Alpha-1 Antitrypsin Deficiency: Have We Got the Right Proteinase?
نویسندگان
چکیده
منابع مشابه
Alpha 1 antitrypsin deficiency.
Alpha-1 antitrypsin deficiency is an inherited disorder that may cause severe lung and liver disease.
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Introduction: α1-antitrypsin deficiency (α1-ATD) is one of the most common genetic disorders in white race, a usual cause of liver disease in children, and hepatopulmonary involvement in children and adult. The aim of this case description is presenting a child with early lung disease without liver parenchymal disorder. Case presentation: We describe a 13 year old boy because of exertional dysp...
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1 Computational Systems Biology Group, Center for Biomedical Technology, Universidad Politécnica de Madrid, Madrid, Spain 2 Departamento de Engenharia Electrotecnica, Faculdade de Ciencias e Tecnologia, Universidade Nova de Lisboa, Lisboa, Portugal 3 Innaxis Foundation & Research Institute, Madrid, Spain 4 Laboratory of Biological Networks, Center for Biomedical Technology, Universidad Politécn...
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Alpha-1 antitrypsin (AAT) deficiency remains an underrecognized genetic disease with predominantly pulmonary and hepatic manifestations. AAT is derived primarily from hepatocytes; however, macrophages and neutrophils are secondary sources. As the natural physiological inhibitor of several proteases, most importantly neutrophil elastase (NE), it plays a key role in maintaining pulmonary protease...
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ژورنال
عنوان ژورنال: Chronic Obstructive Pulmonary Diseases: Journal of the COPD Foundation
سال: 2020
ISSN: 2372-952X
DOI: 10.15326/jcopdf.7.3.2019.0151