Kidney transplantation in patients with Fabry disease
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Kidney transplantation in patients with Fabry disease. / Cybulla, Markus; Walter, Kerstin Nanette; Schwarting, Andreas; Divito, Raffaelle; Feriozzi, Sandro; Sunder-Plassmann, Gere; European FOS Investigators Group.
In: TRANSPL INT, Vol. 22, No. 4, 4, 04.2009, p. 475-481.Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review
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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 - European FOS Investigators Group
AU - Gal, Andreas
AU - Muschol, Nicole Maria
PY - 2009/4
Y1 - 2009/4
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.
AB - 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.
KW - Adolescent
KW - Adult
KW - Comorbidity
KW - Fabry Disease/complications
KW - Female
KW - Humans
KW - Isoenzymes/therapeutic use
KW - Kidney Failure, Chronic/epidemiology
KW - Kidney Function Tests
KW - Kidney Transplantation
KW - Male
KW - Middle Aged
KW - Recombinant Proteins
KW - Young Adult
KW - alpha-Galactosidase/therapeutic use
U2 - 10.1111/j.1432-2277.2008.00824.x
DO - 10.1111/j.1432-2277.2008.00824.x
M3 - SCORING: Journal article
C2 - 19207191
VL - 22
SP - 475
EP - 481
JO - TRANSPL INT
JF - TRANSPL INT
SN - 0934-0874
IS - 4
M1 - 4
ER -
