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New Patient-Centric Approaches to the Management of Alpha-1 Antitrypsin Deficiency

<p>New Patient-Centric Approaches to the Management of Alpha-1 Antitrypsin Deficiency</p>

... Abstract: Alpha-1 antitrypsin de fi ciency (AATD) is a rare and underdiagnosed genetic predisposition for COPD and emphysema and other conditions, including liver ...European Alpha-1 Research ...

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Alpha 1 antitrypsin to treat lung disease in alpha 1 antitrypsin deficiency: recent developments and clinical implications

Alpha 1 antitrypsin to treat lung disease in alpha 1 antitrypsin deficiency: recent developments and clinical implications

... Abstract: Alpha 1 antitrypsin deficiency is a hereditary condition characterized by low alpha 1 proteinase inhibitor (also known as alpha 1 antitrypsin [AAT]) serum ...in ...

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Enhanced inositol trisphosphate response to alpha 1 adrenergic stimulation in cardiac myocytes exposed to hypoxia

Enhanced inositol trisphosphate response to alpha 1 adrenergic stimulation in cardiac myocytes exposed to hypoxia

... to alpha 1-adrenergic stimulation and a reversible increase in alpha 1-adrenergic receptor ...myocytes, alpha 1-adrenergic receptor number increases two- to threefold after 10 ...

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Alpha-1 proteinase inhibitors for the treatment of alpha-1 antitrypsin deficiency: safety, tolerability, and patient outcomes

Alpha-1 proteinase inhibitors for the treatment of alpha-1 antitrypsin deficiency: safety, tolerability, and patient outcomes

... Abstract: Alpha-1 antitrypsin (AAT) deficiency remains an underrecognized genetic disease with predominantly pulmonary and hepatic manifestations. AAT is derived primarily from hepatocytes; however, ...

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Expression of the alpha 1 antitrypsin gene in mononuclear phagocytes of normal and alpha 1 antitrypsin deficient individuals

Expression of the alpha 1 antitrypsin gene in mononuclear phagocytes of normal and alpha 1 antitrypsin deficient individuals

... to alpha-1-antitrypsin (alpha 1AT) production in normal and alpha 1AT- deficient individuals, Northern analysis with a human alpha 1AT complementary DNA was used to demonstrate that ...

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Safety and efficacy of alpha-1-antitrypsin augmentation therapy in the treatment of patients with alpha-1-antitrypsin deficiency

Safety and efficacy of alpha-1-antitrypsin augmentation therapy in the treatment of patients with alpha-1-antitrypsin deficiency

... Abstract: Alpha-1-antitrypsin deficiency (AATD), also known as alpha1-proteinase inhibitor deficiency, is an autosomal co-dominant ...dysfunctional alpha-1-antitrypsin (A1AT) variants, which ...

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Hypersensitivity Vasculitis with Leukocytoclastic Vasculitis Associated with Alpha-1-Proteinase Inhibitor

Hypersensitivity Vasculitis with Leukocytoclastic Vasculitis Associated with Alpha-1-Proteinase Inhibitor

... Here we describe a patient who developed HV 2 weeks following her 2nd Prolastin infusion with fulminant skin disease. As mentioned previously drugs are the most common offending agents, but drug-induced vasculitis is a ...

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Compensatory increase in alpha 1 globin gene expression in individuals heterozygous for the alpha thalassemia 2 deletion

Compensatory increase in alpha 1 globin gene expression in individuals heterozygous for the alpha thalassemia 2 deletion

... in alpha-globin gene activity secondarily to the loss of function of one or more of these genes (alpha-thalassemia [Thal]) have not been directly ...human alpha-globin genes by determining the ...

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Alpha-1-antitrypsin augmentation therapy in deficient individuals enrolled in the Alpha-1 Foundation DNA and Tissue Bank

Alpha-1-antitrypsin augmentation therapy in deficient individuals enrolled in the Alpha-1 Foundation DNA and Tissue Bank

... FEV 1 associated with a submaximal expiratory effort (unfortunately peak flow was not ...the Alpha-1-Antitrypsin Deficiency Registry Study Group, some patients had a positive FEV 1 slope (in ...

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Differentiation of the mRNA transcripts originating from the alpha 1  and alpha 2 globin loci in normals and alpha thalassemics

Differentiation of the mRNA transcripts originating from the alpha 1 and alpha 2 globin loci in normals and alpha thalassemics

... deficient alpha- globin production and results in ...the alpha 1- and alpha-2-globin messenger RNA (mRNA) transcripts and thus delineate the relative level of expression of the two ...

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Treatment of lung disease in alpha-1 antitrypsin deficiency: a systematic review

Treatment of lung disease in alpha-1 antitrypsin deficiency: a systematic review

... involved alpha-1 antitrypsin augmentation and 17 concerned surgical treatments (largely ...in 1 s, diffusing capacity of the lungs for carbon monoxide, health status, and exacerbation rates were ...

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Interaction of mouse macrophage elastase with native and oxidized human alpha 1 proteinase inhibitor

Interaction of mouse macrophage elastase with native and oxidized human alpha 1 proteinase inhibitor

... oxidized alpha 1-proteinase inhibitor (alpha 1-PI) were compared as substrates for the metalloproteinase macrophage ...native alpha 1-PI was readily degraded by macrophage ...

5

The prevalence of alpha 1 antitrypsin deficiency in Ireland

The prevalence of alpha 1 antitrypsin deficiency in Ireland

... Children, Alpha One Foundation (Ireland), Alpha-1 Foundation (USA), Health Research Board (HRB) and Medical Research Charities Group (MRCG), and the Programme for Research in Third Level Institutes ...

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The prevalence of alpha-1 antitrypsin deficiency in Ireland

The prevalence of alpha-1 antitrypsin deficiency in Ireland

... Children, Alpha One Foundation (Ireland), Alpha-1 Foundation (USA), Health Research Board (HRB) and Medical Research Charities Group (MRCG), and the Programme for Research in Third Level Institutes ...

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A comparative ultrastructural and molecular biological study on Chlamydia psittaci infection in alpha 1 antitrypsin deficiency and non alpha 1 antitrypsin deficiency emphysema versus lung tissue of patients with hamartochondroma

A comparative ultrastructural and molecular biological study on Chlamydia psittaci infection in alpha 1 antitrypsin deficiency and non alpha 1 antitrypsin deficiency emphysema versus lung tissue of patients with hamartochondroma

... included five specimens from alpha-1 antitrypsin defi- ciency (AATD) and 34 from non-AATD patients. PCR examinations were conducted on six AATD and 31 non- AATD specimens. History showed a status of ...

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Alpha 1 antitrypsin deficiency caused by the alpha 1 antitrypsin Nullmattawa gene  An insertion mutation rendering the alpha 1 antitrypsin gene incapable of producing alpha 1 antitrypsin

Alpha 1 antitrypsin deficiency caused by the alpha 1 antitrypsin Nullmattawa gene An insertion mutation rendering the alpha 1 antitrypsin gene incapable of producing alpha 1 antitrypsin

... alpha 1-Antitrypsin (alpha 1AT) deficiency is a hereditary disorder associated with reduced serum alpha 1AT levels and the development of pulmonary ...An alpha 1AT gene is defined as ...

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Mineralization of alpha-1-antitrypsin inclusion bodies in Mmalton alpha-1-antitrypsin deficiency

Mineralization of alpha-1-antitrypsin inclusion bodies in Mmalton alpha-1-antitrypsin deficiency

... Alpha-1-antitrypsin (AAT) deficiency (AATD), an auto- somal co-dominant genetic disorder, was discovered in 1963 on the base of the recurrent lack of alpha-1- globulin electrophoretic peak in ...

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The impact of alpha-1 antitrypsin augmentation therapy on neutrophil-driven respiratory disease in deficient individuals

The impact of alpha-1 antitrypsin augmentation therapy on neutrophil-driven respiratory disease in deficient individuals

... Abstract: Alpha-1 antitrypsin (AAT) is the most abundant serine protease inhibitor circulating in the ...neutrophils, alpha-1 antitrypsin deficiency, alpha-1 antitrypsin ...

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Alpha-1 antitrypsin deficiency

Alpha-1 antitrypsin deficiency

... has shown, perhaps unsurpris- ingly, that those children who progressed to end stage liver disease had more severe abnormalities in infancy. They were more likely to have remained jaundiced for more than 6 weeks, to have ...

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Alpha 1 acid glycoprotein as potential biomarker for alpha fetoprotein low hepatocellular carcinoma

Alpha 1 acid glycoprotein as potential biomarker for alpha fetoprotein low hepatocellular carcinoma

... Measurement of plasma AAG concentration was per- formed according to our previous report [14]. Briefly, the plasma AAG level was detected via the Agilent 1200 HPLC system. A 200 μ L of crude plasma was diluted with 600 μ ...

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