1. Cardiopulmonary assessment in primary ciliary dyskinesia
- Author
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Valerio G, Montella S, Vaino N, Mirra V, GIALLAURIA, FRANCESCO, VIGORITO, CARLO, SANTAMARIA, FRANCESCA, Valerio, G, Giallauria, Francesco, Montella, S, Vaino, N, Vigorito, Carlo, Mirra, V, and Santamaria, Francesca
- 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±2·8years) 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 1s, 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
- Published
- 2012