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Update on cystic fibrosis epidemiology
Christopher H Goss1, Margaret Rosenfeld
1Department of Medicine, University of Washington Medical Center, Seattle, Washington 98195, USA. goss@u.washington.edu
Current Opinion in Pulmonary Medicine
|October 29, 2004
Summary
Cystic fibrosis (CF) survival is improving, especially in younger patients. Factors like income, air pollution, and specific gene mutations significantly impact CF patient outcomes and disease progression.
Area of Science:
- Epidemiology
- Pulmonology
- Genetics
Background:
- Cystic Fibrosis (CF) is a complex multisystem disease with evolving clinical features.
- Improving survival rates necessitate understanding disease modifiers and prognosis.
- Focus on older CF patients is crucial due to increased longevity.
Purpose of the Study:
- To review recent advances in understanding CF survival predictors and disease modifiers.
- To highlight studies relevant to the growing population of older CF patients.
- To synthesize current knowledge on CF epidemiology and its impact on patient care.
Main Methods:
- Review of selected recent epidemiologic studies in cystic fibrosis.
- Analysis of factors influencing survival and disease progression.
- Examination of emerging pathogens and their impact.
Main Results:
- US CF patient survival has improved significantly in the last 15 years, primarily in younger age groups.
- Median household income and air pollutants are identified as key disease modifiers.
- Genotype, particularly mutation classes I, II, and III, strongly correlates with mortality.
- Emerging pathogens like B. cepacia complex and B. gladioli are prominent, with some patients acquiring new strains over time.
Conclusions:
- Epidemiologic research continues to guide CF patient care and clinical investigation.
- Survival and outcomes in CF demonstrate ongoing improvement.
- Longitudinal epidemiologic studies are essential for monitoring prognosis and outcomes in the aging CF population.