Skip to main content
Key Specialties
Cardiology Diabetology Endocrinology Gastroenterology Geriatrics and Gerontology Gynecology and Obstetrics Hematology Infectious Disease Internal Medicine Nephrology Neurology Oncology Pediatrics Respiratory Medicine Rheumatology Surgery
Congress Coverage
2024 ACC Congress 2023 ESMO Congress 2023 EASD Congress 2023 ERS Congress 2023 ESC Congress 2023 EHA Congress 2023 ASCO Annual Meeting
Clinical Collections
Advances in Alzheimer’s

Keynote webinar | Spotlight on sleep in brain health

LIVE: Thursday 2nd May 2024, 18:00-19:30 (CEST)

Quality sleep is essential for health. But what happens to our brains when sleep patterns are disturbed? Join our experts to explore the interplay between sleep disruption and neurological diseases, and the questions that you need to be asking your patients to help you prevent the harmful effects of sleep deprivation.
ADVANCED SEARCH
Log in
Top
Orphanet Journal of Rare Diseases
Published in: Orphanet Journal of Rare Diseases 1/2012

Open Access 01-12-2012 | Research

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

Authors: Pierre-Yves Boëlle, Laura Viviani, Pierre-Francois Busson, Hanne V Olesen, Sophie Ravilly, Martin Stern, Baroukh M Assael, Celeste Barreto, Pavel Drevinek, Muriel Thomas, Uros Krivec, Meir Mei-Zahav, Jean-François Vibert, Annick Clement, Anil Mehta, Harriet Corvol

Published in: Orphanet Journal of Rare Diseases | Issue 1/2012

Login to get access

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.
Appendix
Available only for authorised users
Literature
1.
Dodge JA, Morison S, Lewis PA, Coles EC, Geddes D, Russell G, Littlewood JM, Scott MT: Incidence, population, and survival of cystic fibrosis in the uk, 1968-95. uk cystic fibrosis survey management committee. Arch Dis Child. 1997, 77: 493-496. 10.1136/adc.77.6.493.PubMedCentralCrossRefPubMed
2.
Mehta G, Macek M Jr, Mehta A: Cystic fibrosis across europe: eurocarecf analysis of demographic data from 35 countries. J Cyst Fibros. 2010, 9 (Suppl 2): S5-S21.CrossRefPubMed
3.
McCormick J, Mehta G, Olesen HV, Viviani L, Macek M Jr, Mehta A: Comparative demographics of the european cystic fibrosis population: a cross-sectional database analysis. Lancet. 2010, 375: 1007-1013. 10.1016/S0140-6736(09)62161-9.CrossRefPubMed
4.
Hankinson JL, Odencrantz JR, Fedan KB: Spirometric reference values from a sample of the general U.S. population. Am J Respir Crit Care Med. 1999, 159: 179-187.CrossRefPubMed
5.
Knudson RJ, Lebowitz MD, Holberg CJ, Burrows B: Changes in the normal maximal expiratory flow-volume curve with growth and aging. Am Rev Respir Dis. 1983, 127: 725-734.PubMed
6.
Kastner-Cole D, Palmer CN, Ogston SA, Mehta A, Mukhopadhyay S: Overweight and obesity in deltaF508 homozygous cystic fibrosis. J Pediatr. 2005, 147: 402-404. 10.1016/j.jpeds.2005.06.003.CrossRefPubMed
7.
Matthew DJ, Warner JO, Chrispin AR, Norman AP: The relationship between chest radiographic scores and respiratory function tests in children with cystic fibrosis. Pediatr Radiol. 1977, 5: 198-200. 10.1007/BF00972175.CrossRefPubMed
8.
Kulich M, Rosenfeld M, Campbell J, Kronmal R, Gibson RL, Goss CH, Ramsey B: Disease-specific reference equations for lung function in patients with cystic fibrosis. Am J Respir Crit Care Med. 2005, 172: 885-891. 10.1164/rccm.200410-1335OC.CrossRefPubMed
9.
McCormick J, Conway SP, Mehta A: Paediatric northern score centile charts for the chest radiograph in cystic fibrosis. Clin Radiol. 2007, 62: 78-81. 10.1016/j.crad.2006.09.011.CrossRefPubMed
10.
11.
Farrell PM: The prevalence of cystic fibrosis in the European Union. J Cyst Fibros. 2008, 7: 450-453. 10.1016/j.jcf.2008.03.007.CrossRefPubMed
12.
Efrati O, Nir J, Fraser D, Cohen-Cymberknoh M, Shoseyov D, Vilozni D, Modan-Moses D, Levy R, Szeinberg A, Kerem E, Rivlin J: Meconium ileus in patients with cystic fibrosis is not a risk factor for clinical deterioration and survival: the israeli multicenter study. J Pediatr Gastroenterol Nutr. 2010, 50: 173-178. 10.1097/MPG.0b013e3181a3bfdd.CrossRefPubMed
13.
de Onis M, Onyango AW, Borghi E, Siyam A, Nishida C, Siekmann J: Development of a WHO growth reference for school-aged children and adolescents. Bulletin of the World Health Organization. 2007, 85: 660-667. 10.2471/BLT.07.043497.PubMedCentralCrossRefPubMed
14.
Wei Y, Pere A, Koenker R, He X: Quantile regression methods for reference growth charts. Stat Med. 2006, 25: 1369-1382. 10.1002/sim.2271.CrossRefPubMed
15.
Lucotte G, Hazout S, De Braekeleer M: Complete map of cystic fibrosis mutation DF508 frequencies in Western Europe and correlation between mutation frequencies and incidence of disease. Hum Biol. 1995, 67: 797-803.PubMed
16.
de Onis M, Onyango AW, Borghi E, Siyam A, Nishida C, Siekmann J: Development of a who growth reference for school-aged children and adolescents. Bull World Health Organ. 2007, 85: 660-667. 10.2471/BLT.07.043497.PubMedCentralCrossRefPubMed
17.
Corvol H, Beucher J, Boelle PY, Busson PF, Muselet-Charlier C, Clement A, Ratjen F, Grasemann H, Laki J, Palmer CN: Ancestral haplotype 8.1 and lung disease severity in European cystic fibrosis patients. J Cyst Fibros. 2012, 11: 63-67. 10.1016/j.jcf.2011.09.006.CrossRefPubMed
18.
Taylor C, Commander CW, Collaco JM, Strug LJ, Li W, Wright FA, Webel AD, Pace RG, Stonebraker JR, Naughton K, et al: A novel lung disease phenotype adjusted for mortality attrition for cystic fibrosis Genetic modifier studies. Pediatr Pulmonol. 2011, 46: 857-869. 10.1002/ppul.21456.PubMedCentralCrossRefPubMed
19.
Ramsey BW, Davies J, McElvaney NG, Tullis E, Bell SC, Drevinek P, Griese M, McKone EF, Wainwright CE, Konstan MW, et al: A CFTR potentiator in patients with cystic fibrosis and the G551D mutation. N Engl J Med. 2011, 365: 1663-1672. 10.1056/NEJMoa1105185.PubMedCentralCrossRefPubMed
20.
Vaincre-la-Mucoviscidose, Ined: Bilan des données 2008. Registre Français de la Mucoviscidose. 2011, 1-20.
21.
Cystic Fibrosis Foundation: 2009 Annual Data Report. Patient Registry. 2009, 1-28.
22.
Cohen-Cymberknoh M, Shoseyov D, Kerem E: Managing cystic fibrosis: strategies that increase life expectancy and improve quality of life. Am J Respir Crit Care Med. 2011, 183: 1463-1471. 10.1164/rccm.201009-1478CI.CrossRefPubMed
23.
Stark LJ, Powers SW: Behavioral aspects of nutrition in children with cystic fibrosis. Curr Opin Pulm Med. 2005, 11: 539-542. 10.1097/01.mcp.0000183051.18611.e4.CrossRefPubMed
24.
Magoffin A, Allen JR, McCauley J, Gruca MA, Peat J, Van Asperen P, Gaskin K: Longitudinal analysis of resting energy expenditure in patients with cystic fibrosis. J Pediatr. 2008, 152: 703-708. 10.1016/j.jpeds.2007.10.021.CrossRefPubMed
25.
Michel SH, Maqbool A, Hanna MD, Mascarenhas M: Nutrition management of pediatric patients who have cystic fibrosis. Pediatr Clin North Am. 2009, 56: 1123-1141. 10.1016/j.pcl.2009.06.008.CrossRefPubMed
26.
Rosenfeld M, Davis R, FitzSimmons S, Pepe M, Ramsey B: Gender gap in cystic fibrosis mortality. Am J Epidemiol. 1997, 145: 794-803. 10.1093/oxfordjournals.aje.a009172.CrossRefPubMed
27.
Zemel BS, Jawad AF, FitzSimmons S, Stallings VA: Longitudinal relationship among growth, nutritional status, and pulmonary function in children with cystic fibrosis: analysis of the Cystic Fibrosis Foundation National CF Patient Registry. J Pediatr. 2000, 137: 374-380. 10.1067/mpd.2000.107891.CrossRefPubMed
28.
McCormick J, Sims EJ, Green MW, Mehta G, Culross F, Mehta A: Comparative analysis of cystic fibrosis registry data from the uk with usa, france and australasia. J Cyst Fibros. 2005, 4: 115-122. 10.1016/j.jcf.2005.01.001.CrossRefPubMed
29.
Hodson ME, Simmonds NJ, Warwick WJ, Tullis E, Castellani C, Assael B, Dodge JA, Corey M: An international/multicentre report on patients with cystic fibrosis (CF) over the age of 40 years. J Cyst Fibros. 2008, 7: 537-542. 10.1016/j.jcf.2008.06.003.CrossRefPubMed
30.
Wright FA, Strug LJ, Doshi VK, Commander CW, Blackman SM, Sun L, Berthiaume Y, Cutler D, Cojocaru A, Collaco JM, et al: Genome-wide association and linkage identify modifier loci of lung disease severity in cystic fibrosis at 11p13 and 20q13.2. Nat Genet. 2011, 43: 539-546. 10.1038/ng.838.PubMedCentralCrossRefPubMed
31.
Nathan N, Abou Taam R, Epaud R, Delacourt C, Deschildre A, Reix P, Chiron R, de Pontbriand U, Brouard J, Fayon M: A national internet-linked based database for pediatric interstitial lung diseases: the French network. Orphanet J Rare Dis. 2012, 7: 40-10.1186/1750-1172-7-40.PubMedCentralCrossRefPubMed
Metadata
Title
Reference percentiles for FEV1 and BMI in European children and adults with cystic fibrosis
Authors
Pierre-Yves Boëlle
Laura Viviani
Pierre-Francois Busson
Hanne V Olesen
Sophie Ravilly
Martin Stern
Baroukh M Assael
Celeste Barreto
Pavel Drevinek
Muriel Thomas
Uros Krivec
Meir Mei-Zahav
Jean-François Vibert
Annick Clement
Anil Mehta
Harriet Corvol
Publication date
01-12-2012
Publisher
BioMed Central
Published in
Orphanet Journal of Rare Diseases / Issue 1/2012
Electronic ISSN: 1750-1172
DOI
https://doi.org/10.1186/1750-1172-7-64

Other articles of this Issue 1/2012

Orphanet Journal of Rare Diseases 1/2012 Go to the issue

Research

Establishing a network of specialist Porphyria centres - effects on diagnostic activities and services

Research

Clinical features and predictors for disease natural progression in adults with Pompe disease: a nationwide prospective observational study

Research

Array CGH improves detection of mutations in the GALC gene associated with Krabbe disease

Review

Advanced therapies for the treatment of hemophilia: future perspectives

Research

Key outcomes from stakeholder workshops at a symposium to inform the development of an Australian national plan for rare diseases

Review

The trisomy 18 syndrome