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The Capital Region of Denmark - a part of Copenhagen University Hospital
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Multicenter analysis of body mass index, lung function, and sputum microbiology in primary ciliary dyskinesia

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  2. The impact of mannose-binding lectin polymorphisms on lung function in primary ciliary dyskinesia

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  1. SPEF2- And HYDIN-mutant cilia lack the central pair-associated protein SPEF2, aiding primary ciliary dyskinesia diagnostics

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  2. Primary ciliary dyskinesia patients have the same P. aeruginosa clone in sinuses and lungs

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  3. The Controversies and Difficulties of Diagnosing Primary Ciliary Dyskinesia

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  4. The impact of mannose-binding lectin polymorphisms on lung function in primary ciliary dyskinesia

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  5. Long-Term Lung Function and Exercise Capacity in Postinfectious chILD

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  • Marco Maglione
  • Andrew Bush
  • Kim G Nielsen
  • Claire Hogg
  • Silvia Montella
  • June K Marthin
  • Angela Di Giorgio
  • Francesca Santamaria
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BACKGROUND: No studies longitudinally, simultaneously assessed body mass index (BMI) and spirometry in primary ciliary dyskinesia (PCD).

METHODS: We determined BMI and spirometry in 158 PCD children and adolescents from London, UK (n = 75), Naples, Italy (n = 23) and Copenhagen, Denmark (n = 60) at first presentation and during follow-up. Annual BMI and spirometry were prospectively collected and analyzed over blocks of 2, 4, and 6 consecutive years. Sputum pathogens were recorded.

RESULTS: Age at first spirometry was 8.7 years (range, 4.2-17.4). Mean Z scores of first measured BMI, FEV1, FVC, and FEF(25-75) were 0.01, -1.37, -0.84, and -1.68, respectively. First spirometry was not more frequently impaired in patients referred at age ≥6 years than in those referred at preschool age (P = 0.13). There were no differences in slopes for BMI, FEV1, FVC, or FEF(25-75) over any time block. H. influenzae was the most common pathogen, isolated at least once in 65% of patients. P. aeruginosa was found in 58 subjects (37%) of whom 8 (5%) were chronically infected. Neither pathogens was associated with spirometry changes.

CONCLUSIONS: Preschool referral to a PCD center was not associated with better spirometry or BMI. PCD children and adolescents receiving centralized care show steady BMI and spirometry during medium term follow-up. There was a high prevalence of Pseudomonas aeruginosa infection, but the evolution of spirometry or BMI was not affected by this microorganism in medium term. Despite our longitudinal analysis showed no differences between the three centers, the assessment of spirometry and BMI over time represents a quality improvement tool. Future studies are needed to highlight the role of spirometry and BMI in long term PCD management and identify subgroups of patients with a higher risk of early lung failure or nutritional problems.

Original languageEnglish
JournalPediatric Pulmonology
Volume49
Issue number12
Pages (from-to)1243-50
Number of pages8
ISSN8755-6863
DOIs
Publication statusPublished - Dec 2014

ID: 45096478