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Δ4-3-oxosteroid-5β-reductase deficiency: Responses to oral bile acid therapy and long-term outcomes

BACKGROUND: Disorders of primary bile acid synthesis may be life-threatening if undiagnosed, or not treated with primary bile acid replacement therapy. To date, there are few reports on the management and follow-up of patients with Δ4-3-oxosteroid 5β-reductase (AKR1D1) deficiency. We hypothesized th...

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Detalles Bibliográficos
Autores principales: Zhang, Mei-Hong, Setchell, Kenneth DR, Zhao, Jing, Gong, Jing-Yu, Lu, Yi, Wang, Jian-She
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Baishideng Publishing Group Inc 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6385010/
https://www.ncbi.nlm.nih.gov/pubmed/30809085
http://dx.doi.org/10.3748/wjg.v25.i7.859