23例仅C3沉积的儿童原发性肾病的临床病理特征

目的:通过回顾性分析首都医科大学附属北京儿童医院肾组织病理免疫荧光仅有C3沉积(≥2+)的肾病患儿的临床及病理特点,以加深对儿童以C3沉积为主的原发性肾病的认识.方法:选取首都医科大学附属北京儿童医院肾活检病理结果仅补体C3沉积(≥2+),不伴免疫复合物沉积或仅有少许IgM沉积的原发性肾病患儿.收集患者临床、病理信息及肾功能、补体C3,C4水平等资料.同时记录治疗及转归.结果:病理提示仅C3沉积(≥2+)的原发性肾病患儿23例,包括急性链球菌感染后肾炎7例,无明确病因的肾炎综合征6例,肾炎型肾病综合征7例,反复发作性肉眼血尿3例.其中补体C3降低者20例.23例患儿中病理表现为毛细血管内增生性肾炎11例,局灶增生性肾炎1例,新月体肾炎1例,增生硬化或硬化性肾炎6例,膜增生性肾炎4例.免疫荧光C3强度2+9例,3+14例,内皮下、上皮下、系膜区均可见沉积.随访中大部分肾功能正常,6例患儿就诊即出现严重肾功能不全者,预后不佳.结论:以C3沉积为主的肾病患儿临床与病理表现多种多样,预后与病理改变有关.

Clinical manifestations and pathological features of 23 primary glomerulonephritis children with isolated C3 deposits

Objective: To deeply understand clinical and pathological characteristics of primary glomerulonephritis children with C3 deposits through a retrospective analysis. Methods: Primary glomerulonephritis children with the C3 deposits diagnosed by renal biopsy in our hospital were selected. Clinicopathological features, renal functions,laboratory tests including C3 and C4, and pathological information were collected. Treatment and follow-up were recorded. Results: Twenty-three cases was involved, including 7 cases with acute post-streptococcal glomerulonephritis, 6 cases with nephritis syndrome, 7 cases with nephrotic syndrome, 3 cases with recurrent macroscopic haematuria, C3 decreased in 20 cases. Pathological manifestations of capillaries proliferative glomerulonephritis was in 11 cases, focal proliferative glomerulonephritis in 1 case, crescent nephritis in 1 case, sclerosing glomerulonephritis in 6 cases, and membrane proliferative glomerulonephritis in 4 cases. Pathological immune fluorescence intensity of C32+ were in 9 cases, 3+ in 14 cases. Immune complex deposition were all visible at subcutaneous, under the epithelium, mesangial area. Children were given positive treatment according to clinical manifestations. Most of cases recovered well. Conclusion: Clinical and pathological manifestations varied in children with primary glomerulonephritis with isolated C3 deposits. Prognosis is associated with pathological changes.