纤维样肾小球病1例报告并文献复习

A report on one case of fibrillary glomerulopathy and a review of literature

目的结合文献,探讨纤维样肾小球病的诊断、治疗及预后。方法对我院肾病中心经肾活检确诊的1例纤维样肾小球病患者的临床资料,结合国内外相关文献进行分析。结果该例纤维样肾小球病患者为青年男性,临床表现为大量蛋白尿、低蛋白血症、高血压、肾功能异常,肾脏病理光镜表现为膜增殖性肾炎,免疫荧光IgG、IgA、IgM和补体均阳性,电镜下可见<20 nm的纤维丝沉积于肾小球系膜区。给予血管紧张素转化酶抑制剂(ACEI)治疗后患者肌酐上升超过50%,给予糖皮质激素治疗2个月无效,且出现严重不良反应,肾衰竭加重。结论纤维样肾小球病是一种罕见的肾小球疾病,部分患者可找到病因,需依靠电镜诊断,目前缺乏有效的治疗方法,该病预后差,大多数患者会进入终末期肾衰。

Objective To discuss the diagnosis, treatment and prognosis of fibrillary glomerulopathy through analysis of one clinical case in combination with review of related literature. Methods According to the clinical data of one patient admitted to the depart- ment, the experience in diagnosis, treatment and prognosis in combination with review of related literature was discussed in this arti- cle. Results The patient was a young man, whose clinical characteristics manifested as nephrotic syndrome, hypertension and re- nal dysfunction. The light microscopy of renal pathology showed mesangial proliferative glomerulonephritis. By immunofluores- cence, glomerular positivity for IgG, IgA, IgM and complements was found. Fibrin less than 20 mn was seen in the mesangium by electron microscopy. The serum ereatinine was elevated by over 50% after application of ACEI, and the hormone therapy was inef- fective with seriously side-effect. Conclusion Fibrillary glomerulopathy is a rare glomerular disease, some patients maybe have the primary disease, and electron microscopy is the essential method to its diagnosis. Because of lack of effective treatments for this dis- ease, the prognosis is poor and most patients will progress to end stage of renal failure.