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. 2005 Mar;60(3):226–228. doi: 10.1136/thx.2004.028340

Structural changes to airway smooth muscle in cystic fibrosis

S Hays 1, R Ferrando 1, R Carter 1, H Wong 1, P Woodruff 1
PMCID: PMC1747321  PMID: 15741440

Abstract

Background: Chronic airway obstruction is characteristic of cystic fibrosis (CF) but there are few studies of airway smooth muscle remodelling in CF.

Methods: Airway smooth muscle content and mean airway smooth muscle cell size were measured by applying design-based stereology to bronchoscopic biopsy specimens obtained from seven subjects with CF and 15 healthy controls.

Results: The smooth muscle content increased by 63% in subjects with CF (mean (SD) 0.173 (0.08) v 0.106 (0.042) mm3 smooth muscle/mm3 submucosa, mean difference –0.067; 95% CI –0.12 to –0.013, p = 0.017) but there was no increase in mean cell size (2705 (351) v 2654 (757) µm3, mean difference –51; 95% CI –687 to 585, p = 0.87).

Conclusions: These findings indicate hyperplasia of airway smooth muscle cells without hypertrophy and suggest that accumulation of airway smooth muscle cells may contribute to airway narrowing and bronchial hyperresponsiveness in CF.

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Selected References

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  1. Bedrossian C. W., Greenberg S. D., Singer D. B., Hansen J. J., Rosenberg H. S. The lung in cystic fibrosis. A quantitative study including prevalence of pathologic findings among different age groups. Hum Pathol. 1976 Mar;7(2):195–204. doi: 10.1016/s0046-8177(76)80023-8. [DOI] [PubMed] [Google Scholar]
  2. Claman D. M., Boushey H. A., Liu J., Wong H., Fahy J. V. Analysis of induced sputum to examine the effects of prednisone on airway inflammation in asthmatic subjects. J Allergy Clin Immunol. 1994 Nov;94(5):861–869. doi: 10.1016/0091-6749(94)90154-6. [DOI] [PubMed] [Google Scholar]
  3. Danel C., Erzurum S. C., McElvaney N. G., Crystal R. G. Quantitative assessment of the epithelial and inflammatory cell populations in large airways of normals and individuals with cystic fibrosis. Am J Respir Crit Care Med. 1996 Jan;153(1):362–368. doi: 10.1164/ajrccm.153.1.8542144. [DOI] [PubMed] [Google Scholar]
  4. Ferrando Ronald E., Nyengaard Jens R., Hays Steven R., Fahy John V., Woodruff Prescott G. Applying stereology to measure thickness of the basement membrane zone in bronchial biopsy specimens. J Allergy Clin Immunol. 2003 Dec;112(6):1243–1245. doi: 10.1016/j.jaci.2003.09.038. [DOI] [PubMed] [Google Scholar]
  5. Holgate S. T., Davies D. E., Lackie P. M., Wilson S. J., Puddicombe S. M., Lordan J. L. Epithelial-mesenchymal interactions in the pathogenesis of asthma. J Allergy Clin Immunol. 2000 Feb;105(2 Pt 1):193–204. doi: 10.1016/s0091-6749(00)90066-6. [DOI] [PubMed] [Google Scholar]
  6. James A. L., Paré P. D., Hogg J. C. The mechanics of airway narrowing in asthma. Am Rev Respir Dis. 1989 Jan;139(1):242–246. doi: 10.1164/ajrccm/139.1.242. [DOI] [PubMed] [Google Scholar]
  7. James A., Carroll N. Airway smooth muscle in health and disease; methods of measurement and relation to function. Eur Respir J. 2000 Apr;15(4):782–789. doi: 10.1034/j.1399-3003.2000.15d25.x. [DOI] [PubMed] [Google Scholar]
  8. Mitchell I., Corey M., Woenne R., Krastins I. R., Levison H. Bronchial hyperreactivity in cystic fibrosis and asthma. J Pediatr. 1978 Nov;93(5):744–748. doi: 10.1016/s0022-3476(78)81070-1. [DOI] [PubMed] [Google Scholar]
  9. Ormerod L. P., Thomson R. A., Anderson C. M., Stableforth D. E. Reversible airway obstruction in cystic fibrosis. Thorax. 1980 Oct;35(10):768–772. doi: 10.1136/thx.35.10.768. [DOI] [PMC free article] [PubMed] [Google Scholar]
  10. Tiddens H. A., Koopman L. P., Lambert R. K., Elliott W. M., Hop W. C., van der Mark T. W., de Boer W. J., de Jongste J. C. Cartilaginous airway wall dimensions and airway resistance in cystic fibrosis lungs. Eur Respir J. 2000 Apr;15(4):735–742. doi: 10.1034/j.1399-3003.2000.15d18.x. [DOI] [PubMed] [Google Scholar]
  11. Tomashefski J. F., Jr, Bruce M., Goldberg H. I., Dearborn D. G. Regional distribution of macroscopic lung disease in cystic fibrosis. Am Rev Respir Dis. 1986 Apr;133(4):535–540. doi: 10.1164/arrd.1986.133.4.535. [DOI] [PubMed] [Google Scholar]
  12. Van Asperen P., Mellis C. M., South R. T., Simpson S. J. Bronchial reactivity in cystic fibrosis with normal pulmonary function. Am J Dis Child. 1981 Sep;135(9):815–819. doi: 10.1001/archpedi.1981.02130330027009. [DOI] [PubMed] [Google Scholar]
  13. Woodruff Prescott G., Dolganov Gregory M., Ferrando Ronald E., Donnelly Samantha, Hays Steven R., Solberg Owen D., Carter Roderick, Wong Hofer H., Cadbury Peggy S., Fahy John V. Hyperplasia of smooth muscle in mild to moderate asthma without changes in cell size or gene expression. Am J Respir Crit Care Med. 2004 Jan 15;169(9):1001–1006. doi: 10.1164/rccm.200311-1529OC. [DOI] [PubMed] [Google Scholar]
  14. van Haren E. H., Lammers J. W., Festen J., van Herwaarden C. L. Bronchial vagal tone and responsiveness to histamine, exercise and bronchodilators in adult patients with cystic fibrosis. Eur Respir J. 1992 Oct;5(9):1083–1088. [PubMed] [Google Scholar]

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