Abstract
Introduction: Weak coughing is perceived as the cause for recurrent respiratory system infection leading to lung function deterioration in Ataxia telangiectasia (A-T) disease. The cough profile of these patients has not been studied.
Aim: To explore the feasibility of the cough-flow-volume profile for detecting cough performance in A-T patients.
Methods: Thirty five A-T patients (age 12.7±4.9yrs) were studied. Patients performed forced expiratory flow volume (FVC) and maximal voluntary cough (FVC-cough) maneuvers. Analysis of data included: Inspiratory volume (IC) prior to cough; FVC-cough, Peak cough flow and number of spikes per maneuver. Values were related to publish data of healthy population of similar ages and are presented as actual and as%predicted.
Results: We found that IC prior to cough was 0.85+0.47 (l) (36.1±15.0%); FVC-cough was 1.00±0.51 (l) (43.6±15.4%); Peak cough flow was 3.27±1.53 (l/s) (45.5±15.0%); Peak cough flow to Peak expiratory flow ratio was 1.06±0.24 vs.1.48±0.22 in healthy and the number of spikes/maneuver were 2.0±0.8 vs. 6-12 in healthy population. All parameters were significantly lower than healthy (P<0.001). Additionally, Peak cough flow increased with age but the yearly increase rate was significantly lower than normal, (0.157 vs. 0.423 l/s/year; respectively, P<0.005).
Conclusions: Our findings indicate that A-T patients have a weak cough compared to healthy of similar ages and that cough ability worsens with age. Cough flow volume curve, as well as forced vital capacity maneuvers, should be considered a mainstay in the clinical assessment of A-T patients.
The study was funded by the J. Baum foundation of the Israeli Lung Association, Tel Aviv, Israel.
- © 2011 ERS
