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Table 2 Comparison of amino acids among the groups

From: The value of simultaneous determination of blood large neutral amino acids and tetrahydrobiopterin metabolites in the diagnosis of atypical hyperphenylalaninemia

AA

Stat

Classic PKU, n = 46

Atypical PKU, n = 14

Control, n = 50

KW P-value

Phe (μm/L)

M ± SD

793 ± 415

145 ± 50

75 ± 13

 

Min-Max

300–2211

99.7–250

40–92.5

 

Median (IQR)

748 (465)a,c

115 (68)b,c

78 (14)

< 0.05

Tyr (μm/L)

M ± SD

48 ± 14

40 ± 13

56 ± 12

 

Min-Max

23–85

23–65

30–75.6

 

Median (IQR)

46 (25)a,c

39 (22)b,c

59 (18)

< 0.05

P/T ratio

M ± SD

17 ± 8

4 ± 2

1 ± 0

 

Min-Max

4.1–36.2

2.1–10.2

1–1.7

 

Median (IQR)

16 (10)a,c

4 (2)b,c

1 (0)

< 0.05

Cit

Mean ± SD

69.03 ± 33.39

37.01 ± 3.03

16.04 ± 9.71

 

Min-Max

32–188

32–42

2–34

 

Median (IQR)

62.5 (41)a,b,c

37 (5)

13.2 (13)

< 0.05

Orn

Mean ± SD

201 ± 88

101 ± 67

145 ± 81

 

Min-Max

33–348

23–230

29–321

 

Median (IQR)

213 (151)a,b

103.5 (100)

138.5 (115)

< 0.05

Met

Mean ± SD

10.03 ± 6.71

2.61 ± 1.93

6.84 ± 5.29

 

Min-Max

1–23

0.2–8

1.7–23

 

Median (IQR)

8.5 (11.87)a,b,c

2 (1.3)

4.95 (3.4)

< 0.05

Arg

Mean ± SD

3.15 ± 1.62

5.19 ± 1.53

17.63 ± 10.16

 

Min-Max

0–8.2

2.7–7.3

3.8–34

 

Median (IQR)

2.8 (2.6)b

5.2 (2.1)a,c

17.45 (20)

< 0.05

Val

Mean ± SD

32.89 ± 16.47

25.91 ± 20.36

76.43 ± 47.82

 

Min-Max

12–65

2–76

3–192

 

Median (IQR)

30 (23)b,c

22.2 (25)

66 (59)

< 0.05

Leu

Mean ± SD

19.82 ± 6.29

51.14 ± 6.99

154.82 ± 53.60

 

Min-Max

3–32

39–63

56–249

 

Median (IQR)

21 (8)b

52 (10)a,c

157.5 (93)

< 0.05

  1. Non-parametric, Kruskal-Wallis, and Mann-Whitney tests were used to detect the difference across the studied groups, with a P-value < 0.05 indicating a statistically significant difference in the median rank presented as median (IQR). Other parametric descriptive statistics such as mean, standard deviation, minimum, maximum, and range are also displayed in the table
  2. Phe phenylalanine, Tyr tyrosine, Phe/Tyr phenylalanine and tyrosine ratio, Cit citrulline, Orn ornethine, Met methionine, Arg arginine, Val valine, Leu leucine, IQR interquartile range, SD standard deviation, Min minimum, Max maximum
  3. aP < 0.05 in classic PKU vs. control
  4. bP < 0.05 atypical PKU vs. control
  5. cP < 0.05 classical PKU vs. atypical PKU