Pulmonary Complications in Cystic Fibrosis: Past, Present, and Future: Adult Cystic Fibrosis Series.

Abstract

1Cystic 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.

• Authors: Christina M Mingora, Patrick A Flume
• Journal: Chest (Chest) Vol. 160 Issue 4 Pg. 1232-1240 (10 2021) ISSN: 1931-3543 [Electronic] United States
• PMID: 34147501 (Publication Type: Journal Article, Research Support, N.I.H., Extramural, Review)
• Copyright: Copyright © 2021 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.

Chemical References

• Antifibrinolytic Agents
• Chloride Channel Agonists

Topics

• Antifibrinolytic Agents (therapeutic use)
• Bronchoscopy
• Chest Tubes
• Chloride Channel Agonists (therapeutic use)
• Cystic Fibrosis (complications, physiopathology, therapy)
• Disease Progression
• Hemoptysis (etiology, therapy)
• Humans
• Noninvasive Ventilation
• Oxygen Inhalation Therapy
• Palliative Care
• Pleurodesis
• Pneumothorax (etiology, therapy)
• Prosthesis Implantation
• Respiratory Insufficiency (etiology, therapy)
• Severity of Illness Index
• Surgical Instruments
• Thoracostomy