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儿童新月体性肾小球肾炎的病因、临床特征及转归:一项系统评价

Etiology, clinical profile, and outcomes of crescentic glomerulonephritis in children: a systematic review.

作者信息

Meena Jitendra, Nangla Parnika, Ojha Sanjay, Sinha Aditi, Hari Pankaj, Bagga Arvind

机构信息

Division of Nephrology, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India.

出版信息

Pediatr Nephrol. 2025 Mar;40(3):675-684. doi: 10.1007/s00467-024-06521-3. Epub 2024 Sep 16.

Abstract

BACKGROUND

Crescentic glomerulonephritis, if not managed promptly, is associated with unsatisfactory outcomes. There are limited studies reporting the outcomes of crescentic glomerulonephritis in children.

OBJECTIVES

This systematic review is aimed at synthesizing the data on etiology, clinical profile, and outcomes of crescentic glomerulonephritis in children.

DATA SOURCES

We performed a literature search using PubMed, Embase, and Web of Science from inception to January 2024 without language or geographic restrictions.

STUDY ELIGIBILITY CRITERIA

Cohort and cross-sectional studies with at least 10 participants reporting etiology, clinical features, and outcomes on crescentic glomerulonephritis in children were considered eligible.

PARTICIPANTS AND INTERVENTIONS

Children aged less than 18 years with crescentic glomerulonephritis.

STUDY APPRAISAL AND SYNTHESIS METHODS

We used a tool by Hoy et al. for assessing the quality of studies. We calculated pooled estimates using random effect meta-analyses. Primary outcome was the pooled proportion of patients progressing to kidney failure.

RESULTS

From 1706 records, we included 36 studies (1548 participants) from 16 countries. Etiology was immune-complex glomerulonephritis in 76% (95% CI 67 to 85), pauci-immune in 19% (13 to 25), and anti-GBM disease in 5% (3 to 7) of patients. Gross hematuria, oliguria, edema, and hypertension were observed in 63% (41 to 82), 57% (34 to 79), 79% (65 to 90), and 64% (49 to 77), respectively. In-hospital mortality, reported in 11 studies, was 7% (4 to 11). Progression to kidney failure and chronic kidney disease was reported in 27% (21 to 33) and 50% (29 to 71) of patients, respectively. Risk factors for kidney failure included oliguria, dialysis requirement at onset, estimated GFR, proportion of fibrous crescents, and pauci-immune glomerulonephritis as the underlying etiology.

LIMITATIONS

High heterogeneity in pooled estimates of the outcomes.

CONCLUSIONS AND IMPLICATIONS OF KEY FINDINGS

Immune-complex glomerulonephritis is the most common etiology in children, with edema, hypertension, gross hematuria, and oliguria being the chief presenting manifestations. Almost one in every four patients with crescentic glomerulonephritis progressed to kidney failure.

SYSTEMATIC REVIEW REGISTRATION NUMBER

PROSPERO registration number CRD42024500515.

摘要

背景

新月体性肾小球肾炎若不及时治疗,预后不佳。关于儿童新月体性肾小球肾炎预后的研究有限。

目的

本系统评价旨在综合儿童新月体性肾小球肾炎的病因、临床特征及预后数据。

数据来源

我们使用PubMed、Embase和Web of Science进行文献检索,检索时间从数据库建库至2024年1月,无语言或地域限制。

研究纳入标准

纳入至少10名参与者的队列研究和横断面研究,这些研究报告了儿童新月体性肾小球肾炎的病因、临床特征及预后。

参与者和干预措施

年龄小于18岁的新月体性肾小球肾炎患儿。

研究评估和综合方法

我们使用Hoy等人的工具评估研究质量。我们采用随机效应荟萃分析计算合并估计值。主要结局是进展至肾衰竭的患者合并比例。

结果

从1706条记录中,我们纳入了来自16个国家的36项研究(1548名参与者)。病因方面,76%(95%CI 67至85)的患者为免疫复合物性肾小球肾炎,19%(13至25)为寡免疫性,5%(3至7)为抗肾小球基底膜病。63%(41至82)的患者出现肉眼血尿,57%(34至79)出现少尿,79%(65至90)出现水肿,64%(49至77)出现高血压。11项研究报告的院内死亡率为7%(4至11)。分别有27%(21至33)和50%(29至71)的患者进展至肾衰竭和慢性肾脏病。肾衰竭的危险因素包括少尿、起病时需要透析、估计肾小球滤过率、纤维性新月体比例以及潜在病因是寡免疫性肾小球肾炎。

局限性

结局的合并估计值存在高度异质性。

关键发现的结论和意义

免疫复合物性肾小球肾炎是儿童最常见的病因,水肿、高血压、肉眼血尿和少尿是主要临床表现。几乎每四名新月体性肾小球肾炎患者中就有一人进展至肾衰竭。

系统评价注册号

PROSPERO注册号CRD42024500515。

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