AI Article Synopsis
- Benign recurrent intrahepatic cholestasis (BRIC) is a genetic liver disorder causing episodes of jaundice and itching that can last from a week to several months and typically resolve on their own.
- A case study highlights a male patient with BRIC who experienced three episodes of itching and jaundice between ages 14 and 19, with no effective pharmacological treatment during the last episode.
- A procedure called fractionated plasma separation and absorption (FPSA) was successfully used to treat severe pruritus and jaundice, providing immediate relief and lasting improvement over a five-year follow-up period.
Article Abstract
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.
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| http://dx.doi.org/10.1111/hepr.12262 | DOI Listing |
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