Dubin-Johnson氏症候群の肝実質細胞の電子顕微鏡学的観察

とくに色素顆粒の電子顕微鏡学的所見および黄疸発生の形態学的考察

抄録

Four cases including a male sibling with Dubin-Johnson's syndrome, which diagnosis was confirmed by peritoneoscopy and histologic studies of the liver biopsy specimens, were investigated.Electron-microscopic studies were made with special reference to the process of yielding the peculiar pigment granules in the liver-cells of this syndrome. In addition, mechanism of jaundice as well as bromsulphalein metabolism in this disease was analyzed from electron-microscopic findings, and the results were as follws:
1. The cristae and inner membrane of the outer envelope of mitochondria of the liver-cell seemed to play an important role for producing the pigment granules. Namely, first of all, small granules appeared within mitochondria and gradually increased in size and number, at which time the non-functioning mitochondria was separated from cytoplasm by a single membrane. Then, the pigment granules confluenced each other and piled up, covered, as a whole, with a membrane, being, segregated from cytoplasm. Thus the pigment granules demonstrated various morphological changes during the process of their production.
2. There was no sign of destruction in the terminal bar (desmosome) of the bile canaliculi in Dubin-Johnson's syndrome, but the microvilli were reduced in number and size. On the other hand, some parenchymal liver cells were remarkably provided with microvilli on the surfaces where they were placed oppositely each other. And the vacuoles in the liver-cells were directly communicated to the intercellular spaces. These findings suggested the mechanisms of jaundice in this syndrome and also of the increase in conjugated bromsulphalein in the blood with passing of the time.