Clinicopathological studies of the Dubin-Johnson syndrome complicated with chronic hepatitis

Eleven patients with Dubin-Johnson syndrome (DJS) were studied clinicopathologically. In three patients with DJS, concomitant chronic hepatitis was observed. They showed long-standing jaundice with the symptoms of general fatigue and anorexia. Laboratory tests revealed mild hypertransaminasemia, elevated serum bilirubin (over 5.0 mg/dl) and a high level of serum Bromsulphalein (BSP) retention at 45 min (above 18%). Two patients complicated with chronic hepatitis showed a rather slow secondary rise in the BSP excretion curve in comparison with the patients who had no complication. One patient accompanied with the most severe fibrosis showed no secondary rise in the BSP excretion curve. After the treatment of the patient with phenobarbital, however, a secondary rise in the BSP excretion curve appeared and the serum BSP level was also significantly decreased at all points on the BSP excretion curve. Histological examination of the liver revealed the decreased number of the Dubin-Johnson pigments in the patients complicated with chronic hepatitis. Laparoscopically, a patient with a chronic aggressive hepatitis showed a dark gray decololization of the liver surface and another patient with a chronic aggressive hepatitis showed a black color of the liver surface with partial nodule formation.