A nationwide cross-sectional analysis of biopsy-proven Fabry nephropathy: the Japan Renal Biopsy Registry.

ABSTRACT:

Background

Fabry disease (FD) is an X-linked inherited disease where renal complications are associated with a poor prognosis. However, little is known about the prevalence of Fabry nephropathy (FN) in patients with chronic kidney disease (CKD). We extracted FN data from the Japan Renal Biopsy Registry, analyzed the prevalence of FN, and examined the correlation between clinical characteristics and renal involvement according to sex differences and hemi- and heterozygosity in patients with FD.

Methods

A total of 38,351 participants who underwent renal biopsy were retrospectively enrolled, and FN was determined. The clinical characteristics of FD patients were examined based on sex differences.

Results

Twenty-nine patients (0.076%) (19 males and 10 females, mean age: 4

SUBMITTER: Nasu M 

PROVIDER: S-EPMC9845163 | biostudies-literature | 2023 Feb

REPOSITORIES: biostudies-literature