fig3

Figure 3. Changes in DS3 scores from pre-treatment (baseline) to one year prior to fracture occurrence. The sub-population in this figure comprises the 33 patients from the total study population of 133 patients who had a fracture after initiation of treatment for GD1. The baseline DS3 scores are shown in blue. DS3 scores recorded approximately one year before a fracture occurred are shown in red. Five patients (designated with an “X” below the patient number) had a fracture during the first year of treatment and were excluded from the subsequent analysis of change in DS3 score. Of the 28 remaining patients, 13 (46.4%) had a decrease in DS3 ≥ 3.15, indicating a minimal clinically important improvement as defined in the literature^x, despite which they nonetheless suffered a fracture. These thirteen patients are designated with asterisks. The vertical green line separates the 23 patients with a baseline DS3 ≥ 6.00 (severe severity) from the 10 patients with a lower baseline score. Of these, one patient had DS3 < 3.00. The other nine had baseline DS3 3.00-5.99 (moderate severity). DS3: Disease severity scoring system.