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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...
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 |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BioMed Central
2022
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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 |
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