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Pneumothorax as a Complication of Cystic Fibrosis: Report of Twenty Cases

Pneumothorax as a Complication of Cystic Fibrosis: Report of Twenty Cases Abstract PNEUMOTHORAX is a recognized complication of cystic fibrosis.1-3 Although described as unusual3 or rare,2 the actual incidence of this complication has not been defined, nor has the clinical significance been assessed. Furthermore, the literature on this problem is presently restricted to single case reports,3-5 with little opportunity for evaluation of treatment. In particular, there is no information on the management of persistent or recurrent pneumothorax complicating cystic fibrosis. It is the purpose of this paper to review our experience with 20 cases of cystic fibrosis and pneumothorax, and to present four cases in which open thoracotomy and direct surgical intervention were required because of persistent or recurrent pneumothorax. Subjects and Methods The records of all patients with cystic fibrosis seen at Babies Hospital, Columbia-Presbyterian Medical Center, New York, from January 1953 through December 1967 were reviewed. Of a total 710 patients seen over this 15-year period, References 1. Anderson, D.H.: Cystic Fibrosis of the Pancreas: A Review , J Chron Dis 7:58-90 ( (Jan) ) 1958.Crossref 2. Shwachman, H.: " Cystic Fibrosis ," in Kendig, E.L., Jr. (ed.): Disorders of the Respiratory Tract in Children , Philadelphia: W. B. Saunders Co., 1967, pp 541-564. 3. Paxton, D.G., and Scott, E.P.: Pneumothorax: An Unusual Complication in Fibrocystic Disease , Amer J Dis Child 111:311-312 ( (March) ) 1966. 4. Fanconi, G., and Metaxas-Buhler, M.: Spontaneous Pneumothorax in Pancreatic Fibrosis With Bronchiectasis , Helv Paediat Acta 2:289-295 ( (June) ) 1947. 5. Bernard, E., et al: Mucoviscidosis and Idiopathic Spontaneous Pneumothorax , J Franc Med Chir Thorac 16:105-109 ( (Jan) ) 1962. 6. Cook, C.D., and Hamann, J.F.: Relation of Lung Volumes to Height in Healthy Persons Between the Ages of 5 and 38 Years , J Pediat 59:710-714 ( (Nov) ) 1961.Crossref 7. Engstrom, I.; Karlberg, P.; and Kraepelien, S.: Respiratory Studies in Children: I. Lung Volumes in Healthy Children 6-14 Years of Age , Acta Paediat 45:249-277 ( (May) ) 1956.Crossref 8. Banyai, A.L.: " Collapse of the Lung ," in Gordon, B.L. (ed.): Clinical Cardiopulmonary Physiology , New York: Grune & Stratton, Inc., 1957, pp 208-218. 9. Chapman, J.S.: Spontaneous Irruption of Air From the Lung: I. Pneumomediastinum , Amer J Med 18:547-556 ( (April) ) 1955.Crossref 10. Miller, W.S.: A Further Study of Emphysematous Blebs , Amer J Roent 18:42-47 ( (Jan) ) 1927. 11. Rubin, E.H., and Rubin, M.: Thoracic Diseases , Philadelphia: W. B. Saunders Co., 1961, p 422. 12. Hinshaw, H.C., and Garland, L.H.: Diseases of the Chest , ed 2, Philadelphia: W. B. Saunders Co., 1963, p 256. 13. Hughes, F.A.; Kraeft, N.H.; and Lowry, C.C.: Treatment of Spontaneous Pneumothorax , JAMA 146:244-247 ( (May) ) 1951.Crossref 14. Anderson, D.H.: Pathology of Cystic Fibrosis , Ann NY Acad Sci 93:500-517 ( (Aug) ) 1962.Crossref 15. Macklin, M.T., and Macklin, C.C.: Malignant Interstitial Emphysema of the Lungs and Mediastinum as an Important Occult Complication in Many Respiratory Diseases and Other Conditions: An Interpretation of the Clinical Literature in the Light of Laboratory Experiment , Medicine 23:281-358 ( (Dec) ) 1944.Crossref 16. Cook, C.D., et al: Studies of Respiratory Physiology in Children: II. Lung Volumes and Mechanics of Respiration in 64 Patients With Cystic Fibrosis of Pancreas , Pediatrics 24:181-193 ( (Aug) ) 1959. 17. Howell, J.B.L., et al: Effect of Inflation of the Lung on Different Parts of the Pulmonary Vascular Bed , J Appl Physiol 16:71-76 ( (Jan) ) 1961. 18. Mellins, R.B., et al: Pericapillary Pressure in the Lung , Fed Proc 25:629 ( (April) ) 1966. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png American Journal of Diseases of Children American Medical Association

Pneumothorax as a Complication of Cystic Fibrosis: Report of Twenty Cases

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References (20)

Publisher
American Medical Association
Copyright
Copyright © 1968 American Medical Association. All Rights Reserved.
ISSN
0002-922X
DOI
10.1001/archpedi.1968.02100020637010
Publisher site
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Abstract

Abstract PNEUMOTHORAX is a recognized complication of cystic fibrosis.1-3 Although described as unusual3 or rare,2 the actual incidence of this complication has not been defined, nor has the clinical significance been assessed. Furthermore, the literature on this problem is presently restricted to single case reports,3-5 with little opportunity for evaluation of treatment. In particular, there is no information on the management of persistent or recurrent pneumothorax complicating cystic fibrosis. It is the purpose of this paper to review our experience with 20 cases of cystic fibrosis and pneumothorax, and to present four cases in which open thoracotomy and direct surgical intervention were required because of persistent or recurrent pneumothorax. Subjects and Methods The records of all patients with cystic fibrosis seen at Babies Hospital, Columbia-Presbyterian Medical Center, New York, from January 1953 through December 1967 were reviewed. Of a total 710 patients seen over this 15-year period, References 1. Anderson, D.H.: Cystic Fibrosis of the Pancreas: A Review , J Chron Dis 7:58-90 ( (Jan) ) 1958.Crossref 2. Shwachman, H.: " Cystic Fibrosis ," in Kendig, E.L., Jr. (ed.): Disorders of the Respiratory Tract in Children , Philadelphia: W. B. Saunders Co., 1967, pp 541-564. 3. Paxton, D.G., and Scott, E.P.: Pneumothorax: An Unusual Complication in Fibrocystic Disease , Amer J Dis Child 111:311-312 ( (March) ) 1966. 4. Fanconi, G., and Metaxas-Buhler, M.: Spontaneous Pneumothorax in Pancreatic Fibrosis With Bronchiectasis , Helv Paediat Acta 2:289-295 ( (June) ) 1947. 5. Bernard, E., et al: Mucoviscidosis and Idiopathic Spontaneous Pneumothorax , J Franc Med Chir Thorac 16:105-109 ( (Jan) ) 1962. 6. Cook, C.D., and Hamann, J.F.: Relation of Lung Volumes to Height in Healthy Persons Between the Ages of 5 and 38 Years , J Pediat 59:710-714 ( (Nov) ) 1961.Crossref 7. Engstrom, I.; Karlberg, P.; and Kraepelien, S.: Respiratory Studies in Children: I. Lung Volumes in Healthy Children 6-14 Years of Age , Acta Paediat 45:249-277 ( (May) ) 1956.Crossref 8. Banyai, A.L.: " Collapse of the Lung ," in Gordon, B.L. (ed.): Clinical Cardiopulmonary Physiology , New York: Grune & Stratton, Inc., 1957, pp 208-218. 9. Chapman, J.S.: Spontaneous Irruption of Air From the Lung: I. Pneumomediastinum , Amer J Med 18:547-556 ( (April) ) 1955.Crossref 10. Miller, W.S.: A Further Study of Emphysematous Blebs , Amer J Roent 18:42-47 ( (Jan) ) 1927. 11. Rubin, E.H., and Rubin, M.: Thoracic Diseases , Philadelphia: W. B. Saunders Co., 1961, p 422. 12. Hinshaw, H.C., and Garland, L.H.: Diseases of the Chest , ed 2, Philadelphia: W. B. Saunders Co., 1963, p 256. 13. Hughes, F.A.; Kraeft, N.H.; and Lowry, C.C.: Treatment of Spontaneous Pneumothorax , JAMA 146:244-247 ( (May) ) 1951.Crossref 14. Anderson, D.H.: Pathology of Cystic Fibrosis , Ann NY Acad Sci 93:500-517 ( (Aug) ) 1962.Crossref 15. Macklin, M.T., and Macklin, C.C.: Malignant Interstitial Emphysema of the Lungs and Mediastinum as an Important Occult Complication in Many Respiratory Diseases and Other Conditions: An Interpretation of the Clinical Literature in the Light of Laboratory Experiment , Medicine 23:281-358 ( (Dec) ) 1944.Crossref 16. Cook, C.D., et al: Studies of Respiratory Physiology in Children: II. Lung Volumes and Mechanics of Respiration in 64 Patients With Cystic Fibrosis of Pancreas , Pediatrics 24:181-193 ( (Aug) ) 1959. 17. Howell, J.B.L., et al: Effect of Inflation of the Lung on Different Parts of the Pulmonary Vascular Bed , J Appl Physiol 16:71-76 ( (Jan) ) 1961. 18. Mellins, R.B., et al: Pericapillary Pressure in the Lung , Fed Proc 25:629 ( (April) ) 1966.

Journal

American Journal of Diseases of Children – American Medical Association

Published: Dec 1, 1968

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