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

Autor: Jean-Meidi Alili, Marie-Pierre Berleur, Marie-Caroline Husson, Karine Mention, Manuel Schiff, Jean-Baptiste Arnoux, Anaïs Brassier, Anne-Sophie Guemman, Coraline Grisel, Sandrine Dubois, Marie-Thérèse Abi-Wardé, Christine Broissand, Aude Servais, Myriam Dao, Pascale de Lonlay

Publikováno v: Orphanet Journal of Rare Diseases, Vol 17, Iss 1, Pp 1-12 (2022)

Abstract 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 patien

Externí odkaz: https://doaj.org/article/8f59208a49e64da6bb37d62f57cb423c

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.

Autor: Alili, Jean-Meidi^1,2 (AUTHOR) jean-meidi.alili@aphp.fr, Berleur, Marie-Pierre¹ (AUTHOR), Husson, Marie-Caroline¹ (AUTHOR), Mention, Karine^3,4 (AUTHOR), Schiff, Manuel^4,5 (AUTHOR), Arnoux, Jean-Baptiste^4,5 (AUTHOR), Brassier, Anaïs^4,5 (AUTHOR), Guemman, Anne-Sophie^4,5 (AUTHOR), Grisel, Coraline^4,5 (AUTHOR), Dubois, Sandrine^4,5 (AUTHOR), Abi-Wardé, Marie-Thérèse^4,5 (AUTHOR), Broissand, Christine⁶ (AUTHOR), Servais, Aude⁷ (AUTHOR), Dao, Myriam⁷ (AUTHOR), de Lonlay, Pascale^4,5 (AUTHOR)

Publikováno v: Orphanet Journal of Rare Diseases. 5/16/2022, Vol. 17 Issue 1, p1-12. 12p.