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Table 2 Number of patients with hyperphenylalaninaemia with altered biochemical concentrations of lipid profile and other related parameters

From: Lipid profile status and other related factors in patients with Hyperphenylalaninaemia

 

TC (<120 mg/dL)

TC (>200 mg/dL)

TGC(>100 mg/dL below 10 years of age >130 mg/dL above 10 years of age)

TGC (<30 mg/dL)

HDL-C (<40 mg/dL)

LDL-C (>130 mg/dL)

ApoA (<115 mg/dL)

ApoB (>110 mg/dL)

tHcy. (<5 μmol/L)

tHcy. (>15 μmol/L)

Zn (<65 μg/dL)

Cu (<65 μg/dL)

Cu (>140 μg/dL)

B12 (>1900 pg/mL)

hsCRP (>1.1 mg/dL)

over total of patients N (%)

19 (13.47)

6 (4.25)

26(18.44)

2(1.42)

17 (12.06)

4 (2.84)

6(4.25)

4(2.84)

58(41.13)

2(1.42)

28(19.86)

31(21.98)

3(2.13)

2(1.42)

5(3.55)

Age (below 18 years of age) N (%)

14 (73.68)

4 (66.67)

10(38.46)

1(50)

9(52.94)

1(25)

6(100)

2(50)

50(86.2) p = 3.18e−4

0(0)

23(82.14)

17(54.84)

0(0)

0(0)

0(0)

Gender (Male) N (%)

12(63.16)

3(50)

9(34.62)

0(0)

10(58.82)

1(25)

4(66.67)

2(50)

26(44.83)

2(100)

10(35.71)

12(38.7)

0(0)

0(0)

1(20)

PKU patients N (%)

19 (100) p = 0.0125

2(33.33)

21(80.77)

2(100)

14(82.35)

3(75)

5(83.33)

2(50)

42(72.41)

2(100)

19(67.86)

26(83.87)

1(33.33)

2(100)

5(100)

6R-BH4 therapy N (%)

1 (5.26)

0(0)

0(0)

1(50)

0(0)

0(0)

1(16.67)

0(0)

9(15.52)

0(0)

3(10.71)

4(12.9)

0(0)

0(0)

2(40)

Adherence to treatment (adequate) N (%)

16 (84.21)

6(100)

22(84.62)

1(50)

14(82.35)

3(75)

5(83.33)

4(100)

50 (86.2)

1(50)

26(92.86)

26(83.87)

3(100)

2(100)

4(80)

Phe tolerance (under 500 mg/day) N (%)

18 (94.73) p = 2.29e−4

3(50)

22(84.62)

2(100)

13(76.47)

2(50)

5(83.33)

3(75)

24(41.38)

0(0)

8(28.57)

22(70.97)

1(33.33)

0(0)

3(60)

  1. *p-values were obtained using Fisher’s exact test and adjusted by Benjamini-Hochberg’s correction to determine statistically significant differences between patients with deficient micronutrient concentrations andpatients in the normal range according to: age (below/above 18 years of age), gender (male/female), diagnosis (PKU / MHPA), 6R-BH4 therapy (yes/no), adherence to dietary treatment (adequate/inadequate) and Phe tolerance (below/above 500 mg/day). Statistically significant differences are shown in the Table (p < 0.05). N number of patients, % percentage of patients, TC total cholesterol, TGC triglycerides, HDL-C high-density lipoprotein cholesterol, LDL-C low- density lipoprotein cholesterol, ApoA apolipoprotein A, ApoB apolipoprotein B, tHcys total homocysteine, Zn Zinc, Cu Cupper, B 12 vitamin B12, hsCRP high sensitivity C-Reactive Protein, PKU phenylketonuria, MHPA mild hyperphenylalaninaemia, Phe phenylalanine