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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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Detalhes bibliográficos
Publicado no:J Pediatr Gastroenterol Nutr
Main Authors: Heubi, James E., Bove, Kevin E., Setchell, Kenneth D.R.
Formato: Artigo
Idioma:Inglês
Publicado em: Lippincott Williams & Wilkins 2017
Assuntos:
Acesso em linha: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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