Volume CXIV, Number 4, November 7, 1996

Objective: Turner syndrome (TS) is a chromosomal disorder caused by complete or partial X chromosome monosomy that manifests various clinical features depending on the karyotype and on the genetic background of affected girls. This study aimed to systematically investigate the key clinical features of TS in relationship to karyotype in a large pediatric Turkish patient population.Methods: Our retrospective study included 842 karyotype-proven TS patients aged 0-18 years who were evaluated in 35 different centers in Turkey in the years 2013-2014.Results: The most common karyotype was 45,X (50.7%), followed by 45,X/46,XX (10.8%), 46,X,i(Xq) (10.1%) and 45,X/46,X,i(Xq) (9.5%). Mean age at diagnosis was 10.2±4.4 years. The most common presenting complaints were short stature and delayed puberty. Among patients diagnosed before age one year, the ratio of karyotype 45,X was significantly higher than that of other karyotype groups. Cardiac defects (bicuspid aortic valve, coarctation of the aorta and aortic stenosis) were the most common congenital anomalies, occurring in 25% of the TS cases. This was followed by urinary system anomalies (horseshoe kidney, double collector duct system and renal rotation) detected in 16.3%. Hashimoto's thyroiditis was found in 11.1% of patients, gastrointestinal abnormalities in 8.9%, ear nose and throat problems in 22.6%, dermatologic problems in 21.8% and osteoporosis in 15.3%. Learning difficulties and/or psychosocial problems were encountered in 39.1%. Insulin resistance and impaired fasting glucose were detected in 3.4% and 2.2%, respectively. Dyslipidemia prevalence was 11.4%.Conclusion: This comprehensive study systematically evaluated the largest group of karyotype-proven TS girls to date. The karyotype distribution, congenital anomaly and comorbidity profile closely parallel that from other countries and support the need for close medical surveillance of these complex patients throughout their lifespa

Wpływ karwedilolu i nebiwololu na stan stresu oksydacyjnego u pacjentów z nie-niedokrwienną niewydolnością serca

Background: Carvedilol and nebivolol have favourable properties such as anti-oxidative effects in addition to other beta-blockers. However, which of these drugs is more effective on oxidative stress is unclear. Aim: To compare the effects carvedilol and nebivolol on oxidative stress status in non-ischaemic heart failure (HF) patients. Methods: We included 56 symptomatic non-ischaemic HF patients with ejection fraction ≤ 40%. The patients were randomised to carvedilol (n = 29, 18 male) or nebivolol (n = 27, 18 male) groups. They were evaluated clinically and echocardiographically after target dose. We evaluated parameters associated with oxidative stress, such as alkaline phosphatase (ALP), gamma-glutamyl transferase (GGT), uric acid, total antioxidant capacity (TAC), total oxidative status (TOS), and oxidative stress index (OSI). Results: TAC, TOS, GGT, and ALP levels and OSI were comparable in both groups. Uric acid levels were lower in the carvedilol group compared with the nebivolol group (5.8 ± 1.6 vs. 7.0 ± 1.7 mg/dL, p = 0.01). In correlation analysis, uric acid (p < 0.001, r = 0.50) and TOS level (p < 0.001, r = 0.73) were positively correlated with OSI. Conclusions: Carvedilol and nebivolol have similar effects on oxidative stress status in patients with non-ischaemic HF. Wstęp: Karwedilol i nebiwolol oprócz działań wspólnych dla grupy leków beta-adrenolitycznych mają dodatkowo korzystne właściwości antyoksydacyjne. Jednak nie ustalono, który z tych leków działa skuteczniej na stan stresu oksydacyjnego. Cel: Badanie przeprowadzono w celu porównania wpływu karwedilolu i nebiwololu na stres oksydacyjny u chorych z nie-niedokrwienną niewydolnością serca (HF). Metody: Do badania włączono 56 chorych z nie-niedokrwienną HF, u których frakcja wyrzutowa wynosiła ≤ 40%. Pacjentów przydzielono losowo do grupy leczonej karwedilolem (n = 29, 18 mężczyzn) lub nebiwololem (n = 27, 18 mężczyzn). Po zastosowaniu docelowej dawki chorych poddano badaniu klinicznemu i echokardiograficznemu. Oceniono parametry związane ze stresem oksydacyjnym, takie jak stężenie fosfatazy zasadowej (ALP), gamma glutamylotransferazy (GGT) i kwasu moczowego, całkowita pojemność antyoksydacyjna (TAC), całkowity stan oksydacyjny (TOS) i wskaźnik stresu oksydacyjnego (OSI). Wyniki: Wartości TAC, TOS, GGT, ALP i OSI były podobne w obu grupach. Stężenie kwasu moczowego było niższe wśród pacjentów przyjmujących karwedilol niż u osób stosujących nebiwolol (5,8 ± 1,6 vs. 7,0 ± 1,7 mg/dl, p = 0,01). W analizie korelacji wykazano dodatnią korelację stężeń kwasu moczowego (p < 0,001; r = 0,50) i TOS (p < 0,001; r = 0,73) z OSI. Wnioski: Karwedilol i nebiwolol podobnie wpływają na stan stresu oksydacyjnego u chorych z nie-niedokrwienną HF.

The association of plasma oxidative status and inflammation with the development of atrial fibrillation in patients presenting with ST elevation myocardial infarction

WOS: 000395146100001PubMed ID: 27905214Atrial fibrillation (AF) is the most common supraventricular arrhythmia following ST elevation myocardial infarction (STEMI). Oxidative stress and inflammation may cause structural and electrical remodeling in the atria making these critical processes in the pathology of AF. In this study, we aimed to evaluate the association between total oxidative status (TOS), total antioxidative capacity (TAC) and high-sensitivity C-reactive protein (hs-CRP) in the development of AF in patients presenting with STEMI. This prospective cohort study consisted of 346 patients with STEMI. Serum TAC and TOS were assessed by Erel's method. Patients were divided into two groups: those with and those without AF. Predictors of AF were determined by multivariate regression analysis. In the present study, 9.5% of patients developed AF. In the patients with AF, plasma TOS and oxidative stress index (OSI) values were significantly higher and plasma TAC levels were significantly lower compared to those without AF (p=.003, p=.002, p<.0001, respectively). Multivariate regression analysis results showed that, female gender (Odds ratio[OR]=3.07; 95% Confidence Interval[CI]=1.26-7.47; p=.01), left atrial diameter (OR =1.28; 95% CI =1.12-1.47; p<.0001), hs-CRP (OR =1.02; 95% CI =1.00-1.03; p=.001) and OSI (OR =1.10; 95% CI =1.04-1.18; p=.001) were associated with the development of AF in patients presenting with STEMI. The main finding of this study is that oxidative stress and inflammation parameters were associated with the development of AF in patients presenting with STEMI. Other independent predictors of AF were female gender, left atrial diameter and hs-CRP

Evaluation of Methyleletetrahydrofolate Reductase 677C > T Polymorphism in Patient with Atrial Fibrillation with Ischemic Stroke

29th Turkish Cardiology Congress of the Turkish-Society-of-Cardiology (TSC) with International Participation -- OCT 26-29, 2013 -- Antalya, TURKEYADALI, MEHMET KORAY/0000-0002-0054-6252WOS: 000329858400101…Turkish Soc Cardio

Association between the use of renin-angiotensin system blockers and development of in-hospital atrial fibrillation in patients with ST-segment elevation myocardial infarction

Background and aim: Atrial fibrillation (AF) is the most common supraventricular arrhythmia following ST-segment elevation myocardial infarction (STEMI). We evaluated the association between use of previous angiotensin converting enzyme inhibitors and/or angiotensin receptor blockers (renin-angiotensin system [RAS] blockers) and started RAS blockers after MI and development of AF in patients presenting with acute STEMI. Materials and methods: This retrospective study enrolled 1000 patients with acute STEMI who were admitted to the coronary care unit. Patients were divided into groups according to the use of RAS blockers before MI and development of AF rates was compared. Predictors of AF were determined by multiple logistic regression analysis. Results: Of the 1000 patients presenting with STEMI, 247 received and 753 did not receive RAS blockers. The incidence of AF was 7.9%. The incidence of AF in patients receiving RAS blockers and did not receiving RAS blockers before MI were similar (5.7% vs. 8.6% respectively, P = 0.13). On the other hand, AF rate was lower in patients in whom RAS blockers were administered during MI as compared to those in whom these agents were not administered (7.2% vs. 28.6%, P < 0.001). Multiple regression analysis results showed that administration of RAS blockers or statins during hospitalization and left atrial diameter were associated with development of AF in patients with acute STEMI. Conclusions: Previous therapy with RAS blockers does not reduce the incidence of AF in STEMI. Administration of RAS blockers at the hospital may decrease the AF rate in STEMI

Anthropometric findings from birth to adulthood and their relation with karyotpye distribution in Turkish girls with Turner syndrome

WOS: 000373099300016PubMed ID: 26788866To evaluate the anthropometric features of girls with Turner syndrome (TS) at birth and presentation and the effect of karyotype on these parameters. Data were collected from 842 patients with TS from 35 different centers, who were followed-up between 1984 and 2014 and whose diagnosis age ranged from birth to 18 years. Of the 842 patients, 122 girls who received growth hormone, estrogen or oxandrolone were excluded, and 720 girls were included in the study. In this cohort, the frequency of small for gestational age (SGA) birth was 33%. The frequency of SGA birth was 4.2% (2/48) in preterm and 36% (174/483) in term neonates (P<0.001). The mean birth length was 1.3cm shorter and mean birth weight was 0.36kg lower than that of the normal population. The mean age at diagnosis was 10.1 +/- 4.4 years. Mean height, weight and body mass index standard deviation scores at presentation were -3.1 +/- 1.7, -1.4 +/- 1.5, and 0.4 +/- 1.7, respectively. Patients with isochromosome Xq were significantly heavier than those with other karyotype groups (P=0.007). Age at presentation was negatively correlated and mid-parental height was positively correlated with height at presentation. Mid-parental height and age at presentation were the only parameters that were associated with height of children with TS. The frequency of SGA birth was found higher in preterm than term neonates but the mechanism could not be clarified. We found no effect of karyotype on height of girls with TS, whereas weight was greater in 46,X,i(Xq) and 45,X/46,X,i(Xq) karyotype groups. (c) 2016 Wiley Periodicals, Inc

Growth curves for Turkish Girls with Turner Syndrome: Results of the Turkish Turner Syndrome Study Group

Objective: Children with Turner syndrome (TS) have a specific growth pattern that is quite different from that of healthy children. Many countries have population-specific growth charts for TS. Considering national and ethnic differences, we undertook this multicenter collaborative study to construct growth charts and reference values for height, weight and body mass index (BMI) from 3 years of age to adulthood for spontaneous growth of Turkish girls with TS