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

doi:10.1016/S0046-8177(76)80023-8

Human Pathology

Volume 7, Issue 2, March 1976, Pages 195-204

https://doi.org/10.1016/S0046-8177(76)80023-8 Get rights and content

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.

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^*: Presented at the 11th Latin American Congress of Pathology, Merida, Mexico, November 1973.

^†: Assistant Professor of Pathology, The University of Texas Health Science Center. Staff, Pathology Department, Hermann Hospital, Houston, Texas.

^‡: Professor of Pathology, Baylor College of Medicine, Houston, Texas.

^§: Associate Professor of Pathology, Baylor College of Medicine, Houston, Texas.

^#: Pathologist, Laboratory Service, U. S. Air Force Regional Hospital, Minot, North Dakota.

^‖: Professor of Pathology, Baylor College of Medicine. Staff, Department of Pathology, Texas Children's Hospital, Houston, Texas.

View full text

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

Abstract

Brit. J. Dis. Chest.

J. Pediat.

Studies in cystic fibrosis: a report on sixty-five patients over 17 years of age

Pediatrics

Cystic fibrosis: recent advances in understanding of its pathogenesis, detection of carriers and improved prognosis

Medical Times

The bronchial mucous glands—their hypertrophy and change in intracellular mucus

Observations in cystic fibrosis of the pancreas. III. Pulmonary lesions

Johns Hopkins Med. J.

A rapid method of inflation fixation for morphologic study of chronic pulmonary disease

Amer. J. Clin. Pathol.

Measurement of the bronchial mucous gland layer: a diagnostic yardstick in chronic bronchitis

Thorax