Document Detail

Pulmonary outcome differences in U.S. and French cystic fibrosis cohorts diagnosed through newborn screening.
MedLine Citation:
PMID:  19926349     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: A comparison of the longitudinal progression of lung disease in cystic fibrosis patients identified through newborn screening (NBS) in cohorts located in two different countries has never been performed and was the primary objective of this study.
METHODS: The study included 56 patients in Brittany diagnosed through NBS between 1989 and 1994 and 69 similar patients in Wisconsin between 1985 and 1994. The onset and progression of lung disease was radiographically quantified using the Wisconsin Chest X-ray (WCXR) scoring system. A single pediatric pulmonologist blinded to all identifiers scored the films.
RESULTS: Generalized estimating equation analyses adjusted for age, genotype, sex, pancreatic insufficiency, and meconium ileus showed worse WCXR scores in Brittany patients compared to Wisconsin patients (average score difference=4.48; p<0.001). Percent predicted FEV1 was also worse among Brittany patients (p<0.001).
CONCLUSIONS: The finding of milder radiographically-quantified lung disease using the WCXR scoring system, as well as better FEV1 values, may be explained by variations in nutrition, environmental exposures, or healthcare delivery.
Aimee C Walsh; Gilles Rault; Zhanhai Li; Virginie Scotet; Ingrid Duguépéroux; Claude Férec; Michel Roussey; Anita Laxova; Philip M Farrell;
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Publication Detail:
Type:  Comparative Study; Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't     Date:  2009-11-18
Journal Detail:
Title:  Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society     Volume:  9     ISSN:  1873-5010     ISO Abbreviation:  J. Cyst. Fibros.     Publication Date:  2010 Jan 
Date Detail:
Created Date:  2010-02-04     Completed Date:  2010-04-22     Revised Date:  2014-09-24    
Medline Journal Info:
Nlm Unique ID:  101128966     Medline TA:  J Cyst Fibros     Country:  Netherlands    
Other Details:
Languages:  eng     Pagination:  44-50     Citation Subset:  IM    
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MeSH Terms
Bronchiectasis / epidemiology,  radiography
Child, Preschool
Cohort Studies
Cystic Fibrosis / epidemiology*,  radiography*
Forced Expiratory Volume
France / epidemiology
Infant, Newborn
Longitudinal Studies
Lung / radiography*
Mass Chest X-Ray
Neonatal Screening*
Retrospective Studies
Risk Factors
United States / epidemiology
Wisconsin / epidemiology
Grant Support
1UL1RR025011/RR/NCRR NIH HHS; DK 34108/DK/NIDDK NIH HHS; M01 RR003186/RR/NCRR NIH HHS; M01 RR003186-225571/RR/NCRR NIH HHS; M01 RR03186/RR/NCRR NIH HHS; R01 DK034108/DK/NIDDK NIH HHS; R01 DK034108-23/DK/NIDDK NIH HHS; UL1 RR025011/RR/NCRR NIH HHS; UL1 RR025011-025923/RR/NCRR NIH HHS

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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