LONG-TERM FOLLOW-UP OF PATIENTS WITH DUCHENNE MUSCULAR-DYSTROPHY RECEIVING VENTILATORY SUPPORT

被引:11
作者
FUKUNAGA, H [1 ]
OKUBO, R [1 ]
MORITOYO, T [1 ]
KAWASHIMA, N [1 ]
OSAME, M [1 ]
机构
[1] DEPT INTERNAL MED 3,KAGOSHIMA,JAPAN
关键词
DUCHENNE MUSCULAR DYSTROPHY; RESPIRATORY FUNCTION; NEGATIVE CHEST RESPIRATOR; NIGHT VENTILATION; OXIMETER;
D O I
10.1002/mus.880160519
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
We, retrospectively, examined the clinical course, decline in pulmonary function, and requirements for ventilatory assistance in 54 patients with Duchenne-type muscular dystrophy (DMD) who were followed in the muscle disease ward of the National Hospital in Kagoshima, Japan, over the past 20 years. The percentage of the predicted vital capacity (%VC) declined in relation to age and stage of disease. Most patients required assisted ventilation when the %VC fell below 10%. Twenty patients were treated with a negative pressure chest respirator. Six of these died at the mean age of 23.2 years after being on the respirator for a mean period of 18 months. Fourteen patients are surviving at a mean age of 23.5 years after being on the respirator for a mean period of 39 months.
引用
收藏
页码:554 / 558
页数:5
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