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Cystic fibrosis year in review 2018, part 1

Adrienne P Savant1,2,3, Susanna A McColley1,2,3

  • 1Department of Pediatrics, Northwestern University Feinberg School of Medicine, Chicago, Illinois.

Pediatric Pulmonology
|May 21, 2019
PubMed

Insights

In 2018, cystic fibrosis research focused on CFTR modulator therapies, inflammation, infection, and disease assessment. This summary highlights key findings and case reports from the year.

Area of Science:

  • Pulmonology
  • Genetics
  • Medical Research

Background:

  • Cystic fibrosis (CF) is a genetic disorder affecting multiple organs.
  • Significant advancements in understanding CF pathophysiology and treatment were made.
  • 2018 saw a surge in research, particularly concerning novel therapeutic approaches.

Purpose of the Study:

  • To provide a comprehensive overview of 2018 cystic fibrosis research.
  • To summarize key findings in CFTR modulator therapies.
  • To report on advancements in understanding inflammation, infection, and disease progression.

Main Methods:

  • Literature review of 2018 publications on cystic fibrosis.
  • Analysis of case reports and clinical trial outcomes.
  • Synthesis of data on epidemiology, physiology, and imaging.

Main Results:

  • CFTR modulator therapies showed promising results in clinical studies.
  • Research elucidated the complex interplay of inflammation and infection in CF.
  • Epidemiologic data and advanced imaging techniques refined disease assessment.

Conclusions:

  • 2018 was a pivotal year for cystic fibrosis research, especially regarding modulator therapies.
  • Continued investigation into inflammation, infection, and disease progression is crucial.
  • Integrated approaches combining therapeutics and monitoring are advancing patient care.

Keywords:
antibiotic therapyclinical trialscystic fibrosis (CF)epidemiologypulmonary function testing (PFT)

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