Cystic fibrosis surgery
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
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Overview
Surgery
Lung transplantation often becomes necessary for individuals with cystic fibrosis as lung function and exercise tolerance declines. Although single lung transplantation is possible in other diseases, individuals with CF must have both lungs replaced because the remaining lung would contain bacteria that could infect the transplanted lung. A pancreatic or liver transplant may be performed at the same time in order to alleviate liver disease and/or diabetes.[1] Lung transplantation is considered when lung function approaches a point where it threatens survival or requires assistance from mechanical devices.[2]
Gene therapy holds promise as a potential avenue to cure cystic fibrosis. Gene therapy attempts to place a normal copy of the CFTR gene into affected cells. Studies have shown that to prevent the lung manifestations of cystic fibrosis, only 5–10% the normal amount of CFTR gene expression is needed.[3] Many approaches have been theorized and several clinical trials have been initiated but, as of 2006, many hurdles still exist before gene therapy can be successful.[4]
References
- ↑ Simultaneous liver and pancreas transplantation in patients with cystic fibrosis. Transplant Proc. 2005 Oct;37(8):3567–9. PMID 16298663
- ↑ Belkin RA, Henig NR, Singer LG, Chaparro C, Rubenstein RC, Xie SX, Yee JY, Kotloff RM, Lipson DA, Bunin GR. Risk factors for death of patients with cystic fibrosis awaiting lung transplantation. Am J Respir Crit Care Med. 2006 Mar 15;173(6):659-66. Epub 2005 Dec 30. PMID 16387803
- ↑ Ramalho AS, Beck S, Meyer M, Penque D, Cutting GR, Amaral MD. Five percent of normal cystic fibrosis transmembrane conductance regulator mRNA ameliorates the severity of pulmonary disease in cystic fibrosis. Am J Respir Cell Mol Biol. 2002 27(5):619-27. PMID 12397022
- ↑ Tate S, Elborn S. Progress towards gene therapy for cystic fibrosis.Expert Opin Drug Deliv. 2005 Mar;2(2):269-80. Review. PMID 16296753