Stratégie thérapeutique pour la prise en charge des hyperammoniémies

Hyperammonemia is a metabolic disorder that could induce neurological side effects, sometimes leading to death of the patient, when an effective treatment is not quickly undertaken. This is particularly critical as diagnosis may be delayed because of various etiologies and lack of specific clinical signs. The etiology and form (acute or chronic) of hyperammonemia determine the type of treatment. For acute hyperammonemia, dialysis, with intravenous sodium benzoate and/or sodium phenylacetate is preferred. Whereas for chronic hyperammonemia, a low-protein diet may be sufficient to control ammonemia level. In more severe case, oral intake of sodium benzoate and/or sodium phenylacetate is needed. Arginine or carnitine supply could be considered in case of hyperammonemia due to parenteral nutrition or sodium valproate treatment.