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。     

特发性醛固酮增多症手术是否必要?

分析39例经手术和病理检查证实的特发性醛固酮增多症(IHA)的临床资料,提出了IHA手术指征:即药物治疗不能控制,或药物剂量过大、副作用严重者;临床上增生结节与腺瘤不能区别者。手术方法是一侧肾上腺全切除。     

单侧性原发性醛固酮增多症的预测模型建立及价值评估

原发性醛固酮增多症(PA)是继发性高血压常见的病因之一, 其病因分型诊断的金标准为肾上腺静脉采血(AVS)。本文回顾性分析了2018年7月至2021年8月在南京鼓楼医院内分泌科诊断为PA并行AVS或单侧肾上腺切除手术患者的数据, 通过多元logistic回归分析确定了与单侧醛固酮优势分泌相关的因素, 并基于这些因素建立了诊断单侧原发性醛固酮增多症(UPA)的预测模型:年龄<40岁, 血浆醛固酮浓度(PAC)>15 ng/dL, 肾上腺CT示单侧典型腺瘤, 自发性低钾血症。该预测模型可使14%的PA患者避免非必要的AVS。     

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.     

应重视从高血压人群中鉴别原发性醛固酮增多症

原发性醛固酮增多症(原醛症)是一组醛固酮分泌增多、肾素-血管紧张素系统受抑制但不受钠负荷调节的疾病,是临床可控制或可治愈的继发性高血压.因肾上腺皮质增生或肿瘤而自主分泌过多醛固酮,可产生心、脑、肾血管损害,高血压,钠潴留、排钾增多而导致低钾血症.原醛症常见原因是肾上腺腺瘤、单侧或双侧肾上腺增生,少见原因为遗传缺陷所导致的糖皮质激素可调节的醛固酮增多症.     

原发性肾上腺增生:原发性醛固酮增多症的一种新亚型

原醛病人中,肾上腺腺瘤与特发性醛固酮增多症(IHA)的临床鉴别很重要,因手术对前者有益,对后者常无效。作者报告一例原发性肾上腺增生(Primary adrenal hyperplasia,PAH),在形态上PAH类似IHA,但生化指标及其对手术的反应酷似腺瘤。     

原发性醛固酮增多症与心血管疾病相关性研究的最新进展

正原发性醛固酮增多症(PA)是继发性高血压的常见因素,发病率在高血压人群中约占11%,较原发性高血压(EH)更易发生心脑血管病,对PA的早期诊断十分重要~([1])。双侧肾上腺增生(BAH)是临床最常见的PA类型,占PA的65%;而单侧醛固酮增多常见于醛固酮腺瘤(APA)和原发性单侧肾上腺增生,分别占PA的30%和3%~([2])。综合近年发表的最新研究,探讨PA对心血管系统的影响和临床意义。     

影响中国原发性醛固酮增多症诊断的关键问题与应对策略

<正>原发性醛固酮增多症(primary aldosteronism,PA)是由肾上腺皮质分泌过量的醛固酮而导致肾素受抑制,临床上以高血压伴(或不伴)低血钾、高醛固酮和低肾素血症为主要表现的临床综合征。其主要病因包括:醛固酮瘤、双侧肾上腺皮质增生(特发性醛固酮增多症)、单侧肾上腺皮质增生、家族性PA等。目前,国内外指南或共识推荐的诊断流程包括:筛查、确诊和分型"三部曲"~([1-2])。明确诊断为单侧病变的PA可以通过手术根治,而不宜手术的PA患者     

警惕漏诊原发性醛固酮增多症

原醛是一个综合征，表现为：高血压、低肾素、血浆与尿中醛固酮增高。最常见的是：①单侧肾上腺产生醛固酮腺瘤(ADA)；②双侧原发性醛固酮增多症(IHA)。过去诊断ADA占70％～80％，IHA只占20％～30％，由于生化检查的进步，现在IHA反而占多数。近年来，国际医学界对原醛的研究发展迅猛，有不少见解推翻了过去的观点。     

原发性醛固酮增多症患者血浆醛固酮水平的刺激因素

在原发性醛固酮增多症(PA)中,醛固酮的分泌相对独立于肾素-血管紧张素系统,但是否受到其他因素的刺激?一项前瞻性队列研究对此进行了研究。共入选了43例经肾上腺静脉穿刺确诊为PA的患者(n=39),其中11例为双侧肾上腺增生(BAH),28例为单侧醛固酮瘤(APA),4例病因不明。     

Unique cases of unilateral hyperaldosteronemia due to multiple adrenocortical micronodules, which can only be detected by selective adrenal venous sampling

Primary aldosteronism is classified as aldosterone-producing adenoma (APA), idiopathic hyperaldosteronism (IHA), unilateral adrenal hyperplasia (UAH), primary adrenal hyperplasia (PAH), adrenal cancer, and glucocorticoid-remediable aldosteronism. We describe here 4 cases of primary aldosteronism due to unilateral hyperaldosteronemia, demonstrating unique histopathologic findings, such as unilateral multiple adrenocortical micronodules in the affected adrenals. Thirty-three patients with primary aldosteronism were consecutively admitted; 27 of them were treated by unilateral adrenalectomy. Four of them also had unilateral adrenal hypersecretion of aldosterone by selective adrenal venous sampling and adrenocortical multiple micronodules without an adenoma. These patients had hyporeninemic hyperaldosteronism with normokalemic hypertension. In these patients, furosemide plus upright test failed to increase plasma renin activity (PRA); the ratio of plasma aldosterone concentration (PAC) to PRA at 90 minutes after captopril administration was similar to that in patients with IHA and APA. Aldosterone concentrations were increased in each unilateral adrenal vein, and poorly encapsulated multiple adrenocortical micronodules from 2 to 3 mm in diameter were microscopically detected in the resected adrenal glands. Immunohistochemical analysis of steroidogenic enzymes, including cholesterol side chain cleavage, 3beta-hydroxysteroid dehydrogenase, 21-hydroxylase, 17alpha-hydroxylase, and 11beta-hydroxylase, indicated that the cortical cells within these micronodules were active in aldosterone production, while the non-nodular zona glomerulosa cells were inactive. We conclude that the clinical and pathologic characteristics of our 4 cases with unilateral multiple adrenocortical micronodules (UMN) are distinct from those of APA, IHA, UAH, and PAH. Furthermore, unilateral hyperaldosteronemia induced by UMN may be frequently misdiagnosed, because standard imaging tests, which cannot always detect tiny abnormalities of adrenals, showed "normal adrenal glands" in these patients. Thus, primary aldosteronism due to UMN should be carefully examined for differential diagnosis of each form of hyperaldosteronemia.     

A case of primary aldosteronism due to unilateral adrenal hyperplasia.

The case of a patient with primary aldosteronism due to unilateral adrenal hyperplasia (UAH) is reported. A 43-year-old man with an 8-year history of hypertension presented at our institution with hypokalemia, increased plasma aldosterone concentration (PAC), and suppressed plasma renin activity (PRA). An abdominal CT scan showed almost normal adrenal glands with slight enlargement in the left gland. 131I-Norcholesterol adrenal scintigraphy under dexamethasone suppression showed bilaterally decreased uptake. To rule out idiopathic hyperaldosteronism, an adrenal vein sampling before and after ACTH stimulation was performed and a left-sided lateralization of PAC was observed. A left adrenalectomy was performed, and the patient had a good clinical and biochemical response. Micronodular hyperplasia was discovered in the adrenal gland histologically, and in the immunohistochemical analysis, positive staining for 3beta-hydroxysteroid dehydrogenase in micronodular lesions confirmed the diagnosis of UAH. Although UAH is a rare subset of primary aldosteronism, it is surgically correctable as a unilateral autonomous aldosterone-producing lesion. Careful investigations, including bilateral adrenal vein sampling, should be performed for the diagnosis.     

The Mineralocorticoid Hormone Pathways in Hypertension with Hyperaldosteronism

Simultaneous measurement of the 0800-hr plasma concentrations of deoxycorticosterone (DOC), corticosterone (B), 18-hydroxycortico-sterone (18-OHB), aldosterone, 18-hydroxydeoxycorticosterone (18-OHDOC) and cortisol (F) in four types of primary aldosteronism provides evidence for primary adrenal disease. Elevated DOC with normal F concentrations in the presence of elevated 18-OHB and aldosterone, and suppressed renin concentration suggests a primary adrenal abnormality of the zona glomerulosa (ZG). Steroid production by the zona fasciculata (ZF), F, 18-OHDOC, and most often B, is normal. These patterns exist only for primary adrenal hyperplasia, aldosterone-producing adenoma (APA), and aldosterone-producing adrenocortical carcinoma (AP-Ca). Elevated DOC levels are rarely found in patients with idiopathic hyperaldosteronism (IHA or adrenal hyperplasia) and suggest that IHA is not a primary adrenal disorder and should be excluded from the syndrome of primary aldosteronism as they have been heretofore.     

Primary aldosteronism due to unilateral adrenal hyperplasia: report of two cases and review of the literature

Unilateral adrenal hyperplasia (UAH) is a rare, surgically correctable subset of primary aldosteronism (PA), which shows similar endocrine features to aldosterone-producing adenoma (APA). We report here two Japanese patients with UAH. Case 1 was a 62-year-old man with a four-year history of hypertension. Hypokalemia and suppressed plasma renin activity (PRA) with elevated plasma aldosterone concentration (PAC) were observed, while no adrenal nodules were identified by abdominal computed tomographic (CT) scan. Adrenal scintigraphy did not reveal definite localization. The selective adrenal-vein sampling for determinations of PAC showed an over-functioning left adrenal gland, and a left adrenalectomy was performed. Diffuse micronodular adrenocortical hyperplasia was observed. Case 2 was a 61-year-old man with a six-year history of hypertension. At the first visit to our hospital, hypokalemia and suppressed PRA with elevated PAC were observed. An abdominal CT scan showed a left adrenal mass 1.5 cm in diameter, while adrenal scintigraphy did not reveal definite laterality. A left adrenalectomy was performed, and three macronodules and diffuse micronodular adrenocortical hyperplasia were observed. Hypokalemia, hypertension and endocrine data became normal, and both patients have been well with no signs of recurrence for eight years (case 1) and seven months (case 2) after surgery. Clinical characteristics and endocrine features of UAH are also reviewed.     

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.     

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。     

肾上腺静脉采血在原发性醛固酮增多症分型诊断中的价值

肾上腺静脉采血是近年来在我国兴起的一项新技术，本文通过不同方案，采用不同的参数及切点，分析其在原发性醛固酮增多症分型诊断中的敏感性和特异性，并与肾上腺计算机断层扫描（CT）等手段相比较，阐明其在分型诊断中的地位和价值。     

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。