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

Bowden SA, Connolly AM, Kinnett K, Zeitler PS. Management of Adrenal Insufficiency Risk After Long-term Systemic Glucocorticoid Therapy in Duchenne Muscular Dystrophy: Clinical Practice Recommendations. Journal of neuromuscular diseases  2019;6:31-41.

Bowden_2019_J Neuromuscul Dis. Jan 2

Most DMD patients are using corticosteroids, which are known to have many side effects, among others kidney failure (adrenal insufficiency). This is less well-known, while it can be life-threatening. This reviews aims at raising awareness and makes suggestions for the management of kidney failure.

 

Abstract

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.

 

 

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