Non-genetic influences on cystic fibrosis lung disease: the role of sociodemographic characteristics, environmental exposures, and healthcare interventions.

Abstract:

:Patients with cystic fibrosis (CF) exhibit significant variability in the course and severity of the disease, and this variability is best interpreted in the context of an interaction between genetic and nongenetic determinants. While a small number of patients with "mild" mutations in the CF transmembrane conductance regulator gene clearly have less severe disease, for the general population of patients with CF the strongest predictor of severity of clinical course appears to be socioeconomic status (SES). Low SES probably exerts an influence via the convergence of detrimental environmental influences on outcome such as environmental tobacco smoke, poor nutrition, and stress. Other important predictors that appear to be independent of SES include gender and acquisition of airway pathogens such as Pseudomonas aeruginosa and Burkholderia cepacia complex. Finally, effective provision of medical interventions is an important determinant of outcome, and this is affected primarily by patient adherence (which bears a poorly defined relationship to SES), and attendance at a CF care center that utilizes a system of care that allows optimal delivery of evidence-based interventions.

authors

Schechter MS

doi

10.1055/s-2004-815660

keywords:

subject

Has Abstract

pub_date

2003-12-01 00:00:00

pages

639-52

issue

6

eissn

1069-3424

issn

1098-9048

journal_volume

24

pub_type

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