Lumacaftor/ivacaftor changes the lung microbiome and metabolome in cystic fibrosis patients.

ABSTRACT:

Rationale

Targeted cystic fibrosis (CF) therapy with lumacaftor/ivacaftor partly restores chloride channel function and improves epithelial fluid transport in the airways. Consequently, changes may occur in the microbiome, which is adapted to CF lungs.

Objectives

To investigate the effects of lumacaftor/ivacaftor on respiratory microbial composition and microbial metabolic activity by repeatedly sampling the lower respiratory tract.

Methods

This was a single-centre longitudinal observational cohort study in adult CF patients with a homozygous Phe508del mutation. Lung function measurements and microbial cultures of sputum were performed as part of routine care. An oral and nasal wash, and a breath sample, were collected before and every 3 months after starting therapy,

SUBMITTER: Neerincx AH 

PROVIDER: S-EPMC8053817 | biostudies-literature | 2021 Apr

REPOSITORIES: biostudies-literature