Pulmonary changes and cor pulmonale in mucoviscidosis

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RESUMO

The lungs of eight cases of cystic fibrosis in patients ranging from age 19 days to 23 years were examined by the large-section technique of Gough and Wentworth (Gough, 1960). All cases showed in life a susceptibility to staphylococcal and pseudomonal bronchopneumonia. In all cases the bronchial tree was plugged with mucus. The lungs from the older patients showed diffuse and pronounced bronchiectasis with luxuriant peribronchial granulation tissue and dilatation of the pulmonary arterial system. The distal air spaces in all cases showed only minimal distension and only in the eldest was there any evidence of destructive emphysema. Three cases developed cor pulmonale which we thought was the result of hypoxaemia due to a ventilation-perfusion imbalance.

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