Familial Mediterranean Fever (FMF) is an autosomal recessive genetic disorder, primarily observed in populations around the Mediterranean Sea, linked to MEFV gene mutations. These mutations disrupt inflammatory responses, increasing pyrin-protein production. Traditional diagnosis relies on clinical symptoms, family history, acute phase reactants, and excluding similar syndromes with MEFV testing, which is expensive and often inconclusive due to heterozygous mutations.
View Article and Find Full Text PDFObjectives: The aim of this study is to investigate the effect of anti-interleukin (IL)-1/-6 biologics on systemic juvenile idiopathic arthritis (sJIA)-associated macrophage activation syndrome (MAS).
Methods: Demographic, clinical and laboratory data of patients followed up with a diagnosis of sJIA-associated MAS assessed from sixteen paediatric rheumatology centres across the country. The clinical and laboratory features of MAS developing while on biological drugs were compared with those without this treatment.
Background: Juvenile idiopathic arthritis (JIA) is a prevalent childhood chronic arthritis, often persisting into adulthood. Effective transitional care becomes crucial as these patients transition from pediatric to adult healthcare systems. Despite the concept of transitional care being recognized, its real-world implementation remains inadequately explored.
View Article and Find Full Text PDFObjectives: The aim of this study was to examine the clinical and phenotypic features of pediatric Behçet's disease (PEDBD) in our clinic and present the rates of fulfilling the diagnostic criteria.
Patients And Methods: Thirty-four patients (20 males, 14 females; mean age: 16.0±2.
Background: The pathogenesis and clinical manifestations of the multisystem inflammatory syndrome in children (MIS-C) has not yet been fully elucidated and there is no clear consensus on its treatment yet.
Objectives: To evaluate our patients diagnosed with MIS-C and present them to the literature in order to contribute to the better understanding of this new disease, which entered paediatric practice with the SARS-CoV-2 peak.
Methods: In this study, 17 MIS-C cases diagnosed according to the Centers for Disease Control and Prevention criteria were included.
Objectives: This study aims to evaluate demographic, clinical, and radiological characteristics of Turkish children with chronic non-bacterial osteomyelitis.
Patients And Methods: Between January 2008 and December 2018, a total of 28 patients (10 males, 18 females; median age: 12.5 years; range, 4.
Background: Juvenile idiopathic arthritis (JIA), is the most common pediatric rheumatologic disorder with unknown etiology. Currently, no population-based data are available regarding the distribution of categories and frequency of uveitis in patients with JIA in Turkey. The purpose of this study was to evaluate the frequency of JIA-associated uveitis (JIAU) and distribution of JIA categories in a Turkish JIA cohort.
View Article and Find Full Text PDFObjectives: This study aimed to evaluate the risk for atherosclerosis by using echocardiographic arterial stiffness (AS) parameters and serum endocan levels, as a biomarker of endothelial dysfunction (ED) in children with FMF.
Methods: Seventy-nine children with FMF (12-18 years) and 41 healthy children were included, and clinical features (age at the first attack, age at the time of diagnosis, diagnosis delay time, colchicine dose, biological agent usage, MEFV mutations, and symptoms of attacks) of patients were noted. Arterial stiffness parameters were calculated by using echocardiographic aortic measurements with blood pressure monitoring.
Purpose: The aim was to compare the effects of yoga and home exercise program on lower extremity functional status, pain, and quality of life in children with enthesitis related arthritis (ERA).
Methods: Twenty-one children with ERA were divided into two groups as yoga (n = 11) and home exercise (n = 10). Yoga group performed supervised yoga exercises twice a week for eight weeks.
Int J Pediatr Otorhinolaryngol
December 2020
Objective: To evaluate middle and inner ear function and hearing status of children with familial Mediterranean fever (FMF).
Methods: We assigned 56 patients with FMF to the study group and 52 healthy volunteers to the control group. The mean age of patients in the study and control groups were 10.
Background: Colchicine is an anti-inflammatory agent used for preventing familial Mediterranean fever (FMF) attacks and amyloidosis. A significant number of patients are non-responsive or intolerant to the domestic drug colchicum dispert.
Aims: To compare the efficacy and side effects of colchicum dispert and colchicine opocalcium in children with FMF.
Objectives: This paper aimed to present real-world data of treatment results of a single center in patients with systemic Juvenile Idiopathic arthritis (SJIA), in which methotrexate (MTX) along with glucocorticoids was preferred as the first-line treatment option.
Methods: The medical records of 50 patients (58 episodes) with SJIA were evaluated. All patients with SJIA were hospitalized and were given high dose glucocorticoid treatment along with subcutaneous MTX.
Kawasaki disease (KD) is the most common cause of childhood coronary artery disease. The incidence of coronary artery lesions (CALs) has declined with the routine use of intravenous immunoglobulin (IVIG) treatment, but there is still considerable risk for resistance to IVIG treatment and development of CALs. The present study was aimed to determine the risk factors in Turkish children with IVIG resistant KD and coronary artery involvement.
View Article and Find Full Text PDFAçarı C, Ünsal E, Hakgüder G, Soylu A, Özer E. Pediatric mesenteric panniculitis: three cases and a review of the literature. Turk J Pediatr 2019; 61: 798-803.
View Article and Find Full Text PDFObjectives: To report our experiences of the juvenile Behçet's disease (BD) patients with cerebral venous sinus thrombosis (CVST) and to review previous studies reporting the clinical characteristics and outcomes of juvenile BD with CVST.
Methods: Clinical characteristics and outcomes of paediatric patients with CVST who met the Paediatric Behçet's Disease (PEDBD) classification criteria for juvenile BD from 3 referral centres in Turkey were reviewed retrospectively. A systematic review of literature of all published data was conducted.
Objective: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are very rare in childhood with an increased risk of morbidity and mortality. We aimed to evaluate renal prognostic factors in childhood AAV from the perspective of ANCA serotype, histopathological classification, and five-factor score (FFS).
Methods: Pediatric AAV patients from 11 referral centers in Turkey had been included to the study.
Açarı C, Ünsal E. Current information about vaccination practice in pediatric rheumatic diseases and recommendations for future applications. Turk J Pediatr 2017; 59: 357-368.
View Article and Find Full Text PDF