Cardiopulmonary assessment in primary ciliary dyskinesia
Giuliana Valerio
Dipartimento di Studi delle Istituzioni e dei Sistemi Territoriali, Università degli Studi di Napoli “Parthenope”
Search for more papers by this authorFrancesco Giallauria
Dipartimento di Medicina Clinica, Scienze Cardiovascolari ed Immunologiche, Area Funzionale di Riabilitazione Cardiologica
Search for more papers by this authorSilvia Montella
Dipartimento di Pediatria, Università degli Studi di Napoli “Federico II”, Naples, Italy
Search for more papers by this authorNicola Vaino
Dipartimento di Studi delle Istituzioni e dei Sistemi Territoriali, Università degli Studi di Napoli “Parthenope”
Search for more papers by this authorCarlo Vigorito
Dipartimento di Medicina Clinica, Scienze Cardiovascolari ed Immunologiche, Area Funzionale di Riabilitazione Cardiologica
Search for more papers by this authorVirginia Mirra
Dipartimento di Pediatria, Università degli Studi di Napoli “Federico II”, Naples, Italy
Search for more papers by this authorFrancesca Santamaria
Dipartimento di Pediatria, Università degli Studi di Napoli “Federico II”, Naples, Italy
Search for more papers by this authorGiuliana Valerio
Dipartimento di Studi delle Istituzioni e dei Sistemi Territoriali, Università degli Studi di Napoli “Parthenope”
Search for more papers by this authorFrancesco Giallauria
Dipartimento di Medicina Clinica, Scienze Cardiovascolari ed Immunologiche, Area Funzionale di Riabilitazione Cardiologica
Search for more papers by this authorSilvia Montella
Dipartimento di Pediatria, Università degli Studi di Napoli “Federico II”, Naples, Italy
Search for more papers by this authorNicola Vaino
Dipartimento di Studi delle Istituzioni e dei Sistemi Territoriali, Università degli Studi di Napoli “Parthenope”
Search for more papers by this authorCarlo Vigorito
Dipartimento di Medicina Clinica, Scienze Cardiovascolari ed Immunologiche, Area Funzionale di Riabilitazione Cardiologica
Search for more papers by this authorVirginia Mirra
Dipartimento di Pediatria, Università degli Studi di Napoli “Federico II”, Naples, Italy
Search for more papers by this authorFrancesca Santamaria
Dipartimento di Pediatria, Università degli Studi di Napoli “Federico II”, Naples, Italy
Search for more papers by this authorAbstract
Eur J Clin Invest 2012; 42 (6): 617–622
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 (VO2peak) and time spent in PA.
Results Forty per cent of patients with PCD had impaired lung function as expressed by FEV1 < 85% predicted. Only patients with impaired lung function exhibited reduced VO2peak (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.
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