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1 Dept of Gastroenterology and Cystic Fibrosis, Robert Debré Hospital AP/HP, Paris, France, 2 Statistics for Research SFR Ltd, Basel, Switzerland, 3 Munich University Children's Hospital, Munich, Germany, 4 Cystic Fibrosis Dept, Royal Brompton Hospital, London, UK, 5 Dept of Paediatrics, Rigshospitalet, University Hospital, Copenhagen, Denmark, 6 Dept of Pulmonary and Digestive Diseases of Developmental Age, Cystic Fibrosis Centre, Verona, Italy, 7 Dept of Paediatrics, Göteborg University, Göteborg, Sweden and 8 Pharmaceuticals Division, F. Hoffmann-La Roche Ltd, Basel, Switzerland
CORRESPONDENCE: J. Navarro, Hôpital Robert Debré, Gastro-entérologie, 48 Boulevard Serrurier, 75019, Paris, France. Fax: 33 140032353
Keywords: Cystic fibrosis, pulmonary function, registry, respiratory function tests
Received: July 28, 2000
Accepted March 10, 2001
The European Epidemiologic Registry of Cystic Fibrosis (ERCF) Advisory Board, an independent team of academics who manage the ERCF with complete editorial freedom, would like to acknowledge financial support from F. Hoffmann-La Roche, Basel, Switzerland for funding the ERCF.
The European Epidemiologic Registry of Cystic Fibrosis began collecting longitudinal data on European cystic fibrosis patients in 1994. A cross-sectional analysis was performed to identify the factors associated with low values of % predicted forced expiratory volume in one second (FEV1) upon patient enrolment.
Data from 7,010 patients aged
Factors associated with FEV1 that were lower by >10% of pred values were: lower weight for age percentiles, haemoptysis, pneumothorax, pulmonary symptoms at presentation, Pseudomonas aeruginosa, Burkholderia cepacia, oral corticosteroids, nonsteroid anti-inflammatory drugs, dornase alfa, oxygen and assisted ventilation and, in patients >12 yrs old only, use of airway clearance techniques, inhaled bronchodilators, oral nutritional supplements, pancreatic enzymes and insulin or oral hypoglycaemics. Slightly impaired lung function (5–10%) was associated with: diabetes (
The results confirm those of previous studies and suggest selective prescribing in sicker patients.
6 yrs were included. Clinical conditions, microbiological isolates and medications reported at enrolment or within the following 180 days were analysed for age-specific associations. 18-yrs-old), gastro-oesophageal reflux, allergic bronchopulmonary aspergillosis, asthma-like symptoms, portal hypertension, Aspergillus spp. and Candida spp. Sex, Haemophilus influenzae and Staphylococcus aureus were not associated with impaired pulmonary status. Regular exercise (especially in older patients) and nasal polyposis were associated with slightly better FEV1.
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