Variables | Type of diagnosis | p 1 | p 2 | ||
---|---|---|---|---|---|
CPKU | MPKU | MHPA | |||
Patients N | 37 | 20 | 26 | ||
Age (y) | 24.92 ± 11.64 | 16.7 ± 7.26 | 13.23 ± 10.24 | 0.002 | 0.0006 |
Sex (F/M) | 19F / 18 M | 13F / 7 M | 17F / 9 M | 0.48 | 0.47 |
TD (E/L) | 27E / 10 L | 18E / 2 L | 25E / 1 L | 0.38 | 0.26 |
Treatment (D/P + D) | 36D / 1P + D | 11D / 9P + D | – | – | – |
BMI (kg/m2) | 25.57 ± 5.96 15Nor/22↑ | 22.12 ± 5.78 14Nor/6↑ | 18.46 ± 3.79 22Nor/4↑ | 0.0003 | 0.0004 |
WC (cm) | 87.08 ± 17.42 13Nor/24↑ | 73.96 ± 13.92 16Nor/4↑ | 64.85 ± 11.59 22Nor/4↑ | 0.002 | 0.001 |
Phe median inter- quartile range (μM). | 484(378–840) | 241.9(205–482) | 295.5(246–310) | 0.007 | 3.2e−05 |
Phe tol (mg/day) | 379 ± 148.57 | 868.9 ± 682.53 | Free diet- | – | – |
Glucose (mg/dL) | 81.24 ± 9.76 | 78.05 ± 4.12 | 82.19 ± 7.4 | 0.29 | 0.29 |
Insuline (mUI/L) | 16.22 ± 10 | 11.12 ± 5.72 | 9.03 ± 5.34 | 0.035 | 0.02 |
Peptide C (ng/mL) | 2.3 ± 1.03 | 1.54 ± 0.78 | 1.31 ± 0.72 | 0.028 | 0.002 |
Urea (mg/dL) | 21.86 ± 6.14 | 23.3 ± 6.36 | 26.15 ± 6.58 | 0.18 | 0.31 |
Fructosamine(μM) | 234.25 ± 44.36 | 222.8 ± 25.2 | 232.65 ± 32.74 | 0.7 | 0.28 |
Lactate (mM) | 1.28 ± 0.61 | 1.01 ± 0.49 | 1.29 ± 0.65 | 0.45 | 0.31 |
IGF-1 (mg/mL) | 204.77 ± 77.7 | 227.89 ± 112.34 | 193.35 ± 125.69 | 0.11 | 0.22 |
IGF-BP3 (μg/mL) | 4.68 ± 1.2 | 4.65 ± 1.39 | 4.39 ± 0.97 | 0.34 | 0.62 |
Ghrelin (pg/mL) | 884.94 ± 454.91 | 687.5 ± 319.34 | 977 ± 517.78 | 0.16 | 0.25 |
HOMA-IR | 3.35 ± 2.26 | 2.17 ± 1.17 | 1.88 ± 1.19 | 0.034 | 0.037 |
QUICK-Index | 0.33 ± 0.03 | 0.35 ± 0.04 | 0.36 ± 0.04 | 0.019 | 0.026 |
Total intake of carbohydrates per day (g) | 282.59 ± 68.9 | 248.23 ± 91.94 | 206.4 ± 47.64 | 0.17 | 0.31 |
% total Kcal as carbohydrates | 57.04 ± 8.55 | 53.47 ± 9.8 | 45.4 ± 7.4 | 0.038 | 0.046 |