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ABSTRACTObjectiveTo evaluate the safety of canakinumab using real-world data in patients with systemic juvenile idiopathic arthritis (sJIA) and autoinflammatory diseases (AID).Research design and methodsThis was a cross-sectional observational, multicenter study. Patients diagnosed with AID and sJIA treated with canakinumab were included in the study. The participating 13 centers retrospectively collected their patients’ data.ResultsA total of 335 patients were involved in the study. Among these patients, 280 were in the AID group and 55 were in the sJIA group. Canakinumab was administered at a median dose of 3 (2.5–4) mg/kg. The median total exposure time to canakinumab was 1.9 (0.8–3.2) years, corresponding to 759.5 patient-years. Seven hundred and seventy-nine total adverse events (AE) were identified. The total incidence of AE, and serious adverse events (SAE) throughout the study period was 1.02 per patient-years. The upper respiratory tract infection rate was 0.7 per patient-years, while the other infection rate was 0.13 per patient-years. While no death was observed in any patient, SAE were observed in 8 patients. Interstitial lung disease, anaphylaxis, or anaphylactoid reactions were not observed in any patient.ConclusionsReal-life data from a large cohort of patients suggests that canakinumab is as safe as claimed in clinical trials.

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ABSTRACTObjectivesThe aim of this study is to compare the demographic, clinical features, treatment results and outcomes in pediatric patients with idiopathic uveitis and uveitis due to juvenile idiopathic arthritis (JIA) and Behçet’s disease (BD).Methods97 pediatric uveitis patients were divided into three groups according to the etiology of uveitis: Group 1 comprised idiopathic uveitis patients, Group 2 uveitis patients who had JIA, and Group 3 uveitis patients with BD.ResultsSymptomatic presentation and intermediate uveitis were more common in Group 1 (p < 0.005). Asymptomatic presentation and anterior uveitis in Group 2 (p < 0.005), whereas symptomatic presentation and posterior uveitis in Group 3 (p < 0.005). Erythrocyte sedimentation rate (ESR) was higher in patients with BD or JIA uveitis than those with idiopathic uveitis (p < 0.005). Biologic therapy was more commonly used in JIA group compared to other groups (p < 0.005). Patients who had a complication related with uveitis were more common in females, asymptomatic disease course, and needed use of biologic treatment than in those without any complication (p < 0.005).ConclusionUveitis accompanying rheumatologic diseases may have asymptomatic and insidious course but have higher ESR as an important notice.

Background: Children with chronic kidney disease and on kidney replacement therapy may have neurocognitive and psychosocial disorders. Although kidney transplantation improves quality of life, psychological problems may exist in children who undergo kidney transplantation. Herein, we aimed to investigate attention-deficit hyperactivity disorder-like symptoms with MOXO-continuous performance test in children with pre-dialysis chronic kidney disease, dialysis and kidney transplantation. Methods: The MOXO-continuous performance test measures four domains of attention-deficit hyperactivity disorder-like symptoms, including attention, timeliness, hyperactivity and impulsivity. Patients with at least three scores &lt; − 1.5 standard deviations were considered as positive to MOXO-continuous performance test. Test scores of the pre-dialysis chronic kidney disease, dialysis (divided into peritoneal dialysis and hemodialysis subgroups) and kidney transplantation groups were compared. Correlations of test scores with the patient’s clinical and laboratory characteristics and effects of hospitalizations and schooling were assessed. Results: Seventy-two patients aged 13.3 ± 3.4 years (23 with kidney transplantation, 23 on dialysis and 26 with pre-dialysis chronic kidney disease) were evaluated. Overall MOXO-continuous performance test positivity was 29%. No differences were detected between the three groups concerning total or z scores. Attention and timeliness z scores were significantly higher in females (p= 0.004 and p= 0.008, respectively). Age was positively correlated to attention and timeliness total scores (p= 0.000, r= 0.445 and p= 0.004, r= 0.243, respectively), and inversely correlated to hyperactivity total scores (p= 0.000, r= − 0.415). Conclusions: Prevalence of attention-deficit hyperactivity disorder-like symptoms in the study population was much higher than that of pediatric attention-deficit hyperactivity disorder. We believe that the MOXO-continuous performance test is a valid supportive measure for evaluation of attention-deficit hyperactivity disorder diagnosis in children with various stages of chronic kidney disease or on kidney replacement therapy. Graphical abstract:

Background:Data on the immunization practices in pediatric chronic kidney disease (CKD) patients are scarce. The purpose of this study was to evaluate current vaccination practices for children on dialysis across European pediatric nephrology centers. Methods:A total of 18 tertiary pediatric nephrology centers from 12 European countries were included in the study. The data on universal national immunization programs and immunization practices for children with chronic disease or risk were recorded from European Center for Disease Prevention and Control and the World Health Organization. The immunization practices and center protocols for monitoring antibody titers after vaccination in dialysis patients were obtained through a questionnaire. Results:All centers included in the study recommended immunization against hepatitis B virus (HBV), diphtheria, tetanus, pertussis, Hemophilus influenzaetype b (Hib), poliomyelitis, measles, mumps, rubella (MMR), and streptococcus pneumonia in dialysis patients. In 16 centers, dialysis patients were vaccinated against influenza virus annually. HBV protective antibody titers were measured in 17 centers (during dialysis period in 14 centers, during pre-renal transplantation preparations in 14 centers or in both times in 11 centers). Hepatitis A virus (HAV) was reported to be followed in 13 centers, in 8 centers during dialysis period, and in 11 centers during pre-RTx preparations. MMR and varicella-zoster virus (VZV) protective antibody titers were measured during the dialysis period or before renal transplantation (RTx) in 12 and 15 centers, respectively, and in 6 centers both titers were checked both times. Conclusion:There are variations in vaccination practice across Europe. Children with CKD, those undergoing dialysis, and transplant candidates should receive age-appropriate vaccinations before RTx as well as before the transition to adult nephrology clinics and antibody levels should be monitored to evaluate the immunization status before and after RTx.