Watanabe S, Nishioka M, Kodama T, Ando K, Numa Y, Fukumoto Y, Okita K, Takemoto T, Mizuta M
Gastroenterol Jpn. 1982 Dec;17(6):576-84. doi: 10.1007/BF02779135.
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.
对11例杜宾-约翰逊综合征（DJS）患者进行了临床病理研究。在3例DJS患者中，观察到合并慢性肝炎。他们表现为长期黄疸，伴有全身乏力和厌食症状。实验室检查显示轻度转氨酶升高、血清胆红素升高（超过5.0mg/dl）以及45分钟时血清磺溴酞钠（BSP）潴留水平较高（超过18%）。与无并发症的患者相比，2例合并慢性肝炎的患者BSP排泄曲线的继发性上升较为缓慢。1例伴有最严重纤维化的患者BSP排泄曲线无继发性上升。然而，用苯巴比妥治疗该患者后，BSP排泄曲线出现继发性上升，且BSP排泄曲线上各点的血清BSP水平也显著降低。肝脏组织学检查显示，合并慢性肝炎的患者杜宾-约翰逊色素数量减少。腹腔镜检查显示，1例慢性侵袭性肝炎患者肝脏表面呈深灰色脱色，另1例慢性侵袭性肝炎患者肝脏表面呈黑色并伴有部分结节形成。
