Natural History and Treatment of Uremia Secondary to Fabry’s Disease: An European Experience

Vol. 46, No. 4, 1987

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Original Paper

Natural History and Treatment of Uremia Secondary to Fabry's Disease: An European Experience
Donato Donati, Raffaele Novario, Luigi Gastaldi

Department of Nephrology, General Hospital, Varese, Italy

Address of Corresponding Author

Nephron 1987;46:353-359 (DOI: 10.1159/000184389)

Key Words

Cardiac complications
Cerebral complications
Fabry's disease
Hemodialysis
Infectious complications
Renal transplantion, survival

Abstract

Fabry's disease, a very unusual cause of end-stage renal disease, is actually included in the contraindications to renal transplantation. The few published studies, based on very few patients, report a high rate of life-threatening complications, mostly infectious, following kidney transplant in these patients. We have evalued retrospectively 12 uremic patients in renal function replacement treatment for Fabry's disease. In transplanted patients (n = 8; whole observation period 241 months), no lethal complication was ever recorded. Fever and acroparesthesias ameliorated. Cardio- and cerebrovascular complications did not progress. Although no increase in serum enzymatic activity was measured, renal transplantation provided an alternative route by excretion of an amount of the metabolic product (ceramide-trihexoside). When undergoing maintenance hemodialysis (whole observation period 291 months), 3 deaths and several cardiac and cerebral complications occurred. Angiokeratomas, fever and pains were unmodified. These data disagree with what has been previously stated. The transplanted patients' survival shifts towards the all-time rate and a satisfying rehabilitation is provided. Fabry's disease should not be considered a high-risk disease; and patients suffering from it should decidedly enter a regular transplantation program.

Author Contacts

Dr. Donato Donati, MD, Divisione di Nefrologia, Ospedale Multizonale - USSL 3, V. le L. Borri, 57, I-21100 Varese (Italy)

Article Information

Accepted: December 1, 1986
Published online: December 05, 2008
Number of Print Pages : 7

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