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Clinical Investigations: BreathingPrevention of Pulmonary Morbidity for Patients With Duchenne Muscular Dystrophy
Section snippets
Materials and Methods
Of all patients referred to a regional Jerry Lewis Muscular Dystrophy Association clinic since 1977, 92 were diagnosed as having DMD on the basis of onset and progression of weakness before age 5 years, calf muscle pseudohypertrophy, loss of unassisted ambulation before age 13 years,1 and characteristic elevations of serum creatinine kinase, muscle biopsy in the patient or male relative, and electrodiagnostic examination. More recently, documentation of a Duchenne-type mutation or identical
Results
Forty-eight patients required treatment for respiratory failure. Two patients became dependent on 24-h noninvasive IPPV without being equipped with oximeters or the expiratory aids of this protocol. Both died from pneumonia and respiratory failure complicating intercurrent URTIs. They are no longer considered. The remaining 46 patients included the following: (1) 22 nonprotocol tracheostomy IPPV users who were referred before 1983, referred already using tracheostomy IPPV and for whom the tube
Discussion
The initial objective, that of identifying DMD patients at risk of developing acute respiratory failure, appears to have been achieved because no patients with (assisted) PCF above 270 L/min developed acute respiratory distress. All but one patient for whom assisted PCF were below 270 L/min had VCs below 1,000 mL. Despite 14 patients eventually requiring full-time noninvasive IPPV, two with VCs under 100 mL, and three with severe dysphagia necessitating indwelling gastrostomy tubes, assisted
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