Paediatric Orthopaedic Unit

Dec 3, 2024 | link.springer.com | Gonzalez Rodriguez |Paediatric Orthopaedic Unit

AbstractOsteogenesis 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.