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Updated: Mar 17, 2026

Evaluation of Respiratory System Mechanics in Mice using the Forced Oscillation Technique
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Evaluation of Respiratory System Mechanics in Mice using the Forced Oscillation Technique

Published on: May 15, 2013

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Respiratory System Disease

Danielle M Goetz1, Shipra Singh1

  • 1Pediatric Pulmonology, Jacobs School of Medicine, Women & Children's Hospital of Buffalo, State University of New York, 219 Bryant Street, Buffalo, NY 14222, USA.

Pediatric Clinics of North America
|July 30, 2016
PubMed

Insights

Cystic fibrosis (CF) primarily affects the respiratory system, leading to infections and lung disease. Emerging CFTR-targeted therapies offer new hope for managing this complex genetic disorder.

Area of Science:

  • Pulmonology
  • Genetics
  • Infectious Diseases

Background:

  • Cystic fibrosis (CF) is a genetic disorder primarily impacting the respiratory system, causing significant morbidity and mortality.
  • Defects in the cystic fibrosis transmembrane regulator (CFTR) gene lead to sinopulmonary infections by various pathogens, including Pseudomonas aeruginosa.
  • Early-onset lung disease in CF necessitates effective monitoring and treatment strategies.

Purpose of the Study:

  • To review the current understanding of sinopulmonary disease in cystic fibrosis.
  • To discuss current and emerging treatment modalities for CF-related lung disease.
  • To highlight the potential of personalized medicine, specifically CFTR modulators, in altering disease trajectory.

Main Methods:

  • Literature review of sinopulmonary involvement in cystic fibrosis.
  • Analysis of current therapeutic approaches including physiotherapy, medications, and surgery.
  • Examination of the role of novel CFTR correctors and potentiators.

Main Results:

  • Respiratory system involvement is the primary driver of morbidity and mortality in CF.
  • Recurrent sinopulmonary infections are a hallmark of the disease, caused by pathogens like P. aeruginosa.
  • Debate exists regarding optimal methods for monitoring lung function decline or improvement.

Conclusions:

  • Effective management of CF sinopulmonary disease requires a multi-faceted approach.
  • Personalized medicine, particularly CFTR modulators, represents a significant advancement in CF treatment.
  • These novel therapies hold the potential to fundamentally change the course of cystic fibrosis lung disease.
Keywords:
BronchiectasisCFTRCystic fibrosisLung diseaseLung functionPulmonary exacerbation

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