Evidence for decline in the incidence of cystic fibrosis: a 35-year observational study in Brittany, France.

Abstract : ABSTRACT: BACKGROUND: Cystic fibrosis (CF) is an autosomal recessive disorder whose incidence has long been estimated as 1/2500 live births in Caucasians. Expanding implementation of newborn screening (NBS) programs now allows a better monitoring of the disease incidence, what is essential to make reliable predictions for disease management. This study assessed time trends in the birth incidence of CF over a long period (35 years: 1975-2009) in an area where CF is frequent (Brittany, France) and where NBS has been implemented for more than 20 years. METHODS: This study enrolled CF patients born in Brittany between January 1st 1975 and December 31st 2009 (n=483). Time trends in incidence were examined using Poisson regression and mainly expressed using the average percent change (APC). RESULTS: The average number of patients born each year declined from 18.6 in the late 1970's (period 1975-79) to 11.6 nowadays (period 2005-09). The corresponding incidence rates dropped from 1/1983 to 1/3268, which represented a decline close to 40% between these two periods (APC=-39.3%, 95% CI=-55.8% to -16.7%, p=0.0020). A clear breakpoint in incidence rate was observed at the end of the 1980's (p<0.0001). However, the incidence rate has remained quite stable since that time (annual APC=-1.0%, 95% CI=-3.0% to 1.1%, p=0.3516). CONCLUSIONS: This study provides an accurate picture of the evolution of the incidence of a genetic disease over a long period and highlights how it is influenced by the health policies implemented. We observed a 40% drop in incidence in our area which seems consecutive to the availability of prenatal diagnosis.
Type de document :
Article dans une revue
Orphanet Journal of Rare Diseases, BioMed Central, 2012, 7 (1), pp.14. 〈10.1186/1750-1172-7-14〉
Liste complète des métadonnées

Littérature citée [28 références]  Voir  Masquer  Télécharger

http://www.hal.inserm.fr/inserm-00681934
Contributeur : Ed. Bmc <>
Soumis le : jeudi 22 mars 2012 - 21:06:03
Dernière modification le : mardi 30 janvier 2018 - 10:42:01
Document(s) archivé(s) le : lundi 26 novembre 2012 - 11:56:24

Fichiers

1750-1172-7-14.pdf
Fichiers éditeurs autorisés sur une archive ouverte

Identifiants

Citation

Virginie Scotet, Ingrid Duguépéroux, Philippe Saliou, Gilles Rault, Michel Roussey, et al.. Evidence for decline in the incidence of cystic fibrosis: a 35-year observational study in Brittany, France.. Orphanet Journal of Rare Diseases, BioMed Central, 2012, 7 (1), pp.14. 〈10.1186/1750-1172-7-14〉. 〈inserm-00681934〉

Partager

Métriques

Consultations de la notice

250

Téléchargements de fichiers

213