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Pediatrics Gastro Intestinal System a3172b80

Which one of the following inherited conditions causes direct hyperbilirubinemia?

A
Gilbert syndrome
B
Type II Crigler-Najjar syndrome
C
Rotor syndrome
D
Type I Crigler-Najjar syndrome
High-Yield Explanation
C. Rotor syndrome(Ref: Nelson's 20/e p 1937-1939)Disorders of bilirubin metabolismType of bilirubin elevatedDiseasesUnconjugated HyperbilirubinemiaCrigler-Najjar syndrome Types I and II (UDP GT gene mutation) Gilbert disease (UPD GT polymorphism)Conjugated HyperbilirubinemiaDubin-Johnson syndrome (multiple drug-resistant protein 2 mutation) Rotor syndrome (deficiency in organic anion uptake)Unlike Dubin-Johnson syndrome, in Rotor syndrome:Total urinary coproporphyrin excretion is elevated, with a relative increase in coproporphyrin I isomerGallbladder is normal by roentgenography and Liver cells contain no black pigment.

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