Liver Metabolism: Urea Cycle Flashcards
formation of bilirubin
Hg - to heme and globin
heme > biliverdin > bilirubin (direct)
carried to liver on albumin
UDPGT
uridyldiphosphate glucuronyl transferase
-conjugates bilirubin in liver at SER
adds glucuronide x2
heme oxygenase
heme > biliverdin
van den berg test
measure conjugated and unconjugated bilirubin levels
urobilinogen
converted in intestine (terminal ileum and colon)
colorless
20% reabsorbed
collection of sterco, meso, urobilinogen
normal range bilirubin
0.2-1
less than 0.2 conjugated
kernicterus
jaundice in infant - bilirubin > 15-20
-brain damage
aka brain encephalopathy
due to polycythemia, Rh incompatibility
pre-hepatic jaundice
excess hemolysis
hepatic jaundice
infection, drugs, alcohol, cirrhosis, pregnancy
post-hepatic jaundice
blockage of duct
cancer of bile duct, pancreas head
excess hemolysis
increased unconjugated and urinary urobilinogen
dark urine
total usually not > 5
biliverdin reductase
biliverdin > biliverdin
stercobilin
stool pigment
urobilin
in urine
causes of jaundice
excess bilirubin production
reduced hepatocyte uptake
impaired conjugation
impaired bile flow
gilberts syndrome
mild unconjugated hyperbilirubinemia
1.5-3
worse with stress
often misdiagnosed - chronic hepatitis
low activity of UDPGT (UGT 1A1 mutation)
crigler-najjar type I
absence of UDPGT
-very high unconjugated bilirubin
autosomal recessive
most patients die of kernicterus
Tx: early liver transplant
crigler najjar type II
autosomal dominant
partial deficiency UDPGT
unconjugated bilirubin elevated 5-20
responds to phenobarbitol
phenobarbitol
Tx for crigler najjar type II
dubin johnson syndrome
autosomal recessive
elevated conjugated
defective canalicular excretion
liver biopsy - black**
rotor syndrome
elevated conjugated
liver biopsy - normal