pulmonary cystic fibrosis
Tuesday 24 October 2006
Cystic fibrosis (CF) results in chronic pulmonary disease in the majority of patients resulting from the production of viscous secretions and impairment of mucociliary clearance due to alterations in airway surface liquid.
Chronic infection results, and the combination of tissue damage by pathogenic organisms and a robust host inflammatory response leads to the development of bronchiectasis and progressive lung disease.
Cystic fibrosis (CF) lung disease involves chronic bacterial infection of retained airway secretions (mucus). CF lung disease pathogenesis reflects the vulnerability of airway surfaces to dehydration and collapse of mucus clearance.
This predisposition is caused by mutations in the CF transmembrane conductance regulator (CFTR) gene, resulting in :
(i) the absence of CFTR-mediated Cl- secretion and regulation of epithelial Na+ channel (ENaC) function;
(ii) the sole dependence on extracellular ATP to rebalance these ion transport processes through P2 purinoceptor signaling.
mucus plugging of the tracheobronchial glands
- hyperplasia of bronchial glands
- papillary hyperplasia of the bronchial mucosa
- bronchial obstruction
- bronchial infections
- chronic bronchitis
- peribronchiolar fibrosis
- pulmonary interstitial inflammation
- pulmonary interstitial finrosis
- bronchial ulceration
- destruction of the bronchial cartilage
- pus-filled bronchiectatic cavities
- penumonic consolidation
lymphoid follicular hyperplasia
- bronchiectatic cysts
- interstitial emphysema
- emphysematous bullae
attenuated and shortened interalveolar septae
pulmonary hypertension lesions
cor pulmonale lesions
- Aspergillus bronchial colonisation (endobronchial aspergillosis)
- endobronchial semi-invasive aspergillosis
- allergic bronchopulmonary aspergillosis
- progressive chronic pulmonary aspergillosis (PCPA) (semi-invasive pulmonary aspergillosis)
- invasive pulmonary aspergillosis