Alpha -1-antitrypsin deficiency, associated diseases and treatments: a review
Journal Title: Pakistan Journal of Pharmaceutical Research - Year 2017, Vol 3, Issue 2
Abstract
Alpha-1-antitrypsin (AAT) is produced in the liver and one of its most important function is to protect the lungs from the neutrophils elastase level enzymes, normal level of AAT in the blood is 1.5-3.5gm/ml, when the AAT is up to 60% the lungs function normally but when it drops below 15% patients are likely to develop Emphysema and liver cirrhosis. Alpha 1-antitrypsin deficiency is heterogeneous genetic disorder due to mutation of chromosome no 14q32.1 (SERPINA1) that leads to the protease anti-protease imbalance. Alveolar And hepatocyte damage were observed causes which is due to the deficiency of Alpha 1-antitrypsin. Disease effects every system of body like respiratory, hepatic and biliary. The aim of study is to evaluate various diseases associated with AATD. Data was collected from internet using sciencdirect.com, googlescholar.com, pubmed.com. The most common associated diseases with alpha 1 antitrypsin deficiency are emphysema, asthma, liver cirrhosis and hepatitis depending the deficiency level of AAT. The best treatment is considered the adminstration of anticholinergics and ipratropium bromide improves the lung functions, however use of bronchodilators improves asthmatic attack also vaccination is carried to prevent the Hepatitis and liver cirrhosis.
Authors and Affiliations
Rauf-ur- Rehman
Keywords
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