Skip to main content
. 2021 Feb 21;59(1):69–80. doi: 10.1002/jmd2.12202

TABLE 1.

Characteristics of patients and controls

Patients (total) Classical PKU MHPA BH4‐sensitive PKU Controls P
Number (n) 49 25 10 14 98
Gender
Male (n) 26 13 5 8 52 1.000 a
Female (n) 23 12 5 6 46

Age (years)

Median [IQR]

9

[4.5; 14.0]

8

[3.5; 13.5]

8.5

[3.0; 15.3]

11.5

[6.8; 14.3]

10

[4.0; 13.3]

.768 a

BMI (kg/m 2 )

Median [IQR]

17.7

[16.2; 19.0]

17.1

[16.0; 18.8]

17.4

[16.2; 20.6]

18.3

[16.9; 19.9]

17.9

[16.1; 19.7]

.763 a

WSI‐Index b

Median [IQR]

13

[10.0; 15.0]

12

[9.0; 14.0]

12.5

[6.0; 14.5]

13

[11.8; 19]

14

[11.3; 16.0] e

.046 a
Median mean dried blood Phe concentration (μmol/L) at study visit [IQR]

213

[152; 398]

306

[138; 499]

207

[141; 212]

224

[173; 365]

n. a. .233
Median mean dried blood Phe concentration (μmol/L) 1 year prior enrollment [IQR]

231

[199; 437]

250

[215; 577]

186

[163; 213]

261

[217; 360]

n. a. .003 c

Median Phe tolerance f (mg/day)

[IQR]

450 g

[339; 1000]

400 h

[260; 480]

n. a.

1170 i

[1000; 1200]

n. a <.001 d

Note: The P values in bold letters represent significant differences (P < .05).

Abbreviations: MHPA, mild hyperphenylalaninemia; n. a, not applicable; PKU, phenylketonuria.

a

Comparison between patients (total) vs controls, Mann‐Whitney‐U‐test.

b

WSI stratification Index: lower class 3‐8, middle class 9‐14, upper class 15‐21.

c

Comparison between patients with classical PKU vs BH4‐sensitive PKU vs MHPA, Kruskal‐Wallis‐test.

d

Comparison between patients with classical PKU vs BH4‐sensitive PKU, Mann‐Whitney‐U‐test.

e

n = 88.

f

Applicable for PKU patients on diet.

g

n = 30.

h

n = 23.

i

n = 7.