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TRANSLATIONAL PHYSIOLOGY
Division of Pulmonary Medicine, Children's Hospital of Philadelphia, and University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104
Submitted 27 November 2002 ; accepted in final form 2 May 2003
Respiratory muscle weakness is common in children with neuromuscular disease (NMD). We hypothesized that weakness puts them at risk for respiratory muscle fatigue, a harbinger of chronic respiratory failure. We therefore measured a noninvasive index of respiratory muscle fatigue, the tension-time index of the respiratory muscles (TTmus), in 11 children with NMD and 13 control subjects. Spirometric flow rates and maximal inspiratory pressure were significantly lower in the NMD group than in controls (43 ± 23 vs. 99 ± 21 cmH2O, P < 0.001). The mean TTmus was significantly higher in the NMD group than in controls (0.205 ± 0.117 vs. 0.054 ± 0.021, P < 0.001). The increase in TTmus was primarily due to an increase in the ratio of average mean inspiratory pressure to maximal inspiratory pressure, indicating decreased respiratory muscle strength reserve. We found a significant correlation between TTmus and the residual volume-to-total lung capacity ratio (r = 0.504, P = 0.03) and a negative correlation between TTmus and forced expiratory volume in 1 s (r = -0.704, P < 0.001). In conclusion, children with NMD are prone to respiratory muscle fatigue. TTmus may be useful in assessing tolerance during weaning from mechanical ventilation, identifying impending respiratory failure, and aiding in the decision to institute therapies.
respiratory muscles; muscular dystrophy
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