TY - T1的撤军替代席拉py and outcomes in alpha-1 antitrypsin deficiency lung transplant recipients JF - European Respiratory Journal JO - Eur Respir J DO - 10.1183/13993003.00055-2019 VL - 53 IS - 5 SP - 1900055 AU - Kleinerova, Jana AU - Ging, Patricia AU - Rutherford, Colin AU - Lawrie, Iain AU - Winward, Sara AU - Eaton, Donna AU - Redmond, Karen C. AU - Egan, Jim J. Y1 - 2019/05/01 UR - //www.qdcxjkg.com/content/53/5/1900055.abstract N2 - Alpha-1 antitrypsin deficiency (AATD) accounts for 5% of lung transplants performed worldwide. Ireland has a high frequency of 1 in 25 for the Z allele and 1 in 10 for the S allele [1]. These variants are associated with decreased production of alpha-1 antitrypsin (AAT), which predisposes to unprotected proteolytic activity of neutrophil elastase (NE) and proteinase 3 (PR3), linked to an increased risk of emphysema and hepatic disease. Associated inflammatory conditions such as vasculitis, panniculitis and inflammatory bowel disease are also suspected to be caused by uncontrolled neutrophil proteinase activity [2–4]. Vascular problems, including aortic aneurysm, in AATD patients are more severe and may reflect abnormal elastic tissue properties potentially impacting on wound healing [5].Our study suggests an association between timing of withdrawal of replacement therapy and bronchial anastomotic complications after lung transplantation http://ow.ly/jhuP30o9dSS ER -