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Eur Respir J. 2017 Dec 21;50(6). pii: 1701659. doi: 10.1183/13993003.01659-2017. Print 2017 Dec.

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

Author information

1
Institute of Social and Preventive Medicine, University of Bern, Bern, Switzerland.
2
Paediatric Respiratory Medicine, Children's University Hospital of Bern, University of Bern, Bern, Switzerland.
3
Primary Ciliary Dyskinesia Centre, NIHR Respiratory Biomedical Research Centre, University of Southampton, Southampton, UK.
4
School of Applied Psychology, University College Cork, Cork, Ireland.
5
Dept of Paediatrics, University Hospital Gasthuisberg, Leuven, Belgium.
6
Primary Ciliary Dyskinesia Centre, Dept of Paediatrics, Royal Brompton and Harefield Foundation Trust, London, UK.
7
Unit for Paediatric Heart, Lung, Allergic Diseases, Rikshospitalet, Oslo, Norway.
8
Dept of Pediatrics, SickKids Hospital, University of Toronto, Toronto, Canada.
9
Dept of Pediatrics, School of Medicine, Washington University, St Louis, MO, USA.
10
Dept of Pediatric Pulmonology, VU University Medical Center, Amsterdam, The Netherlands.
11
Dept of Pediatric Pulmonology, School of Medicine, Marmara University, Istanbul, Turkey.
12
Dept of Medicine, Marsico Lung Institute, School of Medicine, University of North Carolina, Chapel Hill, NC, USA.
13
Dept of Paediatric Pneumology, University Children's Hospital, Ruhr University, Bochum, Germany.
14
Dept of Pediatrics, Marsico Lung Institute, School of Medicine, University of North Carolina, Chapel Hill, NC, USA.
15
Host Defence Unit, Royal Brompton and Harefield NHS Foundation Trust, London, UK.
16
Dept of Pneumonology and Cystic Fibrosis, Institute of Tuberculosis and Lung Disorders, Rabka-Zdrój, Poland.
17
Dept of Respiratory Medicine, Concord Hospital Clinical School, University of Sydney, Sydney, Australia.
18
Danish PCD Centre Copenhagen, Paediatric Pulmonary Service, Copenhagen University Hospital, Copenhagen, Denmark.
19
Dept of Pediatrics, University of Colorado School of Medicine, Aurora, CO, USA.
20
Dept of Translational Medical Sciences, Federico II University, Napoli, Italy.
21
Clinic for Paediatric Pulmonology, Allergiology and Neonatology, Hannover Medical School, Hannover, Germany.
22
School of Medicine, University of Cyprus, Nicosia, Cyprus.
23
Institute of Social and Preventive Medicine, University of Bern, Bern, Switzerland claudia.kuehni@ispm.unibe.ch.

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.

PMID:
29269581
DOI:
10.1183/13993003.01659-2017
[Indexed for MEDLINE]
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