Resistant Chorea Successfully Treated With Intravenous Immunoglobulin: A Case Report*

Sydenham’s chorea (SC) is common cause of acquired chorea in childhood. SC occurs mainly in children with untreated streptococcal infections. An effective list of therapeutic options has been used to treat this disorder: antiepileptic drugs (valproic acid, carbamazepine etc.), haloperidol, chlorpromazine, amphetamines, steroids, plasma exchange and intravenous immunoglobulins (IVIG). We report a 12-year-old girl with carditis and severely generalized chorea and successfully treated with IVIG. This case report shows that IVIG is an effective treatment for the chorea cases resistant to anticonvulsants, dopamine antagonists and steroids, although larger studies are needed to confirm this conclusion

Evaluation of Electrocardiographic Markers for the Risk of Cardiac Arrhythmia in Children with Obesity

Aim:This study was conducted to examine the electrocardiographic markers used in the risk assessment of cardiac arrhythmia in children with obesity.Materials and Methods:In this prospective study, 60 children aged 3-17 years with exogenous obesity and 60 age and sex-matched healthy controls were included. Demographic data, assessment of atrial and ventricular arrhythmia risk markers in electrocardiography, and standard echocardiography measurements were performed. Values of p<0.05 were considered significant.Results:The mean ages of the study and control groups were 11.51±3.48 years and 10.74±3.72 years, respectively. Both groups had 30 males and 30 females. The study group had significantly higher average mean body mass index (BMI) compared to the control group. In electrocardiographic examinations, P-wave dispersion, QT dispersion (QTd), corrected QTd (QTcd), Tpeak-Tend (Tp-e), Tp-e/QT, and Tp-e/QTc values were significantly higher in the obese group compared to the control group. In echocardiographic examinations, the dimensions of the heart chambers and vascular structure and wall thicknesses were found to be significantly higher in those children with obesity.Conclusion:The electrocardiographic risk markers used to predict cardiac arrhythmias were found to be increased in those children with obesity. This may suggest that increased body weight and adiposity may have unfavorable effects on the cardiac conduction system

Fabry Perot Tayfölçeri ve Dört Galaktik Hα Ölçümleri

Özet: Bu çalışma dört farklı iyonize olmuş hidrojen kaynağından (NGC281, NGC6992, NGC7000 ve NGC7635) Hα (=6563Å) alınan tayf çizgilerini vermektedir. Gözlemlerimizde TÜBİTAK Ulusal Gözlemevi’nde (TUG) bulunan RTT150 teleskopunun (f/48) coude çıkışına yerleştirilen DEFPOS Fabry-Perot tayfölçeri kullanılmıştır. Tayfölçer ile birlikte 2048x2048 piksel boyutlu termoelektrik (TE) soğutmalı Andor iKon-L CCD görüntüleme kamerası kullanılmıştır. Tayfların parlaklık kalibrasyonu için NGC7000 bulutsusundan (2000=20h58m04s.0, δ2000=+44◦35′43′′.0) 4 açı dakikalık bölgesinden alınan tayflar kullanılmıştır. Tüm verilerin parlaklık, yarı genişlik (FWHM) ve LSR’a göre hız değerleri belirlenmiştir. Elde edilen tüm kaynakların hız ve yarı genişlik değerleri literatürde verilen sonuçlarla karşılaştırılmış ve sonuçların uyumlu olduğu görülmüştür. Anahtar kelimeler: Yıldızlararası ortam, HII bölgeleri, gezegenimsi bulutsular, Fabry-Perot tayfölçerleri, DEFPOS Fabry Perot Spectrometers and Four Galactic Hα Observations Abstract:This work presents H (=6563Å) spectral lines from four different ionized sources (NGC281, NGC6992, NGC7000 ve NGC7635) was used. The DEFPOS Fabry-Perot spectrometer, located at the f/48 coude focus of the RTT150 telescope at TUBITAK National Observatory (TUG) in Antalya/Turkey, was used in our observations. A new 2048x2048 Andor iKon-L CCD imaging camera with TE cooling has been used along with the spectrometer. For intensity calibration of the data, we used spectral lines obtained from NGC7000 emission nebula (2000=20h58m04s.0,δ2000=+44◦35′43′′.0) with FOV of 4arcmin. We determined values of the intensity, the full weight half maximum (FWHM), and the radial velocity with respect to LSR for all data. The line width and radial velocity values obtained from our data were compared with early results from literature and these results were seen to be in close agreement with them. Key words: Interstellar medium, HII regions, planetary nebulae, Fabry-Perot spectrometer, DEFPO

Impaired brachial endothelial function in patients with primary anti-phospholipid syndrome

Although the precise pathophysiology of thrombosis is unknown in primary anti-phospholipid syndrome (PAPS), it is assumed that autoantibodies developed against endothelial cells and platelets might be one of the primary mechanisms. However, whether interaction between autoantibodies and endothelium leads to an impaired vasodilator response has not been investigated yet. In this study, we aimed to investigate the endothelial functions in patients with PAPS. Thirty-one patients with PAPS (22 female, nine male, mean age: 34.6 +/- 8.9 years) and 27 age- and sex-matched, healthy controls were included in the study. Brachial artery responses to reactive hyperaemia (endothelium-dependent dilatation) [EDD] and sublingual nitroglycerine (endothelium-independent dilatation) [EID] were measured by using high-resolution vascular ultrasound both in patients with PAPS and in the controls. The results were expressed as percentage of change in baseline values. Regarding cardiovascular risk factors, there was no significant difference between the two groups. EDD in patients with PAPS was significantly lower than those of controls (6.9 +/- 4.9 vs. 14.8 +/- 4.1%; p<0.0001). EID measurements were not significantly different between the groups. In the PAPS group, EDD in patients with arterial involvement (17 patients) was significantly lower than those of patients with venous involvement (12 patients) (4.6 +/- 3.9 vs. 7.4 +/- 4.1%; p = 0.02)

Ambulatory blood pressure and endothelial dysfunction in patients with autosomal dominant polycystic kidney disease

Cardiovascular problems are a major cause of morbidity and mortality in patients with autosomal dominant polycystic kidney disease (ADPKD). Endothelial dysfunction (ED), which is an early manifestation of vascular injury, has been shown in patients with ADPKD. However, the association between ambulatory blood pressure and ED has not been investigated in these patients. Forty-one patients with ADPKD having well-preserved renal function were included in the study. Ambulatory blood pressure monitoring was performed in all patients. Patients were divided into dipper and non-dipper groups. Endothelial function of the brachial artery was evaluated by using high-resolution vascular ultrasound. Endothelial-dependent dilatation was expressed as the percentage change in the brachial artery diameter from baseline to reactive hyperemia. The mean 24-hour systolic blood pressure was similar in both groups (125.5 +/- 10.7 mmHg in dippers and 121.2 +/- 14.3 in non-dippers, p > 0.05). There was also no significant difference between the mean 24-hour diastolic blood pressures in both groups (82.3 +/- 9.6 mmHg in dippers and 77.1 +/- 8.6 mmHg in non-dippers, p > 0.05). The nocturnal fall rate in systolic blood pressure was 11.1 +/- 1.2% in dippers and 0.98 +/- 0.9% in non-dippers (p = 0.001). The nocturnal fall rate in diastolic blood pressure was 14.0 +/- 0.9% in dippers and 3.8 +/- 0.8% in non-dippers (p = 0.001). Endothelial-dependent dilatation was significantly higher in dippers compared to non-dippers (6.22 +/- 4.14% versus 3.57 +/- 2.52%, p = 0.025). Non-dipper patients with ADPKD show significant ED, which has an important impact on cardiovascular morbidity and mortality

Improvement of endothelial dysfunction with simvastatin in patients with autosomal dominant polycystic kidney disease

Cardiovascular problems are a major cause of morbidity and mortality in patients with autosomal dominant polycystic kidney disease (ADPKD). Endothelial dysfunction (ED), which is an early manifestation of vascular injury, has been shown in patients with ADPKD. Statins have a beneficial effect in the reversal of ED. The aim of this study was to investigate the effects of a statin, simvastatin, on ED in patients with ADPKD. Sixteen patients with ADPKD having well-preserved renal function were included in the study. Endothelial function of the brachial artery was evaluated by using high-resolution vascular ultrasound. Endothelial-dependent dilatation (EDD) was expressed as the percentage change in the brachial artery diameter from baseline to reactive hyperemia. After the baseline evaluations of EDDs. patients were started treatment with simvastatin at a dose of 40 mg/day and were treated for six months. EDDs were recalculated after one and six months of therapy. Interleukin-6 (IL-6) and high-sensitivity C-reactive protein were also measured as markers of inflammation. Baseline EDD was 11.3 +/- 6.9 % in patients with ADPKD. After one month of simvastatin treatment, EDD increased significantly to 14.6 +/- 4.6% (P = 0.016 versus baseline). Endothelial-dependent dilatation further increased significantly to 18.9 +/- 7.5 % (P = 0.011 versus baseline, P = 0.048 versus first month) after six months of therapy. There was also a significant decrease in the level of IL-6 from 21.6 +/- 21.7 pg/mL to 9.1 +/- 3.5 pg/mL (P = 0.002)