Seza Ozen - Academia.edu (original) (raw)

Papers by Seza Ozen

Research paper thumbnail of A new tool supporting the diagnosis of childhood-onset Behçet’s disease: venous wall thickness

Rheumatology

Objectives The lower extremity venous wall thickness (VWT) of Behçet’s Disease (BD) patients was ... more Objectives The lower extremity venous wall thickness (VWT) of Behçet’s Disease (BD) patients was reported to be significantly increased in adults, suggesting its use for the support of BD diagnosis. This prospective study aimed to investigate the lower extremity VWT in childhood-onset definite and incomplete BD patients and compare it to healthy age-matched controls. Methods Pediatric patients classified with BD according to the 2015 international pediatric BD criteria in our center were included in the study. Intima-media thickness of the lower extremity veins to evaluate VWT was measured by ultrasonography, including common femoral vein (CFV), femoral vein (FV), vena saphena magna (VSM), vena saphena parva (VSP), and popliteal vein (PV). Results In this cross-sectional study, VWT was measured in 35 patients (63% male) and 27 healthy controls (55% male). Thirteen (37%) of 35 patients met the criteria for the diagnosis of BD. The remaining 22 (63%) had incomplete BD and met two crit...

Research paper thumbnail of Update in familial Mediterranean fever

Current Opinion in Rheumatology, 2021

Purpose of review Familial Mediterranean fever (FMF) is the prototypic autoinflammatory disease. ... more Purpose of review Familial Mediterranean fever (FMF) is the prototypic autoinflammatory disease. Although the gene associated with the disease was identified 24 years ago, we still have to learn about the pathogenesis of its inflammation and the variation in the phenotype. In this review, we discuss some recent findings in FMF, such as changes in our understanding of the genetics, aims to define new criteria, and factors contributing to the disease presentation. Recent findings We finally have learned why a mutation causing this disease was selected in ancient times; MEFV gene mutations confer resistance to the microbe of plague. A group of experts have outlined recommendations for the analysis of the genetics of FMF. These recommendations complement the new classification criteria, which includes genetic results. In the past year, a number of studies have addressed the contributing factors to the inflammation caused by the mutations in pyrin; this has included epigenetic studies as well. Finally, we have long-term data for the use of anti-IL1 treatment in colchicine-resistant patients. Summary We now have recommendations for assessing genetic analysis of the MEFV gene and how to reliably classify a patient as FMF. We await further data to understand the contributing genetic and environmental factors that affect the inflammation and final phenotype in FMF and the extent of the disease presentation.

Research paper thumbnail of Defining colchicine resistance/intolerance in patients with familial Mediterranean fever: a modified-Delphi consensus approach

Rheumatology, 2021

Objectives Colchicine is the main treatment for FMF. Although a number of individuals with FMF ar... more Objectives Colchicine is the main treatment for FMF. Although a number of individuals with FMF are intolerant/resistant to colchicine, there is no standard definition of colchicine resistance/intolerance. We developed a set of evidence-based core statements defining colchicine resistance/intolerance in patients with FMF that may serve as a guide for clinicians and health authorities. Methods A set of statements was identified using a modified-Delphi consensus-based approach. The process involved development of an initial colchicine resistance/intolerance-related questionnaire derived from a systematic literature review. The questionnaire, which was completed by an international panel of 11 adult and paediatric rheumatologists with expertise in FMF, was analysed anonymously. The results informed draft consensus statements that were discussed by a round-table expert panel, using a nominal group technique to agree on the selection and wording of the final statements. Results Consensus ...

Research paper thumbnail of Clusters in Pediatric Rheumatic Diseases

Current Rheumatology Reports, 2020

Purpose of the Review Rheumatic diseases usually have very diverse and complex pathophysiology re... more Purpose of the Review Rheumatic diseases usually have very diverse and complex pathophysiology resulting in different clinical phenotypes. Some certain phenotypes cluster together with a common presentation, course, and outcome. Our primary aim is to review the known disease clusters in certain rheumatological conditions. Recent Findings Cluster analysis has been reported in several rheumatic diseases, Behcet disease being the most common. Five individual clusters together with some clinical associations have been reported in Behcet patients. Certain antibody-phenotype associations and damage clusters have been reported in systemic lupus erythematosus (SLE) patients as well. Although there is no proper cluster analysis, two distinct phenotypes are evident in deficiency of ADA2 (DADA2): those with a polyarteritis nodosa-like vasculopathy and hematological involvement with pure red cell aplasia or bone marrow failure. The variants are claimed to be different regions of this gene for these 2 phenotypes. Lastly, there is a recent paper grouping ANCA-associated vasculitis according to disease phenotype and antibody associations. Summary There are distinct clusters in certain rheumatic diseases which might help us to predict comorbidities, disease course, and treatment response. Keywords Clusters. SLE. Behcet. Adenosine deaminase 2 deficiency. ANCA-associated vasculitis This article is part of the Topical Collection on Pediatric Rheumatology

Research paper thumbnail of SAT0493 THE Challenge of Treati̇ng Pulmonary Vasculitis in Behçet’s Disease: Two Pediatric Cases

Saturday, 15 June 2019, 2019

Lipoprotein lipase is a key enzyme in lipid metabolism, ensuring the hydrolysis of plasma triglyc... more Lipoprotein lipase is a key enzyme in lipid metabolism, ensuring the hydrolysis of plasma triglycerides found in chylomicrons and very low density lipoproteins. An increase in the catalytic activity of lipoprotein lipase leads to a decrease in the level of TG and an increase in the level of HDL in the blood. According to the results of the survey in children with JIA, the concentration of lipoprotein lipase was 13.9 [7.1; 25.3] mg/l, with obesity 27.5 [9.9; 47.8] mg/l. A direct relationship was established between its content in blood serum and the concentration of LDL (rs=0.37; p<0.05) and the inverse with the level of LDL (rs =-0.4; p<0.05). There was a significant difference (p<0.05) by gender: the level of lipoprotein lipase in the blood serum in girls was 25.8 [16.7-54.4] mg/l, in boys-15.6 [7.8-29,0] mg/l. A correlation was also established between the level of lipoprotein lipase and body mass index (rs = 0.35; p<0.05). According to the results of the study, a significant (p<0.05) increase in the level of primary (DK233) and secondary (DK278 and MDA) LPO products in the blood serum of children with JIA was found in comparison with the control group. During the correlation analysis, a positive correlation was established between the levels of DK233, DK278 in serum and ESR (rs = 0.587, p<0.01). The results obtained indicate the intensification of lipid peroxidation processes. A significant (p<0.05-0.01) decrease in the content of water-soluble (ACW) and fat-soluble (ACL) antioxidant capacity of substances in the blood serum of children with JIA was found when compared with similar indicators in the control group. During the correlation analysis, a negative correlation was established between the level of CRP and the content of ACL in serum (rs =-0.346, p<0.05), between the content of ACW and CRP in serum (rs =-0.54, p<0, 01). Conclusion: It can be concluded that the activation of lipid peroxidation and the failure of the antioxidant system play a significant role in the development and progression of JIA in the examined children, as well as in the formation of atherogenic disorders by the lipid peroxidation mechanism. In addition, children with JIA have abnormalities in the lipid spectrum, acquiring an atherogenic orientation, which, together with hemostasiological changes, can be regarded as a risk factor for atherothrombosis.

Research paper thumbnail of Predictors of Effectiveness of Anakinra in Systemic Juvenile Idiopathic Arthritis

The Journal of Rheumatology, 2019

Objective.To seek predictors of therapeutic response to the interleukin (IL)-1 inhibitor anakinra... more Objective.To seek predictors of therapeutic response to the interleukin (IL)-1 inhibitor anakinra in children with systemic-onset juvenile idiopathic arthritis (sJIA).Methods.The clinical charts of all patients with sJIA who were newly treated with anakinra at our center between 2004 and 2017 were reviewed retrospectively. Predictors included baseline demographic, clinical, and laboratory variables as well as previous or concomitant therapies. The effectiveness of anakinra was assessed at 1 year after treatment start. Complete clinical response (CCR) was defined as absence of fever, physician’s global assessment ≤ 1, count of active joints ≤ 1, negative C-reactive protein, and ≥ 75% reduction of corticosteroid dose. According to the intention-to-treat principle, patients who had anakinra discontinued before 1 year for any reasons other than disease remission were classified as nonresponders. Statistics included univariate and multivariable analyses.Results.Of the 62 patients include...

Research paper thumbnail of The changing face of polyarteritis nodosa and necrotizing vasculitis

Nature Reviews Rheumatology, 2017

Research paper thumbnail of Tumour necrosis factor α G→A −238 and G→A −308 polymorphisms in juvenile idiopathic arthritis

Rheumatology, 2002

Objectives. To study G £A 2238 and G £A 2308 polymorphisms in the promoter region of the tumour n... more Objectives. To study G £A 2238 and G £A 2308 polymorphisms in the promoter region of the tumour necrosis factor (TNF) a gene in patients with juvenile idiopathic arthritis (JIA). We analysed whether there were any associations between these polymorphisms and the type of JIA anduor the clinical course of the disease in two populations. Methods. The first group consisted of 51 Turkish JIA patients and the second consisted of 159 JIA patients from the Czech Republic. Healthy individuals (93 and 100) from each country served as controls. Subgroups of JIA were defined according to the Durban criteria. The course of the disease was defined on the basis of the physician's global evaluation of disease activity, the swollen and tender joint count and the erythrocyte sedimentation rate. Results. In both JIA cohorts, the distribution of genotypes was not significantly different among the types of JIA. The G £A 2238 polymorphism did not have an effect on the patients' outcome in either group. The G £A 2308 polymorphism was significantly associated with a poor outcome in the Turkish group (P = 0.005) but there was no association in the Czech patients. Some features of JIA in Turkish patients differed from those in Czech patients. Conclusions. Genetic differences may accompany the phenotypic differences found in the Turkish group. Although larger numbers of patients are clearly needed to verify this, we suggest that the G £A 2308 polymorphism may be operative in defining disease outcome in selected groups.

Research paper thumbnail of Gastrointestinal system manifestations in juvenile systemic lupus erythematosus

Clinical Rheumatology, 2017

Systemic lupus erythematosus (SLE) is an autoimmune disease which may involve gastrointestinal sy... more Systemic lupus erythematosus (SLE) is an autoimmune disease which may involve gastrointestinal system (GIS). The aim of this study was to present GIS manifestations of pediatric SLE patients. The medical files of 69 children with SLE followed between January 2011 and January 2016 were reviewed. All fulfilled the Systemic Lupus International Collaborating Clinics criteria. All patients (≤18 years of age) with GIS manifestations were included. GIS manifestations were observed in 19 (27.5%) out of 69 SLE patients and present at the time of SLE diagnosis in 13 (68.4%). The GIS manifestations due to SLE were autoimmune hepatitis (AIH) (n = 8) and lupus enteritis (n = 1). Manifestations associated with SLE were hepatomegaly and hypertransaminasemia due to macrophage activation syndrome (MAS) (n = 3) and hepatic steatosis (n = 1). GIS manifestations as a result of the adverse events of drugs were as follows: toxic hepatitis (n = 3; associated with methotrexate and nonsteroidal antiinflammatory drugs in one, methotrexate in another, and azathioprine in another patient), azathioprine-induced cholestatic hepatitis (n = 1), and gastritis associated with corticosteroid (n = 1). In one patient, acute appendicitis occurred as a coincidence. In this study, one of every five pediatric SLE patients had GIS-related manifestations. GIS involvement may occur as an initial manifestation of the disease.

Research paper thumbnail of Predictors of response in patients with active systemic JIA (SJIA) receiving canakinumab: an exploratory analysis of pooled 12-week data

Pediatric Rheumatology, 2014

Research paper thumbnail of THU0473-HPR Living with childhood vasculitis; a qualitative study

Annals of the Rheumatic Diseases, 2013

Background Childhood systemic vasculitis are a group of rare diseases with multi-organ involvemen... more Background Childhood systemic vasculitis are a group of rare diseases with multi-organ involvement and potentially devastating consequences. The biomedical perspective does not take into account a patient’s own psychological perspective. Patients’ subjective experiences may represent the key domains of illness that differ from clinicians’ views. Objectives The aim of the study is to develop an multidimensional assessment instrument named “Juvenile Vasculitis Multidimensional Assessment Report” (JVAMAR) to measure all these domains. In this study it will be presented the data of “Qualitative Interviews”, one of the steps of item generation in JVAMAR. Methods Twelve children with vasculitis and their mother were enrolled to this study. Data were collected using both a demographic data form and a semi-structured interview form. Study was made on individual patient interview by face-to-face manner. Results Data analysis by grounded theory revealed four categories. These categories were (1) physical impact of disease, (2) emotional impact of disease, (3) social impact of disease and (4) complaints about treatment protocols. In the physical impact category, severe pain, hypersensitivity to cold, restriction in movement, weakness, fatigue, frequently upper respiratory tract infections, anorexia and hypertension were the prominent features. As emotional impact, the most common features were thought of death, pessimism, hopelessness, weak adaptation of social environment relevant to patients age, increased mother dependency, anxiety about treatment and future life, dissatisfaction about body image according to medical therapy and emotional hypersensitivity. In the social impact category, the patients reported that difficulty to access to health services, economic problems, decrease in academic performance, absenteeism to school, increased religious behavior and thoughts, conceal the sickness from friends. In the complaints about treatment protocols category, many patients reported improved health status after treatment but fear about having a chronic disease although the drug use regularly, they complained from life time drug use and frequency of daily drug doses. Conclusions Children with vasculitis imply that they experience several difficulties regarding physical, emotional and social aspects and treatment protocols. There is a need to develop a multidimensional instrument to measure important domains of the illness such as quality of life, the burden of disease in vasculitis, defined as the impact of permanent damage on the patient and its assessment. References Demirkaya E, Luqmani R, Ayaz N.A, Karaoglu A, Ozen S, the FMF Arthritis Vasculitis and Orphan Disease Research in Paediatric Rheumatology (FAVOR). Time to focus on outcome assessment tools for childhood vasculitis. PROJ 2011;9:29 Herlyn K, Hellmich B, Seo P, Merkel P, Patient-Reported Outcome Assessment in Vasculitis May Provide Important Data and a Unique Perspective, AC & R 2010;62:11:1639–45 Disclosure of Interest None Declared

Research paper thumbnail of EMC education at Sakarya University

2003 IEEE International Symposium on Electromagnetic Compatibility, 2003. EMC '03., 2003

ABSTRACT Sakarya University has implemented an EMC education program in co-operation with the Uni... more ABSTRACT Sakarya University has implemented an EMC education program in co-operation with the University of Applied Sciences Zurich since 1998. The EMC education program has been splitted into two parts. Firstly, EMC education during the engineering studies for students of the electrical-electronics and computer science departments. Secondly, EMC education for master and PhD students as advanced EMC education. The first part includes electromagnetic field theory, electromagnetic wave theory, antennas and propagation, some basic experiments in co-operation with the industry and the national research center (TUBITAK), which have an EMC laboratory including measurement possibilities. The second part includes advanced electromagnetic theory, numerical and symbolic computation techniques and thesis in various research areas, such as simulations, modeling, scale modeling. This paper deals with the specific program of the Sakarya University and gives some detailed examples.

Research paper thumbnail of A case of familial Mediterranean fever, Behçet's disease and polyarteritis nodosa complicated by perirenal haematoma

Clinical and experimental rheumatology

... A case of familial Mediterranean fever, Behçet&#x27;s disease and polyarteritis nodosa co... more ... A case of familial Mediterranean fever, Behçet&#x27;s disease and polyarteritis nodosa complicated by perirenal haematoma. Korkmaz C, Zubaroglu I, Kaya T, Akçar N, Gürbüz E, Ozen S. PMID: 11760409 [PubMed - indexed for MEDLINE]. Publication Types: Case Reports; Letter. ...

Research paper thumbnail of Ozaltin F et. al DGKE Supplemental Data

Research paper thumbnail of Vesicoureteral reflux in childhood

Saudi journal of kidney diseases and transplantation : an official publication of the Saudi Center for Organ Transplantation, Saudi Arabia

Research paper thumbnail of Purpura fulminans as the presenting manifestation in a patient with juvenile SLE

The Turkish journal of pediatrics

We present a 12-year-old girl with systemic lupus erythematosus and associated antiphospholipid s... more We present a 12-year-old girl with systemic lupus erythematosus and associated antiphospholipid syndrome who developed an unusual manifestation of purpura fulminans in an accelerated fashion. The patient improved after prompt treatment with anticoagulants, aggressive immunosuppressive drugs and plasmapheresis. This is the first pediatric case of purpura fulminans due to secondary antiphospholipid syndrome of systemic lupus erythematosus. We suggest that SLE patients with lupus anticoagulant should be followed closely for similar complications.

Research paper thumbnail of Takayasu arteritis in a 4-year-old girl: case report and brief overview of the pediatric literature

The Turkish journal of pediatrics

Takayasu arteritis (TA) is a large vessel vasculitis that involves the aorta, its major branches ... more Takayasu arteritis (TA) is a large vessel vasculitis that involves the aorta, its major branches and pulmonary arteries. Diagnosis of TA during childhood remains challenging due to the non-specific symptoms. We report a four-year-old girl presenting with fever, fatigue, weight loss, and elbow pain who was later diagnosed as childhood TA. On admission, she had fever, hypertension, decreased pulses, bruits, hepatosplenomegaly, and increased erythrocyte sedimentation rate and C-reactive protein level. Computed tomography angiography showed luminal narrowing and wall thickening in ascending aorta, brachiocephalic, left common carotid and left vertebral arteries and descending aorta. Oral corticosteroid (prednisone, 2 mg/kg/day) was instituted, later followed by oral methotrexate (12.5 mg/m2/week). TA is rare in children; however, childhood TA must be considered in children who present with non-specific systemic symptoms, hypertension and increased acute phase reactants.

Research paper thumbnail of Systemic juvenile idiopathic arthritis is associated with HLA-DRB1 in Europeans and Americans of European descent

Pediatric Rheumatology, 2012

Systemic juvenile idiopathic arthritis (sJIA) is a complex inflammatory disease whose etiology re... more Systemic juvenile idiopathic arthritis (sJIA) is a complex inflammatory disease whose etiology remains unknown. sJIA is distinguished from other forms of juvenile idiopathic arthritis (JIA) by its characteristic features including requisite quotidian fever and salmon-colored, evanescent skin rash, but also by an absence of autoantibodies. Based on its unique phenotype among JIA subtypes, it has been suggested that sJIA may be autoinflammatory rather than autoimmune in nature, and consistent with this, sJIA is distinct among JIA subtypes for its inconsistently detectable association with HLA genes. In this study, we sought to use SNP genotyping in a large patient collection to identify sJIA susceptibility loci. Methods We genotyped 576 children fulfilling ILAR criteria for systemic arthritis and 366 control subjects free of sJIA or autoimmune disease. The collection included 205 cases and 210 controls from Cincinnati Children's Hospital, 185 cases from the repository at University of Manchester, 56 cases and 60 controls from University of Genova, 54 cases from Hacettepe University, 42 cases from the

Research paper thumbnail of Familial Mediterranean fever: a misnomer?

The Lancet, 1994

when rumours of organ traffic may give transplantation a bad name. We feel, however, that these i... more when rumours of organ traffic may give transplantation a bad name. We feel, however, that these information campaigns should be re-targeted so that we do not preach to those already convinced, as a recent audit of our centre seems to indicate. We found that immigrant families refuse donation significantly more often than autochthonous residents (9/15 or 60% vs 5/31 or 16%; p < 0-005, Fisher's exact test). As it is likely that this kind of behaviour is not limited to our area,2 greater efforts to alter attitudes towards dontion among deprived members of society are clearly needed.

Research paper thumbnail of Pediatric Vasculitis

Current Rheumatology Reports, 2012

The primary systemic vasculitides in childhood are quite rare except for Henoch-Schönlein purpura... more The primary systemic vasculitides in childhood are quite rare except for Henoch-Schönlein purpura and Kawasaki disease. These are usually self-limited diseases. The etiopathogenesis for most of them is not clearly understood. However, it is usually thought that environmental triggers (mostly infectious) evoke an aggravated inflammatory response in susceptible individuals. The classification criteria for most of them were recently validated for children. Clinical manifestations are determined by the organs affected, reflecting the involved vessels on a background of constitutional symptoms. For treatment, the main data are adapted from adult studies, especially for rarer vasculitides in childhood. In this review, the recent classification criteria, current pathophysiologic pathways, and treatment protocols are summarized.

Research paper thumbnail of A new tool supporting the diagnosis of childhood-onset Behçet’s disease: venous wall thickness

Rheumatology

Objectives The lower extremity venous wall thickness (VWT) of Behçet’s Disease (BD) patients was ... more Objectives The lower extremity venous wall thickness (VWT) of Behçet’s Disease (BD) patients was reported to be significantly increased in adults, suggesting its use for the support of BD diagnosis. This prospective study aimed to investigate the lower extremity VWT in childhood-onset definite and incomplete BD patients and compare it to healthy age-matched controls. Methods Pediatric patients classified with BD according to the 2015 international pediatric BD criteria in our center were included in the study. Intima-media thickness of the lower extremity veins to evaluate VWT was measured by ultrasonography, including common femoral vein (CFV), femoral vein (FV), vena saphena magna (VSM), vena saphena parva (VSP), and popliteal vein (PV). Results In this cross-sectional study, VWT was measured in 35 patients (63% male) and 27 healthy controls (55% male). Thirteen (37%) of 35 patients met the criteria for the diagnosis of BD. The remaining 22 (63%) had incomplete BD and met two crit...

Research paper thumbnail of Update in familial Mediterranean fever

Current Opinion in Rheumatology, 2021

Purpose of review Familial Mediterranean fever (FMF) is the prototypic autoinflammatory disease. ... more Purpose of review Familial Mediterranean fever (FMF) is the prototypic autoinflammatory disease. Although the gene associated with the disease was identified 24 years ago, we still have to learn about the pathogenesis of its inflammation and the variation in the phenotype. In this review, we discuss some recent findings in FMF, such as changes in our understanding of the genetics, aims to define new criteria, and factors contributing to the disease presentation. Recent findings We finally have learned why a mutation causing this disease was selected in ancient times; MEFV gene mutations confer resistance to the microbe of plague. A group of experts have outlined recommendations for the analysis of the genetics of FMF. These recommendations complement the new classification criteria, which includes genetic results. In the past year, a number of studies have addressed the contributing factors to the inflammation caused by the mutations in pyrin; this has included epigenetic studies as well. Finally, we have long-term data for the use of anti-IL1 treatment in colchicine-resistant patients. Summary We now have recommendations for assessing genetic analysis of the MEFV gene and how to reliably classify a patient as FMF. We await further data to understand the contributing genetic and environmental factors that affect the inflammation and final phenotype in FMF and the extent of the disease presentation.

Research paper thumbnail of Defining colchicine resistance/intolerance in patients with familial Mediterranean fever: a modified-Delphi consensus approach

Rheumatology, 2021

Objectives Colchicine is the main treatment for FMF. Although a number of individuals with FMF ar... more Objectives Colchicine is the main treatment for FMF. Although a number of individuals with FMF are intolerant/resistant to colchicine, there is no standard definition of colchicine resistance/intolerance. We developed a set of evidence-based core statements defining colchicine resistance/intolerance in patients with FMF that may serve as a guide for clinicians and health authorities. Methods A set of statements was identified using a modified-Delphi consensus-based approach. The process involved development of an initial colchicine resistance/intolerance-related questionnaire derived from a systematic literature review. The questionnaire, which was completed by an international panel of 11 adult and paediatric rheumatologists with expertise in FMF, was analysed anonymously. The results informed draft consensus statements that were discussed by a round-table expert panel, using a nominal group technique to agree on the selection and wording of the final statements. Results Consensus ...

Research paper thumbnail of Clusters in Pediatric Rheumatic Diseases

Current Rheumatology Reports, 2020

Purpose of the Review Rheumatic diseases usually have very diverse and complex pathophysiology re... more Purpose of the Review Rheumatic diseases usually have very diverse and complex pathophysiology resulting in different clinical phenotypes. Some certain phenotypes cluster together with a common presentation, course, and outcome. Our primary aim is to review the known disease clusters in certain rheumatological conditions. Recent Findings Cluster analysis has been reported in several rheumatic diseases, Behcet disease being the most common. Five individual clusters together with some clinical associations have been reported in Behcet patients. Certain antibody-phenotype associations and damage clusters have been reported in systemic lupus erythematosus (SLE) patients as well. Although there is no proper cluster analysis, two distinct phenotypes are evident in deficiency of ADA2 (DADA2): those with a polyarteritis nodosa-like vasculopathy and hematological involvement with pure red cell aplasia or bone marrow failure. The variants are claimed to be different regions of this gene for these 2 phenotypes. Lastly, there is a recent paper grouping ANCA-associated vasculitis according to disease phenotype and antibody associations. Summary There are distinct clusters in certain rheumatic diseases which might help us to predict comorbidities, disease course, and treatment response. Keywords Clusters. SLE. Behcet. Adenosine deaminase 2 deficiency. ANCA-associated vasculitis This article is part of the Topical Collection on Pediatric Rheumatology

Research paper thumbnail of SAT0493 THE Challenge of Treati̇ng Pulmonary Vasculitis in Behçet’s Disease: Two Pediatric Cases

Saturday, 15 June 2019, 2019

Lipoprotein lipase is a key enzyme in lipid metabolism, ensuring the hydrolysis of plasma triglyc... more Lipoprotein lipase is a key enzyme in lipid metabolism, ensuring the hydrolysis of plasma triglycerides found in chylomicrons and very low density lipoproteins. An increase in the catalytic activity of lipoprotein lipase leads to a decrease in the level of TG and an increase in the level of HDL in the blood. According to the results of the survey in children with JIA, the concentration of lipoprotein lipase was 13.9 [7.1; 25.3] mg/l, with obesity 27.5 [9.9; 47.8] mg/l. A direct relationship was established between its content in blood serum and the concentration of LDL (rs=0.37; p<0.05) and the inverse with the level of LDL (rs =-0.4; p<0.05). There was a significant difference (p<0.05) by gender: the level of lipoprotein lipase in the blood serum in girls was 25.8 [16.7-54.4] mg/l, in boys-15.6 [7.8-29,0] mg/l. A correlation was also established between the level of lipoprotein lipase and body mass index (rs = 0.35; p<0.05). According to the results of the study, a significant (p<0.05) increase in the level of primary (DK233) and secondary (DK278 and MDA) LPO products in the blood serum of children with JIA was found in comparison with the control group. During the correlation analysis, a positive correlation was established between the levels of DK233, DK278 in serum and ESR (rs = 0.587, p<0.01). The results obtained indicate the intensification of lipid peroxidation processes. A significant (p<0.05-0.01) decrease in the content of water-soluble (ACW) and fat-soluble (ACL) antioxidant capacity of substances in the blood serum of children with JIA was found when compared with similar indicators in the control group. During the correlation analysis, a negative correlation was established between the level of CRP and the content of ACL in serum (rs =-0.346, p<0.05), between the content of ACW and CRP in serum (rs =-0.54, p<0, 01). Conclusion: It can be concluded that the activation of lipid peroxidation and the failure of the antioxidant system play a significant role in the development and progression of JIA in the examined children, as well as in the formation of atherogenic disorders by the lipid peroxidation mechanism. In addition, children with JIA have abnormalities in the lipid spectrum, acquiring an atherogenic orientation, which, together with hemostasiological changes, can be regarded as a risk factor for atherothrombosis.

Research paper thumbnail of Predictors of Effectiveness of Anakinra in Systemic Juvenile Idiopathic Arthritis

The Journal of Rheumatology, 2019

Objective.To seek predictors of therapeutic response to the interleukin (IL)-1 inhibitor anakinra... more Objective.To seek predictors of therapeutic response to the interleukin (IL)-1 inhibitor anakinra in children with systemic-onset juvenile idiopathic arthritis (sJIA).Methods.The clinical charts of all patients with sJIA who were newly treated with anakinra at our center between 2004 and 2017 were reviewed retrospectively. Predictors included baseline demographic, clinical, and laboratory variables as well as previous or concomitant therapies. The effectiveness of anakinra was assessed at 1 year after treatment start. Complete clinical response (CCR) was defined as absence of fever, physician’s global assessment ≤ 1, count of active joints ≤ 1, negative C-reactive protein, and ≥ 75% reduction of corticosteroid dose. According to the intention-to-treat principle, patients who had anakinra discontinued before 1 year for any reasons other than disease remission were classified as nonresponders. Statistics included univariate and multivariable analyses.Results.Of the 62 patients include...

Research paper thumbnail of The changing face of polyarteritis nodosa and necrotizing vasculitis

Nature Reviews Rheumatology, 2017

Research paper thumbnail of Tumour necrosis factor α G→A −238 and G→A −308 polymorphisms in juvenile idiopathic arthritis

Rheumatology, 2002

Objectives. To study G £A 2238 and G £A 2308 polymorphisms in the promoter region of the tumour n... more Objectives. To study G £A 2238 and G £A 2308 polymorphisms in the promoter region of the tumour necrosis factor (TNF) a gene in patients with juvenile idiopathic arthritis (JIA). We analysed whether there were any associations between these polymorphisms and the type of JIA anduor the clinical course of the disease in two populations. Methods. The first group consisted of 51 Turkish JIA patients and the second consisted of 159 JIA patients from the Czech Republic. Healthy individuals (93 and 100) from each country served as controls. Subgroups of JIA were defined according to the Durban criteria. The course of the disease was defined on the basis of the physician's global evaluation of disease activity, the swollen and tender joint count and the erythrocyte sedimentation rate. Results. In both JIA cohorts, the distribution of genotypes was not significantly different among the types of JIA. The G £A 2238 polymorphism did not have an effect on the patients' outcome in either group. The G £A 2308 polymorphism was significantly associated with a poor outcome in the Turkish group (P = 0.005) but there was no association in the Czech patients. Some features of JIA in Turkish patients differed from those in Czech patients. Conclusions. Genetic differences may accompany the phenotypic differences found in the Turkish group. Although larger numbers of patients are clearly needed to verify this, we suggest that the G £A 2308 polymorphism may be operative in defining disease outcome in selected groups.

Research paper thumbnail of Gastrointestinal system manifestations in juvenile systemic lupus erythematosus

Clinical Rheumatology, 2017

Systemic lupus erythematosus (SLE) is an autoimmune disease which may involve gastrointestinal sy... more Systemic lupus erythematosus (SLE) is an autoimmune disease which may involve gastrointestinal system (GIS). The aim of this study was to present GIS manifestations of pediatric SLE patients. The medical files of 69 children with SLE followed between January 2011 and January 2016 were reviewed. All fulfilled the Systemic Lupus International Collaborating Clinics criteria. All patients (≤18 years of age) with GIS manifestations were included. GIS manifestations were observed in 19 (27.5%) out of 69 SLE patients and present at the time of SLE diagnosis in 13 (68.4%). The GIS manifestations due to SLE were autoimmune hepatitis (AIH) (n = 8) and lupus enteritis (n = 1). Manifestations associated with SLE were hepatomegaly and hypertransaminasemia due to macrophage activation syndrome (MAS) (n = 3) and hepatic steatosis (n = 1). GIS manifestations as a result of the adverse events of drugs were as follows: toxic hepatitis (n = 3; associated with methotrexate and nonsteroidal antiinflammatory drugs in one, methotrexate in another, and azathioprine in another patient), azathioprine-induced cholestatic hepatitis (n = 1), and gastritis associated with corticosteroid (n = 1). In one patient, acute appendicitis occurred as a coincidence. In this study, one of every five pediatric SLE patients had GIS-related manifestations. GIS involvement may occur as an initial manifestation of the disease.

Research paper thumbnail of Predictors of response in patients with active systemic JIA (SJIA) receiving canakinumab: an exploratory analysis of pooled 12-week data

Pediatric Rheumatology, 2014

Research paper thumbnail of THU0473-HPR Living with childhood vasculitis; a qualitative study

Annals of the Rheumatic Diseases, 2013

Background Childhood systemic vasculitis are a group of rare diseases with multi-organ involvemen... more Background Childhood systemic vasculitis are a group of rare diseases with multi-organ involvement and potentially devastating consequences. The biomedical perspective does not take into account a patient’s own psychological perspective. Patients’ subjective experiences may represent the key domains of illness that differ from clinicians’ views. Objectives The aim of the study is to develop an multidimensional assessment instrument named “Juvenile Vasculitis Multidimensional Assessment Report” (JVAMAR) to measure all these domains. In this study it will be presented the data of “Qualitative Interviews”, one of the steps of item generation in JVAMAR. Methods Twelve children with vasculitis and their mother were enrolled to this study. Data were collected using both a demographic data form and a semi-structured interview form. Study was made on individual patient interview by face-to-face manner. Results Data analysis by grounded theory revealed four categories. These categories were (1) physical impact of disease, (2) emotional impact of disease, (3) social impact of disease and (4) complaints about treatment protocols. In the physical impact category, severe pain, hypersensitivity to cold, restriction in movement, weakness, fatigue, frequently upper respiratory tract infections, anorexia and hypertension were the prominent features. As emotional impact, the most common features were thought of death, pessimism, hopelessness, weak adaptation of social environment relevant to patients age, increased mother dependency, anxiety about treatment and future life, dissatisfaction about body image according to medical therapy and emotional hypersensitivity. In the social impact category, the patients reported that difficulty to access to health services, economic problems, decrease in academic performance, absenteeism to school, increased religious behavior and thoughts, conceal the sickness from friends. In the complaints about treatment protocols category, many patients reported improved health status after treatment but fear about having a chronic disease although the drug use regularly, they complained from life time drug use and frequency of daily drug doses. Conclusions Children with vasculitis imply that they experience several difficulties regarding physical, emotional and social aspects and treatment protocols. There is a need to develop a multidimensional instrument to measure important domains of the illness such as quality of life, the burden of disease in vasculitis, defined as the impact of permanent damage on the patient and its assessment. References Demirkaya E, Luqmani R, Ayaz N.A, Karaoglu A, Ozen S, the FMF Arthritis Vasculitis and Orphan Disease Research in Paediatric Rheumatology (FAVOR). Time to focus on outcome assessment tools for childhood vasculitis. PROJ 2011;9:29 Herlyn K, Hellmich B, Seo P, Merkel P, Patient-Reported Outcome Assessment in Vasculitis May Provide Important Data and a Unique Perspective, AC & R 2010;62:11:1639–45 Disclosure of Interest None Declared

Research paper thumbnail of EMC education at Sakarya University

2003 IEEE International Symposium on Electromagnetic Compatibility, 2003. EMC '03., 2003

ABSTRACT Sakarya University has implemented an EMC education program in co-operation with the Uni... more ABSTRACT Sakarya University has implemented an EMC education program in co-operation with the University of Applied Sciences Zurich since 1998. The EMC education program has been splitted into two parts. Firstly, EMC education during the engineering studies for students of the electrical-electronics and computer science departments. Secondly, EMC education for master and PhD students as advanced EMC education. The first part includes electromagnetic field theory, electromagnetic wave theory, antennas and propagation, some basic experiments in co-operation with the industry and the national research center (TUBITAK), which have an EMC laboratory including measurement possibilities. The second part includes advanced electromagnetic theory, numerical and symbolic computation techniques and thesis in various research areas, such as simulations, modeling, scale modeling. This paper deals with the specific program of the Sakarya University and gives some detailed examples.

Research paper thumbnail of A case of familial Mediterranean fever, Behçet's disease and polyarteritis nodosa complicated by perirenal haematoma

Clinical and experimental rheumatology

... A case of familial Mediterranean fever, Behçet&#x27;s disease and polyarteritis nodosa co... more ... A case of familial Mediterranean fever, Behçet&#x27;s disease and polyarteritis nodosa complicated by perirenal haematoma. Korkmaz C, Zubaroglu I, Kaya T, Akçar N, Gürbüz E, Ozen S. PMID: 11760409 [PubMed - indexed for MEDLINE]. Publication Types: Case Reports; Letter. ...

Research paper thumbnail of Ozaltin F et. al DGKE Supplemental Data

Research paper thumbnail of Vesicoureteral reflux in childhood

Saudi journal of kidney diseases and transplantation : an official publication of the Saudi Center for Organ Transplantation, Saudi Arabia

Research paper thumbnail of Purpura fulminans as the presenting manifestation in a patient with juvenile SLE

The Turkish journal of pediatrics

We present a 12-year-old girl with systemic lupus erythematosus and associated antiphospholipid s... more We present a 12-year-old girl with systemic lupus erythematosus and associated antiphospholipid syndrome who developed an unusual manifestation of purpura fulminans in an accelerated fashion. The patient improved after prompt treatment with anticoagulants, aggressive immunosuppressive drugs and plasmapheresis. This is the first pediatric case of purpura fulminans due to secondary antiphospholipid syndrome of systemic lupus erythematosus. We suggest that SLE patients with lupus anticoagulant should be followed closely for similar complications.

Research paper thumbnail of Takayasu arteritis in a 4-year-old girl: case report and brief overview of the pediatric literature

The Turkish journal of pediatrics

Takayasu arteritis (TA) is a large vessel vasculitis that involves the aorta, its major branches ... more Takayasu arteritis (TA) is a large vessel vasculitis that involves the aorta, its major branches and pulmonary arteries. Diagnosis of TA during childhood remains challenging due to the non-specific symptoms. We report a four-year-old girl presenting with fever, fatigue, weight loss, and elbow pain who was later diagnosed as childhood TA. On admission, she had fever, hypertension, decreased pulses, bruits, hepatosplenomegaly, and increased erythrocyte sedimentation rate and C-reactive protein level. Computed tomography angiography showed luminal narrowing and wall thickening in ascending aorta, brachiocephalic, left common carotid and left vertebral arteries and descending aorta. Oral corticosteroid (prednisone, 2 mg/kg/day) was instituted, later followed by oral methotrexate (12.5 mg/m2/week). TA is rare in children; however, childhood TA must be considered in children who present with non-specific systemic symptoms, hypertension and increased acute phase reactants.

Research paper thumbnail of Systemic juvenile idiopathic arthritis is associated with HLA-DRB1 in Europeans and Americans of European descent

Pediatric Rheumatology, 2012

Systemic juvenile idiopathic arthritis (sJIA) is a complex inflammatory disease whose etiology re... more Systemic juvenile idiopathic arthritis (sJIA) is a complex inflammatory disease whose etiology remains unknown. sJIA is distinguished from other forms of juvenile idiopathic arthritis (JIA) by its characteristic features including requisite quotidian fever and salmon-colored, evanescent skin rash, but also by an absence of autoantibodies. Based on its unique phenotype among JIA subtypes, it has been suggested that sJIA may be autoinflammatory rather than autoimmune in nature, and consistent with this, sJIA is distinct among JIA subtypes for its inconsistently detectable association with HLA genes. In this study, we sought to use SNP genotyping in a large patient collection to identify sJIA susceptibility loci. Methods We genotyped 576 children fulfilling ILAR criteria for systemic arthritis and 366 control subjects free of sJIA or autoimmune disease. The collection included 205 cases and 210 controls from Cincinnati Children's Hospital, 185 cases from the repository at University of Manchester, 56 cases and 60 controls from University of Genova, 54 cases from Hacettepe University, 42 cases from the

Research paper thumbnail of Familial Mediterranean fever: a misnomer?

The Lancet, 1994

when rumours of organ traffic may give transplantation a bad name. We feel, however, that these i... more when rumours of organ traffic may give transplantation a bad name. We feel, however, that these information campaigns should be re-targeted so that we do not preach to those already convinced, as a recent audit of our centre seems to indicate. We found that immigrant families refuse donation significantly more often than autochthonous residents (9/15 or 60% vs 5/31 or 16%; p < 0-005, Fisher's exact test). As it is likely that this kind of behaviour is not limited to our area,2 greater efforts to alter attitudes towards dontion among deprived members of society are clearly needed.

Research paper thumbnail of Pediatric Vasculitis

Current Rheumatology Reports, 2012

The primary systemic vasculitides in childhood are quite rare except for Henoch-Schönlein purpura... more The primary systemic vasculitides in childhood are quite rare except for Henoch-Schönlein purpura and Kawasaki disease. These are usually self-limited diseases. The etiopathogenesis for most of them is not clearly understood. However, it is usually thought that environmental triggers (mostly infectious) evoke an aggravated inflammatory response in susceptible individuals. The classification criteria for most of them were recently validated for children. Clinical manifestations are determined by the organs affected, reflecting the involved vessels on a background of constitutional symptoms. For treatment, the main data are adapted from adult studies, especially for rarer vasculitides in childhood. In this review, the recent classification criteria, current pathophysiologic pathways, and treatment protocols are summarized.