Breathing pattern adopted by children with cystic fibrosis with mild to moderate pulmonary impairment during exercise.

It is well known that severe lung impairment in cystic fibrosis (CF) may compromise respiratory muscle function at rest. Even though patients with CF and severe obstructive lung disease exhibit an abnormal breathing pattern during exercise (due to expiratory flow limitation), patients with CF and normal lung function reportedly have a normal breathing pattern. The aim of the study was to assess the precise characteristics of the ventilatory pattern adopted during exercise by children with CF and mild to moderate lung disease. Nine children diagnosed as having mild to moderate CF and 9 healthy children with a similar age distribution participated in this study. Both groups performed a continuous incremental cycling protocol. Breathing and timing components were assessed during exercise. Differences in the breathing pattern between children with CF and controls during exercise are illustrated in Hey plot which described a rapid shallow breathing pattern in children with CF. During exercise, children with CF showed a significantly lower mean inspiratory flow than healthy children (p < 0.001), whereas the mean expiratory flow was higher (p < 0.001). Children with CF also showed a significant increase in the end-tidal carbon dioxide pressure, which may indicate the emergence of hypercapnia. During exercise, children with CF (even those not suffering from advanced disease) showed signs of rapid, shallow breathing and an increase in the ventilatory response. This was essentially due to an increase in the mean inspiratory flow, which in turn suggests an expiratory flow limitation. The children were also predisposed to hypercapnia.

Keochkerian D ，Chlif M ，Delanaud S ，Gauthier R ，Maingourd Y ，Ahmaidi S ... -  《-》

Breathing pattern and chest wall volumes during exercise in patients with cystic fibrosis, pulmonary fibrosis and COPD before and after lung transplantation.

Wilkens H ，Weingard B ，Lo Mauro A ，Schena E ，Pedotti A ，Sybrecht GW ，Aliverti A ... -  《-》

Mechanical ventilatory constraints during incremental exercise in healthy and cystic fibrosis children.

To analyze breathing pattern and mechanical ventilatory constraints during incremental exercise in healthy and cystic fibrosis (CF) children. Thirteen healthy children and 6 children with cystic fibrosis volunteered to perform an incremental test on a treadmill. Exercise tidal flow/volume loops were plotted every minute within a maximal flow/volume loop (MFVL). Expiratory flow limitation (expFL expressed in %Vt) was evaluated and end-expiratory and end-inspiratory lung volumes (EELV and EILV) were estimated from expiratory reserve volume relative to vital capacity (ERV/FVC) and from inspiratory reserve volume relative to vital capacity (IRV/FVC). During the incremental exercise, expFL was first observed at 40% of maximal aerobic speed in both groups. At maximal exercise, 46% of healthy children and 83% of CF children presented expFL, without significant effect of cystic fibrosis on the severity of expFL. According to the two-way ANOVA results, both groups adopted similar breathing pattern and breathing strategies as no significant effect of CF has been revealed. But, according to one-way ANOVA results, a significant increase of ERV/FVC associated with a significant decrease of IRV/FVC from resting value shave been observed in healthy children at maximal exercise, but not in CF children. The hypothesis of this study was based on the assumption that mild cystic fibrosis could induce more frequent and more severe mechanical ventilatory constraints due to pulmonary impairment and breathing pattern disturbances. But, this study did not succeed to highlight an effect of mild cystic fibrosis on the mechanical ventilatory constraints (expFL and dynamic hyperinflation) that occur during an incremental exercise. This absence of effect could be due to the absence of an impact of the disease on spirometric data, breathing pattern regulation during exercise and breathing strategy.

Borel B ，Leclair E ，Thevenet D ，Beghin L ，Gottrand F ，Fabre C ... -  《-》

Timing and driving components of the breathing strategy in children with cystic fibrosis during exercise.

The aim of this study was twofold: first, to determine the breathing strategies of children with cystic fibrosis (CF) during exercise, and secondly, to see if there was a correlation with lung function parameters. We determined the tension-time index of the inspiratory muscles (T(T0.1)) during exercise in nine children with CF, who were compared with nine healthy children with a similar age distribution. T(T0.1) was determined as followed T(T0.1) = P0.1/PImax . T(I)/T(TOT), where P0.1 is mouth occlusion pressure, PImax is maximal inspiratory pressure, and T(I)/T(TOT) is the duty cycle. CF children showed a significant decrease of their forced expiratory volume in 1 sec (FEV1), forced vital capacity (FCV), and FEV1/FVC, whereas the residual volume to total lung capacity ratio (RV/TLC) ratio and functional residual capacity (FRC) were significantly increased (P < 0.001). Children with CF showed mild malnutrition assessed by actual weight expressed by percentage of ideal weight for height, age, and gender (weight/height ratio; 82.3 +/- 3.6%). Children with CF showed a significant reduction in their PImax (69.3 +/- 4.2 vs. 93.8 +/- 7 cmH2O). We found a negative linear correlation between PImax and weight/height only in children with CF (r = 0.9, P < 0.001). During exercise, P(0.1), P0.1/PImax, and T(T0.1) were significantly higher, for a same percent maximal oxygen uptake in children with CF. On the contrary, T(I)/T(TOT) ratio was significantly lower in children with CF compared with healthy children. At maximal exercise, children with CF showed a T(T0.1) = 0.16 vs. 0.14 in healthy children (P < 0.001). We observed at maximal exercise that P0.1/PImax increased as FEV1/FVC decreased (r = -0.90, P < 0.001), and increased as RV/TLC increased (r = 0.92, P < 0.001) only in children with CF. Inversely, T(I)/T(TOT) decreased as FEV1/FVC decreased (r = 0.89, P < 0.001), and T(I)/T(TOT) decreased as RV/TLC increased (r = -0.94, P < 0.001). These results suggest that children with CF adopted a breathing strategy during exercise in limiting the increase of the duty cycle. Two determinants of this strategy were degrees of airway obstruction and hyperinflation.

Keochkerian D ，Chlif M ，Delanaud S ，Gauthier R ，Maingourd Y ，Ahmaidi S ... -  《-》

Respiratory factors do not limit maximal symptom-limited exercise in patients with mild cystic fibrosis lung disease.

Dodd JD ，Barry SC ，Gallagher CG 《RESPIRATORY PHYSIOLOGY & NEUROBIOLOGY》