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Pediatrics Review - Emma Holliday
Ramahi Question with Verified Answers
What does the APGAR tell you? - Answer -General info about how the newborn tolerated labor (1min) and the newborn's response to resuscitation (5min) What does the APGAR not tell you? - Answer -What to do next (does not guide therapy) How the baby will turn out (does NOT predict neurologic outcome) PE: When assessing Moro on an LGA newborn, the right arm remains extended and medially rotated. - Answer -Erb-Duchenne C5-C6. (Klumpke is C7-C8 + T1) Refer if not better by 3- 6mo for neuroplasty PE: When palpating the clavicles on a LGA newborn, you feel crepitus and discontinuity on the left. - Answer -Clavicular Fracture. Will form a callus in 1wk. No tx needed. Can use figure of 8 splint. "Edema. Crosses suture lines." - Answer -Caput succedaneum
"Fluctuance. Doesn't cross suture lines." - Answer - Cephalohematoma Appears in up to half of newborns carried to term, usually between day 2-5 after birth. Resolves within first two weeks of life, and frequently individual lesions will appear and disappear within minutes or hours. It is a benign condition thought to cause no discomfort to the baby - Answer -Erythema toxicum
- Answer -Strawberry Hemangioma
- Answer -Neonatal Acne "an area of alopecia with orange colored nodular skin" Remove before adolescence b/c it can undergo malignant degeneration. - Answer -Nevus Sebaceous "thick, yellow/white oily scale on an inflammatory base". What to do? Gently clean w/ mild shampoo - Answer -Seborrheic Dermatitis
feeding Jaundice. ↓feeding = dehydration = retain meconium & re- absorb deconjugated bili. 14 days old, bili @ 12, direct is 0.5. Baby regained birth weight, otherwise healthy. - Answer -Breast milk Jaundice. Breast milk has glucuronidase and de-conj bili. 1 day old, bili @ 14, direct is 0.5. Are you worried? Next best test? If positive? If negative? - Answer - Pathologic Jaundice = on 1st DOL, bili >12, d-bili >2, rate of rise >5/day. Test: Coombs Positive: Rh or ABO incompatability Negative: twin/twin or mom/fetus transfusion, IDM, spherocytosis, G6p-DH deficiency, etc. 7 days old. Dark urine, pale stool. Bili @ 12, dbili is
- LFTs also elevated. - Answer -Biliary atresia. Bile ducts cannot drain bile. Causes liver failure. Need surgery. Other causes of direct hyperbilirubinemia? - Answer -Always r/o sepsis! Galactosemia Hypothyroid Choledochal cyst CF
Biliary atresia (<2mo) Random inherited causes of indirect hyperbili? (2) - Answer -Gilbert - ↓glucoronyl transferase level Crigler-Najjar (type1) - total deficiency Random inherited causes of direct hyperbili (2) - Answer -Dubin-Johnson Sx - asx, black liver Rotor Sx - NO black liver Why do we care about hyperbilirubinemia? - Answer -Indirect bili can cross BBB, deposit in basal ganglia and brainstem nuclei and cause kernicterus. (esp if bili is >20) Treatment for hyperbilirubinemia - Answer - Phototherapy (Tbil>20)--> ionizes the uncoj bili so it can be excreted. Double volume exchange transfusion (Tbil>25) if that doesn't work. Baby is born w/ respiratory distress, scaphoid abdomen & this CXR.
- Biggest concern?
- Best treatment? - Answer -Diaphragmatic hernia Concern: Pulmonary hypoplasia Txt: If dx prenatally, plan delivery at @ place w/ ECMO. Let lungs mature 3-4 days then do surg
38 wk LGA infant born by C/S to an A2GDM has dyspnea/grunting
- Pathophys?
- Prognosis? - Answer -TTN Pathophys: Lung fluid not squeezed out, retained Prognosis: Usually minimal O2 needed. Self resolves in hrs to days 41 wk AGA infant was born after ROM yielded greenish- brown fluid. *Next best step? *Complications? - Answer -Meconium Aspiration Syndrome Next: intubate and suction before stimulation Complications: Pulmonary artery HTN, pneumonitis Defect lateral (usually R) of midline, no sac
- Assoc w/ other d/o?
- Complications? - Answer -Gastroschisis will see high maternal AFP Not usually associated w/ other d/o Complications: May be atretic or necrotic req removal. Short gut syndrome Defect in the midline. Covered by sac.
- Assoc w/ other disorders? - Answer -Omphalocele Assoc w/ Edwards & Patau Trisomies, Beckwith Wiedemann Syndrome = big baby w/ big tongue, ↓glc, ear pits Defect in the midline. No bowel present.
- Assoc w/ other d/o?
- Txt? - Answer -Umbilical hernia Assoc w/ congenital hypothyroidism. (also big tongue) Repair not needed unless persists past age 2 or 3yo 4wk old infant w/ non- bileous vomiting and palpable "olive"
- Metabolic complications?
- Txt? - Answer -Pyloric stenosis Complications: hypochloremic metabolic alkalosis Txt: immediate surgery referral for myotomy 2wk old infant w/ bileous vomiting. The pregnancy was complicated by poly- hydramnios.
- Assoc w/? - Answer -Intestinal Atresia (Or Annular Pancreas) Assoc w/ Down Syndrome (esp duodenal) 1 wk old baby w/ bileous vomiting, draws up his legs, has abd distension.
- Pathophys? - Answer -Malrotation and volvulus
- Where are they usually?
- Next best test?
- When to do surgery? - Answer -Cryptorchidism Assoc w/ prune belly syndrome
- Where are they usually? Inguinal canal
- Next best test? US if not palpable
- When to do surgery? If not descended by 1yr to avoid sterility/cancer Newborn male with urethral opening on the ventral surface.
- What do you NOT do? - Answer -Hypospadias DON'T circumcise! Foreskin is used in eventual repair Newborn child with ambiguous genitalia. One month later has vomiting & ↓Na ↑K and acidosis.
- Most common Cause?
- Definitive test?
- Tx? - Answer -Congenital Adrenal Hyperplasia
- Most common Cause? 21 Hydroxylase deficiency. (autosomal recessive)
- Definitive test? 17-OH progesterone before and after ACTH bolus
- Tx? Hydrocortisone and fludrocortisone (↑ doses in times of stress) Mothers with pre-existing diabetes (esp type 1)
- Prenatal care?
- Risks to fetus? - Answer -- Prenatal care? Control glc in the 1st trimester & take 4mg folate/day
- Risks to fetus? Placental insufficiency/IUGR, Congenital heart dz, NTD, Caudal regression syndrome, Small left colon syndrome Risks of infants born to mothers with gestational diabetes - Answer -- LGA. ↑risk of birth trauma (clavicle, Erbs), C/S & TTN
- Hypoglycemia. Why? Maternal hyperglycemia --> fetal hyperinsulinemia. Complications? Neonatal seizure (always check glc!). Treatment? Feed frequently if <40. IV dextrose if <
- Hypocalcemia. Neonatal seizure (always check Ca!)
- Polycythemia. Why? Big baby needs more O2, hypoxia-->↑EPO. Complications? Renal or splenic vein thromboses
- Jaundice. More RBCs to bread down. Risk for kernicterus
- RDS. ↑insulin interferes w/ cortisol surge prior to birth that normally stimulates lung maturation. Check L/S ratio > baby < 28 days old has a fever >100.4 - Answer - Sepsis until proven otherwise
Cataracts, deafness and heart defects (esp PDA, VSD), extramedullary hematopoeisis. Txt? - Answer -Rubella No tx. Microcephaly, periventricular calcifications, deafness, thrombo- cytopenia and petechiae. Txt? - Answer -CMV Tx w/ ganciclovir, but won't prevent MR Limb hypoplasia, cutaneous scars, cataracts, chorioretinits, cortical atrophy. Txt? - Answer - Congenital Varicella if mom infected 1st or 2nd trimester. If mom is exposed 5 days before - 2 days after delivery, baby gets VZIG. Neonatal conjunctivitis: DOL 1-3, red conjunctiva and tearing. - Answer -Chemical conjunctivitis caused by silver nitrate drops. Not common anymore b/c we use erythromycin. Neonatal conjunctivitis: DOL 3-5, bilateral purulent conjunctivitis can cause corneal ulceration. - Answer -Gonococcal conjunctivitis tx w/ topical erythromycin and IV 3rd gen ceph. Neonatal conjunctivitis: DOL 7-14, red conjunctiva w/ mucoid discharge & lid swelling - Answer -
Chlamydia conjunctivitis tx w/ oral erythromycin. Complication is chlamydial pneumonia --> cough, nasal drainage, scattered crackles + bilat infiltrates on CXR A newborn baby has decreased tone, oblique palpebral fissures, a simian crease, big tongue, white spots on his iris
- What can you tell his mother about his expected IQ? - Answer -Down's Syndrome He will likely have moderate MR. Speech, gross and fine motor skill delay Common medical complications of Down's Syndrome? - Answer -- Heart? VSD, endocardial cushion defects
- GI? Hirschsprung's, intestinal atresia, imperforate anus, annular pancreas
- Endocrine? Hypothyroidism
- Msk? Atlanto-axial instability
- Neuro? Increased risk of Alzheimer's by 30-35. (APP is on Chr21)
- Cancer? 10x increased risk of ALL Omphalocele, rocker-bottom feet/ hammer toe, microcephaly and clenched hand, multiple others. - Answer -Edward's syndrome (Trisomy 18)
injurious behavior. - Answer -Smith Magenis Syndrome Deletion on Chr Hypotonia, hypogonadism, hyperphagia, skin picking, agression. - Answer -Prader-Willi Syndrome Deletion on paternal Chr Seizures, strabismus, sociable w/ episodic laughter.
- Answer -Angelman Syndrome Deletion on maternal Chr15. Elfin-appearance, friendly, increased empathy and verbal reasoning ability. - Answer -Williams Syndrome Deletion on Chr7. IUGR, hypertonia, distinctive facies, limb malformation, self-injurious behavior, hyperactive. - Answer -Cornelia de Lange Microcephaly, smooth philtrum, thin upper lip, ADHD-like behavior. Most common cause of mental retardation. - Answer -Fetal Alcohol Syndrome Most common type of MR in boys, Macrocephaly, macro- orchidism, large ears. - Answer -Fragile X Syndrome
CGG repeats on the X-chr w/ anticipation. Autosomal dominant, or assoc w/ advanced paternal age. Short palpebral fissures, white forelock and deafness. - Answer -Waardenburg Syndrome 2 y/o M w/ multiple ear infxns, diarrheal episodes & pneumonias. No tonsils seen on exam.
- Labs? - Answer -Bruton agammaglobulinemia
- x-linked
- infx start @ 6-9mo
- Absence of B cells on flow cytometry
- low levels of all Igs 17 y/o F with decreased levels of IgG, IgM, IgE, and IgA but normal numbers of B cells. - Answer - Combined variable immune deficiency. (acquired) Complication of Combined variable immune deficiency? - Answer -Increased lymphoid tissue--> increased risk for lymphoma Most common B-cell defect. Recurrent URIs, diarrhea. - Answer -Selective IgA deficiency Complication of Selective IgA deficiency? - Answer - Anaphylaxis reaction if given blood containing IgA
18mo M baby w/ severe ezcema, petechiae, and recurrent ear infxns. - Answer -Wisckott-Aldrich Syndrome. Often present w/ prolonged bleeding after circumcision. Low IgM, high IgA and IgE, slightly low IgG. Newborns lose 10% of birth weight in 1st week. Why? Should regain BW by? Should double weight by? Should triple weight by? - Answer -Diuresis of extravascular fluid
- Should regain birth weight by? 2wk
- Should double weight by? 6mo
- Should triple weight by? 1yr Newborns increase 50% of length by? Double length by? - Answer -increases 50% by 1 year doubles by 5 years Contraindications to breastfeeding. - Answer - Galactosemia, PKU, HIV, HSV on the breast, chemo, Li, Iodide, alcohol.
Nutritional characteristics of Breast milk vs. Formula
- Answer -Breast milk is
- whey dominant
- more lactose
- more LCFA
- less Fe but its better absorbed 14 y/o boy, always been below 5% in height. Parents are tall & were "late bloomers". Bone age < Real age. - Answer -Constitutional Growth Delay Child is likely to have normal final adult height. 14 y/o boy, always been below 5% in height. Father is 5'2" and mom is 4'10" Bone age = Real age. - Answer -Familial Short Stature 14 y/o boy, 50% in height, 97% for weight. - Answer -Obesity Bone age > Real age. Bone age > Real age. - Answer -Obesity Precocious puberty CAH Hyperthyroidism