促肾上腺皮质激素非依赖性肾上腺皮质大结节样增生的临床研究  

The clinical characteristics of 10 cases of adrenocorticotropic hormone-independent macronodular adrenal hyperplasia

作　　者：李庆玲[1] 郭若汨[2] 陈黎红[3] 尹琼丽[1] 王一娜[1] 陈燕铭[1] 

机构地区：[1]中山大学附属第三医院特诊医疗中心,广州510630 [2]中山大学附属第三医院放射科分子影像学实验室,广州510630 [3]中山大学孙逸仙纪念医院内分泌科

出　　处：《中华内科杂志》2013年第9期737-740,共4页Chinese Journal of Internal Medicine

摘　　要：目的探讨促肾上腺皮质激素（ACTH）非依赖性。肾上腺皮质大结节样增生（AIMAH）的发病机制、诊断及治疗。方法回顾性分析10例AIMAH的临床和实验室资料，复习相关文献并进行讨论。结果10例患者中7例有典型库欣综合征表现，3例仅有高血压或高血糖等非特异性症状，实验室检查显示血浆ACTH水平低下，皮质醇分泌节律紊乱，大、小剂量地塞米松抑制试验均不被抑制，CT或磁共振成像示双侧肾上腺结节样改变，病理诊断符合AIMAH。4例患者体位试验阳性。3例患者行双侧肾上腺切除术；5例行单侧肾上腺切除术；2例口服普萘洛尔治疗。随访10一89个月，单侧肾上腺切除者3例血尿皮质醇在正常范围、对侧肾上腺无明显增大，2例复发后（1年、2．5年）行对侧肾上腺切除术。口服普萘洛尔药物治疗者分别于5个月、18个月病情复发或进展而停药行双侧肾上腺切除术治疗。结论AIMAH是库欣综合征中病因独特的临床亚型，有其特有的发病机制及临床特点，AIMAH的发病可能与肾上腺异位受体的表达有关，对体位试验（＋）的患者可考虑先行普萘洛尔药物治疗，治疗无效者或复发者单侧肾上腺切除术是一个安全、有效的治疗方法。对症状不缓解或复发者可行双侧肾上腺切除术。Objective To evaluate the clinical characteristics of patients with adrenocorticotropin- independent macronodular adrenal hyperplasia （AIMAH）. Methods A total of 10 AIMAH eases were enrolled in this retrospective study. The clinical and laboratory findings of all patients were collected and analyzed. Results All patients manifested some clinical features and biochemical evidence of Cushing＇s syndrome. The plasma adrenocorticotropic hormone （ACTH） level was undetectable in all the patients and their serum eortisol secretion rhythm was abnormal. Low and high-dose dexamethasone suppression tests failed to suppress the cortisol secretion. The bilateral maeronodular adrenal enlargement was shown by CT/ magnetic resonance imaging. The supine-upright posture test was positive in four patients. Three patients were performed bilateral adrenalectomy, five were unilateral adrenalectomy and the remaining two patients were taken propranolol. All the patients had followed up for 10 to 89 months. Contralateral adrenalectomy was performed in two patients with recurrent symptoms after unilateral adrenalectomy and two patients given propranolol were underwent bilateral adrenalectomy when their symptoms had not been improved or recurred. Conclusion AIMAH is a relatively rare subtype of Cushing＇s syndrome with unique clinical and laboratory findings. Propranolol is a good choice if the supine-upright posture test is positive. Unilateral adrenalectomy appears to be an effective and safe alternative treatment for AIMAH. Bilateral adrenalectomy could be performed if the symptoms have not been improved or recurred after unilateral adrenaleetomy.

关 键 词：促肾上腺皮质激素 肾上腺皮质大结节增生 体位试验 

分 类 号：R586[医药卫生—内分泌]