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Cholic acid for primary bile acid synthesis defects: a life-saving therapy allowing a favorable outcome in adulthood
BACKGROUND: Oral cholic acid (CA) replacement has been shown to be an effective therapy in children with primary bile acid synthesis defects, which are rare and severe genetic liver diseases. To date there has been no report of the effects of this therapy in children reaching adulthood. The aim of t...
Autores principales: | Gonzales, Emmanuel, Matarazzo, Lorenza, Franchi-Abella, Stéphanie, Dabadie, Alain, Cohen, Joseph, Habes, Dalila, Hillaire, Sophie, Guettier, Catherine, Taburet, Anne-Marie, Myara, Anne, Jacquemin, Emmanuel |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BioMed Central
2018
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6206929/ https://www.ncbi.nlm.nih.gov/pubmed/30373615 http://dx.doi.org/10.1186/s13023-018-0920-5 |
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