title: Von Willebrand's disease in the Western Cape.
abstract: OBJECTIVE
  To establish the prevalence of the various subtypes of Von Willebrand's disease (VWD) among patients with bleeding disorders in the Western Cape and to review appropriate treatment strategies.
  
  
  METHODS
  A systematic clinical and laboratory study.
  
  
  METHODS
  Haemophilia clinics at two tertiary referral hospitals (Groote Schuur Hospital and Red Cross War Memorial Children's Hospital) in the Western Cape.
  
  
  METHODS
  Twenty-two patients (14 females, 8 males; ages 3 - 55 years) were studied. Those studied were selected for reasons of convenience, as they were compliant and regular attenders at the clinics.
  
  
  METHODS
  History of a bleeding tendency; bleeding time measurements; factor VIII assays, von Willebrand factor (VWF) antigen assays; ristocetin co-factor assays and VWF multimer analysis.
  
  
  RESULTS
  Fourteen patients had typical type I VWD; 2 had type II and 5 had type III variants, and there was 1 unclassifiable variant. Analysis of local factor VIII concentrates showed the presence of high-molecular-weight VWF multimers.
  
  
  CONCLUSIONS
  The results are similar to patterns reported elsewhere in the world. Locally produced factor VIII concentrates, unlike a number of commercially produced concentrates, contain sufficient multimers for use as appropriate replacement therapy.
authors.affiliation: Department of Haematology, Institute of Child Health, Red Cross War Memorial Children's Hospital, Cape Town.
authors.forename: A R
authors.initials: AR
authors.lastname: Bird
authors.forename: M
authors.initials: M
authors.lastname: Shuttleworth
authors.forename: C
authors.initials: C
authors.lastname: Anderson
authors.forename: C
authors.initials: C
authors.lastname: Karabus
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D005169:Factor VIII / Q000627:therapeutic use; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D013019:South Africa / Q000453:epidemiology; D014842:von Willebrand Diseases / Q000145:classification / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy
chemical_list: D005169:Factor VIII