Part of the Handbuch der Inneren Medizin book series (INNEREN, volume 2 / 9)


Die Hämophilie ist die häufigste angeborene hämorrhagische Diathese. Die Inzidenz dürfte generell bei 100/106 Männern für die Hämophilie A und 25/106 Männer für die Hämophilie B liegen (Aledort u.Goodnight 1981). Einzelne Untersucher fanden jedoch Abweichungen nach oben und unten. So schätzteBiggs (1977) die Häufigkeit der Hämophilie auf 50/106 Geburten.Stevenson u.Kerr (1967) nahmen eine Inzidenz von 100–120 Patienten mit Hämophilie A und 20–30 Patienten mit Hämophilie B auf 106 Geburten an. In einer amerikanischen Studie (National Blood Resource Program 1972) wurde hingegen eine Prävalenz von 258 Fällen von Hämophilie auf 106 Männer gefunden (205/106 Hämophilie A und 53/106 Männer Hämophilie B). In Deutschland wurde die Inzidenz auf 100 (Bitter et al. 1963) bzw. 110 (Landbeck u.Kurme 1970) Hämophile auf 106 Männer geschätzt. Die beobachteten Unterschiede dürften zum Teil auf den unterschiedlichen Erfassungsgrad in den untersuchten Populationen zurückzuführen sein.


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