The lung in cystic fibrosis. A quantitative study including prevalence of pathologic findings among different age groups.

The autopsies of 82 patients with cystic fibrosis were reviewed with respect 
to pathologic changes in the lungs and their respective prevalence among 
different age groups. Although bronchitis, mucopurulent plugging, and bronchopneumonia were almost 
universally present among children of all ages, epithelial metaplasia and bronchiectasis 
were rarer among infants and progressively more prevalent in older age 
groups. Emphysema was absent in patients under two years of age 
and affected 11 per cent of the patients two to six 
years of age and 40 per cent of the patients older 
than six years, but was never of a severe degree by 
the point count method. Pulmonary hemorrhage, although uncommon, was usually associated 
with prominent arterial vessels in walls of bronchiectatic airways. Quantitative assessment 
of bronchial glands revealed Reid indices significantly higher in patients with 
cystic fibrosis when compared to noncystic fibrosis patients, but there was 
no increase in these indices with the age of the patients. 
Glandular hypertrophy, predominance of mucous acini within glands, and goblet cell 
hyperplasia of the bronchial mucosa all suggest an explanation for the 
copious mucous secretion of patients with cystic fibrosis. However, it was 
not possible to ascertain whether these findings reflect a general exocrine 
defect of such patients or whether they were merely a response 
to chronic airway infection, even though the latter is a more 
plausible assumption. 