Volume 42, Issue 6 pp. 617-622
ORIGINAL ARTICLE

Cardiopulmonary assessment in primary ciliary dyskinesia

Giuliana Valerio

Giuliana Valerio

Dipartimento di Studi delle Istituzioni e dei Sistemi Territoriali, Università degli Studi di Napoli “Parthenope”

Search for more papers by this author
Francesco Giallauria

Francesco Giallauria

Dipartimento di Medicina Clinica, Scienze Cardiovascolari ed Immunologiche, Area Funzionale di Riabilitazione Cardiologica

Search for more papers by this author
Silvia Montella

Silvia Montella

Dipartimento di Pediatria, Università degli Studi di Napoli “Federico II”, Naples, Italy

Search for more papers by this author
Nicola Vaino

Nicola Vaino

Dipartimento di Studi delle Istituzioni e dei Sistemi Territoriali, Università degli Studi di Napoli “Parthenope”

Search for more papers by this author
Carlo Vigorito

Carlo Vigorito

Dipartimento di Medicina Clinica, Scienze Cardiovascolari ed Immunologiche, Area Funzionale di Riabilitazione Cardiologica

Search for more papers by this author
Virginia Mirra

Virginia Mirra

Dipartimento di Pediatria, Università degli Studi di Napoli “Federico II”, Naples, Italy

Search for more papers by this author
Francesca Santamaria

Francesca Santamaria

Dipartimento di Pediatria, Università degli Studi di Napoli “Federico II”, Naples, Italy

Search for more papers by this author
First published: 10 November 2011
Citations: 28
Francesca Santamaria, MD, Dipartimento di Pediatria, Università degli Studi di Napoli “Federico II”, Via Pansini 5, 80131 Naples, Italy. Tel.: +39 081 7463495; fax: +39 081 7463116; e-mail: [email protected]

Abstract

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.

The full text of this article hosted at iucr.org is unavailable due to technical difficulties.