By Dr. H. Krebs, Prof. Dr. W. Schramm (auth.), Professor Dr. med. I. Scharrer, Professor Dr. med. W. Schramm (eds.)

This booklet includes the contributions to the thirty fourth Hemophilia Symposium, Hamburg 2003. the most subject matters are HIV an infection and epidemiology, administration of bleedings in hemophiliacs with inhibitors, orthopedic difficulties and treatment in hemophiliacs, treatment with protein C and pediatric hemostaseology. the amount is rounded off by means of quite a few unfastened papers and posters on hemophilia, hemophiliacs with inhibitors, thrombophilia and molecular diagnostics.

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Haemophilia 2001;7(1):33-8. 30 A. Gringeri 12. Mauser Bunschoten EP, Nieuwenhuis HK, Roosendaal G, van den Berg HM: Low dose immune tolerance induction in hemophilia A patients with inhibitors. Blood 1995; 86(3):983. 13. Gruppo RA, Valdez LP, Stout RD: Induction of immune tolerance in patients with hemophilia A and inhibitors. Am J Pediatr Oncol 1992;14:82. 14. Freiburghaus C, Berntorp E, Ekman M, Gunnarsson M, Kjellberg B, Nilsson IM. Tole rance induction using the Malmo treatment model 1982-1995.

The administration of rFVIIa by continuous infusion is a wide-spread practice since several authors have shown that it is effective, less expensive [11–15], and more convenient than bolus injections. Table 1. Treatment schedule for recombinant FVIIa – bolus injections. 5 kIU (90 µg)/kg bw, every 3–4h Table 2. Treatment schedule for recombinant FVIIa – continuous infusion. 5 kIU (20–30µg)/kg bw 24 h stable at 25°C after reconstitution I. Scharrer/W. ) 34th Hemophilia Symposium Hamburg 2003 ” Springer-Verlag Berlin Heidelberg 2005 34 C.

In fact, hemostatic effects of by-passing agents is unpredictable on the basis of laboratory assays [37]. No precise guidelines are available for the use of rFVIIa by continuous infusion [41–43], which actually is not licensed. By contrast, large uniformity of approach exists for treatment with APCC, that is recommended at doses of 50-100 IU/kg every 8-24 hours, never exceeding 200 IU/kg per day, in order to prevent adverse events. APCC was also suggested for secondary prophylaxis in patients with recurrent bleedings [44], but a risk/efficacy ratio and a cost/benefit ratio evaluation are still missing as well as the appropriate dose and administration schedule.

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