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Table 2 Biochemical data at diagnosis (excluding asymptomatic patients) and at last visit (all patients)

From: Urea cycle disorders in Spain: an observational, cross-sectional and multicentric study of 104 cases

Biochemical data *

At clinical onset (N = 91)

At last visit (N = 96)

Normal values

Ammonia (μmol/L)

298 (169-615)

33 (27-42)

10-55 (80**)

Glutamine (μmol/L)

1124 (859-1546)

716 (545-908)

350-650

Prothrombin activity (%)

60 (32.6-82)

91 (85-100)

75-130

ALT (IU/L)

76 (38-159)

24 (16-34)

5-39

AST (IU/L)

53.5 (36.7-161)

27.5 (19-37.2)

5-37

Bilirrubin (mg/dL)

0.60 (0.43-3.77)

0.53 (0.30-0.80)

0.20-1.10

Albumin (g/L)

3.79 (3-4.41)

4.30 (3.90-4.50)

3.80-5.40

pH

7.43 (7.36-7.46)

-

7.35-7.45

Bicarbonate (mmol/L)

21 (19-24)

-

23-27

  1. *Median and interquartile range (IQR); **Normal values in newborns.