title: Clinical features and types of von Willebrand disease in Karachi.
abstract: This study was conducted on patients with a history of congenital bleeding disorders or with suspected bleeding tendencies. Laboratory analysis revealed Von Willebrand disease (VWD) in 68 (21.3%) of 318 participants with male to female ratio of 0.8: 1 (31 to 37) and median age 17 years (range 2-45 years). Type 3 being the most frequent, 35 (51.4%) of 68, type 2, 20 (29.4%) of 68, and lastly type 1, 13 (19.1%) of 68. A total of 55.8% patients with VWD presented with mucocutaneous bleeding. Menorrhagia was the most common presentation of female patients. Von Willebrand disease (21.3%) was the second common bleeding disorder and the most common coagulation defect among females with menorrhagia. However, the frequency in the study was quite low when compared to the western world. Similarly, low frequency of VWD type 1 might be due to the fact that only symptomatic patients visited us. Further studies are needed as there is limited information on VWD in the developing countries. This will help in the development of expertise for the accurate diagnosis & proper management.
authors.affiliation: Thrombosis & Haemostasis Department of National Institute of Blood Disease & Bone Marrow Transplantation, Karachi, Pakistan. drmunirashoaib@yahoo.com
authors.forename: Munira
authors.initials: M
authors.lastname: Borhany
authors.forename: Tahir
authors.initials: T
authors.lastname: Shamsi
authors.forename: Arshi
authors.initials: A
authors.lastname: Naz
authors.forename: Tasneem
authors.initials: T
authors.lastname: Farzana
authors.forename: Saqib
authors.initials: S
authors.lastname: Ansari
authors.forename: Muhammad
authors.initials: M
authors.lastname: Nadeem
authors.forename: Zia Ur
authors.initials: ZU
authors.lastname: Rehman
authors.forename: Zainab
authors.initials: Z
authors.lastname: Sangii
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010154:Pakistan / Q000453:epidemiology; D055815:Young Adult; D014842:von Willebrand Diseases / Q000175:diagnosis / Q000453:epidemiology* / Q000473:pathology