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BACKGROUND: To investigate the risk factors for breakthrough urinary tract infection (BT-UTI) in children with vesicoureteral reflux (VUR) receiving continuous antibiotic prophylaxis (CAP). METHODS: This was a single-centre cohort study (January 2016 to December 2019). The clinical data of 256 children with grade I–V VUR receiving CAP were analysed. In this study, exposure variables were sex, younger age at the initial diagnosis of UTI ≤12 months, high-grade VUR, bilateral VUR, aetiology, presence of renal scarring at the initial diagnosis, presence of renal function impairment at the initial diagnosis, ultrasound abnormalities, antibiotic used and bladder and bowel dysfunction (BBD). Outcome was BT-UTI. RESULTS: BT-UTI occurred in 81 out of 256 children with grade I–V VUR who received CAP, an incidence of 31.64%. Univariate analysis showed that younger age at the initial diagnosis of UTI (≤12 months), bilateral VUR, renal scarring on the dimercaptosuccinic acid (DMSA) scan at the initial diagnosis of UTI and BBD were correlated with the occurrence of BT-UTI. Multivariate analysis showed that younger age at the initial diagnosis of UTI (≤12 months) [hazard ratio (HR): 4.629; 95% confidence interval (CI): 1.302–16.462], bilateral VUR (HR: 2.078; 95% CI: 1.084–4.022) and BBD (HR: 3.194; 95% CI: 1.243–8.206) were independent risk factors for the occurrence of BT-UTI. CONCLUSIONS: For VUR children receiving CAP, younger age at the initial diagnosis of UTI (≤12 months), bilateral VUR, and BBD were independent risk factors for the occurrence of BT-UTI.

BACKGROUND: Membranoproliferative glomerulonephritis (MPGN) is a rare histopathologic pattern of glomerular injury with limited studies in pediatric patients. Characteristics and outcomes of children with MPGN have also remained to be further explored. METHODS: We retrospectively reviewed the clinicopathological features, genetic findings, treatments and outcomes in 17 pediatric patients pathologically diagnosed with MPGN from 2007 to 2020 in the Children’s National Medical Center in China. RESULTS: Median age at disease onset was 9.9 years (IQR, 5.6–11.9 years). Most of the patients (12/17) had nephrotic range of proteinuria, and nephritic-nephrotic syndrome was the most common clinical presentation (35.2%). Secondary causes were identified in eight patients including hepatitis B virus (HBV) infection (n=4), methylmalonic acidemia (MMA, n=2), rheumatoid arthritis (RA, n=1) and Aymé-Gripp Syndrome (n=1). The nine patients with primary MPGN were further identified as immune-complex mediated MPGN (n=8), and unclassifiable MPGN (U-MGPN, n=1). Genetic analyses identified pathogenic variants of MMACHC gene in two cases of MMA and established the diagnosis for Aymé-Gripp syndrome in one case with a de novo variant of MAF gene. Comparing study between the complete or partial remission group (n=8) and non-response group (n=9) showed a significant difference in the timing of renal biopsy (P

Congenital anomalies of the kidney and urinary tracts (CAKUT) are the leading cause of kidney failure in children with phenotypic and genotypic heterogeneity. Our objective was to describe the genetic spectrum and identify the risk factors for kidney failure in children with CAKUT.Clinical and genetic data were derived from a multicenter network (Chinese Children Genetic Kidney Disease Database, CCGKDD) and the Chigene database. A total of 925 children with CAKUT who underwent genetic testing from 2014 to 2020 across China were studied. Data for a total of 584 children wereobtained from the CCGKDD, including longitudinal data regarding kidney function. The risk factors for kidney failure were determined by the Kaplan-Meier method and Cox proportional hazards models.A genetic diagnosis was established in 96 out of 925 (10.3%) children, including 72 (8%) with monogenic variants, 20 (2%) with copy number variants (CNVs), and 4 (0.4%)with major chromosomal anomalies. Patients with skeletal abnormalities were more likely to have large CNVs or abnormal karyotypes than monogenic variants. Eighty-two patients from the CCGKDD progressed to kidney failure at a median age of 13.0 (95% confidence interval, 12.4-13.6) years, and twenty-four were genetically diagnosed with variants of PAX2, TNXB, EYA1, HNF1B and GATA3 or the 48, XXYY karyotype. The multivariate analysis indicated that solitary kidney, posterior urethral valves, bilateral hypodysplasia, the presence of certain variants and premature birth were independent prognostic factors.The genetic spectrum of CAKUT varies among different subphenotypes. The identified factors indicate areas that require special attention.