摘要
目的:探讨Alport综合征(AS)的临床与病理关系。方法:回顾性分析1989年1月至2007年4月通过肾活检确诊的AS患儿29例的病理改变及临床特点。结果:29例患儿均有典型的AS病理特征。另外还发现伴有轻度系膜增生11例,足细胞损伤13例,节段和球性硬化2例,间质泡沫细胞5例。伴系膜增生或足细胞损伤患儿尿蛋白显著增高。结论:肾小球基底膜的改变是AS的典型特征。然而,其他非特异性的改变如系膜增生、足细胞损伤、间质泡沫细胞、节段或球性肾小球硬化也可见到,他们的出现预示着疾病向终末肾发展。
Objective:The aim of the present study was to identify the clinicopathological correlation of the Alport syndrome (AS). Methods:We reviewed clinical materials and findings of renal biopsies obtained from 25 children with AS from Jan 1989 to Dec 2005. The electron microscopy changes were emphasized at the diagnosis on AS. Results:All of AS children had typical features of glomerular basement membrane (GBM) changes. Additionally,mild mesangial hypercellurarity was observed in 11 of 25 cases,podocyte injury in 13,segmental and globe glomerulosclerosis in 2,interstitial foam cell in 5. The levels of proteinuria of AS with mild mesangial hypercelluarity and podocyte injury were higher than those of children without these changes. Conclusion:GBM changes are the typical features of AS. However,other non-specific changes that represent progressive toward ESRF such as mild mesangial hypercelluarity,podocyte injury,segmental or globe glomerulosclerosis,interstitial foam cell,and tubular changes may also be found.
