Paediatric Behçet’s Disease: Data From A Single Center Experience In Turkey

ABSTRACT Objectives: Behçet’s disease (BD) is a multisystemic inflammatory disease with unknown etiology. It is characterized by recurrent oral and genital ulcerations, uveitis, and skin lesions, various musculoskeletal, gastrointestinal, central nervous system, and vascular manifestations. The aim of this study was to analyse the demographic characteristics and clinical features, treatment in Turkish paediatric BD from a single center experience.   Methods: The records 36 patients with BD who were diagnosed according to the International Study Group criteria between January 2017 and January 2019 in the department of paediatric rheumatology, were retrospectively reviewed. Data on demographic, clinical features and therapy were collected. Results: A total of 36 (19 male) patients were included in this study. Mean age at disease onset was 9.36 ± 4.45 years and mean age at diagnosis 13.99 ± 2.83 years. The frequencies of signs/symptoms were: recurrent oral aphtosis 100%, genital ulcers 80.6%, musculoskeletal 30.6%, ocular 16.7%, neurological 11.1% and vascular involvement 11.1%, gastrointestinal 2.8%. Colchicine and corticosteroids were the main treatments. Conclusions: In this single-center retrospective study, we analyzed the data of paediatric BD and their treatment from a single center in Turkey. The presented small series and the literature review suggest that paediatric BD is a heterogeneous disease with varied clinical manifestations

The feasibility of withdrawing canakinumab in paediatric colchicine-resistant familial Mediterranean fever patients.

Objective. To determine the feasibility of withdrawing canakinumab (CAN) in a large cohort of paediatric patients with colchicine-resistant familial Mediterranean fever (crFMF)

Toward the integration of biosimilars into pediatric rheumatology: adalimumab ABP 501 experience of PeRA research group.

Optimization of hydrolysis conditions of lignocellulosic biomass is crucial to able to produce value-added products by fermentation. This study not only determines optimal dilute sulfuric acid (H2SO4) hydrolysis conditions of wheat bran (WB) and rye bran (RB) by using one-factor-at-a-time method and subsequently Box-Behnken design but also elucidates chemical composition of hydrolysates yielded under optimal hydrolysis conditions. Based on the results, optimal hydrolysis conditions of WB and RB were 121 and 130 degrees C of temperature, 1/8 and 1/8 w/v of solid to liquid ratio, 2.66 and 1.58% v/v of dilute H2SO4 ratio, and 30 and 16 min of implementation time, respectively. Hydrolysates obtained from WB and RB at these conditions contained 72.7 (0.58 g sugar/g biomass) and 89.4 g/L (0.72 g sugar/g biomass) of reducing sugar concentration, respectively. Hydrolysis rates of WB and RB were 87.79 and 91.33%, respectively. Main reducing sugar in RB hydrolysate was glucose with 31.17 g/L (0.25 g glucose/g biomass) while glucose and xylose were the main monosaccharides with 20.90 (0.17 g glucose/g biomass) and 18.69 g/L (0.15 g xylose/g biomass) in WB hydrolysate, respectively. With acidic hydrolysis of WB and RB, inhibitors such as phenolics, 5-Hydroxymethylfurfural, 2-Furaldehyde (not for RB), acetic acid, and formic acid (not for WB) formed. Catalytic efficiency values of H2SO4 for WB and RB were 15.2 and 24.4 g /g, respectively, indicating that inhibitor concentration in WB hydrolysate was higher than that of RB. These results indicated that WB and RB have a high potential in production of value-added products by fermentation

Remission rates and risk factors for relapse in pediatric morphea: a multicenter retrospective study of Pediatric Rheumatology Academy (PeRA)-Research Group (RG)

Aim: Morphea, also known as localized scleroderma, is an immune-mediated disease and the most common form of scleroderma in children. It is a localized sclerosing disease of the skin, but can also involve such adjacent tissues as the fascia, muscle, bone, and underlying tissues. This multicenter study aimed to evaluate Turkish pediatric morphea patients, regarding demographics, treatments, and response to treatment. Materials and methods: The study was performed by the Pediatric Rheumatology Academy and included pediatric morphea patients from 6 Turkish pediatric rheumatology centers who were followed up for ≥6 months. Demographic, clinical, and laboratory findings and treatment modalities were analyzed. The patients were divided into 3 groups according to treatment response, as follows: group 1: topical treatment response, group 2: methotrexate response, and group 3: methotrexate resistance. Clinical findings were compared between the 3 groups. Results: The study included 76 patients, of which 53 (69.7%) were female. Mean age at diagnosis of morphea was 9.7 ± 4.3 years and mean duration of follow-up was 3.2 ± 2.9 years. Linear morphea was the most common form, accounting for 43.4% (n = 33) of the patients. Extracutaneous features were noted in 17 patients (22.4%) and anti-nuclear antibody positivity was noted in 32 (42.1%). In all, 14.4% of the patients received topical treatment only, whereas 86.6% received both topical and systemic treatment. The methotrexate response rate was 76.9% in the patients that received systemic immunosuppressive therapy. The overall relapse rate while under treatment was 19.7%. Conclusion: In this study, most of the pediatric morphea patients responded well to methotrexate. Bilateral lesions were more common in the methotrexate-resistant group. Multiple involvement, and bilateral lesions, were more common in relapsed patients than in non-relapsed patients. Key points • Most of the pediatric morphea patients respond well to MTX. • Multiple involvement, and bilateral involvement, were more common in relapsed patients than in non-relapsed patients. • Presence of extracutaneous findings in patients increased relapse rate 5.7 times

Clinical characteristics and predictors for recurrence in chronic nonbacterial osteomyelitis: a retrospective multicenter analysis

Background/aim: Chronic nonbacterial osteomyelitis (CNO) is a rare disease of unknown etiology and most commonly occurs during childhood or adolescence. The purpose of this study is to collect data on the clinical features, outcomes, and management of the disease and to identify the factors affecting recurrence. Materials and methods: This is a retrospective multicenter cross-sectional study of pediatric patients diagnosed with CNO. A total of 87 patients with a diagnosis of CNO followed for at least 6 months in 8 pediatric rheumatology centers across the country between January 2010 and December 2021 were included in this study. Results: The study included 87 patients (38 girls, 49 boys; median age: 12.5 years). The median follow-up time was 20 months (IQR: 8.5–40). The median time of diagnostic delay was 9.9 months (IQR: 3–24). Arthralgia and bone pain were the most common presenting symptoms. Multifocal involvement was detected in 86.2% of the cases and a recurrent course was reported in one-third of those included in the study. The most commonly involved bones were the femur and tibia. Vertebrae and clavicles were affected in 19.5% and 20.6% of cases, respectively. The erythrocyte sedimentation rate (ESR) values of 60.9% of the patients were above 20 mm/h and the C-reactive protein values of 44.8% were above 5 mg/L. The remission rate was 13.3% in patients using nonsteroidal antiinflammatory drugs and 75.0% in those using biological drugs. Vertebral and mandibular involvement and high ESR values at the time of diagnosis were associated with recurrence. Conclusion: In this multicenter study, CNO with vertebral and mandibular involvement and high ESR at diagnosis were associated with recurrence