The Levels of Von Willebrand Factor in Adult Patients with Familial Mediterranean Fever and it is Relationship with Platelet Activation

Öz

Objective: Familial Mediterranean Fever (FMF) is an autosomal recessive disease characterized by recurrent inflammatory febrile attacks of serosal and synovial membranes. Recently few studies have shown that FMF is associated with increased atherosclerosis risk. Therefore, this study was designed to answers the following questions: A) do von Willebrand factor (vWf )- a marker of endothelial damage / dysfunction- levels change in adult FMF patients and B) is there any relationship between vWf and mean platelet volume (MPV)- a determinant of platelet activation- levels? Materials and Methods: This study was performed in January 2008. We selected 35 patients with FMF and 35 healthy control subjects matched for age, gender, and body mass index. We measured levels of vWf and MPV in all study population. Results: The level of vWf was significantly higher in the FMF group than in the control group (p=0.001). Also, vWf level was positively correlated with MPV level in FMF group (r=0.694, p=0.001). Conclusion: Our results suggest that (i) adult patients with FMF tend to have relatively endothelial dysfunction, and (ii) vWf seem to be related to platelet volume in patients with FMF.

Anahtar Kelimeler

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Yetişkin Ailesel Akdeniz Ateşi Hastalarında Von Willebrand Faktör Düzeyleri ve Bunun Trombosit Aktivasyonu ile İlişkisi

Öz

Amaç: Ailesel Akdeniz Ateşi (AAA), serozal ve sinovyal membranların tekrarlayan inflamatuar ateşli atakları ile karakterize otozomal resesif bir hastalıktır. Yakın zamandaki bazı çalışmalar AAA'nın artmış ateroskleroz riskine eşlik ettiğini göstermiştir. Bundan dolayı, bu çalışma şu sorulara cevap vermek üzere tasarlanmıştır: (A) endotel hasarının veya disfonksiyonunun bir belirteci olan von Willebrand faktör (vWf) düzeyleri yetişkin AAA hastalarında değişiklik gösteriyor mu? ve (B) vWf ile trombosit aktivasyonunun bir belirleyicisi olan ortalama trombosit volümü (OTV) arasında bir ilişki var mıdır? Gereç ve Yöntem: Bu çalışma Ocak 2008'de gerçekleştirilmiştir. Çalışmaya 35 AAA hastası ile kontrol grubu olarak yaş, cinsiyet ve vücut kitle indeksi açısından benzer 35 sağlıklı kişi alındı. Çalışmaya alınanlarda vWf ve OTV düzeyleri ölçüldü. Bulgular: vWf düzeyi AAA grubunda kontrol grubuna göre anlamlı olarak yüksekti (p=0.001). Ayrıca, AAA grubunda vWf düzeyi ile OTV düzeyi arasında pozitif korelasyon mevcuttu (r=0.694, p=0.001). Sonuç: Sonuçlarımız (i) yetişkin AAA hastalarının rölatif olarak endotelyal disfonksiyona sahip olduklarını ve (ii) AAA hastalarında vWf'ün trombosit volümüyle ilişkili göründüğünü düşündürmektedir.

Anahtar Kelimeler

• Ailesel Akdeniz ateşi, Von Willebrand faktör, Endotelyal disfonksiyon, Ortalama platelet volümü

Kaynakça

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22. Gönderilme Tarihi: 02.10.2010