NAGS and argininosuccinic aciduria: The diagnosis was established in all 123 patients; 61 had citrullinemia type 1, 26 patients had OTC deficiency, 20 were of argininosuccinic aciduria (ASA), 9 had arginase deficiency, 3 had carbamoyl phosphate synthetase (CPS1), 2 had N-acetyl glutamate synthase (NAGS) deficiency and one patient each had lysinuric protein intolerance (LPI) and citrin deficiency (Table 1).