Leger L, Soprani A, Bouvresse M, Liguory C
J Chir (Paris). 1975 Dec;110(6):519-32.
The authors report 6 cases of chronic familial pancreatitis. They review the literature on this disease which is still little recognised and seek the specific characteristics, early onset, transmission as an autosomic dominant with incomplete penetrance, relatively favourable prognosis, together with the suggestive signs, pancreatic lithiasis and certain complications. Apart from these signs, chronic familial pancreatitis differs very little from common chronic pancreatitis. The only cause is apparently genetic. Etiopathogenesis is thus still obscure. Treatment should be started early, but surgical methods are the same as in primary chronic pancreatitis.
作者报告了6例慢性家族性胰腺炎病例。他们回顾了关于这种仍未得到充分认识的疾病的文献，并探寻其特定特征，即发病早、以常染色体显性遗传且外显不全的方式遗传、预后相对良好，以及提示性体征、胰腺结石和某些并发症。除了这些体征外，慢性家族性胰腺炎与普通慢性胰腺炎几乎没有差异。唯一的病因显然是遗传因素。因此，病因发病机制仍不清楚。治疗应尽早开始，但手术方法与原发性慢性胰腺炎相同。
