摘要通过随访2例本院诊断为隐匿性异位促肾上腺皮质激素综合征且服用米非司酮治疗的患者,分析其临床特点、诊疗经过及临床疗效,回顾相关文献,评价米非司酮治疗异位促肾上腺皮质激素综合征患者临床疗效及不良反应.米非司酮治疗可改善患者的临床症状,2例患者均减量至停药,其中病例1合并糖尿病,治疗后可停用降糖药物,其血浆促肾上腺皮质激素(ACTH)、血清皮质醇较前下降;病例2合并高血压,其降压药物种类及剂量减少,血浆ACTH、血清皮质醇较前变化不大,随访过程中发现责任病灶,行手术切除后病情缓解、术后出现皮质醇功能减退症.米非司酮治疗异位促肾上腺皮质激素综合征患者安全有效,但仍需严密随访,若发现责任病灶需进一步明确病灶性质,尽快行手术治疗.

abstractsThis is a follow-up study in two patients who were diagnosed with the occult ectopic adrenocorticotropin syndrome ( EAS) and treated with mifepristone in our hospital. The efficacy and adverse reactions of mifepristone in these two patients with EAS were investigated. The patients had significant improvement in clinical manifestations, the plasma adrenocorticotropic hormone ( ACTH) and cortisol levels decreased in patient 1, while patient 2 remained stable during the treatment. Patient 1 with diabetes mellitus stopped hypoglycemic agents, while the dose of antihypertensive drugs decreased in patient 2 with hypertension, and she was treated with surgery and suffered from transient adrenocortical insufficiency after surgical excision of the lesion. Mifepristone is effective and safe in treating EAS. Surgical treatment should be performed once the lesion is found during the follow-up.