Pulmonary function in Duchenne muscular dystrophy related to stage of disease

摘要: Abstract Thirty-eight patients with muscular dystrophy of the Duchenne type representing disease classification stages 2 through 9 were studied for changes in pulmonary function. Lung volumes, flow rates and airway resistance correlated maximal inspiratory expiratory pressure laboratory evidence muscle including serum aldolase creatine-creatinine excretion. When are grouped according to functional class, deterioration function pressures seems begin between class 5 6. In higher classes a reduction total lung capacity an increase residual volume is associated loss strength. Airway was normal all subjects whom it could be measured. Blood gases showed oxygen tension (pO ) subjects; one slight carbon dioxide (pCO ). Breathing per cent produced hyperventilation subjects. Pulmonary studies, particularly blood conjunction may significant help estimating prognosis determining intensity acute care infection or symptoms respiratory failure develop. Based on our clinical experience, pCO subject without extremely bad prognostic sign. After appropriate therapy, status hypoventilation often returns what before infection. Patients 8 below have good this regard.