Application of carglumic acid in the prevention and treatment of acute metabolic crisis of organic acid metabolism disease
Acute metabolic crises in the patients with organic acidemias are critical conditions of inherited metabolic disorders,where the severity of hyperammonemia directly associates with the prognosis.Except for restricting protein intake to reduce ammonia generation,maintaining adequate energy supply is important to prevent exacerbation of hyperammonemia due to increased breakdown of endogenous proteins.Correction of the underlying metabolic disturbances is also necessary.Carglumic acid,a synthetic analogue of N-acetylglutamate,can increase substrates for the urea cycle,restarting the urea cycle and accelerating ammonia metabolism.This article summarizes the application of carglumic acid in the management of acute metabolic crises due to organic acidemia,providing insights for clinical treatment.
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