Cluster analysis of kidney function decline among males with Fabry disease in a large United States electronic health records database.

ABSTRACT:

Background

Fabry disease (FD) is an X-linked lysosomal storage disorder caused by deficient α-galactosidase A activity. The spectrum of disease includes phenotypes ranging from "classic" to "later-onset," with varying kidney disease progression. Identifying patterns of declining kidney function and involvement of other major organs in patients with FD is important to guide therapy decisions.

Methods

Clusters of patients with FD and similar estimated glomerular filtration rate (eGFR) decline and age were created using agglomerative clustering of data captured between 2007 and 2020 in the United States Optum Market Clarity database. Male patients with a diagnosis of FD and two or more eGFR values ≥6 months apart were included. Disease progression was compared with a control co

SUBMITTER: Chiorean A 

PROVIDER: S-EPMC10539200 | biostudies-literature | 2023 Sep

REPOSITORIES: biostudies-literature