12例ACTH非依赖性肾上腺皮质大结节样增生的诊治

目的 探讨ACTH非依赖性肾上腺皮质大结节样增生(AIMAH)的诊断与治疗.方法 回顾性分析12例AIMAH患者的临床表现、内分泌检查、影像与病理特点以及治疗和预后.男性5例,女性7例,平均年龄48岁(29～66岁).结果 7例有典型库欣综合征表现,5例则仅有高血压或高血糖等非特异性症状.内分泌检查显示皮质醇分泌失去昼夜节律,大、小剂量地塞米松抑制试验均不被抑制.CT或MRI示双肾上腺弥漫性增大伴多发结节.11例行手术治疗,其中双侧肾上腺切除3例;一侧肾上腺全切,对侧肾上腺次全切除1例;单侧肾上腺切除7例.病理诊断肾上腺结节样或腺瘤样增生.随访14～124个月,库欣综合征症状消失.双侧肾上腺切除者无Nelson综合征出现,单侧肾上腺切除者血尿皮质醇在正常范围,对侧肾上腺无明显增大.结论 AIMAH有独特的临床病理特点,单侧肾上腺切除是一种安全、有效的治疗选择.对症状不缓解或复发者可行双侧肾上腺切除或一侧肾上腺全切、对侧肾上腺次全切除.

Abstract：

Objective To explore the diagnosis and treatment of adrenocorticotropin-independent macronodular adrenal hyperplasia (AIMAH). Methods The clinical symptoms, endocrinological and radiological characteristics, treatment modalities, and postoperative clinical course of 12 AIMAH patients were retrospectively reviewed. There were 5 men and 7 women with an average age of 48 years old. Results All cases had some clinical features and biochemical evidence of Cushing's syndrome. The circadian rhythm of serum cortisol was abnormal. Low- and high-dose dexamethasone suppression tests failed to suppress cortisol secretion. CT or MRI scan showed bilateral enlargement of the adrenal glands with multiple macronodules. Bilateral adrenalectomy was performed in three patients and unilateral adrenalectomy combined with contralateral subtotal adrenalectomy was performed in one patient. Seven patients underwent unilateral adrenalectomy of the larger gland. The removed adrenal glands weighted within 8-96 g range and pathologic diagnoses were nodular or adenomatoid hyperplasia of adrenal cortex. The duration of postoperative follow-up was 14 to 124 months. Clinical symptoms of Cushing's syndrome disappeared after surgery in all cases and there was no Nelson's syndrome after bilateral adrenalectomy. Urinary free cortisol and serum cortisol were within normal ranges. No further enlargement of the contralateral gland has been noticed in patients who received unilateral adrenalectomy during the follow-up.Conclusions AIMAH had unique endocrinological and pathological features. Unilateral adrenalectomy appears to be an effective and safe alternative treatment for AIMAH. Bilateral adrenalectomy or unilateral adrenalectomy combined with contralateral subtotal adrenalectomy may be performed if the symptoms had not been improved or recurred after unilateral adrenalectomy.     

ACTH非依赖性大结节样肾上腺增生

探讨促肾上腺皮质激素（ACTH）非依赖性大结节样肾上腺增生（AIMAH）的临床和病理特点。AIMAH是不依赖ACTH的库欣综合征，血浆ACTH水平低，大剂量地塞米松抑制试验不被抑制。CT示双侧肾上腺结节样增大，病理改变为双侧肾上腺结节样或腺瘤样增生，双侧肾上腺切除术后不发生Nelson综合征，AIMAH有特殊的临床病理特点，是库欣综合征的一种病因。     

高危ACTH非依赖性肾上腺皮质大结节样增生的围手术期处理(附一例报告)

目的 提高促肾上腺皮质激素(ACTH)非依赖性肾上腺皮质大结节样增生(AIMAH)的治疗水平.方法 报告了1例AIMAH患者的诊治经过.患者女性,51岁,发现库欣症状半年余,血皮质醇以及24 h尿游离皮质醇升高,血皮质醇昼夜分泌节律消失,2 mg和8 mg地塞米松抑制试验均不被抑制,心肺功能极差,血压最高至300/120 mm Hg(1 mm Hg=0.133 kPa).起始服用米托坦(60 mg/d),效果不佳,后改用酮康唑( 800 mg/d).5天后血压降至180/120 mm Hg,但心、肺功能改善仍不明显,出现端坐呼吸,氧饱和度一度跌至75％,只能尽快行右肾上腺全切术.结果 切除的肿块约10 cm×10 cm大小,称重67.5g.病理示肾上腺非色素性大结节样增生.术后未补充肾上腺皮质激素,术后第6天起继续使用酮康唑( 400 ～ 800mg/d)降皮质醇,血、尿皮质醇逐渐正常(血皮质醇8:00时18.65 μg/dl,24 h尿游离皮质醇78.75 μmol),一般情况好转后出院.随访10个月至今,实验室检查指标正常.结论 AIMAH患者拟采用个体化治疗,对于症状进展迅速,血压极高,心脏受累,低血钾,低蛋白血症者,先通过药物抑制肾上腺分泌糖皮质激素.一旦心肺功能有改善,应尽快切除一侧肾上腺.围手术期不宜补充肾上腺皮质激素.单侧肾上腺全切术依然是一种治疗高危AIMAH有效的方法.     

非ACTH依赖性双侧肾上腺大结节增生患者的临床特点分析

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;24 h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.     

非ACTH依赖性双侧肾上腺大结节性增生病1例报告

非促肾上腺皮质激素(ACTH)依赖性双侧肾上腺大结节性增生(AIMAH)是库欣综合征的一种单独临床亚型。本病比较少见,易与其他库欣综合征相混淆。现将我院收治的1例报道如下。1病历资料患者女,42岁,因头痛3年、夜尿增多2年就诊。3年前,患者感持续性额顶部胀痛,查血压160/100 mmHg(1 mmHg=0.133 kPa),服用多种降压     

ACTH非依赖性大结节样肾上腺增生

探讨促肾上腺皮质激素(ACTH)非依赖性大结节样肾上腺增生(AIMAH)的临床和病理特点。AIMAH是不依赖ACTH的库欣综合征,血浆ACTH水平低,大剂量地塞米松抑制试验不被抑制。CT示双侧肾上腺结节样增大,病理改变为双侧肾上腺结节样或腺瘤样增生,双侧肾上腺切除术后不发生Nelson综合征。AIMAH有特殊的临床病理特点,是库欣综合征的一种病因。     

原发性色素沉着性结节性肾上腺皮质病3例

总结3例原发性色素沉着性结节性肾上腺皮质病(PPNAD)患者的临床特征及诊疗方案。PPNAD除符合促肾上腺皮质激素(ACTH)非依赖性库欣综合征特征外, 有以下特点:(1)Liddle试验后尿游离皮质醇反常升高;(2)伴生长停滞或Carney综合征表现;(3)肾上腺为小结节改变;(4)基因检测分别为PRKACA、PRKAR1A突变;(5)病理均为肾上腺色素沉积的结节改变;(6)治疗最终需要行双侧肾上腺切除, 并予终身激素替代。PPNAD是罕见的库欣综合征类型, 临床不易识别, 认识其特征并行基因检测对明确诊断和治疗具有重要意义。     

非ACTH依赖性库欣综合征合并心功能不全患者的临床特点分析

目的:总结非促肾上腺皮质激素(ACTH)依赖性库欣综合征(CS)合并心功能不全患者的临床特点,为在非ACTH依赖性CS患者中更好地识别出心功能不全的高危人群提供理论依据。方法:收集以"非ACTH依赖性CS"为检索词,在北京大学第一医院住院电子病例系统中检索2014年1月至2019年3月出院诊断为非ACTH依赖性CS的病...     

Case of adrenocorticotropic hormone-independent macronodular adrenal hyperplasia with possible adrenal hypersensitivity to angiotensin II

With increasing case reports, it has been indicated that some cases with adrenocorticotropic hormone (ACTH)-independent macronodular adrenal hyperplasia (AIMAH) show abnormal responses in cortisol to various stimulation tests. Here we report a case of AIMAH that showed an aberrant response to angiotensin II via AT1 receptor in cortisol hypersecretion. A 53-yr-old man was admitted to our division seeking further examinations for the possible diagnosis of Cushing's syndrome. He had hypertension, diabetes mellitus, and physical stigmata, such as moon face and central obesity. His plasma ACTH level was undetectable, and plasma cortisol level was high. Plasma cortisol showed no normal diurnal rhythm and was not suppressed after the administration of 8 mg of dexamethasone. Abdominal computed tomography demonstrated nodular enlargement of bilateral adrenal glands. He was diagnosed with Cushing's syndrome owing to AIMAH. An injection of arginine vasopressin (AVP) increased plasma cortisol and aldosterone levels, whereas ACTH remained undetectable. After 4 h in an upright position, plasma cortisol and aldosterone levels were increased. Pretreatment with candesartan, angiotensin II receptor AT1 antagonist, blocked the increase in plasma cortisol level. These results suggested a possibility of adrenal hypersensitivity to angiotensin II and AVP in cortisol secretion. Bilateral laparoscopic adrenalectomy was performed. The histological findings of the specimen were compatible with AIMAH. In summary, we have made the first report on a case of AIMAH with possible hypersensitivity to angiotensin II.     

Adrenocorticotropin-independent bilateral macronodular adrenal hyperplasia: an unusual cause of Cushing's syndrome

Inappropriate ACTH secretion with bilateral diffuse or macronodular adrenal hyperplasia is the most common cause of Cushing's syndrome. This report describes a patient with Cushing's syndrome and feminization due to ACTH-independent bilateral macronodular adrenal hyperplasia. A 47-yr-old black man presented with Cushingoid features, diabetes mellitus, hypertension, impotence, and gynecomastia. Urinary cortisol and 17-hydroxycorticosteroid excretion were 94 nmol/mmol creatinine (normal, less than 32) and 5.8 mumol/mmol creatinine (normal, 0.6-3.6), respectively. Both decreased by less than 30% after administration of dexamethasone (8 and 16 mg/day), and urinary 17-hydroxycorticosteroid excretion did not increase after metyrapone (750 mg, orally, every 4 h for six doses). Plasma ACTH was undetectable (less than 1 pmol/L) and was not stimulated by administration of metyrapone or ovine CRH. Serum testosterone was 5.2 nmol/L (normal, 7-30), FSH was 5 U/L (normal, 3-18), LH was 2.8 U/L (normal, 1.5-9.2), and estrone was 767 pmol/L (normal, 55-240). Both adrenal glands were enlarged, with a total weight of 86 g (normal, 8-10), and contained multiple nodules (diameter, greater than 0.5 cm) composed of two active cell types, one of which was also observed between the nodules. Cushing's syndrome with feminization due to ACTH-independent bilateral macronodular adrenal hyperplasia is an unusual process of unknown etiology that should be included with the other known causes of Cushing's syndrome.     

肾上腺肿瘤45例临床分析

目的　探讨肾上腺肿瘤的临床特点和检查方法 ,提高术前诊断水平。方法　对 4 5例肾上腺肿瘤及增生的临床表现、激素水平、CT定位诊断及术后病理进行回顾性分析。结果　 4 5例中醛固酮瘤 15例 ,误诊的双侧肾上腺增生 3例 ,嗜铬细胞瘤 8例 ,腺瘤、腺癌型皮质醇增多症 4例和意外瘤 15例。结论　术前肿瘤性质及内分泌功能检查有利于术前准备、术中处理及术后并发症预防