Dubin-Johnson syndrome with varied manifestations

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WHAT is now known as the Dubin-Johnson syndrome was originally reported in 1954 as “chronic idiopathic jaundice.”1 That same year Sprinz and Nelson2 reported four cases with similar clinical and histologic features; in 1958 Dubin3 reviewed 50 cases. Since then there have been other case reports, which have emphasized the familial nature4, 5 and world-wide occurrence of the syndrome.6

Recent studies have concerned selected features of the Dubin-Johnson syndrome with particular attention given to the delay in secretion of sulfobromophthalein in patients with that disease.7, 8 Further, electron microscopic studies of the ultrastructure of the hepatic cell have been done,9 but without positive identification of the abnormal pigment present in the hepatic cell. It is usually considered to be in the lipochrome or lipofuscin family of pigment. The exact mechanism of hyperbilirubinemia remains unknown, although conjugated hyperbilirubinemia is the hallmark of the syndrome. Since hyperbilirubinemia is intermittent, it has been postulated that there is an inherited failure of biliary secretion, with inconstant clinical manifestations.10–13 It has been this unusual aspect of the Dubin-Johnson syndrome which has attracted most recent attention. There has been considerable emphasis on biliary secretion, cholestasis, and excretion of conjugated bilirubin by the liver cell;10–13 and yet the Dubin-Johnson syndrome seems to be a unique form of failure of excretion of conjugated bilirubin. The only syndrome which appears to be similar is the Rotor syndrome,10–14 in which there is also a failure of biliary excretion of conjugated bilirubin, but without the presence of lipochrome pigment.

Recently, we . . .



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