Recently, we have experienced 6 cases of DubinJohnson syndrome with a detailed history of 3 families and confirmed these by clinical, microscopic, and electron-microscopic observations.
There were a total of 22 people in the 3 families and they consisted of 10 males and 12 females. Of these 22 members we observed jaundice in 11 persons; 7 showed predominantly conjugated hyperbilirubinemia, 1 showed predominantly unconjugated hyperbilirubinemia, and the remaining 2 showed normal serum bilirubin. Among these 8 who had jaundice, 3 people stated that the jaundice had appeared intermittently since birth, 1 had jaundice from his age of 5, 1 from 34, and in 3 the time of origin was unknown. We performed the bromsulfophthlein dye test in 6 members; 3 of them were jaundiced and 3 were not jaundiced. We could find abnormal B.S.P. dye retention serum at 120 minutes more than that of 45 minutes in the 3 jaundiced members, while all 3 of the non-jaundiced members showed normal B.S. retention. Intravenous cholangiography was done in 2 members who had jaundice, and the result as non-visualized. Urinary coproporphyrin test showed an increase of isomer I.
It seemed very interesting that 4 members of 2 families had suffered fro- schizophrenia.
Biopsies of the liver were done o 6 jaundiced members of 3 families. The gross appearances of the livers at the time of biopsy were dark-brown or black colored. Histologically those liver specimens showed that the lobular architecture was intact, but there were abundant pigment granules in hepatocytes, especially in the centrilobular areas. Furthermore the electron-microscopic examination revealed numerous oval or irregular) outlined and single membrane-bounded dense bodies throughout the hepatocytes. The dense bodies ere identified roughly as three classes by their electron densities; those with high, medium, and low densities. There were not any abnormal electrion-microscopic findings in the remaining organelles of t e hepatocytes.
Here, we report the cases with a brief review of the literature.
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