The pulmonary function of 55 patients with Duchenne type progressive muscular dystrophy was investigated with distinction of such categories as their age, the curvature of their chest walls and the so-called Disease Grades established by Ministry of Welfare. The movements, due to exercise, of their ventilation and blood gas were also examined.
The results are summarized as follows:
(1) In proportion as the patients grew older, their disease grades and the curvature of their chest walls progressed.
(2) The fall of %VC·%MVV and Pao
2 was caused by the restrictive ventilatory disturbance of their pulmonary function. The older they grew, the worse their restrictive ventilatory disturbance became. The enlargement of their chest wall curvature promoted the development of their pulmonary function disturbance.
(3) The movements of their respiratory muscle force, whose debility gave rise to the low ventilation of their lung alveoli, firstly showed the fall of their inspiratory muscle force, and secondly showed that of their expiratory muscle force.
(4) The patients with Duchenne's DMP, after 13 years of age, went into the group of the disease grades, of which the patients were unable to walk. The pulmonary function of the patients of these disease grades got worse, and turned out to be respiratory failure. When their chest wall curvature was more than 31 degrees and their disease grades were over VII, their %VC decreased below 40% and Pao
2 below 75mmHg. It can be supposed that Paco
2 has a rising tendency.
(5) In the group of 60%>%VC>40%, the increase of their ventilation volume by exercise depended chiefly on their breathing frequency. And the respiration of the patients after exercise changed into a fast and shallow breathing. In this case the fall of Pao
2 and the rise of Paco
2 were found out.
(6) It can be thought that the changes of their pulmonary function for the worse are shown by the disease grades, which can be considered to be an index of debility of the muscle force of bodies, legs and arms. Considering that a group of patients above VII in the disease grades are in a state of chronic respiratory failure, with respect to their respiration good medical care should be taken of these patients. Regarding the V-VI grade, in which their inspiratory muscle force starts to decline, as a previous stage to respiratory failure, we should deal with the patients especially with care.
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