TY - JOUR T1 - PNeumothorax in cystic fibrosis AU - Boat TF, di Sant' Agnese PA, Warwick WJ, Handwerger SA Y1 - 1969/09/08 N1 - 10.1001/jama.1969.03160230032008 JO - JAMA SP - 1498 EP - 1504 VL - 209 IS - 10 N2 - Thirty-two episodes of spontaneous pneumothorax occurred with increasing frequency over a ten-year period in 15 of 400 patients with cystic fibrosis. This complication most often affected adolescents or young adults with significant lung disease. The most likely pathogenetic mechanism was rupture of a subpleural bleb. Tension pneumothorax was found frequently and accounted for most cases of severe dyspnea and cyanosis including three sudden deaths. Silent pneumothorax also occurred. Small asymptomatic pneumothoraces resolved with observation. Most pneumothoraces were treated by tube thoracostomy; rapid resolution resulted in 65%. No pleural infections occurred; two bronchopleural fistulae and one bronchopleural cutaneous fistula complicated recovery. Results of prophylaxis for recurrent pneumothorax with intrapleural installation of chemical irritants were inconclusive. No thoracotomies were performed. Pneumothorax entails vigorous pulmonary care and travel restrictions. SN - 0098-7484 M3 - doi: 10.1001/jama.1969.03160230032008 UR - http://dx.doi.org/10.1001/jama.1969.03160230032008 ER -