<qa>  What is the current evidence supporting the use of carglumic acid for chronic management of patients withThere is no current evidence supporting the use of carglumic acid for the chronic management of patients with OA. The investigators are proposing a randomized multicentre prospective clinical trial to evaluate long-term effects of the use of Carbaglu 50mgkgday combined with standard chronic therapy in patients with PA and MMA compared to standard chronic therapy alone. 