Survival benefit of lung transplantation in individuals with severe α(1)-anti-trypsin deficiency (PiZZ) and emphysema.
(2011) In The Journal of Heart and Lung Transplantation 30(12). p.1342-1347- Abstract
- BACKGROUND:
The objective of lung transplantation (LTx) is to prolong life, but the survival benefit for patients with severe α(1)-anti-trypsin deficiency (PiZZ) and emphysema is still unclear. The aim of this study was to assess whether PiZZ patients who have undergone lung transplantation (the lung transplant group, TxG) do better than patients who have continued on the usual medical therapy (the non-transplant group, NTxG).
METHODS:
Between 1990, when the first patient received a lung transplant in Sweden, until June 2010, a total of 83 PiZZ patients with severe emphysema underwent transplantation. Seventy appropriate controls were identified from the Swedish National AAT Deficiency Registry. Each... (More) - BACKGROUND:
The objective of lung transplantation (LTx) is to prolong life, but the survival benefit for patients with severe α(1)-anti-trypsin deficiency (PiZZ) and emphysema is still unclear. The aim of this study was to assess whether PiZZ patients who have undergone lung transplantation (the lung transplant group, TxG) do better than patients who have continued on the usual medical therapy (the non-transplant group, NTxG).
METHODS:
Between 1990, when the first patient received a lung transplant in Sweden, until June 2010, a total of 83 PiZZ patients with severe emphysema underwent transplantation. Seventy appropriate controls were identified from the Swedish National AAT Deficiency Registry. Each control was matched with a patient who had received a lung transplant, for age, gender, smoking history (number of pack-years) and lung function at the time of transplantation.
RESULTS:
Both controls and lung transplant patients had low spirometric values with a mean FEV(1) of 23 ± 6% and 22 ± 9% of predicted value, respectively (not a statistically significant difference). Of the 83 transplant patients, 62 (75%) underwent single-lung transplantation (SLTx). During follow-up, 37 (45%) deaths occurred in the TxG and 45 (64%) in the NTxG. In the TxG, the estimated median survival time was 11 years (95% confidence interval [CI] 9 to 14 years), compared with 8 years (95% CI 4 to 6 years) for the NTxG (p = 0.006). The most common cause of death was pulmonary infection among the transplant patients (38%) and respiratory failure (60%) among the controls.
CONCLUSION:
Lung transplantation significantly improves long-term survival of patients with severe α(1)-anti-trypsin deficiency (PiZZ) and emphysema. (Less)
Please use this url to cite or link to this publication:
https://lup.lub.lu.se/record/2151358
- author
- Tanash, Hanan LU ; Riise, Gerdt C ; Hansson, Lennart LU ; Nilsson, Peter LU and Piitulainen, Eeva LU
- organization
- publishing date
- 2011
- type
- Contribution to journal
- publication status
- published
- subject
- keywords
- alpha(1)-anti-trypsin deficiency, lung transplantation, survival, mortality, COPD, emphysema, causes of death
- in
- The Journal of Heart and Lung Transplantation
- volume
- 30
- issue
- 12
- pages
- 1342 - 1347
- publisher
- Elsevier
- external identifiers
-
- wos:000297385400006
- pmid:21821433
- scopus:80655147089
- pmid:21821433
- ISSN
- 1557-3117
- DOI
- 10.1016/j.healun.2011.07.003
- language
- English
- LU publication?
- yes
- id
- a3426c3f-651e-480b-9660-c1ed1e5341f2 (old id 2151358)
- alternative location
- http://www.ncbi.nlm.nih.gov/pubmed/21821433?dopt=Abstract
- date added to LUP
- 2016-04-01 10:14:32
- date last changed
- 2022-01-25 21:16:39
@article{a3426c3f-651e-480b-9660-c1ed1e5341f2,
abstract = {{BACKGROUND:<br/><br>
The objective of lung transplantation (LTx) is to prolong life, but the survival benefit for patients with severe α(1)-anti-trypsin deficiency (PiZZ) and emphysema is still unclear. The aim of this study was to assess whether PiZZ patients who have undergone lung transplantation (the lung transplant group, TxG) do better than patients who have continued on the usual medical therapy (the non-transplant group, NTxG).<br/><br>
<br/><br>
METHODS:<br/><br>
Between 1990, when the first patient received a lung transplant in Sweden, until June 2010, a total of 83 PiZZ patients with severe emphysema underwent transplantation. Seventy appropriate controls were identified from the Swedish National AAT Deficiency Registry. Each control was matched with a patient who had received a lung transplant, for age, gender, smoking history (number of pack-years) and lung function at the time of transplantation.<br/><br>
<br/><br>
RESULTS:<br/><br>
Both controls and lung transplant patients had low spirometric values with a mean FEV(1) of 23 ± 6% and 22 ± 9% of predicted value, respectively (not a statistically significant difference). Of the 83 transplant patients, 62 (75%) underwent single-lung transplantation (SLTx). During follow-up, 37 (45%) deaths occurred in the TxG and 45 (64%) in the NTxG. In the TxG, the estimated median survival time was 11 years (95% confidence interval [CI] 9 to 14 years), compared with 8 years (95% CI 4 to 6 years) for the NTxG (p = 0.006). The most common cause of death was pulmonary infection among the transplant patients (38%) and respiratory failure (60%) among the controls.<br/><br>
<br/><br>
CONCLUSION:<br/><br>
Lung transplantation significantly improves long-term survival of patients with severe α(1)-anti-trypsin deficiency (PiZZ) and emphysema.}},
author = {{Tanash, Hanan and Riise, Gerdt C and Hansson, Lennart and Nilsson, Peter and Piitulainen, Eeva}},
issn = {{1557-3117}},
keywords = {{alpha(1)-anti-trypsin deficiency; lung transplantation; survival; mortality; COPD; emphysema; causes of death}},
language = {{eng}},
number = {{12}},
pages = {{1342--1347}},
publisher = {{Elsevier}},
series = {{The Journal of Heart and Lung Transplantation}},
title = {{Survival benefit of lung transplantation in individuals with severe α(1)-anti-trypsin deficiency (PiZZ) and emphysema.}},
url = {{http://dx.doi.org/10.1016/j.healun.2011.07.003}},
doi = {{10.1016/j.healun.2011.07.003}},
volume = {{30}},
year = {{2011}},
}