Intravenous branched-chain amino-acid-free solution for the treatment of metabolic decompensation episodes in Spanish pediatric patients with maple syrup urine disease

BACKGROUND: Metabolic decompensation episodes (DEs) in Maple Syrup urine disease (MSUD) result in brain accumulation of toxic branched-chain amino acids (BCAAs) and their respective branched-chain α-keto acids that could induce neuroinflammation, disturb brain bioenergetics, and alter glutamate and...

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Detalles Bibliográficos
Autores principales: Sánchez-Pintos, Paula, Meavilla, Silvia, López-Ramos, María Goretti, García-Cazorla, Ángeles, Couce, Maria L.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2022
Materias:
Pediatrics
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9420908/
https://www.ncbi.nlm.nih.gov/pubmed/36046474
http://dx.doi.org/10.3389/fped.2022.969741

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