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Context. – Fabry disease is a rare X-linked genetic disease due to pathogenic variants in the GLA gene. Classic Fabry disease is characterized by glycosphingolipids accumulation in all organs including the kidney, resulting in end-stage renal disease in a subset of male patients. Fabry disease should therefore be considered in the differential diagnosis of patients with unexplained end-stage renal disease.Objective. – We performed a prospective screening study in Western France to determine the prevalence of Fabry disease in a large population of dialyzed and transplanted patients.Patients and methods. – Patients meeting the inclusion criteria (males, 18-70 years with end-stage renal disease of unknown or vascular origin) were selected from the REIN1 registry and the CRISTAL1 database. Screening on filter papers was performed after patient consent was obtained during either a dialysis session or a transplantation follow-up visit.Results. – One thousand five hundred and sixty-one end-stage renal disease male patients were screened and 819 consented (dialysis: n = 242; transplant: n = 577). One single patient was found with decreased alpha-galactosidase levels Conclusion. – The prevalence of Fabry disease in end-stage renal disease patients was 0.12%, questioning the efficacy of this screening strategy with respect to the low prevalence. However, beside the benefit for the patient and his family, the increased awareness of Fabry disease among participating nephrologists may be of interest for future patients.