نتایج جستجو برای: 1 antitrypsin deficiency

تعداد نتایج: 2853876  

Journal: :European Respiratory Journal 2014

Journal: :The Journal of clinical investigation 1981
J E Gadek H G Klein P V Holland R G Crystal

The emphysema associated with the inherited serum deficiency of alpha 1-antitrypsin appears to result from an imbalance between neutrophil elastase and its major inhibitor within the alveolar structures. In the present study we assessed the feasibility of reversing this biochemical defect within the lung via parenteral replacement therapy with an alpha 1-antitrypsin concentrate of normal plasma...

Journal: :international journal of pediatrics 0
mohammad esmaeili associated prof. of pediatrics, mashhad university of medical sciences, mashhad, iran. marjan esmaeili resident of pediatrics, iran university of medical sciences, tehran, iran. sayed javad sayedi assistant prof of pediatrics, mashhad university of medical sciences, mashhad, iran. mohammad ali kiani associate prof. of pediatrics, iran university of medical sciences, tehran, iran.

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...

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...

Journal: :The Turkish journal of gastroenterology : the official journal of Turkish Society of Gastroenterology 2012
Halis Şımşek Aslı Pinar Akif Altinbaş Alparslan Alp Yasemin H Balaban Yahya Büyükaşik Osman Özcebe Gülşen Hasçelık Gökhan Gedıkoğlu Gonca Tatar

BACKGROUND/AIMS Alpha-1 antitrypsin deficiency causes accumulation of mutant alpha-1 antitrypsin molecules in hepatocytes, and is attributed to severe liver injury even in heterozygous state. However, there is a question as to whether alpha-1 antitrypsin deficiency is only a cause of liver injury or has a worsening effect on the underlying liver disease. We aimed to determine the role of alpha-...

Journal: :American Journal of Respiratory and Critical Care Medicine 2018

Journal: :Postgraduate Medical Journal 1987

Journal: :Journal of the American Academy of Dermatology 2021

Journal: :Anales de pediatria 2016
B Lara Gallego S Castillo Carullón M T Martínez Martínez M García Reymundo M Miravitlles

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