Dahms B B
Arch Pathol Lab Med. 1979 Jan;103(1):30-3.
Obstructive jaundice, pruritus, and malabsorption developed in twin brothers in infancy. Early liver biopsy specimens showed intracellular and canalicular cholestasis with normal bile ducts. By the age of 3 years, both had cirrhosis and portal hypertension. Each died during the teen years from hepatocellular carcinoma. These brothers represent the tenth reported family with familial cholestatic cirrhosis, and they are the first patients with this syndrome in whom hepatoma developed.
一对双胞胎兄弟在婴儿期出现阻塞性黄疸、瘙痒和吸收不良。早期肝脏活检标本显示细胞内和胆小管胆汁淤积，胆管正常。到3岁时，两人均出现肝硬化和门静脉高压。两人均在青少年时期死于肝细胞癌。这对兄弟是报道的第十个家族性胆汁淤积性肝硬化家族，也是首例发生肝癌的该综合征患者。
