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

被引:11
|
作者
Mingora, Christina M. [1 ]
Flume, Patrick A. [1 ]
机构
[1] Med Univ South Carolina, Dept Med, Div Pulm Crit Care Allergy & Sleep Med, Charleston, SC 29425 USA
关键词
CFTR modulator; complications; cystic fibrosis; hemoptysis; palliative care; pneumothorax; pulmonary hypertension; respiratory failure; ELEXACAFTOR-TEZACAFTOR-IVACAFTOR; BRONCHIAL ARTERY EMBOLIZATION; INHALED TRANEXAMIC ACID; CLINICAL-TRIAL; HEMOPTYSIS; PNEUMOTHORAX; HYPERTENSION; GUIDELINES; LUMACAFTOR; SURVIVAL;
D O I
10.1016/j.chest.2021.06.017
中图分类号
R4 [临床医学];
学科分类号
1002 ; 100602 ;
摘要
(1)Cystic fibrosis (CF) is an autosomal recessive genetic condition with multisystemic disease manifestations, the most prominent of which occur in the respiratory system. Despite signifi-cant 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 ther-apies play in the natural disease course and care of patients with CF.
引用
收藏
页码:1232 / 1240
页数:9
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