Screening for alpha1-Pi deficiency in patients with lung diseases

Screening for a1-Pi deficiency in patients with lung diseases

Screening for a1-Pi deficiency in patients with lung diseases. M. Wencker, A. Marx, N. Konietzko, B. Schaefer, E.J. Campbell. #ERS Journals Ltd 2002. ABSTRACT: In patients with pulmonary emphysema, studies have reported 2–3% of individuals with severe a1-Pi deficiency. The aims of this study were to evaluate the accuracy of a new method for quantifying a1-Pi through phenotyping from dried blood...

Lung deposition of inhaled Alpha-1-proteinase inhibitor (Alpha1-PI) - problems and experience of Alpha1-PI inhalation therapy in patients with hereditary Alpha1-PI deficiency and cystic fibrosis

Alpha-1-proteinase inhibitor (α1-PI) is the most relevant protease inhibitor in the lung. Patients with hereditary deficiency of α1-PI suffer from an impaired hepatic synthesis of α1-PI in the liver and in consequence an insufficient concentration of the protease inhibitor in the lung followed by development of lung emphysema due to an impaired protease antiprotease balance and a local relative...

Alpha1-antitrypsin deficiency allele carriers among lung cancer patients.

Lung cancer (LC) and chronic obstructive pulmonary lung diseases (COPDs; including emphysema and chronic bronchitis) share a common etiology. Despite the known associations of alpha1-antitrypsin deficiency (alpha1AD) with COPD and COPD with LC, few studies examined the association of alpha1AD alleles and LC. We hypothesize that heterozygous individuals who carry a deficient allele of the alpha1...

Glutathione S-transferase P1 and lung function in patients with alpha1-antitrypsin deficiency and COPD.

BACKGROUND The glutathione S-transferase P1 (GSTP1) gene is involved in detoxification of electrophilic substances of tobacco smoke. A polymorphism at nucleotide 315 of this gene alters its enzymatic activity. OBJECTIVE We analyzed the association between the variability in the GSTP1 gene and impairment in lung function in smokers with and without alpha(1)-antitrypsin (AAT) deficiency and COP...

Introducing an index to predict lung involvement deficiency in hypertensive patients with COVID-19