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8 Cards in this Set

  • Front
  • Back
Icterus vs. Jaundice
Patients with icterus have yellow sclera, and patients with jaundice have yellow skin.
At what level of bilirubin does jaundice occur?
Jaundice occurs when systemic retention of bilirubin leads to serum levels above 2.0-2.5mg/dl
Kenicterus
severe neurologic damage in newborn, caused by accumulation of unconjugated bilirubin in brain
• Crigler-Najjar syndrome type I
• Crigler-Najjar syndrome type I
o Autosomal recessive
o Lack of the enzyme responsible for the conjugation of glucuronic acid to bilirubin
o Fatal  death within 18 months of birth secondary to brain damage (kernicterus)
• Crigler-Najjar syndrome type II
• Crigler-Najjar syndrome type II
o Autosomal recessive
o Less severe, nonfatal
o Partial defect in the conjugating enzyme – consequence is extraordinarily yellow skin
• Gilbert syndrome
• Gilbert syndrome
o Common, benign, heterogenous inherited
o Mild, fluctating unconjugated hyperbilirubinemia
o Primary cause: decreased hepatic levels of glucuronosyl-transferase
o Affecting up to 7% of the population
• Dubin-Johnson syndrome
• Dubin-Johnson syndrome
o Autosomal recessive defect in the transport protein responsible for hepatocellular excretion of bilirubin glucuronides across the canalicular membrane
o Conjugated hyperbilrubinemia
o Darkly pigmented liver from polymerized epinephrine metabolites
o hepatomegaly
• Rotor syndrome
o Variant of Dubin-Johnson syndrome in which the liver is not pigmented