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Review ArticleNarrative Review

Cystic Fibrosis Lung Disease: An Overview

Nelson L Turcios
Respiratory Care February 2020, 65 (2) 233-251; DOI: https://doi.org/10.4187/respcare.06697
Nelson L Turcios
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Abstract

Although better insights into the natural course of cystic fibrosis (CF) have led to treatment approaches that have improved pulmonary health and increased the life expectancy of individuals with this disorder, lung disease remains the main cause of morbidity and mortality in patients with CF. Evidence suggests that airway epithelial defects in ions-water transport lead to dehydrated mucus, impaired mucus clearance, and mucus adhesion to airway surfaces. An increase in mucin secretion is also suggested by the formation of endobronchial mucus plaques and plugs, which become the main sites of air flow obstruction, infection, and inflammation conducing to early small airways disease followed by the development of bronchiectasis. The lung involvement is usually progressive with intermittent exacerbations. Aggressive management and advances in treatment delay, but, do not prevent progression of lung disease. Respiratory failure ensues and is the major cause of death. The lung parenchyma is virtually untouched for much of the course of the disease. This review focuses on the lung involvement in cystic fibrosis and summarizes new developments on the diagnostic approach of CF and pathogenesis of related lung disease. Current therapeutic modalities, novel therapies targeting the basic genetic defect, and lung transplantation are also reviewed.

  • cystic fibrosis
  • cystic fibrosis transmembrane regulator
  • airway surface liquid
  • mucociliary clearance
  • endobronchial infection and inflammation
  • Pseudomonas aeruginosa
  • bronchiectasis

Footnotes

  • Correspondence: Nelson L Turcios MD, Medical Staff Office, Robert Wood Johnson University Hospital, One Robert Wood Johnson Place, New Brunswick, NJ 08901. E-mail: nlturcios{at}gmail.com.
  • The author has disclosed no conflicts of interest.

  • Copyright © 2020 by Daedalus Enterprises
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Respiratory Care: 65 (2)
Respiratory Care
Vol. 65, Issue 2
1 Feb 2020
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Cystic Fibrosis Lung Disease: An Overview
Nelson L Turcios
Respiratory Care Feb 2020, 65 (2) 233-251; DOI: 10.4187/respcare.06697

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Cystic Fibrosis Lung Disease: An Overview
Nelson L Turcios
Respiratory Care Feb 2020, 65 (2) 233-251; DOI: 10.4187/respcare.06697
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  • Article
    • Abstract
    • Introduction
    • Clinical Manifestations
    • Establishing the Diagnosis
    • Cystic Fibrosis Genotype/Phenotype
    • Pathogenesis of CF Lung Disease
    • Endobronchial Infections
    • Emerging Bacterial Infections
    • Fungal Infections
    • Pulmonary Exacerbations
    • Airway Clearance and Maintenance Medications
    • Pulmonary Emergencies in Cystic Fibrosis
    • Diagnostic Imaging
    • Pulmonary Function Testing
    • Infection Control
    • Lung Transplantation
    • New Treatment Strategies
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Keywords

  • cystic fibrosis
  • cystic fibrosis transmembrane regulator
  • airway surface liquid
  • mucociliary clearance
  • endobronchial infection and inflammation
  • Pseudomonas aeruginosa
  • bronchiectasis

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