Neonatal Jaundice Flashcards
pathologic jaundice is….
direct hyperbilirubinemia
t/f GB cancer / stones does not really occur in kids
true
congugated or direct hyperbilirubinemia in pediatrics ddx
biliary atresia
sepsis
metabolic derrangement
jaundice categories
- prehepatic
- unconjugated, indirect - intrahepatic
- conjugation - post-obstructive/ excretion
- direct, conjugated
intrahepatic jaundice ddx
mixed:
- crigler-najar
- gilbert’s
(look similiar to prehepatic jaundice)
(UGT1A1 mutations..cannot conjugate)
- dubin johnson (ABCC2 gene cannot excrete) - rotors (SLCO1b1, SLCO1b3) (problem excretion more like post obstructive picture)
- hepatitis
direct bilirubin aka
conjugated
direct bilirubin is good one to have with kernicterus?
true
- it is water soluble
- excreted in urine
- urine dark
- cannot cross BBB
- cannot cause kernicterus
kernicterus
bilirubin reaches basal ganglia of brain and causes permanent damage
unconjugated bilirubin is ___soluble
fat soluble
not excreted in urine
crosses BBB
causes kernicterus
physiologic vs pathologic breakdown depends on what 4 things?
onset
time to resolution
type bilirubin
how fast rises
physiologic jaundice
- onset
- resolution
- type
- rise
onset: over 72 hrs
resolve less than 1 wk (preemie 2 wk)
bilirubin: unconjugated
rise: less than 5 points / day
pathologic jaundice
- onset
- resolution
- type
- rise
occurs first 24 hrs life
resolve over 2 wks
conjugated bilirubin
rises fast over 5/day
treat unconjugated why?
to prevent kernicterus
need to get it conjugated
blue light therapy!
really high transfusion
(based on nomogram)
yellow baby conjugated bilirubin?
pathologic jaundice
pathologic jaundice workup
- u/s liver
- HIDA post phenobarb
- Look sepsis
- look metabolic dz
yellow baby unconjugated bilirubin ?
physiologic error
physiologic jaundice workup
- coombs test
- mom ab attack baby blood (+ test)
= isoimmunization - neg coombs then do Hgb
- if low = hemorrhage
- cephalohematoma
- if Hgb elevated
=transfusion
(twin-twin, one placenta)
= delayed clamp - coombs and Hgb normal. Reticulocyte count
elevated reticulocyte ct
hemolysis:
G6PD
pyruvate kinase def
Hgb SS dz
Coombs normal, Hgb normal, reticulocyte normal, problem is
reabsorption issue
- breast milk jaundice
- breast feeding jaundice
breast milk vs breast feeding jaundice ?
milk:
- quality issue, moms milk inhibits conjugation
- unconjugated bilirubinemia
- post day 7 life
breast feed: - problem not feeding enough - bowel function slows down - more bili reabsorbed = jaundice - occurs day 1-7 - unconjugated
breast feeding jaundice tx
feed baby more!
breast milk jaundice tx
keep feeding baby but get rid of mom enzyme, give hydrolyzed formula
crigler najjar inheritance pattern
autosomal recessive pattern
- both UGT1A1 genes have mutations
Gilbert syndrome 2 types of inheritence patterns
- only one UGT1A1 gene has a mutation in the promoter region = autosomal recessive pattern
- UGT1A1 gene has a missense mutation only 1 causes syndrome = autosomal dominant pattern
sx of kernicterus
- lethargic infant
- hypotonia with episodes of hypertonia and arching of backs
- writhing mvmts of body choreoathetosis
- hearing issues
- intellectual disability
crigler najjar prevelence
1 in 1 million infants or less WORLDWIDE
dubin johnson syndrome inheritance pattern
autosomal recessive
changes in ABCC2 gene
(gene responsible for making proteins that transfer bilirubin out of liver cells into bile)
can have black liver due to bilirubin build up in liver
rotors syndrome inheritance pattern
autosomal recessive pattern
- mutation in both SLCO1b1 and SLCO1b3
- transport protein genes for excretion of bile
- mostly build up of conjugated bilirubin (direct, pathologic, yet not kernicterus)
why does sepsis sometimes cause direct hyperbilirubinemia?
- mechanism : cholestasis
- can be from primary infxn cholangitis or secondarily
pyruvate kinase deficiency
MC form of inherited nonspherocytic hemolytic anemia
