Description of lung function of the european cystic fibrosis patients

INTRODUCTION Cystic Fibrosis (CF) is a rare autosomal recessive disease that causes difficulty breathing and lung infections, disorders for pancreas and liver. From 2003, the European Cystic Fibrosis Society Patient Registry (ECFSPR) has been collecting data on European CF patients. The availability of a European registry for a rare disease is useful because important research questions can be studied with a large number of patients and the collection of information from different countries allows the comparison of the diagnostic and therapeutic protocols. OBJECTIVES We present the 2010 ECFSPR data on lung function. METHODS Once a year, national registries and individual centres send to the ECFSPR data on diagnosis, genetics, lung function, growth, complications, microbiology, therapy and transplantations of patients. RESULTS In 2010, 32,248 patients (48% females, 49% adults, the mean age at 31.12.2010 is 19.5 years) from 22 countries are registered in the ECFSPR . The median age at diagnosis is 5 months, 6% of patients is diagnosed older than 18 years. For almost all the countries, the FEV1% of predicted (FEV1%) has value about 100%-90% for the age group 6-14 years, it declines from the puberty until the age of 20 years, and then stays fairly stable near 60%-50%. However, we observe variability between the median values of FEV1% of the different countries. The percentage of patients with FEV1% <40% (below this threshold a patient is legible for transplantation) increased from 2.6% for patients aged 6-17 years, to 18.2% for patients aged 18-29 years, to 26.1% for patients aged 30 or older. CONCLUSIONS With the availability of the ECFSPR data and with the collaboration of the CF centres, it is possible to study clinical outcomes of the 2,677 patients with FEV1% <40% before and after the transplantation and to compare the national care services in the European countries.