Ng V L, Ryckman F C, Porta G, Miura I K, de Carvalho E, Servidoni M F, Bezerra J A, Balistreri W F
Pediatric Liver Care Center and Division of Gastroenterology, Children's Hospital Medical Center, University of Cincinnati, Ohio 45229-3039, USA.
J Pediatr Gastroenterol Nutr. 2000 Feb;30(2):152-6. doi: 10.1097/00005176-200002000-00011.
Chronic intrahepatic cholestasis is associated with severe pruritus that is often refractory to maximal medical management and leads to significantly impaired quality of life. The hypothesis in this study was that partial external biliary diversion (PEBD) can substantially improve intractable pruritus secondary to intrahepatic cholestasis with subsequent improvement of functional quality of life.
Parents' and/or patients' clinical rating of pruritus, growth percentiles, biochemical parameters, and liver biopsies performed before and after surgery were compared in a retrospective medical record review.
Eight children underwent PEBD from 1990 through 1997. Complete follow-up data were available for seven patients. Before surgery, all patients had intense pruritus, which was not responsive to maximal medical therapy. Specimens obtained in preoperative liver biopsies showed moderate (n = 1), minimal (n = 6), or no (n = 1) portal fibrosis. After PEBD, all patients received ursodeoxycholic acid (10-15 mg/kg/dose two to three times daily) until resolution of pruritus. Of the seven patients with complete follow-up data, six had complete resolution of pruritus and sustained resolution up to 8 years after surgery. The patient with mild to moderate residual pruritus was the youngest to undergo PEBD. Growth improved from below the 5th percentile before surgery to the 5th through the 25th percentiles for five of six patients with more than 6 years' follow-up. All families reported improved quality of life, defined by school attendance and ability to resume normal activity with peers. There has been no clinical evidence of progression of liver disease.
Partial external biliary diversion is effective in the long-term treatment of pruritus refractory to medical therapy and provides a favorable outcome in a select group of patients with chronic intrahepatic cholestasis without cirrhosis.
慢性肝内胆汁淤积症常伴有严重瘙痒，即便采用最大程度的药物治疗，瘙痒往往仍难以缓解，进而导致生活质量显著下降。本研究的假设是，部分外引流术（PEBD）可显著改善肝内胆汁淤积所致的顽固性瘙痒，随后提高生活功能质量。
通过回顾性病历审查，比较手术前后父母和/或患者对瘙痒的临床评分、生长百分位数、生化指标以及肝活检结果。
1990年至1997年期间，8名儿童接受了部分外引流术。7名患者有完整的随访数据。术前，所有患者均有剧烈瘙痒，对最大程度的药物治疗无反应。术前肝活检标本显示中度（n = 1）、轻度（n = 6）或无（n = 1）门静脉纤维化。部分外引流术后，所有患者均接受熊去氧胆酸治疗（10 - 15 mg/kg/剂量，每日2 - 3次），直至瘙痒缓解。在有完整随访数据的7名患者中，6名患者的瘙痒完全缓解，术后长达8年仍持续缓解。有轻度至中度残余瘙痒的患者是接受部分外引流术年龄最小的。6名随访超过6年的患者中，有5名患者的生长情况从术前低于第5百分位数改善至第5至第25百分位数。所有家庭均报告生活质量有所改善，以上学情况和与同龄人恢复正常活动的能力来衡量。尚无肝病进展的临床证据。
部分外引流术对药物治疗难治的瘙痒具有长期治疗效果，对于一组无肝硬化的慢性肝内胆汁淤积症患者，可带来良好预后。
