Table 2.
Short stature of SOFT syndrome: prevalence of clinical, radiological, hormonal characteristics and course of treatment with rhGH
| Total | number of missing data | |
|---|---|---|
| Short stature | 42/42 (100%) | 0 |
| Median (range) height at investigation (SDS) | - 5.5 (([-8.5]-[-2.8])) | 9 |
| Median (range) adult height (cm) | 132.5 (103.5–148) | 34 |
| Disproportionate short stature | 38/42 (90.5%) | 0 |
| Small hands and feet | 40/40 (100%) | 2 |
| Brachydatyly | 37/40 (92.5%) | 2 |
| Clinodactyly | 11/40 (27.5%) | 2 |
| Metaphysical irregularity of long bones | 24/26 (92%) | 16 |
| Hypoplasia of sacrum and pelvis | 14/24 (58%) | 18 |
| Cone‑shaped epiphyses | 27/32 (84%) | 10 |
| Delayed ossification of bones | 14/25 (56%) | 17 |
| Stenosis of craniovertebral junction | 4 | ? |
| Number of patients treated with rhGH | 9/42 (21%) | 0 |
| Median (range) serum IGF-I level before rhGH treatment (SDS) | +2 ([-0.5] – [+3]) | 37 |
| Median (range) age at rhGH onset (years) | 6 (2.5–10.1) | 33 |
| Median (range) age at rhGH withdrawal (years) | 8.7 (5.3–11.6) | 33 |
| Median (range) duration of rhGH therapy (years) | 2.8 (0.66–6) | 33 |
| Reason for stopping rhGH | ||
| - No response or weak response on growth | 7/9 (78%) | 33 |
| - Metabolic cause (diabetes, excessive weight gain) | 4/9 (45%) | |
| Median (range) maximum dose of rhGH (µg/kg/d) | 67.5 (35–100) | 40 |
| Median (range) SDS of height before rhGH therapy | -6.7 ([-8.5]-[-5.5]) | 38 |
| Median (range) SDS of height after rhGH treatment | -5.95 ([-8.5]-[-4.5]) | 38 |
|
Median (range) maximum serum level of IGF-I under rhGH treatment (SDS) |
+3 | 41 |
The median was used to summarize the continuous quantitative variables with extreme values (range) in parentheses. The frequencies were used to summarize the qualitative variables and were reported as "No. (%)"
rhGH recombinant human Growth Hormone, SDS standard deviation score