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Growth and nutritional status, and their association with lung function: a study from the International Primary Ciliary Dyskinesia Cohort

Growth and nutritional status, and their association with lung function: a study from the International Primary Ciliary Dyskinesia Cohort
Growth and nutritional status, and their association with lung function: a study from the International Primary Ciliary Dyskinesia Cohort
Chronic respiratory disease can affect growth or nutrition, which can influence lung function. We investigated height, body mass index (BMI), and lung function in patients with primary ciliary dyskinesia (PCD).In this large study based on the international PCD (iPCD) Cohort, we calculated z-scores for height and BMI using World Health Organization (WHO) and national growth references, and assessed associations with age, sex, country, diagnostic certainty, age at diagnosis, organ laterality, and lung function in multilevel regression models accounting for repeated measurements.We analysed 6402 measurements from 1609 from iPCD Cohort patients. Height was reduced compared to WHO (z-score -0.12, 95% CI -0.17 to -0.06) and national references (-0.27, -0.33 to -0.21) in male and female patients in all age groups, with variation between countries. Height and BMI were higher in patients diagnosed earlier in life (p=0.026 and <0.001, respectively) and closely associated with FEV1 and FVC z-scores (p<0.001).Our study indicates that growth and nutrition are impaired in PCD patients from early life and are strongly associated with lung function. If supported by longitudinal studies, this suggests that early diagnosis with multidisciplinary management and nutritional advice could improve growth and delay disease progression and lung function impairment in PCD.
0903-1936
Goutaki, M.
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Halbeisen, F.
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Spycher, B.
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Maurer, E.
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Fabien, B.
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Israel, A.
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Boon, M.
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Behan, L.
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Carr, S.
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Casaulta, C.
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Clement, A.
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Crowley, S.
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Dell, S.
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Ferkol, T.
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Haarman, E.G.
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Karadag, B.
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Knowles, M.
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Koerner-Rettberg, C.
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Leigh, M.W.
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Loebinger, M.R.
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Nielsen, K.G.
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Phillipsen, M.
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Sagel, S.D.
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Santamaria, F.
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Schwerk, N.
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Yiallouros, P.
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Lucas, J.
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Kuehni, C.E.
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Goutaki, M.
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Halbeisen, F.
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Spycher, B.
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Maurer, E.
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Fabien, B.
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Israel, A.
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Boon, M.
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Behan, L.
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Carr, S.
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Casaulta, C.
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Clement, A.
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Crowley, S.
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Dell, S.
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Ferkol, T.
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Haarman, E.G.
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Karadag, B.
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Knowles, M.
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Koerner-Rettberg, C.
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Leigh, M.W.
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Loebinger, M.R.
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Mazurek, H.
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Morgan, L.
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Nielsen, K.G.
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Phillipsen, M.
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Sagel, S.D.
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Santamaria, F.
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Schwerk, N.
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Yiallouros, P.
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Lucas, J.
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Kuehni, C.E.
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Goutaki, M., Halbeisen, F., Spycher, B., Maurer, E., Fabien, B., Israel, A., Boon, M., Behan, L., Carr, S., Casaulta, C., Clement, A., Crowley, S., Dell, S., Ferkol, T., Haarman, E.G., Karadag, B., Knowles, M., Koerner-Rettberg, C., Leigh, M.W., Loebinger, M.R., Mazurek, H., Morgan, L., Nielsen, K.G., Phillipsen, M., Sagel, S.D., Santamaria, F., Schwerk, N., Yiallouros, P., Lucas, J. and Kuehni, C.E. (2017) Growth and nutritional status, and their association with lung function: a study from the International Primary Ciliary Dyskinesia Cohort. European Respiratory Journal. (doi:10.1183/13993003.01659-2017).

Record type: Article

Abstract

Chronic respiratory disease can affect growth or nutrition, which can influence lung function. We investigated height, body mass index (BMI), and lung function in patients with primary ciliary dyskinesia (PCD).In this large study based on the international PCD (iPCD) Cohort, we calculated z-scores for height and BMI using World Health Organization (WHO) and national growth references, and assessed associations with age, sex, country, diagnostic certainty, age at diagnosis, organ laterality, and lung function in multilevel regression models accounting for repeated measurements.We analysed 6402 measurements from 1609 from iPCD Cohort patients. Height was reduced compared to WHO (z-score -0.12, 95% CI -0.17 to -0.06) and national references (-0.27, -0.33 to -0.21) in male and female patients in all age groups, with variation between countries. Height and BMI were higher in patients diagnosed earlier in life (p=0.026 and <0.001, respectively) and closely associated with FEV1 and FVC z-scores (p<0.001).Our study indicates that growth and nutrition are impaired in PCD patients from early life and are strongly associated with lung function. If supported by longitudinal studies, this suggests that early diagnosis with multidisciplinary management and nutritional advice could improve growth and delay disease progression and lung function impairment in PCD.

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Growth_nutritional_status_and_lung_function_in_patients_with_PCD_postprint - Accepted Manuscript
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Accepted/In Press date: 15 September 2017
e-pub ahead of print date: 21 December 2017

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Local EPrints ID: 414680
URI: https://eprints.soton.ac.uk/id/eprint/414680
ISSN: 0903-1936
PURE UUID: 82f945b0-0176-401f-9004-31d3d5eae700

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Date deposited: 05 Oct 2017 16:31
Last modified: 10 Dec 2019 05:47

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Contributors

Author: M. Goutaki
Author: F. Halbeisen
Author: B. Spycher
Author: E. Maurer
Author: B. Fabien
Author: A. Israel
Author: M. Boon
Author: L. Behan
Author: S. Carr
Author: C. Casaulta
Author: A. Clement
Author: S. Crowley
Author: S. Dell
Author: T. Ferkol
Author: E.G. Haarman
Author: B. Karadag
Author: M. Knowles
Author: C. Koerner-Rettberg
Author: M.W. Leigh
Author: M.R. Loebinger
Author: H. Mazurek
Author: L. Morgan
Author: K.G. Nielsen
Author: M. Phillipsen
Author: S.D. Sagel
Author: F. Santamaria
Author: N. Schwerk
Author: P. Yiallouros
Author: J. Lucas
Author: C.E. Kuehni

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