Dose Requirement for Substitution Therapy in Hemophilia A.

In order to determine the relationship between different doses of factor VIII and their clinical effect,70 children with severe hemophilia A treated with single doses of cryoprecipitate were studied. Plasma factor VIII levels were tested by a one stage assay on samples taken before, and 30 and 60 min. post-infusion. Clinical failure was defined as an absence of clinical improvement or the occurrence of bleeding in another site within 24 hours post-treatment. The relationship between plasma factor VIII levels or doses injected in u/kg and clinical result followed an exponential curve. Calculated from 153 factor VIII recoveries, plasma factor VIII levels of 0.35 and 0.52 u/ml corresponded to 95 and 99% satisfactory treatment, respectively. The same clinical result was obtained with 20 and 31 u of factor VIII/kg when studied in 1043 infusions of lyophilized cryoprecipitate. The in vivo recovery being 0.015 u factor VIII activity/ml for 1 u/kg injected, these dosages provided plasma factor VIII levels of 0.30 and 0.47 u/ml respectively. Since home treatment is largely based on single infusions of factor VIII, it is suggested that most current bleedings can be adequately treated with a dose of factor VIII providing a plasma factor VIII activity of 0.30-0.35 u/ml.

Angaben zum Objekt

• Standort: Deutsche Nationalbibliothek Frankfurt am Main
• Umfang: Online-Ressource
• Sprache: Englisch

Verweise und Beziehungen

• Erschienen in: Dose Requirement for Substitution Therapy in Hemophilia A. ; volume:38 ; number:05 ; year:1977 ; Konferenz: XIIth Congress of The World Federation of Hemophilia; 1977-06-22; Philadelphia ; Thrombosis and haemostasis ; 38, Heft 05 (1977)

Beteiligte, Orts- und Zeitangaben

• Beteiligte Personen und Organisationen: Allain, J.P.

Weitere Informationen

• DOI: 10.1055/s-0039-1682529
• URN: urn:nbn:de:101:1-2412190840488.201634906706
• Rechteinformation: Open Access unbekannt; Der Zugriff auf das Objekt ist unbeschränkt möglich.
• Letzte Aktualisierung: 15.08.2025, 07:23 MESZ