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Respiratory manifestations

Authoring team

Respiratory manifestations include:

  • bronchiectasis with progressive productive cough and green/brown sputum – multiple chest infections
  • pneumothorax
  • Aspergillus fumigatus and allergic bronchopulmonary aspergillosis (ABPA) in up to 20%
  • colonisation of the lungs by Pseudomonas, which is often a difficult problem
  • nasal polyposis
  • pulmonary fibrosis may eventually lead to death from cor pulmonale or ventilatory failure.

Reference:

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