CAJ | 학술논문

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。
야혈목적：마제신시선천성신장융합기형,마제신합병신소구병시,유우담심신천자술후병발증,흔소진행신활검,종이득불도명학적병리진단,난이득도응유적정학치료。본문통과총결분석마제신합병신소구병적림상병리자료,지재제고기진단여치료수평。방법：수집2008년1월~2013년6월재해방군총의원취진,통과영상학학진마제신차신활검병리진단위신소구병적병례。회고분석저사병례적림상자료、실험실검사、영상학검사、신활검괄응증적파악、신천자주의사항、신활검병리검사결과이급치료수방적자료。결과：공5례환자영상학검사학진마제신차신활검병리진단위신소구병。(1)림상상주요표현위야뇨증다、하지수종、고혈압급뇨검이상。(2)뇨단백정량균>1 g/24 h,혈기항정상혹편고。(3)영상검사명학진단위마제신,균위하겁융합。(4)균유신활검적괄응증,몰유신장대혈관기형등금기증,환자혈압균공제재140/90 mmHg이하,응혈공능정상；재환자첨서지정동의서후,유경험봉부적의생재초성인도하용신천자활검창재“신상겁”진행신활검；신천자술후엄밀관찰,환자몰유출현술후병발증。(5)신활검병리진단분별위국조절단성신소구경화、막성신병、원발성IgA신병、과민성자전신염(계발성IgA신병)、랑창신염。(6)근거신활검병리결과급여상응적치료,매월수방1차,수방6개월후결과현시환자뇨단백정량명현하강、혈압급혈기항추우은정。결론：신소구병시마제신적중요합병증,충분평고기신활검적의의급풍험,명학기병리류형,유조우지도치료、연완질병적진전。
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 immunoglobulin A nephropathy) and lupus ne-phritis by renal biopsy. (6) Appropriate medication was administered based on the renal pathological findings. These patients were followed up once a month. The curative effects were followed for six months,revealing that urine protein excretion decreased signifi-cantly and that blood pressure and serum creatinine stabilized. Conclusion:Glomerulopathy is a important complication of HSK. After the value and risks of renal biopsy were comprehensively evaluated,it is important to determine the pathological type of glomerulopathy for HSK patients,which will aid in guiding treatment and postpone progress of the disease.