Identifying Hyperammonemia in the Emergency Department

Children and Adults

In patients with partial enzyme deficiencies, the first recognized clinical episode may be delayed for months or years. Although the clinical abnormalities vary somewhat with the specific urea cycle disorder, a hyperammonemic episode may be marked by a spectrum of symptoms:

Loss of appetite Vomiting
Lethargy Behavioral abnormalities
Agitation Sleep disorder
Confusion Delusions, hallucinations, psychosis
Bizarre/unusual behavior Stroke-like symptoms

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