原发性醛固酮增多症的分型诊断

目的 探讨用于原发性醛固酮增多症(原醛症)分型诊断检查方法的价值.方法 收集本院近7年来57例临床确诊的原醛症患者[醛固酮瘤22例,特发性醛固酮增多症(特醛症)26例,原发性肾上腺增生9例],检测患者的血电解质、血浆肾素活性及血、尿醛固酮,将结果与19例原发性高血压患者对照.再通过肾上腺CT、体位激发试验及肾上腺静脉采血检查对原醛症患者分型并随访.结果 (1)醛固酮瘤患者血压及血、尿醛固酮较特醛症患者高,血钾及血浆肾素活性则低,而原发性肾上腺增生患者临床及生化改变介于两者之间.肾上腺CT检查在原醛症分型诊断中的符合率为醛固酮瘤86.4%,特醛症73.1%,原发性肾上腺增生22.2%;肾上腺静脉采血检查以两侧醛固酮之比作为判定标准时符合率为86.4%、80.8%和77.8%,以醛固酮与皮质醇之比为判定标准则符合率分别为95.5%、92.3%及100.0%.(2)醛固酮瘤及原发性肾上腺增生患者术后随访血醛固酮均下降,血压恢复正常者分别为22.7%及44.9%,血钾恢复正常者为83.3%及100.0%,而特醛症患者随访中各项测值无明显变化,另有33.3%诊断时血钾正常的患者随访中出现低血钾.结论 原醛症的分型诊断需依靠多种检查手段综合分析,单纯依赖影像学检查或体位激发试验并不可靠,肾上腺静脉采血检查可作为影像学检查的补充,用两侧醛固酮与皮质醇的比值分析较单纯比较两侧醛固酮之比更为可靠;醛固酮瘤及原发性肾上腺增生患者术后临床及生化测值均得以明显改善,而特醛症患者随访中无明显变化.     

原发性醛固酮增多症中肾上腺腺瘤与增生的多种鉴别诊断方法比较

该文评价多种临床试验方法对肾上腺腺瘤（APA）和增生（BAH）的敏感性和特异性。方法：在124例临床确诊的原发性醛固酮增多症（PA）患者，入选标准为：1）高血压：血压〉140／90mmHg，应用常规降压药物仍控制不佳；2）低血钾：血钾〈3．5mmol／L，同时伴有尿路失钾；3）高血、尿醛固酮；清晨8：00卧位血醛固酮〉565．1pmol／L，24h尿醛固酮高于12．7μg；4）低血浆肾素活性：清晨8时卧位血浆肾素活性〈0．1ng／mL·h，且不易被激发，站立2h及肌肉注射呋塞米0．7mg／kg后激发值〈0．73ng／mL·h；5）影象学检查有或无肾上腺占位性病变。进行体位激发试验（PST）：患者于清晨8时卧位抽血检测血醛固酮及血浆肾素活性，然后肌肉注射呋塞米0．7mg／kg（通常40mg）并站立2h再抽血。体位激发后血醛固酮较基础值升高30%以上为阳性，低于30％或下降者为阴性。赛庚啶抑制实验：患者清晨卧位顿服赛庚啶8mg，于服药前30min及服药后30、60、90、120min抽血测定醛固酮浓度。服药后血醛固酮浓度下降111pmol／L或较基础值下降30％以上者为阳性，降幅低于30％或服药后血醛固酮值反而上升为阴性。肾上腺静脉插管检查（VSA），并与影像学检查、术后病理结果进行比较。结果：（1）APA组的血钾显著低于BAH组（Pd0．01），血、尿醛固酮则显著增高（P〈0。05）。（2）PST：22例APA患者中，12例血醛固酮升幅〉30%，7例升幅〈30％，较试验前下降者有3例；7例BAH中有4例升幅〉30％。（3）赛庚啶试验：BAH中反应者占69．2oA（18／26），APA中反应者占50．0％（20／40）。（4）VSA：11例行VSA的APA患者中，9例APA插管结果与术后病理结果相符，另2例示醛固酮不呈一侧优势分泌；3例BAH插管结果均与影像学检查结果相符。结论：PST及赛庚啶试验在APA及BAH中有相当一部分重叠，对两者的鉴别价值有限。影像学未能发现明显占位性病变者可进行VSA，如结果提示一侧病变则考虑手术治疗；如提示双侧病变，则予药物治疗，并进行随访。     

原发性醛固酮增多症308例经肾上腺静脉取血术定位诊断结果分析

目的 探讨肾上腺静脉取血(AVS)术在原发性醛固酮增多症（原醛）定位诊断中的应用价值,分析单、双侧病变的构成比和临床特征差异.方法 针对经过醛固酮肾素比值(ARR)初筛、静脉盐水负荷试验等确诊实验定诊并行AVS术定位诊断的原醛患者,回顾性收集其临床及生化资料进行分析.结果 原醛患者308例[男193例,女115例,年龄...     

88例原发性醛固酮增多症临床回顾分析

目的提高对原发性醛固酮增多症（简称原醛）的诊治水平。方法回顾性分析88例原醛患者的临床资料。结果血压水平、血浆醛固酮浓度（PAC）升高,血浆肾素活性（PRA）、血清钾离子浓度下降。醛固酮瘤占84．09％,特发性醛固酮增多症（简称特醛症）占15．91％。95．45％患者的血浆醛固酮／血浆肾素活性比值（ARR）〉20。醛固酮瘤患者具有更高的血浆醛固酮水平及更低的血浆肾素活性和血清钾离子浓度。结论ARR是原醛的重要筛查方法;卧立位试验及CT有助于肾上腺皮质腺瘤和增生的鉴别。     

原发性醛固酮增多症患者术前术后胰岛B细胞功能变化的研究

目的 探讨原发性醛固酮增多症（简称“原醛症”）患者肾上腺腺瘤手术前后胰岛B细胞功能的变化。方法 北京协和医院内分泌科2004-2006年收治的17例原醛症患者，接受肾上腺腺瘤摘除术前后均进行标准的75g葡萄糖耐量试验，分析胰岛素抵抗指数，HOMA分泌功能指数、胰岛素曲线下面积（INSAUC）胰岛素和血糖增量之比（△I30／△G30）等指标的变化以及和血钾、血浆醛固酮的相关关系。结果 △I30／△G30在术后明显升高（P=0．01），按体重指数（BMI）≥25分层后肥胖或超重组术后△I30／△G30增加不明显（P=0．08）；术前低血钾的患者术后胰岛B细胞分泌功能明显改善，而术前血钾正常者改善不明显。手术前后INSAUC变化和空腹血钾正相关（r=0，71，P=0．01），和醛固酮水平的变化以及随访时间无明显相关。结论 原醛症患者肾上腺腺瘤摘除术后低血钾纠正，胰岛B细胞的分泌功能改善，其改善程度受术前血钾和体重的影响。     

肾上腺静脉插管取血在原发性醛固酮增多症分型诊断中的意义

目的　探讨肾上腺静脉插管检查在原发性醛固酮增多症 (原醛 )分型诊断中的应用价值。方法　临床 14例明确为原醛患者 ( 11例腺瘤及 3例增生 )经肾上腺静脉插管检查 ,取双侧肾上腺静脉以及肾静脉水平下的下腔静脉采血 ,测各点醛固酮和皮质醇水平 ,并将结果与影像学检查及术后病理结果进行比较。结果　腺瘤患者插管结果与影像学检查及术后病理结果无统计学差异 ,符合率为 81 82 % ( 9/ 11) ,3例增生患者插管结果与影像学检查结果均相符。结论　肾上腺静脉插管检查在原醛的分型诊断中有较好的准确性 ,是影像学检查有疑问时可选择的良好方法。     

静脉导管术对原发性醛固酮增多症的定位诊断

本文6例原发性醛固酮增多症(简称原醛)患者,经静脉导管分段采血进行血浆醛固酮测定,旨在评价双侧肾上腺静脉、肾静脉血浆醛固酮浓度差及左肾上腺静脉与下腔静脉醛固酮浓度比值对原醛定位诊断的价值。结果表明,其定位诊断符合率高于CT扫描及B超检查,尤其适用于肾上腺的微小病变而非创伤性检查无阳性发现者。     

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目的评价原发性醛固酮增多症(PA)诊断中多项临床试验方法的作用。方法收集1995～2000年上海第二医科大学附属瑞金医院104例PA患者的临床资料,统计分析生化检查、体位激发试验、影像学检查与肾上腺静脉插管在诊断中的阳性率及符合率。结果(1)血醛固酮升高为筛选PA阳性率最高的检测指标。(2)醛固酮腺瘤患者生化异常较明显。(3)与手术病理比较,B超在醛固酮腺瘤和双侧肾上腺增生中的诊断符合率分为95.77%及73.33%;CT为98.51%及31.03%;体位激发试验以升幅30%为标准时在醛固酮腺瘤和双侧肾上腺增生中的符合率分别为61.11%及57.14%,以50%为标准时为72.22%及42.86%;肾上腺静脉插管的符合率为83.33%及100%。结论PA的诊断中,典型患者经血钾、血尿醛固酮及血浆肾素活性等筛查可明确诊断,但部分患者上述生化改变并不典型。体位激发试验结果在醛固酮腺瘤及双侧肾上腺增生中有部分重叠。影像学未能发现明显占位灶者可行肾上腺静脉插管检查。     

探讨促肾上腺皮质激素兴奋在肾上腺静脉取血中的应用价值

目的:探讨促肾上腺皮质激素(ACTH)兴奋在肾上腺静脉取血(AVS)中的应用价值。方法:纳入完成常规AVS和ACTH兴奋的AVS,且至少有一种AVS双侧肾上腺静脉插管成功的原发性醛固酮增多症(原醛症)患者。计算选择指数(SI)、优势侧指数(LI),比较ACTH兴奋与否对插管成功率及优势侧判断的影响。结果:共纳入73名原...     

原发性醛固酮增多症25例临床分析

回顾性分析、总结丁25例PA的临床资料。结果：APA19例，占76％；特发性醛固酮增多症（IHA）6例，占24％；66．67％IHA患者立位后醛固酮上升大于33％，氨体舒酮试验阳性率达80％；B超、CT检查结果与PA的诊断符合率为72％、92％。结论：血、尿醛固酮、肾素活性测定与氨体舒通试验是PA的主要诊断方法；血醛固酮立位试验有助于肾上腺皮质肿瘤和增生的鉴别；双侧肾上腺静脉采样，是PA分型定侧的重要方法；PA的定侧主要依靠B超、CT；外科手术是治疗APA的重要方法；IHA多采用药物治疗。     

A prospective evaluation of postural stimulation testing, computed tomography and adrenal vein sampling in the differential diagnosis of primary aldosteronism

Context In primary aldosteronism (PA), discriminating unilateral from bilateral disease is crucial because adrenalectomy is frequently curative in the former case but rarely helps in the latter. Various series have reported the utility of postural stimulation testing (PST), cross‐sectional imaging and adrenal vein sampling (AVS) in the assessment of PA, but most of these studies were retrospective. Objective To prospectively determine the diagnostic utility of PST, AVS and computed tomography (CT) using a radiological scoring system in the assessment of PA in a tertiary centre, as well as to document the incidence of autonomous cortisol cosecretion. Design and Setting Fifty consecutive patients with PA underwent PST, CT, AVS and a low‐dose dexamethasone suppression test with measurement of serum cortisol at 48 h. For patients who underwent surgery, histological confirmation, and a normal postoperative serum aldosterone concentration and plasma renin activity were taken as evidence for unilateral disease. For other patients, results from successful adrenal vein sampling were the diagnostic evidence against which CT and PST were assessed. Results Postural stimulation testing had a sensitivity and specificity of 44–56% and 71–75%, respectively. CT had an overall sensitivity and specificity of 77% and 80%, respectively, rising to 100% sensitivity and specificity if there was a single, discrete macronodule with an unequivocally normal contralateral gland. Evidence of cosecretion of cortisol occurred in 14% of patients. Conclusions Preliminary experience is presented of an objective radiological scoring system for selecting patients with PA for AVS. PST provides little, if any, useful additional information. A significant minority of patients with PA exhibit evidence of cortisol cosecretion, which may have implications for perioperative management.     

A prospective study of the prevalence of primary aldosteronism in 1,125 hypertensive patients.

OBJECTIVES: We prospectively investigated the prevalence of curable forms of primary aldosteronism (PA) in newly diagnosed hypertensive patients. BACKGROUND: The prevalence of curable forms of PA is currently unknown, although retrospective data suggest that it is not as low as commonly perceived. METHODS: Consecutive hypertensive patients referred to 14 hypertension centers underwent a diagnostic protocol composed of measurement of Na+ and K+ in serum and 24-h urine, sitting plasma renin activity, and aldosterone at baseline and after 50 mg captopril. The patients with an aldosterone/renin ratio >40 at baseline, and/or >30 after captopril, and/or a probability of PA (by a logistic discriminant function) > or =50% underwent imaging tests and adrenal vein sampling (AVS) or adrenocortical scintigraphy to identify the underlying adrenal pathology. An aldosterone-producing adenoma (APA) was diagnosed in patients who in addition to excess autonomous aldosterone secretion showed: 1) lateralized aldosterone secretion at AVS or adrenocortical scintigraphy, 2) adenoma at surgery and pathology, and 3) a blood pressure decrease after adrenalectomy. Evidence of excess autonomous aldosterone secretion without such criteria led to a diagnosis of idiopathic hyperaldosteronism (IHA). RESULTS: A total of 1,180 patients (age 46 +/- 12 years) were enrolled; a conclusive diagnosis was attained in 1,125 (95.3%). Of these, 54 (4.8%) had an APA and 72 (6.4%) had an IHA. There were more APA (62.5%) and fewer IHA cases (37.5%) at centers where AVS was available (p = 0.002); the opposite occurred where AVS was unavailable. CONCLUSIONS: In newly diagnosed hypertensive patients referred to hypertension centers, the prevalence of APA is high (4.8%). The availability of AVS is essential for an accurate identification of the adrenocortical pathologies underlying PA.     

New diagnostic procedure for primary aldosteronism: adrenal venous sampling under adrenocorticotropic hormone and angiotensin II receptor blocker--application to a case of bilateral multiple adrenal microadenomas.

Formerly, the incidence of primary aldosteronism (PA) among patients with hypertension was believed to be less than 1%. However, recent studies have suggested a much higher incidence of 6.59%-14.4% among such patients. These findings suggest that many cases of PA caused by small aldosterone-producing adenoma (APA) or idiopathic hyperaldosteronism (IHA) have not been properly diagnosed. To make a more accurate diagnosis in such cases, we developed a new diagnostic procedure for localization of PA, namely, adrenal venous sampling under continuous infusion of adrenocorticotropic hormone (ACTH) and administration of angiotensin II receptor blocker (AVS with ACTH and ARB). Here, we confirm the efficacy of this procedure in the case of a 37-year-old male suspected of having PA. The anticipated diagnosis of PA was based on the presence of hypokalemia, low plasma renin activity (PRA), elevated plasma aldosterone concentration (PAC) and left adrenal mass. However, AVS with ACTH and ARB revealed the presence of bilateral multiple adrenal microadenomas. In the new AVS method, neither ACTH nor the renin-angiotensin system (RAS) exert any influence on the plasma aldosterone level, and a more accurate aldosterone secretary state and a more accurate assessment of the aldosterone secretion of both adrenal glands can be recognized than by conventional AVS. Use of this new method should enable identification of additional cases of APA among patients diagnosed with essential hypertension.     

Diagnostic accuracy of adrenal venous sampling in comparison with other parameters in primary aldosteronism

This retrospective study was aimed 1) to compare the difference of the findings between adrenal CT scan and adrenal venous sampling (AVS) in 35 cases with definite primary aldosteronism (PA) for assessment of the diagnostic efficacy of PA subgroup (unilateral and bilateral adrenal hypersecretion: UAH and BAH), and 2) to determine the clinical and biochemical parameters as potential predictors for PA subgroup. There were significant discordant results based on AVS and CT scan in subgrouping PA; 9 of 17 BAH patients (53%) had unilateral lesion on CT scan, while 4 of 18 UAH patients (22%) had no apparent or bilateral lesions on CT scan. Among three diagnostic criteria, absolute values of plasma aldosterone concentration (PAC) in both adrenal veins, lateralized and contralateral ratios of aldosterone/cortisol after ACTH stimulation during AVS to determine the laterality, none of them showed 100% diagnostic accuracy if applied alone. Among several clinical and biochemical parameters, hypokalemia (<3.4 mEq/l), younger age (<52 y) and poor response of PAC (<1.45) after furosemide-upright posture, proved to be significant predictors for UAH, with higher specificities (100%, 88%, 94%, respectively). Therefore, despite AVS as a gold standard method to determine the laterality of aldosterone hypersecretion in PA, our study shows that no single criterion could provide definite diagnostic value for its laterality by AVS. It is also suggested that most PA patients, if not all, with a distinct unilateral adrenal lesion on CT accompanied by hypokalemia, younger age and poor aldosterone response to renin stimulation, could undergo adrenalectomy without prior AVS.     

原发性醛固酮增多症

原发性醛固酮增多症(原醛症)是继发性高血压的常见病因之一,部分患者亦可伴有低血钾,醛固酮瘤及特发性醛固酮增多症是其主要的病理亚型。原醛症的诊断包括筛查、确诊及分型诊断3个步骤,传统影像学结合体位刺激的方法进行分型诊断,假阳性及假阴性率均较高,肾上腺静脉插管采血可作为影像学检查的补充。醛固酮瘤及原发性肾上腺增生患者应予手术治疗,特发性醛固酮增多症患者多采用药物治疗,螺内酯是其首选药物。     

Clinical characteristics of aldosterone-producing microadenoma, macroadenoma, and idiopathic hyperaldosteronism in 93 patients with primary aldosteronism.

Primary aldosteronism (PA) due to aldosterone-producing adenoma (APA) is a form of surgically curable secondary hypertension, and distinguishing APA from idiopathic hyperaldosteronism (IHA) is important for treatment. We made a differential diagnosis between APA and IHA using imaging tests such as adrenal CT and MRI as well as adrenal venous sampling (AVS) in all 93 cases of PA presenting at our institutions over the last decade. We identified 27 patients with aldosterone-producing microadenoma (APmicroA), all of whom could be diagnosed by AVS but not by the imaging tests. Then, we compared the clinical and roent-genological findings of these 27 patients with those of 42 patients with aldosterone-producing macroadenoma (APmacroA) and of 24 patients with IHA. Using surgically removed adrenal tissues, histopathological examinations and immunohistochemical analyses of steroidogenic enzymes were conducted. The findings for APmicroA were similar to those for APmacroA, except with respect to the diameter of the adrenal adenomas. Endocrinological and roentgenological findings for APmicroA were similar to those for IHA, but not to those for APmacroA. The rate of cure of hypertension was much greater in patients with APmicroA than in patients with APmacroA after the unilateral adrenalectomy (odds ratio, 4.0; p=0.028). In conclusion, it is important to accurately diagnose APmicroA, in which the laterality of the hyperproduction of aldosterone is only detectable by AVS, and to treat these patients by unilateral adrenalectomy in order to avoid long-term medical treatment and prevent hypertensive vascular complications.     

Hormones Other Than Aldosterone May Contribute to Hypertension in 3 Different Subtypes of Primary Aldosteronism

Blood pressure (BP) level is similar in patients with 3 subtypes of primary aldosteronism (PA), even though aldosterone levels may vary. Glucocorticoids and adrenomedullary hormones may be influenced and may contribute to hypertension in PA. The authors' objective was to investigate the influence of PA on adrenal gland secretion and the roles of these hormones in hypertension. Patients diagnosed with PA (229 cases) were enrolled and classified into 3 subgroups: aldosterone‐producing adenoma (APA), unilateral nodular adrenal hyperplasia (UNAH), and idiopathic hyperaldosteronism (IHA). Patients with essential hypertension served as the control group (100 cases). Concentration of the above hormones was measured and compared between groups. Level of plasma adrenocorticotrophic hormone (ACTH) in patients with APA was significantly lower than that in patients with IHA (P<.001) and UNAH (P<0.5). The 24‐hour urinary free cortisol and adrenomedullary hormone levels were highest in patients with IHA, lower in patients with APA, and lowest in patients with UNAH. Systolic BP level was positively correlated with 8 am plasma cortisol level (r=0.142, P=.039) and plasma ACTH level (r=0.383, P=.016). Cortisol and adrenomedullary hormones were different between PA subtypes and they might involve regulation of BP in those patients.     

Localization of aldosterone-producing adrenocortical adenomas: significance of adrenal venous sampling.

Accurate localization of aldosterone-producing adenoma (APA) is essential for the treatment of primary aldosteronism (PA). In order to confirm the clinical usefulness of adrenal venous sampling (AVS), we retrospectively studied 87 cases of PA in whom AVS was conducted. We collected right and left adrenal venous effluents simultaneously before and after adrenocorticotropic hormone (ACTH) stimulation for measurements of aldosterone concentration (A) and cortisol concentration (C). Based on AVS results, we judged 66 cases as having unilateral aldosterone hypersecretion and the remaining 21 cases as having no apparent laterality. Of the above 66 subjects, 61 underwent laparoscopic removal of the adrenal gland through a retroperitoneal approach. The presence of APA was histopathologically confirmed, and blood pressure decreased significantly with normalization of plasma aldosterone concentration (PAC) in all cases. The receiver operator characteristics (ROC) curve analysis between the operated and no-apparent-laterality groups revealed that the ratio of A/C on the higher side to A/C on the lower side (A/C ratio) after ACTH stimulation is a useful index, with a cutoff value of 2.6, a sensitivity of 0.98 and a specificity of 1.0. The ROC curve analysis between the APA side and contralateral side within the operated patients revealed that the cutoff value of A was 1,340 ng/dL, with a sensitivity of 0.92 and a specificity of 1.00. Our results indicate the usefulness of simultaneous AVS and ACTH stimulation for localizing APA.