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Impaired release of antimicrobial peptides into nasal fluid of hyper-IgE and CVID patients. | LitMetric

AI Article Synopsis

  • Patients with primary immunodeficiency (PID) often experience frequent respiratory infections despite receiving standard treatments, leading researchers to believe there may be additional immune deficiencies.
  • The study aimed to determine if PID patients have reduced production of antimicrobial peptides (AMPs) in their nasal fluid and explore the relationship between AMPs and Th17 cell function.
  • Results showed that while healthy controls and most PID patients increase AMPs in response to bacteria, those with common variable immunodeficiency (CVID) and Hyper-IgE syndrome (HIES) do not, indicating a potential issue with their immune response linked to Th17 cell dysfunction.

Article Abstract

Background: Patients with primary immunodeficiency (PID) often suffer from frequent respiratory tract infections. Despite standard treatment with IgG-substitution and antibiotics many patients do not improve significantly. Therefore, we hypothesized that additional immune deficits may be present among these patients.

Objective: To investigate if PID patients exhibit impaired production of antimicrobial peptides (AMPs) in nasal fluid and a possible link between AMP-expression and Th17-cells.

Methods: Nasal fluid, nasopharyngeal swabs and peripheral blood mononuclear cells (PBMCs) were collected from patients and healthy controls. AMP levels were measured in nasal fluid by Western blotting. Nasal swabs were cultured for bacteria. PBMCs were stimulated with antigen and the supernatants were assessed for IL-17A release by ELISA.

Results: In healthy controls and most patients, AMP levels in nasal fluid were increased in response to pathogenic bacteria. However, this increase was absent in patients with common variable immunodeficiency (CVID) and Hyper-IgE syndrome (HIES), despite the presence of pathogenic bacteria. Furthermore, stimulation of PBMCs revealed that both HIES and CVID patients exhibited an impaired production of IL-17A.

Conclusion: CVID and HIES patients appear to have a dysregulated AMP response to pathogenic bacteria in the upper respiratory tract, which could be linked to an aberrant Th17 cell response.

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Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3246483PMC
http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0029316PLOS

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