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Emília C. Martinez, Claudio Santili, Vivian B. Xavier, Marilia S. Leão, Glauber Alvarenga, Vera Lúcia Alves

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Purpose: Life expectancy in osteogenesis imperfecta (OI) can vary depending on the degree of compromise, and can lead to death in childhood or even at birth in severe cases. In the literature, the most cited causes of death in OI patients are pulmonary compromise and accidental trauma. The objective of this study was to assess pulmonary function of OI patients using spirometry. Methods: A total of 43 patients diagnosed with OI, aged 10 or older, were assessed by spirometry to collect FVC, FEV1 and FEV1/FVC ratio; predicted for age, height and gender. Results: Behavior on spirometry observed in this study diverged greatly from expected patterns, where patients with the most severe form of the disease and greater deformities had higher values of FVC and FEV1 than predicted values. In order to minimize interpretation errors, especially overestimation of values, it is suggested that only raw values in liters be used, without comparison using a prediction equation, since this prediction is performed in anthropometrically similar individuals, whereas the population studied was highly heterogeneous in these aspects. Conclusions: In conclusion the assessment of pulmonary function in individuals with OI revealed higher mean absolute spirometry values in individuals with the type I form.


Brittle Bone Disease; Lobstein Disease; Osteogenesis Imperfecta; Pulmonary Function Test; Spirometry.

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