CitrulinemiaClinical, laboratory and follow up feolures of three patients whith Citrulinemia lhat was identified at ages three months, seven days and seven months are described. AH Ihese palients had abnormal blood amonia (> 200 JJg/dl] and their serum citruline concentrations were 353, 1 759, 289 nn/ml respectively. Nutritional treatment induced a low protein, high calories diet with L-carnitine (100 mg x kg x day), L-arginine [70 to 1 20 mg x kg x day), together with mineral and vitamine supplements. Earlier clinical symptoms are related to more severe argininosuccinale synthetase deficiency and to worse biochemical abnormalities and clinical course.Only one of our palients had ihe acute neonatal form of ihe disease and he was also the one that had the highest blood and urine citruline levels.(