Rare Diseases Symptoms Automatic Extraction

Benign recurrent intrahepatic cholestasis with secondary renal impairment treated with extracorporeal albumin dialysis.

[benign recurrent intrahepatic cholestasis]

Benign recurrent intrahepatic cholestasis (BRIC) is a rare autosomal recessive condition characterized by intermittent episodes of pruritus and jaundice that may last days to months. Treatment is often ineffective and symptoms, particularly pruritus, can be severe. Extracorporeal albumin dialysis (molecular adsorbent recycling system, MARS) is a novel treatment which removes albumin bound toxins including bilirubin and bile salts. We describe a case of a 34-year-old man with BRIC and secondary renal impairment who, having failed standard medical therapy, was treated with MARS. The treatment immediately improved his symptoms, renal and liver function tests and appeared to terminate the episode of cholestasis. We conclude that MARS is a safe and effective treatment for BRIC with associated renal impairment.

Diseases presenting "intermittent episodes" symptom

  • alexander disease
  • benign recurrent intrahepatic cholestasis

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