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患者曾患有感染后肾小球肾炎,现患有 C3 肾小球病。

C3 glomerulopathy in a patient with a history of post-infectious glomerulonephritis.

机构信息

UT Southwestern Medical Center, 5323 Harry Hines Blvd., Dallas, TX, 75390, USA.

Department of Pathology, UT Southwestern Medical Center, 5323 Harry Hines Blvd., Dallas, TX, 75390, USA.

出版信息

Pediatr Nephrol. 2024 Mar;39(3):745-748. doi: 10.1007/s00467-023-06177-5. Epub 2023 Oct 24.

Abstract

Post-infectious glomerulonephritis (PIGN) is an immune complex mediated glomerular injury occurring because of an infection, most commonly with group A beta-hemolytic streptococcus in children. C3 glomerulopathy (C3G) is a distinct clinicopathological entity occurring secondary to dysregulation of alternate complement pathway encompassing both C3 glomerulonephritis (C3GN) and dense deposit disease (DDD). While most patients with PIGN attain complete remission with normalized complement levels by 6-8 weeks after presentation, patients with C3G continue to have hypocomplementemia with high rates of progressive kidney disease. Here, we report a patient diagnosed with dense deposit disease after his initial presentation with PIGN three years prior. While current literature continues to explore the overlapping and distinguishing features of PIGN and C3G, including how underlying defects in the alternate complement pathway may commonly contribute to both diseases, this case further exemplifies the importance of recognizing the clinico-pathogenic features of PIGN and C3G in pediatric patients with glomerulonephritis.

摘要

感染后肾小球肾炎 (PIGN) 是一种免疫复合物介导的肾小球损伤,发生于感染后,最常见于儿童的 A 组乙型溶血性链球菌感染。C3 肾小球病 (C3G) 是一种继发于替代补体途径失调的独特临床病理实体,包括 C3 肾小球肾炎 (C3GN) 和致密沉积物病 (DDD)。虽然大多数 PIGN 患者在出现症状后 6-8 周内通过补体水平正常化达到完全缓解,但 C3G 患者仍持续低补体血症,且进展性肾病的发生率较高。在这里,我们报告了一名患者,他在三年前初次出现 PIGN 后被诊断为致密沉积物病。虽然目前的文献仍在探讨 PIGN 和 C3G 的重叠和区别特征,包括替代补体途径的潜在缺陷如何可能共同导致这两种疾病,但该病例进一步说明了在患有肾小球肾炎的儿科患者中认识 PIGN 和 C3G 的临床病理特征的重要性。

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