Bilirubin Flashcards

1
Q

cregler niger syndrome

A

lack of UGP

  • causes collection of billirubin in the blood
  • glucuronyl transerase deficiency.
  • early buildup causes kernicterus on newborns
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2
Q

dudin johnson syndrome

A

mutation in MR2P- causes conjugated bilirubin

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3
Q

Causes of increased unconjugated bilirubin in neonates

A
  • physiological jaundice of the newborn
  • hemolytic disease owing to Rh or ABO incompatibility
  • presence of breast milk hyperbilirubinemia caused by alpha- glucoronidase in breast milk that causes the hydrolysis of conjugated bilirubin in the neonate
  • usually due to an underdeveloped liver
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4
Q

causes of conjugated hyperbilirubinemia are

A

neonatal hepatisis and billiary atresia

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5
Q

gilberts syndrome

A

transport and conjugation defect, bilirubin = 2-3 mg/dL

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6
Q

direct testing of bilirubin tests for ______ bilirubin

A

conjugated

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7
Q

indirect testing of bilirubin tests for _________ bilirubin

A

unconjugated

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8
Q

Bilirubin

A

transported in the blood to the liver bound to albumin

  • in the hepatocyte the bilirubin is rapidly conjugated with glucuronic acid to produce bilirubin monoglucuronide and diglucuronide which is then secreted into the bile
  • in the intestine the bilirubin is hydroized into unconjugated bilirubin which is then reduced by the microbed into 3 urobilirubins: stercobilin, mesobilin, and urobilin. they are orange brown pigments that give the stool and urine their colors
  • is in a ZZ (trans) formation but when exopsed to light is a EE(cis) formation which is water soluble and more easily excreted in the bile
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9
Q

icterus

A

the deposit of bile pigment in the skin, mucos and scelera resulting in a yellow appearance
- can happen in the serum too

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10
Q

unconjugated billirubin

A

water insoluble

  • causes jaundice
  • soluble in alcohol
  • elevated in kernicterus
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11
Q

Conjugated Bili

A

water soluble

  • causes jaundice
  • present in urine
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12
Q

Diazoreaction

A

bilirubin that reacts with Diazo without alcohol is direct bili
- reactions that occur after the addition of alcohol is indirect bilirubin

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13
Q

Urine testing

A

only conjugated is in the urine

  • use fouchets test, a tablet test(ictotest), or a strip test (bilistix)
  • blilirubin uria seen in biliary obstruction, hepatocellular damage, dubin- johnson syndrome, and rotor’s syndrome
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