非促肾上腺皮质激素依赖性皮质醇增多症伴双侧肾上腺占位1例报告并文献复习

正皮质醇增多症是指多种病因所造成的肾上腺皮质分泌过多的糖皮质激素引起的一系列临床综合征,也称为内源性皮质醇增多症。典型临床表现为向心性肥胖、满月脸、水牛背,多血质、紫纹等。内源性皮质醇增多症病因为促肾上腺皮质激素     

库欣综合征55例临床分析

目的分析库欣综合征患者的病因分类,各种主要临床表现的出现频率以及实验室检查特点,并对各种诊断方法的效率进行评价。方法收集2003～2008年在我院住院经临床和病理确诊为库欣综合征患者的病例资料,对它们进行回顾性分析。结果在本组病例中,库欣病最多见,肾上腺皮质腺瘤次之。临床表现方面,向心性肥胖和高血压出现的频率最高,均在70％以上。血糖异常和血脂异常的发生率分别为51．43％和70．59％,其中糖尿病的比例高达37．14％。四成以上的患者存在低血钾,肾上腺皮质腺癌患者100％有低血钾,且是重度低血钾。在库欣综合征诊断试验中,敏感性从高到低依次为不被小剂量地塞米松抑制,血皮质醇昼夜节律消失,24小时尿游离皮质醇增高,清晨血皮质醇增高。在库欣综合征患者的鉴别诊断中,80％以上的库欣病患者能被大剂量地塞米松抑制,而几乎94％的肾上腺皮质腺瘤患者不能被大剂量地塞米松抑制。有关影像学检查,垂体MRI可以检测出90％以上的库欣病患者存在垂体腺瘤,而肾上腺CT几乎100％可以发现出肾上腺肿瘤。结论本研究中库欣病和肾上腺皮质腺瘤仍然是最常见的病因,但后者的的比例相对较高。而在临床特点方面,高血压和糖尿病的发生率较高。对于库欣综合征的诊断和鉴别诊断,目前没有一种检查方法具有100％的敏感性,我们应对各种结果作出综合判断。     

赛庚啶治疗皮质醇增多症一例报告

本文报告用赛庚啶(Cyproheptadine)成功地治疗皮质醇增多症一例。患者女,15岁,学生。生长迟缓、向心性肥胖及原发性闭经5年。根据午夜血浆皮质醇、促皮质素浓度和尿类固醇排泄的增加,于1975年12月诊断为皮质醇增多症。X线检查垂体凹正常,静脉肾盂造影伴断层摄片征实肾上腺正常。每天使用地塞米松2毫克不能抑制而改用8毫克才能抑制升高的血浆皮质类固醇。虽应用垂体照射,一年后患者仍无临床及实验室的改善。曾考虑施行全肾上腺切除术,决定每天试用赛庚啶24毫克进行治疗。开始一月内,出现月经并持续规则。满月脸及向心性肥胖逐渐减轻。三个月内,第二性征有明显发     

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…     

99例皮质醇增多症的临床与病理分析

作者对本院收治的99例皮质醇增多症进行了临床与病理分析。经病理证实56例,肾上腺皮质增生占64.3%,肾上腺腺瘤占30.3%,肾上腺皮质癌占5.4%。对皮质醇增多症的临床表现、实验室检查、以及引起骨质疏松的机理进行了讨论。     

库欣综合征81例临床分析

81例皮质醇增多症中增生型（库肤病）63例，腺瘤型18例，女：男=3．5：1。30岁以上患者占83．3％，平均病程3．7年、增生型患者表现痤疮、多毛、色素沉着、月经失调和骨质疏松者显著增多。大剂量地塞米松的抑制率增生型为64.8％，腺瘤型只有15．4％。垂体瘤的检出率CT和MRI均为87％，肾上腺CT证实肿瘤的阳性率94．4％。增生型的治疗首选经蝶垂体瘤摘除术，治愈率88．2％，肾上腺腺癌切除的治愈率100％。     

高血压并发低血钾与肾上腺疾病15例临床分析

目的:总结我院15例高血压并低血钾患者的临床表现与诊治结果,提高继发性高血压的诊疗水平。方法:对15例患者的临床表现、激素水平、CT定位诊断及术后病理进行回顾分析。结果:15例患者中原发性醛固酮增多症13例(醛固酮瘤11例、双侧肾上腺增生2例),腺瘤型皮质醇增多症2例。结论:顽固性高血压并发低血钾应高度怀疑肾上腺疾病,诊断依靠临床表现及内分泌功能测定,定位诊断依靠CT,腺瘤型手术效果好。     

大结节样肾上腺增生与Cushing''s综合征

皮质醇增多症是一类常见的内分泌疾病，根据某些临床表现如向心性肥胖，多血质貌，高血压和有关的生化检查可以作出诊断。一旦诊断确定，摆在内科医生面前的主要任务就是确定皮质醇分泌增多的原因。根据皮质醇的分泌是完全自主，还是受ＡＣＴＨ控制的，皮质醇增多症可分为...     

皮质醇增多症的特殊类型

皮质醇增多症系Harvey-Cushing于1932年首先描述并命名。其主要特征是由于慢性糖皮质激素内源性分泌过多,而引起的一系列临床与代谢紊乱,如满月脸、多血症、向心性肥胖、高血压、多毛、痤疮、紫纹、糖代谢紊乱等,这些典型表现已为临床医生所熟悉。习惯上我们把下丘脑-垂体病变所致的双侧肾上     

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。     

Clinical laboratory findings and results of therapy in 55 patients with Cushing's syndrome

In this study, 55 patients with Cushing's syndrome (CS) (50 female, 5 male; mean age 34 +/- 12.3 yr) who attended our clinics between the years 1983 and 2000 were retrospectively evaluated for clinical and laboratory features and modalities and results of therapy, due to a few similar studies over the last ten years. Cushing's disease was diagnosed in 39 patients (71%), adrenal adenoma in 13 patients (23.6%) and adrenal carcinoma in 3 patients (5.5%). Centripedal obesity, moon face, hypertension, hirsutism and purplish stria were the most frequent findings. Loss of normal serum F circadian rhythm was found in all patients with CS. The overnight 1 mg oral dexamethasone suppression test and low-dose dexamethasone suppression test (LDDST) yielded 100% and 100% diagnostic sensitivity for CS, respectively. Sensitivity and specivity of the high-dose dexamethasone suppression test (HDDST) in distinguishing Cushing's disease was found to be 82% and 100%, respectively. All of the patients with adrenal CS were not suppressed with HDDST. Sellar CT and/or MRI accurately identified the tumor in 58% of these patients. Recurrence was observed in 3 (11%) of the 28 patients with Cushing's disease, treated by transsphenoidal adenomectomy. Recurrence was diagnosed 1.5, 3 and 6 yr after the operation in these 3 patients. One patient had residue tumor. In our case series, bilateral adrenalectomy plus pituitary irradiation achieved the highest remission rate (100%) in Cushing's disease. In 2 out of 4 patients (50%) treated by left adrenalectomy associated with pituitary irradiation, recurrence was observed. Panhypopituitarism due to tumor apoplexy was observed in one of the patients with Cushing's disease. All of the patients with adrenal CS, the tumor was accurately localized with imaging methods before the operation. The appropriate operative procedure resulted in complete remission in patients with adrenal adenoma. Consequently, Cushing's disease was the most common form of CS. The overnight 1 mg oral DST and 24-h urine free F excretion (UFC) as screening tests, 2-day LDDST as diagnostic test and 2-day HDDST as differential diagnostic test were good studies. More successful outcomes have been achieved in treatment of Cushing's disease with the development of pituitary surgery in the recent years, as well as in our case series. Surgery is also curative for adrenal adenoma patients. Survival remains poor among carcinoma patients.     

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。     

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.     

主动脉夹层110例临床分析

目的：探讨主动脉夹层（AD）的易患因素、临床表现、诊疗和预后的影响因素。方法：回顾性分析110例AD患者的临床资料。结果：AD发病率呈上升趋势。79例（71，8％）为DeBakey Ⅲ型夹层；80例（72．7％）合并高血压病；CTA和MRI诊断率均为100％。急性期死亡15例（13．7％），平均在4．5d内。21例（19．1％）行介入支架治疗，术中和术后近期均未发生不良并发症。结论：对疑诊AD者应尽快确诊并合理选择治疗方式，急性期CTA优于MRI，介入支架治疗能明显改善AD患者的生活质量和预后。     

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.     

Around-the-clock Ambulatory Blood Pressure Monitoring is Required to Properly Diagnose Resistant Hypertension and Assess Associated Vascular Risk

Obesity is prevalent worldwide and associated with co-morbidities that result in increased cardiovascular risk. Hypertension is the most prevalent obesity comorbidity associated with increased cardiovascular risk. Obesity hypertension is a distinct subtype of essential hypertension. While endogenous Cushing’s syndrome is an uncommon cause of both obesity and hypertension, the recent recognition of other hypercortisolemic states has raised the profile of hypercortisolism as an important contributor in obesity hypertension. The high prevalence of exogenous, iatrogenic, pseudo, and subclinical Cushing’s syndromes makes hypercortisolism an important diagnostic consideration in the evaluation and management of patients with obesity hypertension who are resistant to conventional management. Available data suggest that the renin-angiotensin-aldosterone system modulating antihypertensives have the best efficacy in hypercortisolism-mediated obesity hypertension. Strategies aimed at reducing cortisol production and action also have utility. This review provides a comprehensive overview of the epidemiology, etiopathogenesis and management options available for glucocorticoid-mediated obesity hypertension.     

肾上腺皮质腺瘤患者高血糖的患病率及术后改变情况的观察

目的 评价肾上腺皮质腺瘤患者高血糖的患病情况及术后改变。 方法 选取肾上腺醛固酮瘤（APA）患者65例、皮质醇瘤（CSA）患者43例及无功能瘤（NAA）患者24例行OGTT及检测激素测值,并与原发性高血压（EH）患者53例及正常对照（NC）者58名作比较。APA、CSA及NAA患者行肾上腺肿瘤摘除术后随访激素及OGTT,与术前比较。 结果 ⑴各组糖尿病患病率分为44.6%、48.8%、25.0%、24.5%及17.5%。⑵APA、CSA组激素水平与糖代谢测值有相关性。⑶将APA及CSA组分别分为伴和不伴高血糖亚组进行比较,APA组中伴高血糖亚组血醛固酮高于不伴高血糖亚组;CSA组中伴高血糖亚组血皮质醇高于不伴高血糖亚组。⑷随着术后激素水平的下降,APA、CSA组糖代谢测值也得到改善,NAA组激素及糖代谢测值手术前后比较差异均无统计学意义。3组术后高血糖患病率分为35.4%、32.6%及33.3%。 结论 功能性肾上腺皮质腺瘤（包括肾上腺醛固酮瘤和肾上腺皮质腺瘤）患者高血糖的患病率升高,其原因与激素的高分泌相关。手术摘除肿瘤,激素高分泌状态解除后,患者的高血糖情况得以改善。     

Cushing's syndrome found during long-term glucocorticoid treatment of rheumatoid arthritis in an elderly woman]

A 69-year-old female patient had been treated with glucocorticoid for eight years because of rheumatoid arthritis. She showed characteristic Cushingoid features such as central obesity, moon face, and fragility of skin and vessels. She was disabled because of spinal compression fracture and muscle weakness. The blood pressure was 186/100 mmHg and the laboratory tests revealed serum K: 2.8 mEq/l, WBC: 15, 510/mm3, total cholesterol: 310 mg/dl. These suggested that she had iatrogenic Cushing's syndrome. After discontinuation of glucocorticoid, however, the serum cortisol level remained high. This fact prompted us to conduct further examinations for Cushing's syndrome. Oral dexamethasone administration did not suppress the plasma cortisol level and a left adrenal adenoma was found on abdominal CT scan. Because of the presence of bleeding diathesis, operation for adenoma was contraindicated. Though we tried to treat her with metyrapone, trilostane or opeprim (OP'-DDD), we had to abandon specific treatment because of severe side effects such as acute adrenal dysfunction and gastrointestinal problems. Decrease in the endogenous cortisol level after metyrapone treatment caused exacerbation of symptoms of rheumatoid arthritis. This is a peculiar case in which the long-term administration of glucocorticoid for rheumatoid arthritis might have concealed Cushing's syndrome, and conversely the increased intrinsic adrenal steroid hormone might have suppressed the activity of the rheumatoid arthritis.