Table 2.
PWS Group | All Patients | DEL 15 | UPD 15 | UPD/ID |
---|---|---|---|---|
Number of patients (%) | N = 147 | N = 81 (55.10) | N = 10 (6.80) | N = 30 (20.41) |
F/M (%) | 69/78 (46.94/53.06) | 43/38 (53.09/46.91) | 1/9 (10/90) | 11/19 (36.67/63.33) |
Age of diagnosis [years] | 1.67 ± 2.39 0.53 (0.02–12.49) |
1.38 ± 2.33 0.41 (0.02–12.49) |
3.84 ± 2.86 4.1 (0.13–7.31) (p = 0.003) * |
3.13 ± 2.72 2.55 (0.09–8.84) (p = 0.00) * |
Age of rhGH start [years] | 4.55 ± 3.74 3.03 (0.58–17.43) |
4.24 ± 3.81 2.64 (0.58–16.75) |
7.30 ± 3.03 8.31 (3.29–10.62) (p = 0.003) * |
6.42 ± 3.74 5.62 (0.85–17.43) (p = 0.00)* |
rhGH dose (IU/kg/week; mg/kg/day) |
0.58 ± 0.16; 0.028 ± 0.008 |
0.57 ± 0.14; 0.027 ± 0.007 |
0.61 ± 0.13; 0.029 ± 0.006 |
0.60 ± 0.16; 0.029 ± 0.008 |
Height [cm] | 96.62 ± 23.31 | 95.23 ± 24.18 | 111.78 ± 15.65 | 107.85 ± 20.34 |
Height SDS | −2.11 ± 1.50 | −1.95 ± 1.53 | −2.45 ± 1.07 | −2.18 ± 1.57 |
BMI | 18.05 ± 3.99 | 17.79 ± 3.78 | 20.55 ± 4.24 | 19.31 ± 3.40 |
BMI SDS | 0.41 ± 1.55 | 0.39 ± 1.61 | 1.16 ± 0.91 (p = 0.14) * | 0.86 ± 1.29 (p = 0.15) * |
IGF1 [ng/mL] | 70.31 ± 55.06 | 75.80 ± 64.56 | 84.11 ± 47.63 | 71.93 ± 38.79 |
IGF1 SDS | −0.89 ± 0.43 | −0.83 ± 0.46 | −1.03 ± 0.55 | −1.03 ± 0.43 (p = 0.04) ** |
PWS—Prader–Willi syndrome, DEL 15—deletion of chromosome 15q11-13, UPD 15—uniparental disomy, ID—imprinting defect, F/M—female/male, rhGH—recombinant human growth hormone, BMI—body mass index, IGF1—insulin-like growth factor 1. * group UPD 15 vs. DEL 15, UPD/ID vs. DEL 15, ** group UPD/ID vs. DEL 15.