UNLABELLED: PURPOSE of this study was to determine the prevalence of bronchiectasis in patients suffering from alpha-1-antitrypsin deficiency and to compare its extent with the frequency of infections. MATERIAL AND METHODS: High-resolution CT examinations (HRCT) of 23 patients with alpha-1-antitrypsin deficiency were retrospectively assessed for extent, severity and localisation of bronchiectasis, bronchial wall thickening and extent of emphysema. Chest radiographs and clinical records were available for correlation. RESULTS: HRCT scans showed bronchiectasis in 14 of 23 patients, bronchial wall thickening in 3/14, and panlobular emphysema in 23/23. Chest radiographs showed bronchiectasis in 4/23 patients. Only two of 9 patients without bronchiectasis had a history of infections; all 8 patients with multiple bronchiectasis had a history of recurrent infections. CONCLUSION: Bronchiectasis is a common finding in alpha-1-antitrypsin deficiency, and is associated with recurrent infections. HRCT is superior to chest radiography as an indicator for the risk of infection in these patients.
UNLABELLED: PURPOSE of this study was to determine the prevalence of bronchiectasis in patients suffering from alpha-1-antitrypsin deficiency and to compare its extent with the frequency of infections. MATERIAL AND METHODS: High-resolution CT examinations (HRCT) of 23 patients with alpha-1-antitrypsin deficiency were retrospectively assessed for extent, severity and localisation of bronchiectasis, bronchial wall thickening and extent of emphysema. Chest radiographs and clinical records were available for correlation. RESULTS: HRCT scans showed bronchiectasis in 14 of 23 patients, bronchial wall thickening in 3/14, and panlobular emphysema in 23/23. Chest radiographs showed bronchiectasis in 4/23 patients. Only two of 9 patients without bronchiectasis had a history of infections; all 8 patients with multiple bronchiectasis had a history of recurrent infections. CONCLUSION: Bronchiectasis is a common finding in alpha-1-antitrypsin deficiency, and is associated with recurrent infections. HRCT is superior to chest radiography as an indicator for the risk of infection in these patients.
Authors: Timm Greulich; Christoph Nell; Christian Herr; Claus Vogelmeier; Viktor Kotke; Stefan Wiedmann; Marion Wencker; Robert Bals; Andreas Rembert Koczulla Journal: Orphanet J Rare Dis Date: 2016-06-10 Impact factor: 4.123