抗肾小球基底膜抗体阳性的14岁女孩月牙状肾小球肾炎的缓解临床病程。

Glomerular diseases Pub Date : 2025-01-03 eCollection Date: 2025-01-01 DOI:10.1159/000543339
Noortje M van der Meulen, Michiel J S Oosterveld, Marjolijn S W Quaak, Ester M M van Leeuwen, Joris J T H Roelofs, Rik Westland
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引用次数: 0

摘要

简介:抗肾小球基底膜肾小球肾炎(anti-GBM)伴严重肾衰竭,发病率高,在儿童中极为罕见。发表的关于这种情况的少数儿科研究报告了在出现时依赖肾脏替代治疗和少数病例肾功能部分或完全缓解。病例介绍:我们描述了一名14岁女孩患有月牙状肾小球肾炎的病例,基于抗gbm和髓过氧化物酶-抗中性粒细胞胞浆抗体双阳性,临床病程减轻,表现为疲劳,贫血,肾小球滤过率估计在34至42 mL/min/1.73 m2之间。对每日血浆交换治疗、皮质类固醇和口服环磷酰胺治疗反应迅速。结论:这种罕见的表现强调了即使在不明原因肾小球肾炎不太严重的儿童中,也需要考虑检测抗gbm抗体和/或进行肾活检,并强调了由于潜在的长期后遗症,这种疾病在儿童中的临床治疗困境。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Mitigated Clinical Course of Crescentic Glomerulonephritis with Positive Anti-Glomerular Basement Membrane Antibodies in a 14-Year-Old Girl.

Introduction: Anti-glomerular basement membrane (anti-GBM) glomerulonephritis is associated with severe kidney failure and high morbidity and is exceedingly rare in children. The few pediatric studies published about this condition report dependency of kidney replacement therapy at presentation and partial or complete response in kidney function in a minority of cases.

Case presentation: We describe the case of a 14-year-old girl with crescentic glomerulonephritis based on double positive anti-GBM and myeloperoxidase-antineutrophil cytoplasmic antibodies with a mitigated clinical course presenting with fatigue, anemia, and an estimated glomerular filtration rate range between 34 and 42 mL/min/1.73 m2. Response to treatment with daily therapeutic plasma exchanges, corticosteroids, and oral cyclophosphamide was prompt.

Conclusion: This ultrarare presentation highlights the need to consider determining anti-GBM antibodies and/or obtaining a kidney biopsy even in children with less severe presentations of unexplained glomerulonephritis and underlines the clinical treatment dilemma in this disease for children due to the potential long-term sequelae.

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