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Oral Cholic Acid Is Efficacious and Well Tolerated in Patients With Bile Acid Synthesis and Zellweger Spectrum Disorders

OBJECTIVES: Patients with bile acid synthesis disorders (BASDs) due to single enzyme defects (SEDs) or Zellweger spectrum disorders (ZSDs) accumulate hepatotoxic atypical bile acids resulting in potentially fatal progressive liver disease. We evaluated the efficacy and safety of oral cholic acid in...

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Bibliografski detalji
Izdano u:J Pediatr Gastroenterol Nutr
Glavni autori: Heubi, James E., Bove, Kevin E., Setchell, Kenneth D.R.
Format: Artigo
Jezik:Inglês
Izdano: Lippincott Williams & Wilkins 2017
Teme:
Online pristup:https://ncbi.nlm.nih.gov/pmc/articles/PMC5559188/
https://ncbi.nlm.nih.gov/pubmed/28644367
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1097/MPG.0000000000001657
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