2003; baxter - Supplements - Haematologica

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44J. Tusell et al.SC, Moertel CL, et al. The use of implantable venousaccess devices (IVADs) in children with hemophilia. JPediatr Hematol Oncol 1997; 19:339-44.6. Collins PW, Khair KS, Liesner R, Hann IM. Complicationsexperienced with central venous catheters in childrenwith congenital bleeding disorders. Br J Haematol1997; 99:206-8.7. Ragni MV, Hord JD, Blatt J. Central venous catheterinfection in haemophiliacs undergoing prophylaxis orimmune tolerance with clotting factor concentrates.Haemophilia 1997; 3:90-5.8. Warrior I, Baird-Cox K, Lusher J Use of central venouscatheters in children with haemophilia: one haemophiliatreatment centre experience. Haemophilia 1997;3:194-8.9. Shapiro AD, Donfeld SM. Infectious complications ofcentral venous access devices (CVAD) in children withhaemophilia. Haemophilia 2000; 6:278.10. Geraghty S, Kleinert D. Additional data on the morbidityof central venous access devices in patients withhaemophilia. Haemophilia 1998; 4:66.11. Blanchette VS, Al-Musa A, Stain AM, Ingram J, Fille RM.Central venous access devices in children with hemophilia:an update. Blood Coagul Fibrinolysis 1997; Suppl1:S11-4.12. Liesner RJ, Vora AJ, Hann IM, Lilleymann JS. Use of centralvenous catheters in children with severe congenitalcoagulopathy. Br J Haematol 1995; 91:203-7.13. Bollard CM, Teague LR, Berry EW, Ockelford PA. Theuse of central venous catheters (portacaths) in childrenwith haemophilia. Haemophilia 2000; 6:66-70.14. Santagostino E, Muca-Perga M, Coluccia P, BernardinelliL, Mannucci PM. Study of long-term safety of aport-a-cath use in children with haemophilia: whichalternative? Haemophilia 2000; 6:272.15. Tusell J, Villar A, Lopez MF, Aznar JA, Brito D, Sosa R, etal. High index of port-a-cath (PAC) infections in childrenwith congenital coagulopathies. Spanish Registry.Pediatr Res 2001; 6:883.16. Van Den Berg HM, Fischer K, Roosendaal G, Mauser-Bunschoten EP. The use of the Port-A-Cath in childrenwith haemophilia: a review. Haemophilia 1998; 4:418-20.17. Ljung R, Petrini P, Lingreen AK, Tengborn L. The longtermfeasibility of venous access in children with severhaemophilia. Semin Hematol 1994; Suppl 2:16-8.18. Ljung R, Van Den Berg HM, Petrini P, Tengborn L,Scheibel E, Effenberger W. Experience with the port-acathin children with severe hemophilia A: a multicentrestudy. Haemophilia 1996; Suppl 1:17.19. Hothi DK, Kelsall W, Baglin T, Williams DM. Bacterialendocarditis in a child with haemophilia B: risks of centralvenous catheters. Haemophilia 2001; 7:507-10.20. Yamamoto K, Niiya K, Shigematu T, Kiguchi T, TakenakaK, Shinagawa K, et al. Transient factor VIII inhibitorin a hemophilia patient after staphylococcal septic shocksyndrome. Int J Hematol 2000; 72:517-9.21. Journeycake JM, Quinn CT, Miller KL, Zajac JL,Buchanan GR. Catheter-related deep venous thrombosisin children with hemophilia. Blood 2001; 98:1727-31.haematologica vol. 88(supplement n. 12):september 2003
[Round Table on Immune Tolerance Treatment]review paperThe Malmö International BrotherStudy (MIBS) – An updatehaematologica 2003; 88(suppl. n. 12):45-47http://www.haematologica.org/free/immunotolerance2001.pdfJAN ASTERMARK, ERIK BERNTORP AND THE MIBS STUDY GROUP*Department for Coagulation Disorders, University of Lund,Malmö, SwedenThe Malmö International Brother Study (MIBS) wasinitiated in 1996 to study the issue of predisposingfactors to inhibitor development in hemophiliapatients. As of August 2001, 528 families haveaccrued, of whom 451 suffer from hemophilia A and77 hemophilia B. Twenty-five of the brother pairs aretwins. The inhibitor incidence in all families withsevere hemophilia A was 29.8%. In 36 of the 110inhibitor families (32.7%), at least two brothers hada history of inhibitors. In 24 of these families, theinhibitor was also of the same type, i.e. either highorlow-responding. The overall concordance within249 severe hemophilia A families was found to be78.3% compared to an expected figure of 68.0%and 58.0% using an inhibitor incidence of 20 and30%, respectively (p5 BU and a low-respondinginhibitor was one with a peak titer of ≤ 5 BU.Severe hemophilia A corresponds to a factor VIIIclotting activity of 5%, respectively.Statisticsχ 2 analysis was used for statistical evaluationof expected versus observed frequencies ofinhibitors. An expected number of inhibitor subjectsof at least five was required in each group.A p value below 0.05 was considered to indicatestatistical significance.haematologica vol. 88(supplement n. 12):september 2003
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