Abstract
Pulmonary exacerbations are common events in cystic fibrosis and have a profound impact on quality of life, morbidity, and mortality. Pulmonary exacerbation outcomes remain poor and a significant proportion of patients fail to recover their baseline lung function despite receiving aggressive treatment with intravenous antibiotics. This focused review provides an update on some of the recent advances that have taken place in our understanding of the epidemiology, pathophysiology, diagnosis, and management of pulmonary exacerbations in cystic fibrosis as well as direction for future study.
Highlights
Despite improvements in lung function and nutritional outcomes for individuals with cystic fibrosis (CF) over the past decade, pulmonary exacerbation (PEx) remains common
Exacerbations have a profound impact on the morbidity and quality of life of individuals with CF, and PEx outcomes remain suboptimal with poor recovery of baseline lung function following PEx treatment[2]
The focus of this review is to summarize some of the recent advances that have taken place in our understanding of the epidemiology, pathophysiology, diagnosis, and management of PEx in CF and to provide direction for future study
Summary
Despite improvements in lung function and nutritional outcomes for individuals with cystic fibrosis (CF) over the past decade, pulmonary exacerbation (PEx) remains common. Patients are not randomly assigned and the intensity of treatments is not standardized between comparisons; patients in hospital might receive more intensive treatments Another recent study that used the ESCF employed statistical approaches to control for indication bias and found that PEx with a greater proportion of days treated as an inpatient (versus outpatient) with IV antibiotics was more likely to lead to return of FEV1 % predicted to at least 90% of baseline[44]. This observational study is not definitive (as there was likely residual confounding), the benefits of inpatient PEx treatment are likely due to multiple factors other than the Figure 1. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript
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