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Guides & Guidelines

Management of Adrenal Insufficiency Risk After Long-term Systemic Glucocorticoid Therapy in Duchenne Muscular Dystrophy: Clinical Practice Recommendations

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source: Journal of Neuromuscular Diseases

year: 2019

authors: Bowden SA, Connolly AM, Kinnett K, Zeitler PS


Long-term glucocorticoid therapy has improved outcomes in patients with Duchenne muscular dystrophy. However, the recommended glucocorticoid dosage suppresses the hypothalamic-pituitary-adrenal axis, leading to adrenal insufficiency that may develop during severe illness, trauma or surgery, and after discontinuation of glucocorticoid therapy.

The purpose of this review is to highlight the risk of adrenal insufficiency in this patient population, and provide practical recommendations for management of adrenal insufficiency, glucocorticoid withdrawal, and adrenal function testing. Strategies to increase awareness among patients, families, and health care providers are also discussed.

organization: Nationwide Children's Hospital/The Ohio State University College of Medicine, USA; Washington University School of Medicine in Saint Louis, USA; Parent Project Muscular Dystrophy, USA; University of Colorado School of Medicine, USA

DOI: 10.3233/JND-180346

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