Respiratory function of children and adolescents with osteogenesis imperfecta: respiratory muscle strength, forced vital capacity, and peak expiratory flow

Abstract

Objective: This study aims to evaluate the respiratory function of children and adolescents with osteogenesis imperfecta (OI) followed up at a referral center. Methods: A cross-sectional study was conducted with a non-probabilistic sample. Manovacuometry was performed with the measurement of maximal inspiratory pressure (MIP) and maximal expiratory pressure (MEP), and in addition, peak expiratory flow (PEF) and ventilometry were performed to measure forced vital capacity (FVC). Results: In total, 23 individuals were evaluated, with a mean age of 11.6±3.4 years, 56.5% of whom were females. Regarding the classification of OI, 56.5% of the sample belonged to type IV, 30.5% to type III, and 13% to type I. The mean MIP was 64.4% of the predicted, and the mean MEP was 56.2% of the predicted. Overall, the mean PEF was 213.9 L/min, but only 140.6 L/min in the OI type III group. Median FVC was 1.9 L, corresponding to 110% of the predicted. Conclusions: Respiratory function of the study subjects was altered, with respiratory muscle strength values lower than expected in the whole sample, and peak expiratory flow was significantly reduced in the OI type III group.

Effects of home-based remote rehabilitation on children and adolescents with cystic fibrosis and non-cystic fibrosis bronchiectasis: a systematic review protocol

“…20 In children and adolescents with chronic lung diseases, telehealth interventions may contribute to improvements in HRQoL, treatment adherence, and better control of symptoms. 10 Consequently, they could also lead to better self-management of chronic lung diseases and prevention of exacerbations, thus preventing a decline in lung function. 1 Recently, a systematic review of seven RCTs reported the effectiveness of home-based rehabilitation on pulmonary function, functional capacity, and HRQoL in children and adolescents (6 to 20 years) with CF, emphasizing that home-based rehabilitation may be an alternative way of rehabilitation when conventional center-based programs are not able to be conducted for several reasons.…”

“…9 Especially, using new technology in telehealth seems to be an opportunity for improving clinical outcomes such as symptom control, adherence to treatment, reduction of hospitalizations, and HRQoL in children and adolescents with chronic lung diseases. 10 Home-based remote rehabilitation combines the use of new technology and the home environment in the context of rehabilitation. When circumstances require the restriction of transports (e.g.…”