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Ketone body therapy with D/L-β-hydroxybutyric acid solution in severe MADD

OBJECTIVES: Multiple acyl-CoA dehydrogenase deficiency (MADD) is a severe inborn disorder of mitochondrial fatty acid oxidation. The only treatment option for MADD is the use of exogenous ketone bodies, like sodium β-hydroxybutyrate (NaβHB). However, the use of ketone body salts leads to a high inta...

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Detalles Bibliográficos
Autores principales: Fischer, Tobias, Elpers, Christiane, Och, Ulrike, Fobker, Manfred, Marquardt, Thorsten
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6610240/
https://www.ncbi.nlm.nih.gov/pubmed/31312603
http://dx.doi.org/10.1016/j.ymgmr.2019.100491