| | | | | Alport综合征6例临床分析 | | | 汤洋;唐罗生 | | | 目的总结Alport综合征的临床特征,尤其是[部表现。方法对6例Alport综合征患者的[部、电听力及肾功能检查结果进行回顾性分析。结果有[部改变者4例,高频感音性神经性耳聋者2例,肾功能衰竭者3例,肾组织活检示Alport综合征改变者2例,阳性家族史5例。结论对于有前锥形晶体和/或视网膜黄斑周围微粒改变者应行肾活检以确诊Alport综合症。
【作者单位】：中南大学湘雅二医院眼科;中南大学湘雅二医院眼科
【关键词】：Alport综合征;前锥形晶体;黄斑斑点
【分类号】：R692.3
【DOI】：cnki:ISSN:1006-4443.0.2006-11-029
【正文快照】：
Alport综合征(Alport syndrome,AS)又称眼耳肾综合征,遗传性肾炎,家族性肾炎。首先于1875年由DicRinson研究一个三代血尿的家族时发现,1927年Alport发现家族性肾病伴有听力损害而首次命名。自Flinter于1988年提出诊断标准以来,由于其临床表现多样及诊断手段的缺乏,常常漏诊或误 | | |
| | |  推荐 下载CAJ全文  下载PDF全文 | | | CAJViewer7.0阅读器支持所有CNKI文件格式，AdobeReader仅支持PDF格式 | | | | Cinical analysis of Alport syndrome | | | TANG Yang;TANG Luo-sheng.Department of Ophthalmology;the second affiliated hospital of xiangya medical college;Changsha;410011;China | | | Objective To assess the clinical manifestation of Alport syndrome,especially the ocular features.Methods The ophthalmologic,audiologic and renal function examination results of six patients with Alport syndrome were analyzed retrospectively.Results four patients had ocular changes,two patients had sensorineural deafness,three patients with renal failure,two patients did renal biopsy and had Alport syndrome changes,five patients had family history.Conclusions We should do renal biopsy to the patient with anterior lenticonus and/or granulations surrounding the foveal area in order to final diagnose Alport syndrome.
【Keyword】：Alport syndrome;Anterior lenticonus;Macular flecks |
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