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The lung in cystic fibrosis. A quantitative study including prevalence of pathologic findings among different age
Insights
Autopsies reveal progressive lung pathology in cystic fibrosis (CF) patients, with emphysema increasing with age. While CF patients show higher Reid indices, the cause of mucus overproduction remains unclear, likely due to chronic infection.
Area of Science:
- Pulmonary Pathology
- Pediatric Medicine
- Cystic Fibrosis Research
Background:
- Cystic fibrosis (CF) is a genetic disorder affecting multiple organs, primarily the lungs.
- Understanding the progression of lung pathology in CF is crucial for developing effective treatments.
- Autopsy studies provide valuable insights into the long-term effects of CF on lung structure.
Purpose of the Study:
- To review autopsy findings in cystic fibrosis patients.
- To determine the prevalence of specific lung pathologies across different age groups.
- To investigate quantitative changes in bronchial glands in CF patients.
Main Methods:
- Retrospective review of autopsy records from 82 cystic fibrosis patients.
- Analysis of pathological changes in the lungs, including bronchitis, mucopurulent plugging, bronchiectasis, and emphysema.
- Quantitative assessment of bronchial glands using Reid indices.
Main Results:
- Bronchitis, mucopurulent plugging, and bronchopneumonia were nearly universal in all age groups.
- Epithelial metaplasia and bronchiectasis increased with age.
- Emphysema prevalence rose with age, affecting 40% of patients over six years old, though not severe.
- Reid indices were significantly higher in CF patients, independent of age.
- Glandular hypertrophy, mucous acini predominance, and goblet cell hyperplasia were observed.
Conclusions:
- Lung pathology in cystic fibrosis progresses with age, with emphysema becoming more prevalent in older children.
- Quantitative changes in bronchial glands suggest a role in excessive mucus secretion.
- The exact cause of increased mucus production in CF remains uncertain, with chronic infection being a more likely factor than a generalized exocrine defect.
Abstract:
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.