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比利时弗拉芒地区儿童原发性肾脏疾病的流行病学及临床病理特征

Epidemiology and clinicopathological characteristics of native kidney disease in children in Flanders, Belgium.

作者信息

Deleersnijder Dries, Knops Noël, Trouet Dominique, Van Hoeck Koen, Karamaria Sevasti, Vande Walle Johan, Mauel Reiner, Cools Louise, Meeus Gert, Dendooven Amélie, De Meester Johan, Laurens Wim, Sprangers Ben

机构信息

Laboratory of Molecular Immunology, Department of Microbiology, Immunology and Transplantation, Rega Institute, KU Leuven, Louvain, Belgium.

Division of Nephrology, University Hospitals Leuven, Louvain, Belgium.

出版信息

Pediatr Nephrol. 2023 May;38(5):1533-1545. doi: 10.1007/s00467-022-05719-7. Epub 2022 Oct 13.

Abstract

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. A higher resolution version of the Graphical abstract is available as Supplementary information.

摘要

背景

弗拉芒协作性肾小球肾炎研究组(FCGG)登记处是一个基于人群的肾脏活检登记处,自2017年起纳入了在比利时弗拉芒地区对儿童进行的所有原发性肾脏活检。

方法

2017年至2020年,纳入了148例儿科(<18岁)原发性肾脏活检病例。每例活检均获得组织病理学诊断和最终的肾脏病诊断,并评估两者之间的一致性。采用梅奥诊所慢性ity评分总结疾病慢性程度,对122例有>5个肾小球的活检病例进行了评估。

结果

肾脏活检率较高(每年每百万儿童中有29.0例活检),中位年龄为十岁(四分位间距5.8 - 14.7),男孩占主导(男性占56.1%)。共有140例活检(94.6%)显示出具有代表性的病理结果。肾小球疾病最为常见,IgA肾病/IgA血管炎(43例活检,29.1%)和微小病变病(MCD)(29例活检,19.6%)是总体上最常见的诊断。总体而言,诊断一致性较高(80.7%)。在Alport综合征和局灶节段性肾小球硬化(FSGS)中,一致性较低,因为肾脏病诊断通常由基因分析结果决定。肾病综合征是肾脏活检最常见的指征(31.8%),主要由MCD和FSGS引起。肾脏活检的疾病慢性程度一般较低,尽管27.3%诊断为FSGS的活检显示有中度至重度慢性损伤。

结论

所呈现的流行病学研究结果验证了先前欧洲登记处研究的数据,并可能促使全球肾脏活检登记处采用诸如临床病理一致性和慢性程度分级等新特征。可获取更高分辨率版本的图形摘要作为补充信息。

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