Administration of IV BCAA-free solution at the beginning of a DE could reverse depletion of the amino acids that compete with BCAAs for the LAT1 transporter, and the observed depletion of alanine, despite IV alanine supplementation. The duration of administration ranged from 3–20 days. Leucine normalization was achieved in all cases with resolution or improvement of clinical symptoms following IV BCAA-free solution. Results: We evaluated the use of BCAA-free solution in 5 DEs in 5 MSUD pediatric patients, all with significantly elevated plasma leucine levels at admission (699–3296 μmol/L) and in 1 episode of risk of DE due to surgery. Clinical evolution, amino acid profile and adverse effects were evaluated. Methods: This pediatric series discusses the management of DEs in MSUD patients with IV BCAA-free solution, as an emergency treatment for DEs or as a prophylactic in cases requiring surgery. Intravenous (IV) administration of BCAA-free solution could represent a powerful alternative for emergency treatment of decompensations. These episodes require immediate intervention to prevent irreversible neurological damage. 5Metabolic Diseases Unit, Neurology Department, CIBERER and MetabERN, Sant Joan de Déu Hospital, Barcelona, Spainīackground: 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 glutamine synthesis. 4MetabERN, European Reference Network for Rare Hereditary Metabolic Disorders, Udine, Italy.3CIBERER, Instituto Salud Carlos III, Madrid, Spain.2IDIS-Health Research Institute of Santiago de Compostela, Santiago de Compostela, Spain.1Diagnosis and Treatment Unit of Congenital Metabolic Diseases, Department of Paediatrics, University Clinical Hospital of Santiago de Compostela, Santiago de Compostela, Spain. Paula Sánchez-Pintos 1,2,3,4 Silvia Meavilla 4,5 María Goretti López-Ramos 4,5 Ángeles García-Cazorla 4,5 Maria L.
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