Chest wall volumes, diaphragmatic mobility, and functional capacity in patients with mucopolysaccharidoses.

Disability and rehabilitation(2022)

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摘要
Lung function, respiratory muscle strength, functional capacity, diaphragm mobility, and quality of life are reduced in MPS compared with matched healthy subjects. was mainly reduced due to pulmonary and abdominal ribcage impairment. Implications for RehabilitationReduction in respiratory muscle strength, functional capacity, diaphragm excursion and low lung volumes were found in individuals with Mucopolysaccharidoses (MPS).Chest wall volumes and the upper chest wall compartmental volumes during quiet spontaneous breathing are reduced in MPS.Assessment and monitoring of the respiratory system for individuals with MPS should be performed periodically through standardized assessments to enable identification of changes and early intervention by rehabilitation protocols.This study may provide the necessary basis for carrying out respiratoty rehabilitation protocols that can improving chest wall mechanics with breathing exercise in this group.
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关键词
Optoelectronic plethysmography,diaphragm,disability evaluation,respiratory muscles,six-minute walking test,ultrasound
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