儿童Clq肾病的临床病理特点及治疗  

Clinicopathological features and treatment of Clq nephropathy in children

作　　者：赵三龙[1] 黄松明[1] 朱春华[1] 张维真[1] 鲍华英[1] 吴红梅[1] 张爱华[1] 陈颖[1] 韩媛[1] 赵非[1] 

机构地区：[1]南京医科大学附属南京儿童医院肾内科,210008

出　　处：《中华肾脏病杂志》2011年第12期877-883,共7页Chinese Journal of Nephrology

摘　　要：目的探讨儿童Clq肾病的临床、病理特点及治疗方法。方法回顾性分析本院8年来经肾活检确诊的23例Clq。肾病患JIAt$床、病理和预后资料。结果Clq肾病占同期肾活检的原发性肾小球疾病的4．78％。23例患儿中，男15例，女8例；年龄10个月～12岁5个月，平均发病年龄（5．0＋3．4）岁；。肾病综合征（NS）18例（2例伴镜下血尿），肾病水平蛋白尿4例（1例伴镜下血尿），单纯镜下血尿1例。1例NS起病前曾服用2周中药，发病时同时并发急性。肾功能不全。3例患儿有肾脏病家族史，其中2例（肾病水平蛋白尿）为姐弟，父亲亦有蛋白尿，基因检测证实为家族性Denys-Drash综合征并发Clq肾病。1例患儿（Ns）姐姐亦有大量蛋白尿（未行肾活检）。所有患儿起病时血压均正常，补体正常，抗核抗体、抗dsDNA抗体、抗Sm抗体及乙肝两对半均阴性。18例NS中13例激素耐药（72．2％），4例激素依赖，1例激素敏感。光镜下，13例为微小病变（MCD）（其中1例伴间质性肾炎）；6例为系膜增生性肾小球肾炎（MsPGN）；4例为局灶节段性肾小球硬化（FSGS）。另9例患儿伴有不同程度的小管萎缩和间质纤维化。免疫荧光下，所有患儿均见系膜区弥漫性Clq≥2＋沉积，其中伴IgG沉积18例，IgM沉积18例，IgA沉积8例，c3沉积11例，6例患儿呈“满堂亮”表现。除4例患儿电镜下未见。肾小球外，其余19例中4例系膜区见电子致密物沉积。12例激素耐药（包括2例。肾病水平蛋白尿者）及3例激素依赖患儿在激素治疗基础上加用静脉CTX冲击；3例激素耐药者加用环孢素A（CsA）口服；1例激素依赖患儿给予足量激素重新诱导；1例单纯镜下血尿患儿及2例Denys—Drash综合征并发Clq肾病患儿仅给予血管紧张素转换酶抑制剂（ACEI）治疗。其中1例患儿CTX冲击满疗程无效后换用CsA治疗；1例患儿CTX冲击满疗程无�Objective To investigate the clinicopathological characteristics and treatment of Clq nephropathy in children. Methods Data of 23 Clq nephropathy eases in NanjingChildren＇s Hospital during recent eight years were retrospectively reviewed. Results The incidence of Clq nephropathy was 4.78% in primary glomerulonephritis proven by biopsy. Among 23 patients, 15 were boys and 8 were girls. The mean age at onset was （5.0＋3.4） years old with a range of 0.9-12.4 years. The clinical manifestations included nephrotic syndrome （NS） in 18 cases （78.3%）, nephrotic-range proteinuria in 4 cases （17.4%） and microhematuria in 1 case. Two patients with NS and one patient with nephrotic-range proteinuria also presented microhematuria. One patient with NS who received oral herbal medicine for two weeks developed acute renal insufficiency at the same time of diagnosis. Three cases had a family history of kidney disease, among them two patients （presented nephrotic range proteinuria） were siblings, their father had proteinuria as well, and routine genetic examination confirmed familial Denys-Drash syndrome in association with Clq nephropathy. One NS patient＇s sister had nephrotic-range proteinuria too, but renal biopsy was not performed. No patient had hypertension. None of the patients had low C3 or C4 levels, and serological markers of systemic lupus erythematosus were absent. Light microscopy showed minimal change disease （MCD） in 13 cases （56.5%）, mesangial proliferative glomerulonephritis （MsPGN） in 6 （26.1%） and focal segmental glomernlosclerosis （FSGS） in 4 （17.4%）. Immunofluorescence displayed Clq co-deposits of IgG （78.3%）, IgM （78.3%）, IgA （34.8%） and C3 （47.8%）, and a ＂full-house＂ pattern was found in 6 patients （26.1%）. Electron microscopy revealed 4 out of 19 had mesangial deposits, except for 4 patients whose glomerulus could not be found. Children with either NS （18 cases） or nephrotic-range proteinuria （2 cases）received prednisone, among

关 键 词：Clq肾病 病理学 临床 儿童 

分 类 号：R726.9[医药卫生—儿科]