Alpha 1-antitrypsin deficiency surgery: Difference between revisions
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==Overview== | ==Overview== | ||
== | |||
===Lung Transplantation=== | |||
*Lung transplantation may be recommended for some patients with end-stage lung disease. | |||
*Alpha 1-antitrypsin deficiency accounts for about 5% of all lung transplantations performed in the United States. | |||
*Single lung transplant is more common despite the fact that outcome is better in patient who receive double lung transplant due to limitations on availability. | |||
*Five year survival rates following lung transplant is approximately 50%. | |||
*Bronchiolitis obliterans being the major cause of death post-transplant. | |||
*Outcomes of lung transplantation can vary considerably. | |||
*For example, reports from Washington University Medical Center in St. Louis, the Copenhagen National Lung Transplant Group, and Zurich University Hospital reported no difference in 5-year survival rates between AATD-COPD and usual COPD recipients. | |||
*In contrast, the Toronto group reported that AATD patients had lower 10-year survival (23% vs 43%) compared with usual COPD patients. | |||
*A recent study from the 2 national lung transplantation centers in Sweden documented a median survival time for AATD-COPD patients of 12 years compared with only 6 years for usual COPD patients. | |||
*the rate of FEV1 decline among AATD patients who received double lung transplantation was faster than among single lung recipients. | |||
*This difference persisted after adjustment for age, smoking status, body mass index, oxygen use, exercise capacity, donor age, cytomegalovirus mismatch, and transplant type. | |||
*The question of whether lung transplantation confers a survival advantage over the natural history of the disease has been addressed in preliminary fashion in a case-control study by Tanash and colleagues. | |||
*These investigators matched 83 severe AATD-COPD patients who underwent lung transplantation with 70 control cases (AATD-COPD patients who did not undergo transplantation selected from the Swedish national AATD registry). | |||
*The estimated median survival time was 11 years in transplant recipients versus 5 years in controls. Banga and colleagues98 recently reported the natural history of lung function after lung transplantation for patients with usual (231 patients) versus AATD-COPD (45 patients) at the Cleveland Clinic. | |||
Revision as of 20:53, 27 November 2017
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Alpha 1-antitrypsin deficiency Microchapters |
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Differentiating Alpha 1-antitrypsin deficiency from other Diseases |
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Diagnosis |
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Treatment |
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Case Studies |
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Alpha 1-antitrypsin deficiency surgery On the Web |
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American Roentgen Ray Society Images of Alpha 1-antitrypsin deficiency surgery |
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Directions to Hospitals Treating Alpha 1-antitrypsin deficiency |
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Risk calculators and risk factors for Alpha 1-antitrypsin deficiency surgery |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:
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Overview
Lung Transplantation
- Lung transplantation may be recommended for some patients with end-stage lung disease.
- Alpha 1-antitrypsin deficiency accounts for about 5% of all lung transplantations performed in the United States.
- Single lung transplant is more common despite the fact that outcome is better in patient who receive double lung transplant due to limitations on availability.
- Five year survival rates following lung transplant is approximately 50%.
- Bronchiolitis obliterans being the major cause of death post-transplant.
- Outcomes of lung transplantation can vary considerably.
- For example, reports from Washington University Medical Center in St. Louis, the Copenhagen National Lung Transplant Group, and Zurich University Hospital reported no difference in 5-year survival rates between AATD-COPD and usual COPD recipients.
- In contrast, the Toronto group reported that AATD patients had lower 10-year survival (23% vs 43%) compared with usual COPD patients.
- A recent study from the 2 national lung transplantation centers in Sweden documented a median survival time for AATD-COPD patients of 12 years compared with only 6 years for usual COPD patients.
- the rate of FEV1 decline among AATD patients who received double lung transplantation was faster than among single lung recipients.
- This difference persisted after adjustment for age, smoking status, body mass index, oxygen use, exercise capacity, donor age, cytomegalovirus mismatch, and transplant type.
- The question of whether lung transplantation confers a survival advantage over the natural history of the disease has been addressed in preliminary fashion in a case-control study by Tanash and colleagues.
- These investigators matched 83 severe AATD-COPD patients who underwent lung transplantation with 70 control cases (AATD-COPD patients who did not undergo transplantation selected from the Swedish national AATD registry).
- The estimated median survival time was 11 years in transplant recipients versus 5 years in controls. Banga and colleagues98 recently reported the natural history of lung function after lung transplantation for patients with usual (231 patients) versus AATD-COPD (45 patients) at the Cleveland Clinic.
Lung Volume Reduction Surgery
- Lung volume reduction surgery (LVRS) can help relieve dyspnea and improve exercise capacity in patients with emphysema. Data regarding the efficacy of LVRS for individuals with AATD is limited and generally less favorable in magnitude and duration of FEV1 improvement.
- One study demonstrated that bilateral LVRS in AAT-deficient patients with emphysema can be beneficial but functional measurements except 6 min walk test, returned to baseline at 6–12 months. Although, LVRS was not recommended for management of AAT deficiency.