Which condition is associated with Dubin-Johnson syndrome?

Dubin-Johnson syndrome is a genetic condition characterized by a defect in the hepatic transport mechanism responsible for conjugating bilirubin. The primary issue in this syndrome lies in the defective excretion of conjugated bilirubin from hepatocytes into the bile, leading to an accumulation of conjugated bilirubin in the bloodstream. Therefore, patients with Dubin-Johnson syndrome present with conjugated hyperbilirubinemia.

The condition is often benign, and while it can lead to episodes of jaundice, it does not typically manifest with severe liver dysfunction or significant complications such as hepatocellular carcinoma or acute liver failure. Understanding the physiological and genetic basis of Dubin-Johnson syndrome clarifies why conjugated hyperbilirubinemia is the hallmark laboratory finding.