Lipid profile in adult patients with Fabry disease - Ten-year follow up.

ABSTRACT:

Background

Fabry disease, an X-linked genetic condition, results from alpha-galactosidase deficiency and increased accumulation of glycosphingolipids in cardiovascular tissues. Clinical manifestation includes vasculature associated complications. Hyperlipidaemia is one of the cardiovascular risk factors however it has never been well defined in Fabry disease. Enzyme Replacement Therapy (ERT) is available but its effect on serum cholesterol is unknown. The aim of this project was to assess the influence of long-term ERT on lipid profile in a large cohort of adult patients with Fabry disease.

Methods

This was a retrospective analysis of lipid profile results. Patients with Fabry disease were on ERT for 10 years, were not treated with statins and had no severe renal impairment.

SUBMITTER: Stepien KM 

PROVIDER: S-EPMC5510531 | biostudies-literature | 2017 Dec

REPOSITORIES: biostudies-literature