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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
  1. Abbreviations: BMI: Body Mass Index, BAMF: Brief Assessment of Motor Function, GMFM: Gross Motor Function Measurement.