Pediatric Jaundice - Newman Flashcards

1
Q

what is heme broken down into?

A

biliverdin and CO

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2
Q

what is biliverdin reduced to?

A

bilirubin

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3
Q

how do you get free unconjugated bilirubin?

A

albumin-binding sites are saturated, or unconjugated bilirubin is displaced from albumin by medicine

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4
Q

is unconjugated bilirubin lipid soluble?

A

yes, so it can cross the BBB

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5
Q

high hematocrit and RBC volume

  • RBC with shorter life span
  • inadequate conjugation of bilirubin by the liver due to immaturity of hepatic glucuronosyltransferase
A

newborns

- lots of unconjugated bilirubin present in their blood!

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6
Q

what is the most common type of bilirubin involved in neonatal hyperbilirubinemia

A

UNconjugated

- a result of hemolysis of RBC’s

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7
Q

levels of what, increase rapidly in the first few weeks after birth?

A

UGT1A1

- initially the level is very low, overwhelming the amount of unconjugated bilirubin presented in the liver

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8
Q

what enzyme is located in the intestine, and deconjugates bilirubin (making it water soluble), allowing it to be reabsorbed from the gut into the blood?

A

beta-glucuronidase

- the rest is excreted in the stool

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9
Q

visible jaundice early in life usually means that the transcutaneous serum bilirubin (TSB) is at what level?

A

5 mg/dL

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10
Q

what causes jaundice?

A

hyperbilirubinemia

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11
Q

caused by dehydration and decreased excretion of bilirubin in the stool (more supply related)

A

breast FEEDING jaundice

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12
Q

due to the presence of bilirubin deconjugating enzymes in milk

A

breast MILK jaundice

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13
Q

conjugated hyperbilirubinemia is always what?

A

pathologic!

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14
Q

what causes unconjugated hyperbilirubinemia?

A
  • increased bilirubin production: erythrocyte-enzyme def, ABO incompatibility, RBC structural defects, G6PH def
  • hepatic uptake def
  • impaired conjugated: Gilbert synd, Crigler-Najjar type 1, severe UGT1A1 def
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15
Q

all moms that are type O blood, or Rh (D) neg must have what test done?

A

a direct coombs test
- tests for autoimmune hemolytic anemia (erythroblastosus fetalis)

also check the infant’s cord blood

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16
Q

what causes conjugated hyperbilirubinemia?

A
  • UTI or sepsis
  • **biliary atresia/cholestasis*
  • hypothyroidism
  • galactosemia
17
Q

first 1-2 days of life

  • poor suck
  • high pitched cry
  • stupor
  • hypotonia
  • seizures
A

phase 1 of acute bilirubin toxicity

18
Q

middle of first week of life

  • hypertonia of extensor muscles
  • retrocollis
  • fever
A

phase 2 of acute bilirubin toxicity

19
Q

after the first week of life

- hypertonia

A

phase 3 of acute bilirubin toxicity

20
Q

first year of life

  • active DTR’s
  • obligatory tonic-neck reflexes
  • delayed motor skills
A

phase 1 of BIND (bilirubin induced neurologic dysfunction)

KNOW this phase!

21
Q
  • choreoathetotic cerebral palsy
  • ballismus (spontaneous movements)
  • tremor
  • upward gaze
  • dental dysplasia
  • sensorineuronal hearing loss
  • cognitive impairment
A

phase 2 of BIND

22
Q

what do you assess for icterus?

A

the sclera and mucous membranes

23
Q
  • jaundice in the first 24
  • ABO incompatibility with positive DAT
  • hemolytic disease (G6PD, hereditary spherocytosis)
  • gestational age 35-36 weeks (preterm)
  • previous sibling requiring phototherapy
  • cephalohematoma or bruising (from vaccuum delivery)
  • exclusive breatfeeding (esp if it’s not going well)
  • east Asian race
A

risk factors for severe hyperbilirubinemia

24
Q

what are the 3 biggest risk factors for hyperbilirubinemia?

A
  • prematurity
  • high hematocrit at birth
  • ABO incompatibility
25
Q

what is the measurement for excessive jaundice in infants?

A

jaundice to neck = 5
jaundice to waist = 10
jaundice to toes = 15

26
Q

what tests need to be run when TSB increases rapidly or baby is unexpectedly jaundiced upon initial exam?

A
  • BT and DAT (coombs)
  • CBC and peripheral smear
  • conjugated bili level
  • reticulocyte count
  • repeat TSB in 4-24 hours
27
Q

what labs should be run when conjugated bilirubin is elevated?

A
  • UA
  • blood cultures
  • consider intra-hepatic or post-hepatic abnormalities
28
Q

what labs should be run when baby has prolonged jaundice (greater than 3 weeks)?

A
  • TSB, always order fractionated! (need unconj and conjugated)
  • check newborn screens (hypothyroid, galactosemia)
29
Q

if baby has jaundice for 2 months, what disease should you consider?

A

Gilberts

30
Q
  • most DC babies at 48 hours of life
  • bilirubin greater than 24 hours of life
  • DAT in babies born to Type O and Rh neg moms
  • folluw up of the newborn within 2 days of DC
A

hospital protocol

31
Q
  • increase frequency of feedings
  • continue breast feeding

if inadequate oral intake, excessive weight loss (>12% of birth weight), or dehydration, the baby should receive supplemental breast milk/formula

A

tx for mild jaundice, not a candidate for phototherapy

NOTE: exchange transfusion is considered when bili is >25 mg/dL or when symptoms of acute encephalopathy are present

32
Q

what makes bilirubin water soluble?

A

isomerization (H-bonds to water, rather than internally H-bonded)

33
Q

progressive nad destructive inflammatory process affecting both the extra and intrahepatic biliary tree

  • development of jaundice in the first few post-natal weeks
  • cholestatic jaundice, hepatomegaly, acholic stools
A

conjugated hyperbilirubinemia

34
Q
  • “giant cell” hepatitis
  • prolonged cholestatic jaundice
  • liver biopsy shows a very disrupted hepatic architecture
A

idiopathic neonatal hepatitis

35
Q

syndromic bile duct paucity or arteriohepatic dysplasia

  • AD
  • marked reduction of intrahepatic bile ducts
A

Alagile syndrome

36
Q
  • liver disease resulting from retention of abnormal proteins in the endoplasmic reticulum of the hepatocyte
  • most common genetic cause of acute and chronic liver disease in children
  • most common genetically caused disorder necessitating liver transplantation in children
A

a1-AT deficiency

37
Q

what causes jaundice in older children/adolescents?

A

obstructive or hepatocellular causes