caa a22 4500 469049073 CHVBK 20180323132825.0 cr unu---uuuuu 170328e19920501xx s 000 0 eng 10.1007/BF00174317 doi (NATIONALLICENCE)springer-10.1007/BF00174317 Pulmonary function and resting breathing pattern in myotonic dystrophy [Elektronische Daten] [Jan Bogaard, Frans van der Meche, Irene Hendriks, Carin Ververs] In 17 patients with myotonic dystrophy, spirometric, flow-volume, and maximal mouth occlusion variables were obtained and compared with 8 normal subjects. Ventilatory CO2, response was measured by the estimation of the steady-state effect of a sufficiently large serial dead space. Variability of resting breathing pattern was expressed by the variation coefficients of respiratory cycle time and tidal volume. The group means of the total lung capacity (TLC), vital capacity (VC), forced expiratory volume in 1 sec (FEV)1, and forced inspiratory volume in 1 sec (FIV)1, showed a restrictive pattern. Only maximal static mouth pressure (Pi,max), measured at residual volume (RV) level, showed a significant positive correlation with both VC (p = 0.03) and FIV1, (p = 0.02), suggesting inspiratory muscle weakness as a determinant of the restriction. Although the differences were just not significant, both variation coefficients of the respiratory cycle time and tidal volume were larger in the group with a CO, sensitivity below the lower limit of normal compared to those with a normal ventilatory response to CO2. In 3 patients, fluctuations in FRC were also present. We hypothesize that, in addition to the already documented FRC fluctuations by uncoordinated spontaneous intercostal muscle action, a defect of integration of afferent neural input and chemical drive in the medullary region may also be present in these patients. Springer-Verlag, 1992 Myotonic dystrophy nationallicence Pulmonary function nationallicence Breathing pattern nationallicence Ventilatory CO2 sensitivity nationallicence Bogaard Jan Pathophysiological Laboratory of the Department of Pulmonary Diseases University Hospital Rotterdam ‘Dijkzigt', Erasmus University, Rotterdam, The Netherlands aut van der Meche Frans Department of Neurology University Hospital Rotterdam ‘Dijkzigt', Erasmus University, Rotterdam, The Netherlands aut Hendriks Irene Pathophysiological Laboratory of the Department of Pulmonary Diseases University Hospital Rotterdam ‘Dijkzigt', Erasmus University, Rotterdam, The Netherlands aut Ververs Carin Pathophysiological Laboratory of the Department of Pulmonary Diseases University Hospital Rotterdam ‘Dijkzigt', Erasmus University, Rotterdam, The Netherlands aut Lung Springer-Verlag 170/3(1992-05-01), 143-153 0341-2040 170:3<143 1992 170 408 https://doi.org/10.1007/BF00174317 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/BF00174317 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Bogaard Jan Pathophysiological Laboratory of the Department of Pulmonary Diseases University Hospital Rotterdam ‘Dijkzigt', Erasmus University, Rotterdam, The Netherlands aut NATIONALLICENCE 700 1- van der Meche Frans Department of Neurology University Hospital Rotterdam ‘Dijkzigt', Erasmus University, Rotterdam, The Netherlands aut NATIONALLICENCE 700 1- Hendriks Irene Pathophysiological Laboratory of the Department of Pulmonary Diseases University Hospital Rotterdam ‘Dijkzigt', Erasmus University, Rotterdam, The Netherlands aut NATIONALLICENCE 700 1- Ververs Carin Pathophysiological Laboratory of the Department of Pulmonary Diseases University Hospital Rotterdam ‘Dijkzigt', Erasmus University, Rotterdam, The Netherlands aut NATIONALLICENCE 773 0- Lung Springer-Verlag 170/3(1992-05-01), 143-153 0341-2040 170:3<143 1992 170 408 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer