Kidney transplantation and enzyme replacement therapy in patients with Fabry disease

Markus Cybulla, Christine Kurschat, Michael West, Kathy Nicholls, Joan Torras, Gere Sunder-Plassmann, Sandro Feriozzi

Research output: Contribution to journalReview articlepeer-review

16 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)645-651
Number of pages7
JournalJournal of Nephrology
Volume26
Issue number4
DOIs
Publication statusPublished - Jul 2013

ASJC Scopus Subject Areas

  • Nephrology

PubMed: MeSH publication types

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

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