中文摘要：

目的探讨单侧肾上腺切除术治疗促肾上腺皮质激素（adrenoconjcotropic hormone，ACTH）非依赖性库欣综合征肾上腺大结节增生的疗效。方法回顾性分析2005年1月至2015年12月收治的22例确诊为ACTH非依赖性肾上腺大结节增生且临床表现为库欣综合征患者的临床资料，男17例，女5例。年龄37～56岁，平均46．5岁。22例入院时均有满月脸、向心性肥胖等库欣综合征症状。18例伴高血压，5例空腹血糖升高，4例糖化血红蛋白升高。术前实验室检查：血浆皮质醇昼夜节律消失，24h尿游离皮质醇升高，ACTH受抑制或正常，小剂量地塞米松抑制试验均不受抑制。CT检查示双侧肾上腺弥漫性增大伴多发结节。结果22例均行单侧肾上腺切除术。病理示肾上腺皮质结节样增生。术后16例获得随访，随访时间17～118个月，中位值26个月。术后3～9个月复查血尿皮质醇均恢复正常，库欣综合征症状消失或者明显好转，复查CT对侧肾上腺均无显著改变，未出现肾上腺皮质功能不全的症状；16例患者血压较术前明显下降，中位收缩压为128mmHg（110～140mmHg）（1mmHg=0．133kPa），舒张压为90mmHg（80～100mmHg），之后随访均降至正常水平。16例空腹血糖中位值5．04mmol／L（4．11～8．75mmol／L），糖化血红蛋白5．8％（5．1％～7．4％）。3例患者分别于术后12个月、3年、10年复发，其中2例再次接受对侧肾上腺切除术，术后恢复良好，另1例拒绝再次手术。4例患者电话随访，诉术后血压、血糖均恢复正常。结论单侧肾上腺切除术治疗ACTH非依赖性肾上腺大结节增生引起的库欣综合征疗效显著，复发率低，可以考虑作为临床一线治疗方式。

英文摘要：

Objective To investigate the therapeutic outcomes of unilateral adrenalectomy for Cushing syndrome in patients with adrenoeorticotropic hormone（ACTH） independent bilateral macronodular adrenal hyperplasia. Methods 22 patients diagnosed with Cushing syndrome caused by ACTH independent bilateral macronodular adrenal hyperplasia from January 2005 to December 2015 were retrospectively concluded. There are 17 male patients and 5 female patients with the median age of 46. 5 years. All patients were presented with Cushing syndrome such as moon face, buffalo hump and other typical symptoms of Cushing syndrome. The laboratory tests showed disappearance of cortisol rhythm, elevated 24-hour urinary free cortisol, suppressed or normal ACTH and unsuppressed low dose dexamethasone suppressing test. CT scan showed bilateral macronodular adrenal hyperplasia in all patients. Results All the patients received unilateral adrenalectomy. Pathological results showed adrenal cortex nodular hyperplasia. 16 patients had reexamination in the endocrine department. The median follow-up time was 26 （ 17 - 118 ）months for these 16 patients. In the follow-up between 3 to 9 months after surgery, laboratory test showed serum and urinary cortisol level returned to normal range and Cushing syndrome also disappeared in these 16 patients. CT scan showed no recurrence. And no adrenal insufficiency occurred. One patient had recurrence one year after surgery and two patients turned better after surgery but had recurrence at 3 years and 10 years after surgery. Two patients received contralateral adrenalectomy and cortisol hormone replacement therapy after surgery. Another patient with recurrence refused second surgery. No recurrence was observed in other patients. Four patients were followed up by telephone, and all recovered well after surgery. 18 patients had hypertension before surgery and their blood pressure significantly decreased after surgery. Among them 13 cases blood pressure returned to normal range. 5 patients had elevated blood gluc