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Changes in height and BMI in children and adolescents with primary ciliary dyskinesia (PCD) during the growth period: An iPCD cohort study

Myrofora Goutaki, Florian Halbeisen, Philipp Latzin, Elisabeth Maurer, Carmen Casaulta, Suzanne Crowley, Eric Haarman, Jane S. Lucas, Lucy Morgan, Kim G. Nielsen, Francesca Santamaria, Nico Schwerk, Guillaume Thouvenin, Panayiotis Yiallouros, Alban Ramette, Claudia E. Kuehni
European Respiratory Journal 2016 48: PA3127; DOI: 10.1183/13993003.congress-2016.PA3127
Myrofora Goutaki
1Institute of Social and Preventive Medicine, University of Bern, Bern, Switzerland
2Department of Paediatrics, University Hospital of Bern, Bern, Switzerland
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Florian Halbeisen
1Institute of Social and Preventive Medicine, University of Bern, Bern, Switzerland
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Philipp Latzin
2Department of Paediatrics, University Hospital of Bern, Bern, Switzerland
3Department of Pediatric Pulmonology, University Children's Hospital of Basel, Basel, Switzerland
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Elisabeth Maurer
1Institute of Social and Preventive Medicine, University of Bern, Bern, Switzerland
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Carmen Casaulta
2Department of Paediatrics, University Hospital of Bern, Bern, Switzerland
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Suzanne Crowley
4Department of Pediatric Medicine, Oslo University Hospital, Oslo, Norway
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Eric Haarman
5Department of Pediatric Pulmonology, VU University Medical Center, Amsterdam, Netherlands
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Jane S. Lucas
6PCD Centre, University Hospital Southampton NHS Foundation Trust, Southampton, United Kingdom
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Lucy Morgan
7Department of Respiratory Medicine, Concord Hospital Clinical School, University of Sydney, Sydney, Australia
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Kim G. Nielsen
8Danish PCD Centre Copenhagen, Paediatric Pulmonary Service, Copenhagen University Hospital, Copenhagen, Denmark
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Francesca Santamaria
9Department of Translational Medical Sciences, Federico II University, Naples, Italy
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Nico Schwerk
10Clinic for Paediatric Pulmonology, Allergology, Lung Transplantation, Hannover Medical School, Hannover, Germany
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Guillaume Thouvenin
11Paediatric Pulmonary Department, Université Pierre et Marie Curie, Paris, France
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Panayiotis Yiallouros
12Cyprus International Institute for Environmental & Public, Cyprus University of Technology, Limassol, Cyprus
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Alban Ramette
1Institute of Social and Preventive Medicine, University of Bern, Bern, Switzerland
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Claudia E. Kuehni
1Institute of Social and Preventive Medicine, University of Bern, Bern, Switzerland
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Abstract

Background: In a cross-sectional collaborative analysis within the EU project BESTCILIA, we had found significantly lower height and slightly lower BMI in PCD patients younger than 20 years of age compared to national reference data. We now assessed changes in height and BMI over time during the growth period.

Methods: We used longitudinal measurements of height and BMI from PCD patients of the iPCD Cohort from 10 countries. We calculated height and BMI z-scores using national references and used generalized estimating equations (GEE) to identify changes over time, as a function of sex.

Results: We analysed 1833 repeated measurements of height and BMI from 343 patients aged 0-20 years. We found a significantly lower height (z-score: -0.16, 95%CI: -0.30 to -0.02, p=0.03) and BMI (-0.15, -0.31 to -0.03, p=0.05) in PCD patients compared to reference. We found no significant change in height z-scores (p=0.93) but a small increase in BMI z-scores (0.02 per year, 0.01 to 0.03, p<0.001) over time compared to normal values. Height (p=0.30) and BMI (p=0.13) z-scores did not differ by sex.

Conclusions: Height z-scores were significantly reduced in children with PCD compared to reference values, but they did not change over time. This might suggest that PCD affects growth already at an early stage of life but there is no catch-up until the age of 20. In the ongoing study, we will look at birth weights, stratify by levels of diagnostic certainty and determine patient characteristics associated with growth, such as lung function, microbiology and clinical characteristics.

Funding: FP7 grant 305404,SNF 32003B-162820,Lungenliga Bern,Milena Carvanal–Pro Kartagener.

  • Orphan disease
  • Epidemiology
  • Adolescents
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Changes in height and BMI in children and adolescents with primary ciliary dyskinesia (PCD) during the growth period: An iPCD cohort study
Myrofora Goutaki, Florian Halbeisen, Philipp Latzin, Elisabeth Maurer, Carmen Casaulta, Suzanne Crowley, Eric Haarman, Jane S. Lucas, Lucy Morgan, Kim G. Nielsen, Francesca Santamaria, Nico Schwerk, Guillaume Thouvenin, Panayiotis Yiallouros, Alban Ramette, Claudia E. Kuehni
European Respiratory Journal Sep 2016, 48 (suppl 60) PA3127; DOI: 10.1183/13993003.congress-2016.PA3127

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Changes in height and BMI in children and adolescents with primary ciliary dyskinesia (PCD) during the growth period: An iPCD cohort study
Myrofora Goutaki, Florian Halbeisen, Philipp Latzin, Elisabeth Maurer, Carmen Casaulta, Suzanne Crowley, Eric Haarman, Jane S. Lucas, Lucy Morgan, Kim G. Nielsen, Francesca Santamaria, Nico Schwerk, Guillaume Thouvenin, Panayiotis Yiallouros, Alban Ramette, Claudia E. Kuehni
European Respiratory Journal Sep 2016, 48 (suppl 60) PA3127; DOI: 10.1183/13993003.congress-2016.PA3127
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