ACTH非依赖性库兴综合征(附89例报告)

目的　总结促肾上腺皮质激素 (ACTH)非依赖性库兴综合征的诊断和治疗方法。　方法　对 89例ACTH非依赖性库兴综合征患者的临床资料进行总结。男 11例 ,女 78例。年龄 13～5 6岁 ,平均 34岁。其中腺瘤型皮质醇症 85例、肾上腺皮质癌 2例、大结节样肾上腺皮质增生 2例。回顾性总结其临床、生化特征 ,影像学特点和治疗方法。　结果　 85例腺瘤型皮质醇症术后效果良好 ,库兴综合征症状消失 ;2例肾上腺皮质癌未能切除者术后分别存活 7和 9个月 ;大结节样肾上腺皮质增生 2例行双侧肾上腺切除后治愈。　结论　血浆ACTH水平、地塞米松抑制试验及B超、CT等影像学检查有利于ACTH非依赖性库兴综合征的诊断和鉴别诊断 ,手术治疗腺瘤型皮质醇症和大结节肾上腺增生可获得较理想的疗效 ,肾上腺皮质癌预后差。

CTH-independent Cushing syndrome(report of 89 cases)

Objective To study the diagnosis and treatment of adrenocorticotrophic hormone (ACTH) independent Cushing syndrome. Methods A retrospective review was done on 89 patients with ACTH independent Cushing syndrome, including 85 cases of adrenal tumors with hypercorticism, 2 cases of adrenocortical adenocarcinoma and 2 cases with ACTH independent macronodular adrenal hyperplasia. Results Favorable results were observed from the surgery for 85 cases of adrenal tumors combined with hypercorticism.Two cases with ACTH independent macronodular adrenal hyperplasia were cured by bilateral adrenolectomy. Adrenal cortical adenocarcinoma was unresectable for both the patients who survived 7 and 9 months respectively. Plasma ACTH,dexamethasone suppression test,B ultrasonography and CT scan were useful for the diagnosis and differentiated diagnosis of ACTH independent Cushing syndrome. Conclusions Prognosis of adrenal tumors with hypercorticism and ACTH independent macronodular adrenal hyperplasia is favorable after surgery,whereas the prognosis of adrenal cortical adenocarcinoma is poor.