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Case Rep Gastroenterol. 2012 May;6(2):550-6. doi: 10.1159/000342348. Epub 2012 Aug 23.

Long-term therapy of a patient with summerskill-walshe-tygstrup syndrome by applying prometheus® liver dialysis: a case report.

Case reports in gastroenterology

Mikolaj Walensi, Ali Canbay, Oliver Witzke, Guido Gerken, Alisan Kahraman

Affiliations

  1. Department of Gastroenterology and Hepatology, University Hospital Essen, Essen, Germany.

PMID: 22949896 PMCID: PMC3433004 DOI: 10.1159/000342348

Abstract

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.

Keywords: Benign recurrent intrahepatic cholestasis; Cholestasis; Prometheus® dialysis; Summerskill-Walshe-Tygstrup syndrome

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