This Article Full Text Full Text (PDF) Online Supplement All Versions of this Article: 200303-398OCv1 168/8/989    most recent Alert me when this article is cited Alert me if a correction is posted Services Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Pinet, C. Articles by Estenne, M. Search for Related Content PubMed PubMed Citation Articles by Pinet, C. Articles by Estenne, M.

Function and Bulk of Respiratory and Limb Muscles in Patients with Cystic Fibrosis

Departments of Chest Medicine, Radiology, Physiotherapy, and Intensive Care Medicine, Erasme University Hospital, Brussels, Belgium

Correspondence and requests for reprints should be addressed to Marc Estenne, M.D., Chest Service, Erasme University Hospital, 808, Route de Lennik, B-1070 Brussels, Belgium. 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 control subjects; 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 with cystic fibrosis. Twitch transdiaphragmatic pressure was 23% lower and twitch gastric pressure was 22% greater in patients with cystic fibrosis than in control subjects, but diaphragm mass and abdominal muscle thickness were similar in the two groups. For any given lean body mass and quadriceps cross-section, patients with cystic fibrosis had greater diaphragm mass and abdominal muscle thickness. Diaphragm mass had greater intersubject variability in patients with cystic fibrosis than in control subjects. 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 with cystic fibrosis.

Key Words: cystic fibrosis • diaphragm • inflammation • respiratory muscles • training

This article has been cited by other articles:

				V. Dufresne, C. Knoop, A. Van Muylem, A. Malfroot, M. Lamotte, C. Opdekamp, G. Deboeck, M. Cassart, B. Stallenberg, G. Casimir, et al. Effect of Systemic Inflammation on Inspiratory and Limb Muscle Strength and Bulk in Cystic Fibrosis Am. J. Respir. Crit. Care Med., July 15, 2009; 180(2): 153 - 158. [Abstract] [Full Text] [PDF]

				T. Troosters, D. Langer, B. Vrijsen, J. Segers, K. Wouters, W. Janssens, R. Gosselink, M. Decramer, and L. Dupont Skeletal muscle weakness, exercise tolerance and physical activity in adults with cystic fibrosis Eur. Respir. J., January 1, 2009; 33(1): 99 - 106. [Abstract] [Full Text] [PDF]

				P. J. Barry, D. F. Waterhouse, C. M. Reilly, T. J. McKenna, E. F. McKone, and C. G. Gallagher Androgens, Exercise Capacity, and Muscle Function in Cystic Fibrosis Chest, December 1, 2008; 134(6): 1258 - 1264. [Abstract] [Full Text] [PDF]

				W. D. Reid, E. L. Geddes, K. O'Brien, D. Brooks, and J. Crowe Effects of inspiratory muscle training in cystic fibrosis: a systematic review Clinical Rehabilitation, October 1, 2008; 22(10-11): 1003 - 1013. [Abstract] [PDF]

				F. Bellemare and A. Jeanneret Sex differences in thoracic adaptation to pulmonary hyperinflation in cystic fibrosis Eur. Respir. J., January 1, 2007; 29(1): 98 - 107. [Abstract] [Full Text] [PDF]

				M. E. Sahlberg, U. Svantesson, E. M. L. M. Thomas, and B. Strandvik Muscular Strength and Function in Patients With Cystic Fibrosis Chest, May 1, 2005; 127(5): 1587 - 1592. [Abstract] [Full Text] [PDF]

				C Pinet, P Scillia, M Cassart, M Lamotte, C Knoop, C Melot, and M Estenne Preferential reduction of quadriceps over respiratory muscle strength and bulk after lung transplantation for cystic fibrosis Thorax, September 1, 2004; 59(9): 783 - 789. [Abstract] [Full Text] [PDF]

				M. J. Tobin Sleep-Disordered Breathing, Control of Breathing, Respiratory Muscles, Pulmonary Function Testing in AJRCCM 2003 Am. J. Respir. Crit. Care Med., January 15, 2004; 169(2): 254 - 264. [Full Text] [PDF]

				M. J. Tobin Pediatrics, Surfactant, and Cystic Fibrosis in AJRCCM 2003 Am. J. Respir. Crit. Care Med., January 15, 2004; 169(2): 277 - 287. [Full Text] [PDF]

				F. Maltais Skeletal Muscles in Chronic Airflow Obstruction: Why Bother? Am. J. Respir. Crit. Care Med., October 15, 2003; 168(8): 916 - 917. [Full Text] [PDF]