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Aim: Systemic lupus erythematosus (SLE) is the prototype of chronic autoimmune diseases. One-fifth of all SLE disease occurs in childhood. In this study, it was aimed to compare the SLE classification criteria that have been used so far. These are the ACR (American College of Rheumatology) 1997, SLICC (Systemic Lupus International Collaborating Clinics) 2012, and EULAR (European League against Rheumatism) / ACR 2019 criteria. Materials and Methods: Patients with 34 pediatric SLE and 32 antinuclear antibodies (ANA) (+) control groups who were followed up in Ankara Dr. Sami Ulus Obstetrics and Children's Hospital Pediatric Rheumatology Clinic were recruited to study. All of these patients were diagnosed by three pediatric rheumatologists. The control group consisted of ANA (+) patients with other rheumatologic diseases. Results: The sensitivities of the ACR 1997, SLICC 2012, and EULAR/ACR 2019 criteria were 76.5%, 94.1%, and 88.2%, respectively. The specificities of the criteria for ACR 1997, SLICC2012, and EULAR/ACR 2019 were 93.8%. Conclusion: Although SLICC 2012 showed the best sensitivity, all three classification criteria had the same specificity. [ABSTRACT FROM AUTHOR]

Objective: A subset of typical Hemolytic-uremic syndrome (HUS) patients requires initiation of renal replacement therapy (RRT) despite aggressive medical intervention. This study aims to evaluate the clinical and laboratory findings of patients diagnosed with typical HUS and efficacy and safety of peritoneal dialysis (PD) and hemodialysis (HD) were compared in managing these cases. Material and Methods: Patients having a diagnosis of typical HUS were retrospectively determined, and assessment the effects of RRT modalities on renal survival. Results: The present study included 49 children with typical HUS, and the mean age was 2.99 ± 1.88 years. Neurological involvement was detected in 15 (30.6%) patients. The neutrophil/lymphocyte ratio (NLR) was significantly higher in the neurological involvement group [odds ratio (OR) 15.42, 95% CI (3.39-70.1), p = 0.005]. In total, 43 (87.7%) patients received RRT and the median time from the first recorded symptom to RRT was six days (range 3-11). While throughout RRT, 10.2% of patients required mechanical ventilation. In this study, 26 (53.1%) patients were managed with PD, and 17 (34.6%) patients were managed with HD. During follow-up, 10.2% of all patients progressed to chronic kidney disease (CKD), proteinuria was detected in 6.1% and hypertension in 4.08%. The risk of CKD was significantly lower in the 3-month period in PD patients than in HD patients (odds ratio (OR) 7.69, 95% CI (0.77-76.07), p = 0.04). Conclusion: We concluded that PD might be as effective and safer as HD concerning kidney recovery. Clinical features and inflammatory markers can predict neurological involvement in typical HUS cases. [ABSTRACT FROM AUTHOR]

Objective: We aimed to investigate the efficacy of mycophenolate mofetil (MMF) for maintaining remission and reduce the number of relapses in childhood steroid-sensitive nephrotic syndrome. The effects of MMF on growth and blood pressure parameters were also evaluated. Material and Methods: This retrospective, single-center observational study included patients with steroid-sensitive nephrotic syndrome who were treated using MMF between 2009 and 2019 in the Department of Pediatric Nephrology in our hospital. Results: Ten patients had steroid-dependent nephrotic syndrome; six patients frequently had relapsing nephrotic syndrome in this study. The mean duration of the disease was 93.3±25.0 months and the mean duration of the MMF onset was 33.9±16.7 months after diagnosis. Ten patients showed a 50% or greater reduction in the relapse rate and the prednisolone treatment was discontinued in eight patients for six months or more. Compared to the previous year, before the start of the MMF treatment, there was a 52.7% reduction in the relapse rate and a 36.6% reduction in the cumulative annual dose of steroid after 12 months of MMF treatment. The height z score and the median office systolic blood pressure standard deviation scores of the patients improved after MMF treatment (respectively p = 0.003, p = 0.01). Conclusion: The findings suggest that MMF may lead to decreased relapse rates and cumulative steroid dose, which has a positive effect on growth and blood pressure parameters in steroid-sensitive nephrotic syndrome. [ABSTRACT FROM AUTHOR]

Since previous research suggests a role of a circulating factor in the pathogenesis of steroid-sensitive nephrotic syndrome (NS), we speculated that circulating plasma extracellular vesicles (EVs) are a candidate source of such a soluble mediator. Here, we aimed to characterize and try to delineate the effects of these EVs in vitro. Plasma EVs from 20 children with steroid-sensitive NS in relapse and remission, 10 healthy controls, and 6 disease controls were obtained by serial ultracentrifugation. Characterization of these EVs was performed by electron microscopy, flow cytometry, and Western blot analysis. Major proteins from plasma EVs were identified via mass spectrometry. Gene Ontology classification analysis and Ingenuity Pathway Analysis were performed on selectively expressed EV proteins during relapse. Immortalized human podocyte culture was used to detect the effects of EVs on podocytes. The protein content and particle number of plasma EVs were significantly increased during NS relapse. Relapse NS EVs selectively expressed proteins that involved actin cytoskeleton rearrangement. Among these, the level of RAC-GTP was significantly increased in relapse EVs compared with remission and disease control EVs. Relapse EVs were efficiently internalized by podocytes and induced significantly enhanced motility and albumin permeability. Moreover, relapse EVs induced significantly higher levels of RAC-GTP and phospho-p38 and decreased the levels of synaptopodin in podocytes. Circulating relapse EVs are biologically active molecules that carry active RAC1 as cargo and induce recapitulation of the NS phenotype in podocytes in vitro. [ABSTRACT FROM AUTHOR]