We clinically investigated the process through which the pulmonary function of patients with progressive muscular dystrophy declined to respiratory failure. With the object of taking preventive measures against respiratory failure and heart failure, we made a joint study of the cardiopulmonary function. Clinical pictures of 232 cases with PMD were investigated by disease types—Duchenne type, limb-girdle type, and other types. The cases of Duchenne type were especially examined by disability stage.
(1) The cases of Duchenne type were found in large numbers in young patients of male sex, those of limb-girdle type in older patients, and the cases of other types were found in various ages.
(2) The results of the cases examined by disease types were summarized as follows:
1) The cases of the thoracic vertebrae scoliosis of more than 20°, of the contraction of thorax anteroposterior diameter and of cardio-thoracic rate >0.5 were respectively found in large numbers in Duchenne type.
2) As for enzyme system such as GOT, GPT, LDH or CPK, in 'limb-girdle type and in other types the normal cases were 70%. In Duchenne type the abnormal cases were about 60%, and the value of enzyme activity was high.
3) As for ventilatory function, the cases of %VC<59% were found in large numbers. The cases of heavy ventilatory disturbance with %VC<39% formed 43.5%. The normal cases of FEV
1.0% were more than 95% in each disease type. The normal cases of blood gas and SaO
2 were found 65%. The cases, the PaO
2 of which was less than 79mmHg, were found 40%. The cases being lower than the normal value of PaCO
2 were found both in Duchenne type and in other types. The cases with higher value of PaCO
2 were found 6.3% in each disease type. As for pH, the acidosis cases were found only in Duchenne type.
4) The cases, examined by EKG, pulmonary P wave, and right ventricle overloading, had no difference among the disease types.
(3) The results about cases of Duchenne type in each disability stage were summarized as follows:
1) With the progress of disability stage the cases of the thoracic vertebrae scoliosis and of the contraction of thorax anteroposterior diameter increased the more. The cardio-thoracic rate had no connection with the progress of disability stage.
2) As for enzyme system such as GOT, GPT, LDH or CPK, which was concerned with muscular metabolism, the value of its activity became lower with the progress of muscular atrophy or degeneration.
3) The ventilatory function of the patients in Duchenne type lowered with the progress of disability stage. The cases of PaO
2 below 79mmHg were about half. Lower value of PaCO
2 than the normal one formed about half of the cases of Duchenne type. Higher value of PaCO
2 was found in the cases above Stage VII. The cases, examined by EKG, pulmonary P wave and right ventricle overloading, had no difference among all disability stages. There was no relation between the results of EKG and the development of the disease. The results suggested the necessity of our synthetic judgement which should incorporate other medical ways to judge the cardio-function.
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