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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 and 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 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 that accounted for repeated measurements.We analysed 6402 measurements from 1609 iPCD Cohort patients. Height was reduced compared to WHO (z-score -0.12, 95% CI -0.17 to -0.06) and national references (z-score -0.27, 95% CI -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 p<0.001, respectively) and closely associated with forced expiratory volume in 1 s and forced vital capacity z-scores (p<0.001).Our study indicates that both growth and nutrition are affected adversely in PCD patients from early life and are both strongly associated with lung function. If supported by longitudinal studies, these findings suggest 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, Myrofora
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Halbeisen, Florian S.
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Spycher, Ben
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Maurer, Elisabeth
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Belle, Fabien
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Amirav, Israel
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Behan, Laura
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Boon, Mieke
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Carr, Siobhan
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Casaulta, Carmen
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Clement, Annick
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Crowley, Suzanne
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Dell, Sharon D.
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Ferkol, Thomas W.
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Haarman, Eric G.
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Karadag, Bulent
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Knowles, Michael R.
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Koerner-Rettberg, C.
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Leigh, Margaret W.
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Loebinger, Michael R.
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Morgan, Lucy
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Nielsen, Kim G.
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Phillipsen, Maria
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Schwerk, Nicolause
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Yiallouros, Panayiotis
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Kuehni, Claudia E.
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Goutaki, Myrofora
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Halbeisen, Florian S.
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Belle, Fabien
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Crowley, Suzanne
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Dell, Sharon D.
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Ferkol, Thomas W.
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Haarman, Eric G.
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Karadag, Bulent
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Knowles, Michael R.
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Koerner-Rettberg, C.
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Leigh, Margaret W.
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Loebinger, Michael R.
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Mazurek, H.
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Morgan, Lucy
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Nielsen, Kim G.
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Phillipsen, Maria
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Sagel, Scott D.
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Santamaria, Francesca
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Schwerk, Nicolause
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Yiallouros, Panayiotis
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Lucas, Jane S.
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Kuehni, Claudia E.
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Goutaki, Myrofora, Halbeisen, Florian S., Spycher, Ben, Maurer, Elisabeth, Belle, Fabien, Amirav, Israel, Behan, Laura, Boon, Mieke, Carr, Siobhan, Casaulta, Carmen, Clement, Annick, Crowley, Suzanne, Dell, Sharon D., Ferkol, Thomas W., Haarman, Eric G., Karadag, Bulent, Knowles, Michael R., Koerner-Rettberg, C., Leigh, Margaret W., Loebinger, Michael R., Mazurek, H., Morgan, Lucy, Nielsen, Kim G., Phillipsen, Maria, Sagel, Scott D., Santamaria, Francesca, Schwerk, Nicolause, Yiallouros, Panayiotis, Lucas, Jane S. and Kuehni, Claudia E. (2017) Growth and nutritional status, and their association with lung function: a study from the International Primary Ciliary Dyskinesia Cohort. European Respiratory Journal, 20 (6). (doi:10.1183/13993003.01659-2017).

Record type: Article

Abstract

Chronic respiratory disease can affect growth and 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 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 that accounted for repeated measurements.We analysed 6402 measurements from 1609 iPCD Cohort patients. Height was reduced compared to WHO (z-score -0.12, 95% CI -0.17 to -0.06) and national references (z-score -0.27, 95% CI -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 p<0.001, respectively) and closely associated with forced expiratory volume in 1 s and forced vital capacity z-scores (p<0.001).Our study indicates that both growth and nutrition are affected adversely in PCD patients from early life and are both strongly associated with lung function. If supported by longitudinal studies, these findings suggest 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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Accepted/In Press date: 15 September 2017
e-pub ahead of print date: 21 December 2017

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Local EPrints ID: 416059
URI: https://eprints.soton.ac.uk/id/eprint/416059
ISSN: 0903-1936
PURE UUID: 148eacc9-9347-4e42-8167-1befac31804b

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Date deposited: 01 Dec 2017 17:30
Last modified: 29 Nov 2019 05:01

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Contributors

Author: Myrofora Goutaki
Author: Florian S. Halbeisen
Author: Ben Spycher
Author: Elisabeth Maurer
Author: Fabien Belle
Author: Israel Amirav
Author: Laura Behan
Author: Mieke Boon
Author: Siobhan Carr
Author: Carmen Casaulta
Author: Annick Clement
Author: Suzanne Crowley
Author: Sharon D. Dell
Author: Thomas W. Ferkol
Author: Eric G. Haarman
Author: Bulent Karadag
Author: Michael R. Knowles
Author: C. Koerner-Rettberg
Author: Margaret W. Leigh
Author: Michael R. Loebinger
Author: H. Mazurek
Author: Lucy Morgan
Author: Kim G. Nielsen
Author: Maria Phillipsen
Author: Scott D. Sagel
Author: Francesca Santamaria
Author: Nicolause Schwerk
Author: Panayiotis Yiallouros
Author: Jane S. Lucas
Author: Claudia E. Kuehni

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