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
1   CFF Therapeutics Development Network Coordinating Center, Department of Pediatrics, Seattle Children's Research Institute, Seattle, Washington
2   Division of Pulmonary, Critical Care and Sleep Medicine, Department of Medicine and Pediatrics, University of Washington School of Medicine, Seattle, Washington
› Author Affiliations
Funding C.H.G.'s research time is supported by the Cystic Fibrosis Foundation Therapeutics (GOSS13A0) and NIH (P30 DK089507, UL1TR000423). There was no role of funding sources in writing of this manuscript, or the decision to submit for publication.
Further Information

Publication History

Publication Date:
28 October 2019 (online)

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.

 
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