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.     

肾上腺静脉采血在原发性醛固酮增多症分型诊断中的应用

目的探讨肾上腺静脉采血（AVS）检查在原发性醛固酮增多症（原醛症）分型诊断中的应用价值。方法收集瑞金医院近4年来39例临床确诊的原醛症患者[23例特发性醛固酮增多症（特醛症），16例醛固酮瘤]，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血液，测各点醛固酮和皮质醇水平，并将结果与影像学检查、体位激发试验（PST）及术后病理结果进行比较。结果（1）23例特醛症患者体位激发后血醛固酮较基础值均升高；16例醛固酮瘤患者血醛固酮升高者占56．3％（9／16）；（2）特醛症患者肾上腺B超检查符合率为69．6％（16／23），醛固酮瘤患者为56．3％（9／16）；肾上腺CT检查特醛症患者符合率为73．9％（17／23），醛固酮瘤患者为81．3％（13／16）；（3）AVS检查以两侧醛固酮之比作为判定标准时符合率为71．8％，以醛固酮与皮质醇之比为判定标准则达到100％。醛固酮瘤患者生化异常程度较特醛症患者明显。PST在特醛症及醛固酮瘤中有部分重叠；体位激发后血醛固酮升高者不能排除醛固酮瘤，而血醛固酮下降者可诊断为醛固酮瘤。结论单纯依赖影像学检查对于原醛症患者进行分型诊断易发生误诊。AVS检查的准确性高，对于影像学检查未能发现明显占位性病变者须进行该检查以明确诊断；对于AVS结果，用两侧醛固酮与皮质醇的比值之比分析较单纯比较两侧醛固酮之比更为可靠。     

原发性醛固酮增多症的影像学诊断

原发性醛同酮增多症(PA)是继发性高血压最常见原因,根据其临床和生化特点可确定PA的诊断。影像学检查对PA中醛固酮瘤和特发性肾上腺皮质增生的分型诊断有着重要的价值,其中CT对PA的定位诊断和鉴别诊断尤为重要。肾上腺静脉采血为有创性检查,但其是PA分型诊断最为可靠的方法。     

原发性醛固酮增多症外科治疗中的若干问题

原发性醛固酮增多症是继发性高血压中最常见的病因。目前腹腔镜手术己成为治疗原醛症的最佳手段,在醛固酮瘤的手术中多提倡保留肾上腺组织的手术。本文介绍了原醛症术后影响血压改善情况的因素,并就特醛症中适宜于手术治疗的患者情况作了阐述。     

原发性醛固酮增多症分型定侧诊断进展

原发性醛固酮增多症（原醛）临床分型不仅具有理论意义,更重要的是有助于指导治疗。目前所知原醛至少有７种类型。其中醛固酮腺瘤（ＡＰＡ）和特发性醛固酮增多症（ＩＨＡ）两型为多见。鉴别腺瘤和增生的方法中以双侧肾上腺静脉采样检查（ＡＶＳ）法最为可靠,但由于其本身具有局限性而难以广泛开展。ＣＴ应用最为广泛,对于单侧大结节腺瘤ＣＴ诊断的结论可靠,不必考虑ＡＶＳ检查;对于其它病例,特别是ＩＨＡ,ＣＴ检查的结论不可     

原发性醛固酮增多症分型定侧诊断进展

原发性醛固酮增多症 (原醛 )临床分型不仅具有理论意义 ,更重要的是有助于指导治疗。目前所知原醛至少有 7种类型 ,其中以醛固酮腺瘤 (APA)和特发性醛固酮增多症 (IHA)两型为多见。鉴别腺瘤和增生的方法中以双侧肾上腺静脉采样检查 (AVS)法最为可靠 ,但由于其本身具有局限性而难以广泛开展。CT应用最为广泛 ,对于单侧大结节腺瘤CT诊断的结论可靠 ,不必考虑AVS检查 ;对于其它病例 ,特别是IHA ,CT检查的结论不可靠 ,必需进行AVS检查以及早作出分型诊断和相应治疗。     

原发性醛固酮增多症的内分泌功能诊断

原发性醛固酮增多症(原醛症)是继发性高血压最常见的原因之一,以低肾素和高醛固酮血症为特征,血浆醛固酮/肾素比值(ARR)是筛查原醛症的可靠指标.而口服高钠负荷试验、生理盐水试验、氟氯可的松抑制试验或卡托普利试验中的任何一项均可作为ARR阳性患者的确诊试验;肾上腺静脉插管采血(AVS)是原醛症分型诊断的金标准.     

原发性醛固酮增多症

原发性醛固酮增多症(primary aldosteronism,简称原醛)是指由于肾上腺皮质自主性醛固酮分泌增多而导致以高血压、低血浆肾素活性(PRA)、高醛固酮血症和低钾血症为特征的临床综合征,是继发性高血压常见病因之一。原醛最常见的病因是特发性醛固酮增多症和肾上腺腺瘤,分别占65%和3     

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。     

血浆醛固酮与肾素比值阴性的原发性醛固酮增多症诊断（附1例分析）

目的 分析总结1例血浆醛固酮与肾素比值（ARR）阴性的原发性醛固酮增多症（PA）患者的诊断过程,提高临床对该病的认识。方法 回顾性分析1例ARR阴性的PA患者的临床资料,总结其诊疗过程。结果 患者男,44岁,阵发性头晕,出汗、乏力,发作时测血压高,曾应用多种降压药物治疗效果不佳。其舅舅和女儿均患特发性醛固酮增多症,患者查ARR阴性,卡托普利试验结果显示服药2 h后醛固酮浓度未被抑制,体位试验结果显示立位醛固酮浓度升高,肾上腺增强CT示右侧肾上腺外侧支结节,基因检测未见异常,PA诊断明确,考虑特发性醛固酮增多症可能性大。给予螺内酯联合硝苯地平控释片、琥珀酸美托洛尔缓释片降压治疗,患者血压控制可,头晕、出汗症状较前好转,出院继续联合降压治疗。结论 对于临床怀疑PA但ARR阴性的患者,应考虑有无其他因素致ARR假阴性,尤其是降压药物的影响,行确证试验排除或明确诊断。     

Detecting and treating primary aldosteronism: primary aldosteronism.

Primary aldosteronism (PA) is the most common cause of mineralocorticoid hypertension. Different studies, using the plasma aldosterone concentration to plasma renin activity ratio (PAC/PRA) for the screening of patients with hypertension, have shown a marked increase in the detection rate of PA. Idiopathic bilateral adrenal hyperplasia (IHA) and aldosterone-producing adrenal adenoma (APA), are the leading causes of primary aldosteronism. Glucocorticoid-remediable aldosteronism (GRA), also called familial hyperaldosteronism type I, familial hyperaldosteronism type II and carcinomas are rare causes of PA. Patients with hypertension and hypokalemia, those with a family history of hypertension and stroke at an early age, or patients with medication-resistant hypertension should be screened for PA using the PAC/PRA ratio. If a high ratio is found, a sodium loading test or a captopril test is warranted to confirm the diagnosis. Adrenal gland imaging is important in subtype differentiation (APA vs IHA). Adrenal venous sampling should be used when other tests prove inconclusive. Genetic testing has facilitated detection of GRA. Surgery is considered the treatment of choice for patients with APA, while bilateral hyperplasia subtypes are treated medically. Normalization of aldosterone levels or aldosterone receptor blockade are necessary to prevent the morbidity and mortality associated with hypertension, hypokalemia, and cardiovascular damage.     

Medical treatment as an alternative to adrenalectomy in patients with aldosterone-producing adenomas

Primary aldosteronism (PA) and, in particular, its two commonest subtypes (i.e. idiopathic hyperaldosteronism (IHA) and aldosterone-producing adenoma (APA)) have been recognized as the most common cause of secondary hypertension. While 'conservative' medical treatment with aldosterone receptor antagonists is the therapeutic approach of choice in controlling blood pressure in patients with PA due to IHA, the more invasive (laparoscopic) adrenalectomy seems to be the most suitable therapy for patients with APA. In this review, we focus on the medical approach for the management of APA in cases where surgical excision of the adrenal is not possible.     

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.     

The syndromes of low-renin hypertension: "separating the wheat from the chaff"

Primary aldosteronism (PA) is characterized by hypertension and suppressed renin activity with or without hypokalemia and comprises the aldosterone-producing adenoma (APA) and bilateral adrenal hyperplasia or idiopatic hyperaldosteronism (IHA). In recent series employing the aldosterone (aldo, ng/dL):renin (ng/mL.h) ratio (ARR) for screening, prevalence of PA among hypertensives soars to 8-20%; current predominance of IHA (>80%) over APA suggests the inclusion of former low-renin essential hypertensives (LREH), in whom plasma aldo can be reduced by suppressive maneuvers. We evaluated the test characteristics of the ARR obtained retrospectively from 127 patients with PA (81 APA; 46 IHA) and 55 with EH (30 LREH; 25 NREH) studied from 1975 to 1990. Using the combined ROC-defined cutoffs of 27 for the ARR and 12ng/dL for aldo, we obtained 89.8% sensitivity (Ss) and 98.2% specificity (Sp) in discriminating PA from EH: all APA and 72% of the IHA patients had values above these limits, but only one (3%) with LREH. Among the 46 IHA patients, 10 (21.7%) had ARR <27, four of whom with aldo <12ng/dL, virtually indistinguishable from LREH. Use of higher cutoff values (ARR > or =100; aldo > or =20) may attain 84%Ss and 82.6%Sp in separating APA from IHA. Because IHA and LREH ("the chaff") may be spectrum stages from the same disease, definite discrimination between these entities seems immaterial. However, precise identification of the APA ("the wheat") is critical, since it is the only surgically curable form of PA. Thus, while patients who may harbor an APA must be thoroughly investigated and surgically treated, non-tumoral disease (IHA and LREH) may be best treated with an aldo-receptor antagonist that will also prevent the aldo-mediated inflammatory effects involved in myocardial fibrosis and abnormal cardiac remodeling.     

The role of biochemical tests and clinical symptoms in differential diagnosis of primary aldosteronism

BACKGROUND: Primary aldosteronism (PA) is a secondary form of hypertension resulting from the autonomous hypersecretion of aldosterone. The recognition of PA has an important impact on clinical management, since the choice of therapy is different - surgical for adenoma and medical for hyperplasia. AIM: To evaluate patients with PA in regard to clinical and biochemical factors differentiating between adenoma of adrenal cortex (APA) and idiopathic adrenal hyperplasia (IHA). METHODS: We retrospectively analysed 62 patients with PA (33 females, 29 males, mean age 49.3+/-12.5 years, range 26-78) diagnosed in the Department of Hypertension between 1990-2001. In 37 patients (mean age 47.4+/-12.1 years, 22 females, 15 males) APA was diagnosed whereas in the remaining 26 patients (mean age 52.2+/-12.6 years, 14 males, 11 females) IHA was detected. Clinical manifestation, biochemical, serum aldosterone (SA), plasma renin activity (PRA) as well as echocardiographic parameters and blood pressure (BP) levels were evaluated. Diagnostic accuracy of computed tomography (CT) and scintigraphy was also assessed. RESULTS: Mean systolic BP was significantly higher in the patients with APA. Both groups had similar mean diastolic BP. Severe hypertension, resistant to three or more medications, was found in 63.3% of all patients. Muscle weakness was reported by 39.7% of patients, polyuria - by 19%, and polydypsia - by 10.3% of patients. Patients with muscle weakness had higher mean systolic BP level and lower plasma potassium level than patients without this complaint. Symptoms suggesting cardiac arrhythmia were reported by 45% of patients. A normal potassium level was found in 25.8% of all patients. The hypokalemic patients were younger, had shorter known duration of hypertension, higher mean systolic BP level and higher SA concentration than the normokalemic patients. Supine SA levels were significantly higher in the APA group than in the IHA group (50.3+/-29.0 ng% vs 30.5+/-14.7 ng%; p<0.001). The SA/PRA ratio higher than 30:1 was found in all patients. Response to postural test with a rise in SA concentration higher than 30% was observed in 40% patients with APA and in 87.5% patients with IHA (p<0.0001). There was a strong correlation between supine and upright SA level, and systolic and diastolic BP level as well as plasma potassium level. Left ventricular hypertrophy was present in 60% of patients. The differentiation between APA and IHA was possible using CT, scintigraphy or both methods in 75%, 89.2% and 100% of patients, respectively. CONCLUSIONS: One quarter of patients with PA were normokalemic. PA should be suspected especially in patients with severe hypertension, resistant to three or more antihypertensive drugs. Changes in SA concentration during the postural tests such as CT and scintigraphy are useful for differentiation between APA and IHA.     

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.     

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.     

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.