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Multicenter analysis of body mass index, lung function, and sputum microbiology in primary ciliary dyskinesia
Pediatric pulmonology, 2014-12, Vol.49 (12), p.1243-1250
Maglione, Marco
Bush, Andrew
Nielsen, Kim G.
Hogg, Claire
Montella, Silvia
Marthin, June K.
Di Giorgio, Angela
Santamaria, Francesca
2014
Volltextzugriff (PDF)
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Autor(en) / Beteiligte
Maglione, Marco
Bush, Andrew
Nielsen, Kim G.
Hogg, Claire
Montella, Silvia
Marthin, June K.
Di Giorgio, Angela
Santamaria, Francesca
Titel
Multicenter analysis of body mass index, lung function, and sputum microbiology in primary ciliary dyskinesia
Ist Teil von
Pediatric pulmonology, 2014-12, Vol.49 (12), p.1243-1250
Ort / Verlag
United States: Blackwell Publishing Ltd
Erscheinungsjahr
2014
Quelle
MEDLINE
Beschreibungen/Notizen
Summary 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 FEF25–75 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 FEF25–75 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. Pediatr Pulmonol. 2014; 49:1243–1250. © 2014 Wiley Periodicals, Inc.
Sprache
Englisch
Identifikatoren
ISSN: 8755-6863
eISSN: 1099-0496
DOI: 10.1002/ppul.22984
Titel-ID: cdi_crossref_primary_10_1002_ppul_22984
Format
–
Schlagworte
Adolescent
,
Body Mass Index
,
Child
,
Child, Preschool
,
Cross-Sectional Studies
,
Europe - epidemiology
,
Female
,
Humans
,
Kartagener Syndrome - epidemiology
,
Longitudinal Studies
,
Male
,
Medical research
,
Microbiology
,
primary ciliary dyskinesia
,
Prospective Studies
,
Respiratory Function Tests
,
Spirometry
,
Sputum - microbiology
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