[Hyperammonemia: a suggestion for diagnostic and therapeutic procedures]

Hyperammonemia in pediatrics leads to emergency situations. Adequate decisions for treatment have to be taken rapidly. These depend on the underlying disorder. While the patient's history and symptoms should lead to a search for hyperammonemia, the further steps will depend on biochemical results of aminoacid determinations in plasma and urine, and orotic acid excretion. In some cases where this latter metabolite is not increased, the determination of organic acids in urine and enzyme assays in liver are needed. A scheme for the diagnostic and therapeutic measures is proposed.