Antitrypsin Deficiency Assessment and Programme for Treatment (ADAPT): The United Kingdom Registry

References

• Parr DG, Stoel BC, Stolk J, Stockley RA. Pattern of emphysema distribution in α-1-antitrypsin deficiency influences lung function impairment. Amer J Respir Crit Care Med 2004; 170(11):1172–1178.
   PubMed Web of Science ®Google Scholar
• Needham M, Stockley RA. Exacerbations in α-1-antitrypsin deficiency. Eur Respir J 2005; 25:992–1000.
   PubMed Web of Science ®Google Scholar
• Dawkins PA, Dawkins CL, Wood AM, et al. Rate of progression of lung function impairment in alpha-1-antitrypsin deficiency. Eur Respir J 2009; 33:1338–1344.
   PubMed Web of Science ®Google Scholar
• Stockley RA, Hill AT, Hill SL, Campbell EJ. Bronchial inflammation. Its relationship to colonising microbial load and α1-antitrypsin deficiency. Chest 2000; 117(suppl):291S–293S.
   PubMed Web of Science ®Google Scholar
• Hill AT, Bayley DL, Campbell EJ, et al. Airways inflammation in chronic bronchitis: the effects of smoking and α1-antitrypsin deficiency. Eur Respir J 2000; 15:886–890.
   PubMed Web of Science ®Google Scholar
• Dowson LJ, Guest PJ, Hill SL, et al. High-resolution computed tomography scanning in α1-antitrypsin deficiency: relationship to lung function and health status. Eur Respir J 2001; 17:1097–1104.
   PubMed Web of Science ®Google Scholar
• Dowson LJ, Guest PJ, Stockley RA. Longitudinal changes in physiological, radiological, and health status measurements in a1-antitrypsin deficiency and factors associated with decline. Am J Respir Crit Care Med 2001; 164:1805–1809.
   PubMed Web of Science ®Google Scholar
• Ward H, Turner AM, Stockley RA. Spirometric and gas transfer discordance in alpha-1-antitrypsin deficiency; patient characteristics and progression. Chest 2014; 145(6):1316–1324.
   PubMed Web of Science ®Google Scholar
• Holme J, Stockley RA. Radiologic and clinical features of COPD patients with discordant pulmonary physiology: Lessons from α-1-antitrypsin deficiency. Chest 2007; 132:909–915.
   PubMed Web of Science ®Google Scholar
• Wood AM, Needham M, Simmonds MJ, et al. Phenotypic differences in alpha 1 antitrypsin deficient sibling pairs may relate to genetic variation. J COPD 2008; 5:353–359.
   PubMedGoogle Scholar
• Parr DG, Guest PG, Reynolds JH, et al. Prevalence and impact of bronchiectasis in α1-antitrypsin deficiency. Amer J Respir Crit Care Med 2007; 176:1215–1221.
   PubMed Web of Science ®Google Scholar
• Dowson LJ, Guest PJ, Hill SL, et al. High-resolution computed tomography scanning in α1-antitrypsin deficiency: relationship to lung function and health status. Eur Respir J 2001; 17:1097–1104.
   Google Scholar
• Vijayasaratha K, Stockley RA. Relationship between frequency, length, and treatment outcome of exacerbations to baseline lung function and lung density in alpha-1 antitrypsin-deficient COPD. Inter J COPD 2012; 7:789–796.
   Web of Science ®Google Scholar
• Dirksen A, Dijkman JH, Madsen F, et al. A randomised clinicl trial of alpha(1)-antitrypsin augmentation therapy. Am J Respir Crit Care Med 1999; 160(5 Pt 1):1468–1472.
   PubMed Web of Science ®Google Scholar
• Dirksen A, Piitulainen E, Parr DG, et al. Exploring the role of CT densitometry: a randomised study of augmentation therapy in alpha-1-antitrypsin deficiency. Eur Respir J 2009; 33:1345–1353.
   PubMed Web of Science ®Google Scholar
• Holme J, Stockley JA, Stockley RA. Age related development of respiratory abnormalities in non-index α−1 antitrypsin deficient studies Respir Med 2013; 107:387–393.
   PubMed Web of Science ®Google Scholar
• Piitulainen E, Montero LC, Nystedt-Düzakin M, Stoel BC, Sveger T, Wollmer P, Tanash HA, Diaz S. Lung function and CT densitometry in subjects with alpha-1-antitrypsin deficiency and healthy controls at 35 years of age. COPD 2014; Oct 3 Epub ahead of print.
   PubMedGoogle Scholar
• American Thoracic Society/European Respiratory Society statement: standards for the diagnosis and management of individuals with alpha-1 antitrypsin deficiency. American Thoracic Society; European Respiratory Society. Am J Respir Crit Care Med 2003; 168(7):818–900.
   PubMed Web of Science ®Google Scholar
• Stockley RA, Miravitlles M, Vogelmeier C. Augmentation therapy for alpha-1 antitrypsin deficiency: towards a personalised approach. Orphanet J Rare Dis 2013; 8:149.
   PubMed Web of Science ®Google Scholar
• Holme J, Dawkins PA, Stockley EK, et al. Studies of gamma-glutamyl 1 transferase in alpha-1 antitrypsin deficiency. COPD 2010; 7(20):126–132.
   PubMedGoogle Scholar
• Stockley RA, Bayley DL, Unsal I, Dowson L. The effect of augmentation therapy on bronchial inflammation in α1-antitrypsin deficiency. Am J Respir Crit Care Med 2002; 165:1494–1498.
   PubMed Web of Science ®Google Scholar
• Stolk J, Cooper BG, Stoel B, et al. Retinoid treatment of emphysema in patients on the Alpha-1 International Regsitry. The REPAIR study: study design, methodology and quality control of study assessments. Ther Adv Respir Dis 2010; 4(6):319–332.
   PubMedGoogle Scholar
• International study evaluating the safety and efficacy of inhaled human alpha-1 antitrypsin (AAT) in alpha-1 antitrypsin deficient patients with emphysema. 2010 Clinical Trials Gov NCT01217671.
   Google Scholar
• Chapman KR, Stockley RA, Dawkins C, et al. Augmentation therapy for alpha-1 antitrypsin deficiency: A meta-analysis. J COPD 2009; 6(3):177–184.
   PubMedGoogle Scholar
• Stone H, Pye A, Stockley RA. Disease associations in alpha-1-antitrypsin deficiency. Respir Med 2014; 108:338–343.
   PubMed Web of Science ®Google Scholar
• Wood AM, Simmonds MJ, Bayley DL, et al. The TNF alpha gene relates to clinical phenotype in alpha-1-antitrypsin deficiency. Respir Res 2008; 9(52):1–8.
   PubMedGoogle Scholar
• McAloon CJ, Wood AM, Gough SC, Stockley RA. Matrix metalloprotease polymorphisms are associated with gas transfer in alpha 1 antitrypsin deficiency. Ther Adv Respir Dis 2009; 3:23–30.
   PubMedGoogle Scholar
• Carter RI, Mumford RA, Treonze KM, et al. The fibrinogen cleavage product Aa-Val360, a specific marker of neutrophil elastase activity in vivo. Thorax 2011; 66:686–691.
   PubMed Web of Science ®Google Scholar
• Carter R, Ungurs M, Pillai A, et al. The relationship of the fibrinogen cleavage biomarker Aα-Val 360 with disease severity and activity in lpha-1-antitrypsin deficiency. Chest 2015; in press.
   PubMedGoogle Scholar
• Parr DG, Dirksen A, Piitulainen E, et al. Exploring the optimum approach to the use of CT densitometry in a randomised placebo-controlled study augmentation therapy in alpha-1-antitrypsin deficiency. Respir Res 2009; 10:75.
   PubMed Web of Science ®Google Scholar