米非司酮在库欣综合征治疗中的应用

Mifepristone in Cushing syndrome

目的:探讨米非司酮在库欣综合征(Cushing’s syndrome,CS)患者中的应用,提高医生对米非司酮治疗库欣综合征临床应用的认识。方法:回顾性分析西安交通大学第一附属医院2015-2018收治的5例接受米非司酮治疗的库欣综合征患者的临床特征及诊治经过。结果:5例患者中3例为异位ACTH综合征,因病灶无法定位而接受米非司酮及补钾等治疗,其中1例随访2年后发现肺部病灶并手术切除,1例随访过程中因严重肺部感染、呼吸衰竭死亡,1例仍在随访中。余下1例为肾上腺皮质腺癌Ⅳ期患者,因无法耐受手术而接受化疗及米托坦治疗,后因胃肠道反应严重调整治疗方案为靶向药物联合米非司酮治疗;另1例为库欣病接受手术及伽马刀治疗后复发。5例患者服药后均有可耐受的胃肠道反应,2例有头痛,3例水肿稍有加重。5名患者使用米非司酮后血糖、血压均下降。5名患者入院时均存在严重低钾血症,应用米非司酮后血钾可升高至正常水平,其中1例患者应用米非司酮初期低钾血症加重并出现房性及室性早搏,调整补钾方案后血钾及心电图恢复正常。结论:(1)米非司酮用于复发性库欣病、病灶未明或无法耐受手术的异位ACTH综合征、晚期肾上腺皮质癌所致库欣综合征患者,可明显改善高皮质醇血症所致临床症状。(2)米非司酮用药初期需密切监测血钾,必要时调整补钾方案。

OBJECTIVE To explore the application of mifepristone in patients with Cushing’s syndrome(CS), and to improve doctors’ understanding of the clinical application of mifepristone in the treatment of CS. METHODS The clinical features, diagnosis and treatment of 5 patients with Cushing’s syndrome treated with mifepristone in the First Affiliated Hospital of Xi’an Jiaotong University from 2015 to 2018 were analyzed retrospectively. RESULTS 3 of the 5 patients had ectopic ACTH syndrome and the lesions could not be located. Among them, One patient was found to have pulmonary lesion and underwent surgical resection after 2 years of follow-up, one patient died of severe pulmonary infection and respiratory failure during follow-up, and one patient was still in follow-up. The remaining 1 patient with stage Ⅳ adrenocortical adenocarcinoma was treated with chemotherapy and mitotane due to intolerance to surgery, and then the treatment regimen was adjusted to targeted drugs combined with mitotane due to severe gastrointestinal reactions;the other patient with Cushing’s disease relapsed after surgery and gamma knife treatment.All 5 patients had tolerable gastrointestinal reactions after medication, 2 had headache, and 3 had slightly aggravated edema. Blood glucose and blood pressure decreased in all 5 patients after the use of mifepristone. All the 5 patients had severe hypokalemia at admission, and 1 patient underwent aggravated hypokalemia accompanied with atrial and ventricular premature beats. After adjustment of the potassium supplementation, the serum potassium of all the patients returned to the normal level. CONCLUSION(1)Mifepristone can rapidly relieve severe hypercorticosteremia in patients with recurrent Cushing’s disease, ectopic ACTH syndrome and Cushing’s syndrome caused by advanced adrenocortical carcinoma when surgery is unfeasible.(2)Close monitoring of serum potassium is required at the beginning of miristone administration, and potassium supplementation regimen should be adjusted if necessary.