Article
Clinical implications of maximal respiratory pressure determinations for individuals with duchenne muscular dystrophy,☆☆

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Abstract

Objective: To analyze the relationship between disease progression, pulmonary volumes, respiratory muscle strength (maximum inspiratory [MIP] and expiratory [MEP] pressures), and arterial blood gases for patients with Duchenne muscular dystrophy (DMD).

Design: An inception cohort study of pulmonary volumes, MIPs, and MEPs, correlated with age and Paco2 levels and with each other using linear and nonlinear regression analyses.

Setting: Outpatient clinic.

Patients: Fifty-two consecutive DMD patients who presented for regular evaluations at a regional DMD center.

Results: Maximum expiratory pressures were 47.7% ± 10.9% of normal in the 16 7- to 14-year-old patients and decreased linearly thereafter (MEP% = −2.7 X age +73.8; p < .001). Declines in MEP also correlated linearly with expiratory reserve volume (p < .001) and inversely with residual volume (p < .001). By contrast, MIP was 66.3% ± 19.0% in the 35 7- to 14-year-old patients and then declined to 30.2% ± 19.5% after age 14. No linear relationships were found with age but declines did correlate linearly with inspiratory reserve volume (p < .001) and total lung capacity (p < .001). Paco2 elevations correlated best with decreases in MIP (p < .0001) and appeared when MIP was below 30cmH2O.

Conclusions: Lung volume changes in DMD patients correlate with respiratory muscle weakness, and although inspiratory muscle dysfunction plays a key role in the development of chronic ventilatory insufficiency, reductions in expiratory muscle strength are the first signs of dysfunction and lead to the first episodes of respiratory failure.

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    This work was performed at the Institut Duchenne de Boulogne, Poitiers, France.

    ☆☆

    No commercial party having a direct or indirect interest in the subject matter of this article has or will confer a benefit upon the authors or upon any organization with which the authors are associated.

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