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Abstract
Background: The German cystic fibrosis (CF) quality assurance (CFQA) project is a patient registry for CF which was founded in 1995. Relevant clinical and laboratory data, respiratory function test results, complications, and CF treatments are entered into the database once a year for each patient. Using the database, a study was undertaken to elucidate the relationship between nutrition and lung function in a large patient cohort by cross sectional and longitudinal analysis.
Methods: A cohort of 3298 patients above 2 years of age was analysed. Patients were grouped according to the presence or absence of malnutrition (wasting and/or stunting). Cross sectional and longitudinal analyses over 2 and 3 years including mixed model analyses were performed.
Results: The prevalence of abnormal weight for height (<90% predicted) increased with age from 19% in children aged <6 years to 38% in adults with CF. Patients with malnutrition had significantly lower mean values of vital capacity, arterial oxygen tension (Po2), and forced expiratory volume in 1 second (FEV1) and higher serum IgG (p<0.05). Pseudomonas aeruginosa infection was also associated with decreased pulmonary function. Malnourished adolescents aged 12–18 years experienced a serious decline in FEV1 of about 20% predicted, whereas mean FEV1 values remained stable at above 80% predicted in adolescents of normal weight. Longitudinal follow up showed that malnourished patients of all ages and those with P aeruginosa infection had significantly worse lung function than their normally nourished counterparts and a greater yearly loss of FEV1 % predicted. During 1 year of observation adolescents who experienced a >5% predicted decrease in weight for height had a concomitant mean loss of FEV1 of 16.5% predicted during that year, whereas patients who gained relative weight had a parallel increase in FEV1 of 2.1% predicted.
Conclusions: These data emphasise the close relationship between nutrition, lung function, and clinical course in CF. Normal body weight and absence of P aeruginosa infection was associated with better preservation of lung function.
- cystic fibrosis
- nutrition
- lung function
- children
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Supplementary materials
Acknowledgements
The authors thank the Scientific Advisory Board of the German CFQA project (Chairman: Professor Dr Martin Stern) for the permission to analyse data from the registry, and acknowledge the large amount of work by staff from the 97 CF centres who reported patient data to the project:
Aachen, Kinderarztpraxis; Aue, Klinikum Aue, Kinderklinik; Augsburg, Kinderkrankenhaus Josefinum; Bad Saarow, Humaine Klinikum Bad Saarow, Klinik f�r Kinderheilkunde; Berlin, Krankenhaus Lichtenberg, Kinderklinik Lindenhof; Berlin, Krankenhaus Zehlendorf, Lungenklinik Heckeshorn, Kinderambulanz; Berlin, Kreiskrankenhaus Nauen, Betriebsst�tte Staaken; Berlin, Krankenhaus Buch von Berlin-Pankow, "C W Hufeland"; Bochum, Ruhr-Universit�t Bochum, Klinik f�r Kinder- und Jugendmedizin; Bodenheim, Praxis f�r P�diatrie und Allergologie; Bonn, Universit�t Bonn, Zentrum f�r Kinderheilkunde; Borkum, Reha-Klinik Borkum Riff; Brandenburg, St�dt. Klinikum Brandenburg GmbH, Kinderklinik; Bremen, Zentralkrankenhaus St.-J�rgen-Stra�e, Prof. Hess - Kinderklinik; Bremen, Krankenhaus "Links der Weser", Kinderklinik; Chemnitz, Kinderarztpraxis; Coswig, Fachkrankenhaus Coswig, Zentrum f�r Pneumologie u. 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