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Related Concept Videos

Cystic Fibrosis: Pathogenesis01:23

Cystic Fibrosis: Pathogenesis

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Cystic fibrosis (CF), an autosomal recessive disorder, significantly affects the function of exocrine glands. This genetically inherited disease is characterized by the production of thick and sticky mucus, which can severely affect various organs and systems in the body.
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Cystic fibrosis (CF) is an autosomal recessive disorder that predominantly affects individuals of Northern European descent, occurring at a rate of 1 in 3500. It is caused by a genetic mutation in a gene on chromosome 7, most commonly the ΔF508 mutation, that codes for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. This results in thicker mucus secretions and obstruction pathologies in multiple organs, including the lungs and sinuses.
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Rheumatic heart disease or RHD is a chronic condition that results from rheumatic fever, causing permanent damage to the heart valves.Etiology and Risk FactorsIt primarily arises from rheumatic fever, an inflammatory disease that can develop after untreated or inadequately treated group A streptococcal (GAS) pharyngitis. Streptococcus spreads through direct contact with oral or respiratory secretions. While the bacteria are the causative agents, factors like malnutrition, overcrowding, poor...
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Related Experiment Video

Updated: Nov 27, 2025

Implementation of Non-invasive Point of Care Transient Elastography for Evaluation of Liver Disease in Pediatric Populations with Cystic Fibrosis
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Vasculitis in Cystic Fibrosis.

Francesca Sposito1, Paul S McNamara1, Christian M Hedrich1,2

  • 1Department of Women's and Children's Health, Institute of Life Course and Medical Sciences, University of Liverpool, Liverpool, United Kingdom.

Frontiers in Pediatrics
|December 7, 2020
PubMed
Summary

Cystic fibrosis (CF) vasculitis involves inflammation and immune cell activation, potentially driven by bacterial infections and leading to autoantibodies. Treatment strategies are evolving, exploring immune modulation and CFTR modulators.

Keywords:
cystic fibrosisdamageimmune complexinflammationpathophysiologytreatmentvasculitis

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Area of Science:

  • Immunology
  • Rheumatology
  • Pulmonology

Background:

  • Cystic fibrosis (CF) is a multi-organ disease with airway obstruction and inflammation.
  • Vasculitis is a severe complication in 2-3% of CF patients, often linked to a poor prognosis.

Purpose of the Study:

  • To review the molecular mechanisms, clinical presentation, and treatment of CF-associated vasculitis.
  • To explore current and future therapeutic options for this condition.

Main Methods:

  • Review of published case reports and small case series on CF-associated vasculitis.
  • Summary of proposed pathogenic mechanisms including bacterial colonization, ANCA production, and immune complex formation.

Main Results:

  • CF-associated vasculitis involves persistent inflammation, immune cell activation, and potential immune complex deposition.
  • Current treatments include NSAIDs, corticosteroids, hydroxychloroquine, and DMARDs, but carry risks.

Conclusions:

  • CF-associated vasculitis is complex, involving a interplay of infection, inflammation, and autoimmunity.
  • Future treatments may focus on targeted cytokine blockade or novel CFTR modulators to mitigate risks associated with immunosuppression.