TERMS

Question 1

degradation product of heme

Answer 1

bilirubin

Question 2

yellow discoloration of skin attributed to systemic accumulation and deposition of unconjugated and conjugated bilirubin in tissues (icterus-yellowing of sclera)

Answer 2

jaundice

Question 3

unconjugated bilirubin accumulates (toxic and non-soluble)

Answer 3

hemolytic disease of newborn

Question 4

with prolonged conjugated hyperbilirubinemia, a fraction becomes covalently bound to albumin

Answer 4

delta bilirubin

Question 5

what is this unconjugated hereditary hyperbilirbinemia?

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

Answer 5

gilbert syndrome

Question 6

what is this unconjugated hereditary hyperbilirbinemia?

two types
type I-absent bilirubin UGT, fatal, death

type II-decreased bilirubin UGT activity, less severe, non fatal, kernicterus

Answer 6

crigler-najjar syndrome

Question 7

what is this conjugated hereditary hyperbilirbinemia?

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

Answer 7

dubia johnson syndrome

Question 8

what is this conjugated hereditary hyperbilirbinemia?

asymptomatic, multiple defects in hepatocellular uptake and excretion of bilirubin; autosomal recessive

Answer 8

rotor syndrome