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GI II Exam II > Pediatric Jaundice > Flashcards

Pediatric Jaundice Flashcards

1
Q

Which type of bilirubin is toxic to the CNS? (Be specific!)

A

FREE (not bound to albumin); UNCONJUGATED bilirubin

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2
Q

The large amounts of unconjugated bilirubin in the blood of a new baby is due to what factors?

A
  1. Hemolysis
  2. Inadequate conjugation (due to RBC with shorter life span and immature glucouronosyltransferase) and clearance from GI tract.
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3
Q

__________ is the most common type of bilirubin in neonatal hyperbilirubinemia due to ___________.

A
  1. Unconjugated/indirect bilirubin (lipid soluble type of bilirubin bound to albumin, before reaching the liver)
  2. Hemolysis of RBCS
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4
Q

Differentiate breast feeding jaundice vs. breast milk jaundice?

A
  • Breast feeding jaundice is more of a function of dehydration and decreased excretion of bilirubin in the stool (related to the supply of the breast milk, which is sometimes low in first few days)
  • Breast milk jaundice is due to presence of deconjugating enzymes in milk
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5
Q

What is jaundice?

A

Yellow-orange tint found in the sclera and skin in infants due to hyperbilirubinemia.

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6
Q

_____ of newborns appear jaundiced during the first weeks of life.

A

2/3

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7
Q

What 3 characters are newborns born with?

A
    1. High hematocrits and RBC volume
    1. RBC have a shorter life span (70-90 days)
    1. Immature glucuronosyltransferase => cant conjugate bilirubin
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8
Q

Conjugation of bilirubin occurs where?

A

Liver

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9
Q

______ it initially low, overwhelming the amount of UCB that goes to the liver. BUT increases over the first few weeks.

A

UGT1A1

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10
Q

What is the fate of conjugated/direct BR?

A
  • => intestines via the gallbladder and bile duct.
  • Once in the intestines:
      1. Beta-glucuronidase can deconjugate conjugated BR (water-soluble) from gut => blood
      1. Rest is pooped out.
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11
Q
  • Conjugated/direct BR is _____-soluble
  • UCB is ______- soluble
A
  • CBR = water soluble
  • UCB = lipid soluble
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12
Q

Visible jaundice early in life usually means that the TSB is at least ______

A

5mg/dL

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13
Q

Non-pathologic hyperbilirubinemia is ALWAYS __________-

A

UNCONJUGATED

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14
Q

Which type of hyperbilirubinemia is always pathologic?

A

Conjugated hyperbilirubinemia

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15
Q

What are common causes of unconjugated hyperbilirubinemia?

A
  1. Increase production of BR
  2. Deficiency of liver upatake (bc must be with albumin)
  3. Impaired conjugation (Gilbert, CN syndrome type 1, severe UGT1A1 deficiency)
  4. Impaired enterohepatic circulation (decreased intake, decreased passage of stool)
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16
Q

What are some of the main signs of Kernicterus in a newborn?

A
    • Choreoathetotic movements
    • Ballismus
    • Upward gaze
    • Dental dysplasia
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17
Q

What are 4 pathologic abnormalities resulting in ↑ production unconjugated bilirubin in a new born?

A
    1. Erythrocyte-enzyme deficiencies
    1. Blood group incompatibility (ABO)
    1. Structural defects in RBC’s
    1. G6PD deficiency (enzyme deficiency)
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18
Q

What is Rh and why should Rh testing be done on all pregnant women?

A
  • Rh is inherited and found on surface of RBC’s
  • If mom is Rh (-) and baby is Rh (+) –> some of babies RBC’s get into Mom’s circulation during pregnancy and Mom develops Rh antibodies
  • No big deal in 1st pregnancy, but w/ subsequent pregnancies the Rh antibodies can cross placenta and wreak havoc on Rh (+) baby (hydrops fetalis or erythroblastosis fetalis)
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19
Q

When does ABO incompatability occur and what can it cause?

A
  • Mom = O blood type; Baby has A/B/AB blood type => unconjugated hyperbilirubinemia due to increase BR production.
20
Q

_____________ and ________ testing should be done on all pregnant women?

A
  • ABO
  • Rh (D) testing
21
Q

If Mom has type O blood or if Mom is Rh (-), the infant’s cord blood should be evaluated for what 3 things/how?

A
  1. Blood type
  2. Determine what their Rh is
  3. DAT (Direct antibody/Combs test)
22
Q

If mom is ______ or ________, DO NOT test the cord blood.

A
  1. Not O
  2. Rh (+)
23
Q

If you see a high conjugated/direct BR, where is there a problem?

A

IN the liver or PAST the liver.

Bc conjugating occurs IN the liver

24
Q

What is the difference between Direct and Indirect Combs?

When is Direct Combs use?

A
  • Direct: You are looking for moms antibodies that have attached themselves directly on the bbs RBC.
    • Used in situations where hyperbili is thought to be due to hemolysis, esp in ABO incompatibility.
25
Q

When do we have conjugated hyperbillirubinemia?

A
  1. Conjugated BR is > 1mg/dL when TSB (Total serum BR) is less than 5mg/dL
  2. Or when >20% of our TSB is conjugated BR.
26
Q

_______ hyperbilirubinemia in a newborn bb is RARE.

A

Conjugated hyperbilirubinemia

27
Q

Causes of conjugated hyperbilirubinemia?

A
  1. UTI/sepsis
  2. Biliary atresia/cholestasis
  3. Hypothyroidism
  4. Galactosemia
28
Q

If a newborn has conjugated hyperbilirubinemia in the first few post-natal weeks what is the first thing you should think?

A

Biliary atresia/cholestasis

29
Q

What is acute bilirubin toxicity?

A

When you have a super super high indirect bilirubin (& bc lipid-soluble), can cross BBB.

30
Q

What is seen in Phase 1 (the first 1-2 days) of acute bilirubin toxicity in an infant (high unconjugated bilirubinemia)?

A
    • Poor suck
    • High pitched cry
    • Stupor
    • Hypotonia
    • Seizures
31
Q

What is seen in Phase 2 (middle of the 1st week of life) of acute bilirubin toxicity in an infant (w/ high unconjugated bilirubinemia)?

A
  1. Hypertonia of extensor ms.
  2. Opisthotonus
  3. Retrocollis
  4. Fever
32
Q

What is seen in Phase 3 (after 1st week of life) of acute bilirubin toxicity in an infant (w/ high unconjugated bilirubinemia)?

A

Hypertonia

33
Q

What is the chronic and permanent sequelae of Bilirubin Induced Neurologic Dysfunction (BIND)?

why does this happen?

A

Kernicterus: UCB is lipid soluble and passwes through the BBB.

34
Q

If there is jaundice in the first 24 hrs or the jaundice is excessive for infants age; which tests should be run?

When should Total Serum Bilirubin (TSB) be rechecked?

A
  • TSB
35
Q

What are te 3 biggest risk factors for hyperbilirubinemia?

A
  1. Prematurity (before 35/36 weeks)
  2. High hematocrit at birth
  3. ABO incompatibility
36
Q

What is the BiliTool?

A

assess the risks of developing hyperbilirubinemia or “jaundice” in newborns over 35 weeks gestational age

  • tells you treatment
37
Q

What info do you enter in Bili Tool?

A
  1. Newborn’s gestational age,
  2. Birthday
  3. Date and time of sampling and bilirubin levels.
38
Q

How to treat mild jaundice, but not a candidate for phottherapy?

A

1. increase frequency of feeding

2. Continue breast feeding

39
Q
  1. Explain how phototherapy decreases bilirubin levels in infants undergoing treatment for hyperbilirubinemia.
A
  • Phototherapy isomerizes BR by exposing as much surface of BARE skin as possible, making it water-soluble so that you can get rid of it
40
Q

Home phototherapy can be used depending on?

A
  1. BR levels
  2. Home situation
  3. Ability and willingness to follow up
  4. Comfort level of parents
41
Q

What are 3 signs/symptoms of Biliary Atresia (progressive and destructive inflammatory process that affects BOTH extra and intrahepatic biliary trea => jaundice in 1st few post-natal weeks) in an infant?

A
  1. - Cholestatic jaundice (conjugated hyperbilirubinemia)
  2. - Hepatomegaly
  3. - Acholic stools
42
Q

If there is prolonged jaundice in an infant and you are approaching 2 months, what should you start thinking about?

A

Gilberts

43
Q

Crigler-Najjar Type 1 and Type 2 differ in their response to what drug?

A
  1. Type 1 (total UDPGT deficiency) results in SEVERE hyperbilirubinemia with high risk of BIND/Kernicterus
  2. Type 2 (partial UDPGT deficiency) results in mild hyperbilirubinemia with low risk of BIND/Kernicterus
44
Q

Extrahepatic biliary atresia occurs where in the hepatobilirary system?

A

Common bile duct

45
Q
A

GI II Exam II (10 decks)

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