基于地塞米松抑制试验的原发性色素性结节样肾上腺皮质病鉴别诊断切点探讨

目的 通过比较原发性色素性结节样肾上腺皮质病（PPNAD）、原发性双侧肾上腺大结节增生（PBMAH）和肾上腺皮质腺瘤（ADA）三种疾病的地塞米松抑制试验后尿游离皮质醇变化比值,探讨PPNAD患者的鉴别诊断切点。方法 收集2008年1月-2020年12月于解放军总医院第一医学中心住院病理明确诊断为PPNAD（7例）、PBMAH（31例）及ADA（130例）患者的临床资料进行回顾性分析。通过受试者工作特征（ROC）曲线,以患者小剂量地塞米松抑制试验（LDDST）和大剂量地塞米松抑制试验（HDDST）抑制后24 h尿游离皮质醇（24 h UFC）与抑制前（基线）24 h UFC比值（post UFC/pre UFC）为检测变量,以是否为PPNAD为状态变量,寻找鉴别诊断PPNAD的最佳切点。结果 PPNAD、PBMAH及ADA三组患者基线及LDDST、HDDST后血皮质醇（F）水平均明显升高,组间差异无统计学意义（_P>0.05）;三组患者促肾上腺皮质激素（ACTH）水平均受抑制,且PBMAH组基线及HDDST后ACTH水平最高,ADA组ACTH水平最低（_...     

ACTH非依赖性大结节样肾上腺增生症7例报告并文献复习

目的 探讨ACTH非依赖性大结节样肾上腺增生(AIMAH)的临床及病理特点,提高对AIMAH诊断和治疗的认识.方法 分析1997～2005年收治的7例AIMAH的临床和实验室资料.结果 7例患者中5例有典型库欣综合征(CS)的临床表现,包括满月脸,向心性肥胖,高血压等;2例以高血压等非特异性症状就诊.部分患者有低血钾(43%)和继发性糖尿病(71%).实验室检查血浆ACTH水平低下,皮质醇水平增高或节律紊乱,6例小剂量、大剂量地塞米松抑制试验均不被抑制.CT检查示双侧肾上腺明显增大,呈结节样改变.2例行单侧肾上腺全切术,4例行双侧切除术,1例在等待手术期间因脑出血死亡.术后5例行糖皮质激素替代治疗.病理示肿瘤多呈黄色或灰黄色弥漫性结节样改变,直径0.3～7.0cm.6例术后随诊至今未复发,未发生Nelson综合征.结论 AIMAH是库欣综合征独特的亚型,有其特有的内分泌、影像及病理改变.治疗以双侧肾上腺切除、分期双侧肾上腺切除或单侧切除为主.术后辅以激素替代治疗.     

ACTH非依赖性肾上腺皮质大结节样增生的CT及MR表现

目的探讨ACTH非依赖性肾上腺皮质大结节样增生(AIMAH)的CT及MR特征性表现。方法回顾性分析4例AIMAH患者的临床及CT、MR资料,结合相关文献进行讨论。结果 4例患者CT表现均为双侧肾上腺不对称增大,伴双侧肾上腺内多发、大小不等低密度结节,增强扫描结节呈中度均匀强化;其中2例结节较多、较大,致增大肾上腺呈典型"生姜样"改变。2例MR检查示结节呈稍长T_1、稍长T_2信号,化学位移成像上信号减低。结论双侧肾上腺的多发大结节,部分病例结节融合呈"生姜样"改变;结节于CT成像呈低密度,MR成像呈稍长T1、稍长T2信号,并于反相位上信号减低,为AIMAH的典型影像学表现。     

136例库欣综合征病人垂体-肾上腺皮质激素分泌特征分析

为评估库欣综合征病人血浆ACTH(pACTH)、血清皮质醇 (SC)、尿游离皮质醇 (UFC)、小剂量和大剂量地塞米松抑制试验的诊断价值 ,回顾性分析了 1 36例已确诊为库欣病、肾上腺皮质肿瘤或不依赖ACTH性肾上腺皮质结节性增生症 (AINAH)等患者的pACTH、SC、UFC水平及地塞米松抑制试验的特点。结果显示 ,绝大多数库欣综合征患者SC和UFC(尤其SC 0 :0 0和UFC)水平升高 ;pACTH水平可明确区分库欣病和肾上腺皮质肿瘤或AINAH ;pACTH水平对库欣病诊断价值高于CT/MRI检查 ;激素昼夜分泌节律紊乱及不被小剂量地塞米松抑制是所有库欣综合征的共同特征 ,而肾上腺皮质肿瘤或AINAH则不受大剂量地塞米松抑制。结果表明 ,SC和UFC水平升高、激素分泌节律丧失及不受小剂量地塞米松抑制是诊断库欣综合征的最重要依据 ,pACTH水平是鉴别库欣病和肾上腺皮质肿瘤或AINAH的关键性指标     

双侧增生和单侧醛固酮腺瘤致醛固酮增多症的区别:CT的可靠性

作者对经肾上腺CT扫描和ACTH刺激后双侧肾上腺静脉取血诊断原发性醛固酮增多症的24例病人进行了研究。男16例,女8例。年龄15～66岁。除6例CT扫描层厚3mm外,其余病例均为5mm。单侧存在低衰减结节而对侧肾上腺正常者诊为腺瘤。双侧结节、单侧多发结节或腺体正常者为增生。所有病例,不管CT所见,均经股静脉进行双侧肾上腺静脉同时插管取血,并从肘前静脉获得周围血标本,测定醛固酮(A)和皮质醇(C)基础值后,再静脉团注0.25mg的ACTH,接着4～6ml/min(250ml盐水中加ACTH0.25mg)滴注,滴注开始后15分钟获得第二次双肾上腺静脉和周围静     

17α-羟化酶缺陷症合并男性女性化的影像学特征

【摘要】目的：探讨17α-羟化酶缺陷症合并男性女性化患者的影像学特征。方法：回顾性分析9例17α-羟化酶缺陷症合并男性女性化患者的临床及影像学资料,9例患者社会性别均为女性,年龄9~26岁。所有患者均进行肾上腺激素水平测定、染色体核型分析、双侧肾上腺和盆腔下腹部CT和/或MRI检查,14岁以上患者行X线骨龄测定。结果：9例患者染色体核型均为46XY,临床均表现为原发醛固酮增多症、ACTH水平增高、血尿皮质醇增高,成年患者性激素水平异常。影像学特征包括双侧肾上腺显著弥漫性增粗伴结节、生殖系统发育异常和骨骺闭合延迟。结论：双侧肾上腺显著弥漫性增粗伴结节、生殖系统发育异常和骨骺闭合延迟是17α-羟化酶缺陷合并男性女性化患者的特征性表现。     

肺类癌致异位促肾上腺皮质激素综合征的影像学特点分析

目的：探讨影像手段在隐匿性小结节型样肺类癌致异位促肾上腺皮质激素综合征（EAS）中的诊断价值。 方法：回顾性分析我院收治并确诊的两例小结节样肺类癌致EAS患者的影像资料,包括：MRI、PET-CT、胸部CT、双侧岩下窦静脉采血、肺结节穿刺活检术;结合患者临床表现和实验室检查,通过文献分析总结此类病例的影像特点。 结果：两例患者均有明显的库欣综合征表现,外周血促肾上腺皮质激素（ACTH）明显升高,皮质醇分泌无昼夜节律。MRI资料提示不能排除垂体微腺瘤,双侧岩下窦静脉采血排除了垂体来源ACTH瘤,因胸部CT均发现类圆形小结节,一例右上肺结节穿刺活检证实肺类癌来源ACTH瘤;另一例左下肺结节选择直接胸腔镜手术切除。手术切除后行病理学检查示一例为低分化类癌,另一例为不典型类癌。 结论：支气管肺部类癌是导致EAS的常见病因之一,如青壮年库欣综合征患者同时发现肺部小结节,MRI或双侧岩下窦静脉取血排除了垂体来源的ACTH瘤,应警惕排除肺部隐匿性异位分泌ACTH的肿瘤,必要时穿刺活检及影像学检查随访,早期发现并手术切除肺部肿瘤。     

原发性肥大性骨关节病1例诊治分析

1病例报告患者男,29岁。主因四肢关节肿痛3年就诊。自述3年前无明显诱因出现四肢关节肿痛,以双腕、肘、膝、踝为主,活动后明显加重,手指及足趾端增粗明显,并呈进行性加重,偶感晨僵。近来感觉面部较前肥大明显,皮肤亦增厚。入院后各项常规实验室检查均未见明显异常;另查血ACTH节律、血清皮质醇节律、小剂量地塞米松抑制试验等亦无异常,除外皮质醇增多症;     

血浆ACTH测定在柯兴综合征鉴别诊断中的价值

报告经手术、病理证实的24例柯兴综合征患者血浆ACTH水平的改变,并与大剂量地塞米松抑制试验作了比较,结果表明,柯兴病患者17例中14例血浆ACTH(上午8点)水平增高,3例正常高限,0点AGTH全部升高,且无昼夜节律变化;肾上腺皮质腺瘤6例血浆AGTH均明显降低;异位ACTH综合征1例血浆ACTH异常升高。3组之间无一例重叠,与手术、病理的符合率为100％。大剂量地塞米松抑制试验的符合率为67～70.6％。提示血浆AGTH测定是鉴别柯兴综合征病因十分可靠的方法。     

非ACTH依赖性肾上腺皮质增生症

库欣综合征的病因可分为ACTH依赖性和ACTH非依赖性两大类 ,后者中非ACTH依赖性肾上腺皮质增生症是库欣综合征一种独立的罕见类型 ,根据发病机制及病理变化特点可分为 :①原发性色素性结节性肾上腺皮质病/异常增生 (primarypigmentednodularadrenocorticaldisease/displasia,PPNAD) ;②ACTH非依赖性肾上腺大结节性增生 (ACTH independentbilateralmacronodulara drenalhyperplasia,AIMAH)。尽管两者的发病率很低 ,但对库欣综合征的鉴别诊断具有重要意义。1　发病及发病机制PPNAD是 2 0世纪 80年代中期发现的 ,到目前为止国外报…     

Adrenocorticotropin-independent macronodular adrenal hyperplasia: an uncommon cause of primary adrenal hypercortisolism

PURPOSE: To describe the imaging findings in the adrenal glands of 12 patients with adrenocorticotropin (ACTH)-independent macronodular adrenocortical hyperplasia (AIMAH). MATERIALS AND METHODS: Computed tomographic (CT) and magnetic resonance (MR) imaging findings in the adrenal glands were reviewed retrospectively in 12 patients (three men, nine women) with ACTH-independent Cushing syndrome and with bilateral nonpigmented multinodular adrenal hyperplasia. The results of pituitary MR imaging, adrenal scintigraphy, and petrosal sampling were available in nine, five, and six patients, respectively. Eleven patients underwent bilateral and one patient underwent unilateral adrenalectomy. RESULTS: Eleven patients had enlarged multinodular adrenal glands: Nodules were 0.1-5.5 cm. The combined weight of both adrenal specimens for the 11 bilateral adrenalectomy specimens was 28-297 g, with a mean weight of 122 g. Glands were hypointense compared with the liver on T1-weighted images and were hyperintense on T2-weighted images. Pituitary MR imaging findings were negative in nine of nine patients. Iodomethylnorcholesterol scintigraphy showed bilateral uptake in four of five patients. Petrosal sinus sampling revealed no petrosal-to-peripheral ACTH gradients before corticotropin-releasing hormone (CRH) stimulation in six of six patients, but three patients had gradients after CRH stimulation. After undergoing bilateral or unilateral adrenalectomy, all patients were cured. CONCLUSION: AIMAH is a rare cause of ACTH-independent Cushing syndrome, with characteristic CT findings of massively enlarged multinodular adrenal glands. Bilateral adrenalectomy is indicated on the basis of clinical and CT findings.     

Efficacy of iodine-131 6beta-methyl-iodo-19-norcholesterol scintigraphy and computed tomography in patients with primary aldosteronism.

In order to define the role of scintigraphy in determining the aetiology of primary aldosteronism, 41 patients were examined by computed tomography (CT) scan and adrenal scintigraphy using iodine-131 6beta-methyl-iodo-19-norcholesterol with the dexamethasone suppression test. Hormonal and scintigraphic examinations were conducted while avoiding interference by medical treatment. The aetiological diagnosis was established by taking account of the clinical context, the endocrine profile, and CT scan and scintigraphic data, as well as possible hormone assays after catheterization of the adrenal veins (12 cases) and postoperative pathology data (14 cases). The aetiological diagnoses established were Conn's adenoma (insensitive to angiotensin II) in 12 cases, idiopathic hyperplasia in 11 and macronodular hyperplasia (with functional autonomy of the nodules) in 18. Unilateral and bilateral lesions were correctly distinguished by scintigraphy in 92% of cases as compared with only 58% using CT scan alone; this was because the CT scan appearance was normal in 3/12 cases of adenoma and because a single nodule was visible in 2/11 cases of idiopathic hyperplasia and in 12/18 cases of macronodular hyperplasia. It is concluded that scintigraphy using noriodocholesterol with the dexamethasone suppression test should be performed systematically in conjunction with hormonal tests and adrenal CT scan in all cases of primary aldosteronism, as part of a strategy aimed not only at detecting adenoma but also at determining whether the hyperfunctional lesions are bilateral.     

Efficacy of iodine-131 6β-methyl-iodo-19-norcholesterol scintigraphy and computed tomography in patients with primary aldosteronism

In order to define the role of scintigraphy in determining the aetiology of primary aldosteronism, 41 patients were examined by computed tomography (CT) scan and adrenal scintigraphy using iodine-131 6β-methyl-iodo-19-norcholesterol with the dexamethasone suppression test. Hormonal and scintigraphic examinations were conducted while avoiding interference by medical treatment. The aetiological diagnosis was established by taking account of the clinical context, the endocrine profile, and CT scan and scintigraphic data, as well as possible hormone assays after catheterization of the adrenal veins (12 cases) and postoperative pathology data (14 cases). The aetiological diagnoses established were Conn’s adenoma (insensitive to angiotensin II) in 12 cases, idiopathic hyperplasia in 11 and macronodular hyperplasia (with functional autonomy of the nodules) in 18. Unilateral and bilateral lesions were correctly distinguished by scintigraphy in 92% of cases as compared with only 58% using CT scan alone; this was because the CT scan appearance was normal in 3/12 cases of adenoma and because a single nodule was visible in 2/11 cases of idiopathic hyperplasia and in 12/18 cases of macronodular hyperplasia. It is concluded that scintigraphy using noriodocholesterol with the dexamethasone suppression test should be performed systematically in conjunction with hormonal tests and adrenal CT scan in all cases of primary aldosteronism, as part of a strategy aimed not only at detecting adenoma but also at determining whether the hyperfunctional lesions are bilateral. Received 1 October 1998 and in revised form 3 June 1999     

Does the corticoadrenal adenoma with "pre-Cushing's syndrome" exist?

An adrenal tumor was discovered fortuitously in a patient with no clinical features of Cushing's syndrome. On adrenal imaging, there was good uptake in the nodule but no visualization of the contralateral adrenal. The latter was seen, however, in a second scan performed under ACTH treatment. In the hormone assessment, basal cortisol and 17-hydroxycorticoids were normal and cortisol diurnal variation was near normal, but a dexamethasone suppression test and ACTH responses to metyrapone and insulin hypoglycemia were abnormal. Eight months after excision of a spongiocytic-type adenoma, the remaining adrenal was visible on scintigram and the hormonal tests were normal. This pattern suggests that the clinical Cushing's syndrome but enough to suppress partially ACTH and, consequently, visualization of the contralateral gland.     

Aortic dissection without intimal rupture: diagnosis with MR imaging and CT

Computed tomographic (CT) scans of the adrenal glands were obtained in nine patients with Cushing disease as a result of an adrenocorticotropic hormone (ACTH)-secreting pituitary adenoma and macronodular hyperplasia of the adrenal glands. The findings were compared retrospectively with those in six patients with Cushing syndrome as a result of an autonomous adrenal adenoma and 16 with Cushing syndrome as a result of ectopic ACTH production. Seven of nine patients with macronodular adrenal hyperplasia had hyperplastic adrenal glands in addition to single or multiple focal adrenal nodules. The six patients with hypercortisolism caused by an autonomous adrenal adenoma showed atrophic (four patients) or normal (two patients) ipsilateral and contralateral adrenal glands. Fourteen of 16 patients with the ectopic ACTH syndrome had hyperplasia of the adrenal glands without nodularity, and only two had focal nodules.     

CT and MR imaging of massive macronodular adrenocortical disease: a rare cause of autonomous primary adrenal hypercortisolism.

We studied four patients with adrenocorticotropic hormone (ACTH)-independent hypercortisolism due to bilateral massive enlargement of the adrenal glands. The combined weight of the adrenal glands ranged from 69 to 149 g and the adrenal cortex was replaced in three of four patients by multiple nodules ranging from microscopic to 4 cm in diameter. One patient had massive diffuse enlargement. All patients had low or undetectable levels of serum ACTH, absence of petrosal sinus to peripheral gradients of ACTH in bilateral samples from the inferior petrosal sinuses before and after stimulation by corticotropin releasing hormone, and absence of an adenoma on MR imaging of the pituitary gland. The marked degree of adrenocortical enlargement and absence of ACTH dependency separates this massive macronodular disease from the more common ACTH-dependent macronodular hyperplasia encountered in older patients with pituitary-dependent Cushing disease. All patients required bilateral adrenalectomy to control hypercortisolism. We present the spectrum of nodular adrenal disease associated with hypercortisolism and a differential diagnosis based on morphologic criteria.     

女性假两性畸形影像表现

目的评价女性假两性畸形的影像学特点。方法回顾性分析9例女性假两性畸形的影像资料。结果7例未经激素治疗者，双侧肾上腺内外支一致性增粗、延长、扭曲，其中2例呈结节状、肿块状；2例长期补充激素者中，1例肾上腺延长但不增粗，另1例与未治疗者相仿，且有髓质瘤形成；4例有子宫、卵巢发育不良。结论女性假两性畸形为遗传性疾病，其肾上腺增生、内生殖器官发育不良为继发性，影像学检查可早期发现，如及早治疗，上述改变可望恢复正常。     

Preoperative diagnosis and localization of aldosteronomas by measurement of corticosteroids in adrenal venous blood.

Adrenal venous aldosterone/cortisol ratios were determined in 14 patients with primary aldosteronism. Of 12 patients diagnosed as having an adrenal adenoma, ten underwent surgery in which an adenoma was found. Bilateral adrenal hyperplasia was thought to have developed in two other patients. Measurement of adrenal vein cortisol and establishment of aldosterone/cortisol ratios were done to correct for catheter placement and dilute adrenal efflux. Determination of steroids after ACTH stimulation was helpful in distinguishing quiescent from suppressed adrenal in two patients and in confirming the diagnosis of the others.