Conn’s syndrome

康恩综合征
  • 文章类型: Journal Article
    肾上腺诱发的高血压是由潜在的肾上腺疾病引起的,包括康恩综合征,库欣综合征,和嗜铬细胞瘤.这些肾上腺疾病是心血管和肾脏发病率和死亡率的风险。临床上,肾上腺诱发的高血压的治疗涉及药物或手术方法。前者表现出非常明显的副作用,而后者在其余对侧肾上腺中再次出现肾上腺疾病的情况下可能无效。由于现有治疗方法的局限性,微波消融(MWA)等微创治疗方案在肾上腺疾病的治疗中受到了广泛关注.对人类肾上腺介电特性的精确理解将有助于为MWA治疗定制能量输送,从而提供了优化治疗和减少对周围组织损伤的潜力。这项研究报告了人肾上腺的离体介电特性,包括大脑皮层,髓质,胶囊,和肿瘤,根据从四名患者获得的数据(诊断为康恩综合征,库欣综合征,和嗜铬细胞瘤)在戈尔韦大学医院接受了单侧肾上腺切除术,爱尔兰。使用开放式同轴探针测量技术来测量0.5-8.5GHz频率范围内的介电特性。使用两极德拜模型拟合介电特性,采用加权最小二乘法对模型参数进行优化。此外,肾上腺组织和肿瘤的介电特性在MWA常用的频率上进行了比较,包括915MHz,2.45GHz,5.8GHz。研究发现,肾上腺肿瘤的介电特性受到富含脂质的腺瘤的存在的影响,与诊断为Conn\'s综合征和嗜铬细胞瘤的患者相比,库欣综合征肿瘤的介电特性最低。此外,在诊断为康恩综合征的患者中,观察到髓质和皮质的介电特性存在显着差异,库欣综合征,和嗜铬细胞瘤.这些发现对肾上腺肿瘤的诊断和治疗具有重要意义。包括优化MWA治疗以精确消融肾上腺肿块。
    Adrenal gland-induced hypertension results from underlying adrenal gland disorders including Conn\'s syndrome, Cushing\'s syndrome, and Pheochromocytoma. These adrenal disorders are a risk for cardiovascular and renal morbidity and mortality. Clinically, treatment for adrenal gland-induced hypertension involves a pharmaceutical or surgical approach. The former presents very significant side effects whereas the latter can be ineffective in cases where the adrenal disorder reoccurs in the remaining contralateral adrenal gland. Due to the limitations of existing treatment methods, minimally invasive treatment options like microwave ablation (MWA) have received significant attention for treating adrenal gland disorders. A precise comprehension of the dielectric properties of human adrenal glands will help to tailor energy delivery for MWA therapy, thus offering the potential to optimise treatments and minimise damage to surrounding tissues. This study reports the ex vivo dielectric properties of human adrenal glands, including the cortex, medulla, capsule, and tumours, based on the data obtained from four patients (diagnosed with Conn\'s syndrome, Cushing\'s syndrome, and Pheochromocytoma) who underwent unilateral adrenalectomy at the University Hospital Galway, Ireland. An open-ended coaxial probe measurement technique was used to measure the dielectric properties for a frequency range of 0.5-8.5 GHz. The dielectric properties were fitted using a two-pole Debye model, and a weighted least squares method was employed to optimise the model parameters. Moreover, the dielectric properties of adrenal tissues and tumours were compared across frequencies commonly used in MWA, including 915 MHz, 2.45 GHz, and 5.8 GHz. The study found that the dielectric properties of adrenal tumours were influenced by the presence of lipid-rich adenomas, and the dielectric properties of Cushing\'s syndrome tumour were lowest in comparison to the tumours in patients diagnosed with Conn\'s syndrome and Pheochromocytoma. Furthermore, a notable difference was observed in the dielectric properties of the medulla and cortex among patients diagnosed with Conn\'s syndrome, Cushing\'s syndrome, and Pheochromocytoma. These findings have significant implications for the diagnosis and treatment of adrenal tumours, including the optimisation of MWA therapy for precise ablation of adrenal masses.
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  • 文章类型: Journal Article
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  • 文章类型: Case Reports
    原发性醛固酮增多症(PA)是继发性高血压的最常见形式,最常见于肾上腺腺瘤或双侧肾上腺增生。微创肾上腺切除术是单侧疾病的首选治疗方法。这里,我们报道了一例57岁的既往膀胱癌患者,他被转诊以评估顽固性高血压和低钾血症.诊断检查显示PA,计算机断层扫描成像显示左肾上腺腺瘤,肾上腺静脉采样偏侧至左肾上腺。因此,他被转诊为使用后腹腔镜方法进行左肾上腺切除术。然而,手术并发了与以前的膀胱癌免疫疗法相关的明显的肾炎,鉴于可识别的肾上腺腺瘤,进行了肾上腺部分切除术.尽管组织学证实切除了肾上腺腺瘤,他仍然是高血压和低血钾和持续性PA。他对残余的肾上腺结节进行了计算机断层扫描引导的经皮热(微波)消融,并立即对PA进行生化逆转。手术后六年,他仍然在生化和临床上从PA治愈。本文介绍了该病例的详细信息,并回顾了有关接受热消融和肾上腺切除术的患者的长期预后的文献。表明热消融可能是选定患者的可行替代方案。
    Primary aldosteronism (PA) is the most prevalent form of secondary hypertension and is most commonly caused by an adrenal adenoma or bilateral adrenal hyperplasia. Minimally invasive adrenalectomy is the treatment of choice for unilateral disease. Here, we report the case of a 57-year-old man with previous bladder cancer who was referred for evaluation of resistant hypertension and hypokalemia. Diagnostic workup indicated PA with computed tomography imaging revealing a left adrenal adenoma and adrenal venous sampling lateralizing to the left adrenal. He was therefore referred for a left adrenalectomy using a retroperitoneoscopic approach. However, surgery was complicated by significant perinephritis related to previous bladder cancer immunotherapy and, in view of an identifiable adrenal adenoma, a partial adrenalectomy was performed. Despite histology confirming removal of an adrenal adenoma, he remained hypertensive and hypokalemic with persistent PA. He underwent a computed tomography-guided percutaneous thermal (microwave) ablation of the residual adrenal nodule with immediate biochemical reversal of PA. Six years postprocedure, he remains biochemically and clinically cured from PA. This article presents the details of the case and reviews the literature on long-term outcomes for patients undergoing thermal ablation and adrenalectomy, suggesting that thermal ablation may be a viable alternative for selected patients.
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  • 文章类型: Editorial
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  • 文章类型: Journal Article
    背景:功能性肾上腺肿瘤(FAT)很少见,如果不及时治疗,由于不受控制的过量激素分泌,有相当大的发病和死亡风险.三种最常见的FAT是产生可的松的肿瘤(皮质醇增多症),产生醛固酮的肿瘤(醛固酮增多症),和产生儿茶酚胺的肿瘤(嗜铬细胞瘤)。该研究旨在评估腹腔镜肾上腺切除术后30天的人口统计学特征和结果。
    方法:从ACS-NSQIP数据库(2015-2017)中选择腹腔镜肾上腺切除术治疗FATs的患者,并分为三组(醛固酮增多症,皮质醇增多症,和嗜铬细胞瘤)。术前人口统计学,医疗合并症,和术后30天的结果三组使用卡方检验进行分析,方差分析(ANOVA)和Kruskal-Wallis单向方差分析。进行多变量逻辑回归以评估独立变量对总体发病率增加的可能性的影响。
    结果:在总共2410例接受腹腔镜肾上腺切除术的患者中,345例(14.3%)FATs患者被纳入。皮质醇增多症患者更年轻,女性比例较高,BMI较高,白人比例较高,糖尿病比例较高。醛固酮增多症组的黑人种族比例更高,需要药物治疗的高血压(HTN)比例更高。术后30天结果显示,嗜铬细胞瘤组的严重发病率较高,总体发病率,和最高的再入院率。有3人死亡,嗜铬细胞瘤1和皮质醇增多症2。皮质醇增多症组的手术时间(分钟)更长。皮质醇增多症(2天)和嗜铬细胞瘤(1.5天)组的中位住院时间较高。
    结论:功能性肾上腺肿瘤在患者的人口统计学和术后结局方面表现出明显的差异。在术前使用这些信息来优化患者,并就潜在的术后结果为患者提供咨询。
    Functional adrenal tumors (FATs) are rare and if left untreated, there is a substantial risk of morbidity and mortality due to uncontrolled excess hormone secretion. The three most common FATs are cortisone-producing tumors (hypercortisolism), aldosterone-producing tumors (hyperaldosteronism), and catecholamines-producing tumors (pheochromocytomas). The study aims to evaluate demographic characteristics and 30-day outcomes after laparoscopic adrenalectomy of FATs.
    Patients who underwent laparoscopic adrenalectomy for FATs were selected from the ACS-NSQIP database (2015-2017), and divided into three groups (hyperaldosteronism, hypercortisolism, and pheochromocytoma). Preoperative demographics, medical comorbidities, and 30-day postoperative outcomes among the three groups were analyzed using the chi-squared test, analysis of variance (ANOVA) and Kruskal-Wallis one-way analysis of variance. Multivariable logistic regression was performed to assess the effects independent variables on the likelihood of increased overall morbidity.
    Of a total of 2410 patients who underwent laparoscopic adrenalectomy, 345 (14.3%) patients with FATs were included. Patients in the hypercortisolism group were younger, had higher proportion of females, had higher BMI, had a higher proportion of White ethnicity and had a higher proportion of diabetes. The hyperaldosteronism group had a higher proportion of Black ethnicity and a higher proportion of hypertension (HTN) requiring medication. Thirty day postoperative outcomes showed that the pheochromocytoma group had a higher rate of serious morbidity, overall morbidity, and highest readmission rates. There were three deaths, 1 in the pheochromocytoma and 2 in the hypercortisolism groups. Operative time (in minutes) was longer in the hypercortisolism group. Median length of stay was higher in hypercortisolism (2 days) and pheochromocytoma (1.5 day) groups.
    Functional adrenal tumors show distinct variations in patient demographics and postoperative outcomes. It is essential to use this information during the preoperative period to optimize patients prior to intervention and counsel patients about potential postoperative outcomes.
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  • 文章类型: Editorial
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  • 文章类型: Journal Article
    背景:在某些指南中,建议将高血压合并阻塞性睡眠呼吸暂停(OSA)作为筛查原发性醛固酮增多症(PA)的指征,然而,先前的数据使这一建议的有效性受到质疑.鉴于这种情况,目前尚不清楚这项筛查建议是否正在临床实践中实施.
    方法:我们对2009-2020年所有患有高血压合并OSA的安大略省(加拿大)成年居民进行了一项基于人群的回顾性队列研究,并利用省级卫生行政数据进行了2021年的随访。我们测量了通过醛固酮与肾素比率进行PA筛查的个体的比例。我们进一步检查了合并低钾血症和顽固性高血压的高血压合并OSA患者的筛查率。通过Cox回归模型评估与筛查相关的临床预测因子。
    结果:研究队列包括53,130名患有高血压和OSA的成年人,其中只有634(1.2%)进行了PA筛查。在高血压患者中,OSA,和低钾血症,筛选合格患者的比例上升至2.8%.在≥65岁的高血压患者中,OSA,和处方≥4种降压药物,筛选合格患者的比例为1.8%.年龄较大与筛查的可能性降低相关,而低钾血症和内科亚专科护理,心脏病学,内分泌学,或肾脏病与筛查的可能性增加相关.没有发现筛查与性别有关,农村住宅,心血管疾病,糖尿病,或者呼吸专科护理.
    结论:筛查所有高血压合并OSA患者PA的指南建议的人群水平极低。
    Hypertension plus obstructive sleep apnea (OSA) is recommended in some guidelines as an indication to screen for primary aldosteronism (PA), yet prior data has brought the validity of this recommendation into question. Given this context, it remains unknown whether this screening recommendation is being implemented into clinical practice.
    We conducted a population-based retrospective cohort study of all adult Ontario (Canada) residents with hypertension plus OSA from 2009 to 2020 with follow-up through 2021 utilizing provincial health administrative data. We measured the proportion of individuals who underwent PA screening via the aldosterone-to-renin ratio by year. We further examined screening rates among patients with hypertension plus OSA by the presence of concurrent hypokalemia and resistant hypertension. Clinical predictors associated with screening were assessed via Cox regression modeling.
    The study cohort included 53,130 adults with both hypertension and OSA, of which only 634 (1.2%) underwent PA screening. Among patients with hypertension, OSA, and hypokalemia, the proportion of eligible patients screened increased to 2.8%. Among patients ≥65 years with hypertension, OSA, and prescription of ≥4 antihypertensive medications, the proportion of eligible patients screened was 1.8%. Older age was associated with a decreased likelihood of screening while hypokalemia and subspecialty care with internal medicine, cardiology, endocrinology, or nephrology were associated with an increased likelihood of screening. No associations with screening were identified with sex, rural residence, cardiovascular disease, diabetes, or respirology subspecialty care.
    The population-level uptake of the guideline recommendation to screen all patients with hypertension plus OSA for PA is exceedingly low.
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  • 文章类型: Journal Article
    未经评估:评估单侧原发性醛固酮增多症(UPA)肾上腺手术结果的几个预测评分,已经怀孕了。我们将总结UPA肾上腺手术结果的新型三联疗法与Vorselaars提出的临床治疗方法进行了比较。
    UNASSIGNED:在2011年3月至2022年1月之间,查询了多机构数据集UPA。基线,收集围手术期和功能数据。根据原发性醛固酮增多症手术结果(PASO)标准,对整个队列进行临床和生化完全和部分成功率评估。临床治愈的定义为不使用抗高血压药物的血压正常,或使用较低或同等程度的抗高血压药物的血压正常。Trifecta定义为≥50%的抗高血压治疗强度评分(TIS)降低(ΔTIS),3个月时无电解质损伤,无Clavien-Dindo(2-5)并发症。Cox回归分析用于确定长期临床和生化成功的预测因素。对于所有分析,双侧p<0.05被认为是显著的。
    未经评估:基线,分析围手术期和功能结局。在90名患者中,在中位随访42个月(IQR27-54)时,在60%和17.7%的病例中观察到完全和部分临床成功,而在83.3%和12.3%的病例中观察到完全和部分生化成功。分别。总体三联和临床治愈率分别为21.1%和58.9%,分别。关于多变量Cox回归分析,trifecta成就(HR2.87;95%CI1.45-5.58;p=0.02)是长期随访中临床完全成功的唯一独立预测因素。
    UASSIGNED:尽管其估计复杂且标准更严格,Trifecta而不是临床治愈允许从长远来看独立预测复合PASO终点。
    UNASSIGNED: Several predictive scores to evaluate outcomes of adrenal surgery for unilateral primary aldosteronism (UPA), have been conceived. We compared a novel trifecta that summarizes outcomes of adrenal surgery for UPA with the clinical cure proposed by Vorselaars.
    UNASSIGNED: Between March 2011 and January 2022, a multi-institutional dataset was queried for UPA. Baseline, perioperative and functional data were collected. Clinical and biochemical complete and partial success rates according to Primary Aldosteronism Surgical Outcome (PASO) criteria were assessed for the overall cohort. Clinical cure was defined either as normotension without antihypertensive medications or normotension with lower or equal use of antihypertensive medications. Trifecta was defined as the coexistence of ≥50% antihypertensive therapeutic intensity score (TIS) reduction (ΔTIS), no electrolyte impairment at 3-months and no Clavien-Dindo (2-5) complications. Cox regression analyses were used to identify predictors of long-term clinical and biochemical success. For all analyses, a two-sided p <0.05 was considered significant.
    UNASSIGNED: Baseline, perioperative and functional outcomes were analyzed. Out of 90 patients, at a median follow-up of 42 months (IQR 27-54) a complete and partial clinical success was observed in 60% and 17.7% of cases while a complete and partial biochemical success was achieved in 83.3% and 12.3% of cases, respectively. Overall trifecta and clinical cure rates were 21.1% and 58.9%, respectively. On multivariable Cox regression analysis, trifecta achievement (HR 2.87; 95% CI 1.45-5.58; p = 0.02) was the only independent predictor of complete clinical success at long-term follow-up.
    UNASSIGNED: Despite its complex estimation and more restrictive criteria, trifecta but not clinical cure allows to independently predict composite PASO endpoints on the long run.
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  • 文章类型: Journal Article
    背景:评估治疗强度评分(TIS)预测UPA的肾上腺部分切除术(PA)和全切除术(TA)的临床结果的能力。
    方法:在2011年至2022年之间,查询四中心肾上腺切除术数据集“单侧肾上腺肿块”和“UPA”(n=90)。术前每种降压药的TIS分别计算并合并,累积变量。完成临床的可能性,局部,通过Kaplan-Meier对整个队列进行评估,根据TIS不存在合并成功率.Cox分析用于确定完全临床和部分/缺失成功的预测因子,分别。对于所有分析,双侧p<0.05被认为是显著的。
    结果:在42个月(IQR27-54)的中位随访中,没有临床成功的观察到60%,17.7%,和22.3%,分别。关于Kaplan-Meier分析,TIS<1预测更高的完全成功率(p<0.001),而TIS≥1是部分和无临床成功的预测因子(p=0.008)。在多变量分析中,TIS<1(HR0.25;95%CI0.11-0.57;p=0.001)和腺瘤大小(HR1.11;95%CI1-1.23;p=0.0049)是临床完全成功的独立预测因子,而TIS≥1(HR2.84;95%CI1.32-6.1;p=0.007)是无临床成功的唯一独立预测因子。
    结论:TIS评分和腺瘤大小可能有助于识别手术后可能有持续性高血压风险的患者。
    BACKGROUND: To evaluate the ability of therapeutic intensity score (TIS) in predicting the clinical outcomes of partial (PA) and total adrenalectomy (TA) for UPA.
    METHODS: Between 2011 and 2022, a four-center adrenalectomy dataset was queried for \"unilateral adrenal mass\" and \"UPA\" (n = 90). Preoperative TIS of each antihypertensive medication were individually calculated and merged to create a single, cumulative variable. Probability of complete clinical, partial, and absent pooled success rates according to TIS were assessed for the overall cohort by Kaplan-Meier. Cox analyses were used to identify predictors of complete clinical and partial/absent success, respectively. For all analyses, a two-sided p < 0.05 was considered significant.
    RESULTS: At a median follow-up of 42 months (IQR 27-54) complete partial, and absent clinical success were observed in 60%, 17.7%, and 22.3%, respectively. On Kaplan-Meier analysis, TIS < 1 predicted higher complete success rates (p < 0.001), while TIS ≥ 1 was predictor of either partial and absent clinical success (p = 0.008). On multivariable analysis, TIS < 1 (HR 0.25; 95% CI 0.11-0.57; p = 0.001) and adenoma size (HR 1.11; 95% CI 1-1.23; p = 0.0049) were independent predictors of complete clinical success, while TIS ≥ 1 (HR 2.84; 95% CI 1.32-6.1; p = 0.007) was the only independent predictor of absent clinical success.
    CONCLUSIONS: TIS score and adenoma size may help to identify patients who are likely to be at risk of persistent hypertension after surgery.
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  • 文章类型: Case Reports
    Cervical radiculopathy is a common disease in everyday neurology clinical practice. However, a number of symptoms of cervical radiculopathy can occur in other diseases of nervous system and musculoskeletal apparatus. In particular, pain and weakness are observed in diseases of skeletal muscles and can be incorrectly interpreted as a damage of peripheral nerves, roots and plexuses. Careful collection of anamnesis and topical diagnosis make it possible not only to suspect the clinical development of myopathy, but also to exclude the pathology of other nervous structures. Additional methods of laboratory and instrumental investigation play an important role in etiology clarifying the myopathy causes and determining the further directions of patient management. A clinical case of progressive hypokalemic myopathy due to Conn\'s syndrome, initially diagnosed as «cervical radiculopathy», is presented. Surgical removal of the aldosterone-producing adenoma resulted in relief of symptoms and positive dynamics of laboratory parameters.
    Шейная радикулопатия является распространенным заболеванием в повседневной клинической практике врача-невролога. Однако ряд симптомов шейной радикулопатии может встречаться при патологии других структур нервной системы и мышечно-скелетного аппарата. В частности, боль и слабость наблюдаются при заболеваниях поперечно-полосатой мускулатуры и могут быть неверно интерпретированы как признаки поражения периферических нервов, корешков и сплетений. Тщательный сбор анамнеза и топическая диагностика позволяют не только заподозрить клиническое развитие миопатии, но и исключить патологию других структур нервной системы. Дополнительной лабораторно-инструментальное обследование играет важную роль в уточнении этиологии миопатии и определения дальнейшей тактики ведения пациента. Представлено клиническое наблюдение прогрессирующей гипокалиемической миопатии на фоне синдрома Конна, изначально диагностированной как «шейная радикулопатия». Хирургическое удаление альдостерон-продуцирующей аденомы позволило добиться купирования симптомов и положительной динамики лабораторных показателей.
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