Pulmonary function, working capacity and strength in young adults with Marfan syndrome.
OBJECTIVE
To investigate pulmonary function, working capacity and isokinetic muscle strength in subjects with Marfan syndrome.


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.


CONCLUSIONS
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.
National Institute of Occupational Health, Oslo, Norway.
Liv
L
Giske
Johan K
JK
Stanghelle
Svend
S
Rand-Hendrikssen
Vegard
V
Strøm
Jan-Erik
JE
Wilhelmsen
Cecilie
C
Røe
D000328:Adult; D005080:Exercise Test; D017079:Exercise Tolerance / Q000502:physiology*; D005260:Female; D006801:Humans; D007537:Isometric Contraction / Q000502:physiology; D008297:Male; D008382:Marfan Syndrome / Q000378:metabolism / Q000503:physiopathology*; D018482:Muscle, Skeletal / Q000378:metabolism / Q000503:physiopathology*; D010101:Oxygen Consumption / Q000502:physiology; D012129:Respiratory Function Tests / Q000379:methods; D012143:Respiratory Physiological Phenomena