儿童C_(1q)肾病10例临床与病理分析  

Clinical and Pathological Analysis of Complement 1q Nephropathy in 10 Cases of Children

作　　者：陈慧敏[1] 卫敏江[1] 陈难[1] 

机构地区：[1]上海第二医科大学附属新华医院儿内科,上海200092

出　　处：《实用儿科临床杂志》2005年第11期1102-1103,共2页Journal of Applied Clinical Pediatrics

摘　　要：目的探讨C1q肾病的临床与病理改变的关系。方法对10例经肾活检确诊为C1q肾病患儿临床表现、肾小球、肾小管及免疫病理特征进行分析比较,6例肾病综合征中环磷酰胺冲击治疗3例,环胞素、霉酚酸酯和甲泼尼龙冲击治疗各1例。结果临床表现为单纯性血尿2例,肾炎综合征、急性肾炎各1例,肾病综合征6例;病理类型为轻微病变、系膜增生性肾小球肾炎各2例,局灶节段性肾小球硬化5例,新月体肾炎1例;肾小管间质1例无改变,Ⅰ级和Ⅱ级各3例,Ⅲ级2例,Ⅳ级1例;免疫荧光:系膜区均有娃著的以C1q为主的沉积。10例患儿平均随访25.7个月;6例肾病综合征均对激素抵抗,加用免疫抑制剂治疗,5例缓解,1例无效,肾功能渐减退。结论C1q肾病临床病理改变多样化,临床以肾病综合征为主,病理以局灶节段性肾小球硬化为主,对激素多不敏感,预后与间质损害程度相关,与C1q沉积无相关性。Objective To explore the relationship between clinical and pathological changes of complement 1 q（C1 q） nephropathy. Methods Clinical manifestation, pathologic features including glomerulus change, renal tubule - interstitial change and immunopathology were compared between 10 cases of C1q nephropathy in children,who were diagnosed by renal biopsy. Results Presentation included idiopathic nephritic syndrome（ 6 cases）, simple hematuria （ 2 cases）, nephritic syndrome （ 1 case）, rapidly progressive glomerulonephritis（ 1 case） ; Renal biopsy revealed focal segmental glomerulosderosis（FSGS） in 5, minimal - change disease（MCD） and mesangial proliferative glomerulonephritis （MsPGN） respectively in two and crescentic glomerulonephritis in one. In addition, there were renal- tubule interstitial changes with 3 cases of grade Ⅰ and grade Ⅱ each other, 2 of grade Ⅲ,1 of grade IV. The prominent immunofluorescent features was the presence of bright mesangial deposition of C1q. The average follow- up time was 25.7 months. Six cases presenting nephrotic syndrome were resistant to steroid, but 5 were released after imanunosuppressive therapy, the other had progressive renal insufficiency. Conclusions C1q nephropathy falls with the clinical- pathologic spectrum of FSGS generally. It is also presented as steroid-resistant nephritic syndrome. Moreover, the prognosis of C1q nephropathy is related to renal tubulointerstitial pathologic lesions not to C1q deposition.

关 键 词：补体1q 肾病 病理学 临床 

分 类 号：R726.91[医药卫生—儿科]