Two-year results after lung volume reduction surgery in α1- antitrypsin deficiency versus smoker's emphysema

Dept of Thoracic Surgery, Ruhrlandklinik, Essen, Germany.
European Respiratory Journal (Impact Factor: 7.64). 12/1998; 12(5):1028-32.
Source: PubMed


Lung volume reduction surgery (LVRS) improves exercise capacity and relieves dyspnoea in patients with smoker's emphysema (SE). It is unclear, however, whether LVRS similarly improves lung function in alpha1-antitrypsin-deficiency emphysema (alpha1 E). To address this question, this study prospectively compared the intermediate-term functional outcome in 12 consecutive patients with advanced alpha1E and 18 patients with SE who underwent bilateral LVRS. Before surgery there were no statistically significant differences between the two groups in the six-minute walking distance, dyspnoea score, respiratory mechanics or lung function data, except for the forced expiratory volume in one second, which was lower in the deficient group (24 versus 31% of the predicted value; p<0.05). In both groups, bilateral LRVS produced significant improvements in dyspnoea, the six-minute walking distance, lung function and respiratory mechanics. In the alpha1E group, the functional data, with the exception of the six-minute walking distance, returned to baseline at 6-12 months postoperation and showed further deterioration at 24 months. The functional status of the SE group remained significantly improved over this period. In conclusion, the functional improvements resulting from bilateral lung volume reduction surgery are sustained for at least 2 yrs in most patients with smoker's emphysema, but this type of surgery offers only short-term benefits for most patients with alpha1E.

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    • "The results of lung volume reduction surgery in patients with AAT deficiency have been inconclusive , and these patients are not considered to be ideal candidates for this intervention owing to the morphological characteristics of their pulmonary impairment [Cassina et al. 1998]. "
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    ABSTRACT: Alpha-1 antitrypsin deficiency (AAT) is a hereditary recessive autosomal disease caused by mutations in the AAT gene. This disease is characterized by abnormally low AAT concentrations in plasma, which, in its homozygote form, carries a high risk for the development of early pulmonary emphysema and liver damage. Since the end of the 1980s augmentation therapy with AAT from human plasma has been available for specific treatment of emphysema due to AAT deficiency. Intravenous augmentation therapy has been demonstrated to be safe and weekly infusions of AAT have resulted in plasma AAT concentrations above those considered protective for the lungs. However, life-long weekly infusions are not well accepted by patients, therefore pharmacokinetic studies have been performed to try to individualize the therapeutic regimen in order to obtain adequate trough serum AAT levels with prolonged intervals of administration. Therapeutic regimens administered every two weeks appear to be safe and result in adequate trough serum concentrations, but less-frequent administrations result in trough levels below the target. Alpha-1-antitrypsin deficiency is largely unrecognized and underdiagnosed. The foundation of national and international registries is a valid strategy to increase awareness about the disease and collect information about the natural history of this deficiency. Furthermore, the identification of a large number of patients will allow the development of new clinical trials aimed at finding better treatments for this infrequent condition.
    Therapeutic Advances in Respiratory Disease 03/2008; 2(1):13-21. DOI:10.1177/1753465807088159 · 1.95 Impact Factor
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    • "While several teams have identifi ed predictive factors of good functional results (Wang et al 1997; NETT 2003; Ingenito et al 1998, 2001; Thurnheer et al 1999), the infl uence of α 1 -AT defi ciency on functional results remains debated. The studies which have focused on the results of LVRS in patients with α 1 -AT defi ciency have yielded controversial results (Cooper et al 1996; Cassina et al 1998; Gelb et al 1999; Rischer et al 1999). Taking these results into account, the ATS–ERS statement on the standards for the diagnosis and management of individuals with α 1 -AT defi ciency has recently concluded that LVRS offers only short-term benefi ts for most defi cient patients and that LVRS should not be recommended in these patients pending additional studies (ATS–ERS 2003). "
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    ABSTRACT: Lung volume reduction surgery (LVRS) has been shown to improve lung function and exercise tolerance in patients with severe emphysema. Some predictors of poor outcome have been described but the role of alpha1-antitrypsin (alpha1-AT) deficiency is still not well known. The aim of this study was to analyze the results of unilateral LVRS in our center according to the alpha1-AT status. The results of LVRS in 17 deficient patients and 35 nondeficient patients were analyzed at 3-6 months and 1 year after surgery. Compared with baseline, a significant improvement of FEV1, partial pressure in arterial blood (PaO2), dyspnea score and walking distance was observed in the two groups at 3-6 months after surgery and the studied parameters remained significantly improved at 1 year in the nondeficient group. By contrast, PaO2 and walking distance returned towards baseline in the deficient group at 1 year whereas improvement of FEV1 and dyspnea score was persistent. Mean values of FEV, at baseline, 3-6 months, and 1 year were 22 +/- 6%, 29 +/- 11%, and 26 +/- 9% and 28 +/- 12%, 38 +/- 17%, and 40 +/- 17% predicted in the deficient group and in the non-deficient group, respectively. In conclusion, the functional benefit is short-lasting in alpha1-AT deficient patients after unilateral LVRS.
    International Journal of COPD 02/2006; 1(2):201-6. · 3.14 Impact Factor
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    • "Lung tissue of adequate quality from patients with advanced emphysema undergoing lung volume reduction surgery was used for the present study [18,21]. Samples examined by transmission electron microscopy (TEM) included five specimens from alpha-1 antitrypsin deficiency (AATD) and 34 from non-AATD patients. "
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    ABSTRACT: Chlamydiales are familiar causes of acute and chronic infections in humans and animals. Human pulmonary emphysema is a component of chronic obstructive pulmonary disease (COPD) and a condition in which chronic inflammation manifested as bronchiolitis and intra-alveolar accumulation of macrophages is common. It is generally presumed to be of infectious origin. Previous investigations based on serology and immunohistochemistry indicated Chlamydophila pneumoniae infection in cases of COPD. Furthermore, immunofluorescence with genus-specific antibodies and electron microscopy suggested involvement of chlamydial infection in most cases of pulmonary emphysema, but these findings could not be verified by PCR. Therefore, we examined the possibility of other chlamydial species being present in these patients. Tissue samples from patients having undergone lung volume reduction surgery for advanced alpha-1 antitrypsin deficiency (AATD, n = 6) or non-alpha-1 antitrypsin deficiency emphysema (n = 34) or wedge resection for hamartochondroma (n = 14) were examined by transmission electron microscopy and PCR. In all cases of AATD and 79.4% of non-AATD, persistent chlamydial infection was detected by ultrastructural examination. Intra-alveolar accumulation of macrophages and acute as well as chronic bronchiolitis were seen in all positive cases. The presence of Chlamydia psittaci was demonstrated by PCR in lung tissue of 66.7% AATD vs. 29.0% non-AATD emphysema patients. Partial DNA sequencing of four positive samples confirmed the identity of the agent as Chlamydophila psittaci. In contrast, Chlamydophila pneumoniae was detected only in one AATD patient. Lung tissue of the control group of non-smokers with hamartochondroma was completely negative for chlamydial bodies by TEM or chlamydial DNA by PCR. These data indicate a role of Chlamydophila psittaci in pulmonary emphysema by linking this chronic inflammatory process to a chronic infectious condition. This raises interesting questions on pathogenesis and source of infection.
    BMC Infectious Diseases 10/2004; 4(1):38. DOI:10.1186/1471-2334-4-38 · 2.61 Impact Factor
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