84 Results Participant characteristics In total 23 patients were identified during the study period. One patient was excluded because laboratory testing failed as the blood samples could not be analysed within the normal timeframe. Therefore, 22 patients were available for analysis. Fourteen of the 22 (63.6%) OI patients in our cohort were female. The mean and median age of participants with OI was 40 (IQR 28-52) According to the Kolmogorov-Smirnov test, the age distribution of this study was normal (p = 0.179). Skewness and kurtosis were 0.393 and -0.781, respectively, thus confirming the normal distribution 26. Seventeen of the 22(77%) subjects had OI type 1, 2 (9%) type 3 and 4 patients (14%) had OI type 4. None of the patients suffered from renal or liver diseases. None of the patients reported smoking or alcohol abuse (>2 units/day). All patients were in a moderate to good condition (Karnofsky Index mean 82%, mode 80%) (Table 2). Table 2 Overview of general patient characteristics Variable OI patients Female, n (%) 14 (63.6) Current age, years, median (IQR) 39 (28–52) Karnofsky index, years, median (IQR) 80 (80–90) Age at diagnosis, years, median (IQR) 4 (0–33) OI subtype, n (%) Type 1 17 (77.3) Type 3 2 (9.1) Type 4 3 (13.6) Response rate ISTH-BAT, n (%) 20 (91%) Abbreviations: IQR, Interquartile range; OI, Osteogenesis imperfecta. Bisphosphonates Sixty percent of the patients had previously used bisphosphonates. The group who had never used bisphosphonates had a mean bleeding score of 1.38 (standard deviation, SD 1.19), whereas the group who used bisphosphonates in the past had a mean bleeding score of 3.00 (SD 3.16). The difference between these mean values is not statistically significant (p = 0.113).
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