Skip to Main content Skip to Navigation
Journal articles

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.
Document type :
Journal articles
Complete list of metadatas

Cited literature [28 references]  Display  Hide  Download

https://www.hal.inserm.fr/inserm-00681934
Contributor : Ed. Bmc <>
Submitted on : Thursday, March 22, 2012 - 9:06:03 PM
Last modification on : Wednesday, June 24, 2020 - 4:19:22 PM
Long-term archiving on: : Monday, November 26, 2012 - 11:56:24 AM

Files

1750-1172-7-14.pdf
Publisher files allowed on an open archive

Identifiers

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⟩

Share

Metrics

Record views

396

Files downloads

523