诊断库欣综合征时多种检查方法的比较

目的评价诊断库欣综合征时多种临床试验方法的敏感性和特异性。方法在173例临床确诊库欣综合征患者中进行血、尿皮质醇测定,血皮质醇昼夜节律观察和地塞米松抑制试验,并与术后病理结果进行比较。结果库欣综合征患者血皮质醇节律消失者为92.9%,其中二点法节律消失率为85.1%(8:00,16:00)及91.8%(8:00,24:00),三点法为94.7%(8:00,16:00,24:00)。尿游离皮质醇(UFC)升高者为94.7%。小剂量地塞米松抑制试验不能抑制者为79.7%(1mg)和84.3%(2mg)。血皮质醇基础值升高者为75.6%。库欣病患者行8mg地塞米松抑制试验,以抑制率50%为标准时,敏感性为50%~70%,特异性高于95%。结论诊断库欣综合征敏感性最强的检测指标为血皮质醇昼夜节律的消失和UFC增高。血皮质醇昼夜节律用三点法评价较二点法敏感性高。8mg地塞米松抑制试验是鉴别库欣病和肾上腺皮质腺瘤最合适的方法。     

Cushing综合征的病因诊断与临床特点分析

目的分析Cushing综合征患者的不同病因、临床表现及内分泌实验室检查和影像学检查的特点,评价各种病因鉴别诊断方法的效率。方法对经手术病理确诊为Cushing综合征的42例患者的临床资料和实验室检查、影像学检查等资料进行回顾分析。结果 42例患者的病因以Cushing病占的比例最高,主要临床表现在不同病理类型之间发生率差异无统计学意义。但满月脸、紫纹、皮肤瘀斑在肾上腺瘤中的发生率比其他病理类型为高,64.3%的病人有典型的Cushing外貌,血糖异常、高血压的发生率分别为61.9%、61.9%。Cushing综合征诊断试验的敏感性从高到低依次为不被午夜1mg地塞米松抑制、小剂量地塞米松抑制试验、24h尿游离皮质醇升高、血皮质醇昼夜节律消失、清晨血皮质醇升高。在Cushing综合征的病因鉴别诊断中,93.3%的肾上腺腺瘤病人不被8mg大剂量地塞米松抑制,71.4%的病人被8mg大剂量地塞米松实验抑制。在影像学检查中,肾上腺CT可100%检查出肾上腺腺瘤。结论 Cushing病和肾上腺腺瘤是Cushing综合征最常见的病因。在临床上关注一些有倾向性的表现,并结合ACTH检测、8mg地塞米松抑制试验以及影像学检查,方能对Cushing综合征的病因作出准确的鉴别诊断。     

肾上腺占位合并垂体病变的库欣综合征九例临床分析并文献复习

目的探讨垂体及。肾上腺均有病变的库欣综合征患者的病因诊断方法。方法回顾性分析9例垂体及肾上腺均病变的患者,行大小剂量地塞米松抑制试验,查血促。肾上腺皮质激素（ACTH）水平,并行鞍区MRI及肾上腺CT。结果（1）血ACTH值在4例库欣病患者中3例高于正常上限,1例正常。5例非ACTH依赖性库欣综合征患者ACTH均〈20ng／L。（2）大剂量地塞米松抑制试验服药第2日24小时尿游离皮质醇在ACTH依赖组均可被抑制到对照值的20％以下,ACTH非依赖组5例患者均未被抑制到对照的50％以下。（3）术后病理支持临床诊断。结论对于兼有肾上腺及垂体两处病变的库欣综合征患者需采用多种方法进行鉴别,最后的判定是术后病理及疗效。     

双侧岩下窦静脉采血诊断ACTH依赖性库欣综合征的价值

目的 评估双侧岩下窦静脉采血(BIPSS)测定ACTH、垂体MRI动态增强和大剂量地塞米松抑制试验在ACTH依赖性库欣综合征鉴别诊断中的价值.方法 纳入BIPSS并经病理明确诊断的ACTH 依赖性库欣综合征患者87例,行BIPSS、大剂量地塞米松抑制试验和垂体影像学评估.结果 78例病理明确诊断为垂体ACTH瘤,9例为胸腺类癌所致异位ACTH综合征.大剂量地塞米松抑制试验、垂体MRI动态增强和BIPSS诊断敏感性分别为82.1％、79.5％和92.3％,特异性分别为100％、44.4％和100％,诊断准确率分别为83.9％、77.0％和93.1％.库欣病患者BIPSS分侧定位符合率为83.9％,而垂体MRI分侧定位符合率为64.5％.结论 ACTH依赖性库欣综合征中,BIPSS比大剂量地塞米松抑制试验、垂体MRI 动态增强诊断符合率更高.与垂体MRI检查相比,BIPSS分侧定位更为可信.     

库欣综合征与低血钾

库欣综合征又称皮质醇增多症 ,是肾上腺皮质分泌过量的糖皮质激素所致 ,临床上主要表现为满月脸、向心性肥胖、皮肤紫纹、高血压和低血钾等。其发病原因有垂体分泌促肾上腺皮质激素 (ＡＣＴＨ)过多 (库欣病 )、原发性肾上腺皮质腺瘤或腺癌、异位ＡＣＴＨ综合征以及不依赖ＡＣＴＨ的双侧小结节性增生或小结节性发育不良等。1　库欣综合征伴低血钾的发生率库欣综合征病人经常发生低血钾 ,但明显的低血钾性碱中毒主要见于异位ＡＣＴＨ综合征和肾上腺皮质腺癌病人。尤其是异位ＡＣＴＨ综合征 ,其 90 %可发生低血钾。其它原因所致的库欣综合征则…     

22例皮质醇增多症的临床研究

目的：探讨皮质醇增多症的临床表现和内分泌检查等辅助检查的意义.方法：从年龄、性别、病程及实验室检查等方面,观察22例不同原因所致皮质醇增多症患者不同的临床表现和测定实验室检查指标.结果：22例中诊断库欣病（增生型）14例[63.6％,其中13例（92.9％）得到MRI检查证实],肾上腺腺瘤6例[27.3％,均得到MRI检查证实（100％）],另有肾上腺结节样增生1例（4.5％）,异位ACTH综合征1例（4.5％）.临床表现：按出现的频率前4位依次为,库欣病：高血压（100％）、满月脸（92.9％）、向心性肥胖（85.7％）、多血质（85.7％）,肾上腺腺瘤：高血压（100％）、满月脸（100％）、向心性肥胖（100％）、多血质（83.3％）.实验室检查：小剂量地塞米松不能抑制：库欣病与肾上腺腺瘤均为100％.结论：根据高血压、满月脸、向心性肥胖,小剂量地塞米松抑制试验和MRI检查可诊断绝大多数皮质醇增多症.     

迷走肾上腺皮质腺瘤所引起的库欣综合征一例

迷走肾上腺皮质腺瘤所引起的库欣综合征一例吴国梁高皮质醇症一般是由垂体嗜碱细胞瘤或肾上腺腺瘤所引起，然而少数患者是由垂体和肾上腺外的产生皮质醇或产生ＡＣＴＨ的肿瘤引起。本文报告一例迷走肾上腺腺瘤所引起的库欣综合征（Ｃｕｓｈｉｎｇｓｙｎ－ｄｒｏｍｅ）患者...     

2022年亚临床库欣综合征专家指导建议

亚临床库欣综合征较库欣综合征更常见, 指生化检查皮质醇增多而无特异性的典型库欣综合征临床表现, 可以导致多种代谢异常, 临床上易忽视而相关研究较少, 需要重视和规范诊治。亚临床库欣综合征常见于肾上腺意外瘤患者。对于肾上腺或垂体意外瘤以及与年龄、体重不匹配的骨代谢异常或经规范治疗后糖尿病、高血压和脂代谢控制不佳的患者, 应进行亚临床库欣综合征筛查。初筛首选1 mg过夜地塞米松抑制试验, 确诊试验为小剂量地塞米松抑制试验。诊断后再根据促肾上腺皮质激素水平, 结合影像学和其他功能试验进一步明确病变部位及病因。符合手术指征的肾上腺瘤亚临床库欣综合征患者, 手术后获得最佳代谢改善。围手术期需进行糖皮质激素补充, 术后糖皮质激素替代应个体化。所有患者均应进行定期随访。     

库欣综合征患者小剂量与大剂量地塞米松抑制试验之间的关系

目的 探讨库欣综合征患者小剂量地塞米松抑制试验中血清皮质醇水平抑制程度与大剂量地塞米松抑制试验可受抑制(血清皮质醇水平被抑制50%以上)之间的关系,及其对库欣病的诊断价值.方法 回顾性分析广州中山大学附属第二医院1990年1月至2009年1月收治的91例库欣综合征患者,观察过夜小剂量(1 mg)地塞米松抑制试验中血清皮质醇水平被抑制20%、30%、40%和50%以上与过夜大剂量(8 mg)地塞米松抑制试验可受抑制之间的关系,并比较上述各切点对库欣病诊断的敏感性与特异性.结果 在过夜1 mg地塞米松抑制试验中血清皮质醇水平被抑制的程度(较基础08:00血清皮质醇水平下降的百分比)与在过夜8 mg地塞米松抑制试验中血清皮质醇水平被抑制的程度相关(r=0.649,P<0.001);在过夜1 mg地塞米松抑制试验中血清皮质醇水平被抑制20%、30%、40%和50%以上的例数分别为30、22、13和9例.在这些患者中,过夜8 mg地塞米松抑制试验可受抑制的例数分别为23例(76.7%)、20例(90.9%)、12例(92.3%)和9例(100.0%).血清皮质醇水平被1 mg地塞米松抑制20%、30%、40%和50%以上对库欣病诊断的敏感性分别为52.8%、32.7%、22.6%和15.7%,特异性分别为94.7%、94.7%、97.4%和97.4%.结论 对于库欣综合征患者,若血清皮质醇水平可被过夜1 mg地塞米松抑制20%以上者,大多数也可被过夜8 mg地塞米松抑制试验所抑制,并且其大多数为库欣病患者.     

过夜小剂量地塞米松抑制试验对库欣综合征诊断价值的再探讨

目的重新探讨过夜小剂量地塞米松抑制试验对库欣综合征的诊断价值。方法回顾性分析我院1990年1月到2005年7月收治的52例库欣综合征患者和153例经住院检查排除库欣综合征的肥胖症和高血压病患者，比较过夜小剂量（1mg）地塞米松抑制试验的不同血清皮质醇切点对库欣综合征诊断的敏感性和特异性。结果在过夜1mg地塞米松抑制试验中，以试验当日晨8：00血清皮质醇为基础值，次日8：00血清皮质醇为基础值的50％以及次日晨8：00血清皮质醇275、200、138和50nmol／L为切点，诊断库欣综合征的敏感性分别为92．3％、92．3％、92．3％、92．3％和100．0％，特异性分别为90．8％、98．7％、96．1％、91．5％和78．4％。结论在过夜1mg地塞米松抑制试验中，以次日晨8：00血清皮质醇50nmol／L为切点具有较高的敏感性，可作为库欣综合征的第一线筛选试验。     

Continuous dexamethasone infusion for seven hours in patients with the Cushing syndrome. A superior differential diagnostic test

STUDY OBJECTIVE: To determine the usefulness of the continuous intravenous dexamethasone suppression test in the differential diagnosis of patients with the Cushing syndrome. DESIGN: Comparison of the results of this test with those of other tests in the study population and with data from the literature. SETTING: Referral-based endocrine division in a university hospital. PATIENTS: One hundred and twenty-one patients with the Cushing syndrome were evaluated: corticotropin (ACTH)-secreting pituitary tumors, 90 cases; adrenal adenoma, 13; adrenal carcinoma, 9; ectopic ACTH-secreting tumors, 6; corticotropin-releasing-hormone (CRH)-secreting tumors, 2; and autonomous bilateral micronodular adrenal hyperplasia, 1. The continuous intravenous dexamethasone suppression test was done in 101 patients. MEASUREMENTS AND MAIN RESULTS: Dexamethasone (1 mg/h) was administered intravenously for 7 hours. A cortisol decrease of at least 190 nmol/L was defined as a positive response. In identification of patients with ACTH-secreting pituitary adenomas, this test had a sensitivity of 100% (95% CI, 95% to 100%), a specificity of 90% (CI, 70% to 99%), and a diagnostic accuracy of 98% (CI, 93% to 100%). Only two patients had false test results. They both had CRH-secreting tumors. Cortisol secretion was also shown to be sensitive for feedback control in other tests, however. CONCLUSION: The continuous intravenous dexamethasone suppression test has better diagnostic accuracy than other tests that are currently used in the differential diagnosis of the Cushing syndrome, and this test is very convenient. The only false test results were found in patients with CRH-secreting tumors.     

Urine free cortisol in the high-dose dexamethasone suppression test for the differential diagnosis of the Cushing syndrome.

OBJECTIVE: To develop criteria for interpreting the high-dose dexamethasone suppression test using urine free cortisol as an end point for the differential diagnosis of the Cushing syndrome. DESIGN: Retrospective review. SETTING: Inpatient research ward. PATIENTS: Patients (118) with surgically confirmed causes of the Cushing syndrome: 94 with pituitary disease, 14 with primary adrenal disease, and 10 with ectopic adrenocorticotropic hormone (ACTH) secretion. MAIN OUTCOME MEASURES: The sensitivity, specificity, and diagnostic accuracy were determined for the high-dose dexamethasone suppression test using urine free cortisol and using 17-hydroxysteroid excretion. For each analysis, patients with pituitary disease were considered to be "diseased" and patients with nonpituitary disease were considered to be "non-diseased". The level of suppression that gave 100% specificity was determined for each steroid. RESULTS: The accuracy of urine free cortisol when used as an end point in the high-dose dexamethasone suppression test was equivalent to that of 17-hydroxysteroid excretion. At all levels of sensitivity and specificity, however, the degree of suppression of urine free cortisol used for the diagnosis of pituitary disease was greater than that of 17-hydroxysteroid excretion. The likelihood ratios for pituitary disease based on urine free cortisol suppression of greater than 50%, of greater than 80%, and of greater than 90% were 4.2, 10.1, and "infinite," respectively. Suppression of urine free cortisol greater than 90% or suppression of 17-hydroxysteroid excretion greater than 64% was associated with 100% specificity. When these criteria were combined, the percentage of correct predictions (102 of 118 [86%; 95% CI, 78% to 92%]) was higher than that obtained using either steroid alone (89 of 118 [75%; CI, 65% to 83%]) (P = 0.009) and higher than that obtained using the traditional criterion of 50% suppression for 17-hydroxysteroid excretion (95 of 118 [80%; CI, 71% to 87%]) (P = 0.016). CONCLUSIONS: In the high-dose dexamethasone suppression test, the degree of suppression of urine free cortisol used for the diagnosis of pituitary disease is greater than that traditionally used for 17-hydroxysteroid excretion. The diagnostic performance of the test is improved by measuring both urine free cortisol and 17-hydroxysteroid excretion and by requiring greater suppression of both steroids.     

Screening for Cushing’s syndrome in obese type 2 diabetic patients and the predictive factors on the degree of serum cortisol suppression

The aim of this study was to examine the frequency of Cushing??s syndrome (CS) in obese type 2 diabetic patients devoid of specific clinical symptoms of Cushing??s syndrome. A total of 148 obese (BMI ??30?kg/m² ) type 2 diabetic patients (113 female, 35 male) were included in the study. An overnight 1-mg dexamethasone suppression test (DST) was performed on all patients. Suppression of serum cortisol to <1.8???g/dL after administration of 1?mg dexamethasone was considered normal suppression. Low dose dexamethasone suppression test was performed on the patients who had serum cortisol level over 1.8???g/dL after overnight 1?mg DST. Regression analysis was applied to determine the effective factors on suppression of serum cortisol. Mean age, BMI and HbA1c levels respectively were 50,82?±?8,50?year, 31,78?±?4,66?kg/m², %8,96?±?2,42 in males and 54,15?±?10,348?year, 34,32?±?5.71?kg/m², % 8,18?±?2,06 in females. Serum cortisol level was found 1.64?±?5.62???g/dl after overnight DST. A total of 9 (6.2?%) patients had non-suppressible overnight DST. Only four (2.6 %) of these patients were diagnosed with Cushing Syndrome after low-dose DST. Diagnosis was confirmed pathologically. Etiologic reasons for Cushing??s syndrome were pituitary microadenoma (2 patients) and adrenocortical adenoma (2 patients). Age and duration of diabetes was found to be related to the degree of suppression. Cushing??s syndrome should be investigated in high-risk groups like uncontrolled diabetes, obesity. All the related factors on the degree of suppression must be considered for the final diagnosis.     

Cyclic Cushing's disease with paradoxical response to dexamethasone

Cyclic Cushing's disease is an unusual disorder characterised by ACTH-dependent periodical increase of serum cortisol levels, clinically accompanied by peripheral edema, abnormalities of cardiac rhythm and hypokalemia. The condition may be unrecognised for years, since the typical features of Cushing's disease are usually absent due to the intermittent and brief duration of cortisol hypersecretion. We describe the case of a 42-yr-old man with Cyclic Cushing's disease due to an ACTH-producing pituitary macroadenoma, who presented two episodes of hypercortisolism in a 3-yr-period, clinically characterised by peripheral edema, hypokalemia and arrhythmia. The diagnosis was suspected because of a paradoxical increase of plasma ACTH and cortisol after dexamethasone administration during an asymptomatic period and was confirmed by pituitary imaging and by final histology after transphenoidal resection of the pituitary adenoma. After surgery, the patient resumed a normal pituitary-adrenal function with restoration of the normal ACTH and cortisol suppression after dexamethasone. Cyclic Cushing's disease should be considered in the differential diagnosis of several conditions characterised by recurrent episodes of idiopathic edema, hypokalemia or unexplained cardiac arrhythmia. In such patients, the pituitary-adrenal axis should be tested possibly during the acute phase of their disease or using the dexamethasone suppression test during asymptomatic intervals.     

Discriminatory value of the low-dose dexamethasone suppression test in establishing the diagnosis and differential diagnosis of Cushing's syndrome

Cushing's syndrome requires a screening test of high sensitivity, followed by biochemical evaluation of the source of the tumor when the cause is ACTH dependent. The high-dose dexamethasone suppression test is still in common use as an aid in differential diagnosis, although its value has been queried. We have routinely used the low-dose dexamethasone suppression test for many years in the diagnosis of Cushing's syndrome but noticed that patients with pituitary-dependent Cushing's syndrome or Cushing's disease, usually showed some degree of suppression of their serum cortisol, compared to those with the ectopic ACTH syndrome. We therefore analyzed retrospectively the serum cortisol responses during the low-dose dexamethasone suppression test and the high-dose dexamethasone suppression test in 245 patients with ACTH-dependent Cushing's syndrome and compared the diagnostic utility of each test either alone or in combination with a standard test using CRH. Evaluation of the serum cortisol response at 24 and 48 h during the low-dose dexamethasone suppression test correctly identified 98% of patients with ACTH-dependent Cushing's syndrome and distinguished between pituitary and ectopic causes with a sensitivity of 82% and a specificity of 79%. In the same patients, the serum cortisol response to the high-dose dexamethasone suppression test had a slightly higher sensitivity (91%) and specificity (80%). However, the combined criteria of a more than 30% suppression of serum cortisol during the low-dose dexamethasone suppression test and/or a more than 20% increase in the CRH test had a significantly higher sensitivity (97%) and specificity (94%) than either the high-dose dexamethasone or the CRH tests alone in the differential diagnosis of ACTH-dependent Cushing's syndrome. It produced equivalent information to that when high-dose and CRH test results were combined. We therefore conclude that in our patient series, the serum cortisol response during the low-dose dexamethasone suppression test is highly sensitive in diagnosing Cushing's syndrome and, combined with the results of the serum cortisol response to the CRH test, offered a safe and cost-effective test in the differential diagnosis of ACTH-dependent Cushing's syndrome. There does not appear to be any necessity for retaining the high-dose dexamethasone suppression test in this diagnostic work-up.     

OVERNIGHT DEXAMETHASONE PRE-TREATMENT IMPROVES THE PERFORMANCE OF THE LYSINE-VASOPRESSIN TEST IN THE DIAGNOSIS OF CUSHING'S SYNDROME

OBJECTIVE There is no endocrine test which is completely reliable for the confirmation of Cushing's syndrome and in separation of the various aetiologies. We have tested the hypothesis that overnight dexamethasone pre-treatment should result in a better performance of the lysine-vasopressin (LVP) test in the diagnosis of Cushing's syndrome. STUDY DESIGN AND PATIENTS We studied 61 subjects, including 25 pituitary-dependent and 9 pituitary independent Cushing's (7 adrenal tumours and 2 ectopic ACTH syndromes), 18 euadrenal controls, 4 depressed subjects, and 5 cushingoid patients. The subjects received 1 mg of dexamethasone orally at 2300 h and the following morning they were given 10 IU of lysine-vasopressin im. MEASUREMENTS Plasma cortisol (RIA) was measured at times ?15, 0, 15, 30, 45, 60, 75, 90 and 120 minutes. RESULTS The dexamethasone-modified LVP (Dx/LVP) test resulted in four patterns of cortisol response. The dexa sensitive pattern (positive suppression and negative response to LVP) was found in euadrenal subjects; the dexa insensitive pattern (negative suppression and positive response to LVP) was seen in Cushing’s disease; a non-responsive pattern (negative suppression and negative response to LVP) was observed only in pituitary independent Cushing's; and an indeterminate pattern (positive suppression and positive response to LVP) was equivocal, being observed in 2 control subjects, 1 patient with Cushing’s disease and 1 depressed patient. In separating control subjects from Cushing's syndromes the Dx/LVP test had 88.9% sensitivity, 100% specificity and 96.2% diagnostic accuracy; when the test was used to segregate Cushing's disease from control subjects we found 96.0% sensitivity, 100% specificity and 97.7% diagnostic accuracy. The performance variables for the Dx/LVP test in separating pituitary dependent from pituitary independent Cushing's were uniformly 100%. Depressed and cushingoid subjects did not differ from control subjects in their cortisol patterns during the test. Successful removal of the pituitary microadenoma in Cushing's disease was invariably followed by a reversal of the abnormal cortisol pattern (dexa insensitive) during the test to a dexa sensitive pattern indistinguishable from that of control subjects. CONCLUSION These results confirm our hypothesis and suggest that an improved performance of any corticotroph stimulus (oCRH, LVP, AVP or desmopressin) in the diagnosis of Cushing's syndrome should result from pre-treatment with dexamethasone.