Effect of carglumic acid with or without ammonia scavengers on hyperammonaemia in acute decompensation episodes of organic acidurias

Effect of carglumic acid with or without ammonia scavengers on hyperammonaemia in acute decompensation episodes of organic acidurias

Abstract Background Hyperammonaemia is a key sign of decompensation in organic acidurias (OAs) and can contribute to severe neurological complications, thus requiring rapid treatment. Methods A post-hoc analysis of two retrospective studies analysed the efficacy of carglumic acid ± ammonia (NH3) sca...

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Bibliographic Details
Main Authors: Anupam Chakrapani, Vassili Valayannopoulos, Nuria García Segarra, Mireia Del Toro, Maria Alice Donati, Angeles García-Cazorla, María Julieta González, Celine Plisson, Vincenzo Giordano
Format: Article
Language:English
Published: BMC 2018-06-01
Series:Orphanet Journal of Rare Diseases
Subjects:
Carglumic acid
Ammonia scavengers
Organic aciduria
Hyperammonaemia
Decompensation
Extracorporeal detoxification
Online Access:http://link.springer.com/article/10.1186/s13023-018-0840-4

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