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Multiple Choice

Which condition is associated with conjugated hyperbilirubinemia (direct bilirubin) with normal indirect?

Conjugated hyperbilirubinemia with a normal indirect level points to a problem with excretion of conjugated bilirubin from the liver into bile, not with its formation. Dubin-Johnson syndrome fits this pattern: a defect in the canalicular transporter ABCC2/MRP2 impairs secretion of conjugated bilirubin, so direct bilirubin rises while indirect stays normal. Gilbert syndrome and Crigler-Najjar disorders involve impaired conjugation or excessive production of unconjugated bilirubin, so they do not produce the described direct-dominant profile. Hence, this pattern is characteristic of Dubin-Johnson syndrome.

Conjugated hyperbilirubinemia with a normal indirect level points to a problem with excretion of conjugated bilirubin from the liver into bile, not with its formation. Dubin-Johnson syndrome fits this pattern: a defect in the canalicular transporter ABCC2/MRP2 impairs secretion of conjugated bilirubin, so direct bilirubin rises while indirect stays normal. Gilbert syndrome and Crigler-Najjar disorders involve impaired conjugation or excessive production of unconjugated bilirubin, so they do not produce the described direct-dominant profile. Hence, this pattern is characteristic of Dubin-Johnson syndrome.