World Federation of Hemophilia 2008 WFH WFH World Federation of Hemophilia 1425 René Lévesque Boulevard West, Suite 1010 Montréal, Québec

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1 WORLD FEDERATION OF HEMOPHILIA

2 World Federation of Hemophilia 2008 WFH WFH World Federation of Hemophilia 1425 René Lévesque Boulevard West, Suite 1010 Montréal, Québec H3G 1T7 CANADA (514) (514) WFH Man-Chiu POON

3 B /

4 PPP EQAS

5 32 32 DDAVP A FVIII 37 FVIII 37 /FFP 38 DDAVP 38 B FIX 38 FIX 38 FFP /

7 1 30 WFH 2003 Paul Giangrande Man Chiu Poon Mary Chua A McCraw Jerome Wiedel Alok Srivastava WFHElizabeth Myles WFH WFH Alok Srivastava, MD WFH

9 3 X- VIII FVIII AIX FIXB A B80% 85% FVIII FIX 1/3 APTT 1 1

10 1-1 % (IU/ml) 1% <0.01 1% 5% % 40% / // 70 80% / 10 20% 5 10% <5% 45% 3% 30% 2% 15% 2% 3% 4

11 5 FVIII/FIX HIV HBV HCV HAV) B19 X- <30%

12 FFP / 6

13 h /

14 8 DDAVP (ASA) NSAIDs, COX-2 A DDAVPFVIII RICE( ) DDA 5 10d

15 9 COX-2 Port-A-Cath 1% IU/kg A 3B 2

16 % 15% A1% 3% B 10 20d <5 BU 5BU

17 Bethesda 1. 5d 5BU a FEIBA Auto 2. B B FIX ) 3. A ITI ITIFVIII FEIBA ITI IU/kg B ITI BFIX ITI 11

18 4. FVIII / CVS RhD D VIII 50% 50% VIII 12

19 HIV HIV 13

21 AFVIII>5% DDAVP 5%1 2min4 7d 15

22 NSAIDs 5 10d, 6 12 HIV HCV HBV 16

23 WFH BT PT(AP 1. 17

24 2. 1h 30min 1min SWG SWG 2ml 3.2% 3.8% 9: 1 PPP4 C2h -30 C PPP 2 000g 3500r/min15mi 4 C PPP20 25 C 4 C 2hPPP -30 C C 18

25 BT PT APTT 1-2 FXIII PAI PT APTT BT A B VWD NPP FVIII FIX FVIII/FIX WFH 19

26 FVIII 80 U FVI FVIII C/VWF:Ag % WFH FVIII C/VWF:Ag WFH WFH WFH 37 ± 0.5 C 0.1ml 0.2ml 20

27 21 7.5cm 1.2cm QA QA (IQC) IQC

28 QA EQAS QA EQAS WFH/WHO EQAS WHO ml 2 000g15min4 C 500ul-70 C 37 C WHO NIBSCWHO 22

29 <1% 1%~5% h 23

30 12h, NSAIDs COX-2NSAIDs 48 72h 30 50%48 72h 16 1h 24

31 Volkmann X MRI 6 8 NSAIDs COX-2 25

32 /

33 NSAIDsCOX-2 serial casting bracing orthotics X CTMRI 27

34 50%3 5d 10 14d 28

35 29 FVIII FIX Dr.Car 1997 WFH PCR WFH WFH HB HCV HIV GMPs NAT ;

36 GMPs HIV HBV HCV HAV B19 Creutzfeldt-JakobvCJD vcjd vcjd / 1. FVIII 10 IU IU IU IU FII FVII FX FIX 30

37 FVIII 2. HIV HAV HCV HCV HIV HAV B19 WFH FFP4 C 10 24h FVIII5 IU/ml VWFFXIII FIX FXI FVII FIX FX FXI WFH 31

38 HIV HBsAg PCRHIV HBV HCV FVIII FFP FFPFIX B FVFFP FFP FXI FFP/ / FFPWFH FFP DDAVP DDAVP DDAVPADH FVIII VWF 32

39 0.3mg/kg1FVIII3 6 90min DDAVP 1 2d A DDAVPFIX B FVIII FVIII10% 30 40% DDAVP DDAVP DDAVP DDAVP50 100ml, 2 2DDAVP DDAVP DDAVP DDAVP 0.3mg/kg50kg 50kg 150mg DDAVP 33

40 FXI 3 4g,, 5ml 500mg 25mg/kg75mg/kg, APCCsFEIBAAutoplex APCC 4 6h FVIIa 34

41 5g1g8 250mg/ml mg/kg5g 35

43 2h min NSAIDs 2 3 FVIII FVIII FVIII U 37

44 1U/kg FVIIIFVIII 2% kg 50kg 40 % 0.5=1 000U FVIII 45 FVIII 3ml 100U VIII 24 48h, 24 48h / FFP 30 40mlFVIII60 100U FFPFFP 1ml FFP 1U DDAVP FVIII5% DDAVP B FIX FIX FIX FIX FIX PCCs 38

45 FIX FIX DIC FIX U 1U/kg FIX1% FIX BeneFIX Wyeth1U/kg 0.8%150.7%FIX 50kg 40 %=2 000UFIX =2 500U =2 860U 45 FIX 3ml 100U PCC APCC 24 48h 24 48h FFP B FIX FIX 25% /FFP 39

46 X A 12hB 24h 3d,, d ( ) h CT 40

47 100 mg / CTMRI FVIII FIX

48 L/m 2 48h FIX FVIII 42

49 / DDAV DDAVP A 50% 40% d 24h24g 6 8h 110d 43

50 / d 10 14d

51 A B 40 60% % 60% % 60% % 60% % 100% % 80% % 60% % 60% 3 5 CNS/ 80% 100% % % 100% % % 80% 30% 60% 80% 30% % 100% % % 80% 30% % % % % % 100% 60% 80% 40% 60% 30% 50% % 80% 40% 60% % 50% % 40%

52 A B 10% 20% % 20%1 2 10% 20% % 20%2 3 20% 40% % 30% % 20% % 20% 3 5 CNS/ 50% 80% % 50% % 80% % 50% % 40% % 40% % 50% 10% 20% % 50% % 20% % 50% % 20% % 50% % 20% % 40% % 30%3 5 20% 40% % 80% 30% 40% % 30% % 20% % 30%5 7 50% 70% 30% 40% % 30% % 20%

53 Association of Hemophilia Clinic Directors of Canada. Clinical practice guidelines:hemophilia and von Willebrand s disease: 2.Management. Edition 2, Update Association of Hemophilia Clinic Directors of Canada. Hemophilia and von Willebrand s disease: 1. Diagnosis, comprehensive care and assessment. Edition 2. Update Farrugia, A. Guide for the assessment of clotting factor concentrates for the treatment of hemophilia. World Federation of Hemophilia Hemophilia of Georgia. Protocols for the treatment of hemophilia and von Willebrand disease Kitchen, S. and Angus McCraw. Diagnosis of hemophilia and other bleeding disorders: A laboratory manual. World Federation of Hemophilia Karabus, C, ed. Treatment guidelines for hemophilia in South Africa. South African Hemophilia Foundation. Kasper, C.K.,and Meirione Costa e Silva. Registry of clotting factor concentrates. Fifth edition. World Federation of Hemophilia National Hemophilia Foundation. Standards and criteria for the care of persons with congenital bleeding disorders Santagostino, E., P.M. Mannucci, and A. Bianchi bonomi. Guidelines for replacement therpy for hemophilia and inherited coagulation disorders in italy. Hemophilia :1-10. Srivastava, A., ed. Guidelines for management of hemophilia in India. Hemophilia Federation (India). 47

56 World Federation of Hemophilia 1425 René Lévesque Boulevard West, Suite 1010 Montréal, Québec H3G 1T7 CANADA (514) (514)

《中国血友之家》第三期

《中国血友之家》第三期 2002 10 ----2002.. 2. 7-32... 8 A B. 9. 13. 18. 25. 28. 29. 33. 34. 35. 37. 38 KELLEY WFH 2 38 2002 2000 9 5 14 AF129 777 9 45 (HTC) ( WFH) WFH 2002 5 14 2002 5 24 25 8100 10 AF8436 3 38 ( ) WFH 18 20