Neonatal Jaundice Flashcards

1
Q

pathologic jaundice is….

A

direct hyperbilirubinemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

t/f GB cancer / stones does not really occur in kids

A

true

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

congugated or direct hyperbilirubinemia in pediatrics ddx

A

biliary atresia
sepsis
metabolic derrangement

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

jaundice categories

A
  1. prehepatic
    - unconjugated, indirect
  2. intrahepatic
    - conjugation
  3. post-obstructive/ excretion
    - direct, conjugated
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

intrahepatic jaundice ddx

A

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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

direct bilirubin aka

A

conjugated

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

direct bilirubin is good one to have with kernicterus?

A

true

  • it is water soluble
  • excreted in urine
  • urine dark
  • cannot cross BBB
  • cannot cause kernicterus
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

kernicterus

A

bilirubin reaches basal ganglia of brain and causes permanent damage

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

unconjugated bilirubin is ___soluble

A

fat soluble
not excreted in urine
crosses BBB

causes kernicterus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

physiologic vs pathologic breakdown depends on what 4 things?

A

onset
time to resolution
type bilirubin
how fast rises

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

physiologic jaundice

  • onset
  • resolution
  • type
  • rise
A

onset: over 72 hrs
resolve less than 1 wk (preemie 2 wk)
bilirubin: unconjugated
rise: less than 5 points / day

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

pathologic jaundice

  • onset
  • resolution
  • type
  • rise
A

occurs first 24 hrs life
resolve over 2 wks
conjugated bilirubin
rises fast over 5/day

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

treat unconjugated why?

A

to prevent kernicterus

need to get it conjugated

blue light therapy!
really high transfusion

(based on nomogram)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

yellow baby conjugated bilirubin?

A

pathologic jaundice

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

pathologic jaundice workup

A
  1. u/s liver
  2. HIDA post phenobarb
  3. Look sepsis
  4. look metabolic dz
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

yellow baby unconjugated bilirubin ?

A

physiologic error

17
Q

physiologic jaundice workup

A
  1. coombs test
    - mom ab attack baby blood (+ test)
    = isoimmunization
  2. neg coombs then do Hgb
    - if low = hemorrhage
    - cephalohematoma
    - if Hgb elevated
    =transfusion
    (twin-twin, one placenta)
    = delayed clamp
  3. coombs and Hgb normal. Reticulocyte count
18
Q

elevated reticulocyte ct

A

hemolysis:
G6PD
pyruvate kinase def
Hgb SS dz

19
Q

Coombs normal, Hgb normal, reticulocyte normal, problem is

A

reabsorption issue

  • breast milk jaundice
  • breast feeding jaundice
20
Q

breast milk vs breast feeding jaundice ?

A

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
21
Q

breast feeding jaundice tx

A

feed baby more!

22
Q

breast milk jaundice tx

A

keep feeding baby but get rid of mom enzyme, give hydrolyzed formula

23
Q

crigler najjar inheritance pattern

A

autosomal recessive pattern

  • both UGT1A1 genes have mutations
24
Q

Gilbert syndrome 2 types of inheritence patterns

A
  • 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
25
Q

sx of kernicterus

A
  • lethargic infant
  • hypotonia with episodes of hypertonia and arching of backs
  • writhing mvmts of body choreoathetosis
  • hearing issues
  • intellectual disability
26
Q

crigler najjar prevelence

A

1 in 1 million infants or less WORLDWIDE

27
Q

dubin johnson syndrome inheritance pattern

A

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

28
Q

rotors syndrome inheritance pattern

A

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)
29
Q

why does sepsis sometimes cause direct hyperbilirubinemia?

A
  • mechanism : cholestasis

- can be from primary infxn cholangitis or secondarily

30
Q

pyruvate kinase deficiency

A

MC form of inherited nonspherocytic hemolytic anemia