Walensi Mikolaj, Canbay Ali, Witzke Oliver, Gerken Guido, Kahraman Alisan
Department of Gastroenterology and Hepatology, University Hospital Essen, Essen, Germany.
Case Rep Gastroenterol. 2012 May;6(2):550-6. doi: 10.1159/000342348. Epub 2012 Aug 23.
Summerskill-Walshe-Tygstrup syndrome is a rare benign chronic liver disease characterized by recurring cholestasis with jaundice and severe pruritus. Due to insufficient conservative treatment, liver dialysis by Prometheus(®) was applied to a 45-year-old female patient with resistant pruritus. Initially, other possible liver diseases were excluded and the patient was treated symptomatically since the diagnosis of Summerskill-Walshe-Tygstrup was stated in 1998. As conservative and endoscopic methods progressively failed to relieve the patient's suffering, Prometheus(®) liver dialysis was performed regularly since 2006 at 3-month intervals and successfully led to a decrease in the patient's symptoms. Cholestatic liver enzymes and also serum bile acids could be lowered significantly from an average of 22.5 ± 2.7 to 7.3 ± 1.7 µmol/l. Consequently, Prometheus(®) liver dialysis may be a beneficial option for patients with benign recurrent intrahepatic cholestasis suffering from therapy-resistant symptoms and may be used as well as other extracorporeal liver support devices which have already been reported to improve cholestatic pruritus.
萨默斯克尔-沃尔什-泰格斯特鲁普综合征是一种罕见的良性慢性肝病，其特征为黄疸和严重瘙痒反复发作的胆汁淤积。由于保守治疗效果不佳，对一名45岁患有顽固性瘙痒的女性患者采用了普洛米修斯（®）肝脏透析治疗。最初，排除了其他可能的肝脏疾病，自1998年确诊萨默斯克尔-沃尔什-泰格斯特鲁普综合征以来，对该患者进行了对症治疗。随着保守治疗和内镜治疗逐渐无法缓解患者的痛苦，自2006年起定期每3个月进行一次普洛米修斯（®）肝脏透析，成功减轻了患者的症状。胆汁淤积性肝酶以及血清胆汁酸水平从平均22.5±2.7显著降至7.3±1.7µmol/l。因此，普洛米修斯（®）肝脏透析对于患有治疗抵抗性症状的良性复发性肝内胆汁淤积患者可能是一种有益的选择，并且可以与其他已报道能改善胆汁淤积性瘙痒的体外肝脏支持设备一起使用。
