Epidemiologic international registry of cystic fibrosis: comparison of French data with European data for 1995.

Background. - The Epidemiologic Registry of Cystic Fibrosis (ERCF) is an international registry, sponsored by Roche Laboratories, collecting data about CF patients in Europe. The aim of our study is to compare the French data with the European data collected during the year 1995. Results. - By December 31st 1995, 8,831 patients have been enrolled in Europe, including 1,457 patients in France. French CF patients are younger (mean age = 12.6 years) than European CF patients (mean age = 14.6 years). Genotype is better characterised in France (89 vs 75% for European patients), but only 49% of CF patients are homozygote for the DF508 deletion in France versus 77% in Denmark. Two clinical features of French CF patients are interesting: 1) presence of Staphylococcus aureus and Haemophilus influenzae (52%) is more frequent in France than in Europe (65 vs 48% and 52 vs 29%, respectively), 2) lung function tests (forced vital capacity [FVC]), forced expiratory volume per second [FEV1] are worse in France (P < 0.001) particularly in the older patients (> 18 years): 39% of these patients in France have a FEV1 < 40% of predicted value compared to only 29% in Europe. Similarly there are fewer patients in this age group in France (22 vs 31% in Europe) having a FVC > 90% of the predicted value in France. With regard to the treatment, three differences emerge: 1) dornase alfa is more used in France (55 vs only 34% in Europe); 2) use of prophylactic inhaled and oral antibiotics is less common in France than in all age groups; 3) the use of inhaled corticosteroids and bronchodilators is also less common in France despite the same incidence of asthma-like symptoms. Finally we notice that the mean age at death in 1995 is 18.2 years (+/- 2.38) in France and 20.6 years (+/- 0.85) in Europe. Conclusion. - These results are preliminary because 1995 is the first year for ERCF in France and a low percentage of French CF patients are included for this year. Therefore they must be interpreted with caution. Nevertheless, we carl hypothesise about a relationship between these results and a less aggressive treatment regimen. The impact of dornase a(fa use on prognosis seems interesting to analyse in future years. (C) 1998, Elsevier, Paris.