Teufel J, Farack U M
Dept. of Gastroenterology, Kreiskrankenhaus Pfaffenhofen, FRG.
Scand J Gastroenterol Suppl. 1987;139:76-80. doi: 10.3109/00365528709089778.
Two case reports of Caroli's disease type I are given. The main clinical features were recurrent severe cholangitis, biliary colics, and persistent cholestasis. Both cases demonstrate the predominance in males and the prevalence of symptoms in adults up to the sixth decade. No features of congenital hepatic fibrosis or biliary cirrhosis and no renal pathomorphological changes could be detected. Investigations of their family members did not reveal genetic aspects.
本文报告了2例I型卡罗利病。主要临床特征为反复严重胆管炎、胆绞痛和持续性胆汁淤积。两例均显示男性居多，症状在60岁之前的成年人中更为普遍。未发现先天性肝纤维化或胆汁性肝硬化的特征，也未检测到肾脏病理形态学改变。对其家庭成员的调查未发现遗传方面的问题。
