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Secondary AND Hyperaldosteronism AND adrenal AND adenoma

Dylan J Meyer, Craig Gatto, Pablo Artigas
Primary hyperaldosteronism (Conn's syndrome), a common cause of secondary hypertension, is frequently produced by unilateral aldosterone-producing adenomas that carry mutations in ion-transporting genes, including ATP1A1, encoding the Na/K pump's α1-subunit. Whether Na/K-pump-mutant-mediated inward currents are required to depolarize the cell and increase aldosterone production remains unclear, as such currents were observed in four out of five mutants described so far. Here, we use electrophysiology and uptake of the K+-congener 86Rb+, to characterize the effects of eight additional Na/K pump mutations in transmembrane segments TM1 (delM102-L103, delL103-L104, and delM102-I106), TM4 (delI322-I325 and I327S), and TM9 (delF956-E961, delF959-E961, and delE960-L964), expressed in Xenopus oocytes...
February 27, 2019: Biochemistry
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
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
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
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
Gregory Kline, Daniel T Holmes
Primary aldosteronism (PA) is the most common form of secondary hypertension and is critical to identify because when caused by an aldosterone-producing adenoma (APA) or another unilateral form, it is potentially curable, and even when caused by bilateral disease, antihypertensives more specific to PA treatment can be employed (ie, aldosterone antagonists). Identification of unilateral forms is not generally accomplished with imaging because APAs may be small and elude detection, and coincidental identification of a non-functioning incidentaloma contralateral to an APA may lead to removal of an incorrect gland...
November 2017: Journal of Clinical Pathology
Leticia A P Vilela, Madson Q Almeida
Primary aldosteronism (PA) is the most common form of secondary hypertension (HTN), with an estimated prevalence of 4% of hypertensive patients in primary care and around 10% of referred patients. Patients with PA have higher cardiovascular morbidity and mortality than age- and sex-matched patients with essential HTN and the same degree of blood pressure elevation. PA is characterized by an autonomous aldosterone production causing sodium retention, plasma renin supression, HTN, cardiovascular damage, and increased potassium excretion, leading to variable degrees of hypokalemia...
May 2017: Archives of Endocrinology and Metabolism
Silvia Monticone, Jacopo Burrello, Davide Tizzani, Chiara Bertello, Andrea Viola, Fabrizio Buffolo, Luisa Gabetti, Giulio Mengozzi, Tracy A Williams, Franco Rabbia, Franco Veglio, Paolo Mulatero
BACKGROUND: Despite being widely recognized as the most common form of secondary hypertension, among the general hypertensive population the true prevalence of primary aldosteronism (PA) and its main subtypes, aldosterone-producing adenoma (APA) and bilateral adrenal hyperplasia (BAH), remains a matter of debate. OBJECTIVES: This study sought to determine the prevalence and clinical phenotype of PA in a large cohort of unselected patients with hypertension, consecutively referred to our hypertension unit, by 19 general practitioners from Torino, Italy...
April 11, 2017: Journal of the American College of Cardiology
Barry A Sacks, Ari C Sacks, Salomao Faintuch
PURPOSE OF REVIEW: To review the current status of radiofrequency ablation as a primary treatment for hyperfunctioning adrenal nodules, predominantly aldosterone-producing adenomas (APAs). RECENT FINDINGS: Radiofrequency ablation is an established treatment for focal benign and malignant lesions, including metastatic disease to the adrenal gland. Hyperaldosteronism is the leading cause of secondary hypertension with up to 80% due to APA, statistically the most common functioning adrenal nodule...
June 2017: Current Opinion in Endocrinology, Diabetes, and Obesity
Jacek Kądziela, Aleksander Prejbisz, Ilona Michałowska, Sylwia Kołodziejczyk-Kruk, Leo Schultze Kool, Marek Kabat, Hanna Janaszek-Sitkowska, Sadegh Toutounchi, Zbigniew Gałązka, Urszula Ambroziak, Tomasz Bednarczuk, Dorota Ptasińska-Wnuk, Michał Hoffmann, Magdalena Januszewicz, Andrzej Januszewicz, Adam Witkowski
BACKGROUND: Primary aldosteronism is one of the most common causes of secondary hypertension. Adrenal vein sampling (AVS) remains a "gold standard" procedure in differentiation between unilateral (adenoma) and bilateral (hyperplasia) disease. AIM: The aim of this study was to present our single-centre experience in establishing and implementating the AVS procedure. METHODS: All patients had primary aldosteronism confirmed in a salt-infusion test...
2017: Kardiologia Polska
Geeta Gyamlani, Carol M Headley, Adnan Naseer, Ganpat S Valaulikar, Stephen A Geraci
Primary aldosteronism (PA) is an important and commonly unrecognized cause of secondary hypertension. Idiopathic hyperaldosteronism and aldosterone-producing adenomas account for more than 95% of PA and are characterized, respectively, by bilateral or unilateral involvement of the adrenal glands. When there is suspicion for the presence of PA, a plasma aldosterone to renin ratio should be obtained initially. Localization to determine adrenal gland involvement is done by imaging, with computerized tomography or magnetic resonance imaging...
October 2016: American Journal of the Medical Sciences
Satoshi Umemura
Primary aldosteronism (PA) is a heterogeneous group of disorders including both sporadic and familial forms (familial hyperaldosteronism type I, II and III). PA is the most frequent endocrine cause of secondary hypertension and associated with a higher rate of cardiovascular complications, compared with essential hypertension.Here I review the recent progress in understanding of the genetic and molecular mechanisms leading to autonomous aldosterone production in PA.Systematic screening detects primary aldosteronism in 5 to 10% of all patients with hypertension and in approximately 20% of patients with resistant hypertension...
September 2016: Journal of Hypertension
Ravi Kumar Dutta, Peter Söderkvist, Oliver Gimm
Hypertension is a common medical condition and affects approximately 20% of the population in developed countries. Primary aldosteronism is the most common form of secondary hypertension and affects 8-13% of patients with hypertension. The two most common causes of primary aldosteronism are aldosterone-producing adenoma and bilateral adrenal hyperplasia. Familial hyperaldosteronism types I, II and III are the known genetic syndromes, in which both adrenal glands produce excessive amounts of aldosterone. However, only a minority of patients with primary aldosteronism have one of these syndromes...
October 2016: Endocrine-related Cancer
Tanja Dekkers, Aleksander Prejbisz, Leo J Schultze Kool, Hans J M M Groenewoud, Marieke Velema, Wilko Spiering, Sylwia Kołodziejczyk-Kruk, Mark Arntz, Jacek Kądziela, Johannes F Langenhuijsen, Michiel N Kerstens, Anton H van den Meiracker, Bert-Jan van den Born, Fred C G J Sweep, Ad R M M Hermus, Andrzej Januszewicz, Alike F Ligthart-Naber, Peter Makai, Gert-Jan van der Wilt, Jacques W M Lenders, Jaap Deinum
BACKGROUND: The distinction between unilateral aldosterone-producing adenoma or bilateral adrenal hyperplasia as causes of primary aldosteronism is usually made by adrenal CT or by adrenal vein sampling (AVS). Whether CT or AVS represents the best test for diagnosis remains unknown. We aimed to compare the outcome of CT-based management with AVS-based management for patients with primary aldosteronism. METHODS: In a randomised controlled trial, we randomly assigned patients with aldosteronism to undergo either adrenal CT or AVS to determine the presence of aldosterone-producing adenoma (with subsequent treatment consisting of adrenalectomy) or bilateral adrenal hyperplasia (subsequent treatment with mineralocorticoid receptor antagonists)...
September 2016: Lancet Diabetes & Endocrinology
E Asbach, T A Williams, M Reincke
Primary aldosteronism (PA) is the most frequent endocrine cause of secondary arterial hypertension. Sporadic forms of PA caused mainly by an aldosterone producing adenoma (APA) or idiopathic adrenal hyperplasia (IAH) predominate; in contrast, familial forms (familial hyperaldosteronism types I, II and III) affect only a minor proportion of PA patients. Patient based registries and biobanks, international networks and next generation sequencing technologies have emerged over recent years. Somatic hot-spot mutations in the potassium channel GIRK4 (encoded by KCNJ5), in ATPases and a L-type voltage-gated calcium-channel correlate with the autonomous aldosterone production in approximately half of all APAs...
June 2016: Experimental and Clinical Endocrinology & Diabetes
B Rodríguez Maya, I Rodríguez Goncer, C Diego Hernández
We report a case of a 37 year-old man with a long history of hypertension under treatment, who was admitted at our institution with intense fatigue and weakness of lower limbs. The laboratory results at Emergency Department showed severe hypokalemia. A study of secondary hypertension was carried out. With the initial suspicion of primary hyperaldosteronism, complete blood test was done including plasma renine activity, which was completely suppressed, and plasma aldosterone concentration, which resulted normal...
April 2016: Hipertensión y Riesgo Vascular
Oskar Ragnarsson, Andreas Muth, Gudmundur Johannsson, Bo Wängberg
Primary aldosteronism is the most common cause of secondary hypertension with an estimated prevalence of 5-13 % among patients with hypertension. The most common causes are aldosterone producing adrenal adenoma and idiopathic adrenal hyperplasia. Patients with primary aldosteronism have a higher prevalence of cardiovascular morbidity and mortality compared to patients with essential hypertension. An effective treatment is available for patients with primary aldosteronism, with mineralocorticoid receptor antagonists in bilateral, and minimal invasive adrenal surgery in unilateral disease, which emphasizes the importance of early detection, adequate diagnostic work-up and treatment...
2015: Läkartidningen
Adam Morton
Primary aldosteronism is the most common cause of secondary hypertension. Less than 50 cases of pregnancy in women with primary aldosteronism have been reported, suggesting the disorder is significantly underdiagnosed in confinement. Accurate diagnosis is complicated by physiological changes in the renin-angiotensin-aldosterone axis in pregnancy, leading to a risk of false negative results on screening tests. The course of primary aldosteronism during pregnancy is highly variable, although overall it is associated with a very high risk of fetal and maternal morbidity and mortality...
October 2015: Pregnancy Hypertension
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