No abstract

Objective: 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 İzmir 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 İzmir 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.Conflict of interest:None declared.

Objectives. Ulcerative colitis (UC) and Crohn's disease (CD) are chronic inflammatory bowel diseases (IBD) with unclear underlying aetiologies. Severe cardiac arrhythmias have been emphasised in a few studies on adult IBD patients. This study aimed to investigate the alteration of the P-wave and QT interval dispersion parameters to assess the risk of atrial conduction and ventricular repolarisation abnormalities in pediatric IBD patients. Patients and Methods. Thirty-six IBD patients in remission (UC: 20, CD: 16) aged 3–18 years and 36 age- and sex-matched control patients were enrolled in the study. Twelve-lead electrocardiograms were used to determine durations of P-wave, QT, and corrected QT (QTc) interval dispersion. Transthoracic echocardiograms and 24-hour rhythm Holter recordings were obtained for both groups. Results. The P-wave dispersion, QT dispersion, and QTc interval dispersion (Pdisp, QTdisp, and QTcdisp) were significantly longer in the patient group. The mean values of Pminimum, Pmaximum, and QTcminimum were significantly different between the two groups. The echocardiography and Holter monitoring results were not significantly different between the groups. Furthermore, no differences in these parameters were detected between the CD and UC groups. Conclusion. Results suggest that paediatric IBD patients may carry potential risks for serious atrial and ventricular arrhythmias over time even during remission.

Objective: The aim of this study was to evaluate the association of tenascin-C (TnC) and total oxidant-antioxidant status to rheumatic or congenital 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. (Anadolu Kardiyol Derg 2013; 13: 350-6) Key words: Children, rheumatic valve disease, congenital valve disease, tenascin-C, total antioxidant capacity, total oxidant status Original Investigation Özgün Araşt›rma 350ÖZET Amaç: Bu çalışmanın amacı çocukluk döneminde romatizmal veya konjenital kapak hastalıklarının serum tenascin-C (TnC) ve total oksidan-antioksidan 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, p<0.01; 4.72±1.77 ng/mL, p<0.05]. Ancak konjenital grup ile kontrol grubunun TnC düzeyleri arasında istatistiksel 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 sisteml...

The increased level of ischaemia-modified albumin in children with acute rheumatic fever seems to be associated with inflammation. However, further studies are needed to provide stronger evidence.

This study aimed to investigate homogeneity disorders of ventricular repolarization and atrial conduction via QT dispersion and P-wave dispersion in children with congenital heart disease (CHD) and pulmonary arterial hypertension (PAH). Three groups of 20 each were generated and involved in this study. The first group included 20 children with both CHD and PAH. The second group consisted of 20 children with CHD but no PAH, and the third group was composed of 20 age- and sex-matched healthy children. Electrocardiographic records were used to determine P-wave, QT, and corrected QT (QTc) dispersions. The pulmonary hypertension group compared with the group having no pulmonary hypertension and the control group showed a significantly longer P-wave dispersion duration (39.10 ± 9.54 vs. 26.30 ± 4.99 ms, p < 0.001; and 24.80 ± 6.94 ms, p < 0.001, respectively) and QT dispersion duration (52.80 ± 15.11 vs. 37.60 ± 6.00 ms, p < 0.001; and 35.00 ± 7.77 ms, p < 0.001, respectively). In addition, the durations of maximum QTc and QTc dispersion were significantly longer in pulmonary hypertension group than in the other two groups. The risk of atrial and ventricular arrhythmia was found to be increased in the patients with both CHD and PAH. Physicians should pay close attention to possible atrial and ventricular arrhythmias during the treatment and follow-up evaluation of these patients.

Obesity is a major risk factor for cardiovascular diseases. In our study, we showed that obese children have early subclinical systolic and diastolic dysfunctions. Also, these cardiac impairments are correlated with the increase in IMT, EAT thickness and LVM.

PHACE syndrome is characterized by posterior fossa malformations (P), large facial hemangiomas (H), arterial anomalies (A), cardiac anomalies or coarctation of aorta (C), and eye anomalies (E) and has striking female predominance. Endocrine abnormalities have recently been described in these patients, involving the thyroid and pituitary glands. We report the case of a 2-year-old girl with the clinical features of PHACE with absence of bilateral internal carotid arteries and isolated growth hormone deficiency.