Hepatic fibrosis: a manifestation of the liver disease evolution in patients with Ataxia-telangiectasia.

ABSTRACT:

Background

Ataxia-telangiectasia (A-T) is a DNA repair disorder characterized by changes in several organs and systems. Advances in clinical protocols have resulted in increased survival of A-T patients, however disease progression is evident, mainly through metabolic and liver changes.

Objective

To identify the frequency of significant hepatic fibrosis in A-T patients and to verify the association with metabolic alterations and degree of ataxia.

Methods

This is a cross-sectional study that included 25 A-T patients aged 5 to 31 years. Anthropometric data, liver, inflammatory, lipid metabolism and glucose biomarkers (oral glucose tolerance test with insulin curve-OGTT) were collected. The Cooperative Ataxia Rating Scale was applied to assess the degree of ataxia. The f

SUBMITTER: Barreto TLN 

PROVIDER: S-EPMC10161655 | biostudies-literature | 2023 May

REPOSITORIES: biostudies-literature