The Prevalence of von Willebrand Disease and Significance of in Vitro Bleeding Time (PFA-100) in von Willebrand Disease Screening in the Izmir Region

WOS: 000316239000008PubMed ID: 24385752Objective: von Willebrand disease (vWD) is the most common hereditary bleeding disorder. The purpose of this investigation was to determine the prevalence of vWD among adolescents in Izmir and to assess the sensitivity and specificity of PFA-100 as a screening method in detecting this disease. Material and Methods: Our study was conducted on adolescents in the city of Izmir between October 2006 and March 2007. A total of approximately 1500 high school students between 14 and 19 years of age were planned to be included in the investigation. Survey forms prepared for assessing hemorrhagic diathesis were completed by 1339 individuals (512 males, 827 females). The necessary laboratory tests were performed after having obtained written informed consent from 40 individuals suspected to have hemorrhagic diathesis. Results: Based on the von Willebrand factor antigen (vWF:Ag) and ristocetin cofactor activity (vWF:RCo) levels and bleeding symptoms, vWD type-1 was diagnosed in 14 individuals (4 males, 10 females; prevalence: 1.04%). The most common bleeding symptom in these patients was found to be epistaxis (10/14). Screening with PFA-100 revealed prolongation in both cartridges (Col/ADP and Col/Epi) in 3 of the 14 patients. PFA-100 was determined to exhibit 21.4% sensitivity and 100% specificity in the diagnosis of vWD. Conclusion: The PFA-100 device was found to have high specificity but to have exhibited low sensitivity. Therefore, its utilization as a screening test may be problematic in patients with mild type-1 vWD. Specific tests (vWF:RCo, vWF:Ag) are required for the definite diagnosis of vWD. However, further studies with a large number of patients are needed

Tenascin-C ve oksidatif stresin romatizmal ve konjenital kalp kapak hastalığındaki rolü: Gözlemsel bir çalışma

WOS:000322679800009PubMed ID: 23531872Amaç: Bu çalışmanın amacı çocukluk döneminde romatizmal veya konjenital kapak hastalıklarının serum tenascin-C (TnC) ve total oksidan-antiok- sidan seviyeleri ile ilişkisini değerlendirmektir. Yöntemler: Yaşları 3-17 arasındaki 50 çocuk hasta (25 romatizmal kapak hastası, 25 konjenital kapak hastası) ile yaşça uyumlu 20 sağlıklı birey bu enine kesitli ve gözlemsel çalışmaya alındı. Tenascin-C, total anti-oksidan kapasite (TAC), total oksidan seviye (TOS) ve oksidatif stres indeks (OSİ) değerleri gruplar arasında karşılaştırıldı. Gruplar arası karşılaştırmalarda ANOVA ve Kruskal-Wallis testi kullanıldı. Bulgular: Romatizmal kalp hastalığı grubunun TnC düzeyleri [ortanca 9.09 (0.94-46.30) ng/mL] konjenital ve kontrol gruplarından yüksek bulundu [sırasıyla ortanca 2.97 (0.66-11.80) ng/mL, p0.01; 4.721.77 ng/mL, p0.05]. Ancak konjenital grup ile kontrol grubunun TnC düzeyleri arasında ista- tistiksel olarak anlamlı bir farklılık saptanmadı. Gruplar arasında TAC, TOS ve OSI değerleri açısından da farklılık saptanmadı. Tenascin-C düzeyi ile TOS ve OSI arasında da korelasyon saptanmadı. Sonuç: Tenascin-C, romatizmal kapak hastalıkları ile konjenital kapak hastalıkları ayırıcı tanısında bir biyokimyasal marker olarak kullanılabilir. Romatizmal ve konjenital kapak hastalıklarında oksidan ve antioksidan sistemlerin denge içinde olması, çocukluk döneminde oksidatif stresin romatizmal kalp hastalığı etyopatogenezinde belirgin rolü olmadığını düşündürmektedir.Objective: The aim of this study was to evaluate the association of tenascin-C (TnC) and total oxidant-antioxidant status to rheumatic or con- genital heart valve diseases (HVD) in pediatric patients. Methods: Fifty pediatric patients (25 rheumatic HVD patients and 25 congenital HVD patients) and 20 healthy age-matched control subjects, aged 3-17 years, were enrolled in this observational and cross-sectional study. Serum total antioxidant capacity (TAC), total oxidant status (TOS), oxidative stress index (OSI) and TnC levels were compared among the groups. ANOVA and Kruskal-Wallis tests were used for statistical analysis. Results: Serum TnC level of the patients with rheumatic HVD [median 9.09 (0.94-46.30) ng/mL] was significantly higher than both congenital HVD and control groups [median 2.97 (0.66-11.80) ng/mL; p>0.01, 4.72±1.77 ng/mL; p>0.05, respectively]. However, there was no statistically significant difference between the congenital and control groups in terms of serum TnC level. The levels of serum TAC, TOS and OSI were found to be statistically similar in all groups. In addition, there were no correlations between the level of TnC, and TOS and OSI. Conclusion: Tenascin-C can be used as a biochemical marker in the differential diagnosis of rheumatic and congenital HVD. As the oxidant and antioxidant systems were found to be in equilibrium in rheumatic and congenital HVD, oxidative stress can be thought not to have a marked role in the etiopathogenesis of rheumatic HVD during childhood