Author:
Boëlle Pierre-Yves,Viviani Laura,Busson Pierre-Francois,Olesen Hanne V,Ravilly Sophie,Stern Martin,Assael Baroukh M,Barreto Celeste,Drevinek Pavel,Thomas Muriel,Krivec Uros,Mei-Zahav Meir,Vibert Jean-François,Clement Annick,Mehta Anil,Corvol Harriet
Abstract
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 FEV
1
and BMI allows referencing patients with respect to their peers. These data allow peer to peer and population comparisons in CF patients.
Publisher
Springer Science and Business Media LLC
Subject
Pharmacology (medical),Genetics(clinical),General Medicine
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