Treatment of adult-type citrullinemia with administration of citrate.

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Abstract
A 48 yr old man who showed a regular diurnal fluctuation of blood ammonia level was diagnosed as adult-type citrullinemia with quantitative estimation of plasma amino acids and urea cycle enzymes in the liver. The restriction of daily protein intake < 50 g and oral administration of lactulose had no effect on the blood ammonia level. The efficacy of 4 amino acid solutions which were used to improve the hepatic encephalopathy was compared in this case. Glutamate-arginine mixture had the same effect as glutamate solution in lowering the blood ammonia level and glutamate was essential. Oral administration of citrate showed a remarkable effect almost comparable to i.v. administration of glutamate. Analysis of plasma amino acids 3 h after citrate administration increased glutamate and decreased citrulline. A metabolic pathway of citrate .sbd. .alpha.-ketoglutarate .sbd. glutamate .sbd. glutamine may take part in disposing free ammonia in the blood.

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