Epidemiology and early predictors of Fabry nephropathy: evaluation of long-term outcomes from a national Fabry centre.

ABSTRACT:

Background

Fabry disease is a rare genetic lysosomal storage disorder, whereby the accumulation of sphingolipids consequently leads to kidney structural damage and dysfunction. We explored the epidemiology of chronic kidney disease (CKD) among patients with Fabry disease at a major UK referral centre in Greater Manchester serving over 7 million people, to inform early predictors of kidney disease and possible treatment planning.

Methods

Data were sourced from the electronic records of registered participants from November 2020 to February 2022 of adults diagnosed with Fabry disease, with at least 1 year of follow-up. Four hundred and five participants (female = 223, male = 182) met the initial eligibility criteria. Our study focused on identifying factors linked to incident

SUBMITTER: Mannan F 

PROVIDER: S-EPMC11961510 | biostudies-literature | 2025 Mar

REPOSITORIES: biostudies-literature