Table 2. Urinary excretion levels (μM/g of creatinine) of branched amino acids of the patients.

Patients	Cystine	Ornithine	Lysine	Arginine	NP test
Patient 1	7,036	2,332	11,813	7,740	n.d.
Patient 2	14,875	2,760	21,533	4,058	n.d.
Patient 3	2,778	1,436	9,884	1,101	n.d.
Patient 4	5,358	7,849	14,910	9,746	n.d.
Patient 5	276	1,074	8,532	3,342	n.d.
Patient 6	2,893	1,304	3,028	2,687	Positive
Mother	156	0	162	175	Positive
Father	0	0	196	0	Negative
Patient 7	8,230	9,367	41,367	20,151	n.d.
Patient 8	2,881	369	1,360	1,051	Positive
Mother	60	0	0	0	Negative
Father	58	0	284	24	Negative
Sister	201	0	0	0	Negative

Reference values: Cystine 27–150 μM/g creatinine, Ornithine 0–44 μM/g creatinine, Lysine 62–513 μM/g creatinine, Arginine 0–44 μM/g creatinine.

NP test = cyanide nitroprusside test, n.d. = not done.