ACTH非依赖性肾上腺皮质大结节增生症3例报告

Corticotropin (ACTH) independent macronodular adrenal lyperplasia (Report of 3 cases)

目的 :提高ACTH非依赖性肾上腺皮质大结节增生症 (AIMAH )的诊断水平。方法 :回顾性分析3例AIMAH患者的临床资料。结果 :3例AIMAH患者除具有库兴综合征的临床和生化特点之外 ,还有如下特点 :大剂量地塞米松抑制试验 (HDDST)不被抑制 ,血浆ACTH低水平 ,CT显示双侧肾上腺大结节样增生改变 ,病理检查为双侧肾上腺大结节样或腺瘤样增生 ,单侧肾上腺切除症状可缓解 3～ 4年 ,双侧肾上腺切除可治愈 ,术后无Nelson征。结论 :AIMAH具有独特的内分泌、CT和病理学特点 ,是一种独立的库兴综合征病因。

Purpose:To improve the diagnosis of corticoptropin(ACTH ) independent macronodular adrenal hyperplasia ( AIMAH ). Methods:A retrospective review was done on 3 patients with AIMAH. Results:All the 3 patients present signs and biochemical evidence of cushing's syndrome, High dose dexamethasone suppression tests failed to suppress cortison secretion. There were no elevated ACTH levels with 3 patients. It was showed bilateral massively enlarged multi nodular adrenal gland on CT scan. On microscopic examination, the bilateral adrenal glands were nodular or adenoma like hyperplasia. The signs of cushing's syndrome were resolved for 3～4 years by unilateral adrenolectomy. AIMAH were cured by bilateral adrenalectomy and no one developed Nelson's syndrome. Conclusions:As AIMAH had unique endocrinology, CT scan and pathology finds, AIMAH should be regarded as a separate cause of cushing's syndrome.