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Intravenous administration of a branched-chain amino-acid-free solution in children and adults with acute decompensation of maple syrup urine disease: a prospective multicentre observational study

BACKGROUND: Patients with maple syrup urine disease (MSUD) experiencing metabolic decompensations have traditionally been treated with branched-chain amino acid (BCAA)-free mixture via oral or nasogastric administration routes. In some patients, enteral administration is not possible, either because...

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Detalles Bibliográficos
Autores principales: Alili, Jean-Meidi, Berleur, Marie-Pierre, Husson, Marie-Caroline, Mention, Karine, Schiff, Manuel, Arnoux, Jean-Baptiste, Brassier, Anaïs, Guemman, Anne-Sophie, Grisel, Coraline, Dubois, Sandrine, Abi-Wardé, Marie-Thérèse, Broissand, Christine, Servais, Aude, Dao, Myriam, de Lonlay, Pascale
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9112564/
https://www.ncbi.nlm.nih.gov/pubmed/35578286
http://dx.doi.org/10.1186/s13023-022-02353-2