原发性醛固酮增多症诊治分析

目的 探讨原发性醛固酮增多症的诊断及治疗.方法 回顾性分析68例原发性醛固酮增多症患者的临床资料.结果 全部患者均有不同程度的低血钾,卧、立位血浆醛固酮平均值分别为(682±332)pmol/L、(881±406)pmol/L,29例患者尿醛固酮值升高,其中48例立位的醛固酮/肾素活性比值≥50.通过影像学检查62例为原发性醛固酮增多症腺瘤型,其余6例为双侧肾上腺增生;32例予以手术治疗,24例予以口服安体舒通治疗,经治疗后56例患者血压及血钾均有不同程度的恢复.结论 原发性醛固酮增多症主要通过影像学检查定位,血、尿醛固酮测定定性诊断.肾上腺腺瘤切除仍是当前的首选治疗方法,安体舒通也是重要的治疗手段.     

右肾上腺静脉经导管逆行超声造影1例

患者女, 发现右肾上腺占位3个月入院。患者8年前常规测量发现血压升高(150+/80+mmHg)(1 mmHg=0.133 kPa), 诊断为"高血压病", 2年前开始出现口干多饮多尿、夜尿增多症状。3个月前因肌肉无力急诊就诊, 血钾1.78 mmol/L, 醛固酮肾素比值(aldosterone-renin ratio, ARR) 36.12。肾上腺CT:右侧肾上腺外侧支旁偏囊性结节灶, 肾上腺来源可能性大, 腺瘤考虑, 左侧肾上腺增生(图1～3), 诊断为"原发性醛固酮增多症"。     

原发性醛固酮增多症28例诊治分析

目的：提高原发性醛固酮增多症（PA）的诊断和治疗方法。方法：对我院1996/2007年手术治疗的PA患者进行回顾性分析和总结。结果：28例患者均有高血压病史2-26 a,其中仅64.3%生化检查有低钾血症,余病例血钾正常,血清肾素（PRA）均低于正常值,血清醛固酮（ALD）82.1%高于正常值,A RR（ALD/PRA）≥50,CT对于肾上腺腺瘤诊断率较高为85.7%,术后血压及血钾完全恢复正常者为53.6%。结论：PA的定性诊断须行血钾,醛固酮,肾素水平及醛固酮和肾素比值（ARR）检查,CT为原发性醛固酮增多症的主要定位诊断依据。外科手术是原发性醛固酮增多症的重要治疗方法。     

醛固酮水平正常的原发性醛固酮增多症的诊治

原发性醛固酮增多症（primary akdisteribusnmm,PA）是继发性高血压最常见的原因之一,也是原发于肾上腺的常见疾病之一.绝大多数PA有典型的临床表现,即高血压、低血钾、低肾素活性的醛固酮升高.如病变原发于肾上腺,影像学检查可发现肾上腺腺瘤或肾上腺增生.但临床工作中发现少数PA 患者血浆醛固酮水平并不高,如果患者没有其他定性诊断依据和（或）定位诊断依据,将可能影响PA 的正确诊断和选择适当的手术方案.     

原发性醛固酮增多症患者临床特点分析与研究

目的研究原发性醛固酮增多症患者临床表现特点,并对相关特点进行比较与分析。方法 58例确诊原发性醛固酮增多症患者,按照血钾水平分组,正常组16例为正常血钾;低钾组42例为低血钾或存在低血钾病史。比较与分析两组临床生化检测指标和合并症及肾上腺CT检查等。结果两组患者年龄、血压、病程比较差异无统计学意义(P0.05);低钾组男性患者比率、肌酐(Cr)水平均大于正常组,差异有统计学意义(P0.05)。两组患者肾素活性(PRA)、餐后2 h血糖、肾小球滤过率(GFR)、空腹血糖和LDL-C水平比较差异无统计学意义(P0.05);低钾组立位血醛固酮、联合降压药物使用量、24 h尿蛋白、尿K+、三酰甘油和24 h尿醛固酮高于正常组,血K+水平均低于正常组(P0.05)。两组患者心脑血管和糖脂代谢疾病发生率及颈动脉内膜厚度(IMT)、左心室质量指数(LVMI)、颈动脉粥样斑块、左心室肥厚发生率比较差异无统计学意义(P0.05)。经肾上腺CT检查,低钾组腺瘤发生率高于正常组,皮质增生发生率低于正常组(P0.05)。结论原发性醛固酮增多症男性患者易发生低血钾,低血钾原发性醛固酮增多症患者较正常血钾原发性醛固酮增多症患者血压、血脂、血糖均升高,而低血钾患者主要以肾上腺瘤样为主,正常血钾原发性醛固酮增多症患者以肾上腺皮质增生为主,低血钾原发性醛固酮增多症患者并不增加心脑血管疾病发病率。     

特发性醛固酮增多症的诊治进展

原发性醛固酮增多症（primary aldosteronism,PA）是指肾上腺腺瘤、肾上腺皮质增生等多种病理改变造成的高血压伴有低钾血症、低血浆肾素活性（plasma rennin activity,PRA）、     

CT对原发醛固酮增多症亚型的诊断价值

目的探讨CT在原发醛固酮增多症亚型醛固酮瘤和肾上腺增生鉴别诊断中的价值。方法回顾性分析94例肾上腺肿瘤患者的CT影像资料,与手术组织病理进行对照,分析术前CT诊断醛固酮瘤及肾上腺增生的准确率,比较CT诊断直径≥1.5cm与直径〈1.5cm醛固酮瘤的准确率。结果手术组织病理诊断原发醛固酮增多症79例,其中肾上腺醛固酮瘤62例,肾上腺皮质增生17例;CT术前诊断醛固酮瘤的准确率为85.5%（53/62）,诊断肾上腺增生的准确率为64.7%（11/17）,二者比较差异有统计学意义（P〈0.05）;CT诊断直径≥1.5cm的醛固酮瘤的准确率为92.0%（46/50）,诊断直径〈1.5cm醛固酮瘤的准确率为58.3%（7/12）,二者比较差异有统计学意义（P〈0.05）。结论 CT术前诊断醛固酮瘤的准确率高于肾上腺增生,但对直径〈1.5cm的醛固酮瘤术前正确诊断率较低。     

原发性醛固酮增多症合并亚临床型皮质醇增多症临床特点及其发病机制探讨

目的探讨原发性醛固酮增多症合并亚临床型皮质醇增多症（即醛固酮/皮质醇共分泌瘤）的临床特点及其可能的发病机制,提高醛固酮/皮质醇共分泌瘤的诊治水平。方法回顾分析13例临床诊断为原发性醛固酮增多症（其中单纯性醛固酮腺瘤8例,合并亚临床型皮质醇增多症5例）的临床资料。通过免疫组化及RT-PCR检测腺瘤及瘤旁组织类固醇合成限速酶编码基因（CYP11B1和CYP11B2）的表达情况。结果醛固酮/皮质醇共分泌瘤的最大直径[（3.48±1.51）cm]大于单纯醛固酮腺瘤[（1.61±0.36）cm],差异具有统计学意义（t=-3.412,P=0.006）;醛固酮/皮质醇共分泌瘤患者血钾水平[（4.00±0.29）mmol/L]高于单纯醛固酮腺瘤[（3.15±0.72）mmol/L],差异具有统计学意义（t=-2.460,P=0.032）。结论原发性醛固酮增多症合并亚临床型皮质醇增多症并不少见,其具有独特的临床特点。     

原发性醛固酮增多症的临床特点

目的了解原发性醛固酮增多症(原醛症)的常见症状和早期症状,比较病理为肾上腺皮质腺瘤和增生的原醛症的临床表现的不同.方法回顾性调查原醛症患者的临床资料,选择标准为血浆醛固酮水平增高和手术病理证实为肾上腺皮质腺瘤或皮质增生的患者共90例.结果原醛症患者临床常见症状依次为高血压(100%)、口渴多尿(26.6%)、四肢乏力(23.3%)、四肢麻木(15.5%)、四肢抽搐(4.4%);早期表现为高血压,高血压至确诊时病程为(5±7)年,最高血压为(191±25)/(116±15) mm Hg,平均血压为(154±16)/(97±10) mm Hg,左心室肥厚的频率为43.5%;94.4%的患者存在低血钾;低肾素活性血症卧位发生率70.3%,立位发生率为94.3%;血浆醛固酮/血浆肾素活性比值以25/1为截取点,立位及卧位的阳性率均为72.4%,以50/1为截取点,敏感性明显下降;腺瘤组平均血压明显高于增生组[(157±17)/(99±10) mm Hg )vs(148±13)/(92±10) mm Hg];低钾血症发生率在腺瘤组更易发生(98.5% vs 84.0%);腺瘤组血浆醛固酮水平亦较增生组明显增高(P<0.05).结论原醛症的患者临床上常见症状依次为高血压、口渴多尿、四肢乏力、四肢麻木、四肢抽搐等,早期表现为高血压,且往往是在高血压多年后才因出现低血钾而确诊,部分患者可无低血钾;高血压的特点为重度高血压,药物对其有一定效果,但不能达标,易发生高血压的并发症;低肾素血症和血浆醛固酮/血浆肾素活性的比值对诊断原醛症有一定意义,但也会漏掉一部分患者;在临床表现和实验室检查上肾上腺皮质腺瘤严重程度高于肾上腺皮质增生的患者.     

肾上腺醛固酮瘤切除术后，为何患者血压仍高？

原发性醛固酮增多症（简称为“原醛”）的原因主要为肾上腺醛固酮瘤及双侧肾上腺特发性增生，临床研究表明1／2以上的原醛病例为醛固酮瘤患者。理论上醛固酮瘤经手术切除治疗后血压应恢复正常，事实上有约30％～40％的患者醛固酮瘤切除术后仍血压持续升高。原醛醛固酮瘤切除术后1个月是临床观察血压能否正常的关键时间点，与血钾、血醛固酮相比，血压恢复所需时间较长，其原因为醛固酮瘤切除术后，机体的肾素-血管紧张素-醛固酮系统需要一定的调节时间（一般需1～6个月）才能恢复正常。     

经手术病理证实的原发性醛固酮增多症102例临床分析

目的在经手术病理证实的原发性醛固酮增多症(PA)患者中评价术前各项诊断筛查方法的临床价值。方法回顾性分析经手术病理证实的102例PA患者的临床特征、生化资料及血浆醛固酮浓度(PAC)、血浆醛固酮与肾素比值(ARR),比较不同指标筛查PA的阳性率,不同试验对PA的确诊率及影像学检查对PA的检出率和诊断符合率。结果在上述患者中:(1)ARR>20 ng/dl·(ng·ml-1·h-1)-1筛查PA的阳性率为86.27%;ARR>20 ng/dl·(ng·ml-1·h-1)-1且PAC>15 ng/dl筛查PA的阳性率为68.63%(P<0.01)。(2)静脉盐水抑制试验和卡托普利抑制试验对PA的确诊率分别为100%和80%(P>0.05)。(3)影像学检查中,CT和MRI对PA的检出率明显高于超声(P<0.01);与术后病理对照,CT对醛固酮腺瘤(APA)和单侧肾上腺皮质增生(UAH)的诊断符合率分别为97.33%和78.26%(P<0.01),MRI对APA和UAH诊断符合率分别为88.89%和75.00%(P>0.05)。结论 ARR>20 ng/dl·(ng·ml-1·h-1)-1筛查PA的阳性率高于ARR>20 ng/dl·(ng·ml-1·h-1)-1联合PAC>15 ng/dl;盐水抑制试验和卡托普利抑制试验都有较高的确诊率;影像学检查中,CT对PA有较高的检出率和诊断符合率,对于分型而言,CT对APA的诊断符合率高于UAH。     

肾球旁细胞瘤6例临床分析及文献复习

目的探讨肾球旁细胞瘤的临床特点,提高对该病的诊断和治疗水平。方法回顾性分析6例肾球旁细胞瘤患者临床资料并复习相关文献。男5例,女1例,初诊年龄[26.2±5.7（18～37）]岁,就诊时高血压6例,血浆肾素活性[基础（6479.6±2349.3）ng·L-1·h-1,激发（8446.3±1926.4）ng·L-1·h-1]、血管紧张素Ⅱ[基础（114.8±34.2）pg/ml,激发（297.3±87.1）pg/ml]及血浆醛固酮[基础（0.35±0.04）nmol/L,激发（0.63±0.18）nmol/L]水平增高6例,低血钾4例。除1例拒行CT检查外,5例由动态增强CT定位。结果保守治疗1例,根治性患肾切除1例,保留肾单位的肾肿瘤切除4例,病理组织学及免疫组化证实肾球旁细胞瘤的诊断。术后随访4～160个月,血压、血钾、肾素、醛固酮均在正常范围内,未见肿瘤复发和转移。结论肾球旁细胞瘤具有高血压、高肾素血症、高醛固酮血症、低血钾的临床特点,动态CT增强扫描有助于定位,免疫组化染色CD34、肌动蛋白（Actin）阳性,细胞角蛋白（CK）阴性是其特征,保留肾单位的肿瘤切除术是有效的治疗方法。     

Primary aldosteronism in childhood due to primary adrenal hyperplasia

We present an unusual case of primary aldosteronism in childhood. A 9-year-old boy had hypertension, hypokalemia, hyporeninemia and hyperaldosteronism. Dexamethasone administration decreased plasma aldosterone transiently but failed to correct the hyperaldosteronism, excluding dexamethasone-suppressible hyperaldosteronism. Plasma aldosterone decreased with upright posture and showed a circadian rhythm. Spironolactone treatment normalized blood pressure and serum potassium and lowered aldosterone secretion. During the studies, plasma aldosterone correlated with serum cortisol but not with plasma renin. Preoperative results indicated that this patient presented the functional features of aldosteronoma. Adrenal computed tomography, scintigraphy and left venography were not diagnostic of adrenal lesions. The left adrenal venous sampling showed hypersecretion of aldosterone from the left adrenal gland. The left adrenalectomy revealed micronodular hyperplasia but resulted in a prompt and sustained reversal of hypertension and hyperaldosteronism. These findings suggest that primary aldosteronism in this patient resulted from primary adrenal hyperplasia. Thus, adrenal hyperplasia is a heterogenous group of disorders and carefully selected studies allow prospective selection of appropriate treatment.     

Activation of aldosterone secretion in primary aldosteronism

Angiotensin infusion evokes marked increases in aldosterone secretion in primary aldosteronism and little change in secondary aldosteronism. The low plasma renin activity of primary aldosteronism and the elevated plasma renin activity of secondary aldosteronism are thought to account for this differential response. The effect of angiotensin on aldosterone and 18-hydroxycorticosterone secretion was studied during adrenal vein catheterization in seven patients with primary aldosteronism (whose plasma renin activity had been elevated following spironolactone therapy), one hypertensive patient with normal plasma renin activity and normal aldosterone secretion, two patients with secondary aldosteronism who had elevated plasma renin activity, and one anephric patient whose plasma renin activity was 0. Adrenal venous aldosterone and 18-hydroxycorticosterone were measured before and after a ten min sub-pressor angiotensin infusion.The cells of the aldosterone-producing adenoma (APA) respond to small increases in plasma angiotensin with large increases in secretion of aldosterone and 18-hydroxycorticosterone. The dose of angiotensin capable of evoking this response from the aldosterone-producing adenoma produces little or no change in the secretion of the steroids from nontumorous glands. The augmentation of aldosterone secretion, induced by angiotensin, in primary aldosteronism is due solely to increased secretion by the adenoma and not by the contralateral zona glomerulosa. The increased sensitivity of the aldosterone-producing adenoma is characteristic of the tumor. This response is independent of fluctuations in endogenous plasma renin activity. This sensitivity is not blunted by high plasma renin activity, nor is it a function of tumor mass for the effect is observed in aldosterone-producing adenomas regardless of size. ACTH injection after angiotensin infusion resulted in a marked increase in aldosterone concentration in the effluent from the nontumorous adrenal, but was not capable of producing further increases in aldosterone concentration in the effluent from the APA. In view of this exquisite sensitivity to infused angiotensin, it may be that the small variations in endogenous plasma renin activity that have been observed in primary aldosteronism may be capable of evoking large changes in aldosterone secretion in patients with aldosterone-producing adenomas.     

Primary aldosteronism

Primary aldosteronism is a potentially curable form of hypertension. Recent studies using the plasma aldosterone to plasma renin activity ratio as screening test in hypertensive populations have demonstrated a high prevalence of primary aldosteronism close to 10%. This frequency is clearly higher than the classically described when hypokalemia is used as the screening method. The most common subtypes of primary aldosteronism are idiopathic aldosteronism and aldosterone-producing adenoma. Other causes are glucocorticoid-remediable aldosteronism, unilateral or primary adrenal hyperplasia and adrenal carcinoma. The diagnosis of primary aldosteronism is advocated to confirm the autonomy of aldosterone secretion from the renin-angiotensin system and to differentiate the clinical subtypes of the disease. This article reviews the new data about prevalence, diagnosis criteria and describes the clinical, biochemical and genetic characteristics of the different subtypes of the disease. We also discuss the treatment, and the differential diagnosis with other hyper-mineralocorticoid states.     

维生素D_3对自发性高血压大鼠肾素-血管紧张素-醛固酮系统的影响

目的观察维生素D3对自发性高血压大鼠血压及肾素一血管紧张素一醛固酮系统的影响。方法自发性高血压大鼠20只分为2组各10只,实验组给予维生素D3注射液3μg／（kg·d）溶于100pL丙二醇中腹腔注射,连续注射7d;对照组给予单纯丙二醇100μL腹腔注射7d。药物干预完成后观察2组大鼠血压变化。分别于1,3周后分批处死大鼠,收集血清和组织标本。ELISA法检测血清中25（OH）D3、血钙、甲状旁腺激素、肾素、血管紧张素Ⅱ和醛固酮水平。结果药物干预结束后1周,实验组大鼠收缩压较对照组低14mmHg（P〈0．01）,25（0H）D3（156．00±28．50ng／mL）、血钙水平（59．00±7．67ng／mL）均高于对照组（（2．28±0．17）mmol／L）和（（2．06±0．12）mmol／L）（P〈0．05）,肾素、血管紧张素Ⅱ、甲状旁腺激素水平均低于对照组（P〈0．05）;3周后2组大鼠血压和上述指标比较差异均无统计学意义（P〉0．05）。结论维生素D3可抑制。肾素的合成和分泌,抑制肾素-血管紧张素-醛固酮系统,从而降低血压。     

Activity of [des-Aspartyl1]-Angiotensin II in Primary Aldosteronism

This study describes the effects of [des-Aspartyl(1)]-angiotensin II ([des-Asp]-AII) on blood pressure and aldosterone production in patients with primary aldosteronism due to aldosterone-producing adrenal adenoma (APA) and idiopathic adrenal hyperplasia (IHA), and in normotensive control subjects. 10 patients with primary aldosteronism, 7 with APA and 3 with IHA, and 6 normotensive control subjects were placed on a constant 150-meq sodium diet for 4 days. [des-Asp]-AII was infused for 30 min at 6, 12, and 18 pmol/kg per min. Three groups of patients were identified on the basis of aldosterone response to [des-Asp]-AII. Group I, composed of normotensive control subjects, showed incremental increases in plasma aldosterone concentration from 6+/-1 to 14+/-3 ng/100 ml (P < 0.01) with [des-Asp]-AII infusion. Group II, composed of patients with primary aldosteronism, showed incremental increases in plasma aldosterone concentration from 33+/-8 to 65+/-13 ng/100 ml (P < 0.05) with 12 pmol/kg per min of [des-Asp]-AII. Group III, also composed of patients with primary aldosteronism, showed no increase of plasma aldosterone concentration with [des-Asp]-AII. Groups I and II showed similar percentage increases in plasma aldosterone concentration (P = NS). Group III showed significantly lower aldosterone responses than group I (P < 0.01). Group II included all patients with IHA and two patients with APA. Group III included only patients with APA. The blood pressure responses to [des-Asp]-AII of subjects in group I did not differ significantly from those of groups II or III.Thus, patients with IHA and a subgroup of patients with APA showed responsiveness to [des-Asp]-AII which was limited to adrenal cortical stimulation of aldosterone biosynthesis. This suggests that adrenal responsiveness to angiotensin is a major control mechanism in some forms of primary aldosteronism. The differential adrenal responsiveness to [des-Asp]-AII in patients with APA indicates either that there are two distinct subpopulations of APA, or that alteration in tumor response to angiotensin occurs during the natural progression of the disease history.     

高血压鉴别诊断性实验的研究分析

目的 探讨生理盐水滴注抑制试验、卡托普利抑制试验诊断原发性醛固酮增多症的临床意义及护理措施。方法 收集2012年1～7月在我科治疗的56例高血压患者的临床资料,行生理盐水滴注抑制试验和卡托普利抑制试验诊断高血压原因,比较确诊病例与疑似病例的血浆醛固酮、肾素、醛固酮/肾素活性比值。结果 10例原发性高血压,46例继发性高血压,其中确诊原发性醛固酮增多症18例,疑似病例28例,两组的性别、年龄、收缩压及舒张压比较差异无显著意义（P〉0.05）,具有可比性。两组血浆醛固酮的平均值分别为30.8ng/L、12.9ng/L,肾素0.26ng/（ml·h）、0.54ng/（ml·h）,醛固酮/肾素活性比值150.1、23.2,差异有显著意义（P〈0.001）。结论 生理盐水滴注抑制试验和卡托普利抑制试验是筛选、确诊原发性醛固酮增多症的主要手段,护理是准确地进行试验的关键,试验前仔细准备,试验中严格用药、病情观察、准确采集标本,试验后观察及记录检查结果非常重要。