HIGH YIELD II

Question 1

what are the types of unconjugated hereditary hyperbilirubinemia?

Answer 1

gilbert syndrome

crigler-najjar syndrome

Question 2

what are the types of conjugated hereditary hyperbilirubinemia?

Answer 2

dubin-johnson syndrome

rotor syndrome

Question 3

what is this unconjugated hereditary hyperbilirubinemia?

common, benign inherited (AD) condition presenting with fluctuating hyperbilirubinemia

detected at time of stress
no clinical consequence
glucoronidating activity reduced to 30% of normal

normal liver architecture

Answer 3

Gilbert syndrome

Question 4

what is this unconjugated hereditary hyperbilirubinemia?

two types

type 1 is autosomal recessive, absent bilirubin UGT activity, fatal death

type 2 is autosomal dominant with variable penetrance, decreased bilirubin UGT activity; less severe, non-fatal, neuro damage from kernicterus

Answer 4

crigler najjar syndrome

Question 5

what is this conjugated hereditary hyperbilirubinemia?

hereditary defect in hepatocellular excretion of bilirubin glucoronidase across canalicular membrane; autosomal recessive

deeply pigmented
normal life expectancy

Answer 5

dubin johnson syndrome

Question 6

what is this conjugated hereditary hyperbilirubinemia?

rare, asymptomatic, multiple defects in hepatocellular uptake and excretion of bilirubin; autosomal recessive; normal life expectancy

Answer 6

rotor syndrome