Sai Katta, Lal Amos, Lakshmi Maradana Jhansi, Velamala Pruthvi Raj, Nitin Trivedi
Department of Internal Medicine, Saint Vincent Hospital at Worcester Medical Center, Worcester, Massachusetts, USA.
Department of Endocrinology, Diabetes, and Metabolism, Saint Vincent Hospital at Worcester Medical Center, Worcester, Massachusetts, USA.
Endocrinol Diabetes Metab Case Rep. 2019 Nov 12;2019. doi: 10.1530/EDM-19-0064.
Mifepristone is a promising option for the management of hypercortisolism associated with hyperglycemia. However, its use may result in serious electrolyte imbalances, especially during dose escalation. In our patient with adrenocorticotropic hormone-independent macro-nodular adrenal hyperplasia, unilateral adrenalectomy resulted in biochemical and clinical improvement, but subclinical hypercortisolism persisted following adrenalectomy. She was started on mifepristone. Unfortunately, she missed her follow-up appointments following dosage escalation and required hospitalization at an intensive care level for severe refractory hypokalemia.
Mifepristone, a potent antagonist of glucocorticoid receptors, has a high risk of adrenal insufficiency, despite high cortisol levels. Mifepristone is associated with hypokalemia due to spill-over effect of cortisol on unopposed mineralocorticoid receptors. Given the lack of a biochemical parameter to assess improvement, the dosing of mifepristone is based on clinical progress. Patients on mifepristone require anticipation of toxicity, especially when the dose is escalated. The half-life of mifepristone is 85 h, requiring prolonged monitoring for toxicity, even after the medication is held.
米非司酮是治疗与高血糖相关的皮质醇增多症的一种有前景的选择。然而，其使用可能导致严重的电解质失衡，尤其是在剂量增加期间。在我们这位促肾上腺皮质激素非依赖性大结节性肾上腺增生患者中，单侧肾上腺切除术带来了生化指标和临床症状的改善，但肾上腺切除术后仍存在亚临床皮质醇增多症。她开始服用米非司酮。不幸的是，在剂量增加后她错过了随访预约，因严重难治性低钾血症而需要在重症监护病房住院治疗。
米非司酮是一种强效糖皮质激素受体拮抗剂，尽管皮质醇水平较高，但仍有较高的肾上腺功能不全风险。米非司酮与低钾血症有关，这是由于皮质醇对未受拮抗的盐皮质激素受体的溢出效应。鉴于缺乏评估改善情况的生化参数，米非司酮的给药基于临床进展。服用米非司酮的患者需要预料到毒性反应，尤其是在剂量增加时。米非司酮的半衰期为85小时，即使停药后也需要长期监测毒性反应。
