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Hyperammonemia Hyperammonemia is a metabolic disturbance characterised by an excess of ammonia in the blood. It is a dangerous condition that may lead to encephalopathy and death.
An elevated serum ammonia level, particularly with normal liver enzymes and the absence of relevant medications, should suggest an inborn error of metabolism and prompt treatment of the hyperammonemic state followed by measurement of plasma amino acid and urine orotic acid levels.