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Kidney Int. 2011 Mar;79(6):663-670. doi: 10.1038/ki.2010.484. Epub 2010 Dec 15.

Progressive podocyte injury and globotriaosylceramide (GL-3) accumulation in young patients with Fabry disease.

Author information

1
Department of Pathology, University of Washington, Seattle, Washington, USA. Electronic address: najafian@u.washington.edu.
2
Department of Medicine, Haukeland University Hospital, Bergen, Norway; Institute of Medicine, University of Bergen, Bergen, Norway.
3
Department of Pathology, University of Bergen, Bergen, Norway; Institute of Gade, University of Bergen, Bergen, Norway.
4
INSERM, U983, Université René Descartes, Hôpital Necker-Enfants Malades AP-HP, Paris, France.
5
Institute of Medicine, University of Bergen, Bergen, Norway; Department of Pediatrics, Haukeland University Hospital, Bergen, Norway.
6
Department of Pediatrics, University of Minnesota, Minneapolis, Minnesota, USA.
7
Department of Pediatrics, University of Minnesota, Minneapolis, Minnesota, USA; Department of Medicine, University of Minnesota, Minneapolis, Minnesota, USA.

Abstract

Progressive renal failure often complicates Fabry disease, the pathogenesis of which is not well understood. To further explore this we applied unbiased stereological quantitative methods to electron microscopic changes of Fabry nephropathy and the relationship between parameters of glomerular structure and renal function in 14 young Fabry patients (median age 12 years). Renal biopsies were obtained shortly before enzyme replacement therapy from these patients and from nine normal living kidney donors as controls. Podocyte globotriaosylceramide (GL-3) inclusion volume density increased progressively with age; however, there were no significant relationships between age and endothelial or mesangial inclusion volume densities. Foot process width, greater in male Fabry patients, also progressively increased with age compared with the controls, and correlated directly with proteinuria. In comparison to the biopsies of the controls, endothelial fenestration was reduced in Fabry patients. Thus, our study found relationships between quantitative parameters of glomerular structure in Fabry nephropathy and age, as well as urinary protein excretion. Hence, podocyte injury may play a pivotal role in the development and progression of Fabry nephropathy.

PMID:
21160462
PMCID:
PMC3640823
DOI:
10.1038/ki.2010.484
[Indexed for MEDLINE]
Free PMC Article

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