Lung Transplantation for Cystic Fibrosis

Cystic fibrosis (CF) is an inherited disease in which the fundamental physiological defect is failure of cAMP regulation of chloride transport. More than 90% of patients with CF will die of chronic, suppurative, obstructive lung disease, with the median survival in the United States currently being...

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Veröffentlicht in:Seminars in thoracic and cardiovascular surgery 1998-07, Vol.10 (3), p.202-212, Article 202
1. Verfasser: Mendeloff, E N
Format: Artikel
Sprache:eng
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Zusammenfassung:Cystic fibrosis (CF) is an inherited disease in which the fundamental physiological defect is failure of cAMP regulation of chloride transport. More than 90% of patients with CF will die of chronic, suppurative, obstructive lung disease, with the median survival in the United States currently being 29 years of age. Currently, although other therapies are being aggressively investigated, bilateral lung transplantation offers the only hope for short-term and mid-term survival in patients with CF and end-stage pulmonary disease. Since 1989, 103 bilateral sequential lung transplants (BLT) for CF have been performed at our institution (46 pediatric, 48 adult, 9 redo) at a mean age of 21+/-10 years. Cardiopulmonary bypass was used in all but one pediatric (age
ISSN:1043-0679
1532-9488
DOI:10.1016/S1043-0679(98)70038-3