Lo N S, Chan C W, Hutchison J H
Arch Dis Child. 1979 Jul;54(7):529-33. doi: 10.1136/adc.54.7.529.
Three cases of chronic nonhaemolytic jaundice with conjugated bilirubin in the serum are described in a Chinese family. Bromsulphthalein excretion tests gave results typical of the Dubin-Johnson syndrome. Liver histology in the proband showed cytoplasmic pigment of the lipofuscinmelanin variety, and intravenous cholecystography failed to show visualisation of the gallbladder. Unusual findings included onset during the neonatal period in the proband and the presence of some iron pigment in the hepatic cells with a little canalicular cholestasis. It is suggested that the infant may have had a concomitant nonspecific hepatitis. These cases are regarded as belonging to a disease group in which the Dubin-Johnson syndrome is at one end of a spectrum. The mode of inheritance is discussed.
在中国一个家族中描述了3例血清中结合胆红素升高的慢性非溶血性黄疸病例。磺溴酞钠排泄试验结果符合杜宾-约翰逊综合征的典型表现。先证者的肝脏组织学检查显示有脂褐素-黑色素样的细胞质色素,静脉胆囊造影未能显示胆囊显影。异常表现包括先证者在新生儿期发病,肝细胞内存在一些铁色素并有轻度胆小管胆汁淤积。提示该婴儿可能同时患有非特异性肝炎。这些病例被认为属于一个疾病组,其中杜宾-约翰逊综合征处于该疾病谱的一端。文中还讨论了遗传方式。