124 Table 2 (part1) Patient characteristics and laboratory measurements Patient BS Age (years) Gender OI type BT Ht Hb MCV INR Platelet count 1 0 low 35 M 1 2.58 0.44 9.2 90.2 1.0 260 2 1 low 66 M 1 4.31 0.44 9.1 93.4 1.0 304 3 2 low 27 M 1 2.05 0.46 9.5 85.8 1.2 224 4 2 low 23 M 1 7.17 0.45 10.1 83.0 1.0 207 5 3 low 25 F 1 9.13 0.43 8.9 86.1 1.0 245 6 3 low 49 M 1 5.33 0.43 9.1 90.7 1.0 344 7 5 low 45 F 4 5.3 0.44 9.2 93.5 1.0 254 8 6 low 36 F 1 5.26 0.39 8.2 94.7 1.1 218 9 6 low 31 F 3 5.45 0.37 7.6 92.8 1.1 239 10 10 high 33 F 1 >10 0.4 8.6 91.8 1.0 279 11 10 high 33 M 1 4.41 0.44 9.7 85.5 1.0 320 12 11 high 34 F 1 8.23 0.37 7.5 88.8 1.0 212 13 11 high 47 F 4 4.45 0.41 8.1 92.6 1.0 252 14 11 high 60 F 1 8.57 0.41 8.8 92.4 1.1 195 15 11 high 31 F 1 >10 0.34 7.1 86.6 1.0 233 16 12 high 46 F 1 7.16 0.35 7.3 92.3 1.0 168 17 15 high 63 M 1 3.37 0.42 8.6 96.1 1.0 227 18 18 high 61 F 1 3.45 0.38 7.7 94.5 1.0 244 19 18 high 40 F 1 4.3 0.39 8.2 87.9 1.0 156 20 20 high 41 F 1 6.22 0.4 7.6 82.4 1.0 331 Reference ranges: aPTT: 20-35 s; BT:<10min; INR: ; Fibrinogen: 2-4 g/l; FVIII: 60-150%; Hb: 7,5 – 10 mmol/L (F), 8.5 – 11.0 mmol/L (M); Ht: 0.36 – 0.47 l/l (F), 0.41 – 0.50l/l (M); MCV: 82-100 fl; Platelet count: 150-400 x 109/l; PT: 0.9-1.1 s. Abbreviations: aPTT, activated partial thromboplastin time; BS, bleeding score; BT, bleeding time; CT, clotting time; F, female; FVII, factor VIII; Hb, hemoglobin; Ht, hematocrit; INR, international normalized ratio; M, male; MCF, maximum clot firmness; OI, Osteogenesis Imperfecta; PFA-200, Platelet Function Analyser- 200; PT, prothrombin time; ROTEM: Thromboelastogram; VWF, von Willebrand factor.
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