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[摘要]
Alport综合征(Alport syndrome,AS)是一种累及肾脏、耳、眼的基底膜结构异常的遗传性疾病,发病率约为1:5000。其眼部异常的报道较少见,但对疾病的诊断却有重要的价值,眼部异常相关病理机制分析将为我们揭开眼部异常发生的真正原因,对于疾病的认识和治疗具有重要意义。
[Key word]
[Abstract]
Alport syndrome(AS)is a genetic disease characterized by abnormal basement membrane structure of the kidneys, ears and eyes. The incidence of the disease is 1:5000 approximately. The report on ocular manifestations is relatively scarce, however, it is of great value to diagnosis of the disease. The ocular tissue histopathological analysis provides an effective method to uncover the pathological mechanisms of AS. Besides, it is good for understanding and treatment of AS.
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