Bellis Gil, Cazes Marie-Hélène, Parant Alain, Gaimard Maryse, Travers Cécile, Le Roux Evelyne, Ravilly Sophie, Rault Gilles
Institut national d'études démographiques, 133 boulevard Davout, 75980 Paris Cedex 20, France.
J Cyst Fibros. 2007 May;6(3):179-86. doi: 10.1016/j.jcf.2006.07.001. Epub 2006 Aug 17.
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.
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.
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%.
These provisional results demonstrate the need to convert the ONM observatory into a registry providing exhaustive coverage of all patients.
1992年，法国设立了国家囊性纤维化监测站（国家黏液黏稠病监测站，ONM），以便每年监测患者的健康状况。本研究利用ONM的数据估算了预期寿命的主要指标，并评估了囊性纤维化患者的总数。
1994年至2003年的数据被划分为3年时间段。为这些时间段编制生命表，从中确定平均寿命和中位寿命。利用最新的生命表、2003年的出生人数和疾病发病率，假设人口稳定，可估算出患者的总人口数。
在2001 - 2003年，在ONM登记的患者出生时的预期寿命为39.1岁，中位寿命为36.4岁。这些结果比1994 - 1996年的结果有显著改善，这与ONM数据库中患者纳入条件的改善、医疗保健的改善以及生命表的局限性有关。根据2003年的数据，患者的理论总数为6490人，因此ONM数据库的覆盖率为63.2%。
这些初步结果表明，有必要将ONM监测站转变为一个能全面覆盖所有患者的登记机构。
