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Lung Transplantation for Cystic Fibrosis

  • T. M. Egan

Abstract

Cystic fibrosis (CF) is the most common lethal genetic disease of Caucasians. Although it is a multisystem disease it is estimated that 95% of patients with CF will succumb from end-stage pulmonary disease[1]. Up to 400 patients with CF die annually in the United States. The past decade has seen remarkable advances in understanding the pathophysiology of CF, including identification of a chloride channel abnormality 3. identification of the gene responsible for the chloride channel[4], and creation of a transgenic mouse with CF[5]. Even before the genetic defect was identified, it was apparent that the chloride channel epithelial abnormality present in CF, evidenced by an altered potential difference[3], was not manifested in the epithelium of heart-lung grafts in transplanted CF patients[6]. This provided assurance that the pulmonary abnormalities observed in CF patients were unlikely to recur following lung transplantation.

Keywords

Cystic Fibrosis Lung Transplant Bronchiolitis Obliterans Syndrome Lung Transplant Recipient Airway Complication 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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Copyright information

© Kluwer Academic Publishers 1996

Authors and Affiliations

  • T. M. Egan

There are no affiliations available

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