[in Japanese]

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[in Japanese]

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[in Japanese]

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[in Japanese]

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[in Japanese]

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We reviewed the management of inpatients with Duchenne muscular dystrophy (DMD) treated at 27 hospitals specializing in muscular dystrophy treatment based on the results of the 2005 to 2007 muscular dystrophy ward inpatient surveys conducted annually since 1999. Most of the patients surveyed had advanced DMD based on age, severity, and ventilator usage. Use of ventilators was started at the mean age of 21.4±4.9 years. Non-invasive positive pressure ventilator (NIPPY) was used in 69% of the patients; however, more patients underwent tracheotomy after the age of 30. NIPPY was initially used in 93.8% of 275 patients who started to use ventilators after their hospital admission. The 5-year survival of these patients was 85%. One hundred and eight (108) patients died at the mean age of 29.5±6.2 years. The cause of death was heart failure in 54 patients (49.1%), respiratory failure in 14 patients (12.7%), and respiratory infection in 9 patients (8.2%). Two patients died of arrhythmia. Use of ventilators prolonged the survival of DMD patients by about 10 years. The prolonged survival may be due to thorough monitoring and prompt respiratory management in the hospital setting. However, that 37% of patients on NIPPY died of respiratory failure suggests the current respiratory management in DMD patients and its limitations need to be reviewed.

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[in Japanese]

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