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Kidney transplantation in patients with Fabry disease.
Transpl Int. 2009 Apr; 22(4):475-81.TI

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.

Authors+Show Affiliations

Department of Nephrology, University Hospital of Freiburg, Germany. markus.cybulla@uniklinik-freiburg.deNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

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

Language

eng

PubMed ID

19207191

Citation

Cybulla, Markus, et al. "Kidney Transplantation in Patients With Fabry Disease." Transplant International : Official Journal of the European Society for Organ Transplantation, vol. 22, no. 4, 2009, pp. 475-81.
Cybulla M, Walter KN, Schwarting A, et al. Kidney transplantation in patients with Fabry disease. Transpl Int. 2009;22(4):475-81.
Cybulla, M., Walter, K. N., Schwarting, A., Divito, R., Feriozzi, S., & Sunder-Plassmann, G. (2009). Kidney transplantation in patients with Fabry disease. Transplant International : Official Journal of the European Society for Organ Transplantation, 22(4), 475-81. https://doi.org/10.1111/j.1432-2277.2008.00824.x
Cybulla M, et al. Kidney Transplantation in Patients With Fabry Disease. Transpl Int. 2009;22(4):475-81. PubMed PMID: 19207191.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Kidney transplantation in patients with Fabry disease. AU - Cybulla,Markus, AU - Walter,Kerstin Nanette, AU - Schwarting,Andreas, AU - Divito,Raffaelle, AU - Feriozzi,Sandro, AU - Sunder-Plassmann,Gere, AU - ,, Y1 - 2009/01/22/ PY - 2009/2/12/entrez PY - 2009/2/12/pubmed PY - 2009/5/20/medline SP - 475 EP - 81 JF - Transplant international : official journal of the European Society for Organ Transplantation JO - Transpl. Int. VL - 22 IS - 4 N2 - 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. SN - 0934-0874 UR - https://www.unboundmedicine.com/medline/citation/19207191/Kidney_transplantation_in_patients_with_Fabry_disease_ L2 - https://doi.org/10.1111/j.1432-2277.2008.00824.x DB - PRIME DP - Unbound Medicine ER -