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Case Report: Ursodeoxycholic acid treatment in Niemann-Pick disease type C; clinical experience in four cases
In this case series, we demonstrate that Ursodeoxycholic acid (UDCA) improves liver dysfunction in Niemann-Pick type C (NPC) and may restore a suppressed cytochrome p450 system. NPC disease is a progressive neurodegenerative lysosomal storage disease caused by mutations in either the NPC1 or NPC2 ge...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
F1000Research
2017
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5649119/ https://www.ncbi.nlm.nih.gov/pubmed/29119141 http://dx.doi.org/10.12688/wellcomeopenres.11854.1 |