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Related Concept Videos

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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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COPD: Pathogenesis and Clinical Features01:20

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Non-CF bronchiectasis: Orphan disease no longer.

Jaafer Saadi Imam1, Alexander G Duarte1

  • 1Division of Pulmonary, Critical Care and Sleep Medicine, Department of Internal Medicine, University of Texas Medical Branch (UTMB), Galveston, TX, USA.

Respiratory Medicine
|April 7, 2020
PubMed
Summary
This summary is machine-generated.

Bronchiectasis is a chronic respiratory disease causing cough and shortness of breath. Early diagnosis and tailored therapies, including airway clearance and antibiotics, can manage symptoms and slow progression.

Keywords:
BronchiectasisChronic pulmonary infectionCystic fibrosisNon-CF bronchiectasisNon-cystic fibrosis bronchiectasis

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Area of Science:

  • Pulmonology
  • Respiratory Medicine
  • Medical Diagnostics

Background:

  • Bronchiectasis is a chronic respiratory condition characterized by persistent cough and dyspnea.
  • Historically classified as cystic fibrosis or non-cystic fibrosis, non-cystic fibrosis bronchiectasis is increasingly recognized globally.
  • Advancements in chest computed tomographic imaging have improved the detection and diagnosis of bronchiectasis.

Purpose of the Study:

  • To review the current understanding and management of bronchiectasis, particularly non-cystic fibrosis bronchiectasis.
  • To highlight the importance of identifying the underlying causes of bronchiectasis for targeted treatment.
  • To discuss various therapeutic strategies, including nonpharmacologic and pharmacologic interventions.

Main Methods:

  • Review of existing literature and clinical guidelines on bronchiectasis.
  • Discussion of diagnostic approaches including chest imaging, laboratory testing, and microbiologic assessment.
  • Analysis of therapeutic options such as airway clearance, pulmonary rehabilitation, inhaled corticosteroids, inhaled antibiotics, and macrolide therapy.

Main Results:

  • Identification of the cause of bronchiectasis is crucial for initiating specific therapies.
  • Nonpharmacologic therapies like airway clearance and pulmonary rehabilitation improve symptoms.
  • Inhaled antibiotics and chronic macrolide therapy can reduce exacerbations in select patients.
  • Routine use of inhaled corticosteroids is not recommended unless comorbid asthma or COPD is present.

Conclusions:

  • Effective management of bronchiectasis involves accurate diagnosis, identification of underlying causes, and personalized treatment plans.
  • Therapeutic strategies should be tailored to the individual patient, distinguishing between cystic fibrosis and non-cystic fibrosis bronchiectasis.
  • Early intervention with appropriate therapies can significantly improve patient outcomes and slow disease progression.