Kidney transplantation and enzyme replacement therapy in patients with Fabry disease.
J Nephrol. 2013 Jul-Aug; 26(4):645-51.JN

Abstract

During Fabry disease, progressive glycosphingolipid deposition in the kidney causes gradual deterioration of renal function with proteinuria, uremia and hypertension. This results in end-stage renal disease (ESRD) which is one of the leading causes of morbidity and premature mortality in affected patients. Given the excellent graft and patient survival generally nowadays, kidney transplantation is the first choice to correct renal dysfunction and improve the overall prognosis of patients with renal failure because of Fabry disease. The benefit of enzyme-replacement therapy (ERT) in kidney transplanted Fabry patients has been controversially discussed and long-term trials focusing on the effectiveness of agalsidase in this patient population are needed.

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Authors+Show Affiliations

Cybulla M
FGM, Center of Internal Medicine, Department of Nephrology and Rheumatology, Müllheim, Germany.
Kurschat C
No affiliation info available
West M
No affiliation info available
Nicholls K
No affiliation info available
Torras J
No affiliation info available
Sunder-Plassmann G
No affiliation info available
Feriozzi S
No affiliation info available

MeSH

Disease ProgressionEnzyme Replacement TherapyFabry DiseaseHumansIsoenzymesKidney Failure, ChronicKidney Transplantationalpha-Galactosidase

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't
Review

Language

eng

PubMed ID

23023720