Growth and nutritional status, and their association with lung function: a study from the international Primary Ciliary Dyskinesia Cohort
Myrofora Goutaki
(1, 2)
,
Florian S Halbeisen
(1)
,
Ben D Spycher
(1)
,
Elisabeth Maurer
(1)
,
Fabiën Belle
(1)
,
Israel Amirav
(3)
,
Laura Behan
(4, 5)
,
Mieke Boon
(6)
,
Siobhan Carr
(7)
,
Carmen Casaulta
(2)
,
Annick Clement
(8, 9, 10)
,
Suzanne Crowley
(11)
,
Sharon Dell
(12)
,
Thomas Ferkol
(13)
,
Eric G Haarman
(14)
,
Bulent Karadag
(15)
,
Michael Knowles
(16)
,
Cordula Koerner-Rettberg
(17)
,
Margaret W Leigh
(16)
,
Michael R Loebinger
(7)
,
Henryk Mazurek
(18)
,
Lucy Morgan
(19)
,
Kim G Nielsen
(20)
,
Maria Phillipsen
(20)
,
Scott D Sagel
(21)
,
Francesca Santamaria
(22)
,
Nicolaus Schwerk
(23)
,
Panayiotis Yiallouros
(24)
,
Jane S. Lucas
(4)
,
Claudia E Kuehni
(1)
1
University of Bern
2 Inselspital Bern
3 University of Alberta
4 University of Southampton
5 UCC - University College Cork
6 University Hospital Gasthuisberg [Leuven]
7 Royal Brompton and Harefield NHS Foundation Trust
8 Centre de référence national pour les maladies respiratoires rares de l’enfant RespiRare [CHU Trousseau]
9 Service de Pneumologie pédiatrique [CHU Trousseau]
10 Physiopathologie des maladies génétiques d'expression pédiatrique
11 Oslo University Hospital [Oslo]
12 University of Toronto
13 Washington University School of Medicine in St. Louis
14 VU University Medical Center [Amsterdam]
15 Marmara University [Kadıköy - İstanbul]
16 UNC - University of North Carolina [Chapel Hill]
17 RUB - Ruhr University Bochum
18 ITLD - Institute of Tuberculosis and Lung Disorders [Rabka-Zdrój, Poland]
19 The University of Sydney
20 Copenhagen University Hospital
21 University of Colorado Anschutz [Aurora]
22 University of Naples Federico II = Università degli studi di Napoli Federico II
23 MHH - Hannover Medical School [Hannover]
24 University of Cyprus = Université de Chypre
2 Inselspital Bern
3 University of Alberta
4 University of Southampton
5 UCC - University College Cork
6 University Hospital Gasthuisberg [Leuven]
7 Royal Brompton and Harefield NHS Foundation Trust
8 Centre de référence national pour les maladies respiratoires rares de l’enfant RespiRare [CHU Trousseau]
9 Service de Pneumologie pédiatrique [CHU Trousseau]
10 Physiopathologie des maladies génétiques d'expression pédiatrique
11 Oslo University Hospital [Oslo]
12 University of Toronto
13 Washington University School of Medicine in St. Louis
14 VU University Medical Center [Amsterdam]
15 Marmara University [Kadıköy - İstanbul]
16 UNC - University of North Carolina [Chapel Hill]
17 RUB - Ruhr University Bochum
18 ITLD - Institute of Tuberculosis and Lung Disorders [Rabka-Zdrój, Poland]
19 The University of Sydney
20 Copenhagen University Hospital
21 University of Colorado Anschutz [Aurora]
22 University of Naples Federico II = Università degli studi di Napoli Federico II
23 MHH - Hannover Medical School [Hannover]
24 University of Cyprus = Université de Chypre
Myrofora Goutaki
- Function : Author
- PersonId : 817480
- ORCID : 0000-0001-8036-2092
Israel Amirav
- Function : Author
- PersonId : 1239818
- ORCID : 0000-0002-6917-5285
Annick Clement
- Function : Author
- PersonId : 1029287
- IdHAL : nadianathan
- ORCID : 0000-0001-5149-7975
Jane S. Lucas
- Function : Author
- PersonId : 1168631
- ORCID : 0000-0001-8701-9975
Claudia E Kuehni
Connectez-vous pour contacter l'auteur
- Function : Correspondent author
- PersonId : 1239819
Connectez-vous pour contacter l'auteur
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.