Lung microhaemorrhage drives oxidative/inflammatory damage in α₁-antitrypsin deficiency.

ABSTRACT:

Background

Animal models using intratracheal instillation show that elastase, unopposed by α₁-antitrypsin (AAT), causes alveolar damage and haemorrhage associated with emphysematous changes. The aim of the present study was to characterise any relationship between alveolar haemorrhage and human AAT deficiency (AATD) using bronchoalveolar lavage (BAL) and lung explant samples from AATD subjects.

Methods

BAL samples (17 patients, 15 controls) were evaluated for free haem (iron protoporphyrin IX) and total iron concentrations. Alveolar macrophage activation patterns were assessed using RNA sequencing and validated in vitro using haem-stimulated, monocyte-derived macrophages. Lung explants (seven patients, four controls) were assessed for iron sequestration pro

SUBMITTER: Saltini C 

PROVIDER: S-EPMC10258718 | biostudies-literature | 2023 May

REPOSITORIES: biostudies-literature