The lungs and airways in achondroplasia. Do little peopla have little lungs?

D. C. Stokes, M. E.B. Wohl, R. A. Wise, R. E. Pyeritz, D. L. Fairclough

Research output: Contribution to journalArticlepeer-review

36 Scopus citations

Abstract

Achondroplaia is a unique model of the effects of skeletal dysplasia and dwarfism on the respiratory system. We measured chest dimensions, spirometry, lung volumes, maximal expiratory flow volume curves, nasal and airways resistance, closing volume, maximal inspiratory/expiratory pressures, and tracheal area by acoustic reflection in 12 healthy subjects with achondroplasia. Anterio-posterior thoracic diameter was mildly reduced in men. Vital capacity for all subjects was 108 percent ± 18.6 percent (SD) of that predicted for achondroplatic subjects, but was reduced when compared with values for people of average stature that were predicted, based on either sitting height or thoracic height. The reduction was relatively greater in male than in female subjects. The RV/TLC and FRC/TLC ratios were normal. Other measurements were similar to those in average-statured adults. We conclude that achondroplasia results in a reduction in vital capacity out of proportion to what would be expected if these subjects had normal limb size. Although the lungs may be small, they are functionally normal, as are the airways.

Original languageEnglish (US)
Pages (from-to)145-152
Number of pages8
JournalCHEST
Volume98
Issue number1
DOIs
StatePublished - 1990
Externally publishedYes

ASJC Scopus subject areas

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

Fingerprint

Dive into the research topics of 'The lungs and airways in achondroplasia. Do little peopla have little lungs?'. Together they form a unique fingerprint.

Cite this