Actualización para el tratamiento de la hiperamonemia aguda en pacientes con errores innatos del metabolismo.

Acute hyperammonemia (HAA) is a medical emergency, that cause neurological damage, complications or even death. HAA occurs most frequently in patients with inborn errors of metabolism (IEM), especially in events of metabolic decompensation, and require intensive care. The IEM that mainly cause HAA include; urea cycle defects, organic acidemias, fatty acid oxidation disorders and carnitine cycle defects. In HAA, the primary aim is to preserve life and the integrity of the central nervous system, decreasing blood ammonia levels to normal, with urgent medical treatment, individualized life support and nutrition. Knowledge of the available drugs, the ammonium removal methods, as well as the energy and protein requirements, supplements, cofactors and vitamins; is very important for individualize the medical and nutritional treatment according to their biochemical, clinical profiles and underlying disease. The objective of this work is to present current concepts regarding the treatment of HAA in patients with inborn errors of metabolism. [ABSTRACT FROM AUTHOR]