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

Abstract

Carglumic acid is a well-tolerated and efficacious treatment for OA decompensation episodes. When given alone or combined with NH₃ scavengers, the reduction in NH₃ was greater than with NH₃ scavengers alone in the first 72 h.