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- W2754702448 abstract "We investigated the cause of death, the provision of ventilatory support and steroid use in a cohort of Duchenne muscular dystrophy (DMD) patients, born between 1999-1980.One hundred and one subjects were identified. Data on outcome were available in 88: 29 (33%) died at a mean age of 21.3 years (SD 4.56; range 13–29), 59 subjects are currently alive (67%) (mean age 23.25 years; SD 4.55; range 18–37). In the deceased population 27 (93 %) never received steroids. Respiratory insufficiency was the cause of death in 6 (20.6%), all born between 1980–1982 and not provided with ventilatory support. 18 patients (62%) died from cardiac events and 3 (10%) subjects expressed explicitely their wish to stop interventions. Other causes of death were gastro intestinal complications (1) and fat embolism (1). Of the 59 subjects currently alive 19 (32%) never received steroids,6 (10%) previously and 34 (58%) are currently on steroids. Of the 37 subjects born between '99-'95, 29 (78 %) are on steroid,6 (16%) had been previously and 2 (5%) never. Nine subjects were born between ‘94-‘90, 3 (33%) are on steroids, 2 (22%) previously and 4 (44%) never. Six subjects were born between ‘89-‘85, 3 (50%) are currently on steroids, 3 (50%) have never been. In the oldest cohort born ‘84-‘80 (N = 7), one subject is still on steroids, 6 never were offered. Five steroid treated subjects needed scoliosis surgery after the age of 18. Use of (non-invasive) ventilation (NIV) increased with age : 16%, 77%,66% and 100 % respectively for the cohorts born between ’99-'95,'94-90,'89-'85,'84-'80. Steroid treatment and the provision of NIV have increased life expectancy for adults with DMD, however cardiac complications remain a major cause of early death. Long term steroid treatment raises many new challenges in the management of adult DMD among which delayed onset of scoliosis." @default.
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- W2754702448 date "2017-10-01" @default.
- W2754702448 modified "2023-09-26" @default.
- W2754702448 title "Has outcome changed for adults with Duchenne muscular dystrophy?" @default.
- W2754702448 doi "https://doi.org/10.1016/j.nmd.2017.06.033" @default.
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