• Chest · Oct 2021

    Review

    Pulmonary Complications in Cystic Fibrosis: past, present and future.

    • Christina M Mingora and Patrick A Flume.
    • Department of Medicine, Division of Pulmonary, Critical Care, Allergy, and Sleep Medicine, Medical University of South Carolina, Charleston, SC. Electronic address: mingora@musc.edu.
    • Chest. 2021 Oct 1; 160 (4): 1232-1240.

    Abstract1Cystic fibrosis (CF) is an autosomal recessive genetic condition with multisystemic disease manifestations, the most prominent of which occur in the respiratory system. Despite significant developments in disease understanding and therapeutics, each contributing to improved lung function and survival in patients with CF, several pulmonary complications, including pneumothorax, massive hemoptysis, and respiratory failure, continue to occur. In this review, we briefly describe each of these complications and their management and discuss how they impact the care and disease trajectory of individuals in whom they occur. Finally, we discuss the evolving role that palliative care and CF transmembrane conductance regular modulator therapies play in the natural disease course and care of patients with CF.Copyright © 2021 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.

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