skin Integerity at bilateral hands M/B red rash with exfoliation" 4."Impaired Skin Integrity at bilateral feet M/B red rash with exfoliation" 5.Hand edema: "Impaired Tissue Integrity M/B 2+ pitting edema at bilateral hands" 6.Foot edema: "Impaired Tissue Integrity M/B 3+ pitting edema at bilateral feet" Joint inflammation: "Pain M/B warmth, redness and swelling at bilateral knees and pt. states 6/10 pain on 0-10 P/S" Implementation Monitoring 1. Monitor pain level and child¶s response to analgesics. 2. Institute continual cardiac monitoring and assessment for complications; report arrhythmias. o Take vital signs as directed by condition; report abnormalities. o Assess for signs of myocarditis (tachycardia, gallop rhythm, chest pain). o Monitor for heart failure (dyspnea, nasal flaring, grunting, retractions, cyanosis, orthopnea, crackles, moist respirations, distended jugular veins, edema). Closely monitor intake and output, and administer oral and I.V fluids as ordered. Monitor hydration staus by checking skin turgor, weight, urinary output, specific gravity, and presence of tears.

Observe mouth and skin frequently for signs of infection.

Supportive care 1. Allow the child periods of uninterrupted rest. Offer pain medication routinely rather than as needed during stage I. Avoid NSAIDS if the child is in aspirin therapy. 2. Perform comfort measures related to the eyes. o Conjunctivities can cause photosensitivity, so darken the room, offer sunglasses. o Apply cool compress. o Discourage rubbing the eyes. o Instill artificial tears to soothe conjunctiva. 3. Monitor temperature every 4 hours. Provide sponge bath if temperature above normal. 4. Perform passive range of motion exercises every 4 hours while the child is awake because movement may be restricted. 5. Provide quiet and peaceful environment with diversional activities. 6. Provide care measures for oral mucous membrane. o Offer cool liquids like ice chips and ice pops. o Use soft toothbrush only. o Apply petroleum jelly to dried, cracked lips. 7. Provide skin measures to improve skin integrity. o Avoid use of soap because it tends to dry skin and make it more likely to breakdown. o Elevate edematous extremities. o Use smooth sheets. o Apply emollients to skin as ordered. o Protect peeling of skin, observe for signs of infection. 8. Offer clear liquids every hour when the child is awake. 9. Encourage the child to eat meals and snack with adequate protein. 10. Infuse I.V fluids through a volume control device if dehydration is present, and check the site and amount hourly. 11. Explain all procedures to the child and family. 12. Encourage the parents and child to verbalize their concerns, fears, and questions. 13. Practice relaxation techniques with child, such as relaxation breathing, guided imagery, and distraction. 14. Prepare the child for cardiac surgery or thrombolytic therapy if complications develop. 15. Keep the family informed about progress and reinforce stages and prognosis.

Prognosis: A large majority of children who develop Kawasaki disease recover within two weeks and experience no long-lasting effects. As many as 25 percent of sufferers may develop heart problems, but that percentage drops dramatically to below 5 percent with quick treatment. Only about 1 percent of cases prove fatal, but because that possibility exists, you must know what symptoms to watch for and what steps to take to prevent immediate and future complications.

Infective Endocarditis Endocarditis is inflammation and infection of the endocardium or valves of the heart. It may occur in a child without heart disease but more commonly occurs as a complication of congenital heart disease such as tetralogy of Fallot, VSD, or coarctation of the aorta. Bacteria or other infectious substance can enter the bloodstream during certain medical procedures, including dental procedures, and travel to the heart, where it can settle on damaged heart valves. The bacteria can grow and may form infected clots that break off and travel to the brain, lungs, kidneys, or spleen. Etiology: Streptococci of the viridans type Pathophysiology: A high-velocity flow through a stenotic or incompetent valve or an abnormal communication between systemic and pulmonary circulations causes turbulence downstream from the opening. This turbulence damages or denudes the endothelium, to which platelets and fibrin adhere, and a small, sterile" nonbacterial thrombotic endocardial lesion" forms. In addition, indwelling intravascular catheters in the right heart may directly traumatize the endocardium or valvular endothelium. Circulating bacteria and inflammatory cells adhere to and grow in these thrombi, forming infected vegetation. Infection may occur on the wall, where the turbulent jet strikes, or downstream, near the orifice, where the flow eddies. Once vegetation forms, the constant blood flow may result in embolization to virtually any organ in the body. A brisk immunologic response is produced. Manifestation:  Paleness  Anorexia  weight loss  Chills  Arthralgia  Sweating at night  murmur become audible  petechiae in conjunctiva  RUQ abdominal pain

Laboratory Test  CBC count: Anemia is present in 70-90% of patients and is usually normocytic and normochromic. Leukocytosis is noted in 20-30% of patients.  ESR and C-reactive protein level: The ESR is elevated in almost all patients except for those with congestive heart failure (CHF), renal failure, and disseminated intravascular coagulation (DIC). The mean ESR is 55 mm/h. The C-reactive protein, although nonspecific, is elevated in most patients but decreases with successful treatment. Levels of C-reactive protein may be used to monitor response to antibiotic therapy..  Urinalysis may reveal proteinuria (50-60%) and/or microscopic hematuria (30-50%). Treatment Prophylactic administration antibiotic Penicillinase-resistant penicillin (Nafcillin) UNIPEN via IV (cvc) Nursing Diagnosis:  Decreased cardiac output related to congenital structural disorder  Ineffective tissue perfusion related to inadequate cardiac output Prognosis: The prognosis of bacterial endocarditis varies with the etiologic agent. Infection by a penicillin-sensitiveStreptococcus, diagnosed early, has a cure rate of almost 100%. Because many infections are diagnosed late or due to resistant organisms, the average mortality rate is approximately 20-25%.

UTI- Urethritis Background: Urethritis is inflammation of the urethra. The main symptom is dysuria, which is painful or difficult urination. Etiology: >gonococcocal urethritis Other causes include:  Adenovirus  Uropathogenic Escherichia coli (UPEC)  Herpes simplex 

  

Mycoplasma genitalium Reiter's syndrome Trichomonas spp. Ureaplasma urealyticum

Signs and symptoms/ Clinical Manifestations: >In men- purulent discharge >Dysuria >Frequency Labs and Diagnostics: >Culture and sensitivity testing Nursing Management: >Removal of etiologic agent- by administering doctor-prescribed systemic and topical antibiotics

>Sitz bath >Increases fluid intake >Advise client to avoid coitus

NANDA problems: Impaired Urinary elimination r/t irritation and inflammation of the urethral mucosa Acute pain r/t irritation and inflammation of the urethral mucosa Prognosis: With the correct diagnosis and treatment, urethritis usually clears up without any complications. However, urethritis can lead to permanent damage to the urethra (scar tissue called urethral stricture) and other urinary organs in both men and women.

UTI: Cystitis Background: Is a term that refers to urinary bladder inflammation that results from any one of a number of distinct syndromes. It is most commonly caused by a bacterial infection in which case it is referred to as a urinary tract infection Etiology: >gram negative bacteria (E. coli, Klebsiella, Enterobacter, Proteus) >Candida spp. >Chlamydia trachomitis, Trichomonas vaginalis, Neiseria gonorrhea Signs and symptoms/ Clinical Manifestations: >change in voiding habits >voiding in small amounts >burning pain on urination (dysuria) >incomplete bladder emptying >frequency >hematuria >urgency Labs and Diagnostics: >urine culture >dipstick test Nursing Management: >Inhibit bacterial growth- give adequate instructions about antibiotics therapy and dietary and activity restrictions. >Advice patient to Modify Diet- dietary changes needed to keep urine acidic and to reduce bladder irritation by avoiding spicy foods, caffeinated and alcoholic beverages > Advice patient to Increase fluid intake- to flush urinary system >Prevent complications- educate client about inc manifestations that might result from infection of the upper UT NANDA problems: Impaired Urinary elimination r/t irritation and inflammation of the bladder mucosa Acute pain r/t irritation and inflammation of the bladder and mucosa Prognosis: The prognosis for recovery from uncomplicated cystitis is very good. With proper treatment, the infection usually clears up quickly. In many cases, the condition may reoccur. However, it can be treated in essentially the same way each time it appears. More complicated infections in men may be difficult to treat if antibiotics are not able to clear up the problem.

Enuresis (bed wetting) y Background  Enuresis is an involuntary passage of urine past the age when a child should be expected to have attained bladder control Etiology  Unclear  Possible causes: anatomical malformation of kidneys and bladder; lack of Anti-diuretic hormone secretion; mental disorder Classification according to time of urine passage  Nocturnal- at night  Diurnal- at morning Major types  Primary- occurs when the child never establish bladder control  Secondary- occurs when a person acquires bladder control for the past 6 months then having relapses and started bed wetting. Clinical Findings  5-7 years old  Most common in boys Laboratory and Diagnostic Findings  Abnormal ECG patterns  IVP  VCUG  UTz Management  If the cause is stress; stress modification is recommended  Limit fluids after dinner  Administration of synthetic Anti-diuretic hormone  Desmopressin Nursing Management  Urge parents to exercise common sense  Caution parents of children with sickle-cell anemia not to restrict fluid because sickling of RBC is increased in case of dehydration  Advise bladder stretching exercises by drinking lots of water and trying not to void as long as possible to increase functional size of the bladder

y

y

y

y

y

y

y

Prognosis  Good if readily prevented Acute Glomerulonephritis y y Background Acute Glomerulonephritis is the sudden inflammation of the glomeruli of the kidney. Etiology  History of recent infection to Group A Beta-Hemolytic Streptococcus such as: tonsillitis, otitis media, strep throat and impetigo  Tissue damage from complement fixation reaction in the glomeruli Clinical Findings  B- oys  R- ecent respiratory infection (7-14days)  A- ges 5-10  S- pring and winter seasons  S- trep infection Signs and Symptoms  H- ematuria  E- dema Periorbital area  L- ow grade fever  P-roteinuria  V-omiting  A- norexia  H- eadache  A-bdominal pain Laboratory and Diagnostic Findings  CXR & UTz- hepatocardiomegaly, pulmonary edema  ECG- Galloping HR, T wave inversion, prolong P-R interval, orthopnea  Urinalysis: RBC casts, WBC, epithelial cells, hyaline and granules are present; Increase CRea and BUN  Blood analysis: Antistreptolysin O Albumin Serum Complement Hct & Hgb RBC Sedimentation

y

y

y

y

y

Nursing Management  Bed rest  Diet: Protein; Sodium or DAT with normal sodium content  Health teachings  Positioning semi fowlers  Digitalis  Oxygen therapy 

Weighing  I & O monitoring

y

Nursing Diagnosis  Situational low self-esteem related to feelings of responsibility for onset of serious illness. Prognosis  Good if readily prevented

y

Nephrotic Syndrome y Background  Nephrotic syndrome is an abnormal loss of protein in urine involving immunologic mechanism that may be caused by hypersensitivity to an antigen-antibody reaction or an immune process. y Etiology  hypersensitivity to an antigen-antibody reaction Pediatric Nephrotic Syndrome 1. Congenital- autosomal recessive disorder (rare) 2. Secondary- as progression of glomerulonephritis, sickle cell anemia or SLE 3. Idiopathic- primary; acquired; common y Classification of NS according to Membrane Destruction  Minimal Change Nephrotic Syndrome  Focal Segmental Glomerulosclerosis  Membranoproliferative glomerulonephritis Signs and Symptoms  P- roteinuria  I- ncrease blood lipid  D- ecrease serum albumin  E- dema Periorbital area and abdomen (ascites) Laboratory and Diagnostic Findings  + Proteinuria  Entirely albumin  Minimal Hematuria  RBC sedimentation  Dx Pocedure: RENAL BIOPSY Management

y

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y 

Supportive-Symptomatic (no cure) FOCUS: Reducing proteinuria and edema  Meds: Corticosteroids IV Methylprednisone: until diuresis w/o protein loss Oral Prednisone: SE- halt growth and suppress adrenal gland secretion  Keeping child free from infection  Instruct parents to test the first urine of the day for protein. Approx. 1x a wk.  Instruct to alternate drug therapy If diuretics are necessary WOF: tendency for Hypo-K  Diet: may need to K & K-supplementation  Albumin Infusion  If prednisone resistant (w/ FGS & MPGN):  cyclophosphamide (anti-neoplastic)  Mycophenolate  Cyclosporine **Rationale: NS is an autoimmune disorder **Nsg. Consideration: Should take with adequate fluid intake to avoid bladder irritation and bleeding. y Nursing Diagnosis  Imbalance nutrition less than body requirements related to poor appetite, restricted diet and protein loss Prognosis  MCNS- responds to steroids  FGS & MPGN- 2 relapses at irregular intervals for several years

y

Hemolytic-Uremic Syndrome y Background  Hemolytic-Uremic Syndrome is the inflammation of glomerular arterioles because of occlusion with particles of fibrin and platelets. Etiology  Recent E. coli GI infection Clinical Findings  Summer  6 mos. ± 4 years  Transient diarrhea severe fluid loss and bowel wall necrosis  Fever stupor and hallucination  Oliguria proteinuria, hematuria and protein casts  Edema  Pale  Petichiae thrombocytopenia Laboratory and Diagnostic Findings  BUN and Creatinine Management  Symptomatic Approach  FOCUS: to maintain heart and kidney function  Oliguria- Peritoneal dialysis  Anemia- Blood transfusion Nursing Management  Educate parents about the need for P.D.  Ensure parents understand the importance of follow up care. Prognosis  Good

y

y

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y

Bladder Exstrophy- Epispadia Complex Background: Bladder exstrophy-epispadia complex is a congenital abnormality in which part of the urinary bladder is present outside the body. It is rare, occurring once every 30,000 live births with a 2:1 male:female ratio. The diagnosis involves a spectrum of anomalies of the lower abdominal wall, bladder, anterior bony pelvis, and external genitalia. It occurs due to failure of the abdominal wall to close during fetal development and results in protrusion of the posterior bladder wall through the lower abdominal wall. Etiology: The cause of bladder exstrophy is maldevelopment of the lower abdominal wall, leading to a rupture which causes the bladder to communicate with the amniotic fluid. Signs and symptoms / Clinical manifestations: The typical manifestations of exstrophy-epispadias complex are:  bladder everted through a midline lower abdominal wall defect  widening of the pubic symphysis  epispadias in males (dorsal cleft in the penis, exposing the urethral mucosa)  the anus and vagina appear anteriorly displaced  The testicles may be undescended.  Bifid clitoris in females, with a short "urethral strip" indistinguishable from bladder mucosa. The spectrum of disease extends from spade penis and epispadias on one hand, to exstrophy with cloaca (also known as cloacal exstrophy). Nursing Management: y In neonates with exstrophy and epispadias, initiate general supportive care appropriate for the overall condition and associated anomalies. y Institute parenteral nutrition early for patients with cloacal exstrophy. y Place clean plastic wrap over the bladder plate. Avoid moistened or impregnated gauze, which is irritating to the delicate bladder mucosa. Mist tents may be used to protect exposed tissue. y Start antibiotic therapy with doctor¶s order after delivery and continue through the early postoperative period. y Daily prophylactic antibiotic therapy may be continued in the weeks after bladder closure. Surgeon's philosophy on this matter varies. y Infections may be related to poor emptying and are to be prevented in light of the high incidence of vesicoureteral reflux. Institute latex precautions due to high incidence of latex sensitization in patients with exstrophyepispadias complex.

NANDA problems: Impaired urinary elimination r/t anatomical malformation of the bladder and ureters Risk for infection r/t broken skin Prognosis: Even with successful surgery, patients may have long-term problems with  incontinence  urinary reflux (see Vesicoureteral_reflux)  repeated urinary tract infections  bladder adenocarcinoma  colonic adenocarcinoma  sexual dysfunction  pain  uterine prolapse

Polycystic Kidney Disease Background: It occurs in humans and some other animals. PKD is characterized by the presence of multiple cysts (hence, "polycystic") in both kidneys. The cysts are numerous and are fluid-filled resulting in massive enlargement of the kidneys. The disease can also damage the liver,pancreas, and in some rare cases, the heart and brain. The two major forms of polycystic kidney disease are distinguished by their patterns of inheritance. Polycystic Kidney Disease is the most common genetic, life threatening disease affecting an estimated 12.5 million people worldwide Etiology: It is an autosomal recessive trait, and both parents must have carried the gene. Signs and symptoms/ Clinical Manifestations: >ESRD >Uremia (when renal nephrons are destroyed and renal function deteriorates) >UTI (bec of distorted renal archithecture) >hyponatremia (PKD tends to waste Na+) Labs and Diagnostics: Urinalysis (hema/proteinuria) CBC (low Hct and Hgb) Cerebral angiography ((+) aneurysm)

Nursing Management: 1) Aggressive control of HPN 2) Inc sodium intake (but if + HPN, dietary sodium is restricted) 3) Dialysis or renal transplant (if ESRD develops) 4) Genetic counseling (bec of hereditary nature of disease) NANDA problems: Pain (acute) related to compression of tissues, trauma to structures from calculi, inflammation, and infection Prognosis: Many infants and children with recessive PKD die from hapatic fibrosis, which obstructs blood flow and causes bile buildup in the liver. Its symptoms are enlargement of the liver and the spread of a fibrous connective tissue over the liver. Those who survive into their 20s may develop splenic, pancreatic, and vascular problems. Children with recessive PKD often have smaller than average stature.

Hydronephrosis Background: Hydronephrosis is distension and dilation of the renal pelvis calyces, usually caused by obstruction of the free flow of urine from the kidney, leading to progressive atrophy of the kidney. In case of hydroureteronephrosis, there is distention of both the ureter and the renal pelvis and calices Etiology: Hydronephrosis is the result of several abnormal pathophysiological occurrences. Structural abnormalities of the junctions between the kidney, ureter, and bladder that lead to hydronephrosis can occur during fetal development. Some of these congenital defects have been identified as inherited conditions, however the benefits of linking genetic testing to early diagnosis have not been determined. Other structural abnormalities could be caused by injury, surgery, or radiation therapy. Compression of one or both ureters can also be caused by other developmental defects not completely occurring during the fetal stage such as an abnormally placed vein, artery, or tumor. Bilateral compression of the ureters can occur during pregnancy due to enlargement of the uterus. Changes in hormone levels during this time may also affect the muscle contractions of the bladder, further complicating this condition. Sources of obstruction that can arise from other various causes include kidney stones and blood clots. The obstruction may be either partial or complete and can occur anywhere from the urethral meatus to the calyces of the renal pelvis. Hydronephrosis can also result from the reverse flow of urine from the bladder back into the kidneys. This reflux can be caused by some of the factors listed above as well as compression of the bladder outlet into the urethra by prostatic enlargement or impaction of feces in the colon, as well as abnormal contractions of bladder muscles resulting from neurological dysfunction or other muscular disorders. Signs and symptoms/ Clinical Manifestations:         onset of intense flank or back pain radiating to the groin, nausea, vomiting, Sweating. Colicky pain Blood seen in the urine. Chronic hydronephrosis weakness, malaise

If electrolyte abnormalities occur because the kidneys are unable to regulate sodium, potassium, and calcium, there may be heart rhythm disturbances and muscle spasms. Labs and Diagnostics: The following laboratory tests may be ordered depending upon what potential diagnosis is being considered. y Urinalysis to look for blood, infection or abnormal cells y Complete blood count (CBC) may reveal anemia or potential infection y Electrolyte analysis may be helpful in chronic hydronephrosis since the kidneys are responsible for maintaining and balancing their concentrations in the blood stream. y BUN (blood urea nitrogen), creatinine and glomerular filtration rate (GFR) are blood tests that help assess kidney function. «Imaging Studies CT scan of the abdomen can be performed to evaluate the kidney anatomy and make the diagnosis of hydronephrosis. It also may allow the health care practitioner to look for the underlying cause including kidney stones or structures that are compressing the urinary collecting system. Depending upon the situation and the health care practitioner's concerns, the CT may be done with or without contrast dye injected into a vein, and with or without oral contrast (that the patient drinks) to outline the intestine. Most commonly, for kidney stones, neither oral nor intravenous contrast is needed. Ultrasound is another imaging study that can be done to look for hydronephrosis. The quality of the test depends upon the skill of the ultrasonographer to evaluate the structures in the abdomen and retroperitoneum. Ultrasound is also useful inwomen who are pregnant where radiation concerns exist. Intravenous pyelography (IVP) has mostly been replaced by CT scanning but does have a role in diagnosing some patients and its use is now limited. KUB X-rays (an X-ray that shows the kidney, ureter, and bladder) are used by some urologists to classify a kidney stone as radiodense or radiolucent and may use KUB X-rays to determine if the stone is able to migrate down the ureter into the bladder. Nursing Management: 1) Asses: Pain Urine input& output Palpate kidney Urine for labs (spec gravity, albumin, glucose, and edema) 2) Intervention: Administer fluids (hourly fluid replacement) Watch out for pain and reduced U.O.

Avoid urinary infections(keep urine bag above not touching the floor)

NANDA problems: Impaired urinary elimination r/t obstruction (mechanical or anatomical) Risk for infection r/t urinary stasis Prognosis: Left untreated, bilateral obstruction (occurring to both kidneys rather than one) has a poor prognosis

Wilm¶s Tumor Background: Wilm¶s Tumor or nephroblastoma is cancer of the kidneys that typically occurs in children, rarely in adults. Its common name is an eponym, referring to Dr. Max Wilms, the German surgeon (1867±1918) who first described this kind of tumor. Approximately 500 cases are diagnosed in the U.S. annually. The majority (75%) occurs in otherwise normal children; a minority (25%) is associated with other developmental abnormalities. It is highly responsive to treatment, with about 90% of patients surviving at least five years. Etiology: Wilms tumor may arise in 3 clinical settings, the study of which resulted in the discovery of the genetic abnormalities that lead to the disease. Wilms tumors can arise sporadically, can develop in association with genetic syndromes, or can be familial. Although some of the molecular biology of Wilms tumor is coming to light, the exact cellular mechanisms involved in the etiology of the tumor are still being investigated. Signs and symptoms/ Clinical Manifestations: ...More common findings:  Palpable abdominal mass  Gross Hematuria (onset: 9 mos), Flank pain, Fever , Weight loss, Cachexia fatigue, HPN, amyloidosis, thromblophlebitis, anemia,erythrocytosis, hypercalcemia, abnormal serum liver profile, elevated ESR, «Less frequent findings:  Peripheral neuropathy, inferior vena cava obstruction, priapism, variocele, hydronephrosis (if tumor blocks ureteropelvic junction)

Labs and Diagnostics: Plasma erythropoietin, renin, chorionic gonadotropin ang prostaglandin are elevated IV Pyelogram UTZ CT Scan Nephrotomography Nursing Management: PRE- OP: Inc fluid intake if indicated, emotional support POST-OP: V/S, WOF signs of hemorrhage, pneumothorax

NANDA problems: Anxiety r/t threat of death Risk for injury Interrupted family process r/t expensive treatments Knowledge deficit of the disorder and therapy Prognosis: Tumor-specific loss-of-heterozygosity (LOH) for chromosomes 1p and 16q identifies a subset of Wilms' tumor patients who have a significantly increased risk of relapse and death. LOH for these chromosomal regions can now be used as an independent prognostic factor together with disease stage to target intensity of treatment to risk of treatment failure. Genome-wide copy number and LOH status can be assessed with virtual karyotyping of tumor cells (fresh or paraffin-embedded). The overall prognosis with surgical removal is positive. Early removal tends to promote positive outcomes.

Hypertrophic Pyloric Stenosis A condition that causes severe vomiting in the few months of life. There is narrowing (stenosis) of the opening from the stomach to the intestines, due to enlargement (hypertrophy) of the muscle surrounding this opening (pylorus), which spasms when the stomach empties. It is uncertain whether there is a real congenital narrowing or whether there is a functional hypertrophy of the muscle which develops few weeks of life. Hypertrophic pyloric stenosis may also cause almost complete gastric outlet obstruction. It affects 1 of 250 infants and is common among males by a 4:1 ratio, particularly firstborn males. It occurs most often between 3 to 5 weeks of age and rarely after 12 weeks. Etiology There is no known etiology of pyloric stenosis, but a genetic component is likely because siblings and offspring of affected people are at increased risk. Proposed mechanisms include lack of neuronal nitric oxide synthase and abnormal innervations of the muscular layer. Infants exposed to certain macrolide antibiotics in the first few weeks of life are at significantly increased risk. Clinical Findings - Olive shaped mass palpated on the epigastrium. - Palpable or even visible peristaltic waves - Thickened pylorus in ultrasound Signs and Symptoms - Progressive worsening non-bile stained projectile vomiting - Poor feeding - Weight loss

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Narrowed pyloric outlet in upper GI series Hypokalemic, hypochloremic metabolic alkalosis in blood chemistry Hypovolemia Hyperaldosteronism

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Crying without tears and less wet or dirty diapers (dehydration) Constant hunger Belching Colic

Laboratory and Diagnostic procedures - Ultrasound ( most common diagnostic exam) - Upper gastro-intestinal series ( most common confirmatory diagnostic exam) - Blood chemistry and arterial blood gases Nursing Management - Immediate fluid replacement (dehydration and electrolyte imbalances)

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Oral atropine If the client undergoes surgery, secure consent and properly educated the client¶s parents and guardians about the procedure. If the client has an ostomy, clean the stoma properly and regularly, and educated the client¶s parents and guardians in how to clean the stoma.

Nursing Diagnosis - Fluid volume deficit - Imbalanced nutrition less than body requirement - Risk for aspiration Prognosis If intervention and surgery are immediately done, good prognosis is accomplish.

CLEFT LIP (CL) AND/ OR CLEFT PALATE (CP) Cleft lip and cleft palate are facial malformations that occur during embryonic development and can constitute a severe disability to the affected individual. Cleft lip, also known as cheiloschisis, and cleft palate, also known as palatoschisis, are types of abnormal developments of the face during pregnancy - they are types of clefting congenital deformities. They can occur together as cleft lip and palate. Before birth, there are natural structures that form in the body and then join together (fuse). A cleft is a non-fusion of these structures - a fissure or a gap. In this case, the gap (cleft) occurs in the upper lip, the palate (roof of the mouth), or both. When just one side of the lip is affected it is called a unilateral cleft; a bilateral cleft affects both sides. According to the National Institutes of Health (NIH), USA, about 1 in every 700 newborns has a cleft lip and/or cleft palate. The National Health Service (NHS), UK says the incidence is 1 in 600.

ETIOLOGY Researchers believe that most cases of cleft lip and cleft palate are caused by an interaction of genetic and environmental factors. In many babies, a definite cause isn't discovered.  Genetic factors. Either the mother or the father can pass on genes that cause clefting, either as an isolated defect or as part of a syndrome that includes clefting as one of its signs. In some cases, babies inherit a gene that makes them more likely to develop a cleft, and then an environmental trigger actually causes the cleft to occur.  Environmental factors. Fetal exposure to cigarette smoke, alcohol, certain medications, illicit drugs and certain viruses have been linked to the development of a cleft. Other risk factors: Several factors may increase the likelihood of a baby developing a cleft lip and cleft palate. 

   

Family history. Parents with a family history of cleft lip or cleft palate face a higher risk of having a baby with a cleft. Race. Cleft lip and palate are most common in American Indian and Asian children. Black children are least likely to have a cleft. Sex. Males are twice as likely to have a cleft lip with or without cleft palate. Cleft palate without cleft lip is more common in females. Environmental factors. Exposure in early pregnancy to cigarette smoke, alcohol or illicit drugs may put a baby at higher risk of developing a cleft. Maternal obesity. Obesity in the mother is associated with a slightly increased risk of cleft lip and palate. PATHOPHYSIOLOGY During embryonic development the lateral and medial tissues forming the upper lip palates fuse between weeks 7 and 8 of gestation; the palatal tissues forming the hard and soft palates fuse between weeks 7 and 12 gestation. Cleft lip and cleft palate result when these tissues fail to fuse. MANIFESTATIONS Clinical manifestations a. Cleft lip and cleft palate are readily apparent at birth. Careful physical assessment should be performed to rule out other midline birth defects. Palpate the palate with the fingers to check for defects. b. Cleft lip and cleft palate appear as incomplete or complete defects, and may be unilateral or bilateral. Laboratory and diagnostic study findings. Obstetric ultrasound will reveal cleft lip while the infant is in utero at 18 to 20 weeks of gestation can identify a cleft in a fetus. COMPLICATIONS Children with cleft lip with or without cleft palate face a variety of challenges, depending on the type and severity of the cleft. Feeding difficulties. One of the most immediate concerns after birth is feeding. While most babies with cleft lip can breast-feed, a cleft palate can make sucking difficult or cause gagging or nasal regurgitation. Your health care team will discuss feeding strategies with you, such as using a special bottle nipple or a small artificial palate (obturator) that fits into the roof of the mouth. Ear infections and hearing loss. Babies with cleft palate are especially susceptible to middle ear infections. Over time, repeated ear infections can damage hearing, but hearing loss may resolve with treatment. It's important for children with cleft palate to be evaluated regularly by an audiologist or an ear, nose and throat doctor. Most children with clefts have tubes inserted in their ears to drain fluids and help prevent infections.     

Dental problems. If the cleft extends through the upper gum, tooth development will likely be affected. A pediatric dentist should monitor tooth development and oral health from an early age. Speech difficulties. Because both the lip and palate are used in forming sounds, the development of normal speech can be affected. A speech pathologist can evaluate your child and provide speech therapy. Psychological challenges. Children with clefts may face social, emotional and behavioral problems due to differences in appearance and the stress of intensive medical care. A psychologist and a social worker can help you and your child deal with the stresses your family encounters. NURSING MANAGEMENT Assess for problems with feeding, breathing parental bonding, and speech. Provide child and family teaching. Ensure adequate nutrition and prevent aspiration. a. Provide special nipples or feeding devices (eg, soft pliable bottle with soft nipple with enlarged opening) for a child unable to suck adequately on standard nipples. b. Hold the child in a semi-upright position; direct the formula away from the cleft and toward the side and back of the mouth to prevent aspiration. c. Feed the infant slowly and burp frequently to prevent excessive swallowing of air and regurgitation. d. Stimulate sucking by gently rubbing the nipple against the lower lip.

Feeding bottle for infant with Cleft lip and cleft palate. Support the infant¶s and parents¶ emotional and social adjustment. a. Help facilitate the family¶s acceptance of the infant by encouraging the parents to express their feelings and concerns and by conveying

an attitude of acceptance toward the infant. .

b. Emphasize the infant¶s positive aspects and express optimism regarding surgical correction

Provide preoperative care. a. Depending in the defect and the child¶s general condition, surgical correction of the cleft lip (cheiloplasty ) usually occurs at 1 to 3 months of age; repair of the cleft palate (palatoplasty) is usually performed between 6 and 18 months of age. Repair of the cleft palate may require several stages of surgery as the child grows. b. Early correction of cleft lip enables more normal sucking patterns and facilitates bonding. Early correction of cleft palate enables development of more normal speech patterns. c. Delayed closure or large defects may require the use of orthodontic appliances. d. The responsibilities of the nurse are to: 1. Reinforce the physician¶s explanation of surgical procedures. 2. Provide mouth care to prevent infection. Provide postoperative care. a. Assess airway patency and vital signs; observe for edema and respiratory distress. b. Use a mist tent, if prescribed, to minimize edema, liquefy secretions, and minimize distress. c. Position the child with cleft lip on her back, in an infant seat, or propped on a side to avoid injury to the operative site; position the child with a cleft palate on the abdomen to facilities drainage. d. Clean the suture line and apply an antibacterial ointment as prescribed to prevent infection and scarring. Monitor the site for signs of infection. e. Use elbow restraints to maintain suture line integrity. Remove them every 2 hours for skin care and range-of-motion exercises. f. Feed the infant with a rubber-tipped medicine dropper, bulb syringe, Breck feeder, or soft bottle-nipples, as prescribed, to help preserve suture integrity. For older children, diet progresses from clear fluids; they should not use straws or sharp objects. g. Attempt to keep the child from putting tongue up to palate sutures. h. Manage pain by administering analgesic as prescribed. NURSING DIAGNOSIS y Altered nutrition : less than body requirements related to physical defect y Risk for altered parenting related to infant with a highly visible physical defect

Risk for trauma of the surgical site related to surgical procedure, dysfunctional swallowing y Altered nutrition: less than body requirements related to difficulty eating following surgical procedure y Pain related to surgical procedure y Altered family processes related to child with a physical defect, hospitalization PROGNOSIS Although treatment may continue for several years and require several surgeries, most children with a cleft lip and palate can achieve normal appearance, speech, and eating. However, some people may have a continued speech problem that needs further therapy. CLEFT LIP/ CLEFT PALATE MIND MAP y

ESOPHAGEAL ATRESIA WITH TRACHEOESOPHAGEAL FISTULA . A rare congenital malformation that is believed to result from failed separation of the esophagus and trachea by a septum that forms in the 4th week of gestation. The esophagus ends in a blind pouch and is accompanied by Tracheoesophageal Fistula. ETIOLOGY AND INCIDENCE The cause of esophageal atresia and TEF is unknown. ‡ 1:2000 to 1:1500 live births. ‡ Happens in both sexes. ‡ EA/TEF is often present in VATER or VATERL syndromes ‡ V-ertebral ‡ A-norectal ‡ ‡ ‡ ‡ ‡ ‡ ‡ C- ardiovascular, T-racheo E-sophageal R-enal L-imb abnormalities Low birth weight babies. And high incidence in premature infants

TYPES: y Type A (7.7%): Esophageal atresia in which both segments of the esophagus end in blind pouches. Neither segment is attached to the trachea. y Type B (0.8%): Esophageal atresia with tracheoesophageal fistula in which the upper segment of the esophagus forms a fistula to the trachea. The lower segment of the esophagus ends in a blind pouch. This condition is very rare. y Type C (86.5%): Esophageal atresia with tracheoesophageal fistula, in which the upper segment of the esophagus ends in a blind pouch (EA) and the lower segment of the esophagus is attached to the trachea (TEF). y Type D (0.7%): Esophageal atresia with tracheoesophageal fistula, in which both segments of the esophagus are attached to the trachea. This is the rarest form of EA/TEF. y Type H (4.2%): Tracheoesophageal fistula in which there is no esophageal atresia because the esophagus is continuous to the stomach. Fistula is present between the esophagus and the trachea.

PATHOPHYSIOLOGY

SIGN AND SYMPTOMS ‡ Excessive salivation or drooling ‡ Three C¶s OF TEF ‡ C-hoking ‡ C-oughing ‡ C-yanosis ‡ Apnea ‡ Increased respiratory distress after feeding ‡ Abdominal distention DIAGNOSTIC EXAM ‡ Esophageal Atresia- maternal polyhydramnios. ‡ Determined by radiographic studies ‡ A size 10 or 12 French catheter passed through the nose meets an obstruction (esophageal atresia) approximately 4" to 5" (10 to 12.5 cm) distal from the nostrils. Aspirate of gastric contents is less acidic than normal. ‡ Chest X-ray demonstrates the position of the catheter and can also show a dilated, air-filled upper esophageal pouch, pneumonia in the right upper lobe, or bilateral pneumonitis. Both pneumonia and pneumonitis suggest aspiration. ‡ Abdominal X-ray shows gas in the bowel in a distal fistula (type C) but none in a proximal fistula (type B) or in atresia without fistula (type A). ‡ Cinefluorography allows visualization on a fluoroscopic screen. After a size 10 or 12 French catheter is passed through the patient¶s nostril into the esophagus, a small amount of contrast medium is instilled to define the tip of the upper pouch and to differentiate between overflow aspiration from a blind end (atresia) and aspiration due to passage of liquid through a tracheoesophageal fistula. COMPLICATIONS y The infant may breathe saliva and other secretions into the lungs, causing aspiration pneumonia, choking, and possibly death. y Other complications may include:  Feeding problems  Reflux (the repeated bringing up of food from the stomach) after surgery  Narrowing (stricture) of the esophagus due to scarring from surgery  Tracehomalacia ± weakness in the tracheal wall that occurs when a dilated proximal pouch compresses the trachea in early fetal life. y Prematurity may complicate the condition.

THERAPEUTIC/NURSING MANAGEMENT

‡ ‡ ‡ ‡ ‡

Keep infant warm and oxygenated Keep infant supine with the HOB elevated to keep gastric secretions from entering the lungs NGT aspirate every 5 to 10 minutes to keep the keep the proximal pouch clear Intravenous IV fluids are essential Surgical repair: Ligation of the fistula and end-to-side anastomosis of the atresia. NURSING DIAGNOSIS

y y y y y

Impaired gas exchange and ineffective airway clearance related to Respiratory distress/ EA& TEF Risk for aspiration related to infants immature gag and cough reflex Risk for imbalanced nutrition: less than body requirements related to inability to take in oral feedings Risk for infection related to aspiration or seepage of stomach secretions on the lungs Risk for impaired skin integrity related to gastrostomy tube insertion site.

PROGNOSIS Surgery to correct esophageal atresia is usually survival rates close to 100 percent in otherwise healthy condition is corrected. Postoperative complications may swallowing, since the esophagus may not contract

successful, with infants after the include difficulty efficiently, and

gastrointestinal reflux, in which the acidic contents of stomach back up into the lower part of the esophagus, possibly causing ulcers.

BACKGROUND OF THE DISEASE Intussusception- the invagination of one portion of the intestine into another, usually occurs in the second half of the first year of life. ETIOLOGY <1 year old-occurs for Idiopathic reasons >1 year old-a ³lead point´ on the intestine likely cues the invagination Meckel¶s diverticulum-a polyp, hypertrophy of Peyer¶s patches (lymphatic tissue of the bowel that increases in size with viral diseases) or bowel tumors. The point of the invagination is generally at the juncture of the distal ileum and proximal colon. SIGNS AND SYMPTOMS Assessment: y Suddenly draw up their legs and cry and they vomit y After the peristaltic wave, they are symptom free and play happily y (Approx. 15 min.) Same phenomenon of intense abdominal pain strikes again y Vomitus will begin that contains bile bec. the obstruction is invariably below the ampulla of Vater (the point in the intestine where bile empties into the duodenum y (Approx. 12 hrs.)blood appears into the stool and possibly in vomitus (currant jelly appearance) y The abdomen becomes distended as the bowel above the intussusceptions distends. DIAGNOSTIC TEST The presence of intussusceptions is confirmed by ULTRASOUND or CT scan MANAGEMENT y Surgical Emergency y Instillation of a water soluble sol¶n, barium enema or air (pneumatic insufflation) into the bowel or surgery to reduce invagination. y After this type of reduction, children must observe for 24 hrs. because some children will have recurrence of the intussusceptions. NURSING DIAGNOSIS y Pain related to abnormal abdominal peristalsis y Risk for deficient fluid volume related to bowel obstruction y Risk for impaired parenting related to infant¶s illness

BACKGROUND OF THE DISEASE Hirschsprung¶s Disease-is absence of ganglionic innervations to the muscle of a section of the bowel. In most instances, the lower portion of the sigmoid colon just above the anus. The absence of nerve cells means there are no peristaltic waves in this section to move fecal material through the segment of the intestine. ETIOLOGY Assessment:  If infants fail to pass meconium by 24 hours of age  Increasing abdominal distention  History of constipation or intermittent constipation and diarrhea  What is the duration of the constipation? (It may have been a problem from birth)  What do parents mean constipation? (Children do not have a bowel movement more than a week)  What is the consistency of the stool? (Ribbonlike or watery)  Is the child ill in any other way? (Children with aganglionic disease of the intestine tend to be thin and undernourished, sometimes deceptively so because their abdomen is large and distended) Normal If a gloved finger is inserted into the rectum of a child with true constipation, the examining finger will touch hard, caked stool. Hirschsprung Disease With aganglionic disease, the rectum is empty because fecal material cannot pass into the rectum through the obstructed portion.

DIAGNOSTIC TEST  A barium enema is generally ordered to substantiate the diagnosis. The barium will outline on x-ray film the narrow, nerveless portion and the proximal distended portion of the bowel  Biopsy of the affected segment to show the lack of innervations or by anorectal manometry (a technique to test the strengthor innervations of the internal rectal sphincter by inserting a balloon catheter into the rectum and measuring the pressure exerted against it.

MANAGEMENT SOAVE PULL THROUGH OPERATION Repair of aganglionic megacolon involves dissection and removal of affected section, with anastomosis of the intestine (termed a pull-through operation)

Two stage surgery:  Temporary colostomy  Bowel repair at 12 to 18 months of age *After the final surgery, the children should have functioning, normal bowel. In the few instances in which the anus is deprived of nerve endings, a permanent colostomy will established. NURSING DIAGNOSIS y Constipation related to reduced bowel function y Imbalanced nutrition, less than body requirements, related to reduced bowel function

BACKGROUND OF THE DISEASE Anorectal Malformation-are birth defects (problems that happen as a fetus is developing during pregnancy). With this defect, the anus and rectum (the lower end of the digestive tract) do not develop properly. "Ano" refers to the anus (the opening at the end of the large intestine through which stool passes when a baby has a bowel movement) "Rectal" refers to the rectum (the area of the large intestine just above the anus) With an anorectal malformation, several abnormalities can occur, including the following: y a membrane may be present over y the rectum may connect to a part the anal opening of the urinary tract or the reproductive system through an y the rectum may not connect to the abnormal passage called a fistula anus (imperforate anus) Diagnostic test: Abdominal ultrasound/sonography and xray MANAGEMENT Treatment may depend on the ff: y the extent of the problem y the overall health of the baby and the medical history y parental opinion and preference y the opinion of the physicians involved in the baby's care y expectations for the course of the disease The majority of babies with anorectal malformation will need to have surgery to correct the problem. The type and number of operations necessary depends on the type and extent of abnormality the baby has, including the following: y Narrow anal passage - Babies who have the type of malformation that causes the anal passage to be narrow may not need an operation. A procedure known as anal dilatation may be done periodically to help stretch the anal muscles so stool can pass through it easily. However, if the anal opening is positioned wrongly, an operation may be neeeded to correctly relocate the anal opening. y Anal membrane - Babies with this type of malformation will have the membrane removed during surgery. Anal dilatations may need to be done

y

afterward to help prevent any narrowing of the anal passage that is present. Lack of rectal/anal connection, with or without a fistula -These babies may need a series of operations in order to have the malformation repaired.

IMPERFORATE ANUS Imperforate Anus is stricture of anus. It is congenital (present from birth) defect in which the opening to the anus is missing or blocked. Etiology/ cause The problem is caused by abnormal development of the fetus. In week 7 of intrauterine life, the upper bowel elongates to pouch and combine with a pouch invaginating form the perineum Clinical findings  It is a relatively common condition that occurs in about 1 out of 5,000 infants.  Most common in boys Sign and symptoms y Anal opening very near the vaginal opening in girls y Missing or misplaced opening to the anus y No passage of first stool within 24 - 48 hours after birth Diagnostic/ laboratory  Prenatal sonogram, Radiograph Assessment  Inspection of the newborn¶s anal region  May be revealed because a membrane filled with black meconium y Stool passes out of the vagina, base of penis, scrotum, or urethra y Swollen belly area 

No wink reflex in the anal area (touching the skin near the rectum should make it contact)  Inability to insert rubber catheter into the rectum Nursing management  Anastomosis, Colostomy NANDA problems  Imbalanced nutrition, less than body requirements, related to bowel obstruction and inability for oral intake  Impaired tissue integrity at rectum related to surgical incision  Risk for impaired parenting related to difficulty in bonding with infant ill from birth

GASTRO ESOPHAGEAL REFLUX DISEASE Gastro esophageal reflux disease is a condition in which the liquid content of the stomach regurgitates (backs up or refluxes) into the esophagus. The liquid can inflame and damage the lining of the esophagus although visible signs of inflammation occur in a minority of patients Etiology/ cause  Neuromuscular disturbance in which the cardiac sphincter and the lower portion of the esophagus spasm and allow easy regurgitation of gastric contents into the esophagus  Incompetent cardiac sphincter  Maybe related to hiatal hernia Clinical findings  Starts within 1 week after birth  Regurgitation starts after feeding Sign and symptoms y Effortless vomiting not projectile y heartburn, regurgitation, and nausea.

y Ulcers, strictures, esophageal cancer y Inflammation of larynx and throat Diagnostic and laboratory y Endoscopy (fiberoptic endoscopy) esophagography y Esophageal ph testing y X-ray Nursing management y Formula or breast milk with rice cereal y Upright position while eating and an hour after eating y Laparoscopic or surgical myotomy (narrowing of the esophageal sphincter)

y Inflammation and infection of lungs y Fluid in the sinuses and middle ears y Examination of throat and larynx y Esophageal manometry- used to measure the strength of the esophageal sphincter y Ranitidine y Omeprazole y Avoid acidic, fatty and alcoholic foods y Eat small portion of foods

NANDA problems Risk for imbalanced nutrition, less than body requirements, related to regurgitation of food with esophageal reflux

CONSTIPATION Difficulty of passing hardened stools, may occur in children of any age Cause/ etiology  Lack of fluid intake  High fiber meals Clinical findings y Distressing to children (painful, and may have anal fissures) y May experience abdominal pain from force of intestinal contractions  Large and firm stool Sign and symptoms  Hard with discomfort in defecating 

Anal fissures (may cause blood in stool)  Abdominal pain Diagnostic and laboratory findings  Blood occult test (for sign of bleeding) Nursing management y Increase fluid intake y Diet high in fiber y Privacy in bathroom y Determine the cause of stress NANDA problems Constipation related to pain from anal fissure and hardened stool DIARRHEA Frequent bowel evacuation or the passage of abnormally soft of liquid feces Etiology/cause y Caused by virus which is the major cause of infant gastroenteritis y Common viral pathogens : rotavirus, adenovirus y Common bacterial pathogens ; campylobacter jejuni, salmonella, giardia lamblia and clostridium difficile y Acute associated by infection y Chronic associated with malabsorption and inflammatory cause Sign and symptoms y Frequency- unlimited number y Color- green y Effort of expulsion- effortless; may be explosive y pH- less than 7 (acidic) y Odor- sweet or foul y Occult blood- positive; blood may be overt

y Reducing saubstances- positive Assessment (mild diarrhea) y Anorectic and irritable y Episodes of diarrhea 2-10 loose watery y With fever (38.4-39) Assessment (severe diarrhea) y 39.5-40 temperature y Pulse and respiration are weak and rapid y Pale and cool skin y May appear apprehensive, listless and lethargic Therapeutic management y y y y y Oral rehydration solution Rest the GI tract Breastfeeding Zinc administration (zinc deficiencies) Antibiotic therapy y Depressed fontanelle, sunken eyes and poor skin turgor y Elevated hemoglobin and hematocrit because of dehydration y Loss in body weight y Warm skin y Dry mouth y Rapid pulse

NANDA problems Deficient fluid volume related to loss of fluid through diarrhea Risk for impaired skin integrity related to presence of diarrheal stool on skin

APPENDICITIS Appendicitis is a condition characterized by inflammation of the appendix. It is classified as a medical emergency and many cases require removal of the inflamed appendix, either by laparotomy or laparoscopy. Untreated, mortality is high, mainly because of peritonitis and shock y First visible during week 8 y Function Unknown y Most likely caused by luminal obstruction ETIOLOGY: y Fecal material

y

Parasite

Signs and symptoms For the most part symptoms related to disturbed function of bowels. Pain first, vomiting next and fever last has been described as classic presentation of acute appendicitis. Pain starts mid abdomen, and except in children below 3 years, tends to localize in right iliac fossa in a few hours. This pain can be elicited through various signs. Signs include localized findings in the right iliac fossa. The abdominal wall becomes very sensitive to gentle pressure (palpation). Also, there is severe pain on suddenly releasing a deep pressure in lower abdomen rebound tenderness. In case of a retrocecal appendix, however, even deep pressure in the right lower quadrant may fail to elicit tenderness (silent appendix), the reason being that the cecum, distended with gas, prevents the pressure exerted by the palpating hand from reaching the inflamed appendix. Similarly, if the appendix lies entirely within the pelvis, there is usually complete absence of the abdominal rigidity. In such cases, a digital rectal examination elicits tenderness in the rectovesical pouch. Coughing causes point tenderness in this area (McBurney's point) and this is the least painful way to localize the inflamed appendix. If the abdomen on palpation is also involuntarily guarded (rigid), there should be a strong suspicion of peritonitis requiring urgent surgical intervention. Diagnostics Diagnosis is based on patient history (symptoms) and physical examination backed by an elevation of neutrophilic white blood cells. Histories fall into two categories, typical and atypical. Typical appendicitis usually includes abdominal pain beginning in the region of the umbilicus for several hours, associated with anorexia, nausea or vomiting. The pain then "settles" into the right lower quadrant, where tenderness develops. Atypical histories lack this typical progression and may include pain in the right lower quadrant as an initial symptom. Atypical histories often require imaging with ultrasound and/or CT scanning.[23] A pregnancy test is vital in all women of child bearing age, as ectopic pregnancies and appendicitis present with similar symptoms. The consequences of missing an ectopic pregnancy are serious, and potentially life threatening. Furthermore the general principles of approaching abdominal pain in women (in so much that it is different from the approach in men) should be appreciated. Blood Test Most patients suspected of having appendicitis would be asked to do a blood test. 50% of the time, the blood test may be normal, so it is not fool proof in diagnosing appendicitis. Two form of blood tests are commonly done: FBC (Full blood count) or CBC (Complete blood count), is an inexpensive and commonly requested blood test. It involves measuring the blood for its richness in red blood cells as well as the number of the various white blood cell constituents in it. The number of white

cells in the blood is a usually less than 10,000 cells per cubic millimeter. An abnormal rise in the number of white blood cells in the blood is a crude indicator of infection or inflammation going on in the body. Such rise is not specific to appendicitis alone. If it is abnormally elevated, with a good history and examination findings pointing towards appendicitis, the likelihood of having the disease is higher. In pregnancy, there may be a normal elevation of white blood cells, without any infection present. CRP is an acronym for Cryo-Reactive Proteins. It is an acute phase response protein produced by the liver in response to any infection or inflammatory process in the body. Again, like the FBC, it is not a specific test. It is another crude marker of infection or inflammation. Inflammation at ANY site can lead to the CRP to rise. A significant rise in CRP with corresponding signs and symptoms of appendicitis is a useful indicator in the diagnosis of appendicitis.It is said that if CRP continues to be normal after 72 hours of the onset of pain, it is likely that the appendicitis will resolve on its own without intervention. A worsening CRP with good history is a sure signal fire of impending perforation or rupture and abscess formation Urine Test: Urine test in appendicitis is usually normal. It may however show blood if the appendix is rubbing on the bladder, causing irritation A urine test or urinalysis is compulsory in women, to rule out pregnancy in appendicitis, as well to help ensure that the abdominal pain felt and thought to be acute appendicitis is not in fact, due to ectopic pregnancy. X ± Ray In 10% of patients with appendicitis, plain abdominal x-ray may demonstrate hard formed feces in the lumen of the appendix (Fecolith). It is agreed that the finding of Fecolith in the appendix on X ± ray alone is a reason to operate to remove the appendix, because of the potential to cause worsening symptoms. In this respect, a plain abdominal X-ray may be useful in the diagnosis of appendicitis, though plain abdominal x- ray is no longer requested routinely in suspected cases of appendicitis. An abdominal X ± ray may be done with a barium enema contrast to diagnose appendicitis. Barium enema is whitish toothpaste like material that is passed up into the rectum to act as a contrast. It will usually fill the whole of the large bowel. In normal appendix, the lumen will be present and the barium fills it up and is seen when the x-ray film is shot. In appendicitis, the lumen of the appendix will not be visible on the barium film.

NURSING MANAGEMENT The treatment begins by keeping the patient from eating or drinking in preparation for surgery. An intravenous drip is used to hydrate the patient. Antibiotics given intravenously such as cefuroxime and metronidazole may be administered early to help kill bacteria and thus reduce the spread of infection in the abdomen and postoperative complications in the abdomen or wound.

NANDA PROBLEMS IMBALANCED NUTRITION ,LESS THAN BODY REQUIREMENTS RELATEDTO MALABSORPTION OF FOOD PROGNOSIS y Most appendicitis patients recover easily with surgical treatment, but complications can occur if treatment is delayed or if peritonitis occurs. Recovery time depends on age, condition, complications, and other circumstances, including the amount of alcohol consumption, but usually is between 10 and 28 days. For young children (around 10 years old), the recovery takes three weeks. y The real possibility of life-threatening peritonitis is the reason why acute appendicitis warrants speedy evaluation and treatment. The patient may have to undergo a medical evacuation. Appendectomies have occasionally been performed in emergency conditions (i.e., outside of a proper hospital), when a timely medical evaluation was impossible. INFLAMMATORY BOWEL DISEASE ULCERATIVE COLITIS- inflammation of distal colon and rectum Common manifestation: Diarrhea and malnutrition Complication: Hemorrhage from bowel perforation, peritonitis or formation of fistula between bowel and loops CROHN¶S DISEASE ± inflammation of segments of the intestine which involves terminal ileum COBBLE STONE- colon wall becomes thickened and inflamed. Assessment: Diarrhea and steatorrhea develops, melena, recurring fever, growth failure Diagnostic/Laboratory procedure: -colonoscopy - Barium enema - biopsy Management: Enteral and Parenteral nutrition High protein, carbohydrate and vitamin diet Drugs: prednisone (corticosteroid), sulfasilizine (azulfidine sulfamide and salicylic acid), azathioprine (imuran immunosuppressive agent), monoclonal antibodies (infiximab) PEPTIC ULCER DISEASE A peptic ulcer may be referred to as a gastric, duodenal or esophageal ulcer depending on its location. A person who has a peptic ulcer, depending on

its location. A person who has an peptic ulcer has peptic ulcer has an peptic ulcer disease. A peptic ulcer is an excavation (hollowed-out area) that forms in the mucosal wall of the stomach, in the pylorus (the opening between the stomach and duodenum) in the duodenum (the first part of small intestine) or in the esophagus. Erosion of a circumscribed area of mucous membrane is the cause. This erosion may extend as deeply as the muscle layers or through the muscle of the peritoneum. Peptic ulcers are more likely to be in duodenum than in the stomach. Chronic gastric ulcers tend to occur in the lesser curvature of the stomach, near the pylorus. Peptic ulcer disease occur with the greatest frequency in people between 40 and 60 years of age. It is relatively uncommon in women of childbearing age but it has been observed in children and infant. In addition, excessive secretion of HCI in the stomach may contribute to the formation of peptic ulcers and stress may also be a contributing factor. The ingestion of milk and caffeinated beverages, smoking and alcohol also may increase HCl. Stress and eating spicy food may make peptic ulcer worse. Some other factors like genetic tendency, NSAIDS use and cigarettes can be associated. ETIOLOGIC AGENT: Helicobacter pylori ASSESSMENT: Neonate: hematemesis (blood in vomitus), Melena (blood in stool), rupture ulcer leads to respiratory distress, abdominal distention, vomiting, cardiovascular collapse (if extensive). Toddler: Anorexia and vomiting Pre-school: local pain: mild, severe, colicky or continous. School-aged: gnawing or aching on epigastric area, vomiting, epigastric tenderness. Laboratory procedure: fiberoptic endoscopy ± most reliable diagnostic test to confirm peptic ulcer disease. Treatment management: children: Cimetidine (Targamet) Adult: Amoxicillin/Clarythromycin (Biaxin) Nursing Dx: Pain r/t ulceration in intestinal tract NECROTIZING ENTEROCOLITIS BACKGROUND ± most commonly occurring gastrointestinal emergency in preterm infants ± leading cause of emergency surgery in neonates ± overall incidence: 1-5% in most NICU¶s ± most common in VLBW preterm infants ‡ 10% of all cases occur in term infants

ETIOLOGY ‡ W ±with Infections ‡ I - ischemia ‡ P - premature ‡ E ± enteral feeding ‡ S ± shock SIGNS AND SYMPTOMS: PUNASAN mo nah ‡ P ± period of apnea ‡ U ± undigested milk of > 2ml ‡ N ± not normal BP ‡ A ± aw! Stool = positive occult blood ‡ S ± stomach not full empty ‡ A ± abdomen distended ‡ N ± not stabilized temperature LABORATORY/DX ‡ MEASUREMENT OF ABDOMINAL GIRTH IS INCREASED ‡ XRAY NURSING MANAGEMENT ‡ BREAST FEEDING IS DISCONTINUED OR FORMULA FEEDINGS ‡ IV OR TOTALPARENTERAL NUTRITION IS MAINTAINED ‡ HANDLE THE ABDOMEN GENTLY TO LESSEN THE POSSIBILITY OF PERFORATION ‡ TEMPORARY COLOSTOMY TO PROVIDE BOWEL FUNCTION PROGNOSIS ‡ Typical recovery from NEC if medical, non-surgical treatment succeeds, includes 10±14 days or more without oral intake and then demonstrated ability to resume feedings and gain weight. Recovery from NEC alone may be compromised by co-morbid conditions that frequently accompany prematurity. Longterm complications of medical NEC include bowel obstruction and anemia. Despite a significant mortality risk, long-term prognosis for infants undergoing NEC surgery is improving, with survival rates of 70-80%. "Surgical NEC" survivors are at-risk for complications including short bowel syndrome, and neurodevelopmental disability.

‡

CELIAC DISEASE Sensitivity or Abnormal immunologic response to protein, particulary the gluten factor of protein found in grains, wheat, rye, oats and barley. STEATORRHEA- bulky, foul-smelling, fatty stools Deficiency on Fat-soluble vitamins ADEK (vitamins are not absorbed because fat is not absorbed) ASSESSMENT: Characteristics: Anorexic, irritable, appears skinny, splindly extremities, wasted buttocks but face is plump and well-rounded Clinical symptoms: bulky stools, malnutrition, distended abdomen, anemia between 6-18 months of age Diagnostic/laboratory test: - glucose tolerance test- poor absorption - stool analysis- increase of antibodies - serum analysis of antibodies ± IgA antiglandin antibodies - endoscopy ± biopsy for intestinal mucosa Treatment Management: - gluten free diet for life - water soluble forms of vitamin A and D - iron folate for anemia Nursing Diagnosis : imbalance nutrition less than body requirements r/t malabsorption of food LACTOSE INTOLERANCE Lactose intolerance is the inability to digest and absorb lactose (the sugar in milk) that results in gastrointestinal symptoms when milk or food products containing milk are consumed. Also caused by reduced or absent activity of lactase that prevents the splitting of lactose. Lactase deficiency may occur for one of the three reasons, congenital, secondary or developmental. Assessment: Abdominal pain, diarrhea and flatulence Diagnostic/Laboratory procedure: Breath test, blood glucose, stool acidity test and intestinal biopsy Management: - dietary changes- reducing amount of lactose in diet - avoidance of milk-containing product - lactase enzyme- caplets or tablets of lactase are available to take with milk-containing foods.

LEAD POISONING Lead poisoning (also known as plumbism, colica Pictonum, saturnism, Devon colic, or painter's colic) is a medical condition caused by increased levels of the heavy metal lead in the body. Lead interferes with a variety of body processes and is toxic to many organs and tissues including the heart, bones, intestines, kidneys, and reproductive and nervous systems. It interferes with the development of the nervous system and is therefore particularly toxic to children, causing potentially permanent learning and behavior disorders. Symptoms include abdominal pain, confusion, headache, anemia, irritability, and in severe cases seizures, coma, and death. ETIOLOGY Routes of exposure to lead include contaminated air, water, soil, food, and consumer products. Occupational exposure is a common cause of lead poisoning in adults. One of the largest threats to children is lead paint that exists in many homes, especially older ones; thus children in older housing with chipping paint are at greater risk. Prevention of lead exposure can range from individual efforts (e.g. removing lead-containing items such as piping or blinds from the home) to nationwide policies (e.g. laws that ban lead in products or reduce allowable levels in water or soil). Elevated lead in the body can be detected by the presence of changes in blood cells visible with a microscope and dense lines in the bones of children seen on X-ray. However, the main tool for diagnosis is measurement of the blood lead level; different treatments are used depending on this level. The major treatments are removal of the source of lead and chelation therapy (administration of agents that bind lead so it can be excreted). Humans have been mining and using this heavy metal for thousands of years, poisoning themselves in the process. Although lead poisoning is one of the oldest known work and environmental hazards, the modern understanding of the small amount of lead necessary to cause harm did not come about until the latter half of the 20th century. No safe threshold for lead exposure has been discovered²that is, there is no known amount of lead that is too small to cause the body harm. Signs and symptoms Lead poisoning can cause a variety of symptoms and signs which vary depending on the individual and the duration of lead exposure. Symptoms are nonspecific and may be subtle, and someone with elevated lead levels may have no symptoms.Symptoms usually develop over weeks to months as lead builds up in the body during a chronic exposure, but acute symptoms from brief, intense exposures also occur Symptoms from exposure to organic lead, which is probably more toxic than inorganic lead due to its lipid solubility, occur rapidly.Poisoning by organic lead compounds has symptoms predominantly in

the central nervous system, such as insomnia, delirium, cognitive deficits, tremor, hallucinations, and convulsions. The classic signs and symptoms in children are loss of appetite, abdominal pain, vomiting, weight loss, constipation, anemia, kidney failure, irritability, lethargy, learning disabilities, and behavior problems. Diagnosis Blood film examination may reveal basophilic stippling of red blood cells (dots in red blood cells visible through a microscope), as well as the changes normally associated with iron-deficiency anemia (microcytosis and hypochromasia).However, basophilic stippling is also seen in unrelated conditions, such as megaloblastic anemia caused by vitamin B12 (colbalamin) and folate deficiencies. Exposure to lead also can be evaluated by measuring erythrocyte protoporphyrin (EP) in blood samples EP is a part of red blood cells known to increase when the amount of lead in the blood is high, with a delay of a few weeks.Thus EP levels in conjunction with blood lead levels can suggest the time period of exposure; if blood lead levels are high but EP is still normal, this finding suggests exposure was recent.However, the EP level alone is not sensitive enough to identify elevated blood lead levels below about 35 g/dL.Due to this higher threshold for detection and the fact that EP levels also increase in iron deficiency, use of this method for detecting lead exposure has decreased. Blood lead levels are an indicator mainly of recent or current lead exposure, not of total body burden. Lead in bones can be measured noninvasively by X-ray fluorescence; this may be the best measure of cumulative exposure and total body burden. Fecal lead content that is measured over the course of a few days may also be an accurate way to estimate the overall amount of childhood lead intake. This form of measurement may serve as a useful way to see the extent of oral lead exposure from all the diet and environmental sources of lead. NANDA PROBLEMS SITUATIONAL LOW SELF ESTEEM REALATED TO CHILDS POISONING NURSING MANAGEMENT y Child¶s BLL >10g/dL y Provide educational interventions to the parent/caregiver. y Communicate assessments and interventions to the health care provider y Monitor timeliness and levels of follow-up blood lead tests y Initiate appropriate interventions if level rises y Provide ongoing evaluation to see that BLLs decline and outcome measures are achieved.

PROGNOSIS y Outcome is related to the extent and duration of lead exposure.Effects of lead on the physiology of the kidneys and blood are generally reversible; its effects on the central nervous system are not. While peripheral effects in adults often go away when lead exposure ceases, evidence suggests that most of lead's effects on a child's central nervous system are irreversible. Children with lead poisoning may thus have adverse health, cognitive, and behavioral effects that follow them into adulthood.

Omphalocele - Is a protrusion of abdominal contents through the abdominal wall at the point of the junction of the umbilical cord and the abdomen. The herniated organs are usually the intestines, but they may include the stomach and liver. Usually covered and contained by a thin transparent layer of amnion and chorion with the umbilical cord protruding from the exposed sac. ETIOLOGY This condition occurs because at approximately weeks 6-8 of intrauterine life, the fetal abdominal contents, are extruded from the abdomen into the base of the umbilical cord. Normally at 7-10 weeks when the abdomen has enlarged sufficiently, the intestines return to the abdomen. Omphalocele occurs when the abdomen fails to return the usual way. Fetal omphalocele may occur in conjunction with other conditions, including cardiac or genitourinary abnormalities, neural tube defects or twisted intestines, as well as the genetic defects trisomy 13 or 18. In addition, omphalocele may also be associated with Beckwith-Wiedermann Syndrome or Pentalogy of Cantrell. For this reason, fetuses with omphalocele are carefully evaluated to rule out these abnormalities SIGNS AND SYMPTOMS An omphalocele can be clearly seen, because the abdominal contents stick out (protrude) through the belly button area. There are different sizes of omphaloceles. In small ones, only the intestines stick out. In larger ones, the liver or spleen may stick out of the body as well. DIAGNOSTICS The diagnosis of omphalocele is confirmed by ultrasound. Because of the increased risk of an associated anomaly, a targeted ultrasound will be performed by a perinatologist. Omphaloceles can be associated with (up to 30 percent) some chromosomal abnormalities so an amniocentesis may be offered. An ultrasound of your baby's heart (fetal echocardiogram) may also be recommended. These babies will be monitored closely throughout the pregnancy. Ultrasounds will be done every two to four weeks to assess how well the fetus is growing, the amniotic fluid volume and fetal well-being. You will be instructed on daily fetal movement counting at about 26 weeks' gestation. Non-stress tests (a recording of the baby's heart rate while you are sitting and pressing a button each time the baby moves) may be scheduled at around 32 weeks' gestation. Biophysical profiles (BPP) may also be scheduled weekly. The BPP is an ultrasound that monitors amniotic fluid volume, the baby's breathing movements,

and movements of the extremities along with the nonstress test. Also Elevated AFP (Alpha-feto protein) may be an indicative of omphalocele. The method of delivery will be discussed as the time gets closer. The method of delivery is dependent on the size of the omphalocele. If the size is quite large and especially if the liver is involved, the doctor may prefer to do a cesarean section to avoid the risk of injury to the liver. Otherwise, the preferred method of delivery is vaginal. Other reasons to do a C-section for a baby with an omphalocele include those reasons that would affect a routine pregnancy. Labor may be induced or allowed to start on its own.

TREATMENT Omphaloceles are repaired with surgery, although not always immediately. A sac protects the abdominal contents and allows time for other more serious problems (such as heart defects) to be dealt with first, if necessary. To fix an omphalocele, the sac is covered with a special man-made material, which is then stitched in place. Slowly, over time, the abdominal contents are pushed into the abdomen. When the omphalocele can comfortably fit within the abdominal cavity, the manmade material is removed and the abdomen is closed. Sometimes the omphalocele is so large that it cannot be placed back inside the infant's abdomen. The skin around the omphalocele grows and eventually covers the omphalocele. The abdominal muscles and skin can be repaired when the child is older to achieve a better cosmetic outcome. PROGNOSIS Complete recovery is expected after surgery for an omphalocele. However, omphaloceles often occur with other birth defects. How well a child does depends on which other conditions the child also has. If the omphalocele is identified before birth, the mother should be closely monitored to make sure the unborn baby remains healthy. Plans should be made for careful delivery and immediate management of the problem after birth. The baby should be delivered in a medical center that is skilled at repairing omphaloceles. The baby's outcome is improved if he or she does not need to be taken to another center for further treatment. Parents should consider screening their unborn baby for other genetic problems that are associated with this condition. NURSING DIAGNOSIS: y Impaired skin integrity related to protrusion in the umbilicus y Imbalanced nutrition: less than body requirements y Risk for constipation y Risk for infection

Gastroschisis - Gastroschisis (also called paraomphalocele) represents a congenital defect characterized by a defect in the anterior abdominal wall through which the intestinal contents freely protrude. There is no overlying sac and the size of the defect is usually less than 4 cm. The abdominal wall defect is located at the junction of the umbilicus and normal skin, and is almost always to the right of the umbilicus ETIOLOGY High-risk pregnancies such as those complicated by infection, young maternal age, smoking, drug abuse, or anything that contributes to low birth weight can increase the incidence of gastroschisis, which is more frequent in newborns who are small for gestational age. Whether the intrauterine growth retardation can facilitate the apparition of gastroschisis or the abdominal wall defect impairs fetal growth is not clear yet. A change in paternity (childbearing with different fathers) has been implicated as a risk factor in a recent study, suggesting that the immune system of the mother may play a role in the development of gastroschisis. Gastroschisis as a stand-alone congenital defect is usually inherited in an autosomal recessive manner. It may begin as a sporadic mutation, can be associated with non-genetic congenital disorders, but has also been observed to be autosomal dominant. The malformation is slightly more frequent in males than females. The frequency of gastroschisis is associated with young maternal age, and low number of gestations. This abnormality is seen in ratio of 1:10,000 and is usually detected before birth. It has been reported that the incidence of gastroschisis has increased in recent years.

SIGNS AND SYMPTOMS > Lump in the abdomen > Intestine sticks through the abdominal wall near the umbilical cord DIAGNOSTICS Physical examination of the infant is enough for the health care provider to diagnose gastroschisis. The baby will have problems with movement and absorption in the gut, because the unprotected intestine is exposed to irritating amniotic fluid.

The mother may have shown signs of too much amniotic fluid (polyhydramnios). A prenatal ultrasound often identifies the gastroschisis.

TREATMENT If gastroschisis is found before birth, the mother will need special monitoring to make sure her unborn baby remains healthy. Plans should be made for careful delivery and immediate management of the problem after birth. Treatment for gastroschisis is surgery to repair the defect. A surgeon will put the bowel back into the abdomen and close the defect, if possible. If the abdominal cavity is too small, a mesh sack is stitched around the borders of the defect and the edges of the defect are pulled up. Over time, the herniated intestine falls back into the abdominal cavity, and the defect can be closed. Other treatments for the baby include nutrients by IV and antibiotics to prevent infection. The baby's temperature must be carefully controlled, because the exposed intestine allows a lot of body heat to escape. PROGNOSIS Current advances in surgical techniques and intensive care management for neonates have increased the survival rate to 90%, in adequate settings. The possibility of prenatal diagnosis either through echosonogram or any other method available allows the mother to be referred to an adequate center where a caesarean section or induced natural birth can be performed before term (as natural birth is recommended and just as safe as with a normal baby), preferably within 2 weeks of term, and allow the immediate surgery to be performed on the newborn. The general procedure for gastroschisis is to simply tuck the protruding organs back into the opening and apply a belly band pressure until the wound heals itself. New advances have been pioneered in repairing the protruding bowel by placing a protective "silo" around the intestine outside the abdomen, then slowly pressuring the herniated intestine into the abdominal cavity. This new procedure allows for the bowel to return to its intended shape and location without further traumatizing the infant's viscera with undue internal pressure. The main cause for lengthy recovery periods in patients is the time taken for the infants' bowel function to return to normal. The morbidity is closely related to the presence of other malformations and complications of the wound or the intestine. Patients frequently require more than one surgery.

NURSING DIAGNOSIS y y y y Impaired skin integrity related to protrusion in the abdomen Imbalanced nutrition: less than body requirements Risk for constipation Risk for infection

BACKGROUND Biliary Atresia - is a rare condition in newborn infants in which the common bile duct between the liver and the small intestine is blocked or absent. If unrecognized, the condition leads to liver failure, as the liver is still able to conjugate bilirubin, and conjugated bilirubin is unable to cross the blood-brain barrier. ETIOLOGY The cause of biliary atresia is UNKNOWN. The two types of biliary atresia appear to be a ³fetal´ form, which arises during fetal life and is present at the time of birth, and a ³perinatal´ form, which is more typical and does not become evident until the second to fourth week of life. An important fact is that biliary atresia is not an inherited disease. Cases of biliary atresia do not run in families; identical twins have been born with only one child having the disease. Biliary atresia is most likely caused by an event occurring during fetal life or around the time of birth.

SIGNS AND SYMPTOMS Newborns with this condition may appear normal at birth. However, jaundice (a yellow color to the skin and mucous membranes) develops by the second or third week of life. The infant may gain weight normally for the first month, but then will lose weight and become irritable, and have worsening jaundice. Other symptoms may include: y Pale or clay-colored stools y Dark urine y Slow growth y Enlarged spleen y Slow or no weight gain y Floating stools y Foul-smelling stools DIAGNOSTICS y The health care provider will perform a physical exam, which includes feeling the patient's belly area. The doctor may feel an enlarged liver.

Tests to diagnose biliary atresia include: y Abdominal x-ray y Abdominal ultrasound y Blood tests to check total and direct bilirubin levels y Hepatobiliary iminodiacetic acid (HIDA) scan, also called

y

cholescintigraphy, to help determine whether the bile ducts and gallbladder are working properly Liver biopsy to determine the severity of cirrhosis or to rule out other causes of jaundice

y

X-ray

of

the

bile

ducts

(cholangiogram)

TREATMENT Surgery - If biliary atresia appears to be the cause of the jaundice in the newborn, the next step is surgery. At the time of surgery the bile ducts can be examined and the diagnosis confirmed. For this procedure, the infant is sedated. While the infant is asleep, the surgeon makes an incision in the abdomen to directly examine the liver and bile ducts. If the surgeon confirms that biliary atresia is the problem, a Kasai procedure will usually be performed on the spot. The Kasai Procedure Biliary Atresia occurs within the first two months of life when there is a blockage of the bile duct connecting the liver to the gut. Eventually the blockage will cause a buildup of bile in the liver that leads to damage and scarring of the liver cells, and if untreated, this scarring will cause liver failure and the need for liver transplantation. The Kasai operation attempts to reconstruct the bile duct with a loop of intestine. In the Kasai procedure the small intestine is attached to the liver directly so that the bile may drain. The Children's Hospital sees six to ten new patients with biliary atresia every year and the Kasai procedure is the most effective treatment available. While the Kasai procedure allows some children who would otherwise not be able to live normal healthy lives return to normal growth and activities, most patients with this disease will still eventually need a Liver transplant. Liver transplant. If the Kasai procedure is not successful, the infant usually will need a liver transplant within the first 1 to 2 years of life. Children with the fetal form of biliary atresia are more likely to need liver transplants²and usually sooner²than infants with the typical perinatal form. The pattern of the bile ducts affected and the extent of damage can also influence how soon a child will need a liver transplant. (More about liver transplantation follows.) PROGNOSIS Timely Kasai portoenterostomy (e.g. < 60 postnatal days) has shown better outcomes. Nevertheless, a considerable number of the patients, even if Kasai portoenterostomy has been successful, eventually undergo liver transplantation within a couple of years after Kasai portoenterostomy.

Recent large volume studies from Davenport et al. (Ann Surg, 2008) show that age of the patient is not an absolute clinical factor affecting the prognosis. In the latter study, influence of age differs according to the disease etiology²i.e., whether isolated BA, BASM (BA with splenic malformation ), or CBA(cystic biliary atresia). It is widely accepted that corticosteroid treatment after a Kasai operation, with or without choleretics and antibiotics, has a beneficial effect on the postoperative bile flow and can clear the jaundice; but the dosing and duration of the ideal steroid protocol have been controversial ("blast dose" vs. "high dose" vs. "low dose"). Furthermore, it has been observed in many retrospective longitudinal studies that steroid does not prolong survival of the native liver or transplant-free survival. Davenport at al. also showed (hepatology 2007) that short-term lowdose steroid therapy following a Kasai operation has no effect on the mid- and long-term prognosis of biliary atresia patients.

NURSING DIAGNOSIS y y y y Neonatal Jaundice Imbalanced nutrition: less than body requirements Impaired skin integrity Delayed development

BACKGROUND Cirrhosis - is a condition in which the liver slowly deteriorates and malfunctions due to chronic injury. Scar tissue replaces healthy liver tissue, partially blocking the flow of blood through the liver. Scarring also impairs the liver¶s ability to control infections remove bacteria and toxins from the blood process nutrients, hormones, and drugs make proteins that regulate blood clotting produce bile to help absorb fats² including cholesterol²and fat-soluble vitamins A healthy liver is able to regenerate most of its own cells when they become damaged. With end-stage cirrhosis, the liver can no longer effectively replace damaged cells. A healthy liver is necessary for survival. ETIOLOGY Cirrhosis in children often stems from a wide variety of liver disorders, including (but certainly not limited to): y Bile duct diseases: y Hepatitis B and C  Biliary artresia y Autoimmune hepatitis  Sclerosing cholangitis y Inherited diseases:  Congenital hepatic fibrosis  Glycogen storage disease  Choledochal cysts  Tyrosinemia y Drugs and toxins:  Wilson disease  Isoniazid  Alpha1-antitrypsin deficiency  Methotrexate  Cystic fibrosis  Excess vitamin A y Fatty liver disease SIGNS AND SYMPTOMS As the disease progresses, bile flow is blocked or stopped, and jaundice (yellow skin or eyes) appears. The same bile pigment, bilirubin, which is responsible for the yellow skin tones of jaundice can turn urine dark. Bleeding and bruising can occur more easily and take longer to heal. Other later symptoms, some due to complications, include: y y y y y Reddened palms Loss of body hair Enlarged liver Enlarged spleen Appearance of thin, purplish-red, spidery looking blood vessels on the skin, especially around the navel y y y y y y y Water retention and swelling in the legs and abdomen Vomiting blood Itching Abdominal infections Forgetfulness or confusion Tremors Inability to fully process drugs

y

Enlarged, twisted, thin-walled blood vessels called varices in the esophagus and/or stomach

y

that can rupture and lead to lifethreatening bleeding Liver cancer

DIAGNOSTICS y y y Blood Tests ± to assess how well the liver is working and determine a cause CT Scan, Ultrasound, MRI or Liver/Spleen Scan to identify changes in the liver Liver Biopsy ± analyzing a sample of liver tissue removed via a thin needle inserted into the liver

TREATMENT In general, cirrhosis cannot be cured or reversed. Treatment for cirrhosis depends on the cause of the disease and whether complications are present. The goals of treatment are to slow the progression of scar tissue in the liver and prevent or treat the complications of the disease. Hospitalization may be necessary for cirrhosis with complications. A liver transplant is considered when complications cannot be controlled by treatment. Liver transplantation is a major operation in which the diseased liver is removed and replaced with a healthy one from an organ donor. A team of health professionals determines the risks and benefits of the procedure for each patient. Survival rates have improved over the past several years because of drugs that suppress the immune system and keep it from attacking and damaging the new liver. PROGNOSIS When the complications developed are not controlled with the help of the treatment, liver transplant is recommended. Liver transplant dramatically improves the cirrhosis of the liver prognosis. Cirrhosis life expectancy is about 15 to 20 years when cirrhosis is detected during the initial stage. Life expectancy decreases to about 6 to 10 years, when cirrhosis is detected in second stage. But these patients have enough time and can opt for liver transplant. They have various treatment options and medicines which can help control the advancement of the disease. When cirrhosis of the liver is diagnosed during the last stage, the life expectancy is very poor, about 1-3 years, depending upon the patient's overall health, prompt use of advanced treatment, etc. The Child-Pugh Classification The Child-Pugh classification is a scoring system which helps determine the cirrhosis of the liver prognosis. Here, scoring is based upon several factors:

albumin, ascites, total bilirubin, prothrombin time, and encephalopathy, as follows: Name of the Factor Serum Albumin (g/dL) Serum Bilirubin (mg/dL) Prothrombin (seconds) Ascites Encephalopathy Score: 1 Point Score: Points than 3.0 - 3.5 2 Score: 3 Points Lower than 3.0 Higher 3.0 Higher 6.0 Severe Severe than than

Higher 3.5 Lower than 2.0 2.0 - 3.0 4-6 Moderate Mild

time 1 - 4 None None

The three classes are formed and their scores are: y Class A - score of 5 - 6 y Class B - score of 7 - 9 y Class C - score higher than 9 y Patients with a score of 10 or more (in the Class C category): Have a prognosis with 1 year survival being about 50%. y Patients with Class A or B: Have a better prognosis of 5-years, with a survival rate of 70%- 80%. y Refractory ascites, albumin < 3.2 gm/l, and an episode of SBP (spontaneous bacterial peritonitis): A one-year survival of 50% or less. Studies show that liver cirrhosis affects men slightly more often than women and each year, thousands of people die because of liver cirrhosis. I hope you find the above information regarding cirrhosis of the liver prognosis helpful. With healthy diet, exercises and healthy eating habits, you may succeed in keeping deadly diseases away. NURSING DIAGNOSIS: y y Excess fluid volume related to compromised regulatory system Impaired skin integrity

HERNIAS  It is a protrusion of a portion of an organ or organs through an abnormal opening.  The danger from herniation arises when the organ protruding through the opening is constricted to the extent that circulation is impaired or when the protruding organs enroach on and the impair the function of other structures. Incarcerated hernia ± a hernia that cannot be reduced easily Strangulated hernia ± a hernia in which the blood supply to the herniated organ is impaired. Types of Hernia DIAPHRAGMATIC HERNIA A diaphragmatic hernia is a birth defect in which there is an abnormal opening in the diaphragm, the muscle that helps you breathe. The opening allows part of the organs from the belly (stomach, spleen, liver, and intestines) to go up into the chest cavity near the lungs.

CAUSES A diaphragmatic hernia is caused by the improper joining of structures during fetal development. As a result, the abdominal organs such as the stomach, small intestine, spleen, part of the liver, and the kidney appear in the chest cavity. The lung tissue on the affected side is thus not allowed to completely develop. Congenital diaphragmatic hernia is seen in 1 out of every 2,200 to 5,000 live births. Most affect the left side. Having a parent or sibling with the condition slightly increases your risk. SIGNS AND SYMPTOMS  Mild to severe respiratory distress within a few hours after birth  Tachypnea, tachycardia  Cyanosis  Absent breath sound in affected area DIAGNOSTIC TESTS  Chest Ultrasonography ± may show abdominal organs in the chest cavity  Pelvic Ultrasonography (for mother) ± may show  Bluish colored skin due to lack of oxygen  Abdomen generally appears sunken  May lead to R to L shunting through the foramen ovale in the heart and can cause patent ductus arteriosus excessive amounts amniotic fluid  Fetoscopy of

NURSING MANAGEMENT Pre-operative: 1. Reduce stimulation ± environmental/ care activities 2. Prompt recognition, resuscitation and stabilization 3. Maintain suction, oxygen and IV fluids 4. Positioning ± head up 5. Administer medication as ordered. Post-operative; 1. Carry out routine post operative care and observation 2. Relieve pain and provide comfort 3. Support family because this is a critical illness THERAPEUTIC MANAGEMENT  Supportive treatment of respiratory distress and correction of acidosis, possible use of endotracheal intubation, GI decompression  Surgical reduction of hernia and repair of defect

- if the defect in the diaphragm is large, an insoluble polymer(Teflon) patch may be used in reconstruction. - in infant, abdominal incision may not be closed, it is covered by silicon elastomer (Silastic) and left to be closed at a later date after the abdomen has grown. NURSING DIAGNOSIS  Risk for ineffetive airway clearance related to displaced bowel  Risk for imbalanced nutrition related, less than body requirements related to NPO status PROGNOSIS  Congenital diaphragmatic hernia is a very serious disorder. The outcome of surgery depends on how well your baby's lungs have developed. Usually the outlook is very good for infants who have enough lung tissue.  With advances in neonatal and surgical care, survival is now greater than 80%. HIATAL HERNIA Hiatal hernia is a condition in which a portion of the stomach protrudes upward into the chest, through an opening in the diaphragm. The diaphragm is the sheet of muscle that separates the chest from the abdomen. It is used in breathing. CAUSES The cause is unknown, but hiatal hernias may be the result of a weakening of the supporting tissue. Increasing age, obesity, and smoking are known risk factors in adults. Children with this condition are usually born with it (congenital). It is often associated with gastroesophageal reflux in infants. Hiatal hernias are very common, especially in people over 50 years old. This condition may cause reflux (backflow) of gastric acid from the stomach into the esophagus. SIGNS AND SYMPTOMS  Dysphagia  Failure to thrive  Vomiting  Neck contortions DIAGNOSTIC TESTS  Barium swallow x-ray  Esophagogastroduodenoscopy (EGD) NURSING MANAGEMENT 1. Positioning ± a baby can be kept in an upright position to help prevent the condition from reoccurring.  Frequent unexplained respiratory problem  May cause gastric volvulus and obstruction  Heartburn

2. Give medications as ordered. Medication to reduce acid secretion may be helpful. THERAPEUTIC MANAGEMENT  Pharmacologic treatment  Surgical treatment ± laparoscopic surgery NURSING DIAGNOSIS  Pain related to indigestion of food  Imbalanced nutrition, less than body requirements related to limiting of foods PROGNOSIS Most symptoms are alleviated with treatment. UMBILICAL HERNIA Umbilical hernias are especially common in infants of African descent, and occur more in boys. They involve protrusion of intraabdominal contents through a weakness at the site of passage of the umbilical cord through the abdominal wall. These hernias often resolve spontaneously. Umbilical hernias in adults are largely acquired, and are more frequent in obese or pregnant women. Abnormal decussation of fibers at the linea alba may contribute. CAUSES An umbilical hernia in an infant occurs when the muscle through which blood vessels pass to feed the developing fetus doesn't close completely. Umbilical hernias are common in infants. They occur slightly more often in African Americans. Most umbilical hernias are not related to disease. However, umbilical hernias can be associated with rare conditions such as mucopolysaccharide storage diseases, Beckwith-Wiedemann syndrome, and Down syndrome. SIGNS AND SYMPTOMS  Noted by inspection and palpation of the abdomen  About 1 to 2 cm (1/2 to 1inch) in diameter but may be as big as an orange.  There is a soft swelling over the belly button that often bulges when the baby sits up, cries, or strains. The bulge may be flat when the infant lies on the back and is quiet. DIAGNOSTIC TESTS  Ultrasonography  The doctor can find the hernia during a physical exam. NURSING MANAGEMENT 1. Discourage use of home remedies such as belly bands, coins

2. Encourage parents to spongebath the child until they for postoperative visit and until the dressing is removed. THERAPEUTIC MANAGEMENT  No treatment of small defects  Operative repair if persists to age 4 to 6 year or if defect is > 1.5-2.0 cm by age 2  Strangulation requires immediate attention  Herniorrhaphy NURSING DIAGNOSIS  Pain related to the protrusion of intra abdominal organs. PROGNOSIS Most umbilical hernias get better without treatment by the time the child is 3 - 4 years old. Those that do not close may need surgery. Umbilical hernias are usually painless. INGUINAL HERNIA An inguinal hernia is a condition in which intra-abdominal fat or part of the small intestine, also called the small bowel, bulges through a weak area in the lower abdominal muscles. An inguinal hernia occurs in the groin²the area between the abdomen and thigh. This type of hernia is called inguinal because fat or part of the intestine slides through a weak area at the inguinal ring, the opening to the inguinal canal. An inguinal hernia appears as a bulge on one or both sides of the groin. An inguinal hernia can occur any time from infancy to adulthood and is much more common in males than females. Inguinal hernias tend to become larger with time. CAUSES The two types of inguinal hernia have different causes. Indirect inguinal hernia. Indirect inguinal hernias are congenital hernias and are much more common in males than females because of the way males develop in the womb. In a male fetus, the spermatic cord and both testicles²starting from an intra-abdominal location²normally descend through the inguinal canal into the scrotum, the sac that holds the testicles. Sometimes the entrance of the inguinal canal at the inguinal ring does not close as it should just after birth, leaving a weakness in the abdominal wall. Fat or part of the small intestine slides through the weakness into the inguinal canal, causing a hernia. In females, an indirect inguinal hernia is caused by the female organs or the small intestine sliding into the groin through a weakness in the abdominal wall. Indirect hernias are the most common type of inguinal hernia. Premature infants are especially at risk for indirect inguinal hernias because there is less time for the inguinal canal to close.

Direct inguinal hernia. Direct inguinal hernias are caused by connective tissue degeneration of the abdominal muscles, which causes weakening of the muscles during the adult years. Direct inguinal hernias occur only in males. The hernia involves fat or the small intestine sliding through the weak muscles into the groin. A direct hernia develops gradually because of continuous stress on the muscles. One or more of the following factors can cause pressure on the abdominal muscles and may worsen the hernia: y straining on the toilet because y sudden twists, pulls, or of constipation muscle strains y weight gain y lifting heavy objects y chronic coughing SIGNS AND SYMPTOMS  a small bulge in one or both sides of the groin that may increase in size and disappear when lying down; in males, it can present as a swollen or enlarged scrotum  discomfort or sharp pain²especially when straining, lifting, or exercising² that improves when resting  a feeling of weakness or pressure in the groin  a burning, gurgling, or aching feeling at the bulge TESTS  family history taking  physical examination NURSING MANAGEMENT Keep the suture line dry and free of urine or feces to prevent infection. THERAPEUTIC MANAGEMENT  Herniorrhaphy (Hernioplasty, Hernia repair) is a surgical procedure for correcting hernia. A hernia is a bulging of internal organs or tissues, which protrude through an abnormal opening in the muscle wall. Hernias can occur in the abdomen, groin, and at the site of a previous surgery.  An operation in which the hernia sac is removed without any repair of the inguinal canal is described as a 'herniotomy'.  When herniotomy is combined with a reinforced repair of the posterior inguinal canal wall with autogenous (patient's own tissue) or heterogeneous (like steel or prolene mesh) material it is termed Hernioplasty as opposed to herniorrhaphy in which no autogenous or heterogeneous material is used for reinforcement. PROGNOSIS Inguinal hernia repair is a routine operation with very few risks. Without an operation, the hernia will get bigger, become more painful and could lead to complications, such as a strangulated hernia. Surgery will get rid of the hernia

and prevent you from having any serious complications. After having the operation, you should be able to go home the same day or the day after. MALROTATION AND VOLVOLUS Malrotation of the intestines results when the intestinal rotation and fixation that occurs during pregnancy fails to occur. This normally happens in the 4th and 12th weeks of fetal life. In the 4th fetal week, the entire bowel is basically a straight tube with the superior mesenteric artery (SMA). During the course of pregnancy, the bowel rotates in place to the left of the SMA at the ligament of Treitz. In normal rotation and fixation of the intestines, the bowel has plenty of room to function normally. In malrotation, the primary concern becomes volvulus, or twisting of the intestine that causes obstruction and death to that part of the gut. Accidental bodily movements, unusual effort, abnormal peristaltic movement (digestive wavelike motion of the intestines) or distention of the intestine can bring on this volvulus. CAUSES The cause of intestinal malrotation is disruption in the normal embryological development of the bowel. Clinical features depend on the stage of disruption and are discussed as follows. A full understanding of normal development aids in understanding the etiology of malrotation. SIGNS AND SYMPTOMS  Intermittent vomiting  Recurrent abdominal pain DIAGNOSTIC TESTS  Upper GI series THERAPEUTIC MANAGEMENT  Laparotomy  Untwisting by performing sigmoidoscopy and placing rectal tube, monitor for signs of bowel ischemia for 2-3 days, if no improvement, consult surgery for laparotomy (sigmoid resection and primary anastamosis)  Transduodenal band of ladd is divided PROGNOSIS Younger patients have higher rates of morbidity and mortality. In infants, the mortality rate ranges from 2-24%. The presence of necrotic bowel at surgery increases the mortality rate by 25 times for infants, and the presence of other anomalies increases the risk by 22 times. A report of 25 years' experience demonstrated congenital cardiovascular disease in 27.1% of patients with intestinal malrotation; those patients had a morbidity rate of 61.1% after intestinal malrotation surgery. Meckel's Diverticulum  Distention  Lower GI bleeding

Meckel's diverticulum, a congenital diverticulum, is a small bulge in the small intestine present at birth. It is a vestigial remnant of the omphalomesentric duct http://en.wikipedia.org/wiki/Omphalomesenteric_duct(also called the vitelline duct or yolk stalk), and is the most frequent malformation of the gastrointestinal tract. It is present in approximately 2% of the population, with males more frequently experiencing symptoms. Meckel's diverticulum is located in the distal ileum, usually within about 60100 cm (2 feet) of the ileocecal valve. It is typically 3-5 cm long, runs antimesenterically and has its own blood supply. It is a remnant of the connection from the yolk-sac to the small intestine present during embryonic development. Rule of 2s: 2% (of the population). 2 feet (from the ileocecal valve). 2 inches (in length). 2% are symptomatic. 2 types of common ectopic tissue (gastric and pancreatic). 2 years is the most common age at clinical presentation. 2 times more boys are affected. Symptoms y The majority of people afflicted with Meckel's diverticulum are asymptomatic. If symptoms do occur, they typically appear before the age of two. y painless rectal bleeding y black tarry stool y intestinal obstruction y severe pain in upper abdomen y bloating stomach y it is followed by volvulus and intussusception Diagnosis y Technetium-99m (99mTc) pertechnetate scan - detects gastric mucosa y Colonoscopy y Screenings for bleeding disorders y Angiography Treatment y Surgical resection ±done in conditions such as bleeding, strangulation of bowel, bowel perforation or bowel obstruction. y Appendicectomy/ laparotomy ± for asymptomatic meckel¶s diverticulum with acute abdomen; prevents complication. Prognosis y Once a complication arises and surgery is required, the operative mortality and morbidity rates have both been estimated at 12%. The cumulative long-term risk of postoperative complications in this cohort was found to be 7%. If the Meckel diverticulum is removed as an incidental finding, the risk of mortality and morbidity and long-term complications are much less (1%, 2%, and 2%, respectively). y As many as 5% of complicated Meckel diverticulum contain malignant tissu

Short bowel syndrome It is also called short gut syndrome or simply short gut. It is a malabsorption disorder caused by the surgical removal of the small intestine, or rarely due to the complete dysfunction of a large segment of bowel. Most cases are acquired, although some children are born with a congenital short bowel. It usually does not develop unless more than two thirds of the small intestine have been removed. Signs and symptoms y Complications ± deficiencies y Abdominal pain in Vitamin A, D, E, K, y Diarrhea and steatorrhea (oily and B12, calcium, magnesium or sticky stool, which can be , iron, folic acid, and zinc. malodorous) Also, y Fluid retention anemia, hyperkeratosis (scali y Weight loss ng of the skin), easy bruising, y Malnutrition muscle spasms, poor blood y Fatigue clotting, and bone pain appears. Laboratory/Diagnostic Tests y Small intestine x-ray y Blood chemistry tests (such as albumin level) y Vitamin levels in the blood y Complete blood count (CBC) y Fecal fat test Treatments y Anti-diarrheal medicine (e.g. loperamide, codeine) y Vitamin, mineral supplements and L-Glutamine powder mixed with water y H2 blocker and proton pump inhibitors to reduce stomach acid y Lactase supplement (to improve the bloating and diarrhoea associated with lactose intolerance) y Parenteral nutrition (PN or TPN for total parenteral nutrition - nutrition administered via intravenous line). y Nutrition administered via gastrostomy tubes Surgical procedure y Bianchi procedure - where the bowel is cut in half and one end is sewn to the other y Serial Transverse Enteroplasty(STEP)²where the bowel is cut and stapled in a zigzag pattern y Small intestine transplant y Intestinal lengthening and tapering

Prognosis y There is no cure for short bowel syndrome. In newborn infants, the 4-year survival rate on parenteral nutrition is approximately 70%. In newborn infants with less than 10% of expected intestinal length, 5 year survival is approximately 20%. Some studies suggest that much of the mortality is due to a complication of the TPN, especially chronic liver disease. y Although promising, small intestine transplant has a mixed success rate, with postoperative mortality rate of up to 30%. One-year and 4-year survival rate are 90% and 60%, respectively. HERNIAS Pictures of different hernias

MECKEL¶S DIVERTICULUM

Red Blood Cell Disorders

I.

Aplastic Anemia

BACKGROUND OF THE STUDY Aplastic anemia results from depression of hematopoietic activity in the bone marrow. The formation and development of WBCs, platelets, and RBCs can all be affected.

a) Congenital aplastic anemia (Fanconi's syndrome) is inherited as an autosomal recessive trait. Child is born with a number of congenital anomalies. b) Acquired aplastic is a decrease in bone marrow production. The child is exposed excessively to radiation, drugs, or chemicals known to cause bone marrow damage. (sulfonamides, arsenic (contained in rat poison), hydantoin, quinine, insecticides chemotherapeutic drugs). ASSESSMENT: 1. Low RBC count - child appears pale, fatigues easily, and has anorexia. 2. Reduced Platelet - (thrombocytopenia), the child bruises easily or has petechiae (pinpoint, macular, purplish-red spots caused by intradermal or submucous hemorrhage). excessive nosebleeds or gastrointestinal bleeding. 3. Decreased WBCs (leukopenia), a child may contract an increased number of infections and respond poorly to antibiotic therapy.

MANAGEMENT

The ultimate therapy for aplastic anemia is stem cell transplantation.

If a donor cannot be located, the disease is managed by procedures to suppress T lymphocyte-dependent autoimmune responses with antithymocyte globulin (ATG) or cyclosporine or transfusion of new blood elements. NURSING DIAGNOSIS Risk for injury related to ineffective blood clotting mechanisms secondary to inadequate platelet formation OUTCOME EVALUATION: Child exhibits no ecchymotic skin areas, gingival bleeding, or epistaxis; stools are negative for occult blood. Inadequate platelet formation interferes with blood coagulation, placing a child at risk for bleeding.

PLAN OF CARE: Some techniques for reducing bleeding due to inadequate platelet formation include:
y

y y y y y y y y

y y

Limit the number of blood-drawing procedures; combine samples whenever possible; use a blood pressure cuff instead of a tourniquet to reduce the number of petechiae. Apply pressure to any puncture site for a full 5 minutes before applying a bandage. Minimize use of adhesive tape to the skin (pulling for removal may tear the skin and cause petechiae). Pad side and crib rails to prevent bruising. Protect intravenous sites to avoid numerous reinsertions. Administer medication orally or by intravenous infusion to minimize the number of injection sites. Assess diet for foods that the child can chew without irritation (e.g., avoid toast crusts). Urge the child to use a soft toothbrush. Check toys for sharp corners, which may cause scratches. Urge the child to be careful with paper, because paper cuts can bleed out of proportion to their size. Assess the need for routine blood pressure determinations. Tight cuffs could lead to petechiae. Distract the child from rough play; suggest stimulating but quiet activities to minimize risk of injury.

y

Keep a record of blood drawn; do not draw extra amounts ³just in case´ so children do not become more anemic. Iron Deficiency Anemia

II.

BACKGROUND OF THE STUDY RBCs are both small in size (hypocytic) and pale (hypochromic) due to the stunted hemoglobin. The most common anemia of infancy and childhood, occurring when the intake of dietary iron is inadequate. Children are at high risk for iron-deficiency anemia because they need more daily iron in proportion to their body weight to maintain an adequate iron level than do adults. ETIOLOGY: 1. Infants - Infants of low birthweight have fewer iron stores than those born at term because iron stores are laid down near the end of gestation. Because low-birthweight infants grow rapidly and their need for RBCs expands accordingly, they will develop an iron-deficiency anemia before 5 to 6 months. As a preventive measure, they are given an iron supplement beginning at about 2 months of age. 2. Children ± (older than 2 years) This results from gastrointestinal tract lesions such as polyps, ulcerative colitis, Crohn's disease, protein-induced enteropathies, parasitic infestation, or frequent epistaxis. 3. Adolescent ± (girls) can become iron deficient because of frequent attempts to diet and overconsumption of snack foods low in iron. Without sufficient iron, their body cannot compensate for the iron lost with menstrual flow. ASSESSMENT: a) Pallor b) Pale mucous membranes c) Infants may show poor muscle tone and reduced activity. d) Generally irritable from fatigue e) The heart may be enlarged, and there may be a soft systolic precordial murmur as the heart increases its action, attempting to supply body cells with more oxygen. MANAGEMENT: a) Sources of gastrointestinal bleeding must be ruled out.

b) Diet must be rich in iron and should contain extra vitamin C, as this enhances iron absorption. c) An iron compound such as ferrous sulfate for 4 to 6 wks to improve RBC formation and replace iron stores. NURSING DIAGNOSIS: Imbalanced nutrition, less than body requirements, related to inadequate ingestion of iron

OUTCOME EVALUATION: Parents report child's dietary intake includes iron-rich foods; parents administer ferrous sulfate as prescribed; serum iron levels increase to normal by 6 months. PLAN OF CARE: a) When planning care for an infant with iron-deficiency anemia, it is helpful to minimize the child's activities to prevent fatigue, particularly at mealtime, as a fatigued child will not be able to eat, let alone eat iron-rich foods. b) Counsel parents on measures to improve their child's diet, such as adding iron-rich foods while decreasing milk intake to maintain iron levels and prevent recurring anemia. If the child is not fond of meat, suggest parents substitute cheese, eggs, green vegetables, or fortified cereal. Because iron-rich foods are often expensive, remind parents that these items are important and that they should not substitute less expensive, highcarbohydrate foods. c) Before iron therapy is started, alert parents to possible side effects, such as stomach irritation. If oral iron is not tolerated or if there is a doubt the child will take it, an iron-dextran injection (Imferon) can be given intramuscularly. Imferon stains the skin and is extremely irritating unless it is given by deep Z-track intramuscular injection. d) Of all age groups, adolescents tend to do the least well with taking medicine consistently. Help them plan a daily time for taking their iron supplement with a medication reminder chart. At first, they may reject this as childish, but assure them that everyone needs these charts. Review with them the iron-rich foods they will need to eat daily. An iron supplement is effective only if taken with iron-rich foods. e) After 7 days of iron therapy, a reticulocyte count is usually done. If elevated, this means the child is now receiving adequate iron and the rapid proliferation of new erythrocytes is correcting the anemia. Iron medication must be taken for at least 4 to 6 weeks after the RBC count is normal to rebuild iron levels in the blood. In some children, maintenance the

III.

Folic Acid Deficiency

BACKGROUND OF THE STUDY - A deficiency of folic acid combined with vitamin C. - Megaloblastic arrest, or inability of RBCs to mature past an early stage, may occur in the first year of life from the continued use of infant food containing too little folic acid or from an infant drinking goat's milk, which tends to be deficient in folic acid. - Treatment is daily oral administration of folic acid. IV. Pernicious Anemia

BACKGROUND OF THE STUDY a) Vitamin B12 (Cobalamin) is necessary for maturation of RBCs. b) Deficiency or inability to use the Vitamin B12, found primarily in foods of animal origin, including both cow's milk and breast milk. c) An adolescent may be deficient in vitamin B12 if he or she is on a longterm, poorly formulated vegetarian diet. d) For absorption of vitamin B12 from the intestine, an intrinsic factor must be present in the gastric mucosa.

ASSESSMENT - The child appears pale, anorexic, and irritable, with chronic diarrhea. - The tongue appears smooth and beefy red due to papillary atrophy. - In children, neuropathologic findings such as ataxia, hyporeflexia, paresthesia and a positive Babinski reflex are less noticeable than in adults.

MANAGEMENT - If the anemia is caused by a B12-deficient diet, temporary injections of B12 will reverse the symptoms. - If the anemia is caused by lack of the intrinsic factor, lifelong monthly intramuscular injections of B12 may be necessary. -

V.

Sickle Cell Anemia

BACKGROUND OF THE STUDY The presence of abnormally shaped (elongated) RBCs. An autosomal recessive inherited disorder on the beta chain of hemoglobin; the amino acid valine takes the place of the normally appearing glutamic acid.

1. Sickle-cell crisis - term used to denote a sudden, severe onset of sickling. - Occurs when a child has an illness causing dehydration or a respiratory infection that results in lowered oxygen exchange and a lowered arterial oxygen level, or after extremely strenuous exercise (enough to lead to tissue hypoxia). - Laboratory reports reveal a hemoglobin level of only 6 to 8 g/100 mL. Bilirubin and reticulocyte levels are increased. 2. Sequestration Crisis ± may occur when there is splenic sequestration of RBCs or severe anemia occurs due to pooling and increased destruction of sickled cells. 3. Hyperhemolytic Crisis ± occurs when there is increased destruction of RBCs. 4. Aplastic Crisis ± manifested by severe anemia due to a sudden decrease in RBC production. ASSESSMENT - Hemoglobin electrophoresis is used to diagnose sickle-cell anemia at birth. - Stasis of blood and infarction may occur in any body part, leading to local disease. - Some infants have swelling of the hands and feet (a hand±foot syndrome) probably caused by aseptic infarction of the bones of the hands and feet. - A chest syndrome with symptoms similar to pneumonia may occur. - Liver may become enlarged from stasis of blood flow. - Kidneys may have subsequent scarring and kidney function may be decreased. - Sclerae are generally icteric (yellowed) from release of bilirubin from destruction of the sickled cells.

MANAGEMENT Pain relief Acetaminophen (Tylenol) may be adequate pain relief for some children; for others, a narcotic analgesic such as intravenous morphine may be needed. 2. Hydration - intensive intravenous fluid replacement therapy. Tissue hypoxia leads to acidosis. The acidosis must be corrected by electrolyte replacement. 3. Prevention - Blood transfusion (usually packed RBCs) may be necessary to maintain the hemoglobin above 12 g/dL (termed hypertransfusion). Hydroxyurea, an antineoplastic agent that has the potential to increase the production of hemoglobin F (fetal hemoglobin), can be used in children with sickle-cell disease to increase their overall hemoglobin level. Stem cell transplantation is possible for the child who does not respond to usual therapies. 1.

VI.

Thalassemia

BACKGROUND OF THE STUDY Autosomal recessive associated with abnormalities of the beta chain of adult hemoglobin (HgbA). It occurs most frequently in the Mediterranean population, they also occur in children of African and Asian heritage.

1. Thalassemia Minor (Heterozygous Beta-Thalassemia) - It is a mild form of this anemia that produces both defective beta hemoglobin and normal hemoglobin. Because there is some normal production, the RBC count will be normal, but the hemoglobin concentration will be decreased 2 to 3 g/100 mL below normal levels.

The blood cells are moderately hypochromic and microcytic because of the poor hemoglobin formation. - Children may have no symptoms other than pallor. - They require no treatment, and life expectancy is normal. They should not receive a routine iron supplement because their inability to incorporate it well into hemoglobin may cause them to accumulate too much iron. 2. Thalassemia Major (Homozygous BetaThalassemia) - Also called Cooley¶s anemia or Mediterranean anemia. - Unable to produce normal beta hemoglobin. - Child shows symptoms of anemia: pallor, irritability, and anorexia ASSESSMENT - Bone pain - As bone marrow becomes hyperactive, this results in a characteristic change in the shape of the skull - The base of the nose may be broad and flattened; the eyes may be slanted with an epicanthal fold, as in Down syndrome.

MANAGEMENT Digitalis, diuretics, and a low-sodium diet Transfusion of packed RBCs every 2 to 4 weeks

VII.

Polycythemia Vera y y y y y An increase in the number of rbcs Occurs as a compensatory response to insufficient oxygenation of the blood in order to help supply more oxygen to body cells. Plethora (marked reddened appearance of the skin) occurs because of the increase in total RBC volume. The RBC count may be as high as 7 million/mm3. Hemoglobin levels may be as high as 23 g/100 ml. Exchange transfusion to reduce the RBC count may be necessary.

Disorders of the White Blood Cells

Neutropenia -is the reduced number of white blood cells. -neutrophils make-up 50-70% of circulating white blood cells and serve as the primary defense against infections by destroying bacteria in the blood. -as nursing management, the nurse must maintain aseptic technique when interacting with the patient to prevent transmission of organisms and protect the client from infection. (e.g. proper hand washing) -Causes: maybe response therapy with some drugs: 6-mercaptopurine or nitrogen mustard : Possible side effect of Phenytoin sodium (DILANTIN), Chloramphenicol or Chloropromazine. -Treatments: would be WBC transfusion and Prophylactic Antibiotics. -Neutropenia is identified thru complete blood count. Neutrophilia -Increased number of white blood cells (total number increase, immature cells > mature neutrophils) ³Left Shift´ -Usually in response to infection or inflammation -Treatment: Antibiotic therapy to eliminate infectious organisms. Eosinophilia -Increased number of eosinophils -allergic or atopic diseases are the most common causes, especially those of the respiratory or integumentary systems. -A sign of a disorder -Treatment: is directed to underlying cause

-Prednisone can also be taken. Facial Granuloma w/ Eosinophilia

Lymphocytosis -Increased number of lymphocytes -In adults, absolute lymphocytosis is present when the absolute lymphocyte count is greater than 4000 per microliter, in older children greater than 7000 per microliter and in infants greater than 9000 per microliter[2 -Normally occurs in pre-school period, when there is a marked predominance of lymphocytes in relation to neutrophils -Elevated in childhood illnesses: pertussis, infectious mononucleosis and lymphocytic leukemia -Treatment is directed to underlying condition. -Lymphocytosis is identified thru Complete Blood Count.

Leukemia -is the uncontrolled malignant proliferation of white blood cells within a an unknown cause affecting all blood. -this is most common in children. Types: Acute Lymphocytic (Lymphoblastic) Leukemia is characterized by a rapid increase in the numbers of immature blood cells. Chronic leukemia is characterized by the excessive build up of relatively mature, but still abnormal, white blood cells. Typically taking months or years to progress, the cells are produced at a much higher rate than normal cells, resulting in many abnormal white blood cells in the blood. -Treatment:

A ntibiotic B one marrow aspiration/ Blood tansfusion C hemotheraphy -Nursing Care: Check early signs of infection Use good hand washing technique (universal precaution) Provide nutritional intake ( increase calorie, protein, iron) Protect from injury Promote rest and comfort Provide realistic feedback on child¶s appearance Alopecia r/t chemotherapy Bone marrow aspiration

Disorders of Blood Coagulation in Children

Platelets are very necessary for blood coagulation, so disorders that limit the number of platelets limit the effectiveness of this process. A normal platelet value is 150,000/mm . Thrombocytopenia [decreased platelet count] is defined as a platelet count of less than 40,000/mm . Thrombocytopenia often leads to purpura or blood seeping from blood vessels into the skin.

1. Purpura y Hemorrhagic rash or small hemorrhages in superficial layer of skin Has 2 types:

a. Idiopathic Thrombocytopenic Purpura {ITP} y Decrease number of circulating platelets with presence of adequate megakaryocyte Cause is unknown y y Result = increase rate of platelet destruction Occurs 2 weeks after viral infection {rubella, rubeola, varicella, URTI}

y

Assessment: y y Miniature petichiae Ecchymosis ± Legs y y Epistaxis Bleeding into joints

Lab studies y y Thrombocytopenia Less than 20,000/mm³ with normal number of megakaryocytes

Management: y y y Oral prednisone Intravenous immunoglobulin {IVIG} Platelet transfusion y y Acetaminophen administration Vaccinated against viral diseases of childhood

b. Henoch-Schönlein Syndrome y y y y Anaphylactoid purpura Hypersensitivity reaction to an allergen Caused by increase vessel permeability Occurs between 2-8 years old y y y More frequently on boys Mild infection before outbreak of symptoms Runs for 4-6 weeks

Assessment: y Purpural rush {buttocks, posterior thighs and extensor surface of the arms and legs, tips of the ear} Joints are tender and swollen y y y Abdominal pain Vomiting Blood in stools

y

Lab studies y y Normal platelet count Sedimentation rate, WBC, eosinophil are elevated

Management y y y Oral costicosteroid therapy Mild analgesics Assessment of urine of protein and glucose presence

Nursing Diagnoses: y y Health seeking behaviors related to injury-prevention measures Risk for compromised family coping related to diagnosis of child¶s illness

2. Disseminated Intravascular Coagulation {DIC} y Acquired disorder of blood clotting

Assessment y Uncontrolled bleeding y Ecchymoses and petichiae Lab studies y y Thrombocytopenia Large platelets on blood smear y Toes and fingers are pale, cyanotic, mottled and feel cold

y y y

Prolong prothrombin and partial thromboplastin times Low serum fibrinogen levels Elevated fibrin split products

Management y y IV heparin administration Fresh plasma, platelets, fibrinogen infusion

Nursing Diagnosis: y Deficient knowledge about blood clotting disorder related to its paradoxical nature

y y

Prognosis: Prognosis varies depending on the underlying disorder, and the extent of the intravascular thrombosis (clotting). The prognosis for those with DIC, regardless of cause, is often grim: Between 10% and 50% of patients will die. DIC with sepsis (infection) has a significantly higher rate of death than DIC associated with trauma.

3. Hemophilia y Inherited disorder of blood coagulation

a. Hemophilia A {Factor VIII Deficiency} y Caused by factor VIII deficiency {antihemophilic factor} Transmitted as a sex linked recessive trait

y

Assessment y y Epistaxis is common Severe bleeding may also occur into the gastrointestinal tract, peritoneal cavity or central nervous system Become active {crawl, climb, walk} Lower extremities Become heavily bruised Soft tissue bleeding Painful hemorrhage into joints Swollen and warm First time to Recognize the disease Damage to Synovial membrane Severe loss of joint mobility

Infant

Undergoes circumcision

Bleeds excessively after circumcision

Lab studies y y Platelet count and prothrombin time are normal Thromboplastin generation test is abnormal

Management y y Administration of Factor VIII {fresh whole blood, fresh/frozen plasma} Administration of Desmopressin {DDAVP}

*child must be identified as having hemophilia before undergoing surgery

Nursing Diagnoses: y y Pain related to joint infiltration by blood Risk for interrupted family processes related to fears regarding child¶s prognosis and long term nature of illness

b. Von Willebrand¶s Disease y y y referred as angiohemophilia factor VIII deficiency, inability of platelets to aggregate inherited autosomal dominant disorder y y y affect both sexes blood vessels cannot constrict bleeding time is prolonged

Assessment y Epistaxis y Unusual heavy menstrual flow Management y y Factor VIII replenishment Administration of DDAVP

c. Christmas Disease {Hemophilia B, Factor IX Deficiency} y y cause by factor IX deficiency 15% of people having hemophilia have this form

Management y Concentrate of Factor IX

d. Hemophilia C {Factor XI Deficiency} y y y y plasma thromboplastin antecedent deficiency caused by factor XI deficiency occurs in both sexes symptoms are generally mild compared to Factor VIII and IX deficiency

Management

y y

Administration of DDAVP Transfusion of fresh blood or plasma

Prognosis:

y y y

Near normal lifestyle with treatment, but with need to avoid injury. Advances in treatment over the last three decades have permitted a nearnormal lifestyle and life-span for many individuals with hemophilia. Deaths from Hemophilia: 1,681 deaths for coagulation defects (NHLBI 1999)

Estimated mortality rate for Hemophilia from prevalence and deaths statistics:
y

Deaths: 1,681 (USA annual deaths calculated from this data: 1,681 deaths for coagulation defects (NHLBI 1999)) Incidence: 20,000 (USA prevalence calculated from this data: 20,000 people in the United States (NHLBI)

y

8.4% (ratio of deaths to prevalence).

I.

UPPER RESPIRATORY DISORDER

A. Acute Nasopharyngitis Description/ Background Study The common cold (also known as nasopharyngitis, acute viral rhinopharyngitis, acute coryza, or a cold) is a viral infectious disease of the upper respiratory system, caused primarily viruses. Common by rhinoviruses and corona symptoms include a cough, throat, runny, and fever. There is currently no known treatment that shortens the duration; however, symptoms usually resolve spontaneously in 7 to 10 days, with some symptoms possibly lasting for up to three weeks. The common cold is the most frequent infectious disease in humans with the average adult contracting two to four infections a year and the average child contracting between 6±12. Collectively, colds, influenza, and other infections with similar symptoms are included in the diagnosis of influenza-like illness. They may also be termed upper respiratory tract infections (URTI). Etiology More than 200 viruses are known to cause the symptoms of the common cold.
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Rhinoviruses: Coronaviruses: Enteroviruses, Other viruses: Adenoviruses, orthomyxoviruses (including influenza A and B viruses), paramyxoviruses (eg, PIV), RSV, EBV, and hMPV account for many URIs. Varicella, rubella, and rubeola infections may manifest as a nasopharyngitis before other classic signs and symptoms develop. The remainder of URI pathogens are not identified but are presumed to be viral. This group represents greater than 30% of common colds in adults.

Patophysiology The common cold virus is transmitted mainly from contact with saliva or nasal secretions of an infected person, either directly, when a healthy person breathes in the virus-laden aerosol generated when an infected person coughs or sneezes, or by touching a contaminated surface and then touching the nose or eyes.

Symptoms are not necessary for viral shedding or transmission, as a percentage of asymptomatic subjects exhibit viruses in nasal swabs.It is generally not possible to identify the virus type through symptoms, although influenza can be distinguished by its sudden onset, fever, and cough The major entry point for the virus is normally the nose, but can also be the eyes (in this case drainage into the nasopharynx would occur through the nasolacrimal duct). From there, it is transported to the back of the nose and the adenoid area. The virus then attaches to a receptor, ICAM-1, which is located on the surface of cells of the lining of the nasopharynx. The receptor fits into a docking port on the surface of the virus. Large amounts of virus receptor are present on cells of the adenoid. After attachment to the receptor, virus is taken into the cell, where it starts an infection.[7] Rhinovirus colds do not generally cause damage to the nasal epithelium. Macrophages trigger the production ofcytokines, which in combination with mediators cause the symptoms. Cytokines cause the systemic effects. The mediator bradykinin plays a major role in causing the local symptoms such as sore throat and nasal irritation. The common cold is self-limiting, and the host's immune system effectively deals with the infection. Within a few days, the body's humoralimmune response begins producing specific antibodies that can prevent the virus from infecting cells. Additionally, as part of the cell-mediated immune response, leukocytes destroy the virus through phagocytosis and destroy infected cells to prevent further viral replication. In healthy, immune competent individuals, the common cold resolves in seven days on average. Manifestations Signs and symptoms of acute nasopharyngitis include: y Muscle weakness y Sore throat y Uncontrollable shivering y Runny nose y Loss of appetite y Nasal congestion y low grade fever y Sneezing y cervical lymph nodes may be y Sometimes accompanied by swollen 'pink eye' y edematous and inflamed y Muscle aches mucous membrane y Fatigue y constricting airway space y Malaise causing difficulty of breathing y Headaches
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Laboratory and diagnostic tests No explicit diagnostic test exists to isolate the specific organism responsible for the common cold. Consequently, diagnosis rests on the typically mild,

localized, and afebrile upper respiratory symptoms. Despite infection, white blood cell counts and differential are within normal limits. Diagnosis must rule out allergic rhinitis, measles, rubella, and other disorders that produce similar early symptoms. A temperature higher than 100° F (37.8° C), severe malaise, anorexia, tachycardia, exudate on the tonsils or throat, petechiae, and tender lymph glands may point to more serious disorders and require additional diagnostic tests.
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Cold & Flu: Home Testing: o Home Fever Tests o Home Ear Infection Test Kits o Home Flu Tests o Home Strep A Tests

Treatment Cough relief Cough suppression may increase comfort when cough is severe or when it prevents sleep. The following agents may reduce cough in the setting of a URI.26 The risk-to-benefit ratio for using cough and cold medicines in children younger than 2 years requires careful consideration because serious adverse events, including fatalities, have been reported with the use over-the-counter preparations in young children.29 Since 2008, many nonprescription cough and cold product labels state "do not use" in children younger than 4 years.
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Antihistamine and decongestants: Cough associated with the common cold may be treated with a first-generation antihistamine combined with a decongestant (eg, brompheniramine with pseudoephedrine). Inhaled ipratropium: An anticholinergic, inhaled ipratropium may be useful in postinfectious cough (3-8 wk after the onset of the URI) in adults. Inhaled steroids: These agents may be considered in postinfectious cough (3-8 wks after URI onset), Dextromethorphan: This is a centrally acting cough suppressant and it may be considered for the treatment of postinfectious cough in adults if other medications fail. Additional data are required to permit evidencebased recommendations for the use of central-acting antitussives in URIrelated cough in children. Peripherally acting cough suppressants: While these medications may have a role in bronchitis, they may have limited efficacy in relieving cough associated with URI.

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Guaifenesin: As an expectorant, guaifenesin is intended to mobilize secretions. However, consistent data regarding its effectiveness in reducing discomfort from cough associated with URIs are scarce. Long-acting inhaled beta-agonists: Beta-agonists are not thought to be helpful in URI-related cough, including that due to pertussis. Nonsteroidal anti-inflammatory drugs: They may be used to reduce discomfort due to cough. Avoid aspirin in children with viral illness because aspirin is associated with Reye syndrome.

Nursing Management There are currently no medications or herbal remedies which have been conclusively demonstrated to shorten the duration of illness. Treatment comprises symptomatic support usually via analgesics for fever, headache, sore muscles, and sore throat. Symptomatic Treatments that help alleviate symptoms include simple analgesics and antipyretics such as ibuprofen and acetaminophen /paracetamol. Evidence does not show that cough medicine is any more effective than simple analgesics and is not recommended for use in children due to a lack of evidence supporting its effectiveness and the potential for harm. Symptoms of a runny nose can be reduced by a first generation antihistamine, however it can cause drowsiness and other side effects. Anticholinergics such as Ipratropium nasal spray can reduce the symptoms of runny nose with less side effects, however it is a prescription drug. One study has found chest vapor rub to be effective at providing some symptomatic relief of nocturnal cough, congestion, and sleep difficulty. Getting plenty of rest, drinking fluids to maintain hydration, and gargling with warm salt water, are reasonable conservative measures. Evidence for encouraging the active intake of fluids in acute respiratory infections is lacking, as is the use of heated humidified air. Saline nasal drops may help alleviate nasal congestion. Prognosis
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A common cold may last up to 14 days, with symptoms averaging 7-11 days in duration. Fever, sneezing, and sore throat typically resolve early, whereas cough and nasal discharge are among the symptoms that last longest.

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Attendance at daycare may affect the duration of symptoms in young children. In one study, the duration of viral URI ranged from 6.6 days for 1to 2-year-old children in home care to 8.9 days for children younger than 1 year who were in daycare. Young children in daycare were more likely to have protracted respiratory symptoms lasting more than 15 days. Most patients with influenza recover within a week, although cough, fatigue, and malaise may persist for up to 2 weeks. For newborns, elderly persons, and patients with chronic medical conditions, the flu may be life threatening. More than 200,000 people are hospitalized because of complications of the flu, with 0.36 deaths occurring per 100,000 patients each year. The common cold is generally mild and self-limiting

B. Tonsillitis Background Study Tonsillitis is an inflammation of the tonsils, the fleshy clusters of tissue on both sides of the back of the throat that fight off germs that enter the body through the mouth. The tonsils become enlarged and red, and have a yellow or white coating. Most types of tonsillitis are contagious, spreading from person to person by contact with the throat or nasal fluids of someone who is infected. Tonsillitis symptoms include a sore throat, fever, swollen glands in the neck, and trouble swallowing. Etiology/ Patophysiology The most common causes of tonsillitis are the common cold viruses (adenovirus, rhinovirus, influenza, coronavirus, respiratory syncytial virus). It can also be caused by Epstein-Barr virus, herpes simplex virus, cytomegalovirus, or HIV. The second most common causes are bacterial. The most common bacterial cause is Group A -hemolytic streptococcus (GABHS), which causes strep throat. Less common bacterial causes include: Staphylococcus aureus, Streptococcus pneumoniae, Mycoplasma pneumoniae,Chlamydia pneumoniae, pertussis, Fusobacterium, diphtheria, syphilis, and gonorrhea. Under normal circumstances, as viruses and bacteria enter the body through the nose and mouth, they are filtered in the tonsils. Within the

tonsils, white blood cells of the immune system mount an attack that helps destroy the viruses or bacteria, and also causes inflammation and fever. The infection may also be present in the throat and surrounding areas, causing inflammation of the pharynx. This is the area in the back of the throat that lies between the voice box and the tonsils. Tonsillitis may be caused by Group A streptococcal bacteria, resulting in strep throat. Viral tonsillitis may be caused by numerous viruses] such as the Epstein-Barr virus (the cause of infectious mononucleosis) or adenovirus. Sometimes, tonsillitis is caused by an infection of spirochaeta and treponema, in this case called Vincent's angina or Plaut-Vincent angina. Manifestations Tonsillitis Symptoms
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Sore throat Difficulty feeding (in babies) Pain with swallowing Fever Headache Abdominal pain Nausea and vomiting Cough Hoarseness Runny nose Redness of the tonsils and throat Tenderness in the glands of the neck (swollen lymph glands) White patches on the tonsils Redness of the eyes Rash Ear pain (nerves that go to the back of the throat also go to the ear) Laboratory and diagnostic tests The health care provider will look in the mouth and throat for swollen tonsils. The tonsils are usually red and may have white spots on them. The lymph nodes in the jaw and neck may be swollen and tender to the touch. Tests that may be done include: y Blood count y Mononucleosis test

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Rapid strep test Throat swab culture

Treatment If bacteria such as strep are causing the tonsillitis, antibiotics are given to cure the infection. The antibiotics may be given once as a shot, or taken for 10 days by mouth. If antibiotic pills are used, they must be taken for the entire amount of time prescribed by the doctor. DO NOT stop taking them just because the discomfort stops, or the infection may not be cured. Other treatments include: y Drink cold liquids or suck on popsicles y Drink fluids, especially warm (not hot), bland fluids y Gargle with warm salt water y Suck on lozenges (containing benzocaine or similar ingredients) to reduce pain (these should not be used in young children because of the choking risk) y Take over-the-counter medications, such as acetaminophen (Tylenol) reduce pain and fever. Do NOT give a or ibuprofento child aspirin. Aspirin has been linked toReye syndrome. Some people who have repeated infections may need surgery to remove the tonsils (tonsillectomy). Complications of untreated strep tonsillitis may be severe. Children with tonsillitis related to strep throat or pharyngitis should generally be kept home from school or day care until they have been on antibiotics for 24 hours. This helps reduce the spread of illness. Complications y Blocked airway from swollen tonsils y Dehydration from difficulty swallowing fluids y Kidney failure y Peritonsillar abscess or abscess in other parts of the throat y Pharyngitis - bacterial y Post-streptococcal glomerulonephritis y Rheumatic fever and related cardiovascular disorders

Nursing Management Kids with tonsillitis need plenty of nourishment and rest. If swallowing so painful that eating is difficult, try serving liquids and soft foods, like soups, milkshakes, smoothies, ice pops, or ice cream. Make sure that your child drinks lots of fluids and gets plenty of rest, and take his or her temperature regularly. Use a nonprescription pain reliever, such as acetaminophen or ibuprofen, for throat pain. Don't give aspirin or other products that contain aspirin, though, because these can put kids at risk for Reye syndrome, an illness that can have serious complications. Keep your sick child's drinking glasses and eating utensils separate, and wash them in hot, soapy water. All family members should wash their hands frequently. If your child starts antibiotic therapy for strep, throw out his or her toothbrush and replace it with a new one. Management Tonsillectomy is surgery to remove the tonsils. These glands are at the back of your throat. Often, tonsillectomy is done at the same time as adenoidectomy, surgery to remove the adenoid glands. Description Your child will be given general anesthesia before surgery. They will be asleep and pain free. y The surgeon will insert a small tool into your child¶s mouth to prop it open. y The surgeon then cuts or burns away the tonsils. The doctor will control bleeding, and the cuts heal naturally without stitches. Your child will stay in the recovery room after surgery until they are awake and can breathe easily, cough, and swallow. Most children go home several hours after this surgery. Preoperative Careful history taking is needed to evaluate for the following:
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Bleeding disorders or wish to avoid transfusion Anesthesia intolerance Obstructive sleep apnea

Regarding admission planning, insurance plans are increasingly disallowing inpatient admission for tonsillectomy or adenoidectomy. Children who should be admitted are those with obstructive sleep apnea, those with significant comorbid disease such as hypotonia or neuromotor delays, and those younger than 3 years. Postoperative A sore throat will persist for around two weeks after the operation. Most patients do not feel like swallowing anything during the first few days after surgery. Patients should try to get as much fluid down as possible, as it will help speed recovery. Very cold drinks will help bring down swelling. Ice cream, frozen yogurt and other dairy products are not recommended because they leave a film in the mouth that is difficult to swallow. Sorbet and popsicles, on the other hand, are recommended. Additionally, Slushies are particularly helpful for sore throats, especially when sugar-free. Pain following the procedure is significant and may include a hospital stay.[7] Recovery can take from 10 up to 20 days, during which narcotic analgesics are typically prescribed. Patients are encouraged to maintain diet of liquid and very soft foods for several days following surgery. Rough textured, acidic or spicy foods may be irritating and should be avoided. Proper hydration is very important during this time, since dehydration can increase throat pain, leading to a vicious circle of poor fluid intake. At some point, most commonly 7±11 days after the surgery (but occasionally as long as two weeks (14 days) after), bleeding can occur when scabs begin sloughing off from the surgical sites. The overall risk of bleeding is approximately 1%±2% higher in adults. Approximately 3% of adult patients develop significant bleeding at this time. The bleeding might naturally stop quickly or else mild intervention (e.g., gargling cold water) could be needed (but ask the doctor before gargling because it might bruise the area of the skin that has been cauterized). Otherwise, a surgeon must repair the bleeding immediately by cauterization, which presents all the risks associated with emergency surgery (primarily the administration of anesthesiaparticularly on a patient whose stomach may not be empty). Generally speaking, tonsils will be removed if a patient needs antibiotics to be prescribed six times a year for tonsilitis, and the general practitioner's recommendation is based on how the quality of life will be

improved after the operation. Tonsillectomies can be performed while the patient is actually suffering from tonsillitis, however this increases the risk of bleeding

Prognosis Tonsillitis usually gets better on its own within a few days. Treating the symptoms of sore throat and fever will make the patient more comfortable. In cases where the fever lasts for more than forty-eight hours or reaches a temperature of more than 102°F (38°C) the patient should be seen by a doctor. Any medication that has been prescribed should be taken until all of it has been taken. Patients sometimes stop taking their medications when they feel better, but though the symptoms may have cleared up, the infection may not have been cured. The infection may spread to other parts of the upper respiratory (breathing) system. The ears and sinuses are especially subject to such infection. In rare cases, much more serious conditions, such as rheumatic fever (see rheumatic fever entry) or pneumonia (see pneumonia entry) may develop. C. Pharyngitis Background Study Pharyngitis is very common but rarely serious. Most cases clear up on their own after three to ten days and require no therapy other than pain relievers to ease the discomfort. Rarely, though, tissues may swell considerably and obstruct breathing - a life-threatening condition. In addition, strep throat (caused by streptococcal bacteria) requires antibiotics to prevent complications, including rheumatic fever, a condition that can permanently damage the heart valves. Diphtheria is a rare but serious bacterial variety of pharyngitis. Pharyngitis appears in three forms - nonexudative, exudative, and ulcerative: Nonexudative - although group A streptococci may cause nonexudative pharyngitis, viruses are by far the most common causative agents of this group. Exudative - group A streptococcus is the most common bacterial cause of exudative and nonexudative pharyngitis. Beta-hemolytic streptococci in groups C and G have also been associated with exudative pharyngitis and tonsillitis. Ulcerative - coxsackievirus A and herpes virus are the most common cause of ulcerative pharyngitis. Vincent's angina due to fusobacteria and poor oral hygiene

may also cause ulcerative pharyngitis that is associated with malaise and lowgrade fever. The most common finding is a unilateral tonsillar ulceration with a gray necrotic membrane.

Etiology/ Patophysiology This is most often viral in origin. Importantly, group A streptococcal pharyngitis must be recognized because serious complications may follow untreated disease.
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Causes of viral pharyngitis o Adenovirus, which may also cause laryngitis and conjunctivitis o Influenza viruses o Coxsackievirus o HSV o EBV (infectious mononucleosis) o Cytomegalovirus Causes of bacterial pharyngitis o Group A streptococci (approximately 15% of all cases of pharyngitis) o Group C and G streptococci o N gonorrhoeae o Arcanobacterium (Corynebacterium) hemolyticum o Corynebacterium diphtheriae o Atypical bacteria (eg, M pneumoniae, C pneumoniae): Absent lower respiratory tract disease, the clinical significance of these pathogens is uncertain. o Anaerobic bacteria

Manifestations Sore throat Red throat Lump in throat feeling Fever Headache Swollen glands Swollen neck lymph glands Tender neck lymph glands

Difficulty swallowing Pain swallowing Breathing difficulty Laboratory and diagnostic tests Your health care provider will perform a physical exam and look at your throat. A rapid test or throat culture to rule out strep throat may be done. Additional laboratory tests may be done depending on the suspected cause.
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GABHS rapid antigen detection test Rapid antigen detection test for group A beta-hemolytic streptococci. This is the preferred method for diagnosing GAS infection in the emergency department because of difficulties with culture followup. o Only patients with a high clinical likelihood of GAS pharyngitis should be tested. Patients with a Centor score of 0-1 should be treated symptomatically without testing. o Antigens are specific, but sensitivities vary. Children with a negative antigen test should have a follow-up culture unless the antigen being used in the office has been shown to be as sensitive as a culture.9 o The use of a GABHS rapid antigen detection test can decrease the use of unnecessary antibiotics in pediatric patients when used properly. o Adults do not need follow-up culture after a negative antigen test because of the low incidence of GAS in this population. Throat culture o This is the criterion standard for diagnosis of GAS infection (9099% sensitive). Although less expensive than the rapid antigen detection test, it is not be the best test to use in the emergency department because of difficulty with follow-up. The guidelines that recommend cultures for GAS screening are aimed at office-based practices and not the emergency department. o Patients can be treated up to 9 days after onset of symptoms to prevent acute rheumatic fever, so immediate antibiotic therapy is not crucial if patients can be easily contacted for follow-up should a culture become positive. Mono spot is up to 95% sensitive in children (less than 60% sensitivity in infants).
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Peripheral smear may show atypical lymphocytes in infectious mononucleosis.2 y Perform gonococcal culture, as indicated by history. y A complete blood count (CBC), erythrocyte sedimentation rate (ESR), and C-reactive protein have a low predictive value and usually are not indicated. Imaging Studies
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Imaging studies generally are not indicated for uncomplicated viral or streptococcal pharyngitis. Lateral neck film should be taken in patients with suspected epiglottitis or airway compromise. Soft tissue neck CT can be used if concern for abscess or deep-space infection exists; however, peritonsillar abscess is almost always a clinical diagnosis. Imaging is rarely needed for diagnosis.

Procedures
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The procedure for a throat swab is to vigorously rub a dry swab over the posterior pharynx and both tonsils, obtaining a sample of exudate. If any exudate is obtained, then transport it dry (not in a liquid medium).

Treatment If your doctor suspects that you have a sore throat caused by bacteria, he or she will prescribe an antibiotic. But if your sore throat is caused by a virus, there is no medicine that will cure it -- it will go away on its own. Cool air and humidity are suggested to relieve symptoms. In the meantime, your doctor may recommend gargling with salt water and taking an over the counter pain reliever such as acetaminophen (Tylenol) or ibuprofen (Advil, Motrin). Children under 18 should not take aspirin as a pain reliever, because of the risk of a rare but serious illness called Reye's syndrome. Lifestyle
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Rest Drink lots of fluid. Water and warm broths are better than soft drinks Avoid drinking alcohol Gargle several times per day with ½ tsp. of salt in a glass of warm water Try throat lozenges (do not give to a child under 3 years old due to choking hazard).

Medications If your sore throat is caused by a bacterial infection, your doctor will prescribe an antibiotic. Penicillin or, if you have an allergy to penicillin, erythromycin are most commonly prescribed. In children with early colds, begin acetaminophen or ibuprofen in an appropriate dose on a regular schedule (every 4 hours) during the waking hours. Other symptomatic treatments may be used if they appear to be helpful. These include: 
 

Topical and systemic decongestants (neo-synephrine, pseudoephedrine); decongestants may cause excitability. Antihistamine (especially at bedtime). Antihistamine may cause drowsiness. Mucoevacuants (guafenesin)

Children should never be given aspirin because of the danger of it causing brain damage. (Reyes syndrome) Antibiotics do not shorten a cold, reduce the severity of the illness, or prevent secondary bacterial complications. Consider consulting your doctor : 
 

If the child develops a "second fever" later in the course of the cold. If the nasal symptoms and cough are no better or worsen after 10-14 days. If the child complains of ear pain (or the young child pulls at the ear).

Prognosis
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Viral pharyngitis typically resolves in 1-2 weeks. In patients with penicillin-sensitive streptococcal pharyngitis, symptomatic improvement is expected within 24-72 hours after the start of treatment. The clinician should be aware of potential complications. Treatment failures are common and mainly attributed to poor adherence, antibiotic resistance, and untreated close contacts. A chronic carrier state may develop with group A streptococcal infection. Eradicating the pathogen is difficult in these cases; however, carriers without active symptoms are unlikely to spread group A streptococci, and they are at low risk for

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developing rheumatic fever. The risk of mortality is significant in patients who progress to streptococcal toxic shock syndrome, which is characterized by multiorgan failure and hypotension. With infectious mononucleosis from EBV, complete resolution of symptoms may take up to 2 months. Acute symptoms rarely last more than 4 months. EBV typically remains dormant throughout the patient's life. Reactivation of the virus is not usually symptomatic.

D. Retropharyngeal abscess Background Study Retropharyngeal abscesses are deep neck space infections that can pose an immediate life-threatening emergency, with potential for airway compromise and other catastrophic complications. The retropharyngeal space is located immediately posterior to the pharynx (nasopharynx, oropharynx, hypopharynx), larynx, and trachea. The visceral (buccopharyngeal) fascia, which surrounds the pharynx, trachea, esophagus, and thyroid, forms the anterior border of the retropharyngeal space. Bounded posteriorly by the alar fascia, the retropharyngeal space is bounded laterally by the carotid sheaths and parapharyngeal spaces. It extends superiorly to the base of the skull and inferiorly to the mediastinum at the level of the tracheal bifurcation.

Etiology/Patophysiology The retropharyngeal space can become infected in two ways. Infection can either spread from a contiguous area or the space can be directly inoculated from penetrating trauma. The "classic" retropharyngeal abscess observed in pediatric patients occurs when an upper respiratory infection (URI) spreads to retropharyngeal lymph nodes, forming chains in the retropharyngeal space on either side of the superior constrictor muscle. Sources of infection can include pharyngitis, tonsillitis, adenoiditis, adenitis, otitis, sinusitis, and other infections (ie, nasal, salivary, dental). Degeneration/suppuration of these nodes leads to abscess formation. Infectious

sources (eg, osteomyelitis of the spine) also can spread directly anteriorly from the prevertebral space. Of specific interest is the group of lateral retropharyngeal nodes at the base of the skull that bear the name of French anatomist Henri Rouviere. These Rouviere nodes are typically not of great clinical interest, but as the primary lymphatic drainage of the nasopharynx, they can become significant in cases of nasopharyngeal cancer. They are also pertinent to the discussion of retropharyngeal abscess, as they can suppurate and lead to a retropharyngeal abscess. Penetrating trauma can also be involved in retropharyngeal space infection. Accidental lacerations are not uncommon in children who run and fall down after they have placed a sharp object in their mouths. Foreign bodies (for example, fishbones) have been implicated in penetrating trauma to the retropharyngeal space. Iatrogenic causes of inoculation to this space include instrumentation with laryngoscopy, endotracheal intubation, surgery, endoscopy, feeding tube placement, and dental injections and procedures. Complications of retropharyngeal abscesses are secondary to mass effect, rupture of the abscess, or spread of infection. The most urgent complication involves the abscess expanding against the pharynx or trachea, causing airway compression. Rupture of the abscess can cause aspiration of pus, resulting in asphyxiation or pneumonia. The infection can spread, resulting in inflammation and destruction of adjacent tissues. Spread of the infection to the mediastinum can result in mediastinitis, purulent pericarditis and tamponade, or bronchial erosion. pyopneumothorax, pleuritis,empyema, Spread of the infection laterally can involve the carotid sheath and cause jugular vein thrombosis or carotid artery rupture. Posterior spread of infection can result in osteomyelitis and erosion of the spinal column, causing vertebral subluxation and spinal cord injury. The infection itself can evolve into necrotizing fasciitis, sepsis, and death. Hence, accurate and prompt treatment and intervention for presumed retropharyngeal abscess is crucial to prevent significant untoward sequelae.

Clinical Findings History Patients with a retropharyngeal abscess present with constitutional complaints such as fever, chills, malaise, decreased appetite, and irritability. y Patients may complain of a sore throat, difficulty swallowing (dysphagia), pain on swallowing (odynophagia), jaw stiffness (trismus), or neck stiffness (torticollis). Small children with torticollis tend to hold their neck in a non-neutral position and do not turn their head from side to side. y Patients may also complain of muffled voice, the sensation of a lump in the throat, and/or pain in the back and shoulders upon swallowing. y Difficulty breathing may be an ominous complaint that portends impending airway obstruction. y Patient history is not always straightforward. Signs and symptoms can include fever (74%), sore throat (47%), dysphagia (38%), trismus (36%), decreased appetite (22%), voice change (18%), odynophagia (17%), neck pain (15%), irritability (11%), and difficulty breathing (8%). y The course of pharyngeal abscess can be insidious. o Sometimes an upper respiratory illness can precede symptoms by weeks. o Many patients do not recall (or parents are not aware of) incidences of penetrating trauma. o Maintain a high index of suspicion, especially in patients with upper respiratory illnesses that do not appear to resolve in a normal course or with conventional therapy. Physical
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Most patients with a retropharyngeal abscess are febrile. Some appear toxic and irritable. Cervical lymphadenopathy, usually unilateral, is the most common physical finding in these patients. Patients may have decreased or painful range of motion of their necks or jaws. A neck mass or tenderness may be appreciated. These patients may present with a muffled "hot potato" voice (dysphonia). Patients in respiratory distress or those who present with stridor or drooling have potential airway compromise and should be immediately triaged as such.

Address vascular complications in the physical examination. o Jugular vein thrombophlebitis may manifest as tender induration at the anterior sternocleidomastoid border, vocal cord paralysis, or sepsis of an unknown source. It may also be asymptomatic. o Carotid artery rupture can be heralded by sentinel bleeding from the ear, nose, or mouth. o Ecchymosis may be detected in the lateral neck. Causes
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Most retropharyngeal space infections are spread from various sources in the upper respiratory tract due to the retropharyngeal lymph nodes. The lymphadenitis can form a cellulitis, which suppurate and become an abscess. o Possible predisposing infections can include pharyngitis, tonsillitis, otitis, adenitis, adenoiditis, sinusitis, and nasal, salivary, and dental infections. o Retropharyngeal infections are also spread from contiguous spaces, such as the parapharyngeal space (eg, abscesses), submandibular space, or prevertebral space (eg, osteomyelitis). The retropharyngeal space can also be directly inoculated secondary to penetrating trauma. o Running and falling down with a sharp object in the mouth is not unusual in children. Because parents may be unaware of these predisposing events, diagnosis is even more elusive. o Foreign bodies (for example, fishbones) can become lodged in the posterior pharynx. Although this can happen in the pediatric age group, a foreign body lodged in the posterior pharynx is also a cause of abscess formation in adults. Deep space infections can be iatrogenic secondary to instrumentation of the upper respiratory tract. All of the following can predispose to abscess formation: o Laryngoscopy o Endoscopy o Esophagoscopy o Feeding tube insertion o Endotracheal intubation o Head and neck surgery o Dental procedures o Injections Risk factors may include low socioeconomic status, poor oral hygiene, immune disfunction (including HIV, diabetes, and immunosuppression)

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Bacteria are often polymicrobial, with gram-positive organisms and anaerobes predominating, but gram-negative bacteria have also been isolated. The source is usually oropharyngeal flora. o The most common cause is group A beta-hemolytic streptococci. Other nonhemolytic streptococci can be present. Staphylococcus aureus is also fairly common. The most common anaerobes areBacteroides species. o Other causative agents include Haemophilus parainfluenzae and Veillonella, Peptostreptococcus, Fusobacterium, and Eikenella species. o The incidence of beta-lactamase production is high. One study noted 22% beta-lactam resistance. o Suspect mycobacterium tuberculosis, B henselae, and coccidiosis in patients who may be predisposed (immunosupression, recent immigrants), especially if they are not responding to more conventional therapy. o Another consideration when evaluating these patients is the possibility of Lemierre syndrome (septic thrombophlebitis of the internal jugular vein from a head and neck infection (eg, retropharyngeal abscess). This infection is classically associated with the Fusobacterium necrophorum, an anaerobic, gram-negative rod

Laboratory/ Diagnostic tests Laboratory Studies
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Complete blood count o The mean white blood cell (WBC) count in one study was 17,000, with a range of 3100-45,900. o WBC counts in 18% of the patients were less than 8000; thus, a normal WBC count does not rule out the diagnosis of retropharyngeal abscess. o In a study in Germany, the mean WBC ( standard deviation was 14,700 [ 10,500]), with a range from 200-114,000. Blood cultures are indicated before administration of intravenous antibiotics, but culture results may be negative in as many as 82% of retropharyngeal abscess cases. A culture of pus, aspirated at the time of surgical drainage of the retropharyngeal abscess, can grow one or more organisms 91% of the time.

C-reactive protein o In one study of adults and children with deep cervical space infections, patients with C-reactive protein level greater than 100 had longer hospital stays. o In a German study, mean ( standard deviation) C-reactive protein level was 15.7 ( 12.9), with a range from 0.0-74. Imaging Studies
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Lateral neck radiography o Widening of the retropharyngeal soft tissues was observed in 88% of patients with retropharyngeal abscess in a series that defined soft tissue swelling as more than 7 mm at C2 and more than 14 mm at C6. Most authors define retropharyngeal soft tissue swelling as more than 7 mm at C2 and more than 22 mm at C6; thus, lateral neck radiographs may be considerably less sensitive for detecting retropharyngeal abscess than this study indicates. o Generally, the anteroposterior diameter of the prevertebral soft tissue space in children should not exceed that of the contiguous vertebral bodies. o In addition to showing widening of the prevertebral space, the lateral neck radiograph rarely may show a gas-fluid level, gas in the tissues, or a foreign body.

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CT scan of the neck o A CT scan of the neck with intravenous contrast is very useful in the diagnosis and management of retropharyngeal abscess. Retropharyngeal abscess appears as a hypodense lesion in the retropharyngeal space with peripheral ring enhancement. Other findings on CT scan include soft-tissue swelling, obliterated fat planes, and mass effect. o Obtain a CT scan of the neck with intravenous contrast when the findings on the lateral neck radiograph are equivocal or if the clinical suspicion for retropharyngeal abscess is high in patients with negative findings on lateral neck radiograph. Lateral neck radiographic findings may be misleading, especially in young children. o A CT scan of the neck with intravenous contrast also may be useful if the radiographic findings are positive because the CT scan can differentiate between retropharyngeal abscess and cellulitis. The

CT scan also shows the extent of the retropharyngeal abscess and its relation to the great vessels, which is very helpful to the surgeon. o CT scan of the neck can also differentiate between retropharyngeal abscess and retropharyngeal lymphadenopathy in children, which may help the ear, nose, and throat (ENT) surgeon decide whether to treat with intravenous antibiotics alone or intravenous antibiotics plus surgical drainage. y A chest radiograph is indicated to look for aspiration pneumonia and mediastinitis. y An MRI with gadolinium enhancement may demonstrate a retropharyngeal abscess, but this modality has not been used widely. y Ultrasonography may demonstrate the presence of a retropharyngeal abscess, but its use has not yet been clarified. Procedures
y

y

y y

Nasopharyngolaryngoscopy o A review of the literature did not reveal a role for nasopharyngolaryngoscopy use in the diagnosis of retropharyngeal abscess. o Safety of this procedure in the setting of retropharyngeal abscess is unclear. o Nasopharyngolaryngoscopy has been performed preoperatively in 2 adults; no reports of its use in children exist. Endotracheal intubation o Securing the airway may be required if the patient with retropharyngeal abscess is exhibiting signs of impending upper airway obstruction. Endotracheal intubation may be attempted, but it may be difficult because of distortion of the upper airway. o Prophylactic intubation for a patient with retropharyngeal abscess but without respiratory distress generally is not indicated unless an interhospital transfer is planned. If a patient with signs of upper airway obstruction cannot be intubated, a surgical or needle cricothyrotomy may be required. A tracheostomy may be required as definitive airway management in patients with retropharyngeal abscess and respiratory distress.

Treatment Prehospital Care
y

y y

Supplemental oxygen and attention to upper airway patency are the essential components of prehospital care in patients with suspected retropharyngeal abscess. If a child exhibits respiratory distress, the sniffing position may be beneficial. Occasionally, endotracheal intubation or cricothyrotomy may be required if the patient exhibits signs of upper airway obstruction.

Emergency Department Care ED management of retropharyngeal abscess includes attention to the airway, fluid resuscitation if necessary, antibiotic treatment, and preparation for an emergency operation. Frequent vital sign checks and continuous oxygen saturation monitoring are essential.
y

y

Airway management o Apply supplemental oxygen. In young children, this can be completed in a nonthreatening way by letting the parent direct blowby oxygen at the child's face. o Endotracheal intubation may be required if the patient has signs of upper airway obstruction. It may be difficult because of upper airway swelling. o Cricothyrotomy (surgical or needle) may be required in the patient with upper airway obstruction who cannot be intubated. Tracheostomy may be required for definitive airway management. Intravenous fluids are required if the patient is dehydrated because of fever and difficulty swallowing.

Medications The goals of pharmacotherapy are to eradicate the infection, to reduce morbidity, and to prevent complications. Intravenous broad-spectrum antibiotic coverage is indicated in the treatment of retropharyngeal abscess. Antibiotics Gram-positive organisms (including beta-lactamase producing), gramnegative organisms, and anaerobes must be covered. penicillin and oxacillin, second- or third-generation cephalosporin and clindamycin, penicillinaseresistant penicillin combined with either clindamycin or metronidazole, or third-

generation cephalosporin in combination with clindamycin, nafcillin, or both (triple therapy).

In a review of retropharyngeal infections in children by Wald, the recommendation was to add vancomycin or linezolid to the regimen in patients not responding to clindamycin, and in patients who present with severe disease, in order to cover MRSA Prognosis It is important to get immediate medical help. This condition can lead to blockage of the airway, which can be life-threatening. With prompt treatment, you can make a full recovery. E. Epistaxis Background Study Epistaxis, or nosebleed, is a common pediatric complaint. Most incidents are rarely life threatening but cause significant parental concern.1 Most nosebleeds are benign, self-limiting, and spontaneous but may also be recurrent. Many uncommon causes are also noted. Epistaxis can be divided into 2 categories, anterior bleeds and posterior bleeds, based on where the bleeding originates. Etiology/ Patophysiology Ninety per cent of epistaxis in children originates from Little¶s area in the anterior part of the nose, often being either idiopathic or the result of trauma. Idiopathic epistaxis forms the most common aetiological category (Table I). Although bleeding may occur spontaneously, it often results from forceful nose blowing and sneezing which increases arterial and venous pressure in the vascularised nasal septum, which usually accompanies allergic rhinitis, viral/bacterial URIs and trauma/sepsis secondary to foreign bodies. Posterior epistaxis is uncommon in children and is usually the result of bleeding disorders, inflammatory disorders or neoplasms. Persistent or recurrent epistaxis should raise the suspicion of bleeding disorders or neoplasms, necessitating further investigation.

Nosebleeds are due to the rupture of a blood vessel within the richly perfused nasal mucosa. Rupture may be spontaneous or initiated by trauma. Nosebleeds are reported in up to 60% of the population with peak incidences in those under the age of ten and over the age of 50 and appear to occur in males more than females. An increase in blood pressure (e.g. due to general hypertension) tends to increase the duration of spontaneous epistaxis. Anticoagulant medication and disorders of blood clotting can promote and prolong bleeding. Spontaneous epistaxis is more common in the elderly as the nasal mucosa (lining) becomes dry and thin and blood pressure tends to be higher. The elderly are also more prone to prolonged nose bleeds as their blood vessels are less able to constrict and control the bleeding. The vast majority of nose bleeds occur in the anterior (front) part of the nose from the nasal septum. This area is richly endowed with blood vessels (Kiesselbach's plexus). This region is also known as Little's area. Bleeding farther back in the nose is known as a posterior bleed and is usually due to rupture of the sphenopalatine artery or one of its branches. Posterior bleeds are often prolonged and difficult to control. They can be associated with bleeding from both nostrils and with a greater flow of blood into the mouth. Laboratory and diagnostic tests For the most part, laboratory studies are not needed for first-time or infrequent recurrences with a good history of nose picking or trauma to the nose. If significant blood loss, leukemia, or malignancy is suspected or if recurrent bleeding occurs, perform a CBC count with differential. y If a coagulopathy is suspected, perform CBC count and obtain prothrombin time (PT)/activated partial thromboplastin time (aPTT) and bleeding time. Direct visualization with a good directed light source, nasal speculum (see image below), and nasal suction should be sufficient in most patients
y

Treatment Medical Care Initial treatment begins with direct pressure by squeezing the nostrils together for 5-30 minutes straight, without frequent peeking to see if the bleeding is controlled. Usually only 5-10 minutes is required.

Patients should keep their heads elevated but not hyperextended because hyperextension may cause bleeding into the pharynx and possible aspiration. This maneuver works more than 90% of the time. If bleeding is caused by excessive dryness in the home (eg, from radiator heating), patients may benefit from the following care options:
y y y

y

y

Humidify the air with a cool mist vaporizer in the bedroom. Alternately, a metal basin of water may be placed on top of a radiator to humidify the ambient air. Nasal saline sprays are useful. Oxymetazoline (Afrin) may also be used, with fewer cardiac adverse effects. These agents should only be used for 3-5 days at a time to avoid rhinitis medicamentosa and tachyphylaxis. The physician may consider local application of bacitracin or petrolatum ointment directly to the Kiesselbach area with a cotton applicator to prevent further drying (studies recommend 2 wk). If direct pressure is not sufficient, gauze moistened with epinephrine at a ratio of 1:10,000 or phenylephrine (Neo-Synephrine) may be placed in the affected nostril to help vasoconstrict and achieve hemostasis.

Diet While bleeding is occurring and the assessment is in process, the child should remain nothing by mouth (NPO). Once bleeding is controlled, a full diet can be started. Activity Patients with a simple controlled bleed may resume regular activity; however, instruct individuals not to forcefully blow or pick their noses. For a few days, avoiding contact sports or activities that may directly traumatize the nose is probably prudent. No medications are required for simple epistaxis. An antibiotic with staphylococcal coverage (eg, oral cephalexin, intravenous cephazolin) or antistaphylococcal penicillin (eg, orla dicloxacillin, intravenous nafcillin) is required for patients sent home or admitted with anterior or posterior packing in place. Antibiotic agents Antibiotics with staphylococcal and streptococcal coverage are required if nasal packing is placed. The oral route is used most commonly because most patients are treated on an outpatient basis. If the patient requires admission,

initially use intravenous medications. Continue all antibiotics until the packing is removed Management How do you stop the common nosebleed? Most people who develop nose bleeding can handle the problem without the need of a physician if they follow the first aid recommendations below: 1. Pinch all the soft parts of the nose together between your thumb and index finger. 2. Press firmly toward the face - compressing the pinched parts of the nose against the bones of the face. 3. Lean forward slightly with the head tilted forward. Leaning back or tilting the head back allows the blood to run back into your sinuses and throat and can cause gagging or inhaling the blood. 4. Hold the nose for at least five minutes. Repeat as necessary until the nose has stopped bleeding. 5. Sit quietly, keeping the head higher than the level of the heart. Do not lay flat or put your head between your legs. 6. Apply ice (wrapped in a towel) to nose and cheeks. Surgical Care Cauterization of an identified small bleeding area (only one side should be cauterized at a time to avoid possible septal perforation)
y y y y y

Can be performed with silver nitrate sticks Caution advised not to burn the entire septum or cause perforation (septal perforation is a risk) Performed in only one nostril at a time Used very judiciously; must avoid nasal tissues other than the bleeding site of septum Presents risk of nasal stenosis of the vestibule

Nasal packing items and procedures
y y

Quarter-inch strips of gauze impregnated with petroleum jelly, layered with the use of bayonet forceps (see images below) Bayonet forceps.

Vaseline gauze packing.
y y

Oxycel cotton with bacitracin, which dissolves and does not have to be removed, preferred by some (especially helpful in patients with leukemia) Merocel or other tamponlike packing (see image below) that expands when water is injected into it Expandable (Merocel) packing (dry). Epistat, Rapid Rhino, or other balloon inflation catheter (see image below) Epistat anterior and/or posterior nasal catheter. Ligation of vessels Angiographic embolization

y

y y

Prognosis With a little patience and pressure, almost all uncomplicated anterior nosebleeds respond to simple first-aid measures. Even the rare nosebleed that requires a doctor's care usually can be treated successfully with cauterization, packing or other options. Even severe posterior nosebleeds can be controlled with appropriate first-aid measures at home. Some people who have excessive bleeding, multiple medical problems or who are taking anticoagulant medications may need to be hospitalized for treatment of a nosebleed. F. SINUSITIS

y

Sinuses are hollow air spaces in the human body. In relation to the respiratory system, each sinus cavity has an opening into the nose for free exchange of air and mucus and is joined with the nasal passages by a continuous mucous membrane lining. The maxillary (behind the cheek) and ethmoid (between the eyes) sinuses are small but present at birth. The child¶s sinus cavities are not fully developed until 20 years of age, which makes the child vulnerable to sinus infection. Sinusitis is an infection of the sinus cavities.

Etiology: y It occurs as a secondary infection in older children when streptococcal, staphylococcal, or H. influenzae organisms spread from the nasal cavity.

Pathophysiology The ostiomeatal complex (OMC) is believed to be the critical anatomic structure in sinusitis and is entirely present, although not at full size, in newborns. Present within the middle meatus, the OMC is composed of the uncinate process, infundibulum ethmoidalis, hiatus semilunaris, ethmoid bulla, and frontal recess. Although obstruction of the OMC has not been proven to be the primary source for pediatric sinusitis, changes occurring in the anterior ethmoids are known to impair drainage through the OMC, resulting in chronic maxillary sinusitis and, occasionally,frontal sinusitis. The normal metachronous movement of mucous toward the natural ostia of the sinuses and eventually to the nasopharynx can be disrupted by mucosal inflammation. This most commonly occurs secondary to routine viralupper respiratory tract infections (URTIs) or nasal allergies and the host response to these insults. In addition, many other predisposing factors to chronic disease exist, including allergic rhinitis, anatomical abnormalities,gastroesophageal reflux (GER), immune deficiency, and disorders of ciliary function.

S/S: y y y y fever, a purulent nasal discharge headache, tenderness over the affected sinus.

Diagnostic Test: y Treatment:  for acute sinusitis consists of an: o antipyretic for fever o an analgesic for pain o an antibiotic for the specific organism involved.  Oxymetazoline hydrochloride (Afrin), supplied as nose drops or a nasal spray, shrinks the edematous mucous membranes and allows infected material to drain from the sinuses. A nose and throat culture will identify the infectious organism. 

To avoid a rebound effect, this type of nasal spray should be used for only 3 days at a time; otherwise, it actually causes more nasal congestion than was present originally.  Warm compresses to the sinus area may encourage drainage and relieve pain.  Some children need acetaminophen (Tylenol) for pain. Patient Education     Child should not dive. Child should not travel by airplane. Urge parent to eliminate triggers in the home (dust, smoking) Have all members of the family treated, if indicated.

Prognosis of Sinusitis: Sinusitis can be acute (going on less than four weeks), subacute(4-12 weeks) or chronic (going on for 12 weeks or more) . G. LARYNGITIS Laryngitis: Laryngitis is an inflammation of the mucous membrane lining the larynx which is located in the upper part of the respiratory tract CAUSES Viral infection - common cause of acute laryngitis Allergies Vocal straining Larynx cancer Inhaled irritants Persistent coughing (see Severe cough) Chlamydia pneumoniae - laryngitis Chemical poisoning -- Acrylic acid - laryngitis Common cold Inhaled combined corticosteroid bronchodilator use B-hemolytic streptococcus Haemophilus influenzae Streptococcus pneumoniae Rhinovirus - laryngitis Tumour Aging changes Influenza Aspiration of caustic chemical Injury or compression of recurrent laryngeal nerve Misuse or abuse of voice Inhaling irritating substances

Tuberculosis Barrett's esophagus - Laryngitis Moraxella catarrhalis infection - laryngitis Bacterial or fungal infection Inflammation due to overuse of the vocal cords As an isolated local infection of the larynx as part of another, more serious underlying disorder, such as pneumonia or tuberculosis Postnasal drip Inhalation of smoke fumes or caustic chemicals Chronic upper respiratory tract disorders such as sinusitis, bronchitis, nasal polyps Excessive coughing (see Coughing spasms) Smoking Alcohol consumption (see Alcohol use)

Signs and Symptoms
y y y y y y y y y y y y y y y y y

Hoarseness Loss of voice Sore throat Difficulty swallowing Lump in throat feeling Fever Symptoms of laryngitis can vary, depending on the severity and also the cause. The most common, and obvious, symptom is Impaired speech, ranging from a raspy hoarseness to the total loss of ability to speak, except at a whisper. Dry, sore throat Coughing Sensation of swelling in the area of the larynx Cold or flu-like symptoms Swollen lymph glands in the throat, chest, or face Sensation of a lump in the throat or constant need to clear the throat Difficulty breathing Difficulty singing Difficulty eating

Treatment List for Laryngitis
y y y y y y y y y y y

Symptomatic treatment Resting the voice Steam inhalation Analgesics Voice rest Antibiotics, Antifungal, Antacid medication Throat lozenges Warm liquids Cool mist humidifier Cessation of smoking, alcohol Speech therapy Prognosis of Laryngitis: It is a minor ailment and clears up on its own within a few days or weeks.

H. CROUP (LARYNGOTRACHEOBRONCHITIS)  Croup (inflammation of the larynx, trachea, and major bronchi) is one of the most frightening diseases of early childhood for both parents and children.  parainfluenza virus. In previous years, the most common cause wasH. influenzae. Assessment:        children typically have only a mild upper respiratory tract infection at bedtime. Temperature is normal or only mildly elevated. During the night, they develop a barking cough (croupy cough), inspiratory stridor marked retractions. They wake in extreme respiratory distress. The larynx, trachea, and major bronchi are all inflamed.

Therapeutic Management  to run the shower or hot water tap in a bathroom until the room fills with steam, then keep the child in this warm, moist environment.  cool moist air with a corticosteroid such as dexamethasone, or racemic epinephrine, given by nebulizer, can reduce inflammation and produce effective bronchodilationto open the airway  Intravenous therapy may be prescribed to keep the child well hydrated. 

Maintain accurate intake and output records and test urine specific gravity to ensure that hydration is adequate. I. EPIGLOTTITIS  Epiglottitis is inflammation of the epiglottis (the flap of tissue that covers the opening to the larynx to keep out food and fluid during swallowing).  It occurs most frequently in children from 2 to about 7 years of age .  Epiglottitis can be either bacterial or viral in origin. H. influenzae type B has been replaced as the most common bacterial cause of the disorder by pneumococci, streptococci, or staphylococci. Echovirus and respiratory syncytial virusalso can cause the disorder. Assessment:  Symptoms begin as those of a mild upper respiratory tract infection.  After 1 or 2 days, as inflammation spreads to the epiglottis,  the child suddenly develops severe inspiratory stridor  a high fever  hoarseness  a very sore throat.  difficulty swallowing that he or she drools saliva  child may protrude the tongue to increase free movement in the pharynx. If a child's gag reflex is stimulated with a tongue blade, the swollen and inflamed epiglottis can be seen to rise in the back of the throat as a cherry-red structure. It can be so edematous, however, that the gagging procedure causes complete obstruction of the glottis and respiratory failure. Therefore, in children with symptoms of epiglottitis (dysphagia, inspiratory stridor, cough, fever, and hoarseness), never attempt to visualize the epiglottis directly with a tongue blade or obtain a throat culture unless a means of providing an artificial airway, such as tracheostomy or endotracheal intubation, is readily available. This is especially important for the nurse who functions in an expanded role and performs physical assessments and routinely elicits gag reflexes. Diagnostic Test:  leukocytosis (20,000 to 30,000 mm3), with the proportion of neutrophils increased.  A blood culture to evaluate for septicaemia  ABGs to evaluate respiratory sufficiency may be ordered. Therapeutic Management:  Children need moist air to reduce the epiglottal inflammation.  If cyanosis is present, they need oxygen. 

An antibiotic, such as a second-generation cephalosporin (e.g., cefuroxime).  Because they can't swallow, children need intravenous fluid therapy to maintain hydration.  They may need a prophylactic tracheostomy or endotracheal intubation to prevent total obstruction, although it is often difficult to intubate children with epiglottitis because the tube cannot be passed beyond the edematous epiglottis.  After antibiotic therapy begins, the epiglottal inflammation recedes rapidly. By 12 to 24 hours, it has reduced enough that the airway may be removed.  Antibiotic administration will continue for a full 7 to 10 days.  Siblings of the ill child may be prescribed prophylactic antibiotic therapy to prevent them from developing the same symptoms.

Nursing Diagnoses and Related Interventions Nursing Diagnosis: Ineffective airway clearance related to edema and constriction of airway Outcome Evaluation: Respiratory rate is below 22 breaths/min; no cyanosis is present; PO2 is 80 to 100 mm Hg; SaO2 is over 95%. Attach a sensor for pulse oximetry monitoring and remain constantly with a child with croup, not only to observe closely for increasing respiratory distress but also to reduce the child's anxiety. Take vital signs as often as every 15 minutes, because extreme restlessness and thrashing, increased stridor, increased heart and respiratory rates, and cyanosis are symptoms of oxygen deprivation. In some children, it is difficult to distinguish between fright from the newness of the experience (and their sense of their parents' fright) and the anxiety that comes from oxygen deprivation. Keep a continuous record of vital signs and activity as a way to demonstrate increasing respiratory rate and restlessness. ABGs may be obtained to assess for sufficient oxygenation if pulse oximetry is not being used. A tracheostomy or endotracheal intubation along with oxygen therapy may be necessary if symptoms do not diminish. (It is difficult to intubate children with croup because of the severe respiratory tract edema.) Laryngospasm with total occlusion of the airway can occur when a child's gag reflex is elicited or when the child is crying. Therefore, do not elicit a gag reflex in any child with a croupy, barking cough, and provide comfort to prevent crying. Croup is a frightening disease for parents because their child is suddenly ill with severe symptoms. When the severe symptoms disappear by morning, parents may feel foolish they rushed to a hospital with the child in the middle of the night. Assure them that their initial judgment was correct. When they brought the child in, he or she was seriously ill. Parents may be reluctant to see their child

discharged in the morning until they are convinced that he or she is now well enough to go home (Box 40.10). TABLE 40.5 Epiglottitis Comparison of Laryngotracheobronchitis (Croup) and

Assessment Laryngotracheobronchitis Epiglottitis Causative Usually viral Usually pneumococci or organism streptococci Usual age of 6 mo±3 yr 3±6 yr child Seasonal Late fall and winter None occurrence Onset pattern Preceded by upper respiratory Preceded by upper infection; cough becomes worse respiratory infection; at night suddenly very ill Presence of Low grade Elevated to about 103°F fever Appearance Retractions and stridor; prolonged Drooling; very ill-appearing; inspiratory phase of respirations; neck hyperextended to not very ill-appearing breathe. (Do not attempt to view enlarged epiglottis, or immediate airway obstruction can occur.) Cough Sharp, barking Muffled cough Radiographic Lateral neck radiograph showing Lateral neck radiograph findings subglottal narrowing showing enlarged epiglottis Possible Asphyxia due to subglottic Asphyxia due to supraglottic complications obstruction obstruction

II.

LOWER RESPIRATORY DISORDER

A. Influenza - involves inflammation and infection of the trachea and bronchi, caused by orthomyxoviruses types A, B, and C. The virus attaches to and penetrates respiratory epithelial cells where viral replication occurs, which results in the destruction of the host cell. - can be transmitted through direct contact such as kissing, touching or holding hands with an infected person, indirect contact, and droplet when a person coughs or sneezes.

SIGNS/SYMPTOMS ‡ ‡ ‡ ‡ ‡ Sore throat Cough Aching pains Fatigue Fever ‡ ‡ ‡ ‡ Vomiting Diarrhea Chills and shakes Loss of appetite

DIAGNOSTIC PROCEDURES
y y y

nasopharyngeal or nasal swab, and nasal wash or aspirate. Samples should be collected within the first 4 days of illness. Routine serological testing for influenza some respiratory samples should be tested by both rapid tests and by viral culture. The collection of some respiratory samples for viral culture is essential for determining the influenza A subtypes and influenza A and B strains causing illness, and for surveillance of new strains that may need to be included in the next year's influenza vaccine.

MANAGEMENT Antipyretic suh as acetaminophen (Tylenol) are given to reduce fever. A new antiviral drug, Oseltamivir (TamiFlu), is given to children above 1 year of age at the first sign of illness, to halt virus replication. An influenza vaccine must also be readministered yearly. DIAGNOSIS 1. Fluid Volume Deficit: Related to fever, increased secretions, decreased appetite, possible vomiting/diarrhea 2. Imbalanced Body Temperature (Fever): Related to influenza 3. Altered Nutrition: Less Than Body Requirements: Related to decreased appetite 4. Acute Pain: Related to influenza (body aches, possible chest pain, headache, etc) PROGNOSIS Most people recover fully from the flu. But some develop serious complications. Complications can include life-threatening conditions such as pneumonia. B. Bronchitis inflammation of the major bronchi and trachea one of the more common illnesses affecting preschool and school-age children

-

Causative agents include the influenza viruses, adenovirus, and Mycoplasma pneumonia

SIGNS/SYMPTOMS ‡ Fever ‡ Cough ‡ Sore throat DIAGNOSTIC PROCEDURES
y y y y y y

‡ ‡ ‡

Nasal congestion Crackles/rales Malaise

Chest X-Ray Complete Blood Count (CBC) Test History and Physical Exam Sputum Smear Examination Lung Function Tests X-Ray

MANAGEMENT ‡ Analgesic and antipyretic agents - are used to control fever, myalgias, and arthralgias. ‡ Bronchodilators ‡ Antibiotics ‡ Antivirals DIAGNOSIS Ineffective airway clearance related to excessive, thickened mucous secretions PROGNOSIS In an average, healthy person, acute bronchitis typically clears up quickly on its own or with antibiotic treatment. Some people, including the elderly, infants, smokers or people with heart or lung disorders, are at higher risk of developing pneumonia from acute bronchitis. C. Bronchiectasis Greek bronchion, meaning windpipe, and ektasis, meaning stretched dilatation of bronchi with destruction of elastic walls due to acute/chronic infection or inflammation, anatomic airway obstruction chronic dilatation and plugging of the bronchi may follow pneumonia, aspiration of a foreign body, pertussis, or asthma often associated with cystic fibrosis

SIGNS/SYMPTOMS Chronic, productive cough Occasional hemoptysis Wheezing/stridor in infants Shortness of breath

MANAGEMENT ‡ ‡ ‡ Antibiotics Bronchodilators - indicated when bronchial hyperreactivity is evident, used to improve ciliary beat frequency and, thus, facilitate mucus clearance Chest physiotherapy: Manual and mechanical interventions such as chest percussion, vibration, postural drainage

DIAGNOSIS 1. Impaired gas exchange related to ventilation±perfusion inequality 2. Ineffective airway clearance related to bronchoconstriction, increased mucus production, ineffective cough, bronchopulmonary infection, and other complications 3. Ineffective breathing pattern related to shortness of breath, mucus, bronchoconstriction and airway irritants 4. Self-care deficits related to fatigue secondary to increased work of breathing and insufficient ventilation and oxygenation 5. Activity intolerance due to fatigue, hypoxemia, and ineffective breathing patterns 6. Ineffective coping related to reduced socialization, anxiety, depression, lower activity level, and the inability to work 7. Deficient knowledge about self-management to be performed at home. PROGNOSIS Early recognition and adequate treatment can help control bronchiectasis and decrease symptoms. Life- long awareness of the need for treatment may allow people with bronchiectasis to minimize complications and maximize life expectancy. The outlook depends upon the underlying reason for developing bronchiectasis. Congenital causes of bronchiectasis, like cystic fibrosis, may have a worse prognosis than acquired diseases. D. Status Asthmaticus When children fail to respond and an attack continues Caused by: ‡ viral respiratory illness ‡ following exposure to a potent allergen or irritant ‡ after exercise in a cold environment ‡ history of endotracheal intubation and mechanical ventilation ‡ frequent emergency department visits ‡ poor adherence to the medical regimen

SIGNS/SYMPTOMS ‡ ‡ ‡ ‡ ‡ ‡ Chest tightness Hyperexpanded chest, and accessory muscles (sternocleidomastoid and intercostal muscles) are used Audible wheezing Rapidly progressive shortness of breath Tachypnea/tachycardia Dry cough

DIAGNOSTIC PROCEDURES - CBC count and differential to evaluate for infectious causes (eg, pneumonia, viral infections such as croup), allergic bronchopulmonary aspergillosis, and Churg-Strauss vasculitis - arterial blood gas (ABG) value to assess the severity of the asthma attack and to substantiate the need for more intensive care. The 4 stages of blood gas progression in persons with status asthmaticus are as follows:
y y y

y

The first stage is characterized by hyperventilation with a normal partial pressure of oxygen (PO2). The second stage is characterized by hyperventilation accompanied by hypoxemia (ie, a low partial pressure of carbon dioxide [PCO2] and low PO2). The third stage is characterized by the presence of a false-normal PCO2; ventilation has decreased from the hyperventilation present in the second stage. This is an extremely serious sign of respiratory muscle fatigue that signals the need for more intensive medical care, such as admission to the ICU and, probably, intubation with mechanical ventilation. The last stage is characterized by a low PO2 and a high PCO2, which occurs with respiratory muscle insufficiency. This is an even more serious sign that mandates intubation and ventilatory support.

- chest radiograph to evaluate for pneumonia, pneumothorax, congestive heart failure, and signs of chronic obstructive pulmonary disease, which would complicate the patient's response to treatment or reduce the patient's baseline spirometry values MANAGEMENT Ipratropium treatment Oxygen therapy Fluid replacement Antibiotics

DIAGNOSIS 1. Risk for suffocation related to bronchospasm, mucous secretion and edema 2. Risk for fluid volume deficit related to difficulty taking fluids, insensible losses from hypoventilation and diaphoresis 3. Risk for injury related to hypoventilation and dehydration PROGNOSIS

In general, unless a complicating illness such as congestive heart failure or chronic obstructive pulmonary disease is present, with appropriate therapy status asthmaticus has a good prognosis. A delay in initiating treatment is probably the worst prognostic factor. Delays can result from poor access to health care on the part of the patient or even delays in using steroids. Patients with acute asthma should use steroids early and aggressively. E. Respiratory Syncytial Virus Bronchiolitis most common cause of bronchiolitis in young children can be transmitted through: direct contact, indirect contact, and droplet

In the community setting, a number of factors have been associated with increased risk of acquiring RSV disease, including the following: Childcare attendance Older siblings in preschool or school Crowding, lower socioeconomic status Exposure to environmental pollutants (eg, cigarette smoke) Multiple birth sets (especially triplets or greater) Minimal breastfeeding

SIGNS/SYMPTOMS
y y y y y y y y

Fever (typically low-grade) Cough Tachypnea Cyanosis Retractions Wheezing Rales Sepsislike presentation or apneic episodes (in very young infants)

DIAGNOSTIC PROCEDURES - CBC count, serum electrolytes, urinalysis, and oxygen saturation measurement. The CBC count may reveal a normal or mildly elevated WBC count and an elevated percentage of band forms. Blood cultures, although obtained frequently, are rarely positive for pathogenic bacteria. - An arterial blood gas may be indicated if carbon dioxide retention is a concern. - tests can be performed on samples of secretions obtained by washing, suctioning, or swabbing the nasopharynx. Secretions can be analyzed for virus in the laboratory by culture and/or antigen revealing techniques.

- Chest radiography is frequently obtained in children with severe RSV infection. Chest radiography typically reveals hyperinflated lung fields with a diffuse increase in interstitial markings. In 20-25% of cases, focal areas of atelectasis and/or pulmonary infiltrate are also noted. MANAGEMENT ‡ ‡ ‡ Supportive treatment Isolation Ribavirin, antiviral

PROGNOSIS Children hospitalized secondary to RSV infection typically recover and are discharged in 3-4 days. High-risk infants remain hospitalized longer and have higher rates of ICU admission and mechanical ventilation. Infants hospitalized due to RSV infection have higher rates of subsequent wheezing than age-matched controls not hospitalized for this condition over the next 10 or more years. Whether RSV leads to alterations of airways and/or immune responses that contribute to these subsequent events or is just a marker for abnormal airways is still not completely understood.

F. PNEUMONIA

DESCRIPTION y Infection and inflammation of alveoli. It occurs at a rate of 2-4 children in 100. Most common cause of pulmonary death in infants younger than 48 hours.

ETIOLOGY y y It maybe of bacterial origin (pneumococcal, streptococcal or chlamydial) or viral (RSV). Aspiration of lipid or hydrocarbon substances also causes pneumonia.

TYPES 1. PNEUMOCOCCAL PNEUMONIA The onset of pneumococcal pneumonia is abrupt and follows an upper respiratory tract infection. In infants, pneumonia tends to remain

bronchopneumonia with poor consolidation. In older children, pneumonia may localize in a single lobe, and consolidation may occur.  MANIFESTATION High fever Nasal flaring Retractions Chest pain Chills Dyspnea Tachypnea Tachycardia Diminished respiratory function Bronchial breath sounds 

LABORATORY - Chest X ray - Leukocytosis  TREATMENT - Antibiotics: Ampicillin or third generation cephalosphorins - Amoxicillin clavulanate (Augmentin) maybe prescribed for penicillinresistant organisms - Antipyretic: Acetominaphin (febrile)  MANAGEMENT - Intravenous therapy - Humidified oxygen   NURSING MANAGEMENT Turning and repositioning to avoid pooling of secretions Chest physiotherapy NURSING DIAGNOSIS Ineffective airway clearance Impaired gas exchange Acute pain Risk for infection Risk for fluid volume deficit

2. CHLAMYDIAL PNEUMONIA Most often seen in newborns up to 12 weeks of age because the chlamydial organism is contracted from the mother¶s vagina during birth.    MANIFESTATION Nasal congestion Sharp cough Failure to gain weight Tachypnea Wheezing and rales on auscultation LABORATORY Elevated level of immunoglobulin IgG and IgM antibodies Peripheral eosinophilia Specific antibody to C. trachomatis TREATMENT Macrolide antibiotic: erythromycin

3. VIRAL PNEUMONIA Generally caused by the viruses of the upper respiratory tract infection (RSV, myxovirus or adenovirus).    MANIFESTATION Begins as an upper respiratory tract infection Low grade fever Nonproductive cough Tachypnea Diminished breath sounds Fine rales LABORATORY Chest X ray: diffuse infiltrated areas MANAGEMENT Rest Antipyretic (febrile) Intravenous fluid

4. MYCOPLASMAL PNEUMONIA Occurs more frequently in children in children over 5 years of age and more often during winter.   MANIFESTATION Fever Cough Feels ill Enlarged cervical lymph nodes Persistent rhinitis TREATMENT Erythromycin: for children younger than 8 years old

5. LIPID PNEUMONIA Caused by the aspiration of an oily or lipid substance. It is much less common than it was once because children are not given oil-based tonics anymore.  MANIFESTATION - Initial coughing spell at time of aspiration - Period of symptomless - Chronic cough - Dyspnea - General respiratory distress  LABORATORY - Chest X ray: densities at affected site  TREATMENT - Antibiotic therapy if secondary bacterial infection has occurred  MANAGEMENT - Surgical resection of lung portion 6. HYDROCARBON PNEUMONIA Common household products like furniture, polish, cleaning fluids causes childhood poisoning which results to hydrocarbon pneumonia.  MANIFESTATION - Nausea and vomiting - Drowsy 

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Cough Increased and dyspneic respirations Increased percussion sound Rales MANAGEMENT Supplemental oxygen: cool, moist air Antipyretic (febrile) Positioning Chest physiotherapy

G. ATELECTASIS DESCRIPTION y TYPES 1. PRIMARY ATELECTASIS Occurs in newborns who do not breathe with enough respiratory strength at birth to inflate lung tissue or whose alveoli are so immature or lacks in surfactant. This is seen most commonly in immature and with CNS damage. It may occur when infants have mucus or meconium plugs in the trachea.  MANIFESTATION Irregular respirations Nasal flaring Apnea Respiratory grunt The collapse of lung alveoli. It may occur in children as a primary or secondary condition.

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Cyanosis

2. SECONDARY ATELECTASIS Occurs in children when they have respiratory tract obstruction that prevents air from entering a portion of the alveoli. The causes of obstruction in children include mucus plugs that may occur with chronic respiratory distress or aspiration of foreign objects.    MANIFESTATION Asymmetry of chest Decreased breath sounds on affected side Tachypnea Cyanosis LABORATORY Chest X ray: collapsed alveoli MANAGEMENT If atelectasis is caused by foreign object: bronchoscopy is performed to remove the object. If atelectasis is caused by mucus plug: moving out or expectoration of mucus 

NURSING MANAGEMENT - Make sure that chest is kept free from pressure to allow full lung expansion - Check clothing and make sure it is loose and nonbinding - Make sure that child¶s arm are not position across the chest because weight can interfere with deep inspiration - Place in a semi-fowler¶s position because it lowers abdominal contents and increases chest space - Increase humidity of the environment to prevent further bronchial plugging - Suction and chest physiotherapy  NURSING DIAGNOSIS - Ineffective airway clearance - Impaired gas exchange - Ineffective breathing pattern - Risk for volume deficit H. PNEUMOTHORAX  DESCRIPTION AND ETIOLOGY

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Presence of atmospheric air in the pleural space. In children, it usually occurs when air seeps from ruptured alveoli and collects in the pleural cavity. It can also occur when external puncture wounds allow air to enter the chest.  MANIFESTATION - Tachypnea - Grunting respirations - Flaring of the nares - Cyanosis - Absent or decreased breath sounds  LABORATORY - Chest X Ray: darkened area of the air-filled fluid space  TREATMENT - Oxygen therapy: relieve respiratory distress - Thoracotomy catheter or needle with low-pressure suction with water sealed drainage: remove accumulated air  NURSING DIAGNOSIS - Ineffective breathing pattern - Risk for trauma or suffocation - Deficient knowledge I. TUBERCULOSIS 

DESCRIPTION AND ETIOLOGY y A highly contagious pulmonary disease. It is caused by Mycobacterium tuberculosis. The mode of transmission is inhalation of infected droplets. The incubation period is 2-10 weeks.  MANIFESTATION - Fever - Hemoptysis - Sweating  LABORATORY - Tuberculin test - Mantoux test: 5-10 mm of induration is positive - Sputum test  TREATMENT - Isoniazid - Rifampicin - Para-aminosalicylic acid ( PAS) - High protein, calorie and pyridoxine diet  MANAGEMENT 

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Periodic chest x rays BCG vaccine Prophylactic isoniazid NURSING DIAGNOSIS Ineffective airway clearance Risk for impaired gas exchange Risk for spread of infection

J. CYSTIC FIBROSIS  y DESCRIPTION AND ETIOLOGY The disorder is inherited as an autosomal recessive trait. It occurs approximately in 1 in 2500 live births. It occurs most commonly in Caucasian children and rarely in black or Asian children. All newborns can be screened at birth by a simple heel puncture blood sample for the disorder.

y  MANIFESTATION PANCREAS INVOLVEMENT The acinar cells of the pancreas normally produce lipase, trypsin and amylase, enzymes that flow into the duodenum to digest fat, protein and carbohydrate. With Cystic Fibrosis, these enzyme secretions become so thickened that they plug the ducts, there is such a back pressure in the acinar cells that they become atrophied and then are no longer capable of producing the enzymes. Without pancreatic enzymes in the duodenum, children cannot digest fat, protein and some sugars. The child¶s stool become large, bulky and greasy (steatorrhea). The intestinal flora increases because of the undigested food; when combined with fat in the stool, gives the stool an extremely foul odor. The bulk of feces in the intestine leads to a protuberant abdomen. Because children are benefiting from only about 50% of the food they ingest, they show signs of malnutrition ± emaciated extremities and loose, flabby folds of skin on their buttocks. The fat soluble vitamins, particularly A, D and E, cannot be absorbed because fat is not absorbed, so children develop symptoms of low levels of these vitamins. In approximately 10% of children with CF, the meconium may be so thick, because pancreatic enzymes are lacking, that it obstructs the intestine. The newborn develops abdominal distention with no passage of stool. Rectal

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prolapsed from straining to evacuate hard stool is another common findings in infants with CF. y LUNG INVOLVEMENT Thickened mucus pools in the bronchioles. Pockets of infection then begin in these secretions. Secondary emphysema occurs because air cannot be pushed past the thick mucus on expiration, when all bronchi are narrower than they are on inspiration. Bronchiectasis and pneumonia occur. Respiratory acidosis may develop because obstruction interferes with the ability to exhale carbon dioxide. Atelectasis occurs as a result of absorption of air from alveoli behind blocked bronchioles. The child fingers become clubbed because of the inadequate peripheral tissue perfusion. The anterior-posterior diameter of the chest becomes enlarged. y SWEAT GLAND INVOLVEMENT If CF is not diagnosed by a screening blood sample at birth, it can be diagnosed by documenting the chromosomal abnormality, or by ht history and the combination of the abnormal concentration of chloride in sweat, the absence of pancreatic enzymes in the duodenum, the presence of immunoreactive trypsinogen in the blood and pulmonary involvement. CF may be suspected in newborn when a newboern losses the normal amount of weight at birth but then, the infant cannot make use of the fat in milk, does not gain it back at the usual time of 7 to 10 days and perhaps not until 4 to 6 weeks of age. Chromosome analysis or analysis of serum immunoreactive trypsin in the stool, which is elevated because obstruction in the pancreas occurs as early as during fetal life, confirms the diagnosis. Children who are not diagnosed at birth may be seen in a health care setting at about 1 month of age because of a feeding problem. Using only about 50% of their intake because of the poor digestive function, they are always hungry. This cause them to eat so ravenously they tend to swallow air. This leads to colic or abdominal distention and vomiting. The appearance of typical CF stools is an important finding because children with simple colic do not show these changes in stool consistency. Respiratory infections develop at 4 to 6 months of age. Even at this early stage of the disease, wheezing and rhonchi may be heard on chest auscultation. By the time a child with CF is a preschooler, a cough is a prominent finding. On percussion, the chest is hyperresonant, reflecting the emphysema present. Rales

and rhonchi are heard. Clubbing of the fingers may already be apparent. It is rare for a child to go undiagnosed beyond this time because the symptom of the illness have become so persistent and evident.  LABORATORY - Sweat testing: a level of more than 60 mEq/L chloride in children is diagnostic of CF. - Duodenal analysis: alaysis of duodenal secretions for detection of pancreatic enzymes reveals the extent of the pancreatic involvement. - Stool analysis: stool may be collected and analyzed for fat content and lack of trypsin, although description of the large greasy appearance maybe all that is necessary. - Pulmonary testing: Chest radiograph generally confirm the extent of pulmonary involvement. It is done to determine if atelectasis and emphysema are oresent.  MANAGEMENT - Therapy for children with CF consists of measures to reduce the involvement of the pancreas, lungs and sweat glands. - Diet: high sodium

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