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

on behalf of the PCD Israeli Consortium, Laura Behan; on behalf of the Swiss PCD Group, Annick Clement; on behalf of the French Reference Centre for Rare Lung Diseases, Suzanne Crowley

doi:10.1183/13993003.01659-2017

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

on behalf of the PCD Israeli Consortium, Laura Behan
, on behalf of the Swiss PCD Group, Annick Clement
, on behalf of the French Reference Centre for Rare Lung Diseases, Suzanne Crowley

Bar-Ilan University - Azrieli Faculty of Medicine

Research output: Contribution to journal › Article › peer-review

48 Scopus citations

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.

Original language	English
Article number	1701659
Journal	European Respiratory Journal
Volume	50
Issue number	6
DOIs	https://doi.org/10.1183/13993003.01659-2017
State	Published - 1 Dec 2017

Bibliographical note

Publisher Copyright:
Copyright © ERS 2017

Funding

Support statement: The development of the international Primary Ciliary Dyskinesia (iPCD) Cohort is funded by the European Union’s Seventh Framework Programme (EG-GA No.35404 BESTCILIA: Better Experimental Screening and Treatment for Primary Ciliary Dyskinesia). The primary ciliary dyskinesia (PCD) research at the Institute of Social and Preventive Medicine (ISPM) in Bern is supported by the Swiss National Science Foundation (SNF 320030_173044) and receives national funding from the Lung Leagues of Bern, St. Gallen, Vaud, Ticino and Valais, as well as the Milena-Carvajal Pro Kartagener Foundation. C. Kuehni is supported by the Swiss National Science Foundation (SNF32003B_162820 and SNF32003B_144068) while the national PCD service in Southampton and London is funded by the National Health Service (NHS) in England. The researchers participate in the Better Evidence to Advance Therapeutic Options for PCD (BEAT-PCD) network (COST action BM 1407). A Swiss National Science Foundation fellowship (PZ00P3_147987) supports B.D. Spycher. Swiss Cancer Research grants (KLS-3412-02-2014 and KLS-3644-02-2015) support F. Belle. The AAIR Charity (Reg. No. 1129698) supports L. Behan. S. Dell, T. Ferkol, M. Knowles, M.W. Leigh and S.D. Sagel are supported by the National Institutes of Health (U54HL096458). S.D. Sagel is also supported by local funding (CTSA NIH/NCATS Colorado UL1TR000154). Funding information for this article has been deposited with the Crossref Funder Registry. The development of the international Primary Ciliary Dyskinesia (iPCD) Cohort is funded by the European Union's Seventh Framework Programme (EG-GA No.35404 BESTCILIA: Better Experimental Screening and Treatment for Primary Ciliary Dyskinesia). The primary ciliary dyskinesia (PCD) research at the Institute of Social and Preventive Medicine (ISPM) in Bern is supported by the Swiss National Science Foundation (SNF 320030_173044) and receives national funding from the Lung Leagues of Bern, St. Gallen, Vaud, Ticino and Valais, as well as the Milena-Carvajal Pro Kartagener Foundation. C. Kuehni is supported by the Swiss National Science Foundation (SNF32003B_162820 and SNF32003B_144068) while the national PCD service in Southampton and London is funded by the National Health Service (NHS) in England. The researchers participate in the Better Evidence to Advance Therapeutic Options for PCD (BEAT-PCD) network (COST action BM 1407). A Swiss National Science Foundation fellowship (PZ00P3_147987) supports B.D. Spycher. Swiss Cancer Research grants (KLS-3412-02-2014 and KLS-3644-02-2015) support F. Belle. The AAIR Charity (Reg. No. 1129698) supports L. Behan. S. Dell, T. Ferkol, M. Knowles, M.W. Leigh and S.D. Sagel are supported by the National Institutes of Health (U54HL096458). S.D. Sagel is also supported by local funding (CTSA NIH/NCATS Colorado UL1TR000154). Funding information for this article has been deposited with the Crossref Funder Registry. We want to thank all patients in the PCD cohort and their families, and we are grateful to the PCD patient organisations that closely collaborated with us. We thank all the researchers in the participating centres who helped collect and enter data, and worked closely with us throughout building the iPCD Cohort. We thank Zorica Zivkovic (Children's Hospital for Lung Diseases and TB, Medical Centre ?Dr Dragisa Misovic?, Belgrade, Serbia) for contributing patients from her centre. We appreciate the work of Edwige Collaud and Anna Bettina Meier (Institute of Social and Preventive Medicine, University of Bern, Switzerland) who helped us with the height and BMI z-score calculations for some countries. We also thank Kali Tal and Christopher Ritter (Institute of Social and Preventive Medicine, University of Bern, Switzerland) for their editorial suggestions.

Funders	Funder number
Children's Hospital for Lung Diseases
Milena-Carvajal Pro Kartagener Foundation	SNF32003B_162820, SNF32003B_144068
National Health Service
National Institutes of Health	UL1TR000154
National Heart, Lung, and Blood Institute	U54HL096458
Seventh Framework Programme	35404
European Cooperation in Science and Technology	PZ00P3_147987
Schweizerischer Nationalfonds zur Förderung der Wissenschaftlichen Forschung	320030_173044
Seventh Framework Programme
Swiss Cancer Research Foundation	1129698, KLS-3412-02-2014, KLS-3644-02-2015

UN SDGs

This output contributes to the following UN Sustainable Development Goals (SDGs)

SDG 3 Good Health and Well-being

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10.1183/13993003.01659-2017

Cite this

on behalf of the PCD Israeli Consortium, Laura Behan, on behalf of the Swiss PCD Group, Annick Clement, & on behalf of the French Reference Centre for Rare Lung Diseases, Suzanne Crowley (2017). Growth and nutritional status, and their association with lung function: A study from the international Primary Ciliary Dyskinesia Cohort. European Respiratory Journal, 50(6), Article 1701659. https://doi.org/10.1183/13993003.01659-2017

on behalf of the PCD Israeli Consortium, Laura Behan ; on behalf of the Swiss PCD Group, Annick Clement ; on behalf of the French Reference Centre for Rare Lung Diseases, Suzanne Crowley. / Growth and nutritional status, and their association with lung function : A study from the international Primary Ciliary Dyskinesia Cohort. In: European Respiratory Journal. 2017 ; Vol. 50, No. 6.

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title = "Growth and nutritional status, and their association with lung function: A study from the international Primary Ciliary Dyskinesia Cohort",

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.",

author = "\{on behalf of the PCD Israeli Consortium, Laura Behan\} and \{on behalf of the Swiss PCD Group, Annick Clement\} and \{on behalf of the French Reference Centre for Rare Lung Diseases, Suzanne Crowley\} and Myrofora Goutaki and Halbeisen, \{Florian S.\} and Spycher, \{Ben D.\} and Elisabeth Maurer and Fabi{\"e}n Belle and Israel Amirav and Mieke Boon and Siobhan Carr and Carmen Casaulta and Sharon Dell and Thomas Ferkol and Haarman, \{Eric G.\} and Bulent Karadag and Michael Knowles and Cordula Koerner-Rettberg and Leigh, \{Margaret W.\} and Loebinger, \{Michael R.\} and Henryk Mazurek and Lucy Morgan and Nielsen, \{Kim G.\} and Maria Phillipsen and Sagel, \{Scott D.\} and Francesca Santamaria and Nicolaus Schwerk and Panayiotis Yiallouros and Lucas, \{Jane S.\} and Kuehni, \{Claudia E.\}",

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doi = "10.1183/13993003.01659-2017",

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on behalf of the PCD Israeli Consortium, Laura Behan, on behalf of the Swiss PCD Group, Annick Clement & on behalf of the French Reference Centre for Rare Lung Diseases, Suzanne Crowley 2017, 'Growth and nutritional status, and their association with lung function: A study from the international Primary Ciliary Dyskinesia Cohort', European Respiratory Journal, vol. 50, no. 6, 1701659. https://doi.org/10.1183/13993003.01659-2017

Growth and nutritional status, and their association with lung function: A study from the international Primary Ciliary Dyskinesia Cohort. / on behalf of the PCD Israeli Consortium, Laura Behan; on behalf of the Swiss PCD Group, Annick Clement; on behalf of the French Reference Centre for Rare Lung Diseases, Suzanne Crowley.
In: European Respiratory Journal, Vol. 50, No. 6, 1701659, 01.12.2017.

Research output: Contribution to journal › Article › peer-review

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AU - on behalf of the PCD Israeli Consortium, Laura Behan

AU - on behalf of the Swiss PCD Group, Annick Clement

AU - on behalf of the French Reference Centre for Rare Lung Diseases, Suzanne Crowley

AU - Goutaki, Myrofora

AU - Halbeisen, Florian S.

AU - Spycher, Ben D.

AU - Maurer, Elisabeth

AU - Belle, Fabiën

AU - Amirav, Israel

AU - Boon, Mieke

AU - Carr, Siobhan

AU - Casaulta, Carmen

AU - Dell, Sharon

AU - Ferkol, Thomas

AU - Haarman, Eric G.

AU - Karadag, Bulent

AU - Knowles, Michael

AU - Koerner-Rettberg, Cordula

AU - Leigh, Margaret W.

AU - Loebinger, Michael R.

AU - Mazurek, Henryk

AU - Morgan, Lucy

AU - Nielsen, Kim G.

AU - Phillipsen, Maria

AU - Sagel, Scott D.

AU - Santamaria, Francesca

AU - Schwerk, Nicolaus

AU - Yiallouros, Panayiotis

AU - Lucas, Jane S.

AU - Kuehni, Claudia E.

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on behalf of the PCD Israeli Consortium, Laura Behan, on behalf of the Swiss PCD Group, Annick Clement, on behalf of the French Reference Centre for Rare Lung Diseases, Suzanne Crowley. Growth and nutritional status, and their association with lung function: A study from the international Primary Ciliary Dyskinesia Cohort. European Respiratory Journal. 2017 Dec 1;50(6):1701659. doi: 10.1183/13993003.01659-2017

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

Abstract

Bibliographical note

Funding

UN SDGs

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