title: [Pneumothorax as complication of cystic fibrosis of the pancreas].
abstract: During recent years, 14 episodes of pneumothorax in 4 children and adolescents with cystic fibrosis of the pancreas (CF) have been treated in the University Department of Pediatrics in Zurich. Treatment with pleural drainage, associated with instillations of sclerosing agents was usually tedious and did not prevent relapses. One patient died after the second episode, another was successfully treated by pleurectomy. The incidence of pneumothorax in adolescent CF patients increases rapidly mostly due to the longer life expectancy of CF patients. Both lungs are equally affected. In more than 50% one or more relapses occur. The prognosis is usually bad. No specific etiologic factors can be incriminated, pneumothorax occurring mostly during periods of physical inactivity. The pathogenesis seems, in the majority of cases, to be the bursting of a subpleural emphysematous bulla or bleb. Such bullae can mostly be recognised radiologically many years prior to the first pneumothorax episode, usually in severely affected lungs in children over 10 years of age. From the reviewed literature as well as from our own experience it seems that pleurectomy is the only efficient treatment, being well tolerated, curing the actual episode of pneumothorax, and preventing relapses.
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D002648:Child; D003550:Cystic Fibrosis / Q000150:complications*; D005260:Female; D006801:Humans; D008297:Male; D011030:Pneumothorax / Q000209:etiology* / Q000401:mortality / Q000628:therapy; D012737:Sex Factors
vernacular_title: Der Pneumothorax als Komplikation der zystischen Pankreasfibrose.