Sensitive structural and functional measurements and 1-year pulmonary outcomes in pediatric cystic fibrosis.

ABSTRACT:

Background

Two functional measurements (multiple breath washout [MBW] and hyperpolarized ¹²⁹Xe ventilation magnetic resonance imaging [¹²⁹Xe MRI]) have been shown to be more sensitive to cystic fibrosis (CF) lung obstruction than traditional spirometry. However, functional techniques may be sensitive to different underlying structural abnormalities. The purpose of this study was to determine relationships between these functional markers, their pathophysiology, and 1-year clinical outcomes.

Methods

Spirometry, MBW, ¹²⁹Xe MRI, and ultrashort echo-time (UTE) MRI were obtained in a same-day assessment of 27 pediatric CF patients (ages 11.5±5.0) who had not begun CFTR modulator therapies. UTE MRI was scored for structural abnormalities and functi

SUBMITTER: Willmering MM 

PROVIDER: S-EPMC8175447 | biostudies-literature | 2021 May

REPOSITORIES: biostudies-literature