Pathology of the liver I Flashcards
hepatocyte integrity refers to what structure specifically?
membrane
why is LDH not as specific for liver damage?
RBCs also use LDH
what is the role of albumin?
transporter of heme and FAs
maintains blood osmolarity
what enzymes are elevated in response to bile flow blockage?
alkaline phosphatase
5-NT
GGT
where are 5-NT and GGT located?
bile duct epithelium
which type of bilirubin has conjugated sugars? is it more or less soluble?
direct (measured directly in the lab)
more soluble
why is unconjugated bilirubin referred to as indirect?
because the value is obtained by subtracting direct from total bilirubin
at what bilirubin levels do you start getting symptoms of jaundice?
above 2 mg / dL
what is the normal range for bilirubin?
0.1 - 1.0 mg / dL
what are the general mechanisms of jaundice and cholestasis?
- isolated disorders
- liver disease
- bile duct obstruction
prehepatic hyperbilirubinemia will involve what type of bilirubin? what is the main cause? what is the mechanism?
unconjugated
RBCs
heme gets into macrophages - heme - biliverdin - bilirubin-albumin
where does conjugation of bilirubin occur, specifically?
hepatocyte ER
hepatic hyperbilirubinemia will involve what type of bilirubin?
unconjugated
posthepatic hyperbilirubinemia will involve what type of bilirubin?
conjugated
what enzyme is responsible for conjugation of bilirubin?
UGT1A1
which form of bilirubin can be excreted?
bilirubin monoglucuronide
what type of bilirubin metabolism errors will result in elevation of unconjugated bilirubin?
overproduction
reduced uptake
defective conjugation
what type of bilirubin metabolism errors will result in elevation of conjugated bilirubin?
defective excretion
defective secretion
gilbert syndrome and crigler-najjar syndrome involve which general dysfunction in bilirubin metabolism?
reduced glucuronyl transferase activity
what is the only autosomal dominant disorder of bilirubin metabolism?
crigler-najjar syndrome type II
how is bilirubin metabolism affected by crigler-najjar syndrome type I?
absent
how is bilirubin metabolism affected by crigler-najjar syndrome type II?
decreased UGT1A1 activity
how is bilirubin metabolism affected by gilbert syndrome?
decreased
how is bilirubin metabolism affected by dubin johnson syndrome?
impaired biliary excretion of bilirubin glucuronides due to mutation in canalicular multidrug resistance protein 2
what is the liver pathology associated with dubin johnson syndrome?
pigmented cytoplasmic globules
BLACK LIVER
which syndrome of bilirubin metabolism is associated with kernicterus?
crigler-najjar syndrome type I