Bach N, Schaffner F
Samuel Bronfman Department of Medicine, Mount Sinai School of Medicine, City University of New York, New York.
J Hepatol. 1994 Jun;20(6):698-701. doi: 10.1016/s0168-8278(05)80137-0.
To estimate the prevalence of primary biliary cirrhosis in family members with this disease and determine if a family history of primary biliary cirrhosis predisposes to the development of this disease, we performed a retrospective review of charts and a prospective mail survey of patients with primary biliary cirrhosis. Prevalence of primary biliary cirrhosis in family members of 405 patients with disease was compared to known prevalence of disease in the general population. Twenty-six patients with primary biliary cirrhosis had at least one family member with primary biliary cirrhosis. The estimated prevalence per 100,000 was 6420 or 6.4%. Exclusion of family members with primary biliary cirrhosis who were patients in our practice, in order to avoid duplication, changed the prevalence to 4282 per 100,000 or 4.3%. Affected family members included mothers, daughters, sisters, brothers, aunts and cousins. Estimates of prevalence of primary biliary cirrhosis are between 0.7 and 7.5 cases per 100,000. Therefore, compared to estimates in the general population, the prevalence of primary biliary cirrhosis in family members of patients with this disease is markedly increased. A family history of primary biliary cirrhosis is therefore a predisposing factor for the development of this disease.
为了评估原发性胆汁性肝硬化患者家庭成员中该病的患病率，并确定原发性胆汁性肝硬化家族史是否易引发该病，我们对病历进行了回顾性研究，并对原发性胆汁性肝硬化患者进行了前瞻性邮件调查。将405例原发性胆汁性肝硬化患者家庭成员中该病的患病率与普通人群中已知的患病率进行比较。26例原发性胆汁性肝硬化患者至少有一名家庭成员患有原发性胆汁性肝硬化。每10万人的估计患病率为6420，即6.4%。为避免重复，排除我们诊所中患有原发性胆汁性肝硬化的家庭成员患者后，患病率变为每10万人4282，即4.3%。受影响的家庭成员包括母亲、女儿、姐妹、兄弟、阿姨和表亲。原发性胆汁性肝硬化的患病率估计为每10万人0.7至7.5例。因此，与普通人群的估计患病率相比，该病患者家庭成员中原发性胆汁性肝硬化的患病率显著增加。因此，原发性胆汁性肝硬化家族史是该病发生的一个诱发因素。
