Reddy Soumya, Ghante Abhishek, Vankalakunti Mahesha, Vasudevan Anil
Department of Pediatric Nephrology, St. John's Medical College Hospital, St. John's National Academy of Health Sciences, Bengaluru, India.
Department of Laboratory Medicine, Manipal Hospital, Bengaluru, India.
Clin Exp Pediatr. 2025 Apr;68(4):311-318. doi: 10.3345/cep.2024.01256. Epub 2024 Nov 28.
In children, C3 glomerulopathy (C3G) is a heterogeneous disease characterized by diverse clinicopathological profiles and kidney outcomes. However, diagnostic work-up in resource-limited settings is challenging because of the unavailability of complement assays and limited access to electron microscopy or genetic testing.
This study aimed to describe the clinicopathological features and response to immunosuppression and evaluate renal outcomes among children with C3G in a resource-limited setting.
This retrospective cohort study involved a review of the hospital records of 46 children (2013-2021) diagnosed with C3G on kidney biopsy. Their clinical, laboratory, treatment, and outcome details at onset and follow-up were noted.
The mean (standard deviation) age was 9 (4) years. The common presentation was acute nephritis (27 [58.6%]), while 1 in 5 (19.5%) presented with rapidly progressive glomerulonephritis. Focal crescentic glomerulonephritis (14 [30.4%]) was the common histological pattern. Electron microscopy was performed in 22 (47.8%), of which 17 were C3 glomerulonephritis and 4 were dense deposit disease (DDD). None of the patients underwent complement assay or genetic testing. Almost two-thirds (63%) received empirical immunosuppressive therapy, most commonly steroids. Of the 31/46 who completed follow-up (median [interquartile range] duration, 11.5 [6-24] months), 6 (19.4%) demonstrated complete kidney recovery, while the other 25 (80.7%) had kidney sequelae; of them, 5 (16.1%) progressed to end-stage kidney disease and 2 (4.3%) died by the last follow-up.
Pediatric C3G has a variable clinicopathological spectrum, while DDD is less common. Most patients present with glomerulonephritis and significant morbidities. The lack of genetic and C3Nephritic factor testing is a barrier to the comprehensive phenotyping and management of C3G in resource-limited settings.
在儿童中，C3肾小球病（C3G）是一种异质性疾病，具有多种临床病理特征和肾脏转归。然而，在资源有限的环境中进行诊断检查具有挑战性，因为无法进行补体检测，且获得电子显微镜或基因检测的机会有限。
本研究旨在描述资源有限环境下儿童C3G的临床病理特征、对免疫抑制的反应，并评估肾脏转归。
这项回顾性队列研究回顾了46例（2013 - 2021年）经肾脏活检诊断为C3G的儿童的医院记录。记录了他们发病时及随访时的临床、实验室、治疗和转归细节。
平均（标准差）年龄为9（4）岁。常见表现为急性肾炎（27例[58.6%]），而五分之一（19.5%）表现为快速进展性肾小球肾炎。局灶性新月体性肾小球肾炎（14例[30.4%]）是常见的组织学类型。22例（47.8%）进行了电子显微镜检查，其中17例为C3肾小球肾炎，4例为致密物沉积病（DDD）。所有患者均未进行补体检测或基因检测。近三分之二（63%）接受了经验性免疫抑制治疗，最常用的是类固醇。在完成随访的31/46例患者中（中位[四分位间距]病程，11.5[6 - 24]个月），6例（19.4%）实现了肾脏完全恢复，而其他25例（80.7%）有肾脏后遗症；其中，5例（16.1%）进展为终末期肾病，2例（4.3%）在最后一次随访时死亡。
儿童C3G具有可变的临床病理谱，而DDD较少见。大多数患者表现为肾小球肾炎且有显著的发病率。在资源有限的环境中，缺乏基因和C3肾炎因子检测是C3G全面表型分析和管理的障碍。
