PT  - JOURNAL ARTICLE
AU  - Myrofora Goutaki
AU  - Elisabeth Maurer
AU  - Mieke Boon
AU  - Carmen Casaulta
AU  - Jane S. Lucas
AU  - Lucy Morgan
AU  - Kim G. Nielsen
AU  - Panayiotis Yialouros
AU  - Claudia E. Kuehni
TI  - Growth in patients with primary ciliary dyskinesia (PCD): A multinational study
DP  - 2014 Sep 01
TA  - European Respiratory Journal
PG  - P1246
VI  - 44
IP  - Suppl 58
4099  - http://erj.ersjournals.com/content/44/Suppl_58/P1246.short
4100  - http://erj.ersjournals.com/content/44/Suppl_58/P1246.full
SO  - Eur Respir J2014 Sep 01; 44
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.