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. 2010 Jun;3(3):303-305.
doi: 10.1093/ndtplus/sfq036. Epub 2010 Apr 8.

Fabry disease in donor kidneys with 3- and 12-year follow-up after transplantation

Willy Aasebø  1   2 , Erik H Strøm  3 , Torstein Hovig  3 , Liv H Undset  1 , Arvid Heiberg  4 , Trond Jenssen  1   5
Affiliations

Fabry disease in donor kidneys with 3- and 12-year follow-up after transplantation

Willy Aasebø et al. NDT Plus. 2010 Jun.

Abstract

Enzyme replacement therapy (ERT) has been introduced for Fabry disease and has been reported to clear some renal cell types of accumulated glycolipids and to reduce the accumulation in other cell types. We describe two patients without Fabry disease who were transplanted with kidney allografts from a male donor with Fabry disease. Biopsies were taken at transplantation and after 3 years in the first case and after 12 years in the second case. Even though these Fabry kidney allografts for many years had been exposed to normal levels of circulating α-galactosidase A (α-gal-A), the amount of accumulated lysosomal deposits in the podocytes remained unchanged. Additionally, small deposits were also found in tubular cells and glomerular endothelial cells as long as 12 years after transplantation.

Keywords: Fabry disease; enzyme replacement therapy; histological changes; kidney; transplantation.

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Figures

Fig. 1
Fig. 1
Recipient B—baseline biopsy. (a) Lipid deposits (arrows) in podocytes (toluidine blue, ×400) and (b) by electron microscopy (×5200).
Fig. 2
Fig. 2
Recipient B—biopsy taken 12 years after transplantation. Lipid deposits (arrows) in podocytes (toluidine blue, ×400) and by electron microscopy (×5200).
Fig. 3
Fig. 3
Recipient B—biopsy at 12 years after transplantation. (a) Tubular cell with lipid deposition (arrows) (toluidine blue, ×400). (b) Myelin-like substructure of lipid deposits in tubular cell (electron microscopy, ×7000).
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