Duchenne muscular dystrophy: the effect of glucocorticoids on ventilator use and ambulation

American Journal of Physical Medicine & Rehabilitation
John R BachBilal Saulat

Abstract

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. 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. 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 usi...Continue Reading

References

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Dec 3, 2005·Pediatrics·UNKNOWN American Academy of Pediatrics Section on Cardiology and Cardiac Surgery
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Dec 1, 2009·Lancet Neurology·Katharine BushbyUNKNOWN DMD Care Considerations Working Group

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Citations

Sep 24, 2013·Journal of Neurology·Fumi TakeuchiShin'ichi Takeda
Jun 30, 2012·The Journal of Pharmacology and Experimental Therapeutics·Andreas R BaudyEric P Hoffman
Apr 8, 2016·European Journal of Paediatric Neurology : EJPN : Official Journal of the European Paediatric Neurology Society·Anne HuntChristian deGoede
Mar 24, 2016·Pediatric Physical Therapy : the Official Publication of the Section on Pediatrics of the American Physical Therapy Association·Elise L TownsendTara M Holmes
Apr 17, 2012·Annals of Rehabilitation Medicine·Il-Young JungMoon Suk Bang
Oct 13, 2017·Muscle & Nerve·Barbara K Smith, Darin J Falk
May 17, 2014·Jornal brasileiro de pneumologia : publicaça̋o oficial da Sociedade Brasileira de Pneumologia e Tisilogia·Marcos Rojo RodriguesSuely Kazue Nagahashi Marie
Jul 23, 2011·Physiological Research·D FilipovićZ Grubic
Mar 21, 2020·American Journal of Physical Medicine & Rehabilitation·Alexander E BrahmsteadtPaola Pierucci

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