Article

OBJECTIVE: To describe the effect of glucocorticoid treatment on age at
wheelchair dependence and at dependence on part-time (<23 hrs/day) and continuous
noninvasive mechanical ventilation. DESIGN: In this retrospective study, patients
with Duchenne muscular dystrophy who received glucocorticoid therapy were
compared with those who did not for ages at wheelchair dependence and when
beginning part-time (nocturnal) and continuous noninvasive intermittent positive
pressure ventilation (NIV). Respiratory symptoms, end-tidal carbon dioxide,
oximetry, and vital capacity were noted every 4-12 mos, and NIV was initiated for
symptomatic nocturnal hypoventilation. The daily NIV use increased until some
required it continuously to survive. RESULTS: The 117 untreated patients became
wheelchair-dependent at 9.7 +/- 1.3 yrs of age. Four then died from cardiac
failure, and five were older than 19 yrs without using NIV. The remaining 108
began nocturnal NIV at 19.2 +/- 3.7 yrs of age. Ninety of the 108 became
continuously NIV-dependent at 21.9 +/- 4.5 yrs of age, and the 17 treated with
glucocorticoid therapy became wheelchair-dependent significantly later at 10.8
+/- 1.3 yrs of age (P = 0.02). Three died from cardiac failure, and three were
older than 19 yrs without using NIV. The remaining 11 began nocturnal NIV at 22.9
+/- 5.3 yrs of age (P = 0.05). Eight of the 11 became continuously NIV-dependent
at age 28.9 +/- 7.3 yrs (P = 0.005). CONCLUSIONS: Intermittent glucocorticoid
therapy delays wheelchair dependence and may delay ventilator dependence for
patients with Duchenne muscular dystrophy.

Langue : ANGLAIS