Journals / Ege Pediatri Bülteni / 2005 / Cilt: 12 - Sayı: 3

Epidemiological study in İzmir about prevalance of von Willebrand disease

İzmir'de von Willebrand hastalığı sıklığını belirlemeye yönelik epidemiyolojik çalışma

Pages
151–159
Publication Date
2022-10-17
DOI
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AI Summary & Key Takeaways

AI Insight
⚡ TL;DR(Too Long; Didn't Read)

In this study the most common bleeding symptoms in children with vWD were easy bruising and epistaxis.

📌 3 Key Takeaways

🎯
Objective & Hypothesis:Von Willebrand Disease is an autosomally inherited disease resulting from a quantitative or qualitative defect of von Willebrand factor (vWf).
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Key Findings & Data:A total of 3485 children aged 6-13 years attending at six different primary and secondary school in Izmir were given a questionnaire form to determine the presence of bleeding symptoms and their severity in children and their families.
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Conclusion & Impact:In this study the most common bleeding symptoms in children with vWD were easy bruising and epistaxis.
🔬Design:Survey & Empirical Study
📍Scope:Comprehensive Sample
⚙️Variables:Domain Parameters

Abstract

Von Willebrand Disease is an autosomally inherited disease resulting from a quantitative or qualitative defect of von Willebrand factor (vWf). It is considered as the most common cause of hereditary bleeding disorders. The aim of this study was to evaluate the prevalence of vWD in Izmir. A total of 3485 children aged 6-13 years attending at six different primary and secondary school in Izmir were given a questionnaire form to determine the presence of bleeding symptoms and their severity in children and their families. Questionnaire forms were completed by parents and turned back from 3179 children. Children who had bleeding symptoms and at least one other family member with a bleeding history were asked to give blood samples for vWf antigen (vWf: Ag) and its activity (RiCof). A total of 179 children out of 194 who had personal and family history of bleeding accepted to participate in the study and gave the blood samples. Blood analysis revealed decreased vWf: Ag and/or RiCof in 14 children. The prevalence of vWD among the school children in Izmir was found to be.0.44%. In this study the most common bleeding symptoms in children with vWD were easy bruising and epistaxis.

Özet

Von Willebrand Hastalığı (vWH) von Willebrand faktörün nitelik veya niceliksel bozukluğundan kaynaklanan otozomal geçişli kalıtsal bir hastalıktır. En sık görülen kalıtsal kanama bozukluğu olarak kabul edilir. Bu çalışmada amaç İzmir'de vVVH'nın görülme sıklığını belirlemektir. Yaşları 6- 13 yıl arasında değişen toplam 3485 çocuğa kendilerinde ve ailelerinde kanama semptomlarının varlığını, varsa ağırlığını belirlemeye yönelik bir anket formu verildi. Anne ve babaları tarafından bu anket formları dolduruldu ve 3179 çocuğun anket formu geri alındı. Kanama bulguları olan ve ailesinde kendisinden başka en az 1 bireyde daha kanama öyküsü tanımlanan çocuklardan vWf antijen (vWf: Ag) ve aktivitesi (RiCof) çalışılmak üzere kan örneği alınması önerildi. Kişisel kanama semptomları bulunan ve ailesinde kanama öyküsü tanımlanan 194 çocuğun 179'undan kan örneği alındı. Tetkikler sonucunda 14 çocukta azalmış vWf:Ag ve/ veya RiCof değerleri saptandı. İzmir ilinde okul çocuklarında vWH görülme sıklığı % 0.44 olarak belirlendi.

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Cite This Article

KARAPINAR, B., YILMAZ, D., BALKAN, C., YENİAY, S. B., BİLENOĞLU, B., KAVAKLI, K. (2022). Epidemiological study in İzmir about prevalance of von Willebrand disease. Ege Pediatri Bülteni, Cilt: 12 - Sayı: 3, 151–159. https://ajindex.com/articles/495772

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References (11)

  1. Lillicrap D, Dean J, Blanchette VS. von Willebrand disease. In: Lilleyman J, Mann I and Blanchette V, eds. Pediatric Hematology 2nd Edition, Churchill Livingstone, 2000, 601-609.
  2. Qursel T, Kavaklı K, Aktuğlu Q, ve ark. Hemofili ve von Willebrand Hastalığında Tanı ve Tedavi, Türk Hematoloji Derneği - Hemofili Alt Komitesi Rehberi, İstanbul, 1999, 2-5.
  3. Laffan M, Brown SA, Collins PW, et al. The diagnosis of von Willebrand disease: a guideline from the UK Haemophilia Centre Doctors' Organization. Haemophilia 2004; 10: 199- 217. Dean JA, Blanchette VS, Carcao MD et al. Von Willebrand disease in a pediatric- based population- comparison of type 1 diagnostic criteria and use of the PFA-100 and a von Willebrand factor/ collagen - binding assay.1 Thromb Haemost 2000; 84: 401-409.
  4. Rodeghiero F, Castaman Q, Dini E. Epidemiological Investigation of the prevalence of von Wiilebrand's Disease. Blood 1987; 69: 454- 459. İzmir'de von Willebrand Hastalık Sıklığı Qursel T, Bumin Ç, Ozaltın S. Von Willebrand Hastalığının prevalansı. Tr J Med Sci 1992; 16: 324-330.
  5. Sramek A, Eikenboom JC, Briet E, Vanderbroucke JP, Rosendaal FR. Usefulness of patient interview in bleeding disorders (comment). Arch Intern Med 1995; 155: 1409-1415. Sadler JE. A revised classification of von Willebrand disease. For the Subcommittee on von Willebrand Factor of the Scientific and Standardization Committee of the International Society on Thrombosis and Haemostasis. Thromb Haemost 1994; 71: 520-525.
  6. Qill JC, Endres Brooks J, Bauer PJ, Marks WJ Jr, Montgomery RR. The effect of ABO blood group on the diagnosis of von Willebrand disease. Blood 1987; 69: 1691-1695.
  7. Kadir RA, Economides DL, Sabin CA, Owens D, Lee CA. Variations in coagulation factors in women: effects of age, ethnicity, menstrual cycle and combined oral contraseptive. Thromb Haemost 1999; 82: 1456-1461.
  8. Hansen JB, Wilsgard L, Olsen JO, Osterud B. Formation and persistence of procoaglant and flbrinolytic activities in circulation after sttrenuous phyical exercise. Thromb Haemost 1990; 64: 385-389.
  9. Myrup B, Bregengard C, Faber J. Primary haemostasis in thyroid disease. J Intern Med 1995, 238: 59-63.
  10. Rogers JS Jr, Shane SR, Jencks FS. Factor VIII activity and thyroid function. Ann Intern Med 1982; 97: 713-716.
  11. Miller CH, Dilley A, Richardson L, Hooper WC, Ewatt BL. Population differences in von Willebrand factor levels affect the diagnosis of von Willebrand disease in African- American women. Am J Hematol 2001; 67: 125-129.