Cystic fibrosis mortality trends in France.
BACKGROUND
In 1992 France set up a national cystic fibrosis observatory (Observatoire national de la mucoviscidose, ONM) to monitor the state of health of patients on an annual basis. Using the ONM data, this study estimates the main indicators for life expectancy and assesses the total number of cystic fibrosis patients.


METHODS
The data for the years 1994 to 2003 are divided into 3-year periods. Life tables are drawn up for these periods, from which mean and median lengths of life are determined. Using the most recent life table, the number of births in 2003 and the incidence of the disease, the total population of patients can be estimated, assuming a stationary population.


RESULTS
In 2001-2003, life expectancy at birth of patients registered with the ONM was 39.1 years and median length of life was 36.4 years. These results, substantially better than those of 1994-1996, are linked to improved conditions of patient inclusion in the ONM database, to improvements in their healthcare, but also to the limitations of the life tables. Based on the 2003 data, the total theoretical number of patients is 6490, and coverage by the ONM database is thus 63.2%.


CONCLUSIONS
These provisional results demonstrate the need to convert the ONM observatory into a registry providing exhaustive coverage of all patients.
Bellis
Gil
G
Institut national d'études démographiques, 133 boulevard Davout, 75980 Paris Cedex 20, France. bellis@ined.fr
Cazes
Marie-Hélène
MH
Parant
Alain
A
Gaimard
Maryse
M
Travers
Cécile
C
Le Roux
Evelyne
E
Ravilly
Sophie
S
Rault
Gilles
G
D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D003550:Cystic Fibrosis / Q000401:mortality*; D005602:France / Q000453:epidemiology; D006801:Humans; D008017:Life Expectancy; D008875:Middle Aged; D009026:Mortality / Q000639:trends; D012042:Registries / Q000706:statistics & numerical data*