       Document 0272
 DOCN  M9640272
 TI    Current management of von Willebrand's disease.
 DT    9604
 AU    Castaman G; Rodeghiero F; Department of Haematology and Haemophilia, San
       Bortolo Hospital,; Vicenza, Italy.
 SO    Drugs. 1995 Oct;50(4):602-14. Unique Identifier : AIDSLINE MED/96106617
 AB    von Willebrand's disease (vWD) is the most frequent inherited disorder
       and is the result of a deficiency and/or abnormality of von Willebrand
       factor (vWF). As a consequence, the level of factor VIII, the presence
       of which reflects the ability of vWF to stabilise it, is usually low.
       Bleeding time, which reflects the ability of vWF to promote platelet
       adhesion to subendothelium, is therefore prolonged. However, from a
       therapeutic point of view, it appears that the correction of factor VIII
       and bleeding time is sufficient to prevent or treat bleeding in these
       patients. There are 2 main therapeutic tools to improve or normalise
       these major determinants of the bleeding tendency in this disorder. The
       majority of patients, identified by a test infusion, can be successfully
       treated by giving desmopressin (DDAVP), a synthetic analogue of
       vasopressin. Desmopressin is able to induce the increase of autologous
       vWF released from endothelial cells, leading to the correction of factor
       VIII levels and of bleeding time. In the remaining cases, blood
       products, namely factor/vWF concentrates, are required to accomplish
       haemostasis. All these concentrates are able to correct factor VIII
       levels, whereas their effect on bleeding time may not be consistent. The
       modern virucidal techniques abolish the risk of transmission of
       blood-borne viruses (e.g. hepatitis viruses and HIV) and make these
       products safer than blood-bank cryoprecipitate.
 DE    von Willebrand's Disease/DIAGNOSIS/*DRUG THERAPY/PHYSIOPATHOLOGY  Female
       Human  Pregnancy  JOURNAL ARTICLE  REVIEW  REVIEW, TUTORIAL

       SOURCE: National Library of Medicine.  NOTICE: This material may be
       protected by Copyright Law (Title 17, U.S.Code).

