36th Hemophilia Symposium Hamburg 2005 - Epidemiology; Hemophilia Therapy - Management of Bleedings and Inhibitors; Orthopedic Treatment in Hemophiliacs; Hemostaseologic Diagnosis; Pediatric Hemostaseology; Free Lectures

About 31% of the patients with a factor VIII replacement therapy develop a factor VIII inhibitor.From these are 23% low-responder ( 5BE) [8].In the case of severe hemophilia B,about 10.5% of the patients develop inhibitory antibodies [9]. Anti-factor VIII-antibodies are also seen in 15 78% healthy people without hemophilia [7, 17, 19]. Lacroix-Desmazes et al. [10, 11] showed anti-idiotypic antibodies neutralizing the inhibitory activity of the an- factor VIII antibodies in healthy people. Well-known predisposing factors for inhibitor formation are genetic features of factor VIII,which include large deletions,nonsense mutations or intrachromosomal recombinations [5, 23].Also, ethnic groups other than Caucasians (e.g.Africans) have a higher risk of developing inhibitors.Other risk factors are presumably de- ved from the immune system. For instance, a reduction of the inhibitor was seen with lower CD4+ T helper cell counts in HIV positive hemophilic patients [3,4].The development of inhibitors is very likely to be a Th-2 mediated event where cyto- nes and their receptors,T-cell receptors and the Major Histocompatibility Complex may also play an important role. Theoretical Background The substituted factor is an unknown protein for patients with a severe he- philia. Fig. 1. The normal immunoresponse (according to BAENKLER [2]) Abbreviations: TCR T cell-receptor; APC antigen presenting cell 36 I.Wieland et al.

List of contents

Epidemiology.- Hemophilia Therapy - Management of Bleedings and Inhibitors.- Orthopedic Treatment in Hemophiliacs.- Hemostaseologic Diagnostics.- Pediatric Hemostaseology.- Free Lectures.- Poster.

Product details

• Assisted by: Ute Heilmann (Editor), Ing Scharrer (Editor), Inge Scharrer (Editor), Schramm (Editor), Schramm (Editor), Wolfgang Schramm (Editor)
• Publisher: Springer, Berlin
• Languages: English
• Product format: Paperback / Softback
• Released: 14.04.2009
• EAN: 9783540367147
• ISBN: 978-3-540-36714-7
• No. of pages: 319
• Dimensions: 155 mm x 19 mm x 235 mm
• Weight: 540 g
• Illustrations: XXXIV, 319 p.
• Subjects: Natural sciences, medicine, IT, technology > Medicine > Clinical medicine; C, Pädiatrie, Medicine, Orthopädie und Brüche, Pediatrics, Orthopedics, Hematology, Orthopaedics, Surgical orthopaedics & fractures, Paediatric medicine