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Kidney transplantation in patients with Fabry disease.

Abstract
Little is known about the effects of enzyme replacement therapy (ERT) in kidney transplant recipients with Fabry disease. Clinical characteristics of transplant recipients in the Fabry Outcome Survey (FOS) were therefore examined in patients with Fabry disease with or without ERT. Of the 837 European patients in FOS (March 2006), 34 male patients and two female patients had received kidney transplants. Mean age at transplantation was 37.6 +/- 10.9 years, mean time since transplantation was 7.7 +/- 6.4 years, median estimated glomerular filtration rate (eGFR) was 44.4 ml/min/1.73 m(2), and median proteinuria was 296 mg/24 h. Of 27 patients with baseline data, 59% had hypertension, 74% had left ventricular hypertrophy, 22% had cardiac valve disease, 30% had arrhythmia, and 22% had transient ischaemic attacks and 15% stroke. Twenty patients (74%; two female patients, 18 male patients) were receiving ERT with agalsidase alfa. At enrollment or at the start of ERT, median eGFRs were 59 and 35 ml/min/1.73 m(2) (P = 0.05) and median proteinuria levels were 240 and 420 mg/24 h (not significant) in treated and untreated patients respectively. Renal function remained stable in patients receiving ERT. In conclusion, agalsidase alfa is well tolerated in patients with Fabry disease who have undergone renal transplantation.
AuthorsMarkus Cybulla, Kerstin Nanette Walter, Andreas Schwarting, Raffaelle Divito, Sandro Feriozzi, Gere Sunder-Plassmann, European FOS Investigators Group
JournalTransplant international : official journal of the European Society for Organ Transplantation (Transpl Int) Vol. 22 Issue 4 Pg. 475-81 (Apr 2009) ISSN: 0934-0874 [Print] Switzerland
PMID19207191 (Publication Type: Comparative Study, Journal Article, Research Support, Non-U.S. Gov't)
Chemical References
  • Isoenzymes
  • Recombinant Proteins
  • agalsidase alfa
  • alpha-Galactosidase
Topics
  • Adolescent
  • Adult
  • Comorbidity
  • Fabry Disease (complications, drug therapy)
  • Female
  • Humans
  • Isoenzymes (therapeutic use)
  • Kidney Failure, Chronic (epidemiology, etiology, surgery)
  • Kidney Function Tests
  • Kidney Transplantation
  • Male
  • Middle Aged
  • Recombinant Proteins
  • Young Adult
  • alpha-Galactosidase (therapeutic use)

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