[An inhibitor in the Willebrand-Jürgens syndrome and its effect on the factor VIII molecule properties].
In a patient with a clinically serious Willebrand-Jürgen's syndrome an inhibitor appeared at the age of  1 1/2 years, which, contrary to inhibitors in haemophilia A, was directed against all properties of factor VIII molecule. In a quantitative test the height of the inhibitor level to factor VIIIag was determined. Administrations of plasma concentrate resulted in a titre increase which could not be suppressed even by an immunosuppressive therapy with cyclophosphamide. After a long break in the substitution a severe bleeding could be successfully treated and the substitution effect for factor VIIIc, factor VIIIag and Ristocetin co-factor could be identified for several days.
D000941:Antigens / Q000032:analysis; D001803:Blood Transfusion; D002675:Child, Preschool; D005169:Factor VIII / Q000037:antagonists & inhibitors* / Q000276:immunology; D006801:Humans; D008297:Male; D010949:Plasma; D010974:Platelet Aggregation / Q000187:drug effects; D012310:Ristocetin / Q000494:pharmacology; D014842:von Willebrand Diseases / Q000097:blood* / Q000628:therapy
D000941:Antigens; D012310:Ristocetin; D005169:Factor VIII
Ein Hemmkörper bei von Willebrand-Jürgens-Syndrom und seine Wirkung auf die Eigenschaften des Faktor VIII-moleküls.