BRONCHIAL OBSTRUCTION WITH LOBAR ATELECTASIS AND EMPHYSEMA IN CYSTIC FIBROSIS OF THE PANCREAS

[摘要] A. Lobar atelectasis with obstructive emphysema occurs in about 10% of patients with cystic fibrosis of the pancreas. In most instances, it appears in early infancy and during the first episode of pulmonary involvement and usually affects one or more lobes of the right lung. It is an indication of severe respiratory disease, whose major expression is widespread obstruction of the bronchi and whose cardinal clinical features are cough, cyanosis and respiratory distress. Rapid onset of bronchial obstruction due to failure to remove secretions is the initial event; infection is secondary and due to invasion of the obstructed bronchi.The respiratory disease ushered in by lobar atelectasis represents one of the more severe forms of fibrocystic disease of the pancreas; in many instances it is rapidly fatal unless checked in its progress by the use of antibiotics.Bronchoscopy and bronchial aspiration through a tracheotomy tube are usually ineffective. Lobectomy is of doubtful value and justified only by the persistence of lobar atelectasis unchanged for a prolonged period. Early diagnosis and early antibiotic therapy are at present the best weapons.In infants below the age of one year, lobar atelectasis and obstructive emphysema, especially if they occur in white infants and involve the right lung, should suggest the possibility of cystic fibrosis of the pancreas.B. The pathogenetic mechanism of the pulmonary lesions in cystic fibrosis of the pancreas is more acute and perhaps easier to understand in patients with lobar atelectasis, but is essentially the same except in degree for all patients with this disease. Because of failure to remove the secretions, acute respiratory infections usually initiate the cycle by causing increased mucus production. Widespread and severe bronchial obstruction results and secondary infection of the obstructed air passages follows after a variable but usually short period of time. If the patient survives the first episode, the same cycle can be repeated again on the occasion of a subsequent respiratory infection. Any one of these attacks may be fatal.Although respiratory involvement is a virtually constant accompaniment of the pancreatic lesions in cystic fibrosis of the pancreas, their interdependence is still unexplained. The two principal theories regarding the interrelationship of the pulmonary and pancreatic lesions of fibrocystic disease are discussed. It is concluded that a satisfactory understanding of this problem has not yet been attained.

[学科分类] 儿科学