A Case with Dubin-Johnson Syndrome Re-Examination after an Interval of Twenty Years

Abstract

A 41 year old woman who had been diagnosed as having Dubin-Johnson syndrome in 1956 was reexamined in 1976 after an interval of 20 years. The good stable condition of the disorder was noticed but the following three significant changes were observed. 1) After a single dose of an intravenous injection of BSP (5mg/kg) the retention after 45 minutes was markedly increased (90%) but gradually decreased without another rise (Mandema's phenomenon), however it was discovered in a smaller dose of BSP (1mg/kg). 2) A large amount of accumulation of pigment granules was observed in the Kupffer cells, where by in the hepatocytes were decreased. No other histological change was noticed. 3) By oral cholecystography a gallbladder was visualized. Such a case has never been found in the literature. Some problems of constitutional hyperbilirubinemia, including coproporphyrin metabolism, were discussed.