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Long‐term follow‐up of patients with Duchenne muscular dystrophy receiving ventilatory support
Author(s) -
Fukunaga Hidetoshi,
Okubo Ryuichi,
Moritoyo Takashi,
Kawashima Nozomu,
Osame Mitsuhiro
Publication year - 1993
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.880160519
Subject(s) - duchenne muscular dystrophy , respirator , medicine , muscular dystrophy , pulmonary function testing , ventilation (architecture) , surgery , physical therapy , mechanical engineering , materials science , engineering , composite material
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. © 1993 John Wiley & Soncs, Inc.