HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

This Article Full Text (PDF) Correspondence: Submit a response Alert me when this article is cited Alert me when Correspondence are posted Alert me if a correction is posted Services Email this article to a friend 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 Haller, R. G. Articles by Lewis, S. F. Search for Related Content PubMed PubMed Citation Articles by Haller, R. G. Articles by Lewis, S. F.

Abnormal ventilation during exercise in McArdle's syndrome

Modulation by substrate availability

Departments of Neurology and Physiology, The Veterans Administration Medical Center and The University of Texas Health Science Center, Dallas, TX.

We evaluated ventilation during cycle exercise in four men lacking myophosphorylase. In submaximal exercise of similar relative intensity, ventilation was higher relative to oxygen uptake in McArdle patients than in normal men. The exercise ventilatory response returned to normal after glucose infusion, by fasting to increase free fatty acid availability or by combining fasting and submaximal exercise. Excessive ventilation in exercise was potentiated by inhibiting lipolysis with nicotinic acid. The excessive ventilatory effort and resultant respiratory alkalosis may contribute to exercise intolerance.

Address correspondence and reprint requests to Dr. Haller. Neurology Section (127), Veterans Administration Medical Center, 4500 S. Lancaster Road. Dallas, TX 75216.

Supported by the Veterans Administration, by a grant from the Muscular Dystrophy Association, and by a grant-in-aid from the American Heart Association with funds contributed in part by the Texas Affiliate and by National Heart, Lung and Blood Institute Grant HL-06296. Dr. Lewis was the recipient of a New Investigator Research Award HL26958.

Presented in part at the thirty-fifth annual meeting of the American Academy of Neurology, San Diego, CA, April 1983.

Accepted for publication September 27, 1985.

This article has been cited by other articles:

				S. T. Andersen, T. D. Jeppesen, T. Taivassalo, M.-L. Sveen, K. Heinicke, R. G. Haller, and J. Vissing Effect of Changes in Fat Availability on Exercise Capacity in McArdle Disease Arch Neurol, June 1, 2009; 66(6): 762 - 766. [Abstract] [Full Text] [PDF]

				M. C. Orngreen, H. J. Schelhaas, T. D. Jeppesen, H. O. Akman, R. A. Wevers, S. T. Andersen, H. J. ter Laak, O. P. van Diggelen, S. DiMauro, and J. Vissing Is muscle glycogenolysis impaired in X-linked phosphorylase b kinase deficiency? Neurology, May 13, 2008; 70(20): 1876 - 1882. [Abstract] [Full Text] [PDF]