Grosser Daniel S, Persad Paul, Talento Romualdo V, Shoemaker Lawrence R, Hunley Tracy E, Hidalgo Guillermo, Subtirelu Mihail M, Coventry Susan, Baliga Radhakrishna, Fogo Agnes B
Department of Pathology and Laboratory Medicine, University of Texas Health Science Center At San Antonio, San Antonio, TX, USA.
Laboratory for Kidney Pathology, Nashville, TN, USA.
Pediatr Nephrol. 2022 Mar;37(3):593-600. doi: 10.1007/s00467-021-05245-y. Epub 2021 Aug 28.
IgA-dominant infection-associated glomerulonephritis is well-documented in adults but has not been studied in depth in children. We assessed the incidence of pediatric IgA-dominant infection-associated glomerulonephritis and clinical and kidney biopsy findings.
Pediatric native kidney biopsies over a 10-year period with IgA dominance, strong C3, and findings indicative of infection-associated etiology were identified.
We identified 9 cases of IgA-dominant infection-associated glomerulonephritis, 0.8% of pediatric native kidney biopsies. Seven patients presented with elevated creatinine. All had hematuria and proteinuria. Eight patients had clinical evidence of infection: one each with central port infection by methicillin-sensitive Staphylococcus aureus, recurrent streptococcal pharyngitis and recent otitis media, streptococcal pharyngitis demonstrated 8 months after biopsy, suspected streptococcal scalded skin syndrome, and viral gastroenteritis, and three with serologic evidence of Streptococcal infection but no identified site of infection. All but one patient experienced short-term normalization of creatinine and resolution of proteinuria, though two eventually progressed to kidney failure: one 3 years later due to progressive disease and one 11 years later due to focal segmental glomerulosclerosis without concurrent immune deposits.
Pediatric IgA-dominant infection-associated glomerulonephritis is rare, and generally has a favorable prognosis, contrasting that seen in adults with severe comorbidities. A higher resolution version of the Graphical abstract is available as Supplementary.
IgA 为主的感染相关性肾小球肾炎在成人中有充分记录，但在儿童中尚未深入研究。我们评估了儿童 IgA 为主的感染相关性肾小球肾炎的发病率以及临床和肾活检结果。
确定了 10 年间具有 IgA 优势、C3 强阳性且有感染相关病因表现的儿童原发性肾活检病例。
我们识别出 9 例 IgA 为主的感染相关性肾小球肾炎病例，占儿童原发性肾活检病例的 0.8%。7 例患者肌酐升高。所有患者均有血尿和蛋白尿。8 例患者有感染的临床证据：分别为 1 例对甲氧西林敏感的金黄色葡萄球菌引起的中心静脉感染、复发性链球菌性咽炎和近期中耳炎、活检后 8 个月出现的链球菌性咽炎、疑似链球菌性烫伤样皮肤综合征、病毒性胃肠炎，以及 3 例有链球菌感染血清学证据但未发现感染部位的患者。除 1 例患者外，所有患者肌酐短期内恢复正常，蛋白尿消失，不过有 2 例最终进展为肾衰竭：1 例在 3 年后因疾病进展，另 1 例在 11 年后因局灶节段性肾小球硬化且无并发免疫沉积物。
儿童 IgA 为主的感染相关性肾小球肾炎罕见，总体预后良好，这与患有严重合并症的成人情况形成对比。更高分辨率的图形摘要可作为补充材料获取。
