Cystic fibrosis lung disease: genetic influences, microbial interactions, and radiological assessment.

Abstract:

:Cystic fibrosis (CF) is a multiorgan disease caused by mutation of the CF transmembrane conductance regulator (CFTR) gene. Obstructive lung disease is the predominant cause of morbidity and mortality; thus, most efforts to improve outcomes are directed toward slowing or halting lung-disease progression. Current therapies, such as mucolytics, airway clearance techniques, bronchodilators, and antibiotics, aim to suppress airway inflammation and the processes that stimulate it, namely, retention and infection of mucus plaques at the airway surface. New approaches to therapy that aim to ameliorate specific CFTR mutations or mutational classes by restoring normal expression or function are being investigated. Because of its sensitivity in detecting changes associated with early airway obstruction and regional lung disease, high-resolution CT (HRCT) complements pulmonary function testing in defining disease natural history and measuring response to both conventional and experimental therapies. In this review, perspectives on the genetics and microbiology of CF provide a context for understanding the increasing importance of HRCT and other imaging techniques in assessing CF therapies.

journal_name

Pediatr Radiol

journal_title

Pediatric radiology

authors

Moskowitz SM,Gibson RL,Effmann EL

doi

10.1007/s00247-005-1445-3

keywords:

subject

Has Abstract

pub_date

2005-08-01 00:00:00

pages

739-57

issue

8

eissn

0301-0449

issn

1432-1998

journal_volume

35

pub_type

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