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Published on: December 5, 2019
Glucocorticoid-Associated Demise of a Patient With Duchenne Muscular Dystrophy
Alexander E Brahmsteadt1, John R Bach, Reza Pishdad
1From the Arizona College of Osteopathic Medicine, Midwestern University, Glendale, Arizona (AEB); Department of Physical Medicine and Rehabilitation, Rutgers University-New Jersey Medical School, Newark, New Jersey (JRB, RP); Department of Medicine, Division of Endocrinology, Rutgers University-New Jersey Medical School, Newark, New Jersey (LC); and Cardio Thoracic Department, Respiratory and Sleep Disorders Unit, Bari Policlinic, Bari, Italy (PP).
Abstract:
We describe the clinical deterioration of a 26-yr-old man with Duchenne muscular dystrophy on oral daily high-dose deflazacort. Although this daily regimen was targeted to benefit ambulation and respiration, it resulted in premature death with lethal sequelae from liver failure, decubiti, diabetes mellitus, and morbid obesity. This case illustrates the need for further research weighing risk versus benefit of daily glucocorticoid therapy, specifically deflazacort, in Duchenne muscular dystrophy patients. Thus, curtailment of daily dosing to eliminate dire sequelae in patients living longer into adulthood than ever before is recommended.
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