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Table 2 Mean values and ranges (μmol/L) of plasma metabolites in UCD patients

From: Urea cycle disorders in Argentine patients: clinical presentation, biochemical and genetic findings

Disease

Ammonia

Glutamine

Citrulline

Arginine

Argininosuccinate

Control

< 100

333–809

16–32

44–120

0–0.7

OTCD

778.1 (98–2181)

2065.44 (277–4229)

14.6 (0–28)

66.0 (8–135)

ASSD

1017.1 (399–1600)

2863.24 (1123-4504)

3203.8 (758–8500)

12.9 (8–25.2)

ASLD

478.3 (133–1035)

1346.32 (374–2913)

222.3 (75–357)

10.7 (4–23)

80.7 (37–179)

  1. Control: Reference values and ranges for pediatric population (> 1 year old) [10]