UT Peds Flashcards
what is: nevus sebaceous
newborn –> area of alopecia w orange, nodular skin
nevus sebaceous: tx
risk of malig degen –> remv b4 adol
PKU: ssx
1st few mo:
- MR
- vomit
- sz
- athetosis
- dev delay
- fair hair/eye/skin
- musty odor
galactosemia: pathophys
defect G1p-uridyltransferase –> G1p accumulate –> damage kidney, liver, brain
galactosemia: ssx
- MR
- direct hyperbili –> jaundice
- hypogly
- cataract
- sz
galactosemia: comp
E coli sepsis
galactosemia: tx
no lactose
neonatal jaundice: how know is pathologic?
- 1st day of life
- bili >12
- direct bili >2
- rate of rise >5/day
neonatal jaundice –> pathologic –> next step?
Coombs test
Coombs test for pathologic neonatal jaundice –> results?
+: Rh/ABO incomp
- :
- twin/twin, mom/fetus transfusion
- IDM
- spherocytosis
- G6PD def
biliary atresia: comp
liver fail
cause: direct hyperbili
- sepsis (ALWAYS r/o)
- biliary atresia
- galactosemia
- hypothyroid
- choledochal cyst
- CF
bili >20: comp? tx?
kernicterus –> exchange transfusion
MOA: phototherapy
ionize/isomerize bili –> excrete
diag hernia: comp? tx?
pulm hypoplasia –> 3-4 days for lungs to mature –> surg
prenatal US –> diag hernia –> next step?
have mother deliver at facility w ECMO
TE fistula –> assoc?
VACTER: vertebral, anal atresia, cardiac, radial, renal
condition: cyanotic when feed, pink when cry
choanal atresia
choanal atresia –> assoc?
CHARGE: coloboma, heart defect, retarded growth, GU, ear/deaf
gastroschisis: lab result
high maternal AFP
omphalocele –> assoc?
- Edward
- Patau
- Beckwith-Wiedemann
Beckwith-Wiedemann: presentation
big baby –> big tongue, hypogly, ear pits
umbilical hernia –> assoc?
hypothyroid
tx: umbilical hernia
- nothing
- if persist 2-3yo –> surg
newborn –> bilious vomit –> ddx?
- intestinal atresia
- malrotation & volvulus
newborn –> malrotation & volvulus –> presentation?
- bilious vomit
- draw up legs
- abd distention
intestinal atresia –> assoc?
Down’s
newborn –> malrotation & volvulus –> pathophys?
Ladd’s bands –> kink duodenum
not pass meconium –> ddx?
- meconium ileus (CF)
- Hirschsprung’s
meconium ileus: dx? tx?
gastrograffin enema
Condition: DRE –> poo explosion
Hirschsprung’s
Condition: premature –> bloody diarrhea
necrotizing enterocolitis
necrotizing enterocolitis –> XR?
pneumocystis intestinalis (air in wall)
tx: necrotizing enterocolitis
- NPO
- TPN (if necess)
- abx
- resect necrotic bowel
RF: necrotizing enterocolitis
- premature gut
- intro feed
- formula
intussusception: dx? tx?
barium enema
dx: congenital adrenal hyperplasia
17-OH-progesterone b4/after ACTH bolus
tx: congenital adrenal hyperplasia
hydrocortisone & fludrocortisone
condition: ant midline mass, no pee
post urethral valve
tx: post urethral valve
- catheter to remv urine
- surg
diabetic mother: comp
- placental insuff/IUGR
- congenital heart dz
- NTD
- caudal regression synd
- small L colon synd
diabetic mother –> diet supplement?
folate 4mg/day
gestational DM: comp
- LGA
- hypogly
- hypoCa
- polycythemia
- jaundice
- RDS
neonate –> hypogly –> comp? tx?
sz
- freq feed (if glc<40)
- IV dextrose (if glc <20)
neonate –> hypoCa –> comp?
sz
gestational DM –> neonate –> polycythemia –> pathophys? comp?
big baby –> need more O2 –> hypoxia –> increase EPO –> polycythemia –> clot
pathophys: gestational DM –> neonate –> RDS
high insulin –> no cortisol surge prior to birth –> decreased lung maturation
condition: limb hypoplasia, cutaneous scar, cataract, chorioretinitis, cortical atrophy
congenital varicella
congenital varicella: when give VZIG?
if mom exposed 5day b4 to 2day after delivery
Bruton agammaglobulinemia: presentation
starting 6-9mo:
- mult ear infxn, diarrhea episode, pneumonia
- no tonsils
Bruton agammaglobulinemia: labs
- no B cells
- low Ig
condition: 17yo –> low Ig, normal B cell
combined variable immune def (acquired)
combined variable immune def: comp
lymphoma
