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Primary AND Hyperaldosteronism AND adrenal AND adenoma

John W Funder
Primary aldosteronism (PA), currently recognized to be 5-10% of hypertension, has a cardiovascular risk profile double that in age-, sex-, and blood pressure-matched essential hypertensives. Screening for PA is by determining the plasma aldosterone to renin ratio (ARR), followed by one of half a dozen confirmatory/exclusion tests. Unilateral hyperaldosteronism normally reflects an aldosterone producing adenoma; bilateral disease is the more common form, and termed idiopathic hyperaldosteronism (IHA). Subjects confirmed undergo imaging, followed by adrenal venous sampling (AVS) for lateralization...
2019: Vitamins and Hormones
Toshihiro Kita, Eiji Furukoji, Tatefumi Sakae, Kazuo Kitamura
Adrenal venous sampling (AVS) is the gold standard test for distinguishing between unilateral and bilateral primary aldosteronism (PA); however, AVS requires advanced and time consuming technique. The needs for AVS have been increasing due to the increased utilization of screening for PA. An efficient selection of unilateral PA, such as aldosterone-producing adenoma (APA), before AVS is useful to avoid undesirable AVS in bilateral PA, such as idiopathic hyperaldosteronism. In this study, 40 patients who received all three confirmatory tests, including the captopril challenge test, furosemide upright test and adrenocorticotropin (ACTH) stimulation test (AST), and who were diagnosed as having PA by AVS were recruited...
January 8, 2019: Hypertension Research: Official Journal of the Japanese Society of Hypertension
K S Ahmed, D Bogdanet, S Abadi, R Dineen, G Boran, C P Woods, L A Behan, M Sherlock, J Gibney
INTRODUCTION: The aldosterone/ renin ratio is the initial screening test for primary hyperaldosteronism (PHA), but little data exists regarding ethnic variations in this. METHODS: Following clinical observation of a high prevalence of abnormal ARR in patients of African origin, we retrospectively reviewed all ARR measurements in a single centre over 10 years. Rates of hypokalaemia, intraventricular septal thickness (IVS, by echocardiography) and adrenal imaging were recorded when available...
December 21, 2018: Clinical Endocrinology
Natividad Pons Fernández, Francisca Moreno, Julia Morata, Ana Moriano, Sara León, Carmen De Mingo, Ángel Zuñiga, Fernando Calvo
Less than 15% of hypertension cases in children are secondary to a primary hyperaldosteronism. This is idiopathic in 60% of the cases, secondary to a unilateral adenoma in 30% and 10% remaining by primary adrenal hyperplasia, familial hyperaldosteronism, ectopic aldosterone production or adrenocortical carcinoma.To date, four types of familial hyperaldosteronism (FH I to FH IV) have been reported. FH III is caused by germline mutations in KCNJ5, encoding the potassium channel Kir3.4. The mutations cause the channel to lose its selectivity for potassium, allowing large quantities of sodium to enter the cell...
December 19, 2018: Reviews in Endocrine & Metabolic Disorders
Gian Paolo Rossi, Paolo Mulatero, Fumitoshi Satoh
: Nowadays most patients diagnosed with surgically curable primary aldosteronism have small or micro aldosterone-producing adenoma or unilateral micronodular hyperplasia, which are undetectable with available imaging technologies. Therefore, a negative imaging test by no means excludes unilateral primary aldosteronism. Moreover, about 10% of the subjects above the age of 35 years have nonfunctioning adrenal tumors, regardless of being hypertensive or not, with a prevalence that raises with aging. Hence, the finding of an adrenal mass at imaging does not reliably detect the culprit of primary aldosteronism...
November 13, 2018: Journal of Hypertension
Yui Shibayama, Norio Wada, Shuhei Baba, Yukie Miyano, Shinji Obara, Ren Iwasaki, Haruka Nakajima, Hidetsugu Sakai, Hiroaki Usubuchi, Satoshi Terae, Akinobu Nakamura, Tatsuya Atsumi
Context: The involvement of visceral fat in aldosterone secretion has not been reported in patients with primary aldosteronism (PA). Patients with PA are complicated by metabolic syndrome more frequently than those without PA. An excess of visceral fat has been hypothesized to cause an elevation of aldosterone secretion in patients with PA. Objectives: To clarify the role of visceral fat in the pathophysiology of PA, we investigated the correlation between plasma aldosterone concentration (PAC) and visceral fat parameters in patients with PA...
November 1, 2018: Journal of the Endocrine Society
Kei Omata, Fumitoshi Satoh, Ryo Morimoto, Sadayoshi Ito, Yuto Yamazaki, Yasuhiro Nakamura, Sharath K Anand, Zeng Guo, Michael Stowasser, Hironobu Sasano, Scott A Tomlins, William E Rainey
Primary aldosteronism affects ≈5% to 10% of hypertensive patients and has unilateral and bilateral forms. Most unilateral primary aldosteronism is caused by computed tomography-detectable aldosterone-producing adenomas, which express CYP11B2 (aldosterone synthase) and frequently harbor somatic mutations in aldosterone-regulating genes. The cause of the most common bilateral form of primary aldosteronism, idiopathic hyperaldosteronism (IHA), is believed to be diffuse hyperplasia of aldosterone-producing cells within the adrenal cortex...
October 2018: Hypertension
Christian Adolf, Anton Köhler, Anna Franke, Katharina Lang, Anna Riester, Anja Löw, Daniel A Heinrich, Martin Bidlingmaier, Marcus Treitl, Roland Ladurner, Felix Beuschlein, Wiebke Arlt, Martin Reincke
Context: Primary aldosteronism (PA) represents the most frequent form of endocrine hypertension. Hyperaldosteronism and hypercortisolism both induce excessive left ventricular hypertrophy (LVH) compared with matched essential hypertensives. In recent studies frequent cosecretion of cortisol and aldosterone has been reported in patients with PA. Objective: Our aim was to investigate the impact of cortisol cosecretion on LVH in patients with PA. We determined 24-hour excretion of mineralocorticoids and glucocorticoids by gas chromatography-mass spectrometry and assessed cardiac remodeling using echocardiography initially and 1 year after initiation of treatment of PA...
December 1, 2018: Journal of Clinical Endocrinology and Metabolism
Bora Kahramangil, Emin Kose, Eren Berber
BACKGROUND: A number of small studies have reported the use of indocyanine green imaging during adrenalectomy. Nevertheless, imaging properties of different tumors and the indications for indocyanine green imaging use in adrenalectomy have not been defined. METHODS: This is an Institutional Review Board-approved retrospective review of a prospectively maintained database. Consenting patients underwent indocyanine green imaging fluorescence-guided robotic adrenalectomy...
November 2018: Surgery
Weronika Korzyńska, Anna Jodkowska, Katarzyna Gosławska, Katarzyna Bogunia-Kubik, Grzegorz Mazur
Primary hyperaldosteronism (PHA) is the most common form of secondary hypertension of hormonal origin. It affects about 10% of all hypertensive patients. It is connected with increased morbidity and mortality from cardiovascular diseases (CVD) compared to patients with essential hypertension of a similar age. Usually, it is an effect of bilateral adrenal hyperplasia (BAH) or aldosterone-producing adenoma (APA), more rare causes of PHA are: unilateral adrenal hyperplasia, aldosterone-producing adrenocortical carcinoma, ectopic aldosterone-producing tumors and familial hyperaldosteronism...
August 2018: Advances in Clinical and Experimental Medicine: Official Organ Wroclaw Medical University
Elke Tatjana Aristizabal Prada, Isabella Castellano, Eva Sušnik, Yuhong Yang, Lucie S Meyer, Martina Tetti, Felix Beuschlein, Martin Reincke, Tracy A Williams
Primary aldosteronism is the most common form of endocrine hypertension with a prevalence of 6% in the general population with hypertension. The genetic basis of the four familial forms of primary aldosteronism (familial hyperaldosteronism FH types I-IV) and the majority of sporadic unilateral aldosterone-producing adenomas has now been resolved. Familial forms of hyperaldosteronism are, however, rare. The sporadic forms of the disease prevail and these are usually caused by either a unilateral aldosterone-producing adenoma or bilateral adrenal hyperplasia...
April 9, 2018: International Journal of Molecular Sciences
Maria-Christina Zennaro, Fabio L Fernandes-Rosa, Sheerazed Boulkroun
PURPOSE OF REVIEW: Primary aldosteronism is the most common form of secondary hypertension. Early diagnosis and treatment are key to cure of hypertension and prevention of cardiovascular complications. Recent genetic discoveries have improved our understanding on the pathophysiology of aldosterone production and triggered the development of new diagnostic procedures and targeted treatments for primary aldosteronism. RECENT FINDINGS: Different inherited genetic abnormalities distinguish specific forms of familial hyperaldosteronism...
June 2018: Current Opinion in Endocrinology, Diabetes, and Obesity
Mitchell R Ladd, Martha A Zeiger
The first discovery of primary hyperaldosteronism secondary to an aldosterone-secreting adrenal adenoma has been credited solely to Dr. Jerome Conn, an endocrinologist at the University of Michigan and for whom, Conn syndrome was named. Dr. William Baum, a urologist at the University of Michigan, however, was instrumental in the appropriate operation and historical aldosteronoma resection. Despite Dr. Baum's important role in this discovery, he was never included as an author in any of the subsequent papers describing Conn syndrome and, few today would recognize his name...
August 2018: World Journal of Surgery
Silvia Monticone, Fabrizio Buffolo, Martina Tetti, Franco Veglio, Barbara Pasini, Paolo Mulatero
Aldosterone is the main mineralocorticoid hormone in humans and plays a key role in maintaining water and electrolyte homeostasis. Primary aldosteronism (PA), characterized by autonomous aldosterone overproduction by the adrenal glands, affects 6% of the general hypertensive population and can be either sporadic or familial. Aldosterone-producing adenoma (APA) and bilateral adrenal hyperplasia (BAH) are the two most frequent subtypes of sporadic PA and 4 forms of familial hyperaldosteronism (FH-I to FH-IV) have been identified...
March 2018: European Journal of Endocrinology
Lütfi Soylu, Oğuz Uğur Aydın, Mustafa Cesur, Serdar Özbaş, Selçuk Hazinedaroğlu
Acute hypokalemic paralysis is a relatively rare cause of acute weakness. It may resolve spontaneously; however, it may be a potential life-threatening condition. Hypertension may be considered the most important finding in combination with hypokalemic paralysis for raising the suspicion of primary hyperaldosteronism (PHA). A 55-year-old hypertensive Mexican woman was admitted to the Emergency Unit with a sudden onset of generalized paralysis. An endocrinological workup and an abdominal magnetic resonance imaging revealed PHA with a 1...
2017: Turkish Journal of Surgery
S S C Gunatilake, U Bulugahapitiya
Background: Primary hyperaldosteronism is a known cause for secondary hypertension. In addition to its effect on blood pressure, aldosterone exhibits proinflammatory actions and plays a role in immunomodulation/development of autoimmunity. Recent researches also suggest significant thyroid dysfunction among patients with hyperaldosteronism, but exact causal relationship is not established. Autoimmune hyperthyroidism (Graves' disease) and primary hyperaldosteronism rarely coexist but underlying mechanisms associating the two are still unclear...
2017: Case Reports in Endocrinology
Celso E Gomez-Sanchez, Maniselvan Kuppusamy, Martin Reincke, Tracy Ann Williams
Primary aldosteronism is the most common type of secondary hypertension affecting 6-10% of patients with primary hypertension. PA is mainly caused by unilateral hyperaldosteronism due to an aldosterone-producing adenoma, unilateral hyperplasia with or without micronodules or bilateral zona glomerulosa hyperplasias with or without macro or micronodules. The development of antibodies against the terminal enzyme of aldosterone biosynthesis (CYP11B2) has permitted the further characterization of normal adrenals and resected adrenals from patients with primary aldosteronism...
December 2017: Hormone and Metabolic Research, Hormon- und Stoffwechselforschung, Hormones et Métabolisme
A Ferriere, V Kerlan, A Tabarin
The 2017 Endocrine Society annual meeting included several communications and debates on the conservative adrenal surgery in bilateral hereditary pheochromocytomas (BHP), bilateral adrenal macronodular hyperplasia (BAMH) and primary hyperaldosteronism (PHA). The general principle is to preserve a part of the adrenal cortex to prevent the occurrence of a definitive adrenal insufficiency. In BHP, cortical sparing surgery allows more than 50% of patients to maintain normal corticotropic function at 10 years with a low recurrence rate (~ 10%)...
October 2017: Annales D'endocrinologie
H Y Li, P Li, S M Shen, X B Zhang, W H Feng, H Huang, W Chen, D L Zhu
Objective: To investigate the role of adrenal vein sampling (AVS) in identifying the subtype of primary aldosteronism (PA). Methods: AVS was performed in 50 patients who were confirmed as PA between September 2010 and September 2016 in Nanjing Drum Tower Hospital. Clinical, biochemical and follow-up data were reviewed retrospectively. Bilaterally simultaneous catheterization without cosyntropin stimulation and contemporaneous cortisol measurement during AVS were used. Selectivity index (SI)≥1.5 suggested that the sample was from the adrenal vein...
November 14, 2017: Zhonghua Yi Xue za Zhi [Chinese medical journal]
Tjokorda Gde Dalem Pemayun, Ridho Naibaho, Maretina W Wiyati, Ardy Santosa, Siti Amarwati
Primary hyperaldosteronism is an adrenal abnormality in which there is some degree of autonomy of aldosterone secretion. We report a case of thirty three years old Javanese female presented with uncontrolled hypertension, muscular weakness, cramps  and progressing shortness of breath during working for 6 years. She had history of hypertension since age 20. Her serum potassium level was always low that associated with inappropriate kaliuresis. Blood gas analysis revealed metabolic alkalosis. Sonography of the adrenal gland showed right hipoechoic architecture; CT scan of the abdomen confirmed an right adrenal tumor measured 4 cm in its greatest dimension...
July 2017: Acta Medica Indonesiana
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