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     

甲状腺功能状态对ACTH和皮质醇分泌的影响

甲状腺功能亢进患者的促肾上腺皮质激素-皮质醇(ACTH-F)基础分泌节律正常,由于降解加速而致F水平降低,AGTH水平反馈性升高,ACTH对氟美松抑制和胰岛素低血糖兴奋的反应正常。甲状腺功能减退患者的基础分泌节律正常,F增高,ACTH正常。ACTH对抑制和兴奋试验的反应性减低,表明甲状腺功能减退时垂体AGTH细胞功能受损。     

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.     

家族性ACTH非依赖性肾上腺大结节样增生的临床分析

目的分析家族性ACTH非依赖性肾上腺大结节样增生症(AIMAH)的临床特点。方法回顾性分析一家系3例家族性AIMAH患者的临床和实验室资料,复习相关文献并进行讨论。结果3例患者为同一家系,例1为先证者。3例平均发病年龄为59.3岁,平均病程为6.7年。例1、2皮质醇节律紊乱,ACTH均受抑制,所有ACTH值均<2.2pmol/L。例1小剂量地塞米松抑制试验(LDDST)及大剂量地塞米松抑制试验(HDDST)均不被抑制。例2LDDST不被抑制。例3基础ACTH、皮质醇值及节律均正常,LDDST及HDDST均被抑制。3例患者肾上腺B超均发现双侧多发低回声结节,CT均发现双侧肾上腺大结节样增生。3例垂体MRI检查均未发现异常。结论AIMAH的病因及发病机制尚不清楚,家族性AIMAH患者的存在表明其病因可能与遗传因素有关。家族性AIMAH患者的临床特征与散发性患者相比无明显差别,考虑可能存在更多亚临床型患者。     

肾上腺皮质功能减退时垂体-肾上腺皮质激素的改变

为评估血浆ACTH、血清皮质醇、尿游离皮质醇(UFC)水平及垂体和肾上腺皮质储备功能试验对原发性和继发性肾上腺皮质功能减退症的诊断和鉴别诊断价值，回顾性分析了16例已确诊为Addison病和75例垂体或下丘脑功能减退患者的血浆ACTH、血清皮质醇和UFC水平的改变，以及ACTH刺激试验、胰岛素低血糖刺激试验和氨基导眠能试验的特点。结果显示，原发性肾上腺皮质功能减退病人血浆ACTH水平普遍显著升高，血清皮质醇和UFC绝大多数患者降低，但少数病人正常，且对ACTH刺激试验无反应。继发性肾上腺皮质功能减退患者多数人血浆ACTH正常，而血清皮质醇和(或)UFC降低，他们对胰岛素低血糖试验或氨基导眠能试验无反应，对ACTH刺激试验呈延迟或正常反应。结果表明，皮质醇和UFC水平降低是绝大多数肾上腺皮质功能减退症病人的特征。ACTH水平是鉴别原发性或继发性肾上腺皮质功能减退的关键，原发性者ACTH水平升高，继发性者ACTH水平不高。ACTH刺激试验是诊断原发性肾上腺皮质功能减退症的有用工具，胰岛素低血糖试验和氨基导眠能试验是诊断继发性肾上腺皮质功能减退症的可靠依据。     

辛伐他汀降低血浆Hs-CRP水平及改善ACS患者临床预后分析

目的探讨使用不同剂量辛伐他汀对急性冠脉综合征患者（ACS）血浆Hs-CRP水平的影响及其对预后的评估。方法随机将80例确诊急性冠脉综合征患者分为大剂量组和小剂量组治疗12个月,辛伐他汀治疗前、辛伐他汀使用第3、6和12个月时,检测两组患者血浆Hs-CRP水平,并同时检测肝酶和肌酸激酶。随访12个月时,比较两组患者心血管事件发生率。结果辛伐他汀治疗6个月后,大剂量组患者血浆Hs-CRP水平明显低于小剂量组,差别具有统计学意义（P〈0.05）;随访12个月时,大剂量组患者血浆Hs-CRP水平已降到正常值范围,而小剂量组仍高于正常值;小剂量组患者心血管事件总发生率高于大剂量组（P〈0.05）。大剂量组肝酶和肌酸激酶升高患者各2例,但均无严重肝功能异常及横纹肌溶解,小剂量组未出现肝酶和肌酸激酶升高。结论大剂量辛伐他汀能有效降低急性冠脉综合征患者血浆Hs-CRP水平及降低心血管事件发生率,但应严密监测患者肝酶和肌酶激酶水平。     

21-羟化酶缺乏症16例临床分析

为研究21－羟化酶缺乏症的临床特征，提高对本病的诊治水平，作者对16例患者进行了回顾性分析。结果显示，16例患者中男性1例，表现为阴茎增大；女性15例，外生殖器不同程度男性化，伴色素沉着（66．7％）、多毛（60％）、原发闭经（40％）；均无失盐表现。化验检查显示，血ACTH、24h尿17－KS、17－KGS显著升高，中剂量地塞米松抑制试验均阳性。15例患者给予糖皮质激素替代治疗，1例诊断明确后出院；12例女性患者行外阴矫形术。提示21－羟化酶缺乏症在诊断、治疗上仍被人们认识不足，应予以重视。     

血浆ACTH、血清皮质醇测定对肾上腺皮质功能减退的诊断价值

本文报告原发肾上腺皮质功能减退(PAF)患者13例、继发肾上腺皮质功能减退(SAF)患者31例及正常人42名血浆ACTH及血清皮质醇测定的结果。血浆ACTH:PAF组显著高于正常组;SAF组显著低于正常组;PAF组与SAF组或正常组无重叠,但SAF组与正常组有较多重叠(83.9％)。血清皮质醇:PAF组、SAF组均显著低于正常组;PAF和SAF两组相似,但与正常组有少量重叠(13.6％),其中14例SAF、3例PAF和13名正常人做了氨基导眠能试验。SAF和正常人两组服药后血浆ACTH的重叠现象消失。本文结果表明,血清皮质醇是诊断肾上腺皮质功能减退的直接证据。但不能鉴别PAF和SAF,而血浆ACTH可以鉴别两者,氨基导眠能试验有助于鉴别SAF和正常人。     

前庭功能锻炼对犬血液神经内分泌激素水平的影响

目的 探讨前庭功能锻炼对犬旋转刺激后血液神经内分泌指标的影响,阐明其与运动病的内在联系.方法 8只毕格犬,旋转刺激诱发运动病,测定呕吐潜伏期,同时用放射免疫分析法测定血浆皮质醇、促肾上腺皮质激素(ACTH)、精氨酸加压素(AVP)、肾上腺素与去甲肾上腺素水平.前庭功能锻炼3个月,观察呕吐潜伏期变化,再进行激素水平测定.结果 1)前庭功能锻炼后,呕吐潜伏期延长(P＜0.01);2)前庭功能锻炼前,旋转刺激导致血浆皮质醇、AVP水平升高(P＜0.05);3)前庭功能锻炼后,血浆皮质醇、AVP基础水平有所升高(P＜0.05或0.01),但旋转刺激诱导的反应减弱;4)旋转刺激及前庭功能锻炼对血浆ACTH、肾上腺素与去甲肾上腺素水平无显著影响(P＞0.05).结论 1)前庭功能锻炼可以抑制运动病;2)旋转刺激可以引起血浆皮质醇、AVP水平升高;3)前庭功能锻炼可提高血浆皮质醇、AVP基础水平,但会减弱对运动病诱发刺激的反应.     

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.     

亚临床库欣综合征临床及生化特征分析

目的 评价肾上腺意外瘤中亚临床库欣综合征的临床及生化特征,以及各项检查方法在亚临床库欣综合征诊断中的作用.方法 对45例亚临床库欣综合征患者进行8、24 h血皮质醇、尿游离皮质醇检测,同时进行8 h血促肾上腺皮质激素及2 mg地塞米松抑制试验(dexamethasone suppression test,DST),将结果与51例肾上腺无功能瘤患者比较.结果 (1)亚临床库欣综合征组腹型肥胖、高血压、高血糖及脂代谢紊乱的患病率均高于肾上腺无功能瘤组,腰围、收缩压及血三酰甘油高于无功能瘤组患者,根据ATP Ⅲ标准,2组代谢综合征患病率分别为31.1%(14/45)及11.8%(6/51);(2)各项生化检查对于亚临床库欣综合征诊断的敏感性由高至低依次为2 mg DST不抑制(100.0%)、24 h血皮质醇升高(77.8%)、尿游离皮质醇升高(46.7%)及8 h血皮质醇升高(40.0%);特异性以2 mg DST不抑制及8 h血皮质醇升最高(94.1%)最好,其次为尿游离皮质醇升高(84.3%),24 h血皮质醇最低(74.5%).结论 亚临床库欣综合征患者代谢综合征相关症状的患病率升高,可对患者造成一定危害,在其各项检测指标中,2 mg DST不抑制和24 h血皮质醇升高具有较好的临床价值.     

促肾上腺皮质激素依赖性库欣综合征患者岩下窦静脉采血的临床应用探讨

目的 研究并探讨岩下窦静脉采血(IPSS)在疑难库欣综合征(CS)诊断中的应用价值。方法 16例疑难CS患者行双侧岩下窦和外周静脉采血,测定岩下窦与外周静脉血促肾上腺皮质激素(ACTH)浓度比,评价IPSS对库欣病诊断的敏感度和特异度。结果 13例患者岩下窦与外周静脉血ACTH的比值＞2.0,其中12例经蝶窦垂体手术治疗,病理证实为垂体ACTH腺瘤;1例行鞍区γ-刀治疗,治疗后患者症状好转。3例岩下窦与外周静脉血ACTH的比值＜2.0,其中2例为右肺类癌,另1例为垂体ACTH腺瘤。IPSS诊断库欣病的敏感度和特异度分别为13/14和2/2。结论 IPSS诊断疑难库欣病的敏感度和特异度均较高,且操作安全,并发症少,可作为疑难CS的重要鉴别手段,值得临床上进一步推广应用。     

Adrenal incidentalomas showing unilateral concordant visualization by adrenocortical scintigraphy: Comparison with adenomas in Cushing’s syndrome

An adrenocortical adenoma causing Cushing's syndrome (Cushing's adenoma) produces a unilateral concordant visualization (UCV) imaging pattern in which the adenoma is only visualized on radioiodocholesterol adrenocortical scintigraphy. But because this imaging pattern is also noted in some patients with adrenal incidentalomas, we examined whether the UCV-incidentaloma was essentially identical with Cushing's adenoma and would develop Cushing's syndrome. The subjects were 9 patients with UCV-incidentalomas (mean size, 30 mm; range, 20-45 mm) and 6 patients with Cushing's adenomas (mean size, 28 mm; range, 25-35 mm). Endocrinological evaluations showed several abnormalities including blunted diurnal rhythm of plasma cortisol within the normal range, low plasma ACTH and/or high 24-hr urinary 17-OHCS levels in 8 of 9 patients with UCV-incidentalomas, but these abnormalities did not meet the diagnostic criteria of Cushing's syndrome. Adrenal uptake of the tracer in the patients with UCV-incidentalomas was not statistically different from that in the patients with Cushing's adenomas and had no relationship with hormonal values in either patient group. Tumor size on CT correlated with the levels of 24-hr urinary 17-OHCS (r = 0.75, p = 0.02) and plasma cortisol at 7:00 (r = 0.82, p = 0.007) in the patients with UCV-incidentalomas, but not in the patients with Cushing's adenomas. Although 3 UCV-incidentalomas increased slightly in size, none of 9 patients with UCV-incidentalomas has developed Cushing's syndrome for 4 to 52 months. These results suggest that the UCV-incidentaloma may be essentially different from the Cushing's adenoma and unlikely to develop Cushing's syndrome.     

Selective bilateral blood sampling from the inferior petrosal sinus in Cushing's disease: Effects of corticotropin-releasing factor and thyrotropin-releasing hormone on pituitary secretion

We sought to enhance the sensitivity of selective bilateral blood sampling to determine adrenocorticotropin (ACTH) and prolactin levels in the inferior petrosal sinus (IPS) by administering two stimulatory agents—corticotropin-releasing factor (CRF) and thyrotropin-releasing hormone (TRH). We then determined the ACTH and prolactin levels in the IPS of 10 patients with Cushing's disease. After peripheral administration of both CRF and TRH, ACTH levels were significantly higher on the tumor side in all patients. The prolactin level was significantly higher on the tumor side when CRF or TRH was used to stimulate pituitary secretion. Postsurgical immunohistochemistry studies revealed production of both ACTH and prolactin in tumor cells, explaining the abnormal secretion pattern of the pituitary adenoma. The use of CRF and TRH may therefore improve the reliability of selective blood sampling and tests from the IPS in those cases of Cushing's disease for which noninvasive methods have otherwide failed to clarify the diagnosis.     

Adrenal scintigraphy with 131I-19-iodocholesterol in the diagnosis of Cushing's syndrome associated with adrenal tumor

Seven patients with Cushing's syndrome secondary to adrenocortical tumors were studied using ¹³¹I-19-iodocholesterol. The diagnosis of all cases were verified histologically. In three cases with adenoma the uptake of the tracer was in the tumor only, while the two patients with adrenocortical carcinoma failed to show adrenal accumulation of the labelled compound.In two patients there was a hyperplasia-like scintigraphic pattern, while the stimulation and suppression biochemical tests suggested adrenal tumor. One of these cases was verified as a mixed form (adenoma plus hyperplasia), and the tumor bearing gland was significantly larger on the scan which helped the preoperative localization. In the other case, verified as bilateral multiple adrenocortical adenomas, the autonomus function of both adrenals was proved by dexamethasone suppression scanning. It seens reasonable to use the latter as an adjunctive diagnostic procedure in patients where there is a discrepancy between the standart scintiscan and the biochemical indexes of adrenal hyperfunction.