Table 1 Clinical and biological characteristics of Prader–Willi syndrome (PWS) patients before and after one year of growth hormone therapy (GHT).

From: SNORD116 and growth hormone therapy impact IGFBP7 in Prader–Willi syndrome

 

Baseline

After one year GHT

p value

Age (years)

2.1 (0.86–7.79) n = 21

3.1 (1.91–8.82) n = 21

–

Height (m)

0.79 (0.68–1.1) n = 21

0.92 (0.77 –1.17) n = 21

p < 0.0001

Height (SDS)

−1.24 (−2.66–0.57) n = 21

−0.26 (−2.37–1.92) n = 21

p < 0.0001

BMI Z-score

−0.84 (−3.34–1.19) n = 21

−0.34 (−3.12–2.19) n = 21

0.13

IGF1 (ng/ml)

50.3 (7.2–137.6) n = 21

184.5 (25.5–407.7) n = 21

p < 0.0001

IGF1 (SDS)

−0.92 (−2.58–1) n = 21

1.5 (−0.36–3.76) n = 21

p < 0.0001

IGBFP3 (ng/ml)

2290 (1,237–3,565) n = 21

3632.9 (1,874–5,254) n = 21

p < 0.0001

IGFBP3 (SDS)

−0.8 (−2.03–0.58) n = 20

0.6 (−1.28–2.54) n = 21

p < 0.0001

Patients with DXA analysis

   

 Age (years)

2.27 (1.04–7.79) n = 11

3.35 (2.07–8.82) n = 11

–

 BMI Z-score

−0.8 (−2.24–1.92) n = 11

−0.4 (−3.12–2.19) n = 11

0.2

 Fat mass (%)

32.3 (24.65–46.9) n = 11

15.2 (10.4–42.6) n = 11

p < 0.01

 Lean mass (%)

66.6 (44.1–73.68) n = 11

81.8 (54.7–89.6) n = 11

p < 0.01

  1. Twenty-one patients were evaluated. The gender ratio (M/F) was 38%; the genetic diagnosis was as follows: 38% of the patients had deletions, 57% uniparental disomy, and 4% an imprinting defect. Results are presented as mean values (minimum–maximum); IGF1 (SDS) and IGFBP3 (SDS) were calculated according to Feigerlova et al.13 IGF1 and IGFBP3 are in ng/ml. Conversion factor for IGF1 from ng/ml to nmol/l is 0.131. Analysis of the body composition by dual-energy X-ray absorptiometry (DXA) was performed in 11 patients.
Back to article page