非ACTH依赖性双侧肾上腺大结节增生患者的临床特点分析  

A study on clinical features of ACTH-independent bilateral macronodniar adrenal hyperplasia

作　　者：张倩[1] 窦京涛[1] 谷伟军[1] 杜锦[1] 王先令[1] 杨国庆[1] 郭清华[1] 杨丽娟[1] 吕朝晖[1] 巴建明[1] 母义明[1] 陆菊明[1] 潘长玉[1] 

机构地区：[1]解放军总医院内分泌科,北京100853

出　　处：《中华内分泌代谢杂志》2011年第11期892-896,共5页Chinese Journal of Endocrinology and Metabolism

摘　　要：目的探讨非ACTH依赖性双侧肾上腺大结节增生（AIMAH）患者的临床特点。方法回顾性分析1998年至2010年解放军总医院内分泌科收治并确诊的18例非ACTH依赖性双侧肾上腺大结节增生患者的临床资料。结果（1）AIMAH患者男女比例1：1，平均就诊年龄（51．4±10．7）岁，平均病程（9．9±2．7）年；（2）部分患者因体检意外发现。肾上腺占位就诊；高血压、糖代谢异常最常见，满月脸、向心性肥胖等典型的库欣综合征（CS）体征较少见；（3）实验室检查示患者均为非ACTH依赖性CS，部分患者具备亚临床CS的特点；24h动态血ACTH、皮质醇水平监测能全面、清晰显示部分AIMAH患者亚临床库欣综合征的特点；外源性ACTH能刺激皮质醇的显著分泌；（4）CT显示双侧肾上腺结节样增生改变，结节直径多在1．0～3．5cm；（5）病理显示肾上腺组织结节样增生；（6）双侧肾上腺切除治疗效果较好，单侧切除效果不佳。结论AIMAH为库欣综合征的独立少见类型，部分患者表现为亚临床库欣综合征，非ACTH依赖性及双侧肾上腺结节样增生为该病最重要的临床特点。Objective To study the clinical features of patients with ACTH-independent bilateral macronodular adrenal hyperplasia （AIMAH）. Methods Eighteen cases with AIMAH diagnosed in Chinese PLA General Hospital from 1998 to 2010 were analyzed retrospectively. Results （ 1 ） AIMAH was equally distributed between genders, mean age at diagnosis was （51.4±10. 7） years, and average course was （9. 9±2. 7） years. （2） Most patients visited hospital because of adrenal incidentaloma; hypertension and impaired glucose tolerance/diabetes mellitius were the most common clinical presentations; typical signs of Cushing＇s syndrome（CS） such as moon face and central obesity were less frequent. （ 3 ） All cases＇ laboratory findings met the diagnostic criteria of ACTH- independent CS, some cases with the features of subclinical CS. 24 h dynamic plasma ACTH and eortisol monitoring had the advantage of revealing the features of subclinical CS for some AIMAH cases ; there was remarkable elevation of plasma cortisol after exogenous ACTH stimulation in AIMAH patients. （4） Bilateral adrenal macronodular changes could be visualized on CT scan. （ 5 ） Pathology identified bilateral macronodular adrenal hyperplasia. （ 6 ） Bilateral adrenalectomy was the most useful treatment. Conclusions A1MAH is an infrequent cause of CS, and some patients present subclinical CS. Its marked clinical feature is ACTH-independent bilateral macronodnlar adrenal hyperplasia.

关 键 词：促肾上腺皮质激素 肾上腺 增生 库欣综合征 

分 类 号：R736.6[医药卫生—肿瘤]