primary aldosteronism

原发性醛固酮增多症
  • 文章类型: Journal Article
    背景:单侧心血管并发症的发生率可能高于双侧原发性醛固酮增多症(PA)。我们比较了双侧和单侧PA靶向治疗前后的非侵入性血流动力学。
    方法:进行肾上腺静脉取样,使用桡动脉脉搏波分析和全身阻抗心动图记录心血管变量(n=114).在40例患者中(肾上腺切除术n=20,基于螺内酯的治疗n=20),在PA靶向治疗33个月后再次进行血流动力学记录.
    结果:在初始横截面分析中,51例患者为双侧PA,63例患者为单侧PA。平均年龄分别为50.6和54.3岁(p=0.081),体重指数分别为30.3和30.6kg/m2(p=0.724),分别。两组之间的主动脉血压和心输出量没有显着差异,但单侧PA评估的左心功高出约10%(p=0.022)。在后续研究中,主动脉的初始和最终血压水平没有显着差异,而初始心输出量(+13%,p=0.015)和左心脏功(+17%,p=0.009)单侧PA高于双侧PA。中位治疗时间为33个月后,心脏负荷的差异被消除,和细胞外水容量减少了1.3和1.4升的双边与单边PA,分别(p=0.814)。
    结论:这些结果表明,单侧PA比双侧PA对心脏的负担更大,为单侧疾病的心脏并发症发生率较高提供了可能的解释。用PA的靶向手术和药物治疗获得醛固酮诱导的体积过量的类似减少。
    BACKGROUND: The incidence of cardiovascular complications may be higher in unilateral than in bilateral primary aldosteronism (PA). We compared non-invasive hemodynamics before and after targeted therapy of bilateral versus unilateral PA.
    METHODS: Adrenal vein sampling was performed, and cardiovascular variables were recorded using radial artery pulse wave analysis and whole-body impedance cardiography (n=114). In a subset of 40 patients (adrenalectomy n=20, spironolactone-based treatment n=20), hemodynamic recordings were again performed after 33 months of targeted PA treatment.
    RESULTS: In initial cross-sectional analysis, 51 patients had bilateral and 63 had unilateral PA. The mean ages were 50.6 and 54.3 years (p=0.081), and body mass indexes were 30.3 and 30.6 kg/m2 (p=0.724), respectively. Aortic blood pressure and cardiac output did not significantly differ between the groups, but evaluated left cardiac work was ~10% higher in unilateral PA (p=0.022). In the followup study, initial and final blood pressure levels in the aorta were not significantly different, while initial cardiac output (+13%, p=0.015) and left cardiac work (+17%, p=0.009) were higher in unilateral than in bilateral PA. After a median treatment time of 33 months, the differences in cardiac load were abolished, and extracellular water volume was reduced by 1.3 and 1.4 liters in bilateral versus unilateral PA, respectively (p=0.814).
    CONCLUSIONS: These results suggest that unilateral PA burdens the heart more than bilateral PA, providing a possible explanation for the higher incidence of cardiac complications in unilateral disease. A similar reduction in aldosterone-induced volume excess was obtained with targeted surgical and medical treatment of PA.
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  • 文章类型: Journal Article
    背景:原发性醛固酮增多症(PA)的诊断是全面的,其中包括案例检测测试,病例确认,然后进行亚型分类。在某些情况下,例如在自发性低钾血症的背景下,抑制肾素活性(PRA)加上血浆醛固酮浓度(PAC)>15ng/dL,一个人可能不会继续进行验证性测试。然而,这种方法背后的证据质量非常低。本研究旨在通过评估各种预先指定的PAC阈值的诊断性能,结合肾素抑制和自发性低钾血症的发现,评估拟议的“简化的确认途径”,可以节省原发性醛固酮增多症的确认测试。
    方法:这是一个多中心,回顾性诊断准确性队列选择横断面研究.在2010年1月至2024年3月期间,共有133名18岁及以上的参与者接受了盐水输注测试。结果测量包括不同指数测试组合的诊断性能(基线PAC,基线PRA和自发性低钾血症的存在):敏感性,特异性,负预测值,正预测值,正似然比,负似然比,和诊断的准确性。使用SPSS29.0.1.0&MedCalc20.218进行数据分析。
    结果:在133名接受生理盐水输注试验的患者中,88例(66.17%)被诊断为PA。>25ng/dL的PAC加上PRA<1.0ng/dL/hr伴自发性低钾血症的特异性最高,为100%(95%CI90.51%,100.00%),阳性预测值为100%(85.18-100.00%)。最小可接受组合标准被确定为PAC>20ng/dL加上PRA<0.6ng/dL/hr,和自发性低钾血症的存在。它具有很高的特异性(94.59%;95%CI81.81%,99.34%),阳性预测值(93.55%,95%CI78.49%,98.29%),和中度阳性似然比(LR)(6.39,95%CI1.61,25.38)结论:自发性低钾血症的高血压患者,筛查发现PAC>20ng/dL,PRA抑制<0.6ng/ml/hr,可能被归类为“明确的原发性醛固酮增多症确认”,并且可能不需要进行动态确认测试。
    SRCTN34186253。
    BACKGROUND: The diagnosis of primary aldosteronism (PA) is comprehensive, which includes case-detection testing, case confirmation followed by subtype classification. In certain instances, such as in the setting of spontaneous hypokalemia, suppressed renin activity (PRA) plus plasma aldosterone concentration (PAC) of > 15 ng/dL, one may not proceed with confirmatory tests. However, the quality of evidence behind this approach is very low. This study sought to evaluate the proposed \"simplified confirmatory pathway\" that can spare confirmatory testing for primary aldosteronism by evaluating the diagnostic performances of the various pre-specified PAC thresholds in combination with findings of suppressed renin and spontaneous hypokalemia.
    METHODS: This is a multi-center, retrospective diagnostic accuracy cohort-selected cross-sectional study. A total of 133 participants aged 18 years and above underwent saline infusion test between January 2010 to March 2024. The outcome measures comprise of the diagnostic performances of the different index test combinations (baseline PAC, baseline PRA and presence of spontaneous hypokalemia): sensitivity, specificity, negative predictive value, positive predictive value, positive likelihood ratio, negative likelihood ratio, and diagnostic accuracy. Data analysis was performed using SPSS 29.0.1.0 & MedCalc 20.218.
    RESULTS: Of the 133 patients who underwent saline infusion test, 88 (66.17%) were diagnosed with PA. A PAC of > 25 ng/dL plus PRA < 1.0 ng/dL/hr with spontaneous hypokalemia showed the highest specificity at 100% (95% CI 90.51%, 100.00%) and positive predictive value at 100% (85.18 - 100.00%). The minimum acceptable combination criteria were determined to be a PAC of > 20 ng/dL plus PRA < 0.6 ng/dL/hr, and presence of spontaneous hypokalemia. It has high specificity (94.59%; 95% CI 81.81%, 99.34%), positive predictive value (93.55%, 95% CI 78.49%, 98.29%), and moderate positive likelihood ratio (LR+) (6.39, 95% CI 1.61, 25.38) CONCLUSION: A hypertensive patient with spontaneous hypokalemia and screening findings of PAC > 20 ng/dL and suppressed PRA of < 0.6 ng/ml/hr, may be classified as \"overt primary aldosteronism confirmed\" and may not need to proceed with dynamic confirmatory testing.
    UNASSIGNED: SRCTN34186253.
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  • 文章类型: Journal Article
    背景:原发性醛固酮增多症(PA)的亚型诊断用于确定治疗方法,68Ga-pentixa用于PET/CT研究PA的潜在用途早已得到认可。本研究旨在评估68Ga-pentixaforPET/CT在CT诊断双侧病变患者中的临床价值。
    方法:总共,对25例PA和双侧病变的CT进行了回顾性评估。所有患者均接受68Ga-PentixaPET/CT和肾上腺静脉采样。分析重点是建立双侧肾上腺病变SUVmax与双侧肾上腺病变SUVmax比值(CON)与临床诊断的关系,治疗结果,和KCNJ5基因状态。
    结果:68Ga-PentixaPET/CT与肾上腺静脉采样的符合率为65.2%(15/23)。68Ga-pentixaforPET/CT的侧化结果支持了20例PA患者的临床决策,其中90%在治疗中显示出有效性。手术治疗的患者的优势侧的SUVmax高于用药物治疗的患者。KCNJ5突变组的SUVmax高于KCNJ5野生组,68Ga-Pentixafor摄取与KCNJ5基因状态相关。
    结论:68Ga-PentixaforPET/CT证明对CT双侧病变的PA患者有益。基于PET侧向化的结果,治疗通常是有效的。同时,68Ga-PentixaforPET/CT与KCNJ5基因状态存在一定的关系,值得进一步分析。
    BACKGROUND: Subtype diagnosis of primary aldosteronism (PA) is used to determine treatment, and the potential utility of 68Ga-pentixafor PET/CT for investigation of PA has long been recognized. The study aimed to evaluate the clinical value of 68Ga-pentixafor PET/CT in the diagnosis and prognosis of patients with bilateral lesions identified by CT.
    METHODS: In total, 25 patients with PA and bilateral lesions on CT were retrospectively evaluated. All patients underwent 68Ga-Pentixafor PET/CT and adrenal vein sampling. The analysis focused on establishing the relationship between bilateral adrenal lesions SUVmax and the ratio of bilateral adrenal lesions SUVmax (CON) and clinical diagnosis, treatment outcomes, and KCNJ5 gene status.
    RESULTS: The concordance rate between 68Ga-Pentixafor PET/CT and adrenal venous sampling was 65.2% (15/23). The lateralization results of 68Ga-pentixafor PET/CT supported the clinical decisions of 20 patients with PA, 90% of whom showed effectiveness in treatment. The SUVmax on the dominant side of the surgically treated patients was higher than that of patients treated with drugs. The SUVmax of the KCNJ5 mutant group was higher than that of the KCNJ5 wild group, and 68Ga-Pentixafor uptake was correlated with KCNJ5 gene status.
    CONCLUSIONS: 68Ga-Pentixafor PET/CT proves beneficial for patients with PA with bilateral lesions on CT. The treatment is generally effective based on the results of PET lateralization. Simultaneously, a certain relationship exists between 68Ga-Pentixafor PET/CT and KCNJ5 gene status, warranting further analysis.
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  • 文章类型: Journal Article
    高血压导致多种合并症和死亡风险增加。内分泌失调有助于高血压的发展,包括原发性醛固酮增多症(PA)。本文讨论了PA的评估和管理。
    Hypertension leads to multiple comorbidities and increased risk for mortality. Endocrine disorders contribute to the development of hypertension, including primary aldosteronism (PA). This article discusses the evaluation and management of PA.
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  • 文章类型: Journal Article
    目的:评估临床和影像学特征是否可以预测激素亚型,增长,和肾上腺切除术治疗偶发性肾上腺皮质腺瘤(ACA)。
    方法:单中心队列研究。
    方法:2000年至2016年连续诊断为偶发ACA的成年患者。
    结果:在1516例附带ACA患者中(中位年龄59岁,62%的女性),699(46%)患有无功能腺瘤(NFA),482例(31%)有轻度自主皮质醇分泌(MACS),62(4%)患有原发性醛固酮增多症(PA),39人(3%)患有库欣综合征(CS),18(1%)患有PA和MACS(PA-MACS),和226(15%)有不完整的检查。年龄,性别,肿瘤大小,和肿瘤侧向性,但不是未增强的计算机断层扫描Hounsfield单位(HU),与荷尔蒙亚型有关。在多变量分析中,≥1cm的增长与年龄较小相关(每5年增长的比值比,OR=0.8,P=0.0047)和更长的影像学随访(OR=1.2/年,P<.0001)。355例(23%)患者进行了肾上腺切除术,包括38%的MACS和15%的NFA。NFA和MACS的肾上腺切除术在年轻患者中更为常见(OR=0.79/5年增加,P=0.002),较大的初始肿瘤大小(OR=2.3每增加1厘米,P<.0001),生长≥1cm(OR=15.3,P<0.0001),和较高的后地塞米松皮质醇(OR=6.6>5vs<1.8mcg/dL,P=0.002)。
    结论:年龄,性别,肿瘤大小,和侧向性与ACA激素亚型相关,可指导诊断和治疗.随着年龄的增长和随访时间的延长,肿瘤的生长更为常见。未增强的HU不能预测激素亚型或生长。MACS和NFA的肾上腺切除术主要在肿瘤较大的年轻患者中进行,增长,地塞米松后皮质醇升高。
    OBJECTIVE: The aim of this study is to assess whether clinical and imaging characteristics are associated with the hormonal subtype, growth, and adrenalectomy for incidental adrenal cortical adenomas (ACAs).
    METHODS: This is a single-center cohort study.
    METHODS: Consecutive adult patients with incidental ACA were diagnosed between 2000 and 2016.
    RESULTS: Of the 1516 patients with incidental ACA (median age 59 years, 62% women), 699 (46%) had nonfunctioning adenomas (NFAs), 482 (31%) had mild autonomous cortisol secretion (MACS), 62 (4%) had primary aldosteronism (PA), 39 (3%) had Cushing syndrome, 18 (1%) had PA and MACS, and 226 (15%) had incomplete work-up. Age, sex, tumor size, and tumor laterality, but not unenhanced computed tomography Hounsfield units (HU), were associated with hormonal subtypes. In a multivariable analysis, ≥1 cm growth was associated with younger age (odds ratio [OR] = 0.8 per 5-year increase, P = .0047) and longer imaging follow-up (OR = 1.2 per year, P < .0001). Adrenalectomy was performed in 355 (23%) patients, including 38% of MACS and 15% of NFA. Adrenalectomy for NFA and MACS was more common in younger patients (OR = 0.79 per 5-year increase, P = .002), larger initial tumor size (OR = 2.3 per 1 cm increase, P < .0001), ≥1 cm growth (OR = 15.3, P < .0001), and higher postdexamethasone cortisol (OR = 6.6 for >5 vs <1.8 μg/dL, P = .002).
    CONCLUSIONS: Age, sex, tumor size, and laterality were associated with ACA hormonal subtype and can guide diagnosis and management. Tumor growth was more common with younger age and longer follow-up. Unenhanced HU did not predict hormonal subtype or growth. Adrenalectomy for MACS and NFA was mainly performed in younger patients with larger tumor size, growth, and elevated postdexamethasone cortisol.
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  • 文章类型: Journal Article
    背景:调查阻塞性睡眠呼吸暂停(OSA)和原发性醛固酮增多症(PA)的共同发生对于理解它们的相互关系至关重要。
    目的:这项工作旨在评估诊断为PA的个体中OSA的患病率,并评估OSA人群中PA的患病率。特别关注高血压个体。
    方法:在PubMed中进行了详尽的搜索,Embase,CINAHL,Scopus,和WebofScience至2023年9月,没有语言或出版日期的限制。根据他们对PA患者OSA患病率的关注来选择研究,反之亦然。特别是在高血压个体中。使用标准指南提取数据,关注患者特征,患病率,和其他相关临床参数。
    结果:使用随机效应模型的比例荟萃分析显示,OSA在高血压PA患者中的患病率为59.8%,其中45.4%表现为中度至重度OSA。Meta回归显示年龄没有显著影响,性别,身体质量指数,抗高血压药物,收缩压,舒张压,或血清钾对OSA患病率的影响。然而,与肾小球滤过率(GFR)呈显著正相关(P<.001).亚组分析还显示,超滤率(GFR≥100mL/min/1.73m2)可能与OSA的患病率更高(71%,相互作用的P值<.01)。在高血压OSA患者中,11.2%有PA。
    结论:确定了PA患者中OSA的大量患病率,证明了高血压患者这些疾病之间复杂的相互作用。值得注意的是,OSA的患病率与肾脏高滤过率显著相关.
    BACKGROUND: Investigating the co-occurrence of obstructive sleep apnea (OSA) and primary aldosteronism (PA) is crucial for understanding their interrelation.
    OBJECTIVE: This work aimed to evaluate the prevalence of OSA in individuals diagnosed with PA and to assess the prevalence of PA within the OSA population, with a specific focus on hypertensive individuals.
    METHODS: An exhaustive search was performed across PubMed, Embase, CINAHL, Scopus, and Web of Science up to September 2023, without restrictions on language or publication date. Studies were selected based on their focus on the prevalence of OSA in PA patients and vice versa, specifically in hypertensive individuals. Data were extracted using standard guidelines, focusing on patient characteristics, prevalence rates, and other relevant clinical parameters.
    RESULTS: Proportional meta-analysis using a random-effects model revealed a 59.8% prevalence of OSA in hypertensive PA patients, with 45.4% exhibiting moderate-to-severe OSA. Meta-regression showed no significant effect of age, sex, body mass index, antihypertensive medication, systolic blood pressure, diastolic blood pressure, or serum potassium on OSA prevalence. However, a significant positive association was found with the glomerular filtration rate (GFR) (P < .001). Subgroup analysis also revealed that a hyperfiltration rate (GFR ≥ 100 mL/min per 1.73 m2) may be associated with a higher prevalence of OSA (71%, P value for interaction < .01). Among hypertensive OSA patients, 11.2% had PA.
    CONCLUSIONS: A substantial prevalence of OSA in individuals with PA was identified, demonstrating a complex interplay between these conditions in hypertensive patients. Notably, the prevalence of OSA was significantly associated with kidney hyperfiltration.
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  • 文章类型: Journal Article
    由于肾上腺静脉解剖结构的变化,在肾上腺静脉采样(AVS)期间,肾上腺静脉的准确插管具有挑战性。本研究旨在探讨国人肾上腺静脉形态特征,提高AVS的成功率。共纳入221名接受AVS的原发性醛固酮增多症(PA)患者。比较按性别划分的亚组的形态,体重指数(BMI),有或没有腺瘤。正确的成功率,左,双侧AVS为98.60%,97.20%,和96.85%,分别。右侧最常见的是三角形图案(39.37%),而左侧为腺样模式(70.14%)。不同患者肾上腺静脉形态的比例不同(χ2=21.335,P<.001),BMI(χ2=10.642P=0.031),右侧有或没有腺瘤(χ2=10.637,P=0.031),而男性,肥胖和腺瘤组显示腺样模式的比例高于三角形模式。如果仅仅依靠计算机断层扫描,9.05%的患者错误地诊断优势侧,14.48%的患者会进行不适当的手术,而25.34%的患者会错过手术机会。总之,左右肾上腺静脉形态最常见的类型是三角形和腺样,分别。性,BMI,腺瘤的存在影响右肾上腺静脉形态。对肾上腺静脉形态的充分了解对于提高AVS的成功率和对PA进行适当的治疗至关重要。
    Accurate cannulation of the adrenal vein is challenging during adrenal venous sampling (AVS) because of the variations in adrenal vein anatomy. This study aimed to investigate the adrenal venous morphology in Chinese and improve the success rate of AVS. A total of 221 participants with primary aldosteronism (PA) who underwent AVS were enrolled. Compare the morphology among subgroups divided according to sex, body mass index (BMI), and with or without adenoma. The success rate of right, left, and bilateral AVS was 98.60%, 97.20%, and 96.85%, respectively. The triangular pattern was the most common (39.37%) on the right side, while the glandlike pattern (70.14%) on the left. The proportion of adrenal venous morphology varies among patients with different sexecs (χ2 = 21.335, P < .001), BMI (χ2 = 10.642 P = .031), and with or without adenoma (χ2 = 10.637, P = .031) on the right side, and the male, obese and adenoma group showed a higher proportion of glandlike pattern than triangular pattern. If only dependent on computed tomography, 9.05% of patients incorrectly diagnose the dominant side, 14.48% of patients would have inappropriate surgery meanwhile 25.34% of patients would miss the surgical opportunity. In conclusion, the most common types of right and left adrenal venous morphology were triangular pattern and glandlike pattern, respectively. Sex, BMI, and the presence of adenoma affected right adrenal venous morphology. Adequate knowledge of the adrenal venous morphology is critical for improving the success rate of AVS and making an appropriate treatment for PA.
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  • 文章类型: Journal Article
    目的:对于原发性醛固酮增多症(PA)的亚型,建议进行肾上腺静脉采样(AVS)。然而,在PA的情况下,并发亚临床库欣综合征(SCS)有可能混淆AVS结果。Pentixafor,CXC趋化因子受体4型特异性配体,已被报道为评估肾上腺腺瘤功能性质的有希望的标志物。本研究旨在探讨正电子发射断层扫描-计算机断层扫描(68Ga-PentixaforPET/CT)在PA加SCS患者定位诊断中的临床价值。
    方法:两名确诊为PA加SCS的患者接受了AVS和68Ga-PentixaforPET/CT。
    结果:AVS结果显示两名患者均无侧化,而68Ga-PentixaforPET/CT显示单侧肾上腺结节,对68Ga-Pentixafor的摄取增加。根据68Ga-PentixaforPET/CT的结果进行单侧肾上腺切除术。随后,在两种情况下,自主醛固酮和皮质醇分泌均实现了完全的生化缓解。
    结论:68Ga-PentixaforPET/CT在PA加SCS患者中显示出潜在的醛固酮和皮质醇共分泌肾上腺腺瘤的定位。
    OBJECTIVE: Adrenal venous sampling (AVS) is recommended for subtyping primary aldosteronism (PA). However, in cases of PA, concurrent subclinical Cushing\'s syndrome (SCS) has the potential to confound AVS results. Pentixafor, a CXC chemokine receptor type 4-specific ligand, has been reported as a promising marker to evaluate functional nature of adrenal adenomas. This study aims to investigate the clinical value of Gallium-68 Pentixafor Positron Emission Tomography-Computed Tomography (68Ga-Pentixafor PET/CT) in the localization diagnosis of patients with PA plus SCS.
    METHODS: Two patients with a confirmed diagnosis of PA plus SCS underwent AVS and 68Ga-Pentixafor PET/CT.
    RESULTS: AVS results revealed no lateralization for both patients while 68Ga-Pentixafor PET/CT showed a unilateral adrenal nodule with increased uptake of 68Ga-Pentixafor. Unilateral adrenalectomy was performed based on the results of 68Ga-Pentixafor PET/CT. Subsequently, complete biochemical remission of autonomous aldosterone and cortisol secretion were achieved in both cases.
    CONCLUSIONS: 68Ga-Pentixafor PET/CT shows promising potential for the localization of aldosterone and cortisol co-secreting adrenal adenoma in patients with PA plus SCS.
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