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Background: The Flemish Collaborative Glomerulonephritis Group (FCGG) registry is a population-based kidney biopsy registry that has been including all native kidney biopsies performed in children in Flanders (Belgium), since 2017. Methods: From 2017 to 2020, 148 pediatric (< 18 years) native kidney biopsies were included. Each biopsy received a histopathological and final nephrological diagnosis, and concordance between both was assessed. Disease chronicity, summarized by the Mayo Clinic Chronicity Score, was determined on 122 biopsies with > 5 glomeruli. Results: Kidney biopsy rate was high (29.0 biopsies per million children per year), median age was 10.0 years (IQR 5.8–14.7), and boys predominated (56.1% males). A total of 140 biopsies (94.6%) showed a representative pathology result. Glomerular disease was most prevalent, with IgA nephropathy/IgA vasculitis (43 biopsies, 29.1%) and minimal change disease (MCD) (29 biopsies, 19.6%) being the overall most frequent diagnoses. In general, diagnostic concordance was high (80.7%). In Alport syndrome and focal segmental glomerulosclerosis (FSGS), concordance was lower, as the nephrological diagnosis was often determined by results of genetic analysis. Nephrotic syndrome was the most frequent indication for kidney biopsy (31.8%) and was mainly caused by MCD and FSGS. The degree of disease chronicity on kidney biopsies was generally low, although 27.3% of biopsies with a diagnosis of FSGS showed moderate-to-severe chronic damage. Conclusions: The presented epidemiological findings validate data from previous European registry studies and may inspire kidney biopsy registries worldwide to implement novel features such as clinicopathological concordance and chronicity grading. [ABSTRACT FROM AUTHOR]

Background: Mutations in complement factor H ( CFH), factor I ( CFI), factor B ( CFB), thrombomodulin ( THBD), C3 and membrane cofactor protein ( MCP), and autoantibodies against factor H (αFH) with or without a homozygous deletion in CFH-related protein 1 and 3 ( ∆CFHR1/3) predispose development of atypical hemolytic uremic syndrome (aHUS). Methods: Different mutations in genes encoding complement proteins in 45 pediatric aHUS patients were retrospectively linked with clinical features, treatment, and outcome. Results: In 47% of the study participants, potentially pathogenic genetic anomalies were found (5x CFH, 4x MCP, and 4x C3, 3x CFI, 2x CFB, 6xαFH, of which five had ∆CFHR1/3); four patients carried combined genetic defects or a mutation, together with αFH. In the majority (87%), disease onset was preceeded by a triggering event; in 25% of cases diarrhea was the presenting symptom. More than 50% had normal serum C3 levels at presentation. Relapses were seen in half of the patients, and there was renal graft failure in all except one case following transplant. Conclusions: Performing adequate DNA analysis is essential for treatment and positive outcome in children with aHUS. The impact of intensive initial therapy and renal replacement therapy, as well as the high risk of recurrence of aHUS in renal transplant, warrants further understanding of the pathogenesis, which will lead to better treatment options. [ABSTRACT FROM AUTHOR]

Background: In Belgium and the Netherlands, up to 40% of the children on dialysis are children with immigrant parents of non-Western European origin (non-Western). Concerns exist regarding whether these non-Western patients receive the same quality of care as children with parents of Western European origin (Western). We compared initial dialysis, post-initial treatment, and outcomes between non-Western and Western patients on dialysis. Methods: All children <19 years old on chronic dialysis in the Netherlands and Belgium between September 2007 and May 2011 were included in the study. Non-Western patients were defined as children of whom one or both parents were born in non-Western countries. Results: Seventy-nine of the 179 included patients (44%) were non-Western children. Compared to Western patients, non-Western patients more often were treated with hemodialysis (HD) instead of peritoneal dialysis (PD) as first dialysis mode (52 vs. 37%, p = 0.046). Before renal transplantation, non-Western patients were on dialysis for a median (range) of 30 (5-99) months, vs. 15 (0-66) months in Western patients ( p = 0.007). Renal osteodystrophy was diagnosed in 34% of non-Western vs. 18% of Western patients ( p = 0.028). The incidence rate ratio [95% confidence interval] for acute peritonitis was 2.44 [1.43-4.17] ( p = 0.032) for non-Western compared to Western patients. Conclusions: There are important disparities between children on chronic dialysis with parents from Western European origin and those from non-Western European origin in the choice of modality, duration, and outcomes of dialysis therapy. [ABSTRACT FROM AUTHOR]

The article describes a collaborative quality improvement approach in caring for children with end-stage renal disease (ESRD) in the Netherlands and Belgium. It focuses on the development of the RICH-Q Project which aims to improve the quality of care (QoC) for children with ESRD. It provides an overview of the first results of the project in the absence of population-specific evidence.

Imbalanced colonic microbial metabolism plays a pivotal role in generating protein-bound uraemic toxins (PBUTs), which accumulate with deteriorating kidney function and contribute to the uraemic burden of children with chronic kidney disease (CKD). Dietary choices impact the gut microbiome and metabolism. The aim of this study was to investigate the relation between dietary fibre and gut-derived PBUTs in paediatric CKD. Sixty-one (44 male) CKD children (9 ± 5 years) were prospectively followed for two years. Dietary fibre intake was evaluated by either 24-h recalls (73%) or 3-day food records (27%) at the same time of blood sampling for assessment of total and free serum levels of different PBUTs using liquid chromatography. We used linear mixed models to assess associations between fibre intake and PBUT levels. We found an inverse association between increase in fibre consumption (g/day) and serum concentrations of free indoxyl sulfate (-3.1% (-5.9%; -0.3%) ( p = 0.035)), free p-cresyl sulfate (-2.5% (-4.7%; -0.3%) ( p = 0.034)), total indole acetic acid (IAA) (-1.6% (-3.0%; -0.3%) ( p = 0.020)), free IAA (-6.6% (-9.3%; -3.7%) ( p < 0.001)), total serum p-cresyl glucuronide (pCG) (-3.0% (-5.6%; -0.5%) ( p = 0.021)) and free pCG levels (-3.3% (-5.8%; -0.8%) ( p = 0.010)). The observed associations between dietary fibre intake and the investigated PBUTs highlight potential benefits of fibre intake for the paediatric CKD population. The present observational findings should inform and guide adaptations of dietary prescriptions in children with CKD.

The aim of this study was to report on the clinical characteristics and outcomes of Belgian children with chronic kidney disease (CKD). Between 2001 and 2005, we followed 143 new successive patients younger than 20 years of age with a glomerular filtration rate of <60 ml/min/1.73 m(2) prospectively in a Belgian department of pediatric nephrology. The incidence of diagnosed CKD was 11.9 per million child population (pmcp), and the incidence of renal replacement therapy was 6.2 pmcp. There were 67% patients in CKD stage 3, 19% in CKD stage 4 and 14% in CKD stage 5. Patients with congenital anomalies of the kidney and urinary tract (CAKUTs), hereditary diseases and glomerular diseases were diagnosed at a median age of 1, 2 and 10 years, respectively. CAKUTs were the main causes of CKD, accounting for 59% of all cases. After 3, 4 and 5 years of follow-up, 27, 31 and 38% of patients treated conservatively, respectively, reached end-stage renal failure (ESRF). The progression rate to ESRF was eightfold higher in patients with CKD stage 4 than in those with CKD stage 3. Among our patient group, hereditary diseases progressed more rapidly to ESRF than CAKUTs. Transplantation was performed preemptively in 22% of these children. Infections and cardiovascular diseases were the main causes of death.