TY - JOUR
T1 - Quantitative aspects of lung pathology in cystic fibrosis
AU - Sobonya, R. E.
AU - Taussig, L. M.
PY - 1986
Y1 - 1986
N2 - The lungs of 9 patients with cystic fibrosis were studied by morphometric techniques to determine the amount of bronchiectasis, emphysema, pneumonia, bronchial gland enlargement, and small airways narrowing and density. The severity of these processes, which should account for the clinical picture of air-flow limitation in these patients, varied greatly among patients of different ages. Bronchiectasis was present in all children, but the amount of bronchiectasis did not appear to increase with age. Mild destructive emphysema was seen only in adults, but many younger patients had overinflation without destructive emphysema, as recognized by increased mean linear intercept (interalveolar distance). Despite clinical evidence of mucous hypersecretion, bronchial gland enlargement was present only in some patients. The youngest patient showed normal small airways, but children and younger teenagers tended to have dilated small airways. Older teenagers and adults predominantly had stenosis of small airways. Varying patterns of pulmonary pathology are seen in cystic fibrosis and may be related to rapidity of progression of disease.
AB - The lungs of 9 patients with cystic fibrosis were studied by morphometric techniques to determine the amount of bronchiectasis, emphysema, pneumonia, bronchial gland enlargement, and small airways narrowing and density. The severity of these processes, which should account for the clinical picture of air-flow limitation in these patients, varied greatly among patients of different ages. Bronchiectasis was present in all children, but the amount of bronchiectasis did not appear to increase with age. Mild destructive emphysema was seen only in adults, but many younger patients had overinflation without destructive emphysema, as recognized by increased mean linear intercept (interalveolar distance). Despite clinical evidence of mucous hypersecretion, bronchial gland enlargement was present only in some patients. The youngest patient showed normal small airways, but children and younger teenagers tended to have dilated small airways. Older teenagers and adults predominantly had stenosis of small airways. Varying patterns of pulmonary pathology are seen in cystic fibrosis and may be related to rapidity of progression of disease.
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M3 - Article
C2 - 3740655
AN - SCOPUS:0022462591
VL - 134
SP - 290
EP - 295
JO - American Journal of Respiratory and Critical Care Medicine
JF - American Journal of Respiratory and Critical Care Medicine
SN - 1073-449X
IS - 2
ER -