Rare Diseases Symptoms Automatic Extraction

Treatment of pruritus with Prometheus dialysis and absorption system in a patient with benign recurrent intrahepatic cholestasis.

[benign recurrent intrahepatic cholestasis]

Benign recurrent intrahepatic cholestasis (BRIC) is an autosomal recessive liver disorder characterized by recurrent episodes of jaundice and itching. Episodes of cholestasis last variously from 1 week to several months, may start at any age and usually resolve spontaneously. No effective treatment has been found as yet. We report a case of genetically proven BRIC in a male patient who developed three episodes of pruritus and jaundice at the age of 14, 16 and 19 years. During the third episode, he did not respond to pharmacological medical therapy, and fractionated plasma separation and absorption (FPSA, Prometheus) was performed to manage intractable pruritus. The treatment immediately alleviated pruritus, lowered serum bilirubin concentration and induced sustained remission in the 5-year follow up. FPSA seems to be a safe and effective way of treatment for BRIC in patients with severe pruritus and prolonged jaundice.

Diseases presenting "recurrent episodes" symptom

  • acute rheumatic fever
  • adrenomyeloneuropathy
  • benign recurrent intrahepatic cholestasis
  • child syndrome
  • cholangiocarcinoma
  • cystinuria
  • familial mediterranean fever
  • hydrocephalus with stenosis of the aqueduct of sylvius
  • kabuki syndrome
  • neuralgic amyotrophy
  • pyruvate dehydrogenase deficiency
  • systemic capillary leak syndrome
  • triple a syndrome

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