PULMONARY FUNCTION IN CYSTIC FIBROSIS OF THE PANCREAS

[摘要] Studies of pulmonary function in patients with chronic pulmonary disease associated with cystic fibrosis of the pancreas have revealed a pattern of dysfunction which may be said to be characteristic of this disease, but which is subject to great variation in severity.Both ventilatory insufficiency (leading to dyspnea) and disturbances in gas exchange (leading to arterial hypoxia and carbon dioxide retention) may occur. The former would seem to be largely the result of factors which bring about a diminished ventilatory capacity, among which an increased resistance to air flow within the tracheobronchial tree, due to obstruction of the larger and smaller bronchi by accumulated secretions and purulent exudate, is probably most important. Defective gas exchange seems to stem largely from disturbances in the normal relationships of alveolar ventilation to perfusion. This seems in large part due to uneven distribution of tidal air to the alveoli as a result of bronchial obstruction, although concomitant variation in the distribution of blood probably occurs also.In general, the severity of the impairment of pulmonary function closely parallels the chronicity and severity of the characteristic secondary bronchial and pulmonary infections. The treatment of these constitutes, logically, the major effort in therapy.

[学科分类] 儿科学