Dubin-Johnson Syndrome

In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan.


In 1954, Dubin and Johnson described a new clinicopathological entity consisting of chronic idiopathic jaundice with unidentified pigment in the liver in 12 cases. In the same year, Sprinz and Nelson published a report of four cases with persistent non-hemolytic hyperbilirubinemia associated with lipochrome-like pigment in the liver cells; soon, the syndrome had come to be known as Dubin Johnson syndrome (DJS). This relatively rare disorder is a variety of hereditary hyperbilirubinemia and is characterized by low-grade elevation of conjugated bilirubin and no other signs of hepatic injury. It results from a mutation that leads to improper excretion of bilirubin from hepatocytes.

In most cases, DJS presents in the second decade of life, and rarely it may present in an infant.

The condition is benign, has no long-term consequences, and does not require medical therapy. Diagnosing DJS is important to eliminate the possibility of other hepatobiliary disorders that may cause hepatic injury and diagnose those which may be potentially treatable.

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