Focal and segmental glomerulosclerosis |
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Authors: | N. Daskalakis M. P. Winn |
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Affiliation: | (1) Duke University Medical Center, Duke Box 2903, Durham, North Carolina 27705, USA;(2) Center for Human Genetics, Duke University Medical Center, Durham, North Carolina 27710, USA |
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Abstract: | 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 |
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Keywords: | Familial focal segmental glomerulosclerosis familial nephropathy genetics kidney hereditary TRPC6 podocin nephrin ACTN4 |
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