36th Hemophilia Symposium Hamburg 2005

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

Inge Scharrer, Wolfgang Schramm (Herausgeber)

Buch | Softcover

XXXIV, 319 Seiten

2006 | 2007
Springer Berlin (Verlag)
978-3-540-36714-7 (ISBN)

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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.

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

Erscheint lt. Verlag	2.11.2006
Mitarbeit	Sonstige Mitarbeit: G. Auerswald, A. Kurth, J. Oldenburg, W. Schramm, B. Zieger
Zusatzinfo	XXXIV, 319 p.
Verlagsort	Berlin
Sprache	englisch
Maße	155 x 235 mm
Gewicht	545 g
Themenwelt	Medizinische Fachgebiete ► Chirurgie ► Unfallchirurgie / Orthopädie
	Medizinische Fachgebiete ► Innere Medizin ► Hämatologie
	Medizin / Pharmazie ► Medizinische Fachgebiete ► Pädiatrie
Schlagworte	epidemiology • Hardcover, Softcover / Medizin/Klinische Fächer • HC/Medizin/Klinische Fächer • Health • Hemophilia • Hemostaseologic Diagnosis • HIV • Orthopedic Treatments • Pediatric Hemostaseology
ISBN-10	3-540-36714-4 / 3540367144
ISBN-13	978-3-540-36714-7 / 9783540367147
Zustand	Neuware