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For a substance or method to be added to the List, it must be determined that it meets at least two of the following three criteria: It has the potential to enhance or enhances sport performance It represents an actual or potential health risk to the athletes It violates the spirit of sport The List is released three months ahead of it taking effect so that athletes, their entourage, and other stakeholders can acquaint themselves with any modifications

1 Introduction Hyperammonemia is a common complication of neonatal organic acidemia, with approximately 61.5% of patients developing hyperammonemia ( Carglumic acid, a structural analog of N-acetylglutamic acid, was granted approval in Europe in 2003 for the treatment of inherited metabolic disorders, including N-acetylglutamate synthase deficiency and organic acidemia