| Literature DB >> 34147501 |
Christina M Mingora1, Patrick A Flume2.
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.Entities:
Keywords: CFTR modulator; complications; cystic fibrosis; hemoptysis; palliative care; pneumothorax; pulmonary hypertension; respiratory failure
Mesh:
Substances:
Year: 2021 PMID: 34147501 PMCID: PMC8727888 DOI: 10.1016/j.chest.2021.06.017
Source DB: PubMed Journal: Chest ISSN: 0012-3692 Impact factor: 10.262