WS17.8 Inhibition of leukotriene B4 signaling by alpha-1 antitrypsin: support for the use of aerosolized alpha-1 antitrypsin therapy in cystic fibrosis
نویسندگان
چکیده
منابع مشابه
[Association between alpha 1 antitrypsin deficiency and cystic fibrosis severity].
OBJECTIVE To ascertain the distribution of alpha 1 antitrypsin genotypes and correlate it with the severity of pulmonary disease in patients with cystic fibrosis. METHOD A clinical and laboratory cross sectional study of 70 patients at the Universidade Estadual de Campinas teaching hospital. Cystic fibrosis diagnoses was confirmed by both clinical and laboratory methods. The severity of cysti...
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Alpha 1-antitrypsin deficiency is associated with pulmonary emphysema and liver cirrhosis. The pathogenesis and treatment is reviewed with particular emphasis on gene therapy for emphysema. The technology of gene transfer using viruses and liposomes is developing fast and animal experiments have confirmed the feasibility of gene therapy for alpha 1-antitrypsin deficiency (AATD). So far only sub...
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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...
متن کاملAugmentation therapy in alpha(1)-antitrypsin deficiency.
alpha(1)-Antitrypsin (AAT) deficiency is a common but under-recognised condition. Since its first description by Laurell and Eriksson in 1963, significant advances have been made in understanding the genetics, physiology and pathophysiology of this condition. The intravenous administration of purified AAT to AAT-deficient individuals has been shown to confer biochemical efficacy by raising the ...
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ژورنال
عنوان ژورنال: Journal of Cystic Fibrosis
سال: 2012
ISSN: 1569-1993
DOI: 10.1016/s1569-1993(12)60126-x