Table 3 Overall metabolic control, nutritional intake, anthropometry and body composition of the 11 patients with PKU taking L-AA versus CGMP-AA

Metabolic control

 Median blood Phe (µmol/L)

11

562 ± 289

628 ± 317

0.065

 Median blood Tyr (μmol/L)

11

52.0 ± 19.2

61.4 ± 23.8

0.027

 Median blood Phe/Tyr ratio

11

15.1 ± 10.8

12.0 ± 8.8

0.379

Nutritional intake

 Natural protein intake (g/kg/day)

11

0.41 (0.26–0.62)

0.34 (0.21–0.69)

0.657

 Protein substitute (g/kg/day)

11

0.86 ± 0.24

0.74 ± 0.23

0.126

 Protein substitute (g/day)

11

50.8 ± 16.3

44.6 ± 12.8

0.118

 Phe intake (mg/day)

11

885 (751–1787)

978 (658–1370)

0.721

 Phe intake from protein substitute (mg/day)

11

0

43.5 ± 17.4

–

 Tyr intake from protein substitute (g/day)

11

5.18 ± 1.77

4.22 ± 1.59

0.145

 Leu intake from protein substitute (g/day)

11

5.85 (4.49–7.61)

7.97 (6.85–8.78)

0.075

 Protein (%)

11

13.8 ± 2.25

13.6 ± 2.25

0.696

 Fat (%)

11

26.9 ± 5.0

27.6 ± 4.2

0.503

 CHO (%)

11

57.0 (52.1–60.1)

58.5 (55.8–60.2)

0.248

 Energy (kcal/day)

11

2277 ± 551

2238 ± 491

0.793

Anthropometry and body composition

 Weight (kg)

11

60.4 ± 15.2

63.4 ± 13.2

0.064

 Height (cm)

11

158.6 ± 6.4

160.6 ± 9.8

0.341

 WC (cm)

11

85.1 ± 15.2

86.2 ± 14.3

0.536

 BMI (kg/m²) *

9

24.3 ± 6.2

25.1 ± 5.6

0.095

 Body fat (kg)

9

17.3 ± 13.5

19.5 ± 12.3

0.056

 Body fat (%)

9

25.5 ± 16.1

28.9 ± 13.4

0.126

 Lean mass (%)

9

74.5 ± 16.1

71.1 ± 13.4

0.126

 Phase angle (°)

9

6.8 ± 0.7

6.8 ± 0.6

0.880