Table 1 Clinical and biochemical parameters for patients with MHPA and PKU; BMI body mass index, WC waist circumference, AC arm circumference, BP blood pressure

Number patients

41

100

Gender – Male

20

47

Gender – Female

21

53

Average Age

9y 7 m

17y 10 m

Under 18 years

35

57

zBMI – obese + overweight

3

34

zBMI – normal

33

61

zBMI – underweight

5

5

WC - obese + overweight

7

45

WC - normal

30

50

WC - underweight

4

5

AC - obese + overweight

5

21

AC - normal

21

27

AC - underweight

4

15

Early Diagnostic

40

85

BH4 treatment

0

17

Adequated Diet adherence

40

80

Phe Tol (mg/day)

1889.6 ± 831.9

481.1 ± 376.3

Phe median (μmol/L)

238.6 ± 93.2

397.3 ± 281.9

Total Cholesterol (mg/dL)

164.2 ± 21.9

149.9 ± 31.5

HDL (mg/dL)

55.8 ± 12.6

49.8 ± 11.9

LDL (mg/dL)

92.1 ± 17.2

82.1 ± 24.7

ApoA (mg/dL)

160.7 ± 26.4

144.1 ± 25.9

ApoB (mg/dL)

75.5 ± 17.6

65.9 ± 19.1

LDL/ApoB

1.26 ± 0.16

1.22 ± 0.19

Triglycerids (mg/dL)

79.2 ± 36.2

91.7 ± 45.7

Homocystein (μmol/L)

5.9 ± 2.4

5.9 ± 3.3

Systolic BP (mmHg)

105.5 ± 12

114.9 ± 14.4

Diastolic BP (mmHg)

65.7 ± 10.5

69.3 ± 13.7

B12 (pg/mL)

584.9 ± 205.6

664.5 ± 333.6

Zinc (μg/dL)

82.4 ± 33.1

81.2 ± 21.5

Copper (μg/dL)

86 ± 22.5

78.4 ± 21.3

Zinc/Copper

1.01 ± 0.46

1.11 ± 0.45