Erkan COBANa Haydar ADANIR

The levels of von willebrand factor in adult patients with familial mediterranean fever and it is relationship with platelet activation

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) arasın- da 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 trombo- sit volümüyle ilişkili göründüğünü düşündürmektedir.

Yetişkin ailesel akdeniz ateşi hastalarında von willebrand faktör düzeyleri ve bunun trombosit aktivasyonu ile ilişkisi

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.

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1.Lidar M, Livneh A. Familial Mediterranean fever: clinical, molecular and management advancements. Neth J Med 2007; 65: 318-324.
2.Chae J, Aksentijevich I, Kastner D. Advances in the unders- tanding of familial mediterranean fever and possibilities for targeted therapy. Br J Haematol 2009; 146: 467-478.
3.Turkish FMF Study Group. Familial mediterranean fever (fmf) in turkey results of a nationwide multicenter study. Medicine 2005; 84: 1-11.
4.Livneh A, Langevitz P. Diagnostic and treatment concerns in familial mediterranean fever. Baillieres Best Pract Res Clin Rheumatol 2000; 14: 477-498
5.Poredos P. Endothelial dysfunction in the pathogenesis of atherosclerosis. Int Angiol 2002; 21: 109-116.
6.Lip GY, Blann AD. Von Willebrand factor: a marker of endothelial dysfunction in vascular disorders? Cardiovasc Res 1997; 34: 255-265.
7.Blann AD, Taberner DA. A reliable marker of endothelial cell dysfunction: does it exist? Br J Haematol 1995; 90: 244-248.
8.Bath PM, Butterworth RJ. Platelet size: measurement, physio- logy and vascular disease. Blood Coagul Fibrinolysis 1996; 7: 157–161.
9.Peru H, Altun B, Dogan M, Kara F, Elmaci AM, Oran B. The evaluation of carotid intima-media thickness in children with familial Mediterranean fever. Clin Rheumatol 2008; 17: 689- 694.
10.Bilginer Y, Ozaltin F, Basaran C, et al. Evaluation of intima media thickness of the common and internal carotid arteries with inflammatory markers in familial Mediterranean fever as possible predictors for atherosclerosis. Rheumatol Int 2008; 28: 1211-1216.
11.Caliskan M, Gullu H, Yilmaz S, et al. Impaired coronary microvascular function in familial Mediterranean fever. Athe- rosclerosis 2007; 195: 161–167.
12.Tsiara S, Elisaf M, Jagroop IA, Mikhailidis DP. Platelets as predictors of vascular risk: Is there a practical index of platelet activity?. Clin Appl Thromb Hemost 2003; 9: 177–190.
13.Thompson CB, Eaton K, Princiotta SM, Rushin CA, Valeri CR. Size dependent platelet subpopulations: relationship of platelet volume to ultrastructure, enzymatic activity, and func- tion. Br J Haematol 1982; 50: 509-519.
14.Park Y, Schoene N, Haris W.indicator of platelet activation:lets 2002; 13: 301–306.
15.Mean platelet volumemethodological issues.as an Plate-Endler G, Klimesch A, Sunder-Plassmann et al. Mean platelet volume is an independent risk factor for myocardial infarction but not for coronary artery disease. Br J Haematol 2002; 117: 399–404.
16.Martin JF, Bath PM, Burr ML. Influence of platelet size on outcome after myocardial infarction. Lancet 1991; 338: 1409– 1411.
17.Livneh A, Langevitz P, Zemer D, et al. Criteria for the diagno- sis of familial Mediterranean fever. Arthritis Rheum 1997; 40: 1879-1885.
18.Blann AD, McCollum CN.Von Willebrand factor and the endothelial cell damage andatherosclerosis. Eur J Vasc Surg 1994; 8: 10–15.
19.Ozbalkan Z, Ozturk MA, Onat AM, et al. Circulating thrombomodulin levels in familial Mediterranean fever. Clin Exp Rheumatol 2006; 24: 95-98.
20.Balat A, Islek I, Cekmen M, et al. Adrenomedullin and total nitrite levels in children with familial Mediterranean fever. J Paediatr Child Health 2006; 42: 240-243.
21.Terekeci HM, Oktemli C, Ozgurtas T, et al. Increased asym- metric dimethylarginine levels in young men with familial Mediterranean fever (FMF): is it early evidence of interaction between inflammation and endothelial dysfunction in FMF? J Rheumatol 2008; 35: 2024-2029.
22.22) Akdogan A, Calguneri M, Yavuz B, et al Are familial Mediterranean fever (FMF) patients at increased risk for athe- rosclerosis? Impaired endothelial function and increased inti- ma media thickness are found in FMF. J Am Coll Cardiol 2006; 48: 2351-2353.
23.Coban E, Adanir H. Platelet activation in patients with Fami- lial Mediterranean Fever. Platelets 2008; 19: 405-408.
24.Makay B, Türkyilmaz Z, Unsal E. Mean platelet volume in children with familial Mediterranean fever. Clin Rheumatol 2009; 28: 975-978.
25.Robinson SD, Harding SA, Cummins P. Functional interplay between platelet activation and endothelial dysfunction in pa- tients with coronary heart disease. Platelets 2006; 17: 158– 162.
26.Yuksel S, Ayvazyan L, Gasparyan AY. Familial Mediterra- nean fever as an emerging clinical model of atherogenesis as- sociated with low-grade inflammation. Open Cardiovasc Med J 2010; 4: 51-56.
27.Wagner DD, Burger PC. Platelets in inflammation and throm- bosis. Arterioscler Thromb Vasc Biol 2003; 23: 2131–2137.
28.Smid J, Braun-Dullaeus R, Gawaz M, Langer HF. Platelet interactions as therapeutic targets for prevention of atheroth- rombosis. Future Cardiol 2009; 5: 285–296.
29.Ihara A, Kawamoto T, Matsumoto K, Shouno S, Morimoto T, Noma Y. Relationship between hemostatic factors and the pla- telet index in patients with ischemic heart disease. Pathophy- siol Haemost Thromb 2006; 35: 388-391.
30.Ihara A, Matsumoto K, Kawamoto T, et al. Relationship between hemostatic markers and platelet indices in patients with aortic aneurysm. Pathophysiol Haemost Thromb 2006; 35: 451-456.