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Öğe Advanced Parameters of Cardiac Mechanics in Children With Chronic Kidney Disease (CKD): a Preliminary Report From the Cardiovascular Comorbidity in Children with CKD (4C) Study(Springer, 2013) Franceschini, Alessio; Chinali, Marcello; Matteucci, Maria Chiara; Doyon, Anke; Bayazit, Aysun K.; Sozeri, Betul; Wuehl, Elke; Schaefer, FranzÖğe Aortic Pulse Wave Velocity in Healthy Children and Adolescents: Reference Values for the Vicorder Device and Modifying Factors(Oxford Univ Press, 2015) Thurn, Daniela; Doyon, Anke; Sozeri, Betul; Bayazit, Aysun K.; Canpolat, Nur; Duzova, Ali; Querfeld, Uwe; Schmidt, Bernhard M. W.; Schaefer, Franz; Wuhl, Elke; Melk, AnetteBACKGROUND Aortic pulse wave velocity (PWV), an indicator of arterial stiffness, independently predicts cardiovascular mortality risk in adults. Arterial stiffening advances with age and seems accelerated in children with certain disease conditions such as chronic kidney disease or diabetes. The Vicorder, an oscillometric device to measure PWV, has been validated in children, but reference values in a large pediatric cohort, association to carotid stiffness and influence of individual and family risk factors have not been determined. METHODS Pulse waves were captured in 1,003 healthy children (aged 6-18 years) in 6 centers and gender-specific reference data normalized to age/height were constructed. In 589 children carotid distensibility and intima media thickness were measured. Gestational and family history was reported. RESULTS PWV correlated with age (r = 0.57, P < 0.0001) with significant gender-related differences starting at age 9. Further significant correlations were seen for height, weight, body mass index, blood pressure, pulse pressure, and heart rate. Independent predictors for PWV in a multivariate regression analysis were gender, age, height, weight, mean arterial pressure, and heart rate. Risk factors for higher PWV included small for gestational age at birth, secondhand smoking, parental hypertension, and obesity. PWV showed weak correlations with 2 of the carotid distensibility measures, but not with intima media thickness. CONCLUSION This study defines reference values for PWV captured by the Vicorder device in children and adolescents and reveals associations with potential cardiovascular risk factors in a healthy population. Gender-specific percentiles for age/height will allow for the assessment of pediatric cohorts using this oscillometric method.Öğe Are we late for the diagnosis of acute kidney injury in the intensive care units in pediatric patients? A preliminary, retrospective observational study among 66 patients(Edizioni Minerva Medica, 2016) Bulut, Ipek Kaplan; Kara, Orhan D.; Ozdemir, Kadriye; Dincel, Nida; Yilmaz, Ebru; Bulut, Mustafa O.; Sozeri, Betul; Bicer, Hasan; Mir, SevgiBACKGROUND: The purposes of this study were to emphasize the importance of Risk, Injury, Failure, Loss, and End Stage (RIFLE) classification in early diagnose and prognosis of acute kidney injury (AKI), and to evaluate the practicability of the RIFLE criteria in intensive care units. METHODS: Sixty-six patients applied acute peritoneal dialysis were included into the study. Patients having acute peritoneal dialysis within the first 24 hours of intensive care unit admission were named group 1, between 24-48 hours group 2, and those who had acute peritoneal dialysis 48 hours or more after admission to the intensive care unit were named group 3. Retrospectively, we evaluated patients by RIFLE criteria at the consultation time, and patients who had been just in AKI were called late referral patients. The mean interval time between the onset of AKI and the consultation time was defined as delay time in late referral patients. RESULTS: There were 20 patients in group 1, 15 were in group 2 and 31 in group 3. In total there were 18/66 patients in risk, 13/66 in injury while 35/66 in failure. There was statistically difference between delay times of in risk and failure class in group 3 (P<0.05). Also delayed patient numbers of both risk and failure class were found statistically highly significant (P<0.001). CONCLUSIONS: Most of the patients were in failure class at the time of consultation. We guess that if they would have been diagnosed earlier, prognosis might have been better. Therefore early diagnosis of AKI with RIFLE criteria and early initiation of acute peritoneal dialysis would probably improve prognosis.Öğe Assessment of tuberculosis infection during treatment with biologic agents in a BCG-vaccinated pediatric population(Springer London Ltd, 2016) Atikan, Basak Yildiz; Cavusoglu, Cengiz; Dortkardesler, Merve; Sozeri, BetulBiologic therapies, such as tumor necrosis factor-alpha (TNF-alpha) blockers, are commonly used to treat rheumatological diseases in childhood. Screening patients for tuberculosis (TB) is highly recommended before starting therapy with TNF-alpha blockers. Despite appropriate screening, TB still remains a problem in patients receiving anti-TNF therapy in countries where TB is not endemic. TB in anti-TNF-treated patients is often diagnosed late due to altered presentation, and this delay results in high morbidity and mortality with a high proportion of extrapulmonary and disseminated disease. The aim of this study is to show the course of TB disease in children who are on biologic therapy, in an era where many of the children are BCG-vaccinated and TB is intermediately endemic. We recruited 71 patients with several types of inflammatory diseases. Six of them had a positive test result during TB screening and began taking isoniazid (INH) prophylactically. During the 3 years of follow-up, none of these patients developed TB disease. Biologic agents can be safely used in a BCG-vaccinated pediatric population, as long as patients are closely monitored to ensure that any cases of TB will be detected early.Öğe Assessment of vascular function in systemic onset juvenile idiopathic arthritis(Springer London Ltd, 2016) Sozeri, Betul; Atikan, Basak Yildiz; Ozdemir, Kadriye; Mir, SevgiAn increased incidence of cardiovascular disease has been found in rheumatic disorders. Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disease in children. Prolonged immunological inflammatory process leads these patients to an early onset of atherosclerosis. We aimed to assess the presence of early vascular dysfunction in patients with systemic onset juvenile idiopathic arthritis (sJIA) and investigate the role of therapy sJIA in vascular health. Thirty-three patients (22 males, 11 females) diagnosed with sJIA according to the International League of Associations for Rheumatology criteria were compared to 72 age- and sex-matched controls. None of the participants was overweight, obese, or had a history of hypertension, dyslipidemia, diabetes mellitus, or cardiovascular disease. Arterial stiffness (As) was evaluated by measurement of carotid-femoral pulse wave velocity (PWV) and augmentation index (AIx) with a Vicorder. The mean age of patients in this study was 9.96 +/- 3.71 years (range 4-16 years) and the mean age of controls was 10.9 +/- 3.52 years (range 4-19 years). These two groups were well matched for age, sex, and BMI. The mean age of patients at the onset of disease was 7.06 +/- 3.9 years (range 3-15 years). The mean duration of disease and active disease was 79 +/- 45 months (range 6-162 months) and 58 +/- 49 months (range 1-101 months), respectively. The highest levels of PWV and AIx were found in the patient group. Seven patients had had macrophage activation syndrome at presentation. In these patients, vascular changes were higher than other patients (6.30 +/- 0.42 m/s vs 5.17 +/- 0.55 m/s, p = 0.01, respectively). The corticosteroid therapy was found associated with higher PWV, (p < 0.05), while there was no difference between vascular parameters and use of non-steroid therapies (methotrexate (MTX), anti-TNF alfa agents). We also find statistically significant correlation between PWV and disease duration (p = 0.003, r = 0.45). Vascular function is impaired in patients with sJIA at a very young age. Vascular dysfunction may be partly attributed to the effects of disease-related characteristics (inflammation, disease activity, and medications).Öğe Bioimpedance for assessing volume status in children with nephrotic syndrome(Tubitak Scientific & Technical Research Council Turkey, 2015) Ozdemir, Kadriye; Mir, Makbule Sevgi; Dincel, Nida; Bozabali, Sibel; Bulut, Ipek Kaplan; Yilmaz, Ebru; Sozeri, BetulBackground/aim: The effectiveness of assessing volume load via bioimpedance in pediatric patients with nephrotic syndrome (NS) was investigated. Materials and methods: Patients with NS (n = 34) were compared with healthy controls (n = 20). The inferior vena cava index and inferior vena cava collapsibility index (IVCCI) scores were determined for all subjects. Bioimpedance measurements were used to directly determine volume load. Clinical findings, relative fluid load, and echocardiographic measurements were determined and compared with bioimpedance in calculating volume load. Results: The sensitivity and specificity of bioimpedance in determining volume load were found to be 65% and 90%, respectively. IVCCI was lower in NS patients than in controls. There were also significant differences in IVCCI values between patients with localized and generalized edema. Relative fluid load was higher in NS patients with generalized edema as opposed to patients with localized edema. For calculating volume load, the sensitivity of bioimpedance, clinical findings, and echocardiographic measurements was 87%, 95%, and 83%, respectively, whereas the specificity of determining volume load by bioimpedance, clinical findings, and echocardiographic measurements was 50%, 10%, and 80%, respectively. Conclusion: Our data suggest that bioimpedance may be superior to echocardiography in determining volume load in children with NS, because it is cost-effective, reliable, and relatively simple to perform.Öğe Biological Agents in Familial Mediterranean Fever Focusing on Colchicine Resistance and Amyloidosis(Bentham Science Publ Ltd, 2015) Sozeri, Betul; Kasapcopur, OzgurFamilial Mediterranean fever (FMF) is the most common hereditary autoinflammatory syndrome. FMF is caused by mutations in the MEFV gene which encodes the pyrin protein. FMF is characterized by sporadic, paroxysmal attacks of fever and serosal inflammation, lasting 1-3 days. Patients may develop renal amyloidosis. Colchicine prevents attacks and renal amyloidosis. 5% to 10% of the patients with FMF are resistant or intolerant to colchicine. Colchicine resistant patients may receive biological therapies. Anti-interleukin-1 drugs are the most important agents of biological treatments. In this review, colchicine resistance and treatment options will be evaluated.Öğe The Cardiovascular Comorbidity in Children with Chronic Kidney Disease (4C) Study: Objectives, Design, and Methodology(Amer Soc Nephrology, 2010) Querfeld, Uwe; Anarat, All; Bayazit, Aysun K.; Bakkaloglu, Aysin S.; Bilginer, Yelda; Caliskan, Salim; Civilibal, Mahrnut; Doyon, Anke; Duzova, Ali; Kracht, Daniela; Litwin, Mieczyslaw; Melk, Anette; Mir, Sevgi; Sozeri, Betul; Shroff, Rukshana; Zeller, Rene; Wuehl, Elke; Schaefer, FranzBackground and objectives: Children and adolescents with chronic kidney disease (CKD) are at high risk for cardiovascular morbidity and mortality. A systemic arteriopathy and cardiomyopathy has been characterized in pediatric dialysis patients by the presence of morphologic and functional abnormalities. Design, setting, participants, & measurements: The Cardiovascular Comorbidity in Children with CKD (4C) Study is a multicenter, prospective, observational study aiming to recruit more than 600 children, aged 6 to 17 years, with initial GFR of 10 to 45 ml/min per 1.73 m(2). The prevalence, degree, and progression of cardiovascular comorbidity as well as its association with CKD progression will be explored through longitudinal follow-up. The morphology and function of the heart and large arteries will be monitored by sensitive noninvasive methods and compared with aged-matched healthy controls. Multiple clinical, anthropometric, biochemical, and pharmacologic risk factors will be monitored prospectively and related to the cardiovascular status. A whole-genome association study will be performed to identify common genetic variants associated with progression of cardiovascular alterations and/or renal failure. Monitoring will be continued as patients reach end-stage renal disease and undergo different renal replacement therapies. Results: While cardiovascular morbidity in adults is related to older age and additional risk factor load (e.g., diabetes), the role of CKD-specific factors in the initiation and progression of cardiac and vascular disease are likely to be characterized with greater sensitivity in the pediatric age group. Conclusions: The 4C study is expected to provide innovative insight into cardiovascular and renal disease progression in CKD. Clin J Am Soc Nephrol 5: 1642-1648, 2010. doi: 10.2215/CJN.08791209Öğe Cardiovascular functional and structural changes in children with primary hypertension(Edizioni Minerva Medica, 2016) Mir, Sergi; Sozeri, Betul; Deveci, Murat; Ozdemir, Kadriye; Gun, Zubeyir H.; Dincel, Nida; Gozuoglu, Gozde; Yilmaz, Ebru; Bulut, Ipek KaplanBACKGROUND: Primary hypertension in children is not a benign condition because of end-organ damage. Early investigations of cardiovascular effects rely on vascular structural and functional changes. We aimed to determine presence of early cardiovascular involvement in children with primary hypertension. For this purposes, we investigated functional and morphological changes in vascular system. METHODS; Seventy-five consecutive patients with primary hypertension and 35 healthy children comparable for age and gender were studied. Patients were classified according to the stages of blood pressure. Intima-media thickness of the carotid arteries (cIMT), carotid-femoral pulse wave velocity (PWVcf) and Pulse Wave Augmentation Index (AIx) and Left Ventricular Mass Index (LVMi) were investigated. RESULTS: PWVcf and AIx were higher in patients (5.87 +/- 0.87 m/s vs. 5.29 +/- 0.67 m/s, P=0.02; 9.41 +/- 8.54% vs. 8.36 +/- 3.59%, P=0.04, respectively) than in controls. cIMT was evaluated significantly higher in study group than controls (0.46 +/- 0.06 mm vs. 0.35 +/- 0.12 mm, respectively, P=0.01). The mean LVMi was higher in patients (32.9 +/- 11.5 vs. 28.8 +/- 1.55, P=0.01). CONCLUSION: Our results demonstrated that vascular findings were more sensitive for target organ changes than the more frequently utilized LVMi in patients with pre-hypertension.Öğe A Case of Henoch-Schonlein Purpura with P369S Mutation in MEFV Gene(Iranian Scientific Society Medical Entomology, 2011) Ertan, Pelin; Tekin, Gokhan; Sahin, Gulseren Evirgen; Kasirga, Erhun; Taneli, Fatma; Kandioglu, Ali-Riza; Sozeri, BetulBackground: Henoch-Schonlein purpura (HSP) is the most common vasculitis of childhood. HSP can affect multiple organs presenting with a characteristic rash in most of the patients. Familial Mediterranean Fever (FMF) is an inherited inflammatory disease common in mediterranean populations. HSP is the most common vasculitis seen in children with FMF. Case Presentation: A 16 year old boy was referred with history of abdominal pain lasting for 20 days. He was hospitalized and had appendectomy. Due to the persistence of his abdominal pain after surgery he was admitted to our hospital. His physical examination showed palpable purpuric rashes symmetrically distributed on lower extremities. Abdominal examination revealed periumbilical tenderness. Laboratory tests showed elevated erythrocyte sedimentation rate, C-reactive protein and fibrinogen. Urinalysis revealed microscopic hematuria and severe proteinuria. The fecal occult blood testing was positive. Based on these clinic findings, the patient was diagnosed as HSP with renal, gastrointestinal tract and skin involvement. We performed DNA analysis in our patient because he had diagnosis of vasculitis with severe symptoms and found that he was carrying heterozygote P369S mutation. Conclusion: Our case is noteworthy as it indicates that it may be important not to overlook presence of FMF mutations in patients with a diagnosis of severe vasculitis.Öğe Chronic Recurrent Multifocal Osteomyelitis(Galenos Yayincilik, 2017) Hekimsoy, Ilhan; Argin, Mehmet; Sozeri, BetulChronic recurrent multifocal osteomyelitis (CRMO) is an autoinflamatory disorder (a nonbacterial osteomyelitis) frequently associated with other inflammatory conditions and characterized by bone pain and fever and waxing and waning periods. In this article, details of our CRMO cases were presented and recent research about its epidemiology, etiology, clinical features, histopathologic and radiological findings, and treatment are discussed.Öğe Comparison of the efficacy of once- and twice-daily colchicine dosage in pediatric patients with familial Mediterranean fever - a randomized controlled noninferiority trial(Bmc, 2016) Polat, Adem; Acikel, Cengizhan; Sozeri, Betul; Dursun, Ismail; Kasapcopur, Ozgur; Gulez, Nesrin; Simsek, Dogan; Saldir, Mehmet; Dokurel, Ipek; Poyrazoglu, Hakan; Bakkaloglu, Sevcan; Delibas, Ali; Ekinci, Zelal; Ayaz, Nuray A.; Kandur, Yasar; Peru, Harun; Kurt, Yasemin G.; Polat, Safiye R.; Unsal, Erbil; Makay, Balahan; Gok, Faysal; Ozen, Seza; Demirkaya, ErkanBackground: In this study, we examined the efficacy and safety of a once-daily dosage schema of colchicine compared with a twice-daily dosage schema in pediatric patients with familial Mediterranean fever (FMF). Methods: In this 24-week, multicenter, randomized controlled noninferiority trial, pediatric patients newly diagnosed with FMF carrying a homozygous or compound heterozygous mutation and not receiving any treatment were included. Patients were randomly assigned using a block randomization method to receive treatment with a once-or twice-daily dosage. Clinical and laboratory characteristics and medication side effects were recorded and compared between groups. The study was carried out in compliance with Good Clinical Practice and the Consolidated Standards for Reporting of Trials (CONSORT) statement. Results: A total of 92 patients were selected, and 79 patients completed the study. There were 42 patients in the once-daily dosage group and 37 in the twice-daily dosage group. The results indicated that the once-daily dosage was not inferior to the twice-daily dosage regarding decrease in attack frequency and duration as well as improvement in clinical findings and Mor severity scores. Alterations in laboratory findings indicating inflammation, such as erythrocyte sedimentation rate, C-reactive protein, and serum amyloid A, were similar in both groups. The rates of drug side effects were similar between the once-and twice-daily dosage groups, implying comparable safety of colchicine, with the exception of diarrhea, which was slightly higher in the once-daily dosage group. Conclusions: Using colchicine with either a once-or twice-daily dosage provides similar clinical and laboratory improvements. Considering both efficacy and safety, colchicine can be prescribed with a once-daily dosage.Öğe Comprehensive Analysis of a Large-Scale Screen for MEFV Gene Mutations: Do They Truly Provide a "Heterozygote Advantage" in Turkey?(Mary Ann Liebert Inc, 2011) Berdeli, Afig; Mir, Sevgi; Nalbantoglu, Sinem; Kutukculer, Necil; Sozeri, Betul; Kabasakal, Yasemin; Cam, Sirri; Solak, MustafaFamilial Mediterranean fever (FMF) is a hereditary autoinflammatory disorder characterized by episodes of inflammation in the absence of high-titer autoantibodies or antigen-specific T cells. The Mediterranean fever (MEFV) gene located on chromosome 16p13.3, which encodes the 781-amino-acid protein pyrin, is the causative gene for this monogenic Mendelian disease. This study presents the molecular analysis of an MEFV gene mutation screen of 5518 Turkish individuals with clinical diagnoses of FMF. Patients were genetically diagnosed using the FMF StripAssay and DNA sequencing analysis. Contrary to the results achieved by the FMF StripAssay, DNA sequencing analysis identified large-scale coding and noncoding novel sequence variants, together with a significant group (76%) of individuals who were receiving colchicine and had a single heterozygous mutation, despite the recessive inheritance of FMF. In conclusion, sequence analysis, unlike other routine laboratory techniques, may enable screening for a broad range of nucleotide variations and may prevent less common, population-restricted, novel sequence variants from being overlooked.Öğe The early cardiovascular changes in pediatric patients with systemic lupus erythematosus(Springer, 2013) Sozeri, Betul; Deveci, Murat; Dincel, Nida; Mir, SevgiIn patients with systemic lupus erythematous (SLE) late-onset deaths are due to morbid cardiovascular changes (CVCs). Inflammatory and immune-mediated mechanisms are involved in promoting atherosclerosis development in SLE that is reflected in both functional and morphological changes in the cardiovascular system. The aim of our study was to determine the presence of these changes in pediatric SLE patients. Fifty-one consecutive patients (13 male, 38 female) with SLE and 25 healthy controls were included in the study. Arterial stiffness was assessed by carotid-femoral pulse wave velocity (PWVcf) and augmentation index (AIx), as measured by the Vicorder. Carotid intima-media thickness (cIMT) and the left ventricular mass index (LVMi) were also determined. Patients with SLE, despite equivalent exposure to "traditional" cardiovascular risk factors, presented a higher mean PWVcf and AIx than controls (6.56 +/- 1.45 vs. 5.29 +/- 0.67 m/s, P =0.01 and 14.7 +/- 8.1 vs. 9.36 +/- 3.59 %, P = 0.02, respectively). SLE patients had greater values of cIMT and LVMi than controls (0.54 +/- 0.06 vs. 0.35 +/- 0.12 mm, P = 0.00 and 32.4 +/- 10.8 vs 28.8 +/- 1.5, P = 0.01, respectively). Nine patients had left ventricular hypertrophy (LVMi > 38 g/m(2.7)). There was no significant difference in PWV, AIx, cIMT and LVMi values between presence of hypertension or nephritis within SLE patients. We found significant correlations between all parameters and activity scores. Our results demonstrate that functional and morphological CVCs are independent of traditional risk factors in pediatric SLE patients and correlate with SLE disease activity scores in the early disease stages.Öğe The early cardiovascular changes in pediatric patients with systemic lupus erythematosus(Springer, 2013) Sozeri, Betul; Deveci, Murat; Dincel, Nida; Mir, SevgiIn patients with systemic lupus erythematous (SLE) late-onset deaths are due to morbid cardiovascular changes (CVCs). Inflammatory and immune-mediated mechanisms are involved in promoting atherosclerosis development in SLE that is reflected in both functional and morphological changes in the cardiovascular system. The aim of our study was to determine the presence of these changes in pediatric SLE patients. Fifty-one consecutive patients (13 male, 38 female) with SLE and 25 healthy controls were included in the study. Arterial stiffness was assessed by carotid-femoral pulse wave velocity (PWVcf) and augmentation index (AIx), as measured by the Vicorder. Carotid intima-media thickness (cIMT) and the left ventricular mass index (LVMi) were also determined. Patients with SLE, despite equivalent exposure to "traditional" cardiovascular risk factors, presented a higher mean PWVcf and AIx than controls (6.56 +/- 1.45 vs. 5.29 +/- 0.67 m/s, P =0.01 and 14.7 +/- 8.1 vs. 9.36 +/- 3.59 %, P = 0.02, respectively). SLE patients had greater values of cIMT and LVMi than controls (0.54 +/- 0.06 vs. 0.35 +/- 0.12 mm, P = 0.00 and 32.4 +/- 10.8 vs 28.8 +/- 1.5, P = 0.01, respectively). Nine patients had left ventricular hypertrophy (LVMi > 38 g/m(2.7)). There was no significant difference in PWV, AIx, cIMT and LVMi values between presence of hypertension or nephritis within SLE patients. We found significant correlations between all parameters and activity scores. Our results demonstrate that functional and morphological CVCs are independent of traditional risk factors in pediatric SLE patients and correlate with SLE disease activity scores in the early disease stages.Öğe Effect of Colchicine-Resistant Familial Mediterranean Fever on Growth Parameters(Turkish League Against Rheumatism, 2011) Sozeri, Betul; Yilmaz, Ebru; Mir, Sevgi; Berdeli, AfigObjectives: This study aims to evaluate the growth and development in patients with Familial Mediterranean fever (FMF) resistant to colchicine treatment. Patients and methods: Eighty-seven patients (45 females, 42 males; mean age 9.6 +/- 3.8 years; range 2 to 17 years) diagnosed with FMF according to Tel Hashomer criteria in the Pediatric Nephrology Department of Ege University Faculty of Medicine were included in the study. All patients had leukocytosis and elevated C-reactive protein, fibrinojen levels, erythrocyte sedimentation rate and serum amyloid A levels during attacks. The patients were divided into two groups according to colchicine treatment response. Twenty-seven patients (13 girls, 14 boys; mean age; 9.9 +/- 3.2 years) having frequent attacks (> 1 attact/3 month) despite receiving 2 mg/day colchicine treatment were defined as colchicine-resistant. Sixty patients were defined as colchicine treatment responders. Anthropometric evaluations of the patients were performed at diagnosis and at the end of the follow-up. The height and weight Z scores of patients were used as growth parameters. Results: No statistically significant difference in mutation frequency was found between the groups. There were no significant differences between both groups in the weight and height Z scores calculated from the anthropometric parameters detected at the diagnosis. In the evaluation performed at the end of the follow-up, we found that the height and weight score for age and the body mass index Z score were significantly decreased in the colchicine resistance group (p=0.008, p=0.013, p=0.027). Conclusion: Effective suppression of inflammatory response in patients with FMF provides a positive impact on growth. While colchicine is known as the most effective drug in the treatment of FMF, it is known that there are also patients who fail to respond adequately. The patients with colchicine-resistant FMF should be identified in the early period of the disease and treatment should be arranged accordingly. The detection of growth retardation was found to be important in detecting resistance to colchicine in patients with FMF.Öğe Efficacy of Interleukin-1 Targeting Drugs in Familial Mediterranean Fever Patients.(Wiley-Blackwell, 2014) Cetin, Pinar; Sari, Ismail; Sozeri, Betul; Cam, Ozlem; Birlik, Merih; Onen, Fatos; Akkoc, Nurullah; Akar, ServetÖğe Efficacy of Interleukin-1 Targeting Treatments in Patients with Familial Mediterranean Fever(Springer/Plenum Publishers, 2015) Cetin, Pinar; Sari, Ismail; Sozeri, Betul; Cam, Ozlem; Birlik, Merih; Akkoc, Nurullah; Onen, Fatos; Akar, ServetHerein, we reported our experience in colchicine-resistant familial Mediterranean fever (FMF) patients who are treated with anti-interleukin-1 (IL-1) drugs. A retrospective review of medical records of anti-IL-1 recipients was performed. The main clinical characteristics of these patients and the evolution after anti-IL-1 were recorded. There were 20 patients (11 male [M] and 9 female [F]). Despite regular colchicine treatment, median number of attacks per month and per year was 1 (1-4) and 12 (4-50), respectively. Twelve patients were receiving anakinra, and eight patients were treated with canakinumab. The number of monthly and yearly attacks after IL-1 treatment was significantly decreased after the biologic agent (p < 0.05). One patient did not respond to the treatment, and one patient developed serious infection during anti-IL-1. We also observed a significant decrease in proteinuria in the amyloidosis complicated FMF patients. Anti-IL-1 targeting drugs seem safe and effective therapies in colchicine-resistant FMF.Öğe Evaluation of the effects of miRNAs in familial Mediterranean fever(Springer London Ltd, 2019) Hortu, Hacer Orsdemir; Karaca, Emin; Sozeri, Betul; Gulez, Nesrin; Makay, Balahan; Gunduz, Cumhur; Atik, Tahir; Tekin, Ismihan Merve; Unsal, Sevket Erbil; Cogulu, OzgurFamilial Mediterranean fever (FMF) is an inherited autoinflammatory disorder that can result in attacks with accompanying recurrent episodes of fever, serositis, and skin rash. MiRNAs are demonstrated to be associated with a number of other diseases; however, no comprehensive study has revealed its association with FMF disease. The aim is to investigate the role of microRNAs in FMF. We included 51 patients with genetically diagnosed FMF who had clinical symptoms and 49 healthy volunteers. Fifteen miRNAs that were found to be associated with autoinflammatory diseases and have a part in immune response were evaluated. The expression levels of 11 miRNAs (miR-125a, miR-132, miR-146a, miR-155, miR-15a, miR-16, miR-181a, miR-21, miR-223, miR-26a, and miR-34a) in the patient group were significantly low, compared with the control group (p<0.05). The patient group was analyzed and compared within itself, and the expression levels of 5 miRNAs (miR-132, miR-15a, miR-181a, miR-23b, miR-26a) in the patients who took colchicine seemed to have increased and levels of 5 miRNAs (miR-146a, miR-15a, miR-16, miR-26a, miR-34a) in the patients who took colchicine were significantly lower (p<0.05). Furthermore, the attack patients were compared with the control group, and their expression levels of 4 miRNAs (miR-132, miR-15a, miR-21, miR-34a) were significantly lower (p<0.05). Levels of 9 miRNAs (miR-132, miR-146a, miR-15a, miR-16, miR-181a, miR-21, miR-223, miR-26a, miR-34a) in non-attack patients decreased significantly (p<0.05). Our study demonstrates that miRNAs could be effective in the pathogenesis of FMF.Öğe Evaluation of the effects of miRNAs in familial Mediterranean fever (vol 38, pg 635, 2018)(Springer London Ltd, 2019) Hortu, Hacer Orsdemir; Karaca, Emin; Sozeri, Betul; Gulez, Nesrin; Makay, Balahan; Gunduz, Cumhur; Atik, Tahir; Tekin, Ismihan Merve; Unsal, Sevket Erbil; Cogulu, Ozgur
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