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(Normal range: 0.2-0.8 mg/ml (12-47 mmol/L

Comment: Ammonia and alpha- ketoglutarate with NADH yield glutamate; glutamate and ammonia yield glutamine. Cerebrospinal fluid glutamine levels are useful in hepatic encephalopathy and with Reye syndrome. In the HHH syndrome, hyperammonemia is intermittent; it presents in infancy often, but symptoms can be delayed. Metabolic acidosis with ketosis are found in organic acidemias, in which hyperammonemia is found. Plasma amino acids, urine organic acids, and amino acids are indicated with NH3, biotinidase, and Carnitine. Recently, hyperinsulinaemic hypoglycaemia with persistent hyperammonia has been described. This disorder is not associated with any of the abnormalities of amino acids or organic acids observed in urea cycle enzyme defects, and is thought to be due to mutations in glutamate dehydrogenase

Sample: EDTA, vacutainer, ice, deliver immediately