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 |