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Rheumatology Advance Access originally published online on February 28, 2007
Rheumatology 2007 46(6):957-962; doi:10.1093/rheumatology/kel450
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© The Author 2007. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org

Muscle strength and body composition in adult women with Marfan syndrome

G. Percheron, G. Fayet1, T. Ningler2, J.-M. Le Parc2, S. Denot-Ledunois, M. Leroy1, B. Raffestin1 and G. Jondeau2

Département STAPS, UFR de Sciences, Université de Versailles Saint-Quentin 1Services d’Explorations Fonctionnelles and, 2Consultation Marfan, Hôpital Ambroise Paré, Assistance Publique des Hôpitaux de Paris, UFR Médicale Paris Ile-de-France Ouest, France

Correspondence to: G. Jondeau Consultation Marfan, Hôpital Bichat, 46 rue Henri Huchard, 75018 Paris, France. E-mail: guillaume.jondeau{at}bch.aphp.fr


  Abstract

Objective. The purpose of this study was to assess skeletal muscle function and body composition in a group of women with Marfan syndrome compared with matched controls.

Methods. The 21 women who were receiving follow-up for Marfan syndrome at our institution, were free of major cardiovascular disease, and consented to the study performed isokinetic and isometric knee extension and flexion maximal strength tests and had their body composition evaluated using dual-energy X-ray absorptiometry (DEXA). The same assessments were done in 19 matched controls.

Results. A significant decrease in lean leg mass with no change in total soft-tissue leg mass was noted in the patients compared with the controls. Peak torque values for the quadriceps and hamstring muscle groups were decreased in the patients, but only quadriceps strength was significantly reduced after normalization for lean leg mass.

Conclusion. The muscle strength reduction in Marfan patients was not fully explained by a decrease in lean leg mass, suggesting qualitative skeletal-muscle alterations related to abnormal fibrillin expression in muscle connective tissue.

KEY WORDS: Skeletal muscle, Marfan syndrome, Isokinetic muscle strength, Body composition

Submitted 13 November 2006; revised version accepted 15 December 2006.
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