Folvik Geir, Hilde Olset, Helge Gilja Odd
Department of Medicine, Division of Gastroenterology, Haukeland University Hospital and Institute of Medicine, University of Bergen, Bergen, Norway.
Scand J Gastroenterol. 2012 Apr;47(4):482-8. doi: 10.3109/00365521.2011.650191. Epub 2012 Jan 10.
Benign recurrent intrahepatic cholestasis (BRIC) is a rare autosomal recessive inherited disorder characterized by intermittent episodes of cholestatic jaundice. For the patients, the disease is a physical and psychological challenge. There is no curable treatment, but symptomatic relief is described following treatment with rifampicin or plasmapheresis.
Five patients suffering from BRIC followed up for 17 years by one consultant are described. Two patients were treated with rifampicin and plasmapheresis, two with rifampicin alone, and one with plasmapheresis.
The treatments showed symptomatic relief, effect on biochemical parameters, and earlier clinical remission compared with no treatment or treatment with other substances like cholestyramine, antihistamines, and ursodeoxycholic acid.
Both rifampicin and plasmapheresis represent important therapeutic options of acute cholestatic attacks in patients with BRIC. As a noninvasive treatment, rifampicin may be the first choice.
良性复发性肝内胆汁淤积症（BRIC）是一种罕见的常染色体隐性遗传性疾病，其特征为胆汁淤积性黄疸的间歇性发作。对患者而言，该病是一项生理和心理挑战。目前尚无治愈性治疗方法，但据描述，使用利福平或血浆置换治疗后症状可缓解。
描述了由一名会诊医生随访17年的5例BRIC患者。2例患者接受了利福平和血浆置换治疗，2例仅接受利福平治疗，1例接受血浆置换治疗。
与未治疗或使用消胆胺、抗组胺药和熊去氧胆酸等其他物质治疗相比，这些治疗显示出症状缓解、对生化指标有影响且临床缓解更早。
利福平和血浆置换均是BRIC患者急性胆汁淤积发作的重要治疗选择。作为一种非侵入性治疗，利福平可能是首选。
