Lung function in adult patients with osteogenesis imperfecta: a cohort study
An increase of FEV<sub>1</sub> and FVC over time was observed in OI patients after adjustment for other variables, suggesting that the defective collagen synthesis may impact the pulmonary interstitium and lead to increased lung compliance and hyperinflation, in contrast to skeletal deformities, which reduce the thoracic volume. Lung function changes in OI thus result from the interplay of several mechanisms.
Authors
- Bérengère Aubry‐Rozier (ORCID: https://orcid.org/0000-0002-5732-9734)
- Elena González Rodríguez (ORCID: https://orcid.org/0000-0001-6512-6008)
- Célia Paquier
- Alexandra Lenoir (ORCID: https://orcid.org/0000-0003-2189-6507)
- Aline Brégou (ORCID: https://orcid.org/0000-0001-8501-2067)
- Romain Lazor (ORCID: https://orcid.org/0000-0002-2933-0754)
- Joëlle Tanniger
- Mohamed Faouzi (ORCID: https://orcid.org/0000-0003-2084-3235)
Institutions
- Centre Hospitalier Universitaire Vaudois (CH)
- LMU Klinikum (DE)
- Centre universitaire de médecine générale et santé publique, Lausanne (CH)
- Ludwig-Maximilians-Universität München (DE)
- University of Lausanne (CH)
Publication Details
- Journal
- Orphanet Journal of Rare Diseases
- Published
- 2024-12-04
- DOI
- https://doi.org/10.1186/s13023-024-03452-y
- Citations
- 1
- Primary Topic
- Connective tissue disorders research
- Type
- article
- Field-Weighted Citation Impact
- 0.55
Funders
- Université de Lausanne