PT  - JOURNAL ARTICLE
AU  - Kerem, Eitan
AU  - Viviani, Laura
AU  - Zolin, Anna
AU  - MacNeill, Stephanie
AU  - Hatziagorou, Elpis
AU  - Ellemunter, Helmut
AU  - Drevinek, Pavel
AU  - Gulmans, Vincent
AU  - Krivec, Uros
AU  - Olesen, Hanne
AU  - on behalf of the ECFS Patient Registry Steering Group
TI  - Factors associated with FEV1 decline in cystic fibrosis: analysis of the data of the ECFS Patient Registry
AID  - 10.1183/09031936.00166412
DP  - 2013 Jan 01
TA  - European Respiratory Journal
PG  - erj01664-2012
4099  - http://erj.ersjournals.com/content/early/2013/04/17/09031936.00166412.short
4100  - http://erj.ersjournals.com/content/early/2013/04/17/09031936.00166412.full
AB  - Pulmonary insufficiency is the main cause of death in cystic fibrosis (CF). The European CF Society Patient Registry (ECFSPR) offers a unique database to evaluate potential risk factors of CF lung disease in a heterogeneous population.We analysed forced expiratory volume in one second (FEV1%) data of 14732 patients registered in the ECFSPR in year 2007. Linear and logistic regressions were used to investigate association between FEV1% and age, age at diagnosis, sex, pancreatic status, chronic P. aeruginosa infection, CF-related diabetes (CFRD), BMI, and genotype. Estimates were adjusted for country.BMI, chronic infection by P. aeruginosa, pancreatic status and CFRD showed a statistically significant (all p<0·0001) and clinically relevant effect on FEV1%. Age at diagnosis, sex and genotype had a statistically significant (p<0·01), but not clinically relevant effect. We estimate that patients with lower BMI experience a 6 fold increased odds (95% CI 5·0;7.3) of having severe lung disease (FEV1<40%) compared to patients with normal BMI, and that patients affected by CFRD have a 1·8 fold increased odds (1·6;2·2) compared to patients not affected. Being chronically infected with P. aeruginosa increases the odds of severe lung disease by 2·4 (2·0;2·7) and pancreatic insufficient patients experience a 2·0 fold increased odds (1·6;2·5) of severe lung disease compared to pancreatic sufficient patients.Nutrition, chronic P. aeruginosa infection, pancreatic status and CFRD were found to be potential risk factors of pulmonary function in patients with CF regardless of age. Since three of these factors are preventable or treatable, we emphasize the importance of their early identification through frequent routine tests, the implementation of infection control measures, and a timely initiation of antibiotic therapies. Furthermore, epidemiological and clinical studies comparing CF patients from different populations should control for these factors to avoid confounding.