A multicenter study to evaluate pulmonary function in osteogenesis imperfecta.

ABSTRACT: Pulmonary complications are a significant cause for morbidity and mortality in osteogenesis imperfecta (OI). However, to date, there have been few studies that have systematically evaluated pulmonary function in individuals with OI. We analyzed spirometry measurements, including forced vital capacity (FVC) and forced expiratory volume in the first second (FEV₁ ), in a large cohort of individuals with OI (n = 217) enrolled in a multicenter, observational study. We show that individuals with the more severe form of the disease, OI type III, have significantly reduced FVC and FEV₁ which do not follow the expected trends of the normal population. We also show that "normalization" of FVC and FEV₁ using general population data to generate percent predicted values

SUBMITTER: Tam A 

PROVIDER: S-EPMC6235719 | biostudies-literature | 2018 Dec

REPOSITORIES: biostudies-literature