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肾单位过少性肾发育不全:病例报告及文献综述

Oligomeganephronia: case report and literature review.

作者信息

Yang Xiang-dong, Shi Weiwei, Li Dengren, Peng Tao

出版信息

Srp Arh Celok Lek. 2014 Nov-Dec;142(11-12):732-5. doi: 10.2298/sarh1412732y.

Abstract

INTRODUCTION

Oligomeganephronia (OMN) is one of rare congenital kidney disease. The number of nephrons reduces and the volume of glomerulus increases. The incidence of OMN is uncertain because it is difficult to diagnose. There are no any special clinical manifestations of OMN. Renal pathology is the only way to diagnose OMN, so missed diagnosis always happens without renal pathology.

CASE OUTLINE

A 26-year-old male was diagnosed OMN associated with proteinuria and increased serum creatinine. The size of both kidneys on ultrasound was smaller than normal. Pathological features involved a reduced number of greatly enlarged glomeruli indicating OMN.

CONCLUSION

OMN is a rare disease and it has been rarely reported. The exact mechanism is not clear. The diagnosis mainly depends on pathological findings. For patients with OMN, proteinuria and renal dysfunction are often the main cause to visit a doctor. Early diagnosis is important.

摘要

引言

少肾小球肾单位症(OMN)是一种罕见的先天性肾脏疾病。肾单位数量减少,肾小球体积增大。由于难以诊断,OMN的发病率尚不确定。OMN没有任何特殊的临床表现。肾脏病理学检查是诊断OMN的唯一方法,因此在没有肾脏病理学检查的情况下,漏诊情况经常发生。

病例概述

一名26岁男性被诊断为OMN,伴有蛋白尿和血清肌酐升高。超声检查显示双肾体积小于正常。病理特征为肾小球数量减少且显著增大,提示为OMN。

结论

OMN是一种罕见疾病,鲜有报道。确切机制尚不清楚。诊断主要依赖于病理检查结果。对于OMN患者,蛋白尿和肾功能不全常常是就诊的主要原因。早期诊断很重要。

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