马蹄肾合并肾小球病的临床病理分析  

Analysis on Clinical and Pathological Data of Horseshoe Kidney Patients with Glomerulopathy

作　　者：胡盼盼[1] 谢院生[1] 金美玲[1,2] 尹忠[1] 张雪光[1] 邱强[1] 刘述文[1] 陈香美[1] 

机构地区：[1]解放军总医院,全军肾脏病研究所,肾脏疾病国家重点实验室,北京100853 [2]南开大学医学院,天津300071

出　　处：《中国中西医结合肾病杂志》2014年第5期401-403,I0003,共4页Chinese Journal of Integrated Traditional and Western Nephrology

基　　金：国家重大科学研究计划项目(No.2011CB944004)

摘　　要：也页目的：马蹄肾是先天性肾脏融合畸形,马蹄肾合并肾小球病时,由于担心肾穿刺术后并发症,很少进行肾活检,从而得不到明确的病理诊断,难以得到应有的正确治疗。本文通过总结分析马蹄肾合并肾小球病的临床病理资料,旨在提高其诊断与治疗水平。方法：收集2008年1月~2013年6月在解放军总医院就诊,通过影像学确诊马蹄肾且肾活检病理诊断为肾小球病的病例。回顾分析这些病例的临床资料、实验室检查、影像学检查、肾活检适应证的把握、肾穿刺注意事项、肾活检病理检查结果以及治疗随访的资料。结果：共5例患者影像学检查确诊马蹄肾且肾活检病理诊断为肾小球病。（1）临床上主要表现为夜尿增多、下肢水肿、高血压及尿检异常。（2）尿蛋白定量均〉1 g/24 h,血肌酐正常或偏高。（3）影像检查明确诊断为马蹄肾,均为下极融合。（4）均有肾活检的适应证,没有肾脏大血管畸形等禁忌证,患者血压均控制在140/90 mmHg以下,凝血功能正常；在患者签署知情同意书后,由经验丰富的医生在超声引导下用肾穿刺活检枪在“肾上极”进行肾活检；肾穿刺术后严密观察,患者没有出现术后并发症。（5）肾活检病理诊断分别为局灶节段性肾小球硬化、膜性肾病、原发性IgA肾病、过敏性紫癜肾炎（继发性IgA肾病）、狼疮肾炎。（6）根据肾活检病理结果给予相应的治疗,每月随访1次,随访6个月后结果显示患者尿蛋白定量明显下降、血压及血肌酐趋于稳定。结论：肾小球病是马蹄肾的重要合并症,充分评估其肾活检的意义及风险,明确其病理类型,有助于指导治疗、延缓疾病的进展。Objective：Horseshoe kidney （ HSK） is common congenital renal fusion anomaly. However,when HSK is com-bined with glomerulopathy,few HSK patients receive renal biopsy because of apprehension about postoperative complications of punc-ture,which leads them to be unable to obtain appropriate treatment. This study aims to raise the level of diagnosis and treatment for HSK patients with glomerulopathy. Methods：We enrolled the HSK patients who received renal biopsy from the patients who came to the Chinese PLA General Hospital between January 2008 and June 2013 and were diagnosed with HSK by imajing. we retrospectively studied clinical data,laboratory examination,imaging and indications,cautions and pathological findings of renal biopsy of them. Fur-thermore,we also retrospectively studied the data on follow-up of curative effect. Results：Five patients who were not only diagnosed with HSK by imaging but also diagnosed with glomerulopathy by renal biopsy were enrolled. （1） These patients＇ clinical manifesta-tion involves polyuria at night,edema of lower extremity,hypertension and abnormal uroscopy. （2） their urine protein excretion was more than 1 g/24 h and their serum creatinine nomal was or increased. （3） The imaging findings all identified the HSK definitely and revealed the fusion of bilateral renal lower poles. （4） All of these five patients had indications of renal biopsy and had not absolute contraindications of renal biopsy as aberrant renal great vessels. Their blood pressure was controlled below 140/90 mmHg and the blood coagulation function of the patients was normal. After securing informed consent, renal biopsy was performed by experienced doctors under ultrasonic guidance using a standard needle biopsy gun at the “renal upper pole”. None of the patients presented any postoperative complications. （5） The patients were diagnosed with focal segmental glomerulosclerosis,membranous nephropathy,pri-mary immunoglobulin A nephropathy,Henoch-Schnlein purpura nephritis（ secondary

关 键 词：也页 马蹄肾 肾小球病 蛋白尿 肾活检 

分 类 号：R692.6[医药卫生—泌尿科学] R692.1[医药卫生—外科学]