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Figure 2 |
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| Data from International Rare Bleeding Disorders Database (RBDD, www.rbdd.org ) |
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| Peyvandi F 1 , Menegatti M 1 , Palla R 1 , Siboni, SM 1 , Mazzucconi MG 2 , Targhetta R 3 , Ciavarella N 4 , Mehran K 5 , Lak M 6 , Mikovic D 7 , Kavakli K 8 . |
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1 A. Bianchi Bonomi Hemophilia and Thrombosis Center, IRCCS Maggiore Hospital, Mangiagalli and Regina Elena Foundation, University of Milan and Luigi Villa Foundation, Italy |
| 2 Dipartimento di Biotecnologie Cellulari ed Ematologia, Università degli Studi di Roma La Sapienza, Rome, Italy |
| 3 Center for Hemophilia, University of Cagliari, Policlinico Universitario, ss 554-09042 Monserrato, Cagliari, Italy |
| 4 Hemophilia and Thrombosis Center, Ospedale Consorziale, Policlinico, Bari, Italy. |
| 5 Hematology and Thrombosis Unit, Haematology Research Centre, Shiraz University of Medical Sciences, Shiraz, Iran |
| 6 Iranian Haemophilia Centre, Iman Khomeini Hospital, Tehran University of Medical Science, Tehran, Iran |
| 7 Blood Transfusion Institute of Serbia Hemostasis Dept and Haemophilia Center, Belgrade-Serbia |
| 8 Department of Pediatric Hematology, Ege University Hospital, Ege Hemophilia Center, Izmir, Turkey |
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| Data are extrapolated by the International Rare Bleeding Disorders Database (RBDD, www.rbdd.org , tailored questionnaire) and regard patients coming from Italy (57%), Iran (32%), Serbia (7%) and other countries (5%). Data on FI, FV, FVII, FX, FXI, FXIII are related to patients with severe laboratory deficiency ( coagulant activity less than 10% and fibrinogen coagulant activity less than 5 mg/dl), whilst d ata on FV+FVIII are related to patients with moderate laboratory deficiency ( coagulant activities between 5% and 10%) . About bleedings during pregnancy/deliver, major surgery, we can not exclude that some patients, particularly patients with FXIII deficiency, were on prophylaxis |
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| Legends: |
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| Rare: 0-10 % of patients |
| Occasional: 10 – 30 % of patients |
| Common: >30 % of patients |
| # treatment could not be ruled out |
| * percentages were calculated on the basis of the numbers of procedures; |
| ** percentages were calculated on one patient; |
| na = not available data |
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