BBDC: Evaluating Lung Disease in Adults with Osteogenesis Imperfecta

Aug 30, 2026 · 1m 25s
BBDC: Evaluating Lung Disease in Adults with Osteogenesis Imperfecta
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New research from the Brittle Bone Disorders Consortium (BBDC). This summary is based on a paper published in the journal Chest in July 2026 titled "Evaluation of Lung Disease in...

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New research from the Brittle Bone Disorders Consortium (BBDC). This summary is based on a paper published in the journal Chest in July 2026 titled "Evaluation of Lung Disease in Adults With Osteogenesis Imperfecta." 

Read the paper here. 

Learn more about BBDC. 

Transcript: 

New research from the Brittle Bone Disorders Consortium (BBDC), a research group of the Rare Diseases Clinical Research Network.

Evaluating Lung Disease in Adults with Osteogenesis Imperfecta.

This summary is based on a paper published in the journal Chest in July 2026.

Osteogenesis imperfecta (OI) is a group of inherited connective tissue disorders associated with a wide range of symptoms, including fragile bones that break easily. Although cardiopulmonary impairment—when the heart and lungs are not functioning properly—is a leading cause of mortality in individuals with OI, not much is known about what causes these issues.

In this study, researchers evaluated cardiopulmonary outcomes and differences in pulmonary function among 60 adults with OI. The team tested for scoliosis, lung disease, lung restriction, and cardiac health.

Results showed a low association between pulmonary function and spinal curve magnitude, suggesting that respiratory impairment may result from OI rather than scoliosis. Authors note that more research is needed to better understand the cause of outcomes like bronchial wall thickening and bronchiectasis in OI. 
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Author RDCRN
Organization RDCRN
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