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

Bowden, Sasigarn A; Connolly, Anne M.; Kinnett, Kathi; Zeitler, Philip

doi:10.3233/jnd-180346

Cited by 25 publications

(32 citation statements)

References 47 publications

(37 reference statements)

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“…Both clinical and biochemical evidence of AS have been well described in children after discontinuation of therapeutic doses of systemic GCs 10 12–14. Shorter term systemic GC exposure is associated with more transient AS 15 16.…”

Section: Introductionmentioning

confidence: 99%

Adrenal suppression from glucocorticoids: preventing an iatrogenic cause of morbidity and mortality in children

Ahmet

Mokashi

Goldbloom

et al. 2019

bmjpo

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Adrenal suppression (AS) is an important side effect of glucocorticoids (GCs) including inhaled corticosteroids (ICS). AS can often be asymptomatic or associated with non-specific symptoms until a physiological stress such as an illness precipitates an adrenal crisis. Morbidity and death associated with adrenal crisis is preventable but continues to be reported in children. There is a lack of consensus about the management of children at risk of AS. However, healthcare professionals need to develop an awareness and approach to keep these children safe. In this article, current knowledge of the risk factors, diagnosis and management of AS are reviewed while drawing attention to knowledge gaps and areas of controversy. Possible strategies to reduce the morbidity associated with this iatrogenic condition are provided for healthcare professionals.

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Section: Introductionmentioning

confidence: 99%

Adrenal suppression from glucocorticoids: preventing an iatrogenic cause of morbidity and mortality in children

Ahmet

Mokashi

Goldbloom

et al. 2019

bmjpo

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“…Patients and families should be aware of the risk of adrenal crisis during illness or with sudden cessation of steroid use, and they should discuss this with their health‐care providers. Appropriate dosing of stress dose corticosteroids has been described in previous studies 12,16,17 . Consultation with an endocrinologist is recommended when steroid changes are being made during hospitalization.…”

mentioning

confidence: 99%

The care of patients with Duchenne, Becker, and other muscular dystrophies in the COVID‐19 pandemic

et al. 2020

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The coronavirus disease 2019 pandemic has resulted in the reorganization of health-care settings affecting clinical care delivery to patients with Duchenne and Becker muscular dystrophy (DBMD) as well as other inherited muscular dystrophies. The magnitude of the impact of this public health emergency on the care of patients with DBMD is unclear as they are suspected of having an increased risk for severe manifestations of COVID-19. In this article, the authors discuss their consensus recommendations pertaining to care of these patients during the pandemic. We address issues surrounding corticosteroid and exon-skipping treatments, cardiac medications, hydroxychloroquine use, emergency/respiratory care, rehabilitation management, and the conduct of clinical trials. We highlight the importance of collaborative Abbreviations: COVID-19, coronavirus disease 2019; DBMD, Duchenne and Becker muscular dystrophy; DMD, Duchenne muscular dystrophy; SARS-CoV-2, severe acute respiratory syndrome coronavirus 2.

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“…It is not only important for cell viability that cortisol levels reenter physiological levels within days, also suppression of the hypothalamic-pituitary-adrenal (HPA) axis should be avoided in the body. DMD patients generally receive dosages of 0.75 mg/kg/day prednisolone, which averages 8 times the physiologic dose of hydrocortisone of 10 mg/m 2 /day, potentially leading to HPA axis disturbance [40] and could have an influence on other types of cells in the body. The 8 mM MP used in this study was within the range of plasma levels in DMD patients, meaning 8 times the physiological dosage of HC.…”

Section: Discussionmentioning

confidence: 99%

Description of a Novel Mechanism Possibly Explaining the Antiproliferative Properties of Glucocorticoids in Duchenne Muscular Dystrophy Fibroblasts Based on Glucocorticoid Receptor GR and NFAT5

Herbelet

Paepe

Bleecker

2020

IJMS

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Glucocorticoids are drugs of choice in Duchenne muscular dystrophy (DMD), prolonging patients’ ambulation. Their mode of action at the protein level is not completely understood. In DMD, muscle tissue is replaced by fibrotic tissue produced by fibroblasts, reducing mobility. Nuclear factor of activated T-cells 5 (NFAT5) is involved in fibroblast proliferation. By treating one DMD fibroblast cell culture and one of unaffected skeletal muscle fibroblasts with methylprednisolone (MP) or hydrocortisone (HC) for 24 h or 12 d, the antiproliferative properties of glucocorticoids could be unraveled. NFAT5 localization and expression was explored by immunocytochemistry (ICC), Western blotting (WB) and RT-qPCR. NFAT5 and glucocorticoid receptor (GR) colocalization was measured by ImageJ. GR siRNA was used, evaluating GR’s influence on NFAT5 expression during MP and HC treatment. Cell proliferation was monitored by IncuCyte ZOOM. In DMD fibroblasts, treatment with MP for 24 h induced dots (ICC) positive for NFAT5 and colocalizing with GR. After 12 d of MP or HC in DMD fibroblasts, NFAT5 expression was decreased (RT-qPCR and WB) and growth arrest was observed (Incucyte ZOOM), whereas NFAT5 expression and cell growth remained unchanged in unaffected skeletal muscle fibroblasts. This study may help understand the antiproliferative properties of glucocorticoids in DMD fibroblasts.

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

Cited by 25 publications

References 47 publications

Adrenal suppression from glucocorticoids: preventing an iatrogenic cause of morbidity and mortality in children

Adrenal suppression from glucocorticoids: preventing an iatrogenic cause of morbidity and mortality in children

The care of patients with Duchenne, Becker, and other muscular dystrophies in the COVID‐19 pandemic

Description of a Novel Mechanism Possibly Explaining the Antiproliferative Properties of Glucocorticoids in Duchenne Muscular Dystrophy Fibroblasts Based on Glucocorticoid Receptor GR and NFAT5

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