Patterns of Growth and Development Growth Measurable quantitative change

Development Qualitative change that cannot be measured

Directional  Cephalocaudal Direction - In about 2 months of intrauterine life, the head is ½ of the body Head lag 1st 3 months of life, infant has lack of head control - May cause SIDS - Absence of head lag start at the 3rd month of life 4th month, body can turn left and right 5th month Infant can roll over voluntarily Control of infant’s abdomen

If there is presence of head lag in the 6th month, this may indicate cerebral palsy  Proximodistal - From the center to the farthest part of the body - Central incisors (lower 60%, upper 40%), takes 2 years to more or less complete - 18th month ~ tooth brushing, baby can’t digest fluoride in the first year of life, water should be given before and after feedings - 2 y/o ~ complete set of deciduous (18) teeth - 5 – 6 y/o ~ falling of deciduous teeth - 16 y/o complete set of deciduous teeth - *Age in Months – 6 = estimated number of baby teeth - Teething starts at 6 months, first eruption occurs at around 7 months General to Specific - Simple tasks to complicated functioning - Language development ~ earliest sign of giftedness - Dada is uttered first rather than Mama - First evidence of baby’s language is crying - Crying -> coos and girgles -> babbling sounds -> monosyllable -> words -> sentences -> sing Sequential Pattern - Predetermined - E.g. ability to ambulate

Creeping ~ crawling ~ sit with support ~ sit without support ~ stand with support ~ walk with support ~ stand without support ~ walk without support Developmental - Universal and individual - Does not happen at the same rate and pace - E.g. height - Accelerated growth (adolescence and infancy) - Weight is 2x in 6 months, 3x in 1 year, 4x in 2 years - Height is 1inch/month in first 6 months, 1 ½inch/month in the next 6 months - Increase in height in girls happens before first menses - Increase in height in boys happens after spermarche (first ejaculatory experience, production of sperm, 13.3 y/o)

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Sigmund Freud    “All human behavior is energized by psychodynamic forces divided into components of personality” Id-refers to infants, “it’s ok to spoil infants”, narcissistic, pleasure principle Ego-develops in late infancy, peaks in toddlerhood; toddler is very possessive, egocentric (I, Me, My), Reality Principle Superego-developed during pre school; moral principle, moral arbitration, ego ideal (feel good about what you feel), conscience (feel bad about what you did); peaks during school age

Toddlerhood 1 ½ - 3 (Erik Erikson), 1-3 Freud P – Period of negativism; parallel play; pounding toys/boards (in order to minimize tantrums); phone toys, push and pull toys; ritualistic behavior (sign if the cross 2 y/o, simple prayers, telling stories repeatedly) - R – Regression when child is ill or hospitalized - A – Accident ~ leading cause of death for pediatric clients, toddlers (poisoning-locks poisons, falls-fences, drowning-supervision), Autonomy vs. Shame and Doubt (struggle to be independent) - I – Independence - S – Separation Anxiety ~ number 1 fear of toddlers, resolved by 7 y/o, fear of abandonment, assure that you’ll be back, toddler has trouble sleeping - E – Egocentricism ~ I, Me, My; Elimination-toilet training whenever child is ready (18-24 months) Criteria for Starting Toilet Training 1. Child is able to sit walk and squat 2. Able to complain discomfort 3. Baby will be able to control urination; dryness for 2 hours before defecation *PRAISE toddlers to gain independence/autonomy Pre-School - 3-6 y/o - Magical thinking ~ imagination is at its peak - M – Magic ~ imagination is at its peak - #1 fear is mutilation - Things unfamiliar to them could harm them - Role playing -

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- Don’t do elective surgery for pre schoolers - Guys have fear of castration - Penis envy - Masturbation - Sexual differentiation A – Associative Play - Group play - No rules and delegation - Art work - Toys: no small removable parts, no sharps, durable, non toxic, age appropriate, dress up toys/clothes G-Guilt Initiative vs. Guilt (Phallic Stage) Imagination (Imaginary Friend) Complexes ~ Oedipal and Electra

School Age - 6-12 y/o, peak or superego - D – Death ~ understands the concept of death - Industry vs. Inferiority (failures, bullies, parents with heightened expectations) - M – Moralistic ~ modesty, peak of superego - P – Play ~ Cooperative/Competitive Play; Toys: board games, chess, snake and ladders, card games mahjongg - L – Latency ~ all sexual energies and urges are directed rapid mental development - E – Engages in activities of mental development - S – Same sex ~ period of homosexuality, grouping of same sex, period of bestfriends Adolescents - 12-20 y/o - P – Peers, wants to belong to a group - A – Accident, vehicular accident - I – Image, image is everything, constant struggle to fit in - R – Role, Identity vs. Role Confusion - S – Secondary sex characteristics Assessment for Newborn’s Well Being A. Physical Examination - Preliminary PE is immediately following birth to detect grossly observable conditions such as meningocele, cleft lip and palate, hydrocephalus, birthmark, imperforate anus, Tracheoesophageal fistula, bowel obstruction, spina bifida (congenital condition in which part of the spinal cord or meninges protrudes through a cleft in the spinal column, resulting in loss of voluntary movement in the lower body) - Meningocele – spina bifida; meninges and sac ~ CSF - Myelomeningocele- spinal cord, meninges and sac ~ CSF; irreversible

NB Assessment  Head Circumference - Assessed until 2 years of age - 33-35 cm (13-14 inches)  Length - 48-53 cm (19-21 inches)  Weight - 2700-4000 (6-9lbs) - Loss of 10% of birth weight in the 1st week but regains it in 10-14 days Vital Signs  Axillary Temperature (best and safest) - 36.5-37  Apical Heart Rate (bell – low pitch, high frequency) 120-140 bpm  Respiratory - 30-60 cpm Skin  At birth, bright red, puffy and smooth  Vernix caseosa (prevents maceration and for thermoregulation), lubricant for delivery, protects the skin from amniotic fluid  Lanugo is the soft downy hair, more lanugo means that the infant is more premature and v.v.  Acrocyanosis – bluish discoloration in the extremities/periphery  Cutis Mammorata (mottling)  Erythema Toxicum (papules)  Harlequin’s Sign (pink or pale)  Mongolian Spots (bluish pigments found on top of buttocks)  Telangiectatic nevi (stork bite 2y/o) Head  6 fontanels upon birth  Fontanels indicate fluid volume  Posterior fontanel is triangular  Anterior fontanel is diamond in shape  Fontanels are flat, smooth and firm  Bulging fontanel, increase intracranial pressure  Cephalhematoma – collection of fluid in the head  Caput Succadaneum – compression of head Eyes  Lids are usually edematous  No purulent discharges (ophthalmia neonatorum)  Absence of tears

Ears  Pinna is in line with the outer canthus of the eyes, if lower ~ down syndrome  Ear development is in the same time as the kidneys  Startle reflex  Pinna is flat against the head Nose  Nasal patency (suction catheter)  Sneezing  Flaring of nostrils, indicates breathing difficulty  NB are nasal breathers  Pedia ~ mouth breathers, tonsillitis is a pediatric disorder Mouth and Throat  Uvula at the midline  Rooting reflex  Gag Reflex (swallowing)  Extrusion (before 4 months old)  Epstein-Pearls (small white epithelial cysts) Neck  Short thick, usually surrounded by skin folds  Tonic Reflex (Fencing Reflex) Chest  Small retractions evident during inspiration  Anteroposterior and lateral diameters equal breaths and enlargement  Witch’s milk Heart  Apex is at the 4th to 5th intercostals space left mid clavicular line  Transient cyanosis when crying/straining Female genitalia  Labia and clitoris are usually edematous  Blood tinged mucoid discharge (pseudomenstruation) Genitourinary  Urinates within 24 hours Rectum  Perforate anus  Passage of Meconium with 36h  By 48h, imperforate anus  Hirschsprung's disease, or congenital aganglionic megacolon, involves an enlargement of the colon, caused by bowel obstruction resulting from an aganglionic section of bowel (the normal enteric nerves are absent) that starts at the anus and progresses upwards. The length of bowel that is affected varies but seldom stretches for more than about 30 cm. Primitive Reflexes  Rooting reflex  Sucking Reflex

 Moro/Startle Reflex  Tonic Neck Reflex  Stepping Reflex (dancing or walking reflex)  Babinski Reflex B. APGAR Score  1952  Designed by Dr. Virginia Apgar  Determines adaptation and need for resuscitation  Purpose: evaluate physical condition of the NB at birth and the need for immediate resuscitation  1min, 10min score is from 1-10 The 5 Adaptations 1. Heart Rate - Most important score, may be auscultated or palpated at the junction of the umbilical cord and skin - HR < 100bpm needs immediate resuscitation; 0=absent 1=slow<100 2=>100 2. Respiratory Effort - 2nd most important score - A mature NB cries spontaneously at about 30s after birth - 0=absent 1=slow, irregular, weak cry 2=good strong vigorous cry 3. Muscle tone - Determined by degree of flexion and resistance to strengthening of extremities - Flexion of extremities is a measure of the degree of maturity of a NB - Normal NB shows elbows and hips flexed with knees positioned up toward the abdomen - 0=flaccid 1=some flexion of extremities 2=well flexed 4. Reflex Irritability - Evaluated by either observing the NB response to having the soles of feet slapped, lightly rubbed - 0=no response 1=grimace 2=cry, cough, sneeze 5. Color - Least important score - Assess for cyanosis and pallor - Acrocyanosis in NB is so common that a score of 1 is thought as normal - 0=blue 1=body is pink, extremities is blue 2=completely pink - Dark babies will not be pink *Note: if total score is <4=serious danger, needs resuscitation; 4-6=conditions is guarded and may need clearing of airway (supplemental 02); 7-10=good C. Silverman-Anderson Index (Respiratory Evaluation) - Evaluates the degree of respiratory distress in NB - Values are added - A total score of 0 indicates no Respi Distress - Scores 4-6 indicate moderate distress - 7-10

D. Rapid Estimation of NB Gestational Age - By Usher, focuses on 5 criteria Criteria 36 weeks or more Sole Creases Breast Nodule Diameter Scalp Hair Earlobe Anterior transverse crease only 2mm Fine and fuzzy Pliable, no cartilage Testes in lower canal, scrotum small, few rugae

37-38 weeks Occasional crease 4mm Fine and fuzzy Some cartilage

39 weeks + Sole covered with many creases 7mm Coarse and silky Stiffened by cartilage Testes pendulous, scrotum, extensive rugae

Testes and Scrotum

Intermediate

E. Dubowitz Maturity Scale/Ballard’s Assessment of Gestational Age - Maturity testing tool-determines the gestational age based on the neuromuscular and physical characteristics - All NB that appear to be immature by Usher’s criteria or who are light in weight at birth or early by date are assessed by this A score is given to each assessment criteria and added to give a total score. It is then translated to NB gestational age in weeks It consists of 2 parts 1. First, a series of observations about such things as skin texture, color, lanugo, foot creases, genitalia, ear and breast maturity. The body is given a score of 0-5. 2. Second, observe for posture, square window (wrist), arm recoil, popliteal angle, scarf sign and heel to ear. Scores range from 0-5 - To estimate gestational age, both sections are compared to a standard F. Brazelton Neonatal Behavioral Assessment Scale(1973) - Provides valuable guidelines for assessing, NB state changes, temperament and individual behavior patterns are organized - Allows families to learn which responses, interventions, activities best meet the special needs of NB, fosters positive attachment exercises - Takes 20-30 minutes (complete assessment is done 3rd day after birth, first few days, behavior is disorganized) - NB response is 28 items arranged into clusters  Habituation – ability to response to and then inhibit responding to specific repeated stimuli (rattle, bell, light, pin prick on heel) while asleep

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Orientation - quality of alert states an ability to attend to visual and auditory stimuli while alert how often and where the NB attends to auditory and visual stimuli - Orientation to the environment is determined by an ability to respond to cues given by others and the ability follow a visual object horizontally and vertically - Mother’s face and soft continuous voice, stimulates NB or orientation to them - Motor maturity is demonstrated by how well NB coordinates and controls motor activities – alertness, general tonus, pull to sit Variation - How often the NB exhibits alertness, state changes, activity and peaks of excitement Self quieting/Self Consoling Abilities - How often, how soon, how NB uses his own resources to quiet and console himself when upset and disturbed Social Behavior/Cuddliness - Encompasses the NB need for and response to being held. Also considered is how often and much the NB smiles and cuddles - Cuddling appears to be an indicator of personality - Cuddlers-appear to enjoy accept and seek physical contact, sleep more, form earlier and more intense attachments - Non Cuddlers-active, restless, have accelerated motor development, intolerance of physical restraint

Other Notes  DDST – Denver Developmental Screening Test  Earliest IQ test should be given at 5-6 y/o  Creeping – chest and abdomen touches the surface  Telarche – breast enlargement/development  Menarche – first menstruation  Play with nurse – therapeutic play  Toddlers – 300 word vocabulary  Pre- school – 900 word vocabulary  Temper tantrums – anxiety that cannot be verbalized  Circumcision – 10 y/o  Masturbation – starts as early as preschool  Metrosexual – Carl Jung  GID – Gender Identity Disorder  Macrocephaly + Microsomnia = Alcoholic Mom  Microcephaly + Macrosomnia = Diabetic Mom  Neurological – tested through reflexes  8months – stranger anxiety  2 months – social smiling

G. Laboratory Studies 1. Heel Stick - Common method for taking peripheral blood samples from NB; used to check Hct level at first hour of life (N= 55-55%) - Before taking sample, the hell is warmed with warm moist compresses for 5-10 minutest6o dilate the vessels in the area - Puncture should not be deeper than 2.4 mm and should be made at the outer aspect of the heel to avoid necrotizing osteochondritis from lancet penetration at the underlying calcaneus bone 2. Dextrostix test for hypoglycemia 3. NB Screening RA 9288 - Determine congenital metabolic disorder that may lead to mental retardation and even death if untreated - Ideally done on the 48th hour or at least 24th hour from birth, some disorders are not detected if it is done before 24 hours. - Uses heel prick method a. Congenital hyperthyroidism - May lead to deafness b. Congenital Adrenal Hyperplasia - Autosomal recessive disease resulting from defects in steps of steroidogenesis of cortisol from cholesterol by the adrenal glands - Involves excessive or deficient production of sex steroids and can distort or impair development or a primary or a secondary sex characteristics in affected infants, children, and adults c. Galactosemia - Caused when a baby cannot digest sugar found in milk, leads to cataracts, liver damage, mental retardation and usually death - Substituting a special formula for milk starting in the first weeks or life helps to avoid these problems d. Phenylketonuria - Caused by the child’s inability to break down phenylalanine; may lead to mental retardation, avoiding foods with phenylalanine, proper nutrition is difficult because phenylalanine occurs in almost all natural CHONS. - Dietary use of special phenylalanine free diet is essential. These include latenalac and Phenyl free - Low CHON foods as fruit, vegetables and some cereals may be allowed e. G6PD Deficiency - An inherited condition in which the body doesn’t have enough of the enzyme glucose-6phosphate dehydrogenase, which helps RBC’s function normally; can cause hemolytic anemia usually after exposure to certain medication, foods, infection or exposed to certain triggers like: illness such as bacterial and viral infections, certain pain killers and fever reducing drugs and certain antibiotics (sulf in names) - Certain anti-malarial drugs (especially those with quine in their names). Othe substances when consumes or even touch as fava beans and naphthalene

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S/S: a child with G6PD deficiency who is exposed to a medication or infection, that triggers the destruction of RBC may have no symptoms at all. Jaundice of the skin and eyes Enlarged spleen Dark tea colored urine If trigger is removed or resolved the symptoms of G6PD deficiency are resolved in a matter of weeks if symptoms are mild, no treatment is needed as body makes new RBS, anemia will improve if symptoms are severe, child may need to be hospitalized for supportive medical care

1. 2. 3. 4. 5.

Play vs. solitary play parallel play associative play cooperative/competitive body image

Fears strangers (infancy) abandonment (toddlerhood) mutilation (pre-school) failure (school age) loss of control (adolescent)

Immediate Priorities in the 1st Days of Life - early management of the NB in the Dr and in the nursery soon after the first few minutes after birth  Establish airway - Suction mouth first, then nose - Postural drainage (by gravity or supine turned in the left side  Prevent hypothermia, infection (Crede’s Prophylaxis), and bleeding (lack of Vit. K, injected in the vastus lateralis) Methods of Heat Loss 1. Evaporation 2. Radiation – heat loss to cooler object not in direct contact (aircon) 3. Conduction – heat loss to cooler object (direct contact) 4. Convection – same with conduction and aided by surrounding air current (electric fan and open window) Day to Day Care I. Bathing - Maybe given anytime convenient to the parents but should not be within 30 minutes after feeding because of regurgitation - Sponge bath is done until the cord falls off. Start from the cleanest to the most soiled are like diaper area - Avoid using powder, lotion, mild soap II. Cord Care - Should be kept dry - Cleanse are with 70% alcohol once/twice a day – drying effect antisepsis

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Nutrition A. Nutritional Allowances for the NB 1. Calories (120Cal/Kg BW/day; 50-55Cal/lb BW/day) 2. CHON – 2.2g/kg BW/day - Human milk and cow’s ,ilk provides the essential amino acid - Histidine, an amino acid, appears to be essential in infants - 16% of Cow’s milk is CHON, 8% of Human Milk is CHON 3. Fats - Linoleic acid is an essential fatty acid needed for growth and skin integrity in infants, human milk has 3x more than cow’s milk 4. CHO - Cow’s milk has 29% of its calories as CHO, 37% of calories are CHO - Lactose, disaccharides in human milk, appears to be most easily digested ; improves Ca absorption and _ retention - Formula should contain adequate CHON as this allow CHON to be used for tissue building and rather than calories encouraging normal H2O balance and preventing abnormal fat metabolism 5. Fluid - 150-200mL/kg BW/day = 2.5-3oz/lb BW/day - High metabolic rate requires large amount of fluid - Breast milk changes: Colostrum, thin, watery, yellow fluid composed of CHON, sugar, fat, water, minerals, vitamins and maternal antibodies (less fat and CHO but high in CHON sand inorganic salts makes it easier to digest) On the 2nd day to 4th day Transitional Milk Foremilk – constant forming milk from lactiferous sinuses not dependent on infant’s suck (that stimulates let down reflex) Hind Milk – formed after let down reflex; higher fat content than Foremilk Advantages of BF to the Mother 1. Convenient and economical 2. Aseptically clean 3. Serve as protective function in Breast CA prevention 4. A form of family planning; delays ovulation 5. Aids in uterine involution-release oxytocin 6. Promotes psychological closeness 7. Restful form the mother 8. Successful breastfeeding can have empowering effect 9. Because it is a skill only a woman can master

Advantages for the Baby 1. Immunologic Content: IgA; Lactoferrin – Fe binding CHON that interferes bacterial growth; Lysozyme – dissolves bacteria; Interferon; Lactobacillus bifidus 2. Nutrition 3. Easily digested 4. Infant is unlikely to have constipation 5. No allergic reaction to breast milk 6. Breastfed babies appear to have higher IQ’s 7. Breast milk contains the ideal electrolyte and mineral composition 8. Breast milk contains the ideal electrolyte and mineral composition 9. Babies appear to have less difficulty in Calcium-Potassium regulation Criteria for Breastfeeding - Must have an appropriate distribution of calories - Must meet infant’s need for water, energy, vitamins and mineral - Must be readily digestible Formula is Available in 3 Types 1. Milk based - used for average NB 2. Soy based formula - used for infants with Lactose Intolerance and Galactosemia 3. Elemental Formula - fat, CHO, CHON ~ modified Common Health Problems in NB a. Constipation - Most common in bottle fed children and almost non-existent in Breast Feed NB b. Loose stools - Excessive intake of human breast milk and drugs c. Colic - Paroxysmal/abdominal pain due to production and accumulation of excess gas – treated by feeding the NB in an upright position, burping or bubble the baby after feeding d. Spitting up or regurgitation - Overflow of milk that occurs after nursing; disappears when coordination in swallowing improves e. Skin Irritation (Diaper Dermatitis) - Prevented by frequent diaper change f. Seborrheic dermatitis/Cradle Cap - Sebaceous gland; poor hygiene g. Miliaria/Prickly Heat - Small erythemous papules and vesicles, causing itching, due to bacterial action after excessive sweating 8 Priorities at the First Days of Life 1. Initiates and maintenance of respi 2. Est. of extrauterine circulation 3. Control of Body temperature 4. Intake of adequate nourishment

5. Est. of waste elimination 6. Prevention of infection 7. Est. of infant parent relationship 8. Development of care that balances rest and elimination for mental development Other Notes 1. NB-16h of sleep 2. Infant-16h of sleep 3. Gooseneck lamp 4. Wrist band-best way to identify NB 5. Eggs and Food (yellow) 12-mos. 6. Infant should be introduced to one food at a time

Chromosomal Abnormalities No treatment but only management 1. Down Syndrome  Trisomy 21  Is a chromosomal anomaly resulting in a moderate to severe mental retardation  A chromosomal disorder mean there is a change in the number of chromosome  Normally, 23 pairs of chromosome for a total of 46, 47 in Down Syndrome, one has an extra part  With the essence if the extra number 21 , this can result to health and mental difficulties  Occurs in 1:800 With the Presence of extra chromosome it requires extra protein to be made Normal growth then disturbs cells will no longer dived as rapid, leading to smaller babies than average As the baby develops, cells could no longer move around the usual way, which means body parts Do not form normally such as the bra, fewer brain cells, provided needed for its formation (mental retardation) Resulting to health problems

Common Health Problems 1. 2. 3. 4. 5. 6. Congenital heart defects Respiratory tract infections (when they get infected last longer) Thyroid dysfunction (Congenital Hypothyroidism) Inc incidence of Leukemia) Problems in stomach or blockage in their intestines that prevent digestion Mental retardation

Cause: Unknown

Factor: Maternal age (above 35 y/o woman or older) Diagnostic Tests 1. Appearance of the baby shortly after birth is used as basis 2. Chromosomal test (blood test) – to confirm genetic abnormality; Chromosomal Analysis 3. Echocardiogram to detect heart problems (Congenital heart defects); *2D Echo Cardiogram-to detect congenital heart defects Signs and Symptoms 1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11. 12. 13. Rounded face with fat profile Small nose, depressed nasal bridge Back of the head is slightly flattened Eyes tend to slant upward Smaller tongue than average, tongue that sticks out Small short low set ears Short broad neck Transverse palmar crease (Simian Line) Short hands with stubby fingers Feet are stubby with wide space between first and 2 nd toe Usually have poor muscle tone (hypotnia) Mottled skin Generally develops at slower rate (physically and mentally)

Management (life span: 40-50 years)     Surgery to correct heart, stomach or intestinal problems Medical treatment if leukemia is present Regular vision and hearing test, they have higher risk of eye problem and hearing loss Annual thyroid test and every 2 y dental check up (tooth infection can cause bacteria to grow)

Nursing Considerations 1. 2. 3. 4. 5. Support family at the time of Dx Constant emotional and psychological support Be careful in answering questions regarding developmental potential (DS is non progressive, static) Assisting families unable or unready to take care of the NB Secondary health management

2. Klinefelter Syndrome  A chromosomal abnormality in which an individual make has one or more extra X chromosome in his cells resulting to either chromosome compliment of XXY or more rarely XXXY  Incidence rate 1:500 Often unnoticed until puberty when secondary sex characteristics at this stage start to develop  Lalaki pero may characteristics ng mga babae

Clinical Manifestation            Gynecomastia Are usually infertile due to azoospermia/aspermia (absence of sperm) Mental retardation is higher Frontal baldness Tendency to grow fewer chest hairs Brest development Female type pubic hair pattern Small testicular size Poor beard growth Narrow shoulders Wide hips longs arms and legs

Dx Test  Tx    No cure Hormonal tx: to include 2ndary sex characteristics, testosterone Genetic counseling Chromosomal analysis

3. Turner’s Syndrome  Chromosomal abnormality affecting woman. May have 45 chromosomes (N=46)  May have x missing chromosome or may have both x chromosome but is defective  Babae pero kulang ang pagkababae Clinical Manifestation     Amenorrhea Coarctation of the aorta Degree of mental retardation Adult height is 1.35 m

Medical Management    Estrogen administration Surgery at early age Growth hormone is given

Gastrointestinal System Defects 1. Cleft Lip  Congenital anomaly = slight dimple on the lop’  1:600 births. Pure genetic causes are rare but genetic fat ors appear to contribute significantly to the etiology of both these malformation  A cleft lip is an opening in the upper lip  Failure of the maxillary and median nasal process to fuse normally  Normal lip is formed of 2 tabs that grow in grim the sides of the face with a central tab that grows down from the tip of the nose. This fusion should take place in the 4th to 6th week of pregnancy. If the union is not complete, the baby is born with a cleft lip. If a cleft extends up to the nostril it is call a complete cleft lip. If the nose is not affected it is called an incomplete cleft lip  Plunger; bottlefed with big hole Clinical Manifestation  Difficulty feeding – breastfeeding is encourage  Mouth breathing - Dry and cracked mucous membrane - Venue for injection - Swallow air Nursing Dx - Altered nutrition less than body requirements R/T difficulty of feeding - Risk for disorganized infant behavior R//T pain and discomfort - Sensory perceptual alteration R/T inability to suck - Aspiration Etiologic Factors  Familial tendency or most likely the transmission of multiple genes  Teratogens present 5-8 wks (viral infection)  Deficiency of folic acid  Occurs more in boy than girls Treatment  Cheiloplasty or Lip Reduction - From Gk. Kheilos – lips - Surgery of the lip. It includes lip reduction, the process of surgically reducing the size of the lip or lips in order to reduce the appearance of abnormally large or protruding lips and the process of forming an artificial tip or part of the lips by using a piece of healthy tissue from some neighboring part - The procedure can also be performed to enhance the upper and lower lip those who wish to make their lips permanently larger - Should be done before language development (before 2y/o) - Rule of 10; 10 weeks, at least 10lbs., 10 grams of Hgb (Weight is the best indicator of health for kids below 5 y/o-DOH) 2. Cleft Palate  Fissure  1:700 live births worldwide

An opening if the palate, usually on the midline and may involve just the anterior hard palate, the posterior soft palate or both  It maybe separate anomaly, but as a rule, it occurs, in conjunction with a cleft lip  The palatal process closes at approx. 9-12 wks of IU life Etiologic Factors 1. More freq in girls than in boys 2. Appears to be the result of polygenic inheritance (multiple genes) 3. Envi/teratogenic factors at 9-12 wks of gestation Assessment  Cleft lip is apparent on inspection at birth  Can also be detected by UZ Clinical Manifestation  Infection – aspiration pneumonia  Speech problem  Excessive dental carries  Hearing problems – otitis media Nursing Dx  Altered family process R/T having a child with physical defect  Risk for infection  Altered nutrition less than body requirements R/T difficulty in feeding  Disturbed body image Classification  Incomplete palate  Unilateral complete lipa ad palate  Bilateral complete lip palate Etiology  Inheritance/ hereditary  Exposure to Teratogens  Excessive smoking of pregnant mothers Nsg Managements  Assess for problems with feeling, breathing, speech and parental biondung  Encourage adequate nutrition and prevention of aspiration  Let the baby burp every now and then  Support the infant’s parents emotionally and socially  Breathing, nutrition, fluid Treatments  Palatoplasty - Used to correct or reconstruct te palate - Close abnormal opening between the nose and the mouth to help the patient develop normal speech and to aid in swallowing breathing and normal development of associated structures in the mouth - Any person with any degree of cleft palate is a candidate for palatoplasty. The procedure is usually performed on infants

The amt of treatment required for a child with cleft lip or palate depends on how severe the conditions at birth - A small cleft lip may need only one surgical procedure in the first months if life. A child with a large cleft involving the lip and palate will need several surgical procedure and ongoing care from the Cleft team - Cleft team: 1. Audiologist 2. Dental specialist 3. ENT surgeons 4. Pedia 5. Plastic surgeon 6. Speech pathologist - Pt should be NPO (TPN) - Position should be Supine (Cheiloplasty) - Prone(Palatoplasty) - no suctions which can cause irritation Cleft lip and Palate S – Soft and hard palate M – Malnutrition I – implement Cheiloplasty and palatoplasty L – Language development is affected E – Etiology (idiopathic, congenital) S – Self image disturbance 3. Tonsillitis  Causative agent: Streptococcus pneumoniae  Inflammation of the tonsillar pillar  1+ - tonsil barely visible outside the tonsillar pillar  2+ - tonsils between the tonsillar pillar and uvula  3+ - tonsils that touch the uvula  4+ - tonsils that touch each other (anti-inflammatory IM- corticosteroids; Epi)  No surgery if there is still infection; infection must be treated first Signs and symptoms  Sore throat  Dysphagia  Jaw and throat tenderness  Hoarseness/voice muffling  Aphonia  Fever, chills, headache  Enlarged cervical lymph node  Foul smelling breath  Otalgia (earache)  Difficulty hearing Management: Tonsillectomy (contraindicated to leukemic clients, not recommended for 2-3 y/o)

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Intervention:  Warm saline gargles, lozenges  Soft to liquid diet  Analgesic, antipyretic, but not aspirin (aspirin is contraindicated in all surgery)  Make sure to treat the infection before surgery Post Op care:  Prone head turned on one side  NPO till fully awake  Obs for signs of complication (#1 airway, #2 bleeding-frequent swallowing  Ice collar  Analgesic  Minimize crying  Discourage coughing, nose blowing  Emergency equipment (Epi, steroids, Tracheostomy set, O2)  Discharge instructions (Diet, medication, Oral care, hygiene, OP check up, pain management, Bayabas-antimicrobial properties) 5 S of Tonsillitis  Streptococcus  Sore throat  Surgery  Soft to liquid  Semi prone 4. Esophageal Atresia  Usually low birth weight  May be associated with other anomalies  Choanal atresia: a nasopharyngeal anomaly that exhibits as a lack of an opening between one or both of the nasal passages and the nasopharynx  Aspiration-Nutrition  Tracheoesophageal anomalies - Absence of esophagus - Atresia of the esophagus without a tracheal fistula - Tracheoesophageal fistula - The most common type of anomaly us proximal esophageal atresia combines with distal Tracheoesophageal fistula - Chalasia – an incompetent cardiac sphincter  Clinical Findings - Excessive drooling - Excessive mucus in nasopharynx causing cyanosis which is easily reversed by suctioning - Chinking sneezing and coughing during feeding with regurgitation of formula - Inability to pass a catheter in to the stomach  Therapeutic Intervention - Surgical repair can be done in one procedure or several, depending on infants condition and defect

Nursing Care o Assessment - Three C’s of Tracheoesophageal fistula  Coughing  Choking  Cyanosis - Monitor for sign of respi distress - Obs parent/infant interaction  Nursing Dx - Ineffective airway clearance related to physical defect - Anxiety related to inability to swallow, surgical pain - Risk for disorganized infant behavior related to pain and discomfort and need to restrict oral intake - Risk for injury related to surgical procedure - Altered family processes related to having an infant with disability - Impaired swallowing related to physical defect  Planning/Implementation o Postoperative Nursing care - Frequently suction mouth and pharynx o Provide high humidity to liquefy thick secretions - Stimulate crying and change of position to prevent pneumonia - Perform proper care of chest tubes if used - Maintain nutrition by oral, parenteral or gastrosomy method - Provide a pacifier if oral feedings are contraindicated or before a tube feeding - Provide comfort and physical contact, because hospital stay is usually long  Evaluation/Outcomes - Airway is maintained and infant does not aspirate secretions - Infant receives adequate calories for G and D - The surgical site is not injured - Infant rests calmly - Family demonstrates ability to care for infant - Infant alert when awake - Family able to list potential complications 5. Epiglottitis  Acute, severe, obstructive, and rapidly progressing inflammation of the epiglottis; requires immediate emergency care  Non contagious  Causative agent: Streptococcus, HiB (Haemophilus influenzae, type B)  S/S: - Abrupt in onset - Sore throat - Dysphagia - High fever - Muffled voice

- Inspiratory stridor - Drooling - TRIPOD Position (to max O2 intake) –definitive manifestation of Epiglottitis  Dx Tests: Neck x-ray, visual examination  Management 1. During-ray keep in upright position 2. Never use restraints/tongue blade 3. Administer antibiotic (parenteral) 4. Corticosteroids 5. Position (tripod/upright) 6. Intubation set at bed side (O2 set)  Threatening  Rapid Progression  Ineffective Airway  Pneumoniae Strep; HIB  Obtain Epi  Drooling, dysphagia, dyspnea 6. Hypertrophic Pyloric Stenosis  Obstruction at the pyloric sphincter by hypertrophy of the circular muscle of the pylorus  Etiology: Unknown  Incidence: Boys  S/S - Projectile Vomiting (containing gastric content) - Metabolic alkalosis (due to frequent vomiting); can be known through ABG - Weight loss/fails to gain weight - Palpable olive shaped mass (Right upper quadrant) - Visible peristaltic waves - Distended upper abdomen - Sign signs of dehy - Constipation  Tests: - UGI series (Upper GI Series) - Abdominal UTZ - ABG/Electrolyte Studies  Mgt. - Pyloromyotomy  Pre Op Care - NPO 4-6h and on IVF with glucose and electrolyte replacement - Daily weight - With gastric decompression and lavage (decrease abdominal pressure) - Protection from infection (antibiotic prophylaxis) - Position (Right side lying)  Post Op Care - NPO on IVF

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- Clear liquid containing glucose and electrolyte (4-6h) - Position: HOB elevated - Skin care Stenosis Treatment (Pyloromyotomy) On Position (Right Sidelying) Metabolic alkalosis ABG, ABD UTZ, UGIS Care: NPO H20 with glucose and electrolytes

7. Intussusception  Invagination or telescoping of one portion of intestine into another  Etiology: Unknown  Incidence: Boys  S/S - Pain - Vomiting (stomach content + intestinal content + fecal matter) - Tender, distended abdomen - Currant jelly stool (fat + blood in the stool) - Palpable sausage mass (depends on the site of intussception  Dx Test - Ba Enema  Mgt 1. Ba enema 2. Laparotomy (exploratory, open the abdomen) Other Notes When do boys develop the ability to reproduce? 13.3 y/o

Does sugar make children hyperactive? No

Serotonin – makes the person Hyperactive How is IQ determined? Mental age divided by chronologic age time 100  130+ Very Superior  120-129 Superior  110-119 High Average  90-109 Average  80-89 Low Average

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70-79 Borderline 50-69 Mild Mental Retardation 35-49 Moderate Mental Retardation 20-34 severe Mental Retardation <20 profound Mental Retardation

What is the best predictor of a child’s eventual height Girls *(Father’s height-13) + (Mother’s height)+ / 2 Boys *(Mother’s height +13) +(father’s height)} /2

When can an infant taste? Taste is present at birth and infants demonstrate a definite preference for sweetness or plain water.

Should mothers with silicone implants breastfeed their infants? No

Must infant formulas be sterilized? As a rule no. the terminal sterilization of prepared formula by boiling the bottle for 25 minutes offers no advantage over simple cleansing pf the bottles and nipples in hot, soapy water and the subsequent use of sterilized.

When is masturbation is considered pathologic? Normal      Pathologic       Frequent compulsive no regard for privacy often preferred over activity or play produces genital discomfort involves penetration of genital orifices bizarre practice or rituals Occasional Discreet private Not preferred over other activity No physical signs and symptoms External stimulation only

What is the avg daily sleep requirement by age?    Birth – 16h 6 mos. – 14.5 12 mos. – 13.5

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2 yrs – 13 4 yrs – 11.5 6 yrs – 9.5 12 yrs – 8.5 18 yrs – 5

What is the difference between night terrors ad nightmares?   Nightmare – frightening dreams occur during REM; last half of the night Night terrors – occur in REM; 1st third of sleep; cannot be recalled

At what age do sleepwalking and sleeptalking occur?   Sleepwalking – ages 5-10 Sleeptalking – end usually before the age of 15

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DM Type 2 Hereditary DM Type 1 Congenital Surgery – bleeding and infection common problems Read – tonsillitis, esophageal disorders, pyloric stenosis, GERD, intussusceptions, Hirschsprung’s, imperforate anus Tonsillitis-NPO-Cold Water (clear liquid) Parenteral – before and after surg – tube feeding – oral feeding (Tracheoesophageal atresia) Fever 37.5-38-5 Hyperthermia 38.5-39.5 Hyperpyrexia 40+ 20 lbs. – child can sit in the front seat Gavage – introducing Lavage – getting something Urinary urgency – busisaw End to end anastomosis

8. Hirschsprung’s Disease - Congenital absence of parasympathetic nerve ganglion cells in the distal bowel - Congenital aganglionic megacolon - Etiology: Unknown; familial tendency - Incidence: Boys - S/S: - Failure to pass Meconium - Vomitus - Constipation - Foul breath - Abdominal distention - Ribbon like stool - Anorexia - Anal absorption (H2O) - Palpable fecal mass  Tests: X-ray, Ba Enema, Rectal Exam, Rectal Biopsy  Mgt: 1. Colostomy (temporary) 2. Abdominal Perineal Pull Through  Pre-Op Mgt: 1. Provide adequate Nutri 2. Saline enemas 3. Antibiotics 4. Measure abdominal girth daily 5. Position (on unaffected side)  Post-Op: stoma care/prevent infection, change position frequently Intussusception Tender, distended abdomen Enema, Ba Laparotomy Etiology Stool monitoring; steatorrhea Currant jelly stool Obs for vomiting of fecal matter and bile products Palpable sausage, shaped mass, pain-colicky (depend on the affected part) Intact suture Congenital Hirschsprung’s Congenital aganglionic megacolon Rectal biopsy and exam (definitive test) Abdominal perineal pull through and colostomy Palpable fecal mass Stoma care

9. Imperforate Anus  Most common intestinal anomaly  Failure if the membrane separating the rectum from the anus to absorb during the 8th week of fetal life. Fistulas within the vagina, urinary tract, or scrotum are common Classification A. Low Anomalies  Puborectalis muscle, internal and external sphincter present and well developed with normal function B. Intermediate Anomalies  Rectum at or below Puborectalis muscle  Rectal opening anterior in perineum  May be persistent opening to the genitourinary tract C. High Anomalies  Rectum ends above Puborectalis muscle  Absence on internal and external sphincters Clinical Findings  Failure to pass Meconium stool  Abdominal distention Therapeutic Interventions  Immediate surgical correction unless fistula is present  Possible colostomy with multistage surgical repair Nursing Care  Assessment - Rectum for opening - Passage of Meconium (best evidence for perforation) - Abdomen for distention  Analysis/Nursing Dx - Risk for disorganized infant behavior related to pain and discomfort and need to restrict oral intake - Altered family processes related to having a child with a defect - Risk for injury related to inability to evacuate rectum - Altered nutrition: less than body requirements related to inability to feed  Evaluation and Outcome - Child achieves bowel function - Child consumes sufficient calories for G and D - Family demonstrates ability to care for a child 10. Hernias - Protrusions of any part of the internal organs through the structures enclosing them 1. Diaphragmatic  Is a protrusion of an abdominal organ (usually the stomach or intestine) though a defect in the diaphragm into the chest cavity; Diaphragmatic, Umbilical, Inguinal Hernia - Usually occurs on the left side causing cardiac displacement to the right side of the chests and collapsed of the left lung - Early in the IU life, the chest and the abdominal cavity are one. At approx 8 wk of preg, the diaphragm forms to divide them

Failure of the pleuroperitoneal cavity to close completely during embryonic life High mortality; a surgical emergency in immediate NB care Clinical Findings - Severe respiratory difficulty with cyanosis - Relatively large chest, especially on the affected side - Failure of affected side of the side (usually on the left side) of the chest to expand during respi, absence of breath sounds (collapsed lungs) - Relatively small abdomen  Therapeutic Interventions o Respiratory support - Position head and thorax higher than abdomen and feet - Nasogastric suction for stomach decompression (lavage) o Surgical Repair  Nursing Care o Assessment - Monitor respi status - Obs for irritability/pain on inc distress o Analysis/Nsg Dx - Activity intolerance related to: - Generalized weakness - Hypoxia - Ineffective breathing pattern related to interference with diaphragmatic breathing - Altered family processes related to situational crisis (critically ill infant) - Risk for infection related ton incision and inc secretions - Altered nutrition: less than body req related to lack of energy to suck and chew - Pain related to difficulty in breathing o Planning/implementation - Support parents because of the critical nature of the condition - Gastric suction to remove secretions and swallowed air from the stomach and intestine before and after surgery o Preoperatively position the infant on the affected side with the head elevated to allow the expansion of the unaffected side o Post-operatively feed the infant by gavage to dec chances of swallowing air o Titrate pain medicates to promote relief o Evaluation/Outcomes - Infant is able to maintain oxygenation - Infant will not show evidence of pain/irritability - Parents should be able to verbalize feelings about serious nature of infant’s condition 2. Umbilical Hernia  a protrusion of a apportion of the intestine through the umbilical ring, muscle and fascia surrounding the umbilical cord Assessment  Bulging protrusion under the skin at the umbilical and becomes more evident when the infant reaches one y/o

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The structure is generally 1-2 cm (1/2-1 inch) in diameter but may maybe as big as orange when children cry or strain Therapeutic Management  If the defect is more than 2 cm. Surgery is indicated to prevent intestinal obstruction or bowel strangulation. Surgery is done when the child is 1-2 y/o  If the size is less than 2cm. Closure of herniated part will occur spontaneously as baby grows and no surgical repair is done 3. Inguinal Hernia  A protrusion or a section of the bowel into inguinal ring. It can be direct or indirect - Direct inguinal hernia – the hernia passes through the posterior inguinal wall, protrudes directly through the transverse fascia of the canal and come out at the external ring (an are known as Hesselbachs’s triangle) - Indirect inguinal hernia – causes the abdominal viscera to protrude though the inguinal ring and follow the spermatic cord (males) and round ligament (females)  An infant, an inguinal are, it exists in undescended testicle or Hydrocele. In the 7 th month of pregnancy, the testicle normally descends into the scrotum, preceded by the peritoneal sac. If the sac closes improperly, it leaves an opening through which the intestine can slip Assessment  There’s a lump to appear over the herniated are when the patient stands or strains. Then, it disappears when the patient is supine  Tension on the herniated contents may cause sharp, steady pain in the groin  X-rays and sonograms may help confirm the condition Therapeutic Management  If the hernia is reductible, the pain maybe temporarily relieved by pushing the hernia back into place  Herniorrhaphy – a surg procedure which replaces the contents of the hernia sac into the abdominal cavity and close the opening  Hernioplasty – the repair of the hernia which reinforces the weakened are with a fascia, a nylon or wire  A strangulated or necrotic hernia requires bowel resection which necessities a temporary colostomy

Cardiovascular System Defects  Exact cause: Unknown  Etiologic Factors:  Maternal viral infections (rubella)  Poor nutrition  Advanced maternal age 35 years and above  Chromosomal disorders Trisomy 13 (Patau’s), Trisomy 18 (Edward’s), Trisomy 21 (Down’s)  Maternal DM, heart defects  Alcoholism  Fetal Infections  Classification of Cardiac Defects 1. Acyanotic: shunt from left to right side  No abnormal communication between pulmonary and systemic circulation  If a connection within the heart chambers exist pressure forces blood from the arterial (left) to the venous (right) side of the heart where it is reoxygenated 2. Cyanotic: shunt from the right side to the left side of the heart  Abnormal connection bet pulmonary ad systemic circ  Venous/unoxy blood enters systemic circ  Polycythemia – compensation for inadequate O2 supply General Assessment Findings Acyanotic Heart Defects Cyanotic Heart defects Murmurs Cyanosis Dyspnea, easy fatigability Squatting – Dx clue Difficult feeding Clubbing of fingers/toes Retarded Physical growth Retarded physical growth Frequent Respi Tract Freq respi tract Infection Infections Tachycardia, tachypnea Tachycardia, tachypnea No cyanosis Poor/diff feeding With CHF (most common complication)-cyanosis Polycythemia RDS Complications: cerebral abscess (most common); Congested Cough left sided heart failure; brain thrombosis, Diaphoresis infarctions

A. Acyanotic Defects  Ventricular Septal Defects (VSD) – most common  Abnormal opening between 2 ventricles  Severity depends on the size of the opening  Effect: O2nated blood go back to right ventricle, back to the pulmonary artery to the lungs for reoxygenation – right ventricular hypertrophy  Types: a) Low septum – small defect; may close spontaneously, no tx needed b) High septum – large defect, no spontaneous closure; plastic patch to close defect is applied early to prevent pulmonary HTN

 S/S: bradycardia, slowing growth pattern  Atrial Septal Defect (ASD)  Opening between right and left atria  Effect: same with VSD but backflow occurs in atria  S/S: dyspnea upon mild exertion; murmur hear high on the chest, with fixed splitting on the second heart sound; prone to resp. infxn  Patent Ductus Arteriosus  Failure of the fetal connection between aorta and pulmonary artery to close  Blood is shunted from the aorta back to the pulmonary artery – pulmonary HPN, cardiomegaly  Most common anomaly in children born to moms exposed to rubella (German measles)  S/S: continuous machinery-like murmur at L intraclavicular area (Pathognomonic sign)  Prominent radial pulses in NB  Limited growth and physical activity  On ECG/CXR – left ventricular enlargement  Therapeutic Mgt: closure of the opening; in critically ill NB, pharmacologic closure may be attempted with prostaglandin inhibitor (Indomethacin)  Prognosis without surgery: life expectancy is shortened due to bacterial endocarditis  Coarctation of Aorta (COA) – narrowing along the aorta usually below the level of the aorctic arch  Effect: O2 blood cannot pass thru narrowed aorta, diminished blood flow to body – left Vent hypertrophy  S/S: absent femoral pulse (Pathognomonic sign)  Leg pains on exertion –dec systemic circ  Muscle spasms – below stricture  Cooler lower extremities  BP higher in upper extremities; normally systolic pressure in lower ext is 20-40mmHg higher in upper ext – gravity  Bounding radial, carotid pulse  Headache, dizziness, epistaxis  Inc pressure in aorta above the defect causes left Vent hypertrophy  Tx: resection of the defect, anastomosis of aorctic ends B. Cyanotic Defects  Tetralogy of Fallot (ToF)  4 associated defects: Pulmonic Valve stenosis  Right ventricular hypertrophy  Overriding aorta, receiving blood from both ventricles; or an aorta arising from R ventricles  Ventricular septal defect. Usually high septum  Major physiologic finding: dec pulmonary blood flow  Hemodynamics: UnO2 blood from body parts empty to SVC/IVC and into the R atrium into the R ventricle via the tricuspid valve. BUT because of the very small pulmonary artery, very little O2 blood passes through so that blood accumulates in the R vent causing building up of pressure in that are (R ventricular hypertrophy). Because of the opening between R and L ventricles and inc pressure in the R ventricle, the unO2 blood will go though the ventricular septal defect

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S/S: related to the severity of the pulmonary stenosis 1. Cyanosis – measures 3-4 gm/100mL of deO2hemoglobin in the arterial blood General Considerations Cyanosis rarely present at birth, except in cases of pulmonary atresia usually manifested when child is 3-6 months old or when he is already active Inadequate tissue oxygenations results in anaerobic catabolism -> accumulation of lactic acid and other ketones dec the energy available for muscular activity Cyanosis tends to appear or be exaggerated by exercise, crying or straining and other activities that lower systemic vascular response On vigorous and persistent crying, cyanosis becomes intense (blue baby), sever DOB develops and infants faints due to cerebral hypoxia (hypoxic episodes/tet spells) 2. Clubbing of fingers and toes – mechanism is unknown. May be due to peripheral hypoxia that leads to compensatory shift of fluid form the intracellular spaces, edema and fibrous tissue proliferation – seen at 1y/o 3. Growth retardation 4. Dyspnea on exertion relieved by squatting -> this is of a Dx value - Exact mechanism is unknown. May be due to the rapid return of arterial saturation to its normal resting value during squatting 5. Polycythemia – compensatory mechanism to deliver as much O2 blood as possible to the body. However, Polycythemia predisposes the child to cerebral thrombosis and brain abscess, leading to CVA 6. Presence of right ventricular hypertrophy 7. Boot shaped configuration of the heart Dx: History and PE Angiocardiography – series of x-ray films showing the blood flow through the heart and great vessels, which are outlines by Radiopaque dye Cardiac catheterization – used to Dx extent of damage in the heart by determining the blood flow pattern of blood, O2 saturation, pressure in the cardiac chambers and vessels, stroke volume and CO Tx: 1. Conservative Adequate hydration – to prevent pulmonary emboli, brain abscess and cerebral thrombosis Prevention of recurrent infections – increases body’s need for O2 Alleviation of dyspnea by knee chest position or squatting O2 and morphine administration – to provide rest and relieve anxiety Do not allow child to cry for long periods of time 2. Palliative tx procedure performed to inc pulmonary blood flow o Blalock-Taussig Procedure – left pulmonary artery is anastomosed to the left subclavian artery. Remember: BP is the left arm is absent because of the anastomosis – BP must be taken on the right arm 3. Corrective Repair – open heart surgery-> closure of the septal defect and resection of thee infundibular stenosis, possibly with a pericardial patch to enlarge the right ventricular outflow tract. Makes us of the heart-lung machine and employs hypothermia

 Transposition of the Great Vessels - Aorta (which is supposed to arise from the L side of the heart) arises from the R ventricle and pulmonary artery (which is supposed to arise from the Right side of the heart) arises from the L ventricle - Effect: Because the aorta is on the R side of the heart, the unO2 blood enters the aorta and delivered to the body parts. Because the pulmonary artery is in the L side, O2 blood will go back to the lungs for reoxygenation - Tx: 1. Palliative tx procedures Rashkind procedure – enlargement of an existing atrial defect during a cardiac catheterization (balloon septostomy) Pulmonary artery banding, if a ventricular septal defect is present -> to dec blood flow to the lungs and inc shunting of O2 blood intraventricularly to the aorta Pharmacologic dilation of a patent Ductus Arteriosus with use of prostaglandins (Prostin VR) Blalock-Hanlon operation: surgical creation of an ASD 2. Complete Repair Rastelli’s Procedure – closure of the ventricular septal defect resulting in physiologic normal circulation but would require revision as the child grows Mustard or Senning Procedure – removing the entire atrial septum and creating a new one from the existing pericardium or prosthesis may be used to baffle blood for more oxygenation Jatene Operation – transposing the great vessels to their corrective anatomic placement with reimplantation of the coronary arteries Treatment Acyanotic VSD – Pulmonary banding (before 2y/o); direct closure/patch work (3-4yrs); no surgery for small defects; treatment of CSF ASD – spontaneous closure for small; direct suture/patch work (3-4yrs) PDA – Indomethacin (Tx for Preterms); Division of PDA (1-2 yrs) Coarctation – excision of coarctated portion with end to end anastomosis @ 4 yrs; Pulmonic Stenosis –surgical valvulotomy Cyanotic Tetralogy of Fallot – Blalock Taussig Shunt (preferred); anstomosing right or left subclavian artery with corresponding pulmonary artery to inc pulmonary blood flow (palliative only) Transposition of Great vessels – Blalock-Hanlon procedure: surgical creation of ASD Rashkind balloon catheter insertion to create ASD during cardiac catheterization Tricuspid atresia – same as tetralogy

Principles of Care  Maintenance of respi and oxygenation  Warmth  Promotion of rest  Prevention of infection  Maintenance of nutrition and hydration  Meds: antibiotics, diuretics, digitalis o Check Cr before giving digitalis and withhold ,refer if: o Give digitalis regularly not with milk or drugs o Obs for digitalis toxicity signs: N and V, anorexia, diarrhea, arrhythmias, headache, dizziness, xanthopsia (yellow vision) o Preparation of child/family for procedures and surg as required. Psychologic support Other Notes  Clubbed fingers: sign lack of O2nation  Hypoxemia – lack of O2 in blood  Hypoxia – lack of O2 in tissue  VSD – seen is cyanotic and Acyanotic defect  3-5 y/o – adequate heart size  Cardiac catheterization  ASD – RV  VSD – RV  PDA – PA  Knee chest – tetralogy  Radiopaque dye – ask if there is allergy to seafood; allergy

CNS Defects Spina Bifida – protrusion of meninges and sometimes spinal cord; infection and motor deficit (if spinal cord involvement) Cause: Unknown Contributing Factors 1. Late maternal age 2. Poor nutrition/Chemical 3. Genetic Influence Dx Test 1. Amniocentesis (IU life) – if determined in IU life, CS delivery is recommended; increased AFP in amniotic fluid. 2. UTZ – can be used to differentiate if spina bifida myelomeningocele or 3. Myelogram – can be used to know if spinal cord is involved 4. Transillumination test (pass through, Meningocele, if not myelomeningocele) 2 Types 1. Spina Bifida Occulta – No problems (no spinal cord involvement); port whine stain in the lower back with patches of hair; evaluate patient for other congenital defect-hydrocephalus, CHD, Trisomy 2. Spina Bifida Cystica – cyst like formation Forms of SB Cystica 1. Meningocele – meninges and sac containing amniotic fluid; biggest problem is infection because of the exposed meninges 2. Meningomylocele – meninges and spinal cord is involved-motor deficit; infection is priority number 1; motor deficit is irreversible Motor Dysfunctions in MM T6-T12 Complete flaccidity; Paralysis (absence of movement and sensation) of lower extremities L1-L2 Paraplegia (absence of movement but with sensation), flaccidity, hip flexion L3-L4 Hip dislocation S1-S2 Preservation of some foot and ankle movement S3 No motor impairment Management 1. Prevent sac damage a. Prevent drying b. Inspect for leaks, abrasion and irritation c. Cleanse carefully d. Position (Prone) HOB elevated, head turned on one side; cover with sterile gauze soaked in sterile water e. No clothing or diaper 2. Provide meticulous care 3. ROM exercises (Passive for paraplegia) 4. MIO

Surgery: Demyelization Post Op Care: 1. Monitor V/S (for post operative complications) 2. Position (Prone) – baby cannot be carried Hydrocephalus CSF – stored cerebral ventricles, absorbed in subarachnoid space, produced in choroid plexus Etiology: 1. Tumor – obstruction 2. Congenital – born with it 3. Associated with other defect 4. Secondary to an infection Sunset eyes – of Dx value probably due to inc intracranial pressure or inc stretching of forehead Ventriculoperitoneal shunting – draining the CSF to the peritoneum Crack pot sound 2 Types: 1. Communicating – non obstructive hydrocephalus; CSF can flow from cerebral ventricle to subarachnoid space; over production of CSF in the chorionic villi or malabsorption in the subarachnoid space; CONGENITAL; shunting is tx 2. Non-communicating – obstructive hydrocephalus; tumor, trauma, infection; shunting is only palliative S/S Early 1. Head enlargement 2. Bulging fontanel 3. Separated sutures 4. Dilated scalp veins (Do not use as IV Site) 5. Irritability (Due to inc ICP) 6. Muscle spasticity (Due to inc ICP) Late (Late sign will become early sign in clients with closed sutures) 1. Frontal enlargement 2. Sunset eyes/setting sun 3. Lethargy, excessive sleepiness 4. Brief, shrill, high pitched cry (cat like cry) 5. Headache 6. Vision changes 7. Ataxia 8. Vomiting Dx Tests: 1. Transillumination Test 2. CT scan (anatomy)/MRI (physiology) MRI-can measure ICP 3. Skull X-ray (will not macrocranium)

4. Spinal Tap/LP – done to know if there is involvement of infection-low glucose level, inc in protein (waste product of pathogen in protein), Culture and Sensitivity; broad spectrum antibiotics is recommended of C/S is not yet done Implementation 1. Support the head very well 2. Provide adequate nutrition 3. Prepare shunting Post Op Care 1. Flat in bedx24h in unoperative site 2. Observe sign s for inc ICP 3. NPOx24h 4. MIO carefully 5. Administer drug as ordered: Non-narcotic analgesic; Antibiotics-IV for infection 6. Observe signs of abdominal distention 7. Avoid/observe sign for sign of inc ICP 8. Provide skin care 9. Teachings Meningitis – complication of spina bifida and hydrocephalus; most of the time a secondary infection Causative Agent: Neisseria Meningitidis; 10-14 days incubation period; bacterial meningitis is harder to treat Predisposing Factors 1. URTI 2. Otitis Media 3. Recent neuro procedure 4. Head trauma 5. Spina bifida Pathophysiology Predisposing factors Vascular dissemination Spread to meninges Into the CSF Organism invade brain tissue Inflammation Brain Becomes Edematous Purulent Exudates Pressure on pituitary gland

Blocks CSF flow Hydrocephalus

inc ADH production Oliguria/Edema

S/S 1. Fever 2. Sever sudden headache 3. Mental changes 4. Vomiting 5. Full tense bulging fontanel 6. Frequent seizure accompanies buy high pitched cry 7. Nuchal rigidity (opisthotonus; + kernigs sign; + Brudzinski); (over extension of neck, or arching of body – opisthotonus) 8. Opisthotonus 9. + Kernigs Sign (kernigs: resistance when flexing knees without flexion of neck; brudzinski’s neck sign = flexion of neck when knees are flexed) 10. + Brudzinski 11. Rashes 12. Photophobia (causes pupil dilatation d/t increased ICP) 13. Hyperactive reflexes Dx Tests: 1. Lumbar puncture and CSF culture Glucose Protein NB – 34-119mg/dl 20-170mg/dl Child – 40-80mg/dl 5-40mg/dl Management 1. Antibiotic 2. Anticonvulsant Nursing Intervention 1. Institute respiratory prec (or universal prec) 2. Take isolation precaution 3. Maintain seizure prec 4. HOB slightly elevated 5. Siblings of ill child may be prescribed with antibiotic Other Disorders Kawasaki – acute systemic vasculitis Etiology: idiopathic; Asian men below 5 y/o (autoimmune or infection caused by S. aureus) S/S:  Fever for at least 5-10 days  Irritability  Changes in peripheral extremities  Bilateral painless conjunctivitis  Changes in lips and oral cavity

 Acute cervical lymph adenopathy  Arthritis  Pink eye  Oral mucosal change  Enlarged lymph nodes  Patchy rash  Peeling skin (desquamation)  Coronary aneurysm (2D echocardiogram)  Pink tongue (Strawberry tongue) Tests:  CBC – inc  ESR – Erythrocyte sedimentation rate (inc)  Echocardiogram Management 1. Administer  Gamma globulin (Given within 10 days)  Aspirin (Anticoagulant and helps in juvenile arthritis) 2. Cool/soft clothes 3. Oral hygiene 4. Passive ROM 5. Place in quiet environment (non stimulating) 6. MIO/diet (soft diet) Hydrocephalus Over accumulation of CSF Need for CT scan and MRI Enlargement of Head Shunting High level of ICP Etiology: congenital and acquired Acetaminophen and antibiotics Dilated scalp veins Meningitis Mannitol (osmotic diuretic, also for hydrocephalus-dec ICP) Acquired Nasopharyngeal Inc ICP Apply respi prec and isolation CSF culture and LP Spina Bifida Paraplegia/protrusion

Incomplete closure of the vertebrae/idiopathic Meningocele/Myelomeningocele Prevent sac from damage Lumbosacral area Exercise: PROM Spina Bifida occulta/cystica Dx Tests CT scan Amniocentesis MRI Myelogram UTZ Transillumination test LP Kawasaki Mucocutaneous lymph node syndrome Idiopathic Test: ESR, CBC, 3D Echo S. Aureus Under 5 Boys: Asian IV IgG Strawberry tongue Hygiene Intake and output measured Altered body temp Celiac Disease/Celiac Sprue - Also known as gluten-induced enteropathy or non-tropical sprue - Is a chronic intestinal malabsorption and inability to digest gluten, a protein found mostly in wheat or grains – rye, oats, barley, wheat - 4 months earliest Dx – solid foods - Is a mucosal disease of the GIT that is believed to be autoimmune in etiology - Cause is unknown; current studies indicate that CD is strongly associated with a group of genes on chromosome 6 (thus considered a genetic disease). These genes are involved in the regulation of the body’s immune response to gluten protein fractions. - Gluten –> mixture of individual proteins, classified in 2 groups (Prolamines and glutelins). The prolamine, GLIADIN is said to be the most troublesome component of gluten. Gliadin antibodies are commonly found in the immune complexes associated with CD - When children with CD ingest gluten, their immune system responds by damaging the small intestine. Specifically, the villi on the lining of the SI are lost. The loss of functioning villi limits the ability of the SI to absorb nutrients thus adversely affecting all body systems

Clinical Manifestations 1. Progressive malnutrition – muscle wasting, anorexia, distended abdomen (due to bulky stools), deficiency in vitamins ADEK (due to non-absorption of fat) 2. Secondary deficiencies – anemia and rickets 3. Steatorrhea –> fatty, foul, frothy, bulky stools 4. Celiac crisis –severe episodes of dehy and acidosis from diarrhea Therapeutic Management 1. Gluten-free diet for life – can lead to GI carcinoma if DC into adulthood 2. High in Cal and proteins 3. Low fat 4. Small, frequent feeding with adequate fluids 5. Vitamin supplement, all in water-soluble form 6. Supplemental iron (as early as 4months) and folate to correct anemia Nursing Dx: 1. Risk for injury r/t knowledge deficit (diet and food composition) 2. Altered Nutrition: LBR r/t impaired intestinal absorption Nursing Care:  Protect child from infection  Teach parents dietary restrictions  Explain need for frequent follow-up supervision, home visit Allowed Foods Amaranth Arrowroot Buckwheat Cassava Corn Flax Indian Rice grass Job’s tears Legumes Millet Nuts Potatoes Rice Wheat Sago Cystic Fibrosis - An autosomal recessive disorder affecting the exocrine glands or perhaps all epithelial surfaces - Most common serious pulmonary and genetic diseases of children

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Disease can be fatal in early life but as many as 50% of children now live to be more than 30y/o. Full life expectancy is possible due to availability of lung transplants as well as early detection of gene disorder through obstetrical procedures done during the prenatal period. - Multi system disorder (GIT and Respi) 3 Organs that are most consistently affected 1. Eccrine sweat gland, produces excessively salty sweat; Elevation in sweat electrolytes; Sweat chloride tests – definitive test, >60meq/L 2. Lungs, in which COPD invariably develops and is usually the cause of mortality; inc viscous mucus in trachea, bronchi, bronchioles leading to emphysema, infection 3. Pancreas – becomes fibrotic, fails to produce adequate bicarbonate ion and water in nearly all patients, and produces inadequate digestive enzymes in most, giving rise to pancreatic insufficiency. Late complication – diabetes 4. Liver – possible cirrhosis form biliary obstruction, malnutrition; portal HPN lead to esophageal varices 5. Repro tracts, intestines, sinuses, and salivary glands are also regularly affected. In the sweat gland and in the airways, passive chloride permeability is reduced, accounting for the salty sweat and probably contributing to the dehydrated mucus in the airways. In organs apart from the sweat glands Clinical Manifestations Early Manifestations during Infancy - Meconium ileus at birth (15%) - Failure to regain normal 10% weight loss at birth - Presence of cough or wheezing during the first 6 months of age, there may be clubbing of fingers, barrel chest cyanosis, distended neck veins - Elevation in sweat electrolytes - Pancreas become fibrotic with a dec in the production of pancreatic enzymes  Lipase – causes steatorrhea  Trypsin – causes inc N in stool  Amylase – polysaccharides - Rectal prolapse - Sweat glands: high electrolyte content of Na and CL (3-5x higher than normal) - Respiratory system: inc viscous mucus in the trachea, bronchi, and bronchioles –> may cause emphysema or infection - Liver: possible cirrhosis - Sexual organs: infertility - Cardiac enlargement, particularly right ventricular hypertrophy (cor pulmonale) Therapeutic Interventions: Pulmonary problems – humidified O2; chest physiotherapy – use of bronchodilators and antibiotics through aerosol therapy - Respiratory hygiene – freq mouth care and tooth brushing - Adequate rest and comfort – prevent exhaustion GI Problems - Pancreatic enzyme supplements - Balanced nutritional intake –> High calorie, high protein, moderate diet Nsg Dx - Activity intolerance r/t imbalance between oxygen supply and demand

- Ineffective airway clearance r/t secretion of thick, tenacious mucus - Ineffective breathing pattern r/t mechanical tracheobronchial obstructions Nsg Care  Prevent RTI - Postural drainage, percussion, vibrating between feedings - Aerosol therapy - Use of expectorants and antibiotics - Avoid antitussives, antihistamines   Promote Optimal Nutrition Replacement of pancreatic enzymes given with cold food in the middle of the meal Replacement of fat soluble vitamins in water miscible form High CHON diet of easily digested food, normal fat, high calorie Promote mobility and activity Encourage activity and regular exercise; help child regulate activity to own tolerance

Nephrotic Syndrome/ Nephrosis Abnormal, increased permeability of the glomerular basement membrane to plasma albumin, causing loss of protein in the urine - Cause is unknown - Possible cause  hypersensitivity to an antigen-antibody reaction and t-lymphocyte (autoimmune process)dysfunction - Peak incidence 2-7yo - Most often in boys - Classification 1. Minimal Change Nephrotic Syndrome (80% incidence) – little scarring occurs within the glomerulus of the kidney; good prognosis: children with this type respond well to therapy 2. Secondary NS – usually occurs after glomerular damage of known or presumed etiology Ex. In cases of sickle cell anemia, GN, systemic lupus erythematosus 3. Congenital NS – caused by autosomal recessive gene Rare and does not usually respond to therapy. Infant dies in the first or second year of life - Classis S/S of NS (referred to as James’ Schema) a. Proteinuria – 3.5gms or more / 24hrs – frothy or foamy tea-colored urine due to high amounts of protein b. Hypoalbuminemia – low serum albumin level – 3gms or less (NV – 3.5-5g/100ml) c. Hyperlipidemia – increased blood lipid level – 300mg% or more d. Edema – insidious onset – worsens during the day; when ABSENT prognosis is POOR - Periorbital edema Other S/S o Normotensive (BP within normal range or slightly lower) o Waxy pallor – pale stretched, taut (due to edema) o Tires easily, poor appetite

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Generalized dependent edema – extracellular fluid collection in cavities, legs and genitalia; ascites – DOB

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Laboratory Tests - 24-hr urine test – marked Proteinuria (+1-+4) – 15gms of CHON - ESR – elevated d/t inflammation of glomerular membrane - Renal biopsy – to determine scarring of the glomerular membrane - BUN creatinine - if high levels of waste product in blood – kidney damage BUN – 8.25mg/100ml Creatinine – 0.6-1.5mg/100ml Therapeutic management = Goal: reduce proteinuria, edema and keep child free from infection  Diet: high protein, extra K, “no added salt” diet (some would require moderate Na restriction)  Corticosteroid therapy (Prednisone – drug of choice) – SE: prone to infection, moon faced, thus not done for long term Response to therapy usually within 7-21days - Dose is lowered or gradually discontinued when satisfactory response is noted, given until proteinuria is absent  Furosemide (Lasix) – given IV after albumin infusion to enhance dieresis and decrease the chance of fluid overload; given only when child responds poorly to corticosteroid, K supplement  Immunosuppressant therapy for children who do not respond to steroids or those who have frequent relapses - Cyclophosphamide (Cytoxan) – prevents relapse - Cyclosporine (Sandimmune); Chlorambucil (Leukeran) Other management IV albumin may be given – hypoalbuminemia - Kidney transplant/dialysis if renal failure develops - Nursing Considerations Continue to monitor fluid retention/excretion - Strict MIO record - Provide skin care - Application of diapers/weighing pads may be necessary - As much as possible limit exposing client to crowd-reverse isolation - Nursing diagnosis  Imbalanced nutrition: LBR r/t poor appetite  Risk for impaired skin integrity r/t edema  Risk for infection r/t chon fluid loss  Activity intolerance r/t fatigue  Knowledge deficit r/t chronic illness  Body image disturbance r/t change in appearance ***AIDS, SARS – isolation, cancer patients – reverse isolation *** UTI – most common infection in nephritic syndrome

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***nephritic – hematuria; nephritic – proteinuria; can occur at the same time Cerebral Palsy - Is a nonspecific term for a group of non-progressive neuromuscular disorders but persists throughout life causing disability or difficulty in controlling voluntary muscles - Caused by damage to some portion of the brain with associated sensory, intellectual, emotional or seizure disorders - Affects young children usually becoming evident before 3yo - Language deficit may be present Enuresis -

Repeated involuntary passage of urine past the age when a child should be expected to have attained bladder control - Normal bladder control 2-3 y/o (daytime) - 4 y/o (night time) Classification 1. Primary Enuresis – is when a child has never been dry at night or would not sleep dry without being taken to the toilet by another person or has some dry nights but continues to average at least 2 wet nights a week with no long period of dryness 2. Secondary Enuresis – occurs when a patient through an extended period of dryness and begins to experience night time wetting again. Secondary enuresis can be caused by stress - Cause: Primarily unknown - May have a genetic component – 75% of all children with enuresis have first degress relative who has or had the disorder - Stress may also be a factor - Dx procedures: 1. Sonogram – to rule out organic disease 2. Sickle cell test – to rule out sickle cell anemia 3. UA – to rule - rule out bacterial infection; to rule out defect in urine concentration 4. Protein and glucose level determination – to determine evidence of kidney problem Nursing Assessment - Older that 5 y/o with enuresis should be evaluated whether there is an organic cause - Assess whether there is a stress at home or at school - If the child wets only when he is engrossed in an interesting activity, he simply needs a reminder to empty his bladder - If the child has symptoms other than bedwetting like abdominal pain, burning, frequency – UTI - Some children have abnormal EEG patterns Therapeutic Management 1. Retention Control Training (RCT)/Bladder stretching exercises – child drinks fluid and delays urination as long as tolerated to stretch the bladder for the purpose of accommodating larger amounts of urine 2. Alarm bell that ring when the child wets at night 3. Use waking schedule treatment – to encourage the child to awaken at intervals to void 4. Limiting oral fld. Intake after dinner as long as a child is free of sickle cell anemia

5. Administration of ADH 6. Administration of an anticholinergic drug (Tofranil0 that inhibits urination: given an hour before bedtime 7. Frank discussion with child regarding what causes stress to help him cope better Hydrocele - Painless swelling of the scrotum caused by accumulation of fluid in the scrotal sac - Processus vaginalis – folds of tissue that precedes when a testis descends into the scrotum in utero, occasional fluid collects Types 1. Non communicating Hydrocele - Most commonly seen at birth - Residual peritoneal fluid is trapped within the o=lower segment of the processus vaginalis (tunica vaginalis) - There is no communication with the peritoneal ca S/S: - enlarged scrotum - Transillumination-glows - Dx procedures: prenatal Treatment 1. If Hydrocele is uncomplicated, the fluid will gradually be reabsorbed into the body and no treatment is necessary 2. If unresolved, repair must be done by one year of age 3. Sclerotherapy – injection of the drug to decrease fluid production 4. If it will form later in life due to inguinal hernia, hernia may be repaired for the Hydrocele to be reabsorbed Cryptorchidism - Undescended testacle - Real cause is unknown - Possible causes: Fibrous bands at the inguinal ring or inadequate length of spermatic vessles preventing descent - Testosterone production is lower than normal level Assessment: 1. Palpate testes when the child is standing or after a warm bath 2. If gender is unclear, karyotyping may be done to determine true gender Nursing Consideration - Early detection is necessary since it can Therapeutic Management - Because the testes sometimes descend spontaneously during the first year of life, tx is usually delayed for 6-12 months - Children may be given chorionic gonadotropin hormone – stimulates testicular descent - Orchiopexy – surgical

Febrile Seizure Disorders - Caused by elevation of temp - Usually occur in children between 6 months and 3 years of age - Affects 3-5% of children in this age group - AKA Benign febrile seizures - Classification: Simple seizure a. Brief b. Generalized Complex seizure a. Prolonged b. May have focal features Clinical Findings: associated with disease outside the CNS - Fever usually exceeds 102F (38.8C) Nursing Dx - Ineffective airway clearance General Seizure Prec - Protect child from injury: do not restrain; pad crib rails, do not use tongue blade - Place in side lying position to prevent aspiration - Obs an record the time of seizure. Duration and body parts involved - Suction the nasopahrynx and administer oxygen after the seizure as required - Obs degree of consciousness and behavior after the seizure - Provide rest Failure to Thrive (FTT) - Infants and children whose weight and sometime height fall below the 5 th percentile for their age. Persistent deviation from established growth curve Classification: - Organic (OFFT) – result of a physical cause (congenital head defects, UTI, neurologic lesions, GERD, microcephaly, cystic fibrosis, endocrine dysfunction, malabsorption syndrome) – often cause marasmus - Non-organic (NFTT) – caused by psychological factors (maternal and environmental Clinical Manifestation - Growth below the 5th percentile, emotional and developmental retardation; apathy, flaccid and unresponsive - History of difficulty of feeding (anorexia, pica), vomiting, sleep disturbance, and excessive irritability, poor hygiene - May exhibit difficult temperament pattern, withdrawn, difficulty in forming meaningful relationships - Poor hygiene, withdrawn behavior, and feeling problems - No fear of strangers (@ age when separate anxiety is normal), eye contact avoidance, stiff or flaccid posture, and minimal smiling, wide-eyed gaze - Vigilant of people @ a distance and then distressed when they come closer, continual scan of environment (radar gaze) - May be more interested in inanimate objects that people

Management of Care - Tx and management of care is directed at reversing the malnutrition and the underlying cause 1. Promote age – appropriate growth and development Assess the child’s present developmental development Provide adequate ad age-appropriate developmental stimulation Foster social interaction and touch 2. Foster appropriate parenting behaviors Nursing Dx - Altered growth and development r/t physiologic factors; physical neglect ; social neglect - Altered nutrition: LBR r/t feeding or eating disorders - Altered parenting r/t knowledge deficit

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