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Published ahead of print on June 26, 2003, doi:10.1164/rccm.200303-398OC

Am. J. Respir. Crit. Care Med., Volume 168, Number 8, October 2003, 989-994

A more recent version of this article appeared on October 15, 2003
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Submitted on April 14, 2003
Accepted on June 17, 2003

Function and bulk of respiratory and limb muscles in cystic fibrosis

Christophe Pinet1, Marie Cassart2, Pietro Scillia2, Michel Lamotte3, Christiane Knoop1, Georges Casimir1, Christian Melot4, and Marc Estenne1*

1 Chest Medicine, Erasme University Hospital, Brussels, Belgium, 2 Radiology, Erasme University Hospital, Brussels, Belgium, 3 Physiotherapy, Erasme University Hospital, Brussels, Belgium, 4 Intensive Care, Erasme University Hospital, Brussels, Belgium

* To whom correspondence should be addressed. E-mail: mestenne{at}ulb.ac.be.

Inspiratory muscle weakness due to lung hyperinflation and muscle wasting may occur in cystic fibrosis. We therefore measured diaphragm function and bulk in 18 stable patients with cystic fibrosis and 15 matched controls; the abdominal and quadriceps muscles were studied for comparison. We assessed diaphragm mass, abdominal muscle thickness, twitch transdiaphragmatic and gastric pressures, quadriceps cross-section and isokinetic strength, and lean body mass. Lean body mass, quadriceps strength and quadriceps cross-section were lower in patients. Twitch transdiaphragmatic pressure was 23% lower and twitch gastric pressure 22% greater in patients than in controls, but diaphragm mass and abdominal muscle thickness were similar in two groups. For any given lean body mass and quadriceps cross-section, patients had greater diaphragm mass and abdominal muscle thickness. Diaphragm mass had a greater inter-subject variability in patients than in controls. We conclude that diaphragm strength is decreased but abdominal muscle strength is increased in patients with cystic fibrosis. Diaphragm and abdominal muscle bulk are not affected by the general muscle wasting, which suggests that there may be a training effect of cystic fibrosis on respiratory muscles. However, the variability of diaphragm mass indicates that this beneficial response does not occur in all patients.
Key words: cystic fibrosis, respiratory muscles, diaphragm, training, inflammation




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