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The aim of this study is to describe the natural history of patients with alpha-1 antitrypsin associated emphysema and to figure out associated prognostic factors.
All patients who suffer from this disease are followed every 6 months during a 5 years period. FEV1 decline is the primary endpoint. Quality of life assessed using the Saint George's Respiratory Questionary is recorded too.
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Inclusion Criteria:
Exclusion Criteria:
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Emphysema associated alpha-1 antitrypsin deficiency
| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Gabriel Thabut, MD | Contact | 1 40 87 57 12 | g.thabut@bch.aphp.fr |
| Name | Affiliation | Role |
|---|---|---|
| Gabriel Thabut, MD | Assistance Publique - Hôpitaux de Paris | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Departement d'épidémiologie, Hôpital Bichat | Recruiting | Paris | 75018 | France |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 25938292 | Derived | Gauvain C, Mornex JF, Pison C, Cuvelier A, Balduyck M, Pujazon MC, Fournier M, AitIlalne B, Thabut G. Health-related quality of life in patients with alpha-1 antitrypsin deficiency: the French experience. COPD. 2015 May;12 Suppl 1:46-51. doi: 10.3109/15412555.2015.1022645. | |
| 24525449 | Derived | Thabut G, Mornex JF, Pison C, Cuvelier A, Balduyck M, Pujazon MC, Fournier M, AitIlalne B, Porcher R. Performance of the BODE index in patients with alpha1-antitrypsin deficiency-related COPD. Eur Respir J. 2014 Jul;44(1):78-86. doi: 10.1183/09031936.00168113. Epub 2014 Feb 13. |
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| ID | Term |
|---|---|
| D019896 | alpha 1-Antitrypsin Deficiency |
| D004646 | Emphysema |
| ID | Term |
|---|---|
| D008107 | Liver Diseases |
| D004066 | Digestive System Diseases |
| D008171 | Lung Diseases |
| D012140 | Respiratory Tract Diseases |
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| D030342 |
| Genetic Diseases, Inborn |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D013352 | Subcutaneous Emphysema |
| D010335 | Pathologic Processes |
| D013568 | Pathological Conditions, Signs and Symptoms |