Document Detail

Cardiopulmonary assessment in primary ciliary dyskinesia.
MedLine Citation:
PMID:  22121832     Owner:  NLM     Status:  Publisher    
Eur J Clin Invest 2011 ABSTRACT: Background  Primary ciliary dyskinesia (PCD) is a rare, usually autosomal recessive disorder of ciliary dysfunction associated with lung involvement, which has a great impact on health. There is limited information concerning the aerobic fitness of children and adolescents with PCD. The aim of this study was to assess cardiopulmonary functional capacity and its relationship with pulmonary function and physical activity (PA) levels in patients with PCD. Design  Ten patients with PCD (age 13·2 ± 2·8 years) underwent spirometry and cardiopulmonary exercise testing. PA was investigated through a questionnaire. Eight age- and body mass index-matched healthy children were enrolled as controls. Main variables were forced expiratory volume at 1 s, peak oxygen uptake (VO(2peak) ) and time spent in PA. Results  Forty per cent of patients with PCD had impaired lung function as expressed by FEV(1)  < 85% predicted. Only patients with impaired lung function exhibited reduced VO(2peak) (18·1 ± 7·9 mL/kg/min). Time spent in total daily PA was slightly lower in patients than controls, with no difference between patients with normal or reduced lung function. In multiple regression models, male gender (β = 0·518, P = 0·018), age (β = 0·752, P = 0·035) and time spent in vigorous PA (β = 0·353, P = 0·049) were independent predictors of aerobic fitness. Conclusions  Assessment of resting pulmonary function and cardiopulmonary functional capacity could contribute to the evaluation of pulmonary impairment in PCD. Given the benefit of physical exercise on airway clearance and on general health and quality of life, patients with PCD should be encouraged to adopt an active lifestyle.
Giuliana Valerio; Francesco Giallauria; Silvia Montella; Nicola Vaino; Carlo Vigorito; Virginia Mirra; Francesca Santamaria
Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2011-11-10
Journal Detail:
Title:  European journal of clinical investigation     Volume:  -     ISSN:  1365-2362     ISO Abbreviation:  -     Publication Date:  2011 Nov 
Date Detail:
Created Date:  2011-11-29     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0245331     Medline TA:  Eur J Clin Invest     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
© 2011 The Authors. European Journal of Clinical Investigation © 2011 Stichting European Society for Clinical Investigation Journal Foundation.
Dipartimento di Studi delle Istituzioni e dei Sistemi Territoriali, Università degli Studi di Napoli "Parthenope" Dipartimento di Medicina Clinica, Scienze Cardiovascolari ed Immunologiche, Area Funzionale di Riabilitazione Cardiologica Dipartimento di Pediatria, Università degli Studi di Napoli "Federico II", Naples, Italy.
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