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8 Cards in this Set
- Front
- Back
Icterus vs. Jaundice
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Patients with icterus have yellow sclera, and patients with jaundice have yellow skin.
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At what level of bilirubin does jaundice occur?
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Jaundice occurs when systemic retention of bilirubin leads to serum levels above 2.0-2.5mg/dl
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Kenicterus
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severe neurologic damage in newborn, caused by accumulation of unconjugated bilirubin in brain
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• Crigler-Najjar syndrome type I
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• 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) |
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• Crigler-Najjar syndrome type II
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• Crigler-Najjar syndrome type II
o Autosomal recessive o Less severe, nonfatal o Partial defect in the conjugating enzyme – consequence is extraordinarily yellow skin |
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• Gilbert syndrome
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• 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 |
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• Dubin-Johnson syndrome
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• 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 |
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• Rotor syndrome
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o Variant of Dubin-Johnson syndrome in which the liver is not pigmented
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