Rapidly progressive glomerulonephritis in children.
Rapidly progressive glomerulonephritis in children.
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DOI：
10.12669/pjms.38.icon-2022.5774
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发表时间：
2022-01
影响因子：
2.2
通讯作者：
Amanullah F
中科院分区：
文献类型：
--
作者：
Moorani KN;Aziz M;Amanullah F
快速进展性肾小球肾炎(RPGN)的特征是肾炎发展迅速，在几天或几周内肾功能丧失，在组织学上通常与大多数肾小球的新月体有关；这是一个具有挑战性的问题，特别是在资源匮乏的环境中。RPGN是一种诊断和治疗紧急情况，需要迅速评估和治疗，以防止不良结果。RPGN包括四大类：抗肾小球基底膜(GBM)病、免疫复合体介导的RPGN、免疫缺乏性疾病和特发性/重叠疾病。临床表现包括肉眼血尿、蛋白尿、少尿、高血压和水肿。诊断性评估，包括肾功能测试、电解质、尿液分析/显微镜和血清学(包括抗GBM抗体、抗中性粒细胞胞浆抗体(ANCA))与治疗同时开始。需要紧急的肾脏活检，以便进行特定的病理诊断，并评估疾病的活动性和慢性化，以指导特定的治疗。目前的儿童RPGN治疗指南是从成人的经验中采纳的，包括诱导和维持治疗。积极的联合免疫抑制显著改善了预后，然而，肾病综合征、需要透析的严重急性肾损伤、纤维新月体的存在和慢性化是肾脏存活率较低的预测因素。RPGN相关性感染后肾小球肾炎(PIGN)在无免疫抑制的儿童中预后较好，而免疫复合物介导的GN和狼疮性肾炎(LN)预后较差，发生终末期肾病(ESKD)的比例分别为50%和30%以上。考虑到需要及时诊断和紧急治疗以避免灾难性后果，我们对RPGN治疗的最新证据进行了审查，以帮助为我们环境中的儿童制定临床实践指南。信息来源和搜索策略：搜索策略在PubMed、Cochrane图书馆、谷歌学者的数字数据库中执行，从成立之日到2020年12月。三位研究者分别以以下检索词“快速进行性肾小球肾炎”、“儿童”、“新月体肾炎”、“管理”同时进行系统检索，回溯检索相关文献。
Rapidly progressive glomerulonephritis (RPGN), characterized by a rapid development of nephritis with loss of kidney function in days or weeks, is typically associated histologically, with crescents in most glomeruli; and is a challenging problem, particularly in low resource settings. RPGN is a diagnostic and therapeutic emergency requiring prompt evaluation and treatment to prevent poor outcomes. Histopathologically, RPGN consists of four major categories, anti-glomerular basement membrane (GBM) disease, immune complex mediated, pauci-immune disorders and idiopathic /overlap disorders. Clinical manifestations include gross hematuria, proteinuria, oliguria, hypertension and edema. Diagnostic evaluation, including renal function tests, electrolytes, urinalysis/microscopy and serology including (anti GBM antibody, antineutrophil cytoplasmic antibody (ANCA)) starts simultaneously with management. An urgent renal biopsy is required to allow specific pathologic diagnosis as well as to assess disease activity and chronicity to guide specific treatment. The current guidelines for management of pediatric RPGN are adopted from adult experience and consist of induction and maintenance therapy. Aggressive combination immunosuppression has markedly improved outcomes, however, nephrotic syndrome, severe acute kidney injury requiring dialysis, presence of fibrous crescents and chronicity are predictors of poor renal survival. RPGN associated post infectious glomerulonephritis (PIGN) usually has good prognosis in children without immunosuppression whereas immune-complex-mediated GN and lupus nephritis (LN) are associated with poor prognosis with development of end stage kidney disease (ESKD) in more than 50% and 30% respectively. Given the need for prompt diagnosis and urgent treatment to avoid devastating outcomes, we conducted a review of the latest evidence in RPGN management to help formulate clinical practice guidance for children in our setting. Information sources and search strategy: The search strategy was performed in the digital databases of PubMed, Cochrane Library, google scholar, from their inception dates to December 2020. Three investigators independently performed a systematic search using the following search terms “Rapidly progressive glomerulonephritis” “children” “crescentic glomerulonephritis” “management” at the same time, backtracking search for references of related literature.