RT Journal Article
SR Electronic
T1 Growth in patients with primary ciliary dyskinesia (PCD): A multinational study
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP P1246
VO 44
IS Suppl 58
A1 Goutaki, Myrofora
A1 Maurer, Elisabeth
A1 Boon, Mieke
A1 Casaulta, Carmen
A1 Lucas, Jane S.
A1 Morgan, Lucy
A1 Nielsen, Kim G.
A1 Yialouros, Panayiotis
A1 Kuehni, Claudia E.
YR 2014
UL http://erj.ersjournals.com/content/44/Suppl_58/P1246.abstract
AB Background: Poor growth is a common problem in children with severe chronic respiratory disease, but little is known for PCD. In the EU project BESTCILIA, we assessed height and body mass index (BMI) in a multinational PCD dataset, compared to WHO reference values.Methods: We analysed 2633 measurements of height and weight from 296 paediatric and adult PCD patients from 6 centres (Australia, Belgium, Cyprus, Denmark, UK and Switzerland). We used World Health Organization (WHO) growth reference data to calculate z-scores for height and BMI. To account for repeated measurements we used a multilevel model, adjusting for age, sex and study centre.Results: The mean age of patients at the time of measurement was 20 years (range 0-72). We found a mean height z-score of 0.07 (SD=1.11) and a mean BMI z-score of 0.93 (3.41). 17/296 (5.74%) patients had BMI z-score <-2. In the multilevel model, there was no difference between measured height in PCD patients and normal values (z-score: -0.03, p=0.673), but we found a higher BMI compared to WHO references (z-score: 1.70, p<0.001). Males, children aged <10 years and older adults deviated more from the normal values (higher BMI). Results differed by country, with BMI z-scores being highest in patients from Australia and Switzerland.Conclusions: In our study, we found higher BMI z-scores compared to WHO growth reference data. This suggests that undernutrition might be a minor problem in our population. In a next step, we will compare the data also to national reference values, determine patient characteristics associated with growth, especially level of lung function and evaluate changes over time.Funding: FP7 grant 305404, Lungenliga Bern, Lungenliga St.Gallen.