HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

This Article Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Starnes, V. A. Articles by Oyer, P. E. Search for Related Content PubMed PubMed Citation Articles by Starnes, V. A. Articles by Oyer, P. E.

The Journal of Thoracic and Cardiovascular Surgery, Vol 103, 1008-1014, Copyright © 1992 by The American Association for Thoracic Surgery and The Western Thoracic Surgical Association

ARTICLES

Cystic fibrosis. Target population for lung transplantation in North America in the 1990s

VA Starnes, N Lewiston, J Theodore, C Stoehr, E Stinson, NE Shumway and PE Oyer
Department of Cardiothoracic Surgery, Stanford University Medical Center, Calif. 94305.

Cystic fibrosis is the most common lethal genetic disease occurring in the white population. It is estimated that 3.5% of the 20,000 individuals with cystic fibrosis in North America will die each year of end-stage lung disease. Lung transplantation (heart-lung or double lung) is becoming more frequent as more patients are referred for this procedure. Since January 1988, we have evaluated 60 patients with cystic fibrosis for lung transplantation and have accepted 30 (50%). Nine patients (30%) died while awaiting a donor. Fifteen patients underwent transplantation (13 heart-lung and two double lung procedures). Actuarial survival at 1, 2, and 3 years is 76%. All survivors are without physical limitations. Pulmonary function, as determined by forced vital capacity, forced expiratory volume in 1 second, and arterial blood gas determinations, is within the normal range. Comparing these data with those of a group of patients without cystic fibrosis who underwent transplantation during the same period did not reveal any significant differences with respect to infection, rejection, and outcome. Preliminary data suggest that obliterative bronchiolitis is less prevalent at 1 year in patients with cystic fibrosis (19%) than in those without cystic fibrosis (41%). Patients with cystic fibrosis present a number of challenges. The problems of pleural adhesions from repeated infections, pleurodesis, and previous thoracic procedures are now readily approached through the bilateral thoracosternotomy (clam shell) incision. Insulin-dependent diabetes mellitus and low-dose corticosteroid therapy are no longer considered absolute contraindications. Both septic lungs must be removed at operation, either with heart-lung transplantation or with double lung transplantation. These data support the therapeutic efficacy of lung transplantation for patients with cystic fibrosis.

This article has been cited by other articles:

				K. Wiebe, Th. Wahlers, W. Harringer, H.v.d. Hardt, H. Fabel, and A. Haverich Lung transplantation for cystic fibrosis - a single center experience over 8 years Eur. J. Cardiothorac. Surg., August 1, 1999; 14(2): 191 - 196. [Abstract] [Full Text] [PDF]

				C. G. Caronia, J. Gorvoy, P. Silver, C. Quinn, L. Nimkoff, and M. Sagy Use of Bilevel Positive Airway Pressure (BIPAP) in End-Stage Patients with Cystic Fibrosis Awaiting Lung Transplantation Clinical Pediatrics, September 1, 1998; 37(9): 555 - 559. [Abstract] [PDF]

				V. A. Starnes, M. L. Barr, R. G. Cohen, J. A. Hagen, W. J. Wells, M. V. Horn, and F. A. Schenkel LIVING-DONOR LOBAR LUNG TRANSPLANTATION EXPERIENCE: INTERMEDIATE RESULTS J. Thorac. Cardiovasc. Surg., November 1, 1996; 112(5): 1284 - 1291. [Abstract] [Full Text]

				G. Wu, F. Zhang, R. K. Salley, M. C. Robinson, and S. Chien A Systematic Study of Hypothermic Lung Preservation Solutions: Euro-Collins Solution Ann. Thorac. Surg., August 1, 1996; 62(2): 356 - 362. [Abstract] [Full Text]

				J. P. A. Couetil, D. P. Houssin, O. Soubrane, P. G. Chevalier, B. E. Dousset, D. Loulmet, A. Achkar, M. J. Tolan, C. I. Amrein, A. Guinvarch, et al. COMBINED LUNG AND LIVER TRANSPLANTATION IN PATIENTS WITH CYSTIC FIBROSIS: A 4 1/2-year experience J. Thorac. Cardiovasc. Surg., November 1, 1995; 110(5): 1415 - 1423. [Abstract] [Full Text]

				T. L. Spray, G. B. Mallory, C. B. Canter, and C. B. Huddleston Pediatric lung transplantationIndications, techniques, and early results J. Thorac. Cardiovasc. Surg., April 1, 1994; 107(4): 990 - 1000. [Abstract] [Full Text]