Spirometry and chest wall dimensions in achondroplasia

D. C. Stokes, R. E. Pyeritz, R. A. Wise, D. Fairclough, E. A. Murphy

Research output: Contribution to journalArticle

Abstract

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.

Original languageEnglish (US)
Pages (from-to)364-369
Number of pages6
JournalCHEST
Volume93
Issue number2
DOIs
StatePublished - Jan 1 1988

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine
  • Critical Care and Intensive Care Medicine
  • Cardiology and Cardiovascular Medicine

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    Stokes, D. C., Pyeritz, R. E., Wise, R. A., Fairclough, D., & Murphy, E. A. (1988). Spirometry and chest wall dimensions in achondroplasia. CHEST, 93(2), 364-369. https://doi.org/10.1378/chest.93.2.364