Saroka Rachel M, Kane Michael P, Robinson Lawrence, Busch Robert S
Department of Pharmacy Practice, Albany College of Pharmacy and Health Sciences, Albany, NY, USA.; Albany Medical Center Division of Community Endocrinology, Albany, NY, USA.
Albany Medical Center Division of Community Endocrinology, Albany, NY, USA.
Clin Med Insights Endocrinol Diabetes. 2016 Jul 26;9:31-6. doi: 10.4137/CMED.S39997. eCollection 2016.
Glucocorticoid replacement is commonly required to treat secondary adrenal insufficiency after surgical resection of unilateral cortisol-secreting adrenocortical adenomas. Here, we describe a patient with unilateral cortisol-secreting adenomas in which the preoperative use of mifepristone therapy was associated with recovery of the hypothalamic-pituitary-adrenal (HPA) axis, eliminating the need for postoperative glucocorticoid replacement.
A 66-year-old Caucasian man with type 2 diabetes mellitus, hyperlipidemia, hypertension, and obesity was hospitalized for Fournier's gangrene and methicillin-resistant Staphylococcus aureus sepsis. Abdominal computed tomography scan revealed three left adrenal adenomas measuring 1.4, 2.1, and 1.2 cm and an atrophic right adrenal gland. Twenty-four-hour urinary free cortisol level was elevated (237 µg/24 hours, reference range 0-50 µg/24 hours). Hormonal evaluation after resolution of the infection showed an abnormal 8 mg overnight dexamethasone suppression test (cortisol postdexamethasone 14.5 µg/dL), suppressed adrenocorticotropic hormone (ACTH; <5 pg/mL, reference range 7.2-63.3 pg/mL), and low-normal dehydroepiandrosterone sulfate (50.5 µg/dL, male reference range 30.9-295.6 µg/dL). Because of his poor medical condition and uncontrolled diabetes, his Cushing's syndrome was treated with medical therapy before surgery. Mifepristone therapy was started and, within five months, his diabetes was controlled and insulin discontinued. The previously suppressed ACTH increased to above normal range accompanied by an increase in dehydroepiandrosterone sulfate levels, indicating recovery of the HPA axis and atrophic contralateral adrenal gland. The patient received one precautionary intraoperative dose of hydrocortisone and none thereafter. Two days postoperatively, ACTH (843 pg/mL) and cortisol levels (44.8 µg/dL) were significantly elevated, reflecting an appropriate HPA axis response to the stress of surgery, and two weeks postoperatively, ACTH was within normal range and a repeat dexamethasone suppression test was normal. Six months postoperatively, ACTH was within normal limits and cortisol was approaching normal. The patient has exhibited no postoperative signs or symptoms of adrenal insufficiency in 12 months.
Preoperative mifepristone therapy was associated with apparent recovery of the HPA axis prior to unilateral adrenalectomy in a patient with unilateral adrenal adenomas. Postoperatively, the patient experienced no signs or symptoms of adrenal insufficiency and no glucocorticoid replacement was required.
单侧分泌皮质醇的肾上腺皮质腺瘤手术切除后，通常需要进行糖皮质激素替代治疗以治疗继发性肾上腺皮质功能减退。在此，我们描述了一名患有单侧分泌皮质醇腺瘤的患者，术前使用米非司酮治疗与下丘脑-垂体-肾上腺（HPA）轴的恢复相关，从而无需术后糖皮质激素替代治疗。
一名66岁的白种男性，患有2型糖尿病、高脂血症、高血压和肥胖症，因福尼尔坏疽和耐甲氧西林金黄色葡萄球菌败血症住院。腹部计算机断层扫描显示左肾上腺有三个腺瘤，大小分别为1.4厘米、2.1厘米和1.2厘米，右肾上腺萎缩。24小时尿游离皮质醇水平升高（237μg/24小时，参考范围0 - 50μg/24小时）。感染消退后的激素评估显示过夜地塞米松抑制试验异常（地塞米松后皮质醇14.5μg/dL），促肾上腺皮质激素（ACTH）受抑制（<5pg/mL，参考范围7.2 - 63.3pg/mL），硫酸脱氢表雄酮处于低正常水平（50.5μg/dL，男性参考范围30.9 - 295.6μg/dL）。由于其病情较差且糖尿病控制不佳，其库欣综合征在手术前采用药物治疗。开始米非司酮治疗，五个月内，其糖尿病得到控制，胰岛素停用。先前受抑制的ACTH升至正常范围以上，同时硫酸脱氢表雄酮水平升高，表明HPA轴恢复且对侧肾上腺萎缩。患者术中预防性使用了一剂氢化可的松，此后未再使用。术后两天，ACTH（843pg/mL）和皮质醇水平（44.8μg/dL）显著升高，反映了HPA轴对手术应激的适当反应，术后两周，ACTH在正常范围内，重复地塞米松抑制试验正常。术后六个月，ACTH在正常范围内，皮质醇接近正常。该患者在12个月内未出现肾上腺功能减退的术后体征或症状。
术前米非司酮治疗与单侧肾上腺腺瘤患者单侧肾上腺切除术前HPA轴的明显恢复相关。术后，患者未出现肾上腺功能减退的体征或症状，无需糖皮质激素替代治疗。
