82 Table 1 Patients characteristics with a general overview of bleeding score and laboratory measurements Patient (text ID) BS OI type Age (years) M/F Bleeding symptoms Platelet count aPTT FVIII VWF antigen VWF activity 1 3 3 24 M Subdural bleeding 202 30 103 74 82 2 2 4 45 F I, oral cavity bleeding 254 33 50 44 36 3 (Patient 5) 0 3 52 M 367 27 121 75 76 4 4 1 19 F I, II, melena with consultation 269 24 154 104 101 5 (Patient 6) 0 1 18 M 163 28 116 88 135 6 (Patient 1) 6 1 51 F I, III*, Haematochezia†, post-partum blood transfusion 180 25 209 120 185 7 1 1 56 F Haematuria 333 23 56 91 87 8 3 1 70 M III*, IV, haematemesis 258 28 181 9 3 1 40 M Epistaxis treated with cauterisation 249 27 99 74 72 10 (Patient 2) 4 1 29 M III treated with blood transfusion 261 28 78 11 (Patient 3) 9 4 55 F II, V, VI, III treated with medication, VII 346 26 173 12 0 1 33 F 290 30 97 76 79 13 1 1 30 F I 302 29 73 64 68 14 0 1 38 F 258 28 123 78 109 15 3 1 45 F Haematuria, spontaneous hemarthrosis 323 25 160 70 69 16 1 1 45 F I 251 24 206 131 151 17 4 1 28 F with iron/hormonal therapy, VIII, oral cavity bleeding 355 31 95 75 72 18 0 4 28 M 213 29 130 101 107 19 (Patient 4) 7 1 27 M IV‡, III treated with re-surgery and blood transfusion 197 27 126 110 117 20 1 1 67 F I 203 25 184 115 129 21 1 1 26 F IV 394 26 222 130 143 22 1 1 53 F II 396 28 108 84 71 Bleeding symptoms: I: menorrhagia; II: ≥5 bruises [>1 cm]; III: bleeding after surgery in <25% of the procedures; IV: bleeding after tooth extraction in <25% of all procedures; V: spontaneous epistaxis >10 min; VI: muscle haematoma after trauma; VII: bleeding umbilical stump with surgical haemostasis and blood transfusion.; VIII: >5 minor bleeds/year. Reference ranges: aPTT: 20-35 s; EPI/ADP: 85-165 s; ExtemCFT: 34-159 s; ExtemCT: 38-79 s; ExtemMCF: 50-72 mm;Extem/IntemML: <15%;Fibrinogen: 2-4 g/l; FVIII: 60-150%; IntemCFT: 30-110 s; IntemCT: 110-240 s; IntemMCF: 50-72 mm; Platelet count: 150-400 x 109/l; PT: 09-11 s; VWF activity: 50%-150%; VWF antigen: 50%-150%. Results outside the reference ranges are marked: .
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