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Arch Phys Med Rehabil. 1997 Jan;78(1):1-6.

Clinical implications of maximal respiratory pressure determinations for individuals with Duchenne muscular dystrophy.

Author information

1
Department of Neuropediatrics, University of Kiel, Germany.

Abstract

OBJECTIVE:

To analyze the relationship between disease progression, pulmonary volumes, respiratory muscle strength (maximum inspiratory [MIP] and expiratory [MEP] pressure), 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 167- 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 357- 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.

PMID:
9014949
DOI:
10.1016/s0003-9993(97)90001-0
[Indexed for MEDLINE]

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