Table 1 Clinical characteristics at the start and during denosumab treatment
From: Two years’ experience with denosumab for children with Osteogenesis imperfecta type VI
Findings | Patient 1 | Patient 2 | Patient 3 | Patient 4 |
|---|---|---|---|---|
Mutation in SERPINF1 | c.324_325dupCT | c.1132C > T | c.696C > G | c.696C > G |
Duration of previous bisphosphonate treatment [years] | 8.1 | 5.75 | 3.4 | 3.4 |
Age at start of denosumab therapy [years] | 9.4 | 6.9 | 5.7 | 18.5 |
Time since first denosumab injection [years] | 2.4 | 2.1 | 2.0 | 2.0 |
Number of denosumab treatment cycles [n] | 12 | 10 | 8 | 8 |
Length [cm] (SD) at start | 109 (-4.0) | 102 (-3.55) | 89 (-5.8) | 120 (-9.9) |
Length [cm] (SD) after two years | 117 (-3.7) | 107 (-4.3) | 95 (-6.2) | 120 (-9.9) |
Weight [kg] / BMI [kg/m2] at start | 19.7/16.6 | 14.7/14.1 | 10.8/13.6 | 51.4/35.7 |
Weight [kg] / BMI [kg/m2] after two years | 28.2/20.6 | 17.5/15.3 | 11.0/12.2 | 54.0/37.5 |
BAMF mobility score at start | 3 | 3 | 4 | 2 |
BAMF mobility score after one year | 3 | 3 | - | - |
BAMF mobility score after two years | 4 | 4 | 5 | 3 |
GMFM 66 score at start | 32.31 | 34.84 | - | - |
GMFM 66 score after one year | 31.78 | 40.20 | - | - |
GMFM 66 score after two years | 34.37 | 40.67 | - | - |
Fractures under denosumab treatment [n] | 2 | 2 | 0 | 0 |