Respiratory System Disease.

Abstract:

:Respiratory system involvement in cystic fibrosis is the leading cause of morbidity and mortality. Defects in the cystic fibrosis transmembrane regulator (CFTR) gene throughout the sinopulmonary tract result in recurrent infections with a variety of organisms including Pseudomonas aeruginosa, methicillin-resistant Staphylococcus aureus, and nontuberculous mycobacteria. Lung disease occurs earlier in life than once thought and ideal methods of monitoring lung function, decline, or improvement with therapy are debated. Treatment of sinopulmonary disease may include physiotherapy, mucus-modifying and antiinflammatory agents, antimicrobials, and surgery. In the new era of personalized medicine, CFTR correctors and potentiators may change the course of disease.

journal_name

Pediatr Clin North Am

authors

Goetz DM,Singh S

doi

10.1016/j.pcl.2016.04.007

subject

Has Abstract

pub_date

2016-08-01 00:00:00

pages

637-59

issue

4

eissn

0031-3955

issn

1557-8240

pii

S0031-3955(16)30015-3

journal_volume

63

pub_type

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