Contamination of werum by certain gram-negative bacteria has been shown to spoil the serum for measurement of trypsin inhibitory capacity (STIC) or for antitrypsin phenotyping. Such sera develop intense fibrinolytic activity when the STIC has dropped to itsminimal level, but antitrypsin concentration as measured by radial immunodiffusion remainsconstant. Cultures of ENTEROBACTER, Klebsiella, Bacillus subtilis, and Pseudomonas species were shown to have this capability, but production of the fibrinolytic enzyme by the bacteria was most proficient in the presence of human serum. The enzyme is believed to be of bacterial origin because of its lack of esterase activity, and because activation of serum plasmin by streptokinase did not affect the STIC. Care mustbe taken to avoid bacterial contamination of blood that is to be submitted for an STICassay and/or antitrypsin phenotyping. Serum should be prepared quickly, frozen soon,and stored and transported in a frozen state.
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Acta Cardiol
January 2025
Department of Cardiology, Université Catholique de Louvain, CHU UCL Namur, Yvoir, Belgium.
Int J Chron Obstruct Pulmon Dis
December 2024
Department of Clinical Sciences, Lund University, Lund, Sweden.
Background: The risk of coronavirus (COVID-19) can be affected by the presence of certain chronic conditions. It is unknown if individuals with severe hereditary alpha-1-antitrypsin deficiency (AATD) faced an increased risk of severe COVID-19 infection during the pandemic and if COPD in this population affected the risk of severe COVID-19 outcomes.
Aim: Our aim was to investigate COVID-19 outcomes in individuals with severe AATD and to identify if COPD was a risk factor for severe disease.
J Bras Pneumol
December 2024
. Vall d'Hebron Institut de Recerca - VHIR - Hospital Universitário Valld'Hebron, Barcelona, España.
Alpha-1 antitrypsin deficiency (AATD) is a relatively rare genetic disorder, inherited in an autosomal codominant manner, that results in reduced serum AAT concentrations, with a consequent reduction in antielastase activity in the lungs, as well as an increased risk of diseases such as pulmonary emphysema, liver cirrhosis, and necrotizing panniculitis. It results from different mutations in the SERPINA1 gene, leading to changes in the AAT glycoprotein, which can alter its concentration, conformation, and function. Unfortunately, underdiagnosis is quite common; it is possible that only 10% of cases are diagnosed.
View Article and Find Full Text PDFJ Cell Mol Med
October 2024
Department of Surgery, Medical University of South Carolina, Charleston, South Carolina, USA.
J Clin Transl Hepatol
October 2024
Division of Pulmonary, Critical Care and Sleep Medicine, College of Medicine, University of Florida, Gainesville, FL, USA.
Background And Aims: Alpha-1 antitrypsin deficiency (AATD) is a genetic disorder characterized by the misfolding and accumulation of the mutant variant of alpha-1 antitrypsin (AAT) within hepatocytes, which limits its access to the circulation and exposes the lungs to protease-mediated tissue damage. This results in progressive liver disease secondary to AAT polymerization and accumulation, and chronic obstructive pulmonary disease (COPD) due to deficient levels of AAT within the lungs. Our goal was to characterize the unique effects of COPD secondary to AATD on liver disease and gene expression.
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