Reference percentiles for FEV1 and BMI in European children and adults with cystic fibrosis
1 AP-HP, Hôpital Trousseau - Pediatric Pulmonary Department, Hopital St Antoine – Public Health Department, Paris, France
2 INSERM, U938, INSERM U707, Paris, France
3 Université Pierre et Marie Curie – Paris6, Paris, France
4 Dipartimento di Scienze Cliniche e di Comunità, Università degli Studi di Milano, Milan, Italy
5 Cystic Fibrosis Centre Aarhus, Aarhus University Hospital, Aarhus N, Denmark
6 Vaincre la Mucoviscidose, Paris, France
7 Universitätsklinik für Kinder und Jugendmedizin, Tübingen, Germany
8 Verona CF center, Verona, Italy
9 Portuguese Registry for Cystic Fibrosis Collaborative Group, Lisbon, Portugal
10 Department of Paediatrics, 2nd Faculty of Medicine, Charles University, Prague, Czech Republic
11 Belgian Cystic Fibrosis Registry, Public health and Surveillance|, Scientific Institute of Public Health, Brussels, Belgium
12 Unit for pulmonary diseases, University Children’s Hospital, University Medical Centre Ljubljana, Ljubljana, Slovenia
13 Israeli National CF registry, Jerusalem, Israel
14 Division of Medical Sciences, University of Dundee, Dundee, United Kingdom
Orphanet Journal of Rare Diseases 2012, 7:64 doi:10.1186/1750-1172-7-64Published: 7 September 2012
The clinical course of Cystic Fibrosis (CF) is usually measured using the percent predicted FEV1 and BMI Z-score referenced against a healthy population, since achieving normality is the ultimate goal of CF care. Referencing against age and sex matched CF peers may provide valuable information for patients and for comparison between CF centers or populations. Here, we used a large database of European CF patients to compute CF specific reference equations for FEV1 and BMI, derived CF-specific percentile charts and compared these European data to their nearest international equivalents.
34859 FEV1 and 40947 BMI observations were used to compute European CF specific percentiles. Quantile regression was applied to raw measurements as a function of sex, age and height. Results were compared with the North American equivalent for FEV1 and with the WHO 2007 normative values for BMI.
FEV1 and BMI percentiles illustrated the large variability between CF patients receiving the best current care. The European CF specific percentiles for FEV1 were significantly different from those in the USA from an earlier era, with higher lung function in Europe. The CF specific percentiles for BMI declined relative to the WHO standard in older children. Lung function and BMI were similar in the two largest contributing European Countries (France and Germany).
The CF specific percentile approach applied to FEV1 and BMI allows referencing patients with respect to their peers. These data allow peer to peer and population comparisons in CF patients.