Stremmel W, Scholten T, Miller B, Strohmeyer G
Z Gastroenterol. 1984 Jun;22(6):300-4.
The diagnosis of benign recurrent intrahepatic cholestasis was made in a 40 year old female. Seven episodes of jaundice persisting up to 6 months in lengths lead to 3 laparotomies, numerous peritoneoscopies and needle biopsies of the liver. Elevation of conjugated serum bilirubin and alkaline phosphatase levels and histopathological confirmation of intrahepatic cholestasis without cholangitis were the main characteristics during the episodes of cholestatic jaundice. However, hepatic histology and liver function were normal in remission periods. Drug induced cholestasis was ruled out by careful history. A sister also suffered from 4 episodes of intrahepatic cholestasis. The etiology of the disease is probably of a genetic origin. The pathogenesis remains unclear. There is no specific treatment to prevent or shorten the occurrence of cholestatic episodes. The patients are advised to avoid pregnancy and oral contraceptives, since both will induce icteric phases.
一名40岁女性被诊断为良性复发性肝内胆汁淤积症。七次黄疸发作持续长达6个月，导致3次剖腹手术、多次腹腔镜检查以及肝脏穿刺活检。胆汁淤积性黄疸发作期间的主要特征为结合血清胆红素和碱性磷酸酶水平升高，以及肝内胆汁淤积且无胆管炎的组织病理学证实。然而，缓解期肝脏组织学和肝功能均正常。通过仔细询问病史排除了药物性胆汁淤积。其姐姐也患有4次肝内胆汁淤积症。该病病因可能源于遗传。发病机制尚不清楚。尚无预防或缩短胆汁淤积发作的特异性治疗方法。建议患者避免妊娠和口服避孕药，因为二者均可诱发黄疸期。
