摘要
目的探讨Clq肾病的临床表现、病理改变、治疗效果和随访结果的特点,以加强对Clq肾病的认识。方法选择上海交通大学医学院附属新华医院儿内科2002年1月—2007年6月明确诊断为Clq肾病的患儿17例,从发病情况、临床表现、病理改变、治疗效果和随访结果5个方面进行分析。结果同期共389例患儿因各种肾脏疾病行经皮肾脏穿刺活组织检查术,参照Clq肾病的诊断标准,明确诊断为Clq肾病者共17例,占同期肾脏活组织检查的4.4%。其中男7例,女10例,行肾脏活组织检查时的年龄为2～11岁。临床表现为肾病综合征6例,大量蛋白尿3例,血尿8例(其中1例表现为持续肉眼血尿).肾功能均正常。肾脏病理检查显示,5例(5/17)为微小病变(MCD),10例(10/17)为局灶节段性肾小球硬化(FSGS),2例(2/17)为轻微病变(表现为系膜节段性轻度增生)。免疫荧光检查示,肾小球系膜区均以Clq沉积为主,强度为(++)～(+++),同时伴C3、IgM沉积者较多,未见"满堂亮"表现。电子显微镜下,除1例未见肾小球外,其余16例均可见电子致密物分布在系膜区和(或)内皮下。1例患儿在肾脏活组织检查后失访,其余16例予以治疗和随访.平均随访时间为(28±18)个月。1例轻微病变患儿仅应用血管紧张素转换酶抑制剂(贝那普利)治疗,目前完全缓解。其余15例予泼尼松治疗,其中3例加用其他免疫抑制剂,完全缓解7例(7/15),分别是2例FSGS、1例轻微病变和4例MCD患儿;部分缓解7例(7/15),分别是6例FSGS和1例MCD患儿;无效1例(1/15),病理表现为FSGS,但目前肾功能正常。结论Clq肾病临床表现多样化,病理改变以FSGS和MCD为主,治疗效果和长期预后与病理改变密切相关,FSGS的疗效和长期预后不容乐观。
Objective To summarize the clinical manifestations, pathological changes, response to treatment and the follow-up outcomes of children with Clq nephropathy, so as to better our understanding of the disease. Methods The incidence, clinical manifestations, pathologic findings, and outcomes of treatment and follow-up were analyzed in 17 children with Clq nephropathy, who were treated in Xinhua Hospital from Jan. 2002 to Jun. 2007. Results Seven- teen （4.4%） Clq nephropathy patients, including 7 boys and 10 girls, aged from 2 to 11 years old, were identified from 389 children who underwent renal biopsy due to various reasons. Six patients presented with nephrotic syndrome, 3 cases with nephritic proteinuria and 8 cases with hematuria, including one with persistent gross hematuria. Light microscopy showed focal segmental glomerulo sclerosis （FSGS） in 10 （10/17）, minimal change disease （MCD） in 5 （5/17） and focal mesangial proliferative glomerulonephritis in 2 （2/17）. Immunofluorescence revealed Clq depositing dominantly in all 17 patients （ ＋ ＋, ＋ ＋ ＋）, whereas IgM and C3 were more consistent. No “full-house” pattern was detected. Electron-dense deposits were evident in 16 patients. Sixteen patients were followed-up and the mean follow-up period was （28±18） months. One patient treated with only angiotensin-converting enzyme inhibitors had good outcome. Fifteen patients were treated with corticosteroids and 3 of them received additional immunosuppressive agents. Seven patients achieved complete remission, in which 4 presented with MCD, 2 with FSGS and 1 with focal mesangial proliferative glomerulonephritis. Six patients with FSGS and 1 patient with MCD experienced partial remission. The re- maining 1 patient with FSGS showed poor response to corticosteroids and other immunosuppressive therapy （cyclophosphamide and mycofenolate mofetil）. All the patients had normal renal function at last follow-up. Conclusion Childhood Clq nephropathy has various clinical presentations.
出处
《上海医学》
CAS
CSCD
北大核心
2008年第6期400-404,共5页
Shanghai Medical Journal
关键词
Clq肾病
病理改变
临床表现
治疗
预后
儿童
Clq nephropathy
Histopathologic findings
Clinical manifestation
Treatment
Prognosis
Children
