Long-term Efficacy of Carglumic Acid in Organic Acidemia.

"A. Study period: 36 months; treatment period: 12 months.~B. Patients will receive Carglumic Acid (Carbaglu®) 50 mg/kg/day in addition to standard therapy (protein restricted diet, L-carnitine, metronidazole and vitamin B12).~C. Patients need to regularly come back for follow up examination on Day 0, month 3, month 6, and month 12.~D. Patients should report any adverse event that occur during treatment period.~E. Patients' number of emergency visits due to hyperammonemia, ammonia level, acylcarnitine, urinary organic acid analysis, plasma amino acid analysis and methylmalonic acid will be recorded during treatment period."