Lung function in adult patients with osteogenesis imperfecta: a cohort study.

ABSTRACT:

Background

Osteogenesis imperfecta (OI) is a rare hereditary bone disease resulting from a defect in collagen synthesis or processing, leading to bone fragility, frequent fractures and skeletal deformities. OI is associated with increased respiratory morbidity and mortality, but the mechanisms of lung involvement are poorly understood, and there are no data on the natural history of lung function. We studied lung function over time in a cohort of adult OI patients at one center.

Methods

We used data from OI patients aged 15 and above followed up at the Lausanne university hospital between 2012 and 2023 with available pre-bronchodilator spirometry. Associations between spirometric measurements at first visit and clinical characteristics were studied through linear regression.

SUBMITTER: Lenoir A 

PROVIDER: S-EPMC11616175 | biostudies-literature | 2024 Dec

REPOSITORIES: biostudies-literature