End-stage renal disease in patients with Fabry disease.

نویسندگان

  • Gregorio T Obrador
  • Akinlolu Ojo
  • Ravi Thadhani
چکیده

Anderson-Fabry disease (AFD) is a rare cause of end-stage renal disease (ESRD). Renal pathology in AFD is notable for diffuse deposition of glycosphingolipid in the renal glomeruli, tubules, and vasculature. Light microscopic findings include a “foamy” appearance of the glomeruli with diffuse swelling and vacuolization of visceral podocytes. Electron microscopic findings show podocytes and mesangial cells filled with lysosomal electron dense granules arranged in a lamellar, myelin pattern. Toluidine blue stain brings out the lipid deposits in these areas. ESRD is a common complication of AFD (see Alroy et al. and Branton et al. in this issue). Current information regarding ESRD in patients with AFD derives primarily from two studies that used data from the United States Renal Data System (USRDS) and the European Renal Association-European Dialysis and Transplant Association (ERA-EDTA) registry. In this article, we review the prevalence of ESRD due to AFD, the characteristics of Fabry patients at initiation of renal replacement therapy (RRT), and the outcomes associated with dialysis and transplantation.

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عنوان ژورنال:
  • Journal of the American Society of Nephrology : JASN

دوره 13 Suppl 2  شماره 

صفحات  -

تاریخ انتشار 2002