Authors:
Stick SM
Authors notes:
European Respiratory Monograph, European Respiratory Society. 64: 77-87.
Keywords:
bronchiectasis, cystic fibrosis, disease surveillance, enzyme replacement; genotype, human, lung disease, Pseudomonas aeruginosa, infection
Abstract:
Lung disease was recognised as an early component of cystic fibrosis (CF) when it was first described as a syndrome.
However, until midway through the 20th century, when the generalised mucous abnormality was identified, it was considered secondary to low vitamin A due to malabsorption of fat.
Improved nutrition resulting from pancreatic enzyme replacement and comprehensive care in specialist clinics has resulted in improved survival, and lung disease is now the major determinant of morbidity and mortality.
Early surveillance studies have demonstrated the very early onset of lung disease associated with neutrophilic airway inflammation and indicate potential strategies to prevent or slow the onset of structural lung disease.