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Background: Myelomeningocele (MMC) is highly prevalent in developing countries, and MMC-related neurogenic bladder is an important cause of childhood chronic kidney disease (CKD). This nationwide study aimed to evaluate demographic and clinical features of pediatric patients with MMC in Turkey and risk factors associated with CKD stage 5. Methods: Data from children aged 0–19 years old, living with MMC in 2022, were retrospectively collected from 27 pediatric nephrology centers. Patients > 1 year of age without pre-existing kidney abnormalities were divided into five groups according to eGFR; CKD stages 1–5. Patients on dialysis, kidney transplant recipients, and those with eGFR < 15 ml/min/1.73 m2 but not on kidney replacement therapy at time of study constituted the CKD stage 5 group. Results: A total of 911 (57.8% female) patients were enrolled, most of whom were expectantly managed. Stages 1–4 CKD were found in 34.3%, 4.2%, 4.1%, and 2.4%, respectively. CKD stage 5 was observed in 5.3% of patients at median 13 years old (range 2–18 years). Current age, age at first abnormal DMSA scan, moderate-to-severe trabeculated bladder on US and/or VCUG, and VUR history were independent risk factors for development of CKD stage 5 (OR 0.752; 95%; CI 0.658–0.859; p < 0.001; OR 1.187; 95% CI 1.031–1.367; p = 0.017; OR 10.031; 95% CI 2.210–45.544; p = 0.003; OR 2.722; 95% CI 1.215–6.102; p = 0.015, respectively). Only eight CKD stage 5 patients underwent surgery related to a hostile bladder between 1 and 15 years old. Conclusion: MMC-related CKD is common in childhood in Turkey. A proactive approach to neurogenic bladder management and early protective surgery in selected cases where conservative treatment has failed should be implemented to prevent progressive kidney failure in the pediatric MMC population in our country. [ABSTRACT FROM AUTHOR]

We evaluated the reasons for requesting anti-nuclear antibody (ANA) analysis in clinical practice at a tertiary center and the performance of ANA in pediatric autoimmune diseases. Patients under 18 years of age who underwent ANA testing for various symptoms between 2013 and 2017 were included. We retrieved data from medical records, including demographic and clinical characteristics, diagnoses, ANA results, titers, and staining patterns. The performance assessment tools were calculated according to the ANA titer for autoimmune diseases. Risk factors for autoimmune diseases in ANA-positive patients were evaluated using logistic regression analysis. Changes in ANA titer and seroconversion were evaluated using repeated ANA analyses. A total of 3812 patients underwent ANA. Medical records of 3320 patients were obtained. The rate of ANA positivity was 27.4%. ANA was requested most frequently because of musculoskeletal findings in 1355 patients (40.8%). Juvenile idiopathic arthritis (n = 174, 20.2%) was the most common diagnosis in ANA-positive patients, followed by systemic lupus erythematosus (n = 52, 6%). For autoimmune diseases, a titer of ≥ 1:100, a sensitivity of 40.1%, and a specificity of 77.1% were observed. At a titer ≥ 1:1000, the sensitivity and specificity were 24.1% and 89%, respectively. Homogeneous staining was an additional risk factor for autoimmune diseases in ANA-positive patients by multivariate logistic regression analysis (OR [95% CI]: 4.562 [3.076–6.766], p < 0.001). Conclusion: Our results revealed that the performance of the ANA test in diagnosing autoimmune diseases in pediatric clinical practice was poor. Therefore, clinical findings should be carefully evaluated before ANA testing is performed. What is Known: • ANA can be detected in systemic autoimmune rheumatic diseases. • The diagnostic role of ANA is controversial, especially in childhood. What is New: • One in four patients who requested the ANA test had an autoimmune disease. • Less than half of patients with an autoimmune disease had ANA positivity. [ABSTRACT FROM AUTHOR]

Background: Having a low nephron number is a well-known risk factor for hypertension. There is an inverse relationship between the filtration surface area and systemic hypertension. A significant percentage of masked hypertension can be detected in children with nephron loss by ambulatory blood pressure monitoring (ABPM). Methods: We prospectively investigated ABPM results of children having reduced kidney mass with normal office blood pressures (BPs) and kidney function. Forty-three children with congenital solitary kidney (group 1), 11 children with acquired solitary kidney (group 2), and 76 children with hypofunctioning kidney (group 3) were compared with age, gender, and BMI-matched healthy control group (group 4). The dietary salt intake of 76 patients was evaluated as salt equivalent (g/day). The primary endpoint was change from baseline in mean 24-h ABPM variables and the proportion of patients with masked hypertension when assessed by ABPM. Results: The masked hypertension ratio of all patients was 12.3% when assessed with ABPM. Night hypertension was significantly higher in all patient groups than in the control group (p = 0.01). Diastolic BP loads of groups 1 and 3 were higher than in controls (p = 0.024). Systolic BP loads were higher only in group 1 than in the control group (p = 0.003). The dietary salt equivalent of patients in group 1 correlated positively with 24-h SBP and mean arterial pressure (MAP) values. Patients with excessive dietary salt intake in group 1 had a significantly higher diastolic BP load than those without excessive salt intake in group 1 (p = 0.002). Conclusions: Masked hypertension can be seen in children with a solitary kidney or when one of the kidneys is hypofunctioning. Systolic BP loads are higher in children with congenital solitary kidney, and salt intake correlates with systolic BP profiles especially in those. Our results suggest that being born with a congenital solitary kidney increases predisposition to hypertension and salt sensitivity. [ABSTRACT FROM AUTHOR]

Background: C3 glomerulopathy (C3G) is characterized by heterogeneous clinical presentation, outcome, and predominant C3 accumulation in glomeruli without significant IgG. There is scarce outcome data regarding childhood C3G. We describe clinical and pathological features, treatment and outcomes, and risk factors for progression to chronic kidney disease stage 5 (CKD5) in the largest pediatric series with biopsy-proven C3G. Methods: Sixty pediatric patients with C3G from 21 referral centers in Turkey were included in this retrospective study. Patients were categorized according to CKD stage at last visit as CKD5 or non-CKD5. Demographic data, clinicopathologic findings, treatment, and outcome data were compared and possible risk factors for CKD5 progression determined using Cox proportional hazards model. Results: Mean age at diagnosis was 10.6 ± 3.0 years and follow-up time 48.3 ± 36.3 months. Almost half the patients had gross hematuria and hypertension at diagnosis. Nephritic-nephrotic syndrome was the commonest presenting feature (41.6%) and 1/5 of patients presented with nephrotic syndrome. Membranoproliferative glomerulonephritis was the leading injury pattern, while 40 patients had only C3 staining. Patients with DDD had significantly lower baseline serum albumin compared with C3GN. Eighteen patients received eculizumab. Clinical remission was achieved in 68.3%. At last follow-up, 10 patients (16.6%) developed CKD5: they had lower baseline eGFR and albumin and higher frequency of nephrotic syndrome and dialysis requirement than non-CKD5 patients. Lower serum albumin and eGFR at diagnosis were independent predictors for CKD5 development. Conclusions: Children with C3G who have impaired kidney function and hypoalbuminemia at diagnosis should be carefully monitored for risk of progression to CKD5. Graphical abstract [ABSTRACT FROM AUTHOR]