|
|
||||||||
Guest Access | Sign In via User Name/Password |
|||||||||
Chest, Vol 93, 364-369, Copyright © 1988 by American College of Chest Physicians
ARTICLES |
DC Stokes, RE Pyeritz, RA Wise, D Fairclough and EA Murphy
Department of Pediatrics, Johns Hopkins Medical Institutions, Baltimore, MD.
Standard values for pulmonary function in short-limbed dwarfism are not available. Therefore, chest diameters and expiratory spirograms were measured in 58 female and 44 male subjects between 7 and 60 years of age with achondroplasia, the most common form of dwarfism. Standing height in adults was 49.6 +/- 3.2 (SD) inches with a sitting/standing height ratio of 0.66 (normal 0.52-0.53). Despite extremely short stature, only AP chest diameters in males were smaller than control subjects of similar age. The following equations were derived for forced vital capacity (FVC): males (under 25 years), FVC(L) = -3.56 + 0.162 X sitting height (in) + 0.067 X age (yrs); males (over 25 years), FVC(L) = -0.73 + 0.162 X sitting height (in) -0.047 X age (yrs); females (under 20 years), FVC(L) = -3.56 + 0.150 X sitting height (in) + 0.067 X age (yrs); females (over 20 years), FVC(L) = -1.92 + 0.150 X sitting height (in) -0.016 X age (years). Similar prediction equations were derived for FEV1 and FEF25-75%: FEV1/FVC % was 84.2 (+/- 6.5) for females and 88.0 (+/- 6.5) for males. We also compared the observed FVC measurements to values calculated using standing heights derived from the subject's sitting height, assuming a normal body proportion. The observed vital capacity in achondroplasia was only 67.6 (+/- 19.2) percent of that predicted for normally proportioned females and 72.4 (+/- 13.6) percent for males, suggesting reduced vital capacity in achondroplasia, due to reduced chest wall compliance or abnormal lung growth.
This article has been cited by other articles:
![]() |
W. O. Collins and S. S. Choi Otolaryngologic Manifestations of Achondroplasia Arch Otolaryngol Head Neck Surg, March 1, 2007; 133(3): 237 - 244. [Abstract] [Full Text] [PDF] |
||||
![]() |
P. Harmatz, D. Ketteridge, R. Giugliani, N. Guffon, E. L. Teles, M. C. S. Miranda, Z.-F. Yu, S. J. Swiedler, J. J. Hopwood, and for the MPS VI Study Group Direct Comparison of Measures of Endurance, Mobility, and Joint Function During Enzyme-Replacement Therapy of Mucopolysaccharidosis VI (Maroteaux-Lamy Syndrome): Results After 48 Weeks in a Phase 2 Open-Label Clinical Study of Recombinant Human N-Acetylgalactosamine 4-Sulfatase Pediatrics, June 1, 2005; 115(6): e681 - e689. [Abstract] [Full Text] [PDF] |
||||
![]() |
R. C Tasker, I. Dundas, A. Laverty, M. Fletcher, R. Lane, and J. Stocks Distinct patterns of respiratory difficulty in young children with achondroplasia: a clinical, sleep, and lung function study Arch. Dis. Child., August 1, 1998; 79(2): 99 - 108. [Abstract] [Full Text] |
||||
| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |