hrp0094p2-30 | Adrenals and HPA Axis | ESPE2021

Development of a novel weight-based steroid emergency plan for patients with Duchenne Muscular Dystrophy

Tollerfield Sally , Atterbury Abigail , Antell Hannah , Craig Sian , Smith Natalie , Sarkozy Anna , Manzur Adnan , Hoskins Steve , Amin Rakesh , Dattani Mehul , Katugampola Harshini ,

Background: Long-term glucocorticoid therapy with Prednisolone or Deflazacort has improved outcomes in patients with Duchenne Muscular Dystrophy (DMD), however recommended dosages suppress the hypothalamic-pituitary-adrenal axis, leading to adrenal insufficiency. All boys prescribed glucocorticoid therapy should be assumed to have adrenal suppression, and therefore at risk of adrenal crisis during illness or stress (eg. surgery, bisphosphonate infusions). The ...