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The WinCF Model - An Inexpensive and Tractable Microcosm of a Mucus Plugged Bronchiole to Study the Microbiology of Lung Infections
Published on: May 8, 2017
Mucus, phlegm, and sputum in cystic fibrosis
1Department of Pediatrics, Wake Forest University School of Medicine, Medical Center Boulevard, Winston-Salem NC 27157-1081, USA. brubin@wfubmc.edu
Abstract:
Although some clinicians still believe that cystic fibrosis (CF) lung disease is largely due to hypersecretion of very viscous mucus, it has never been demonstrated that there is mucus hypersecretion in CF and it is clear that there is almost no intact mucin (the principal polymeric component of normal mucus) in CF sputum. CF sputum has lower viscosity when compared to asthma or bronchitis sputa, but is highly tenacious and biochemically most closely resembles pus. Tenacity and lower viscosity lead to decreased cough clearance of infected phlegm, which is thought to induce a persistent inflammatory state in the airway, leading to bronchiectasis. There are many medications and devices either in use or under development that are meant to improve airway hygiene in CF by assisting with sputum expectoration. This paper discusses the scientific basis and potential mechanism of action for many of these interventions and briefly reviews the clinical evidence of their safety and effectiveness.
Insights
Cystic fibrosis (CF) sputum is not hypersecreted but is tenacious, hindering airway clearance. This review examines interventions aimed at improving mucus expectoration and airway hygiene in CF patients.
Area of Science:
- Pulmonary Medicine
- Biochemistry
- Medical Devices
Background:
- Cystic fibrosis (CF) lung disease is often mistakenly attributed to excessive, viscous mucus secretion.
- CF sputum lacks intact mucin, the primary component of normal mucus, and biochemically resembles pus.
- This tenacious sputum impairs effective cough clearance, promoting chronic airway inflammation and bronchiectasis.
Purpose of the Study:
- To critically evaluate the scientific basis and proposed mechanisms of action for various airway hygiene interventions in CF.
- To review the existing clinical evidence regarding the safety and efficacy of these expectoration-assisting therapies.
Main Methods:
- Literature review and analysis of scientific principles.
- Examination of biochemical properties of CF sputum.
- Assessment of clinical trial data for airway clearance therapies.
Main Results:
- CF sputum's tenacity, not viscosity, is the primary factor impeding clearance.
- Interventions focus on enhancing mucus expectoration through various physical and pharmacological means.
- Evidence for safety and effectiveness varies among different airway clearance strategies.
Conclusions:
- Understanding the true nature of CF sputum is crucial for developing effective airway clearance strategies.
- Numerous interventions aim to improve mucus expectoration, but their clinical utility requires careful consideration of the underlying mechanisms.
- Further research is needed to optimize therapies for managing CF lung disease through enhanced airway hygiene.
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