Pediatric Radiology

, Volume 35, Issue 8, pp 739–757

Cystic fibrosis lung disease: genetic influences, microbial interactions, and radiological assessment

  • Samuel M. Moskowitz
  • Ronald L. Gibson
  • Eric L. Effmann
Review

DOI: 10.1007/s00247-005-1445-3

Cite this article as:
Moskowitz, S.M., Gibson, R.L. & Effmann, E.L. Pediatr Radiol (2005) 35: 739. doi:10.1007/s00247-005-1445-3

Abstract

Cystic fibrosis (CF) is a multiorgan disease caused by mutation of the CF transmembrane conductance regulator (CFTR) gene. Obstructive lung disease is the predominant cause of morbidity and mortality; thus, most efforts to improve outcomes are directed toward slowing or halting lung-disease progression. Current therapies, such as mucolytics, airway clearance techniques, bronchodilators, and antibiotics, aim to suppress airway inflammation and the processes that stimulate it, namely, retention and infection of mucus plaques at the airway surface. New approaches to therapy that aim to ameliorate specific CFTR mutations or mutational classes by restoring normal expression or function are being investigated. Because of its sensitivity in detecting changes associated with early airway obstruction and regional lung disease, high-resolution CT (HRCT) complements pulmonary function testing in defining disease natural history and measuring response to both conventional and experimental therapies. In this review, perspectives on the genetics and microbiology of CF provide a context for understanding the increasing importance of HRCT and other imaging techniques in assessing CF therapies.

Keywords

Cystic fibrosis Genetics Microbiology Current therapies Chest radiograph High-resolution CT Functional imaging 

Copyright information

© Springer-Verlag 2005

Authors and Affiliations

  • Samuel M. Moskowitz
    • 1
  • Ronald L. Gibson
    • 1
  • Eric L. Effmann
    • 2
  1. 1. Department of PediatricsUniversity of Washington School of Medicine and Children’s Hospital and Regional Medical CenterSeattleUSA
  2. 2.Department of RadiologyUniversity of Washington School of Medicine and Children’s Hospital and Regional Medical CenterSeattleUSA

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