Dahili Tıp Bilimleri Bölümü
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Article Citation - WoS: 3Citation - Scopus: 57-15 years of age group children' hypertension and obesity(Derman Medical Publ, 2013) Battaloğlu İnanç, Betül; Battaloğlu, Betül İnanç; Inanc, Betul BattalogluAmaç: Mardin ili, ilköğretim okullarındaki öğrencilerde, hipertansiyon ve obezite değerlendirilmesi amaçlandı. Gereç ve Yöntem: Tanımlayıcı tipteki bu çalışma, Mardin ilinin, farklı sosyoekonomik düzeyindeki, üç ilköğretim okulunda, 3460 öğrenciyle yürütüldü. Öğrencilerin, obeziteleri Dünya Sağlık Örgütü’ne (WHO), kan basınçları Amerikan Pediatri Akademisi’nin yayınladığı standartlara göre tanımlandı. Çalışmanın verileri SPSS paket programında, ki-kare testi ile değerlendirildi. İstatistiksel anlamlılık p <0.05 olarak belirlendi. Bulgular: Fazla ağırlığı olan çocuk % 15.78, obez olan çocuk oranı % 10.57’ dir. Obezite sıklığı kız çocuklar için % 9.05 ve erkek çocuklar için % 11.97’ dir ( p<0.01). Öğrencilerin % 1.30’ unda presistolik hipertansiyon, % 2.02’ sinde sistolik hipertansiyon, % 2.65’ inde prediyastolik hipertansiyon ve % 2.74’ ünde diyastolik hipertansiyon görüldü. Ailesinde hipertansiyon olanlar ve onların çocukları arasında hipertansiyon ilişkisi yoktu. İki saatin üzerinde televizyon seyreden çocuklarda, obezite mevcuttu ( p=0.03). Sonuç: Okul sağlığı açısından, hipertansiyon ve obezite değerlendirmeleri gereklidir. Bu nedenle okul çağı çocuklarının muayenelerinde, bu konu mutlaka yer almalı, hipertansiyon ve obezite konusunda öğrencilere ve ailelerine eğitimler verilmelidir.Article Citation - WoS: 3Aging and cardiac implantable electronic device complications: is the procedure safe in older patients?(ELSEVIER, 2023) Güzel, Tuncay; Aktan, Adem; Kılıç, Raif; Günlü, Serhat; Arslan, Bayram; Arpa, Abdulkadir; Güzel, Hamdullah; Tatlı, İsmail; Aydın, Saadet; Suzan, Veysel; Demir, MuhammedBackground In this study, we investigated whether there is a higher incidence of cardiac implantable electronic devices (CIED) procedures related complications in older (≥75 years) than in younger (<75 years) patients. Methods This retrospective cohort study enrolled patients who had undergone CIED procedures (de novo implantation, system upgrade, generator substitution, pocket revision or lead replacement) at two heart centers in Turkey between January 2011 and May 2023. The primary composite endpoint included clinically signifcant hematoma (CSH), pericardial efusion or tamponade, pneumothorax, and infection related to the device system. Secondary outcomes included each component of the composite end point. Results The overall sample included 1923 patients (1419<75 years and 504 aged≥75 years). There was no diference between the groups in terms of cumulative events defned as primary outcome (3.5% vs. 4.4%, p=0.393). Infection related to device system was signifcantly higher in the≥75 age group (1.8% vs. 3.4%, p=0.034). There was no signifcant diference between the groups in terms of clinically signifcant hematoma and pneumothorax (0.7% vs. 0.4%, p=0.451, 1.4% vs. 1.0%, p=0.477, respectively). In multivariate model analysis, no association was found between age≥75 years and infection related to the device system. Conclusion Infection rates were relatively higher in the patient group aged≥75 years. This patient group should be evaluated more carefully in terms of infection development before and after the procedure.Article Citation - WoS: 3Citation - Scopus: 3Aging and cardiac implantable electronic device complications: is the procedure safe in older patients?(Springer, 2023) Güzel, Tuncay; Aktan, Adem; Kılıç, Raif; Günlü, Serhat; Arslan, Bayram; Arpa, Abdulkadir; Güzel, Hamdullah; Tatlı, İsmail; Aydın, Saadet; Suzan, Veysel; Demir, MuhammedBackground: In this study, we investigated whether there is a higher incidence of cardiac implantable electronic devices (CIED) procedures related complications in older (≥ 75 years) than in younger (< 75 years) patients. Methods: This retrospective cohort study enrolled patients who had undergone CIED procedures (de novo implantation, system upgrade, generator substitution, pocket revision or lead replacement) at two heart centers in Turkey between January 2011 and May 2023. The primary composite endpoint included clinically significant hematoma (CSH), pericardial effusion or tamponade, pneumothorax, and infection related to the device system. Secondary outcomes included each component of the composite end point. Results: The overall sample included 1923 patients (1419 < 75 years and 504 aged ≥ 75 years). There was no difference between the groups in terms of cumulative events defined as primary outcome (3.5% vs. 4.4%, p = 0.393). Infection related to device system was significantly higher in the ≥ 75 age group (1.8% vs. 3.4%, p = 0.034). There was no significant difference between the groups in terms of clinically significant hematoma and pneumothorax (0.7% vs. 0.4%, p = 0.451, 1.4% vs. 1.0%, p = 0.477, respectively). In multivariate model analysis, no association was found between age ≥ 75 years and infection related to the device system. Conclusion: Infection rates were relatively higher in the patient group aged ≥ 75 years. This patient group should be evaluated more carefully in terms of infection development before and after the procedure.Article Citation - WoS: 5Citation - Scopus: 6Bronchiectasis in Türkiye: Data From a Multicenter Registry (Turkish Adult Bronchiectasis Database)(Galenos Publ House, 2024) Edis, Ebru Cakir; Cilli, Aykut; Kizilirmak, Deniz; Coskun, Ayson Sakar; Guler, Nurcan; Cicek, Sedat; Sayiner, Abdullah; Gülmez, İnci; Çağlayan, Benan; Niksarlioglu, Elif Yelda Ozgun; Researchers, TebvebBackground: Bronchiectasis is a chronic lung disease characterized by permanent bronchial wall dilatation. Although it has been known as an orphan disease, it has recently gained attention because of registry -based studies and drug research. Aims: We aimed to use a multicenter database to analyze and compare data regarding the etiology, associated comorbidities, microbiological characteristics, and preventive strategies of bronchiectasis in T & uuml;rkiye to those of other countries. Study Design: A multicenter prospective cohort study. Methods: The multicenter, prospective cohort study was conducted between March 2019 and January 2022 using the Turkish Adult Bronchiectasis Database, in which 25 centers in T & uuml;rkiye participated. Patients aged > 18 years who presented with respiratory symptoms such as cough, sputum, and dyspnea and were diagnosed with non -cystic fibrosis bronchiectasis using computed tomography were included in the study. Demographic information, etiologies, comorbidities, pulmonary functions, and microbiological, radiological, and clinical data were collected from the patients. Results: Of the 1,035 study participants, 518 (50%) were females. The mean age of the patients was 56.1 +/- 16.1 years. The underlying etiology was detected in 565 (54.6%) patients. While postinfectious origin was the most common cause of bronchiectasis (39.5%), tuberculosis was identified in 11.3% of the patients. An additional comorbidity was detected in 688 (66.5%) patients. The most common comorbidity was cardiovascular disease, and chronic obstructive pulmonary disease (COPD) and bronchiectasis was identified in 19.5% of the patients. The most commonly detected microbiological agent was Pseudomonas aeruginosa (29.4%). Inhaled corticosteroids (ICS) were used in 70.1% of the patients, and the frequency of exacerbations in the last year was significantly higher in patients using ICS than in nonusers (p < 0.0001). Age [odds ratio (OR): 1.028; 95% confidence interval (CI): 1.005-1.051], cachexia (OR: 4.774; 95% CI: 2,054-11,097), high modified medical research council dyspnea scale score (OR: 1,952; 95% CI: 1,459-2,611), presence of chronic renal failure (OR: 4,172; 95% CI: 1,249-13,938) and use of inhaled steroids (OR: 2,587; 95% CI: 1,098-6,098) were significant risk factors for mortality. Mortality rates were higher in patients with COPD than in those with no COPD (21.7-9.1%, p = 0.016). Patients with bronchiectasis and COPD exhibited more frequent exacerbations, exacerbation -related hospitalizations, and hospitalization in the intensive care unit in the previous year than patients without COPD. Conclusion: This is the first multicenter study of bronchiectasis in T & uuml;rkiye. The study results will provide important data that can guide the development of health policies in T & uuml;rkiye on issues such as infection control, vaccination, and the unnecessary use of antibiotics and steroids.Conference Object Citation - WoS: 1Clinical characteristics of patients presented with primary adrenal insufficiency due to a p.R451W mutation in the CYP11A1 gene(Karger, 2023) Çayır, Atilla; Demirbilek, Hüseyin; Özbek, Mehmet Nuri; Kurt, İlknur; Karaoğlan, Murat; Albayrak, Serpil; Dündar, Bumin Nuri; Güran, Tülay; Kurt, LknurBackground and objective: The first and rate-limiting step of steroidogenesis is the conversion of cholesterol to pregnanolone which is catalyzed by the P450scc side chain cleavage enzyme (encoded by CYP11A1 gene-SCC). Homozygous recessive mutations of the CYP11A1 gene cause a global steroid hormone deficiency thereby disorders of sexual development in 46, XY individuals with a variable phenotype depending on the mutation characteristics. About 60 cases of SCC deficiency due to CYP11A1 gene mutation have been reported so far. The most common mutation is the c.1351C>T (p.R451W) mutation, which has been detected in 12 cases. We, herein, present the clinical characteristics of 14 cases presented with adrenal insufficiency due to p.R451W mutation in the CYP 11A1 gene. Design and method: Data were retrospectively collected from tertiary pediatric endocrine centers using a standardized proforma. Family history, presenting age, clinical, biochemical, and hormonal characteristics, treatment options, and the follow-up characteristics obtained during their latest follow-up visits were recorded. Results: 14 patients (M/F:7/7) from 10 consanguineous Turkish families were recruited. The mean age of the diagnosis was 3.8±2.4(Range: 1.04-8.5 years). All of the male subjects were completely virilized with no sign of DSD. The main presenting complaints were signs and symptoms of primary adrenal insufficiency. However, despite having signs and symptoms 3 subjects were diagnosed when investigated due to the history of their affected siblings. While glucocorticoid deficiency (elevated ACTH, low cortisol) was present in all cases, none of the male cases had undervirilization excluding androgen deficiency. Mild mineralocorticoid (MC) deficiency was detected in 10/14 of the cases which were recovered in 2 subjects during follow-up. More strikingly, one patient with no MC deficiency at presentation had developed a salt-wasting adrenal crisis during acute illness. Although a deterioration was detected in height SDS, there was not a statistically significant difference between height SDS at presentation (-0.64±1.4), at the latest follow-up visit(-0.90±1.4), and target height SDS (-0.63±0.6). Conclusion: In the present largest case series with a p.R451W mutation in the CYP11A1 gene our results confirmed a milder phenotype for all steroid hormones. Particularly lack of virilization defect in male subjects, and lack of salt-wasting crisis until a relatively late age of diagnosis suggested mild MC and androgen deficiency. Nevertheless, lack of MC deficiency at presentation does not exclude the risk of developing a salt-wasting adrenal crisis. Therefore special caution requires for patients with no MC replacement, particularly during acute illnesses.Article Citation - WoS: 21Citation - Scopus: 20Clinical features of generalized lipodystrophy in Turkey: a cohort analysis(Wiley Online Library, 2023) Özbek, Mehmet Nuri; Yildirim Simsir, Ilgin; Tuysuz, Beyhan; Tanrikulu, Seher; Celik Guler, Merve; Akinci, Baris; Karhan, Asuman NurAim: To describe the Turkish generalized lipodystrophy (GL) cohort with the frequency of each complication and the death rate during the period of the follow-up. Methods: This study reports on 72 patients with GL (47 families) registered at different centres in Turkey that cover all regions of the country. The mean ± SD follow-up was 86 ± 78 months. Results: The Kaplan-Meier estimate of the median time to diagnosis of diabetes and/or prediabetes was 16 years. Hyperglycaemia was not controlled in 37 of 45 patients (82.2%) with diabetes. Hypertriglyceridaemia developed in 65 patients (90.3%). The Kaplan-Meier estimate of the median time to diagnosis of hypertriglyceridaemia was 14 years. Hypertriglyceridaemia was severe (≥ 500 mg/dl) in 38 patients (52.8%). Seven (9.7%) patients suffered from pancreatitis. The Kaplan-Meier estimate of the median time to diagnosis of hepatic steatosis was 15 years. Liver disease progressed to cirrhosis in nine patients (12.5%). Liver disease was more severe in congenital lipodystrophy type 2 (CGL2). Proteinuric chronic kidney disease (CKD) developed in 32 patients (44.4%) and cardiac disease in 23 patients (31.9%). Kaplan-Meier estimates of the median time to diagnosis of CKD and cardiac disease were 25 and 45 years, respectively. Females appeared to have a more severe metabolic disease, with an earlier onset of metabolic abnormalities. Ten patients died during the follow-up period. Causes of death were end-stage renal disease, sepsis (because of recurrent intestinal perforations, coronavirus disease, diabetic foot infection and following coronary artery bypass graft surgery), myocardial infarction, heart failure because of dilated cardiomyopathy, stroke, liver complications and angiosarcoma. Conclusions: Standard treatment approaches have only a limited impact and do not prevent the development of severe metabolic abnormalities and early onset of organ complications in GL.Article Citation - WoS: 4Citation - Scopus: 4Early puberty paradox: an investigation of anxiety levels of mothers and children, children's quality of life, and psychiatric diagnoses(SpringerLink, 2023) Kardaş, Burcu; Kardaş, Ömer; Demiral, Meliha; Özbek, Mehmet NuriEarly puberty signs lead to an increase in anxiety levels of parents and children. The aim of this study was to investigate the quality of life and anxiety levels of girls and their mothers who were admitted to a pediatric endocrinology clinic with concerns about early puberty. Girls and their mothers who were admitted to endocrinology outpatient clinic with concerns about early puberty were compared to healthy control group. Screen for Child Anxiety Related Emotional Disorders (SCARED) parent form, Quality of Life for Children Scale (PedsQL) parent form, and Beck Anxiety Inventory (BAI) were administered to the mothers. Children were evaluated with the Schedule for Afective Disorders and Schizophrenia for School-Age Children (Kiddie-SADS Lifetime Version) (K-SADS-PL). The study sample consisted of 92 girls and 62 of them were administered to clinic with concerns about early puberty. There were 30 girls in early puberty group (group 1), 32 girls were in the normal development group (group 2), and 30 were in the healthy control group (group 3). The anxiety level of group 1 and group 2 was signifcantly higher, and their quality of life was signifcantly lower when compared to group 3 (p<0.001). Mother’s anxiety level was found signifcantly higher in group 2 (p<0.001). It has shown that anxiety level and quality of life of children were associated with anxiety level of mothers and the current Tanner stage (r=0.302, p<0.005). Conclusion: Mothers and children who have concerns about early puberty are negatively afected when early puberty is a possibility. For this reason, educating parents will prevent negative impacts of this situation on children. At the same time, it will decrease health burden.Article Citation - WoS: 2Citation - Scopus: 3The effect of body mass index on complications in cardiac implantable electronic device surgery(WILEY, 2023) Güzel, Tuncay; Demir, Muhammed; Aktan, Adem; Kılıç, Raif; Arslan, Bayram; Günlü, Serhat; Altıntaş, Bernas; Karahan, Mehmet Zülkif; Özbek, Mehmet; Aslan, Burhan; Arpa, Abdulkadir; Coşkun, Mehmet Sait; Altunbaş, Mahsum; Tüzün, Rohat; Akgümüş, Alkame; Karadeniz, Muhammed; Aydın, Saadet; Güzel, Hamdullah; Aslan, Selen Filiz; Söner, Serdar; Taş, Ahmet; Ertaş, FarukBackground: Cardiac implantable electronic device (CIED) procedures are prone to complications. In our study, we investigated the effect of body mass index (BMI) on CIED-related complications. Methods: 1676 patients who had undergone CIED surgery (de novo implantation, system upgrade, generator change, pocket revision or lead replacement) at two heart centers in Turkey and met the study criteria were included in our study. For analysis of primary and secondary endpoints, patients were classified as non-obese (BMI < 25 kg/m2), overweight (25 ≤ BMI < 30 kg/m2), and obese (BMI ≥ 30 kg/m2). The primary endpoint was accepted as cumulative events, including the composite ofclinically significant hematoma (CSH), pericardial effusion or tamponade, pneumoth- orax, and infection related to the device system. Secondary outcomes included each component of cumulative events. Results: The rate of cumulative events, defined as primary outcome, was higher in the obese patient group, and we found a significant difference between the groups (3.0%, 4.3%, 8.9%, p = .001). CSH and pneumothorax rates were significantly higher in the obese patient group (0.3%, 0.9%, 1.9%, p = .04; 1.0%, 1.4%, 3.3%, p = .04, respectively). According to our multivariate model analysis; gender (OR:1.882, 95%CI:1.156–3.064, p = .01), hypertension (OR:4.768, 95%CI:2.470–9.204, p < .001), BMI (OR:1.069, 95%CI:1.012–1.129, p = .01) were independent predictors of cumulative events rates. Conclusions: Periprocedural complications associated with CIED (especially hematoma and pneumothorax) are more common in the group with high BMI.Article The effect of coronary slow flow on ventricular repolarization parameters(ELSEVIER, 2023) Karahan, Mehmet Zülkif; Aktan, Adem; Güzel, Tuncay; Günlü, Serhat; Kılıç, RaifIntroduction: Ischemia due to microvascular dysfunction may be responsible for the heterogeneity of ventricular repolarization in coronary slow flow. To our knowledge, there is no study in which QT interval, Tp-Te interval, index of cardiac-electrophysiological balance (iCEB), and frontal QRS-T angle were evaluated together in patients with CSF. In this study, we examined for the first time the relationship between all these myocardial repolarization parameters and CSF. Materials and methods: The study group included 178 patients (99 female, mean age: 50.6 ± 8.6 years) with isolated CSF without stenotic lesions and with angiographically proven normal coronary arteries. The control group included 120 patients (71 female, mean age: 49.3 ± 9.4 years) with normal coronary angiography. QRS duration, QT interval, QTc interval, Tp-Te interval, Tp-Te/QT, Tp- Te/QTc, iCEB score, and frontal QRS-T angle were calculated from 12‑lead ECGs. Results: There was no significant difference in demographic parameters between the two groups. Compared with the control group, patients with CSF had significantly longer QTmax duration, QT dispersion, Tp-Te interval, and higher iCEB score, wider frontal QRS-T angle. Conclusion: In our study, we found that many of the ventricular repolarization parameters were adversely affected in patients with CSF. Impaired parameters may be associated with the risk of malignant ventricular arrhythmias.Article Citation - WoS: 1Citation - Scopus: 1Effectiveness of Valproic Acid in the Treatment of Sydenham's Chorea and a Literature Review(Sage Publications inc, 2024) Ozgun, Nezir; Akdeniz, OsmanThere is still no evidence-based guideline and consensus on the treatment Sydenham's Chorea (SC). The first-line medication preference of specialists depends on personal experience and is variable. In this study, we evaluate the treatment results of pediatric patients who were treated with valproic acid (VPA). The medical records of 17 patients diagnosed with SC were reviewed retrospectively. The mean time to clinical improvement was found as approximately 5 days, the mean duration of remission as 13.60 & PLUSMN; 3.94 weeks and the mean duration of medication use was found as 17.96 & PLUSMN; 3.81 weeks. No side effects were observed in any of the patients and relapse occurred in 2 patients. A positive correlation was found between the initial C-reactive protein (CRP) level and the duration of medication use. Until evidence-based guidelines are established, VPA can be used as an effective, safe, and inexpensive first-line treatment option, especially in pediatric patients.Article Citation - WoS: 1Citation - Scopus: 2Evaluation and management of neonatal onset hyperinsulinemic hypoglycemia: a single neonatal center experience(Taylor & Francis, 2023) Bezirganoğlu, Handan; Okur, Nilifer; Feryal Taş, Funda; Çelik, Kıymet; Özbek, Mehmet Nuri; Tas, Funda FeryalObjectives: To evaluate the clinical characteristics and treatment options of neonates requiring prolonged hospitalization due to persistent hyperinsulinemic hypoglycemia (HH). Methods: This retrospective cohort study included infants >34 weeks of gestation at birth who were born in our hospital between 2018 and 2021, diagnosed with HH, and required diazoxide within the first 28 days of life. The baseline clinical characteristics, age at the time of diagnosis and treatment options in diazoxide resistance cases were recorded. Genetic mutation analysis, if performed, was also included. Results: A total of 32 infants diagnosed with neonatal HH were followed up. Among the cohort, 25 infants were classified as having transient form of HH and seven infants were classified as having congenital hyperinsulinemic hypoglycemia (CHI). Thirty-one percent of the infants had no risk factors. The median birth weight was significantly higher in the CHI group, whereas no differences were found in other baseline characteristics. Patients diagnosed with CHI required higher glucose infusion rate, higher doses, and longer duration of diazoxide treatment than those in the transient HH group. Eight patients were resistant to diazoxide, and six of them required treatment with octreotide and finally sirolimus. Sirolimus prevented the need of pancreatectomy in five of six patients without causing major side effects. Homozygous mutations in the ABCC8 gene were found in four patients with CHI. Conclusions: The risk of persistent neonatal hyperinsulinism should be considered in hypoglycemic neonates particularly located in regions with high rates of consanguinity. Our study demonstrated sirolimus as an effective treatment option in avoiding pancreatectomy in severe cases.Article Citation - WoS: 1Evaluation of Clinical, Biochemical, and Demographic Characteristics of Paediatric COVID-19 Patients Admitted to Dicle University Hospital(Cureus, 2023) Orhan, Özhan; Akay, Cihan; Şen, VelatIntroduction and aim: In this study, we aim to determine how laboratory parameters were related to the clinical courses of patients admitted to the Dicle University Faculty of Medicine Department of Paediatrics and Paediatric Intensive Care Unit with COVID-19 diagnoses from March 2020 to November 2021. Materials and method: Clinical, biochemical and demographic characteristics of 220 patients between 0 and 16 years old with COVID-19 diagnoses at admission were analysed retrospectively. Results: We found that 57.3% of patients were male and 42.7% female, with a mean age of 107.8 ± 65.5 (range 1-192) months. Of the cases, 48.6% (n = 107) were asymptomatic, 35.5% (n = 78) were mild, 11.8% (n = 26) were moderately severe and 3.6% (n = 8) were severe. The patients’ site of admission, mortality rates, C reactive protein (CRP), lactate dehydrogenase (LDH), ferritin, and fibrinogen levels differed significantly (p < 0.001). Conclusion: It is important to learn about the clinical course of the disease by accurately interpreting the results of blood parameters and appropriate imaging studies.Article Citation - WoS: 2Citation - Scopus: 2Evaluation of Hematological Parameters After Transcatheter Aortic Valve Replacement(Sage Journals, 2023) Karahan, Mehmet Zülkif; Aktan, Adem; Güzel, Tuncay; Kılıç, Raif; Günlü, Serhat; Demir, Muhammed; Ertaş, FarukAlthough transcatheter aortic valve replacement (TAVR) is safe and effective, mortality and bleeding events post procedure are important. The present study investigated the changes in hematologic parameters to evaluate whether they predict mortality or major bleeding. We enrolled 248 consecutive patients (44.8% male; mean age 79.0 ± 6.4 years) undergoing TAVR. In addition to demographic and clinical examination, blood parameters were recorded before TAVR, at discharge, 1 month and 1 year. Hemoglobin levels before TAVR 12.1 ± 1.8 g/dL, 10.8 ± 1.7 g/dL at discharge, 11.7 ± 1.7 g/dL at first month, 11.8 ± 1.4 g/dL at first year (Hemoglobin values compared with pre-TAVR, P < .001, P = .019, P = .047, respectively). Mean platelet volume (MPV) before TAVR 8.72 ± 1.71 fL, 8.16 ± 1.46 fL at discharge, 8.09 ± 1.44 fL at first month, 7.94 ± 1.18 fL at first year (MPV values compared with pre-TAVR, P < .001, P < .001, P < .001, respectively). Other hematologic parameters were also evaluated. Hemoglobin, platelet count, MPV, and red cell distribution width before the procedure, at discharge, and at the first year did not predict mortality and major bleeding in receiver operating characteristic analysis. After multivariate Cox regression analysis, hematologic parameters were not independent predictors of in-hospital mortality, major bleeding, and death at 1 year after TAVR.Article Citation - WoS: 2Evaluation of Hematological Parameters After Transcatheter Aortic Valve Replacement(SAGE, 2023) Karahan, Mehmet Zülkif; Aktan, Adem; Güzel, Tuncay; Kılıç, Raif; Günlü, Serhat; Demir, Muhammed; Ertaş, FarukAlthough transcatheter aortic valve replacement (TAVR) is safe and effective, mortality and bleeding events post procedure are important. The present study investigated the changes in hematologic parameters to evaluate whether they predict mortality or major bleeding. We enrolled 248 consecutive patients (44.8% male; mean age 79.0 ± 6.4 years) undergoing TAVR. In addition to demographic and clinical examination, blood parameters were recorded before TAVR, at discharge, 1 month and 1 year. Hemoglobin levels before TAVR 12.1 ± 1.8 g/dL, 10.8 ± 1.7 g/dL at discharge, 11.7 ± 1.7 g/dL at first month, 11.8 ± 1.4 g/dL at first year (Hemoglobin values compared with pre-TAVR, P < .001, P = .019, P = .047, respectively). Mean platelet volume (MPV) before TAVR 8.72 ± 1.71 fL, 8.16 ± 1.46 fL at discharge, 8.09 ± 1.44 fL at first month, 7.94 ± 1.18 fL at first year (MPV values compared with pre-TAVR, P < .001, P < .001, P < .001, respectively). Other hematologic parameters were also evaluated. Hemoglobin, platelet count, MPV, and red cell distribution width before the procedure, at discharge, and at the first year did not predict mortality and major bleeding in receiver operating characteristic analysis. After multivariate Cox regression analysis, hematologic parameters were not independent predictors of in-hospital mortality, major bleeding, and death at 1 year after TAVR.Article Citation - WoS: 2Citation - Scopus: 2Evaluation of patients diagnosed with congenital hypothyroidism by newborn screening between 2011-2019 in Diyarbakir, Turkey(LWW, 2023) Toktaş, İzzettin; Erdem, Özgür; Sarıbaş, Seyfettin; Özbek, Mehmet NuriThis study aimed to determine the incidence of congenital hypothyroidism in Turkey’s Diyarbakir Province and assess the development and growth conditions of people with congenital hypothyroidism. Patients born between 2011-2019 and diagnosed with congenital hypothyroidism within the scope of the newborn screening program were included. The medical records of these patients were retrospectively reviewed. The length and weight for age, weight for length, and body mass index standard deviation scores were calculated. We investigated the treatment status of the patients, whether their relatives had a similar disorder, and the presence of consanguinity between parents. Blood samples were collected from 380,592 newborns. As a result of further tests, 498 newborns were diagnosed with congenital hypothyroidism (incidence: 1/764). Demographic and anthropometric data of 241 patients were analyzed. The patients comprised 46.9% (n = 113) females and 53.1% (n = 128) males. It was determined that 44.4% of the individuals had transient congenital hypothyroidism and 53.6% had permanent congenital hypothyroidism. The parents of 29.8% of the individuals diagnosed with transient congenital hypothyroidism and 44.2% of the individuals diagnosed with permanent congenital hypothyroidism were consanguineous (P = .02). According to the latest anthropometric assessment, 6.8% of individuals diagnosed with congenital hypothyroidism had a weight z-score below −2 SD and 16.9% had a length z-score below −2 SD. The incidence of congenital hypothyroidism was higher in our region. The ratio of consanguinity between parents was higher in patients diagnosed with permanent congenital hypothyroidism than in those diagnosed with transient congenital hypothyroidism. According to the most recent follow-up, weight and age were found to be similar in patients with transient and permanent congenital hypothyroidism.Article Citation - WoS: 6Citation - Scopus: 7Evaluation of patients diagnosed with phenylketonuria and biotinidase deficiency by the newborn screening program: a ten-year retrospective study(The Turkish Journal of Pediatrics, 2022) Toktaş, İzzettin; Sarıbaş, Seyfettin; Canpolat, Semih; Erdem, Özgür; Özbek, Mehmet NuriBackground. Phenylketonuria (PKU) and biotinidase deficiency (BD) are autosomal recessive diseases. If they are not identified and treated early, severe intellectual disability and developmental delay occur. This study was conducted to calculate the ten-year incidence of PKU and BD in the Diyarbakır province of Turkey. Methods. This cross-sectional study included patients born between 2011-2020 and diagnosed with PKU and BD. Patients with a clear diagnosis had their records evaluated retrospectively. Results. Between 2011 and 2020, blood was taken from 417,525 newborns’ heels in Diyarbakir province. As a result of further diagnostic testing, 53 PKU (Incidence: 1:7878) and 177 BD (Incidence: 1:2359) were detected. Of the patients with BD, 56% had profound BD and 44% had partial BD. The records of a total of 269 patients (PKU: 25; BD: 123; Hyperphenylalaninemia: 121) were examined. Parents of 65% (n=15) of the patients diagnosed with PKU and 46.6% (n=55) of the patients diagnosed with BD were consanguineous. Conclusions. The incidence of both PKU and BD was found to be high in our region. The high number of consanguineous marriages was regarded as the most important explanation for the high frequency of these illnesses.Article Citation - WoS: 2Citation - Scopus: 3Examining the effect of COVID-19 vaccines on the menstrual cycle: A study from Turkey(LWW, 2023) Toktaş, İzzettin; Akelma, Hakan; Araç, EşrefConcerns about a possible relationship between vaccination against Coronavirus Disease 2019 (COVID-19) and menstrual disorders have been raised in the media. In addition, different studies have shown that the COVID-19 vaccine may be associated with menstrual changes. This study was conducted to investigate the effects of COVID-19 vaccines on the menstrual cycle in women. This cross-sectional descriptive study was conducted between August 16 and September 17, 2021. Data were collected through a self-administered questionnaire via an online form sent to the participants through social media. Data of 586 women were included in this study. A total of 82.4% (n = 483) of the participants were aged between 31 and 50 years. The BioNTech vaccine (2 doses) was administered to 75.8% (n = 444), Sinovac (3 doses) to 9.0% (n = 53) of the participants. 53.1% (n = 311) of the women experienced changes in their menstrual cycles. The most common menstrual changes after vaccination were delayed menstruation (n = 176; 30.0%) and prolonged menstrual duration (n = 132; 22.5%). Menstrual delay, prolonged menstrual duration, heavy bleeding, and early menstruation were more common in women than prior to receiving the vaccine (P < .05). More than half of the women experienced menstrual cycle changes after receiving the COVID-19 vaccine. Women experienced significantly higher rates of menstruation delay, prolonged menstrual duration, heavy bleeding, and early bleeding compared to before vaccination.Article Citation - WoS: 10Citation - Scopus: 13Glutaric aciduria and L-2-hydroxyglutaric aciduria: Clinical and molecular findings of 35 patients from Turkey(ScienceDirect, 2023) Özbek, Mehmet Nuri; Ergül Bozaci, Ayse; Er, Esra; Ünal, Aysel Tekmenuray; Tas, Ibrahim; Ayaz, Ercan; Durmaz, Asude; Aykut , Ayçe; Kose, Melis; Bozaci, Ayse ErgulBackground: Cerebral organic acid disorders are progressive neurometabolic diseases characterized by neurologic dysfunction. Glutaric aciduria type I (GA-I) and L-2-hydroxyglutaric aciduria (L2HGA) are the main cerebral organic acid disorders. They are both classified as, and it is suggested that these two disorders may share a common metabolic pathway. Current treatment strategies are based on levocarnitine, vitamin B2, and diet. Recent guidelines recommend a lysine-restricted diet up to six years of age, but there is no consensus for patients over the age of six. Vitamin B2 is exists in the blood as riboflavin and its cofactors, flavin mononucleotide and flavin adenine dinucleotide (FAD). FAD, the cofactor of L2HGD, accelerates the conversion of L-2-hydoxy glutarate to alpha-ketoglutarate. Levocarnitine stimulates the formation and excretion of derivatives of glutaric acid. Also, lysine-associated organic acidurias some results provide principal proof for the beneficial effects of riboflavin in GA-I. It has been previously reported that combination therapy with riboflavin and levocarnitine is effective for L2HGA as well as GA-I. Riboflavin and levocarnitine have been reported to improve not only clinical symptoms but also urinary 2-HGA levels. In our study, we aimed to evaluate the effect of the current treatment strategies and genotype on urinary metabolites and IQ scores in GA-I and L2HGA patients. Methods: The presented retrospective multicenter study included patients followed up in Diyarbakir Children's Hospital and Izmir Katip Celebi University Faculty of Medicine, Division of Pediatric Metabolism. Between 2016 and 2021, we retrospectively evaluated 35 patients with confirmed diagnosis of GA-I and L-2HGA. We analyzed the clinical, biochemical, neuroradiological, molecular data and treatment of the patients. The follow-up period was every 2 months until 12 months old, every 3 months until 6 years of age, and every 6 months thereafter. Therapy monitoring was undertaken during follow-up visits that included evaluation of clinical parameters, laboratory parameters, and dietary consumption records. Denver II was applied in order to evaluate children aged 0–6 years in terms of development. Patients between 6 and 16 years of age were evaluated using the Wechsler Intelligence Scale for Children-Revised. Results: We identified 25 with GA-I and 10 with L2HGA. The most common clinical symptoms were developmental delay, intellectual disability, and movement disorders. Behavioural problems were more common in L2HGA than in GA-I patients. In the same family, there were patients with severe developmental delay despite early diagnosis and treatment and individuals with normal IQ scores. In our study group, we used diet (lysine restricted or protein controlled), levocarnitine and vitamin B2 for GA-I patients. The mean urinary glutaric acid levels were decreased with treatment in GA-I patients. Group I consisted of 14/25 patients receiving lysine restricted diet and levocarnitine, Group II (8/25) received protein-controlled diet and levocarnitine. Group III (3/25) patients whom had p.Pro248Leu (P248L) variant, received riboflavin in combination with protein-controlled diet and levocarnitine. When we evaluated according to the treatment groups, a significant decrease was observed in urinary glutaric acid levels in group I. But there were no significant difference in Group II and III. The patients with c.1018C > T variant in GCDH gene had higher pre-treatment urinary metabolites and significant reduction in urinary metabolites with treatment was detected. In L2HGA patients, we used levocarnitine and vitamin B2. In all L2HGA patients, there was a significant decrease in the mean urinary 2- hydoxy glutarate with treatment. However, there was no significant difference between the c.164G > A and c.1115delT variants. The mean pre- and post-treatment IQ scores of GA-I patients, no significant difference was observed. Relative neurologic improvement was seen in three L2HGA patients. We found two novel variants, including the c.221A > G (p.Tyr74Cys) in the GCDH gene and the c.738 + 5A > G splice variant in the L2HGDH gene. Conclusions: Glutaric aciduria type I and L2HGA are the most common cerebral organic acidurias. Early and correct diagnosis is crucial. Poor prognosis based on metabolic crises and progressive deterioration still appears. In countries where newborn screening is not performed, a clinical suspicion index is required for cerebral organic aciduria. GA-I and L-2HGA are difficult to examine by medical evidence standards because of the small sample size, regional differences in newborn screening, and medical care limits. More clinical studies are needed to identify effective treatments. However, the significant decrease in urinary glutaric acid levels after treatment in patients on lysine-restricted diet raises the question of whether lysine-restricted diet should be continued after six years of age. We also reported our experience in order to contribute to the literature.Article The Importance of Frontal QRS-T Angle in Predicting the Effectiveness and Success of Thrombolytic Therapy in Patients With Acute Pulmonary Embolism(Cureus, 2023) Durgun, Mehmet; Karahan, Mehmet ZülkifObjective: The frontal QRS-T angle (fQRS-T) is associated with myocardial ischemia and ventricular arrhythmias. On the other hand, acute pulmonary embolism (APE) is a major risk factor for cardiac adverse events. This research aimed to determine whether the fQRS-T, a marker of ventricular heterogeneity, can be used to predict successful thrombolytic therapy in patients with APE. Methods: This was a retrospective observational study. Patients diagnosed with APE and hospitalized in the intensive care unit between 2020 and 2022 were included in the research. A total of 136 individuals with APEs were enrolled in this research. The patients were divided into two groups: thrombolytic-treated (n=64) and non-treated (moderate to severe risk, n=72). An ECG was conducted for each patient, and echocardiography was performed. Results: The mean age of the thrombolytic group was 58.2±17.6 years, with 35 females (55.1% of the group) and 29 males (44.9%). The non-thrombolytic group had a mean age of 63.1±16.2, with 41 females (56.5%) and 31 males (43.5%). Respiratory rate, heart rate, and fQRS-T were higher in the thrombolytic group, and oxygen saturation ratio and systolic and diastolic blood pressure were higher in the non-thrombolytic group (p=0.006, p<0.001, p=0.021; p<0.001, p=0.015, p<0.001, respectively). In the thrombolytic therapy group, comparing pre- and post-treatment ECG data revealed a statistically significant change in the fQRS-T value (p=0.019). Conclusion: The fQRS-T may provide important clues for the successful treatment of APEsArticle Citation - Scopus: 1Investigation of the effect of comorbid psychopathologies on glycemic control in children and adolescents with type 1 diabetes mellitus(Turkish J Clinical Psychiatry, 2022) Kardaş, Burcu; Kardaş, Ömer; Demiral, Meliha; Özbek, Mehmet Nuri; Unal, EdipObjective: The presence of comorbid psychiatric conditions in chronic diseases makes the management of the disease difficult. Our study, we aimed to examine the relationship between psychiatric comorbid conditions and glycemic control in children and adolescents with Type 1 Diabetes. Method: In our study, depending on the number of patients, good and moderate controls were evaluated as a single group, and HbA1c levels of 8.5 and below were included in this group. Children for Depression Inventory (CDI), Screen for Child Anxiety-Related Emotional Disorders (SCARED), Turgay Child and Adolescent Behavioral Disorders Based on DSM-IV Screening and Evaluation Scale were applied. The case and parents were evaluated with K-SADS-PL.Among 778 diabetic patients who were followed up in the pediatric endocrinology clinic, 73 cases between the ages of 8 and 17 who were followed up regularly, who did not have any comorbidities and who accepted to participate in the study were evaluated psychiatrically. Results: Of the 73 cases included in the study, 29 were accepted as the patients with good glycemic control (HbA1c <= 8.5 mg / dl), and 44 as with poor glycemic control (HbA1c>8.5mg / dl). In cases with poor glycemic control, parents' education level and income level were significantly lower, while the rate of attention deficit and hyperactivity disorder, major depressive disorder, social anxiety disorder and psychopathology was significantly higher. Discussion: The findings of this study revealed that there are many factors affecting glycemic control and there is a strong relationship between glycemic control and psychopathologies.

