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.

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Blood ammonia concentration in liver diseases was studied with special reference to its relation to hepatic coma.
1) Blood ammonia level was determined by the microdiffusion method of Conway. Elevated blood ammonia level was observed in the patients with liver diseases with the exception of obstructive jaundice and higher level was observed in the patients with hepatic coma. However, the overlapping of the values between these three groups was observed and blood ammonia level did not always correlate with the clinical condition.
2) In some patients with liver diseases, the oral administration of antibiotics which supress the bacterial growth in making ammonia in intestine, and the parenteral administration of arginine, an activator of urea cycle in the liver were effective against hepatic coma, resulting in lowering of blood ammonia.
3) Hepatic coma with hyperammoniemia was provoked in some patients with liver diseases by the administration of chlorothiazide, hydrochlorothiazide and mercury diuretics.
4) A case with probably hepatocerebral syndrome was observed for one and half year. This patient had frequent attacks of unconsciousness and was also provoked by acetazolamide and high intake of protein. These attacks were fairly good correlated with hyperammoniemia.
In summary, ammonia intoxication seems to play an important role in the genesis of hepatic coma.

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