Pulmonary outcome differences in U.S. and French cystic fibrosis cohorts diagnosed through newborn screening.

Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society
Aimee C Walshparticipating CF centres of Brittany, France

Abstract

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. 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. 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). 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.

References

May 15, 1996·American Journal of Epidemiology·M Corey, V Farewell
Oct 3, 2002·American Journal of Respiratory and Critical Care Medicine·David S ArmstrongKeith Grimwood
May 8, 2003·Journal of Clinical Microbiology·David ArmstrongClaire Wainwright
May 15, 2003·Pediatric Pulmonology·Suzanne Terheggen-LagroCornelis van der Ent
Aug 12, 2003·Pediatric Pulmonology·Philip M FarrellMark L Splaingard
Aug 31, 2004·Pediatric Pulmonology·Zhanhai LiPhilip M Farrell
Feb 3, 2005·JAMA : the Journal of the American Medical Association·Zhanhai LiMark L Splaingard
Feb 9, 2005·Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society·Ingrid DuguépérouxUNKNOWN Participating Centres to the French National Cystic Fibrosis Registry
Nov 9, 2005·Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society·Andrew T BraunWilliam M Gershan
Nov 26, 2008·Paediatric Respiratory Reviews·Andrew Bush

❮ Previous
Next ❯

Citations

Jan 15, 2014·Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society·Matthew N HurleyAlan R Smyth
Oct 12, 2015·Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society·Donald R VanDevanterPaul S Hodgkins
Apr 12, 2012·Current Opinion in Pediatrics·Jeffrey S WagenerMarci K Sontag

❮ Previous
Next ❯

Related Concepts

Related Feeds

CFTR Mutant Structural Therapy

Over 1700 different mutations in the CFTR genes have been shown to cause cystic fibrosis. Here is the latest research on structural therapy for CFTR mutants.