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

@article{Saich2005BenignRI,
  title={Benign recurrent intrahepatic cholestasis with secondary renal impairment treated with extracorporeal albumin dialysis},
  author={Rebecca Saich and Peter Collins and Aftab Ala and Richard A Standish and Humphrey Julian Francis Hodgson},
  journal={European Journal of Gastroenterology \& Hepatology},
  year={2005},
  volume={17},
  pages={585-588}
}
Benign recurrent intrahepatic cholestasis (BRIC) is a rare autosomal recessive condition characterized by intermittent episodes of pruritis and jaundice that may last days to months. Treatment is often ineffective and symptoms, particularly pruritis, 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… 
Treatment of pruritus with Prometheus dialysis and absorption system in a patient with benign recurrent intrahepatic cholestasis
TLDR
F fractionated plasma separation and absorption (FPSA) immediately alleviated pruritus, lowered serum bilirubin concentration and induced sustained remission in the 5‐year follow up, and FPSA seems to be a safe and effective way of treatment for BRIC in patients with severePruritus and prolonged jaundice.
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Both rifampicin and plasmapheresis represent important therapeutic options of acute cholestatic attacks in patients with BRIC and may be the first choice as a noninvasive treatment, according to patients suffering from BRIC.
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The Mystery of Episodic Recurrent Jaundice in a Young Male: Cholestasis With a Normal Gamma-Glutamyl Transferase
TLDR
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Progressive familial intrahepatic cholestasis : Clinical, biochemical, genetic and histopathological aspects
TLDR
It is concluded that PFIC encompasses not one, but several cholestatic diseases, all caused by different defects in the formation of bile, and most children with PFIC caused by mutations in ABCB11 who undergo PEBD have a favorable long-term prognosis including histological improvement and long- term survival without the need for liver transplantation.
Long-Term Therapy of a Patient with Summerskill-Walshe-Tygstrup Syndrome by Applying Prometheus® Liver Dialysis: A Case Report
TLDR
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.
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