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目的:探讨单侧肾上腺切除术治疗原发性色素结节性肾上腺皮质病(PPNAD)的有效性。方法:回顾性分析2013年1月至2023年6月北京协和manbet官网登录 收治的26例PPNAD患者的临床资料。男11例，女15例。年龄(19.4±4.7)岁。25例表现为典型库欣综合征，16例诊断为Carney复合征。10例行基因检测，其中8例检出PRKAR1A基因突变。CT检查示双侧肾上腺多发小结节14例，单侧肾上腺小结节或轻度增粗而对侧腺体正常8例，双侧肾上腺无明显异常4例。所有患者24 h尿游离皮质醇(24 h-UFC)水平升高，中位值为408.35(334.28，800.78)μg/24 h，正常血皮质醇节律消失，血浆促肾上腺皮质激素水平均<5 pg/ml。22例行大、小剂量地塞米松抑制试验，结果均为不被抑制。26例均行腹腔镜单侧肾上腺切除术，左侧肾上腺全切8例，右侧肾上腺全切18例。结果:本组26例手术均顺利完成。手术时间(85.2±28.7)min，术中出血量均<50 ml。术后拔除引流管时间中位值为3(2，3)d。1例术后发生肺部感染，3例术后需激素替代治疗。中位随访64(31.5，103.8)个月，术后所有患者库欣综合征临床表现均缓解，其中19例(73.1%)术后8.5(5，46)d 24 h-UFC水平降至正常，中位值为42.0(22.4，58.3)μg/24 h；7例(26.9%)24 h-UFC水平虽未降至正常，但下降幅度为(73.2±10.4)%。术后13例(50%)未复发，中位随访时间51(7，89.5)个月，其中2例随访10年未复发；13例术后皮质醇水平再次升高，中位值为225.6(188.9，397.2)μg/24 h，术后至24 h-UFC升高的中位时间为27(13.5，50.5)个月，最长达104个月，其中9例伴临床症状、体征复发。13例复发患者中9例行对侧肾上腺全切或次全切除术，4例随访观察。结论:单侧肾上腺切除术可作为PPNAD手术治疗的可选择方案之一，尽管部分患者术后仍会复发，但单侧肾上腺切除术确能有效、迅速、长期地降低PPNAD患者皮质醇水平并缓解库欣综合征临床表现。

Objective:Investigating the efficacy of unilateral adrenalectomy in treatment for primary pigmented nodular adrenocortical disease (PPNAD).Methods:Clinical data of 26 patients with PPNAD treated in our hospital from January 2013 to June 2023 was retrospectively analyzed.There were 11 males and 15 females, with an average age of (19.4±4.7) years. 25 cases presented with typical Cushing's syndrome, and 16 cases were diagnosed with Carney's syndrome. PRKAR1A gene mutation detected in 8 out of 10 cases. CT showed multiple small nodules on bilateral adrenal glands in 14 cases, unilateral small nodules or mild thickening with normal contralateral glands in 8 cases, and no obvious abnormalities in 4 cases. All patients showed autonomous oversecretion of cortisol by endocrine laboratory tests, with a median 24 h-UFC of 408.35 (334.28, 800.78) μg/24 h and decreased level of adrenocorticotropic hormone. All 26 patients underwent laparoscopic unilateral adrenalectomy, with left side adrenalectomy in 8 cases and right side adrenalectomy in 18 cases.Results:The average surgical duration was (85.2±28.7) minutes, with intraoperative blood loss <50 ml in all cases. The median time to drainage tube removal post-operation was 3 (2, 3) days. One patient developed a postoperative pulmonary infection, and 3 patients required postoperative hormone replacement therapy. The median follow-up duration was 64 (31.5, 103.8) months, and all patients showed alleviation of Cushing syndrome clinical manifestations after operations. 19 patients (73.1%) had their 24 h-UFC levels normalized to a median of 42.0 (22.4, 58.3) μg/24 h within 8.5 (5, 46) days post-surgery. 7 patients (26.9%) did not achieve normal 24 h-UFC levels, yet experienced an average reduction of (73.2±10.4)%. 13 patients (50.0%) did not experience recurrence, with a median follow-up of 51 (7, 89.5) months, including two cases without recurrence at 10 years post-surgery. 13 patients showed recurrent increase in postoperative cortisol levels, with a median of 225.6 (188.9, 397.2) μg/24 h. The median time to increased 24 h-UFC post-surgery was 27 (13.5, 50.5) months, with the longest duration reaching 104 months. Among these, 9 cases exhibited clinical signs and symptoms of recurrence, while 4 cases did not. Of the 13 patients with recurrence, 9 underwent contralateral adrenalectomy or subtotal resection, while 4 were observed with follow-up.Conclusions:Unilateral adrenalectomy could be a surgical treatment option for PPNAD. Despite the recurrence in some patients postoperatively, unilateral adrenalectomy could effectively and rapidly reduce cortisol levels in PPNAD patients and alleviate the clinical manifestations of Cushing syndrome.