Sheehy T W, Law D E, Wade B H
Arch Intern Med. 1980 Oct;140(10):1364-6.
Only one of eight previously described patients with sickle cell intrahepatic cholestasis (SCIH) survived. Death usually resulted from liver failure and/or a hemorrhagic diathesis. This report deals with the successful treatment of SCIH by partial blood-plasma exchange transfusion. This treatment led to regression of hepatic encephalopathy, impending bleeding diathesis, and noticeable clinical improvement withn 48 hours. Within six days, the serum bilirubin level decreased from 146 to 16 mg/dL. Partial blood-plasma exchange transfusion may be valuable for the treatment of SCIH.
先前描述的8例镰状细胞性肝内胆汁淤积症（SCIH）患者中仅有1例存活。死亡通常由肝功能衰竭和/或出血素质导致。本报告论述了通过部分血浆置换输血成功治疗SCIH的病例。该治疗使肝性脑病消退、即将发生的出血素质得到改善，并在48小时内临床症状显著改善。6天内，血清胆红素水平从146mg/dL降至16mg/dL。部分血浆置换输血可能对SCIH的治疗有价值。
