Gregorio G V, Ball C S, Mowat A P, Mieli-Vergani G
Department of Paediatrics, King's College Hospital, London.
Arch Dis Child. 1993 Jul;69(1):141-3. doi: 10.1136/adc.69.1.141.
Pruritus in hepatic cholestasis has been suggested to be secondary to a high concentration of serum bile acids. Rifampicin, which inhibits the uptake of bile acids by hepatocytes, has been used to treat pruritus. To determine the efficacy of rifampicin as a treatment for refractory pruritus, the medical records of 33 children (median age 25 months, range 4-135; 19 boys) with chronic cholestasis liver disease (21 with Alagille's syndrome, eight with progressive intrahepatic cholestasis, one with extrahepatic biliary atresia, one with an inborn error of bile acid metabolism, and one with cryptogenic cirrhosis) were reviewed retrospectively. The median dose of rifampicin was 5(4-10) mg/kg/day. The median duration of intake was 36(4-120) weeks. Complete relief of pruritus was noted in five (15%) patients and a partial response in 12 (36%). Overall, no significant difference was noted in the laboratory parameters before and after treatment with rifampicin. In the 21 patients with Alagille's syndrome, however, a significant decrease in alkaline phosphatase was seen before and after one and six months of starting treatment. No adverse side effects were seen. Rifampicin appears to be effective in the treatment of refractory pruritus. A prospective study is warranted to assess further the effect of rifampicin treatment in children with hepatic cholestasis.
肝内胆汁淤积引起的瘙痒被认为是血清胆汁酸浓度过高所致。利福平可抑制肝细胞对胆汁酸的摄取,已被用于治疗瘙痒。为确定利福平治疗难治性瘙痒的疗效,我们回顾性分析了33例慢性胆汁淤积性肝病患儿(中位年龄25个月,范围4 - 135个月;19例男孩)的病历,其中21例患有阿拉吉耶综合征,8例患有进行性肝内胆汁淤积,1例患有肝外胆道闭锁,1例患有胆汁酸代谢先天性缺陷,1例患有隐源性肝硬化。利福平的中位剂量为5(4 - 10)mg/kg/天。中位摄入持续时间为36(4 - 120)周。5例(15%)患者瘙痒完全缓解,12例(36%)部分缓解。总体而言,利福平治疗前后实验室参数无显著差异。然而,在21例阿拉吉耶综合征患者中,开始治疗1个月和6个月后碱性磷酸酶显著下降。未观察到不良副作用。利福平似乎对难治性瘙痒有效。有必要进行前瞻性研究以进一步评估利福平治疗对肝内胆汁淤积患儿的效果。