Sanderson F, Quaranta J F, Cassuto-Viguier E, Grimaldi C, Troin D, Dujardin P, Delmont J
Clinique Néphrologique, Hôpital Pasteur, Nice.
Ann Med Interne (Paris). 1988;139 Suppl 1:35-7.
Benign recurrent intrahepatic cholestasis (BRIC) or Summerskill's disease is a rare affection characterized by recurrent episodes of cholestasis of no apparent cause, separated by totally asymptomatic remissions. No specific treatment exists for the disease, and plasma exchange (PE) was proposed for disabling episodes affecting the general condition of two patients: one by two sessions of 4 and 13 exchanges during two episodes respectively (mass exchanged 14 and 35.5 l) and the other by 3 exchanges (total exchanged 4.5 l) during one episode. Improvement in clinical symptomatology and shortening of duration of two cholestatic episodes was noted when PE was carried out at an early stage. Bile acids were purified satisfactorily (42 to 52%), 0.15 to 1.17 mmole being extracted at each acute attack. Although difficult to assess in a rare affection such as this, PE appears to contribute to the symptomatic treatment of BRIC acute episodes if performed early enough, shortening their duration by the probable purification of circulating factors that are cholestatogenic as in primary biliary cirrhosis.
良性复发性肝内胆汁淤积症（BRIC）或萨默斯凯尔病是一种罕见疾病，其特征为无明显病因的胆汁淤积反复发作，发作间期完全无症状缓解。该疾病尚无特效治疗方法，有两例病情严重影响全身状况的患者接受了血浆置换（PE）治疗：一例在两次发作期间分别进行了4次和13次置换（置换量分别为14升和35.5升），另一例在一次发作期间进行了3次置换（总置换量4.5升）。早期进行血浆置换时，临床症状有所改善，两次胆汁淤积发作的持续时间缩短。胆汁酸得到了满意的纯化（42%至52%），每次急性发作可提取0.15至1.17毫摩尔。尽管在这样一种罕见疾病中难以评估，但如果早期进行血浆置换，似乎有助于BRIC急性发作的对症治疗，通过可能纯化循环中如原发性胆汁性肝硬化中导致胆汁淤积的因子来缩短发作持续时间。
