Rare Diseases Symptoms Automatic Extraction

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

[benign recurrent intrahepatic cholestasis]

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.

Diseases presenting "pruritus" symptom

  • benign recurrent intrahepatic cholestasis
  • cholangiocarcinoma
  • cutaneous mastocytosis
  • dystrophic epidermolysis bullosa
  • erdheim-chester disease
  • lamellar ichthyosis
  • pleomorphic liposarcoma
  • pyomyositis
  • zellweger syndrome

This symptom has already been validated