The major pathologic or structural changes associated with cystic fibrosis include:

This site uses cookies to improve performance. If your browser does not accept cookies, you cannot view this site.

Setting Your Browser to Accept Cookies

There are many reasons why a cookie could not be set correctly. Below are the most common reasons:

  • You have cookies disabled in your browser. You need to reset your browser to accept cookies or to ask you if you want to accept cookies.
  • Your browser asks you whether you want to accept cookies and you declined. To accept cookies from this site, use the Back button and accept the cookie.
  • Your browser does not support cookies. Try a different browser if you suspect this.
  • The date on your computer is in the past. If your computer's clock shows a date before 1 Jan 1970, the browser will automatically forget the cookie. To fix this, set the correct time and date on your computer.
  • You have installed an application that monitors or blocks cookies from being set. You must disable the application while logging in or check with your system administrator.

Why Does this Site Require Cookies?

This site uses cookies to improve performance by remembering that you are logged in when you go from page to page. To provide access without cookies would require the site to create a new session for every page you visit, which slows the system down to an unacceptable level.

This site stores nothing other than an automatically generated session ID in the cookie; no other information is captured.

In general, only the information that you provide, or the choices you make while visiting a web site, can be stored in a cookie. For example, the site cannot determine your email name unless you choose to type it. Allowing a website to create a cookie does not give that or any other site access to the rest of your computer, and only the site that created the cookie can read it.

  • Journal List
  • Thorax
  • v.60(3); 2005 Mar
  • PMC1747321

Thorax. 2005 Mar; 60(3): 226–228.

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.

Full Text

The Full Text of this article is available as a PDF (71K).

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  • van Haren EH, Lammers JW, Festen J, van Herwaarden CL. 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]
  • Van Asperen P, Mellis CM, South RT, Simpson SJ. Bronchial reactivity in cystic fibrosis with normal pulmonary function. Am J Dis Child. 1981 Sep;135(9):815–819. [PubMed] [Google Scholar]
  • Tiddens HA, Koopman LP, Lambert RK, Elliott WM, Hop WC, van der Mark TW, de Boer WJ, de Jongste JC. Cartilaginous airway wall dimensions and airway resistance in cystic fibrosis lungs. Eur Respir J. 2000 Apr;15(4):735–742. [PubMed] [Google Scholar]
  • Tomashefski JF, Jr, Bruce M, Goldberg HI, Dearborn DG. Regional distribution of macroscopic lung disease in cystic fibrosis. Am Rev Respir Dis. 1986 Apr;133(4):535–540. [PubMed] [Google Scholar]
  • Claman DM, Boushey HA, Liu J, Wong H, Fahy JV. 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. [PubMed] [Google Scholar]
  • 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 May 1;169(9):1001–1006. [PubMed] [Google Scholar]
  • 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. [PubMed] [Google Scholar]
  • 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. [PubMed] [Google Scholar]
  • James AL, Paré PD, Hogg JC. The mechanics of airway narrowing in asthma. Am Rev Respir Dis. 1989 Jan;139(1):242–246. [PubMed] [Google Scholar]
  • Danel C, Erzurum SC, McElvaney NG, Crystal RG. 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. [PubMed] [Google Scholar]
  • Ormerod LP, Thomson RA, Anderson CM, Stableforth DE. Reversible airway obstruction in cystic fibrosis. Thorax. 1980 Oct;35(10):768–772. [PMC free article] [PubMed] [Google Scholar]
  • Mitchell I, Corey M, Woenne R, Krastins IR, Levison H. Bronchial hyperreactivity in cystic fibrosis and asthma. J Pediatr. 1978 Nov;93(5):744–748. [PubMed] [Google Scholar]
  • Bedrossian CW, Greenberg SD, Singer DB, Hansen JJ, Rosenberg HS. 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. [PubMed] [Google Scholar]
  • Holgate ST, Davies DE, Lackie PM, Wilson SJ, Puddicombe SM, Lordan JL. Epithelial-mesenchymal interactions in the pathogenesis of asthma. J Allergy Clin Immunol. 2000 Feb;105(2 Pt 1):193–204. [PubMed] [Google Scholar]


Articles from Thorax are provided here courtesy of BMJ Publishing Group


What are the major pathologic and structural changes associated with cystic fibrosis?

The anatomic alterations of the lungs associated with cystic fibrosis may result in both restrictive and obstructive lung characteristics, but excessive bronchial secretions, bronchial obstruction, and hyperinflation of the lungs are the predominant features of cystic fibrosis in the advanced stages.

What is the pathology of cystic fibrosis?

In cystic fibrosis, the airways fill with thick, sticky mucus, making it difficult to breathe. The thick mucus is also an ideal breeding ground for bacteria and fungi. Cystic fibrosis (CF) is an inherited disorder that causes severe damage to the lungs, digestive system and other organs in the body.

How can the genetic mutation found with cystic fibrosis be characterized?

General Discussion. Cystic fibrosis is a genetic disorder that often affects multiple organ systems of the body. Cystic fibrosis is characterized by abnormalities affecting certain glands (exocrine) of the body especially those that produce mucus.

Which of the following would you expect to find in a patient with cystic fibrosis?

Symptoms of CF Frequent lung infections including pneumonia or bronchitis. Wheezing or shortness of breath. Poor growth or weight gain in spite of a good appetite. Frequent greasy, bulky stools or difficulty with bowel movements.