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Focal and segmental glomerulosclerosis 总被引:3,自引:0,他引:3
An increasing cause of end-stage renal disease is the pathological lesion focal and segmental glomerulosclerosis (FSGS). FSGS
is characterized by proteinuria and frequently nephrotic syndrome with ensuing renal failure. The etiology remains unknown
in the majority of individuals. The idiopathic form of FSGS is most common; however, secondary forms of FSGS do exist. There
is a form of FSGS that is fulminant that frequently recurs after renal transplantation with an estimated frequency of approximately
30%, suggesting that the pathogenesis is not solely a result of intrinsic kidney disease. Recently, hereditary forms of the
disease were recognized as well as those associated with other congenital syndromes. Known genetic causes of the hereditary
form of this disease have been suggested to account for upwards of 18% of cases. This review will address recent discoveries
of the genetic mechanisms of hereditary FSGS and the current interpretations of their interactions at the slit diaphragm.
Received 17 April 2006; received after revision 23 May 2006; accepted 6 July 2006 相似文献
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