Literature DB >> 14582554

Pulmonary function, working capacity and strength in young adults with Marfan syndrome.

Liv Giske1, Johan K Stanghelle, Svend Rand-Hendrikssen, Vegard Strøm, Jan-Erik Wilhelmsen, Cecilie Røe.   

Abstract

OBJECTIVE: To investigate pulmonary function, working capacity and isokinetic muscle strength in subjects with Marfan syndrome.
MATERIALS AND METHODS: Seventeen subjects, mean age 23 years, performed spirometry, maximal ergometer cycle tests and knee extension and flexion isokinetic torque tests.
RESULTS: The subjects with Marfan syndrome had increased total and residual lung volume values compared with predicted values in healthy subjects and reduced peak oxygen uptake compared with healthy subjects of the same age. The isokinetic peak torque was reduced only at the highest velocity in the women.
CONCLUSION: Thirteen subjects were able to perform the bicycle test until exhaustion without cardiovascular complications or pulmonary restrictions. However, their aerobic capacity was considerably decreased, which we suggest is caused by deconditioning. Further investigations are needed to reveal how pulmonary and cardiovascular limitations change with ageing and to give guidelines for exercise.

Entities:  

Mesh:

Year:  2003        PMID: 14582554     DOI: 10.1080/16501970306095

Source DB:  PubMed          Journal:  J Rehabil Med        ISSN: 1650-1977            Impact factor:   2.912


  7 in total

Review 1.  The Genetics of Pneumothorax.

Authors:  Philip M Boone; Rachel M Scott; Stefan J Marciniak; Elizabeth P Henske; Benjamin A Raby
Journal:  Am J Respir Crit Care Med       Date:  2019-06-01       Impact factor: 21.405

2.  The correlation between spinal and chest wall deformities and pulmonary function in Marfan syndrome.

Authors:  Hila Otremski; Roger F Widmann; Mary F Di Maio; Dror Ovadia
Journal:  J Child Orthop       Date:  2020-08-01       Impact factor: 1.548

Review 3.  Joint hypermobility as a distinctive feature in the differential diagnosis of myopathies.

Authors:  N C Voermans; C G Bonnemann; B C J Hamel; H Jungbluth; B G van Engelen
Journal:  J Neurol       Date:  2009-02-09       Impact factor: 4.849

4.  Lung Function Changes are More Common in Marfan Patients Who Need Major Thoracic Surgery.

Authors:  Abigel M Kolonics-Farkas; Bence Agg; Kalman Benke; Balazs Odler; Aniko Bohacs; Zsuzsanna Kovats; Zoltan Szabolcs; Veronika Müller
Journal:  Lung       Date:  2019-05-14       Impact factor: 2.584

Review 5.  Is physical activity a future therapy for patients with Marfan syndrome?

Authors:  Steeve Jouini; Olivier Milleron; Ludivine Eliahou; Guillaume Jondeau; Damien Vitiello
Journal:  Orphanet J Rare Dis       Date:  2022-02-10       Impact factor: 4.123

6.  Subjective assessment underestimates surgical risk: On the potential benefits of cardiopulmonary exercise testing for open thoracoabdominal repair.

Authors:  Damian M Bailey; Claire L Halligan; Richard G Davies; Anthony Funnell; Ian R Appadurai; George A Rose; Lara Rimmer; Matti Jubouri; Joseph S Coselli; Ian M Williams; Mohamad Bashir
Journal:  J Card Surg       Date:  2022-04-29       Impact factor: 1.778

7.  Early Impairment of Lung Mechanics in a Murine Model of Marfan Syndrome.

Authors:  Juan J Uriarte; Thayna Meirelles; Darya Gorbenko Del Blanco; Paula N Nonaka; Noelia Campillo; Elisabet Sarri; Daniel Navajas; Gustavo Egea; Ramon Farré
Journal:  PLoS One       Date:  2016-03-22       Impact factor: 3.240

  7 in total

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