Vascular disease in male patients with Fabry disease on hemodialysis: a retrospective cohort study in Argentina

Abstract

Introduction: Vascular events (VE), both cardiovascular (CVD) and cerebrovascular (CVA), are the main cause of death in patients with Fabry disease (FD). The aim of this study was to describe the occurrence of VE in patients with FD and end-stage renal disease (ESRD) on hemodialysis during follow-up. Methods: a retrospective, cohort study was carried out at three centers in Argentina between January 2010 and January 2017. Hemodialysis patients with FD and ESRD were included. Information was collected regarding demographic, clinical and VE aspects. Results: Eight adult (40 ± 4.2 year-old) male patients with ESRD on hemodialysis (mean time on dialysis: 39.1 ± 20.6 months) were included; the mean followup time was 55 ± 12 months. Five patients received enzyme replacement therapy during follow-up. Four patients (50%) had VE during follow-up. In three of them the cardiovascular event was fatal. The mean time from admission to dialysis until the onset of VE was 38 ± 8 months. Conclusion: 50% of the patients with FD and ESRD presented a VE in a mean time of approximately 38 months since their admission to dialysis.

Keywords

• enfermedad de Fabry
• enfermedad renal terminal
• epidemiología