Table 1.

Plasma amino acids concentrations in two untreated patients with OAT deficiency

	Glutamine (μmol/L)	Proline (μmol/L)	Citrulline (μmol/L)	Ornithine (μmol/L)	Arginine (μmol/L)	Proline/citrulline
Normal	402–776	97–254	8–36	41–129	7–128	<32
Case 1
17 days	877	1,090	4	56	14	272
38 days	1,228	1,089	<1	32	12	>1,089
Case 2
71 days		570	3	35	42	190