Acute Pulmonary Exacerbations in Cystic Fibrosis

Christopher H. Goss

doi:10.1055/s-0039-1697975

Seminars in Respiratory and Critical Care Medicine, Table of Contents

Semin Respir Crit Care Med 2019; 40(06): 792-803
DOI: 10.1055/s-0039-1697975

Review Article

Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Acute Pulmonary Exacerbations in Cystic Fibrosis

Christopher H. Goss

¹CFF Therapeutics Development Network Coordinating Center, Department of Pediatrics, Seattle Children's Research Institute, Seattle, Washington

²Division of Pulmonary, Critical Care and Sleep Medicine, Department of Medicine and Pediatrics, University of Washington School of Medicine, Seattle, Washington

› Author Affiliations

Abstract

With the improving survival of cystic fibrosis (CF) patients and the advent of highly effective cystic fibrosis transmembrane conductance regulator therapy, the clinical spectrum of this complex multisystem disease continues to evolve. One of the most important clinical events for patients with CF in the course of this disease is an acute pulmonary exacerbation. Clinical and microbial epidemiology studies of CF pulmonary exacerbations continue to provide important insight into the disease course, prognosis, and complications. This work has now led to a number of large scale clinical trials with the goal of improving the treatment paradigm for CF pulmonary exacerbation. The primary goal of this review is to provide a summary of the pathophysiology, the clinical epidemiology, microbial epidemiology, outcome and the treatment of CF pulmonary exacerbation.

Keywords

FEV₁ - respiratory symptoms - pulmonary exacerbation - cystic fibrosis - treatment duration

Full Text

References

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