Reference percentiles for FEV1 and BMI in European children and adults with cystic fibrosis.

Pierre-Yves Boëlle (1, 2) , Laura Viviani (3) , Pierre-Francois Busson (2) , Hanne Olesen (4) , Sophie Ravilly (5) , Martin Stern (6) , Baroukh Assael (7) , Celeste Barreto (8) , Pavel Drevinek (9) , Muriel Thomas (10) , Uros Krivec (11) , Meir Mei-Zahav (12) , Jean-François Vibert (1, 2) , Annick Clément (1, 2) , Anil Mehta (13) , Harriet Corvol (1, 2)

1 ESIM - Epidémiologie des maladies infectieuses et modélisation
2 UMRS893 - Centre de Recherche Saint-Antoine
3 DISCCO - Dpt di Scienze Cliniche e di Comunità [Milano]
4 Cystic Fibrosis Centre Aarhus
5 Vaincre la Mucoviscidose
6 Gastroenterologisches Labor
7 Ospedale Civile Maggiore
8 Portuguese Registry for Cystic Fibrosis Collaborative Group
9 Department of Paediatrics
10 Belgian Cystic Fibrosis Registry [Brussels]
11 Unit for pulmonary diseases
12 Israeli National CF registry
13 Division of Medical Sciences

ABSTRACT: BACKGROUND: 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. METHODS: 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. RESULTS: 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). CONCLUSION: 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.

Mots clés: Cystic fibrosis; Forced expiratory volume in one second; Body mass index; Registry

Domaines: Génétique

Pierre-Yves Boëlle, Laura Viviani, Pierre-Francois Busson, Hanne Olesen, Sophie Ravilly, et al.. Reference percentiles for FEV1 and BMI in European children and adults with cystic fibrosis.. Orphanet Journal of Rare Diseases, 2012, 7 (1), pp.64. ⟨10.1186/1750-1172-7-64⟩. ⟨inserm-00764370⟩