Therapeutic potential of alpha-1 antitrypsin in human disease

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Alpha-1- Antitrypsin Deficiency Liver Disease: Science and Therapeutic Potential 50 Years Later

A1AT deficiency has a complex pathophysiology, is highly variable in clinical course, and is under diagnosed. The association with chronic lung disease was first described by Eriksson and Laurell in 1963, and later, Sharp and colleagues recognized A1AT deficiency as a cause of liver disease. In 2013 an international meeting was held to review the intervening 50 years of basic and clinical scien...

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Assessment of Alpha-1 Antitrypsin Deficiency in Patients with Severe Chronic Obstructive Pulmonary Disease

Background and Aims: Chronic obstructive pulmonary disease (COPD) is a kind of pulmonary diseases characterized by chronic obstruction of lung that is in the form of a diffuse narrowing of airways resulting in air flow resistance. Alpha-1 antitrypsin (AAT) deficiency is genetically relatively common risk factor in patients with COPD throughout the world and the exact cause of its prevalence is ...

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Alpha 1-antitrypsin in human macrophages.

Preliminary studies have suggested that alpha-1-antitrypsin (A1AT) is a useful immunohistochemical marker of histiocytes (monocytes/macrophages) and malignant tumours derived from them. To confirm the reliability of this marker a wide variety of benign and malignant lymphoreticular cells and tissues have been stained by the immunoperoxidase technique for A1AT and positive staining was found to ...

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Liver disease and alpha 1-antitrypsin deficiency.

Discovery of AAT deficiency by Laurell and Eriksson in 1963 [2] provided a foundation for current thinking about the pathogenesis of pulmonary emphysema [3,4]. Although AAT deficiency has become one of the best understood genetic disorders at a molecular and protein level, many questions about the clinical disease remain unanswered. Current American and International research projects should pr...

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Alpha- 1 Antitrypsin Deficiency in Children: Pulmonary Involvement

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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ژورنال

عنوان ژورنال: Annals of Pediatric Endocrinology & Metabolism

سال: 2018

ISSN: 2287-1012,2287-1292

DOI: 10.6065/apem.2018.23.3.131