Clinical Features

by Felix C.

Describe respiratory symptoms.

Respiratory symptoms are common in adulthood. CF should be considered in individuals with the following features:

  • Chronic obstructive lung disease with bronchiectasis

  • Chronic sinusitis: nasal polyps may eventually develop

  • Recurrent or chronic productive cough and pulmonary infections

    • S. aureus is the most common cause of recurrent pulmonary infection in infancy and childhood.

    • P. aeruginosa is the most common cause of recurrent pulmonary infections in adulthood.

    • Other commonly involved bacteria

      • Burkholderia cepacia: can lead to cepacia syndrome, a severe necrotizing pneumonia that is often accompanied by rapid respiratory decline and can progress to sepsis [13]

      • S. pneumoniae

      • H. influenzae

    • Increased susceptibility of individuals with CF to opportunistic, potentially life-threatening pathogens (e.g., Pseudomonas aeruginosa, Aspergillus)

      • Infections with Pseudomonas aeruginosa → rapid decline in pulmonary function (patients with CF go through multiple antibiotic courses in their lifetime → increasing resistance of Pseudomonas aeruginosa to commonly used antibiotics) [14]

      • A chronic infection with Aspergillus species may lead to allergic bronchopulmonary aspergillosis (see “Complications” below)

  • Pulmonary obstruction and airway hyperreactivity may manifest with expiratory wheezing and/or dyspnea.

  • Barrel chest , moist rales (indicate pneumonia), hyperresonance to percussion

  • Hemoptysis

  • Signs of chronic respiratory insufficiency: digital clubbing associated with chronic hypoxia


Felix C.


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