Treatment of congenital hyperammonemias

A rapid recognition of congenital hyperammonemia, a clear diagnostic workup and institution of a combined treatment without delay, by restriction of nitrogen supply, adequate caloric supply, substitution of missing metabolites, and use of alternate routes of nitrogen excretion will help to control hyperammonemic crises and improve the prognosis. For long-term treatment the use of essential amino acid mixtures and perhaps of antiserotoninergic agents is needed.