ACTH非依赖性大结节样肾上腺增生(附三例报告)

目的 探讨ＡＣＴＨ非依赖性大结节样肾上腺增生（ＡＩＭＡＨ）的临床病理特点。方法 回顾性分析３例ＡＩＭＡＨ临床资料，结合文献进行讨论。结果 ３例ＡＩＭＡＨ均有主加兴综合征的临床表现及生化异常，特点是小剂量、大剂量地塞米松抑制，血浆ＡＣＴＨ水平低，ＣＴ示双侧肾上腺结节样增在。３例均行双侧肾上腺切除，病理诊断双侧肾上腺结节样或腺瘤样增生，随诊４２～１０８个月库兴综合征未复发，未发生Ｎｅｌｓｏｎ综合征。结

Corticotropin(ACTH) independent macronodular adrenal hyperplasia(report of 3 cases)

Objectives To study the clinical presentation, laboratory findings, and pathologic characteristics of patients with corticotropin (ACTH) independent macronodular adrenal hyperplasia (AIMAH). Methods Clinical data of 3 patients diagnosed as AIMAH were retrospectively reviewed. Results All the 3 had clinical presentation and biochemical evidence of Cushing's syndrome. Low and high dose dexamethasone suppression tests failed to suppress cortisol secretion. No patient had elevated ACTH levels. CT showed bilateral nodular adrenal enlargement. All the 3 underwent bilateral adrenalectomy and no one developed Nelson's syndrome with follow up ranging from 42 to 108 months. Pathologic diagnosis of these patients was bilateral adrenal nodular or adenoma like hyperplasia. Conclusions As AIMAH patients had unique clinical manifestations and pathologic findings, AIMAH should be regarded as a separate cause of Cushing's syndrome.