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Primary hyperaldosteronism

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https://www.readbyqxmd.com/read/29642543/comparative-genomics-and-transcriptome-profiling-in-primary-aldosteronism
#1
REVIEW
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
https://www.readbyqxmd.com/read/29538199/hypertensive-crisis-with-2-target-organ-impairment-induced-by-glycyrrhizin-a-case-report
#2
Jing Li, Xiaoli Fan, Qin Wang
RATIONALE: Glycyrrhizin is the main active component of licorice. Licorice and glycyrrhizin induced hypertension has been widely reported, yet licorice and glycyrrhizin induced hypertensive crisis has been rarely known. PATIENT CONCERNS: The case of this report was a 47-year-old woman, who took 225 mg of glycyrrhizin daily for 3 years due to primary biliary cholangitis. She was found to have a dramatically elevated blood pressure of about 230/110 mmHg without a history of hypertension and was referred to the emergency department...
March 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29521660/update-in-adrenal-venous-sampling-for-primary-aldosteronism
#3
Gian Paolo Rossi
PURPOSE OF REVIEW: Current guidelines recommend adrenal venous sampling (AVS) to identify the surgically curable causes of hyperaldosteronism. In contrast with this recommendation, AVS remains markedly underutilized in clinical practice, which leads to deny curative adrenalectomy, to many patients with primary aldosteronism. The purpose of this review is to challenge the views that AVS is a technically challenging, invasive and risky procedure, which moreover, is difficult to interpret...
March 7, 2018: Current Opinion in Endocrinology, Diabetes, and Obesity
https://www.readbyqxmd.com/read/29487649/anomalous-adrenal-vein-anatomy-complicating-the-evaluation-of-primary-hyperaldosteronism
#4
Kaitlin M Ford, Sara Smolinski, Juan Carlos Perez Lozada
Adrenal vein development in utero occurs concurrently with the development of the inferior vena cava, the renal veins, and the gonadal veins. The embryologic formation of these veins involves communication of various venous systems. Although the left adrenal-renal vein complex is most commonly described as a shared emptying of the left adrenal vein and the left inferior phrenic vein into the left renal vein, there have been reports of numerous anatomic variations of this complex. In this report, we present a case of a rare variant of the left adrenal vein, in which the left adrenal vein empties into the left gonadal vein, which takes an atypical course superolateral to the left kidney...
February 2018: Radiology Case Reports
https://www.readbyqxmd.com/read/29453922/a-short-review-of-primary-aldosteronism-in-a-question-and-answer-fashion
#5
Frederick-Anthony Farrugia, Nicolaos Zavras, Georgios Martikos, Panagiotis Tzanetis, Anestis Charalampopoulos, Evangelos P Misiakos, Dimitrios Sotiropoulos, Nikolaos Koliakos
OBJECTIVES: The aim of this study was to present up to date information concerning the diagnosis and treatment of primary aldosteronism (PA). PA is the most common cause of endocrine hypertension. It has been reported up to 24% of selective referred hypertensive patients. METHODS: We did a search in Pub-Med and Google Scholar using the terms: PA, hyperaldosteronism, idiopathic adrenal hyperplasia, diagnosis of PA, mineralocorticoid receptor antagonists, adrenalectomy, and surgery...
January 1, 2018: Endocrine Regulations
https://www.readbyqxmd.com/read/29445488/glucocorticoid-remediable-aldosteronism-in-a-young-adult-with-a-family-history-of-conn-s-syndrome
#6
Heiko Methe, Sinan Pehlivanli
Glucocorticoid-remediable aldosteronism is a hereditary form of primary hyperaldosteronism and the most common monogenic cause of hypertension. We present the case of a 24-year-old man with a family history of Conn's syndrome. Yet, in the index patient, classical characteristics of mineralocorticoid excess could be reversed by exogenous glucocorticoids.
February 2018: Clinical Case Reports
https://www.readbyqxmd.com/read/29432258/overview-of-aldosterone-related-genetic-syndromes-and-recent-advances
#7
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...
February 8, 2018: Current Opinion in Endocrinology, Diabetes, and Obesity
https://www.readbyqxmd.com/read/29403011/clcn2-chloride-channel-mutations-in-familial-hyperaldosteronism-type-ii
#8
Ute I Scholl, Gabriel Stölting, Julia Schewe, Anne Thiel, Hua Tan, Carol Nelson-Williams, Alfred A Vichot, Sheng Chih Jin, Erin Loring, Verena Untiet, Taekyeong Yoo, Jungmin Choi, Shengxin Xu, Aihua Wu, Marieluise Kirchner, Philipp Mertins, Lars C Rump, Ali Mirza Onder, Cory Gamble, Daniel McKenney, Robert W Lash, Deborah P Jones, Gary Chune, Priscila Gagliardi, Murim Choi, Richard Gordon, Michael Stowasser, Christoph Fahlke, Richard P Lifton
Primary aldosteronism, a common cause of severe hypertension1 , features constitutive production of the adrenal steroid aldosterone. We analyzed a multiplex family with familial hyperaldosteronism type II (FH-II)2 and 80 additional probands with unsolved early-onset primary aldosteronism. Eight probands had novel heterozygous variants in CLCN2, including two de novo mutations and four independent occurrences of a mutation encoding an identical p.Arg172Gln substitution; all relatives with early-onset primary aldosteronism carried the CLCN2 variant found in the proband...
March 2018: Nature Genetics
https://www.readbyqxmd.com/read/29352336/who-was-dr-william-c-baum
#9
REVIEW
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...
January 19, 2018: World Journal of Surgery
https://www.readbyqxmd.com/read/29348113/genetics-in-endocrinology-the-expanding-genetic-horizon-of-primary-aldosteronism
#10
REVIEW
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
https://www.readbyqxmd.com/read/29275380/double-hit-a-unique-case-of-resistant-hypertension
#11
Kristen Elizabeth DeCarlo, Nidhi Agrawal
A middle-aged woman with obesity, hyperlipidaemia and diet-controlled diabetes was referred for resistant hypertension. Her blood pressure (BP) was uncontrolled on five medications, including a diuretic. Physical exam revealed a systolic ejection murmur, and ECHO demonstrated moderate hypertrophy. Laboratory examination revealed elevated aldosterone level (20.7 ng/dL) and elevated aldosterone:renin ratio (41.4 (ng/dL)/(ng/mL/h)), meeting criteria for primary aldosteronism (PA), and confirmed by saline infusion testing...
December 22, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/29260252/-functional-diagnostics-in-endocrinology
#12
REVIEW
C J Auernhammer, M Reincke
When investigating many endocrinological diseases, basal laboratory parameters are not sufficient to distinguish between physiological and pathological hormone secretion. Functional diagnostics plays a decisive role in this context. Stimulation and suppression tests are used depending on whether under- or over-function needs to be diagnosed. This review article discusses selected functional tests, each of which plays an important role in current guidelines. Indications and test principles, including their performance, reliability, and limitations, are discussed...
January 2018: Der Internist
https://www.readbyqxmd.com/read/29260143/single-incision-laparoscopic-adrenalectomy-in-a-patient-with-acute-hypokalemic-paralysis-due-to-primary-hyperaldosteronism
#13
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
https://www.readbyqxmd.com/read/29214084/coexistence-of-primary-hyperaldosteronism-and-graves-disease-a-rare-combination-of-endocrine-disorders-is-it-beyond-a-coincidence-a-case-report-and-review-of-the-literature
#14
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
https://www.readbyqxmd.com/read/29212953/the-e3-ubiquitin-ligase-siah1-regulates-adrenal-gland-organization-and-aldosterone-secretion
#15
Marzia Scortegagna, Annabel Berthon, Nikolaos Settas, Andreas Giannakou, Guillermina Garcia, Jian-Liang Li, Brian James, Robert C Liddington, José G Vilches-Moure, Constantine A Stratakis, Ze'ev A Ronai
Primary and secondary hypertension are major risk factors for cardiovascular disease, the leading cause of death worldwide. Elevated secretion of aldosterone resulting from primary aldosteronism (PA) is a key driver of secondary hypertension. Here, we report an unexpected role for the ubiquitin ligase Siah1 in adrenal gland development and PA. Siah1a-/- mice exhibit altered adrenal gland morphology, as reflected by a diminished X-zone, enlarged medulla, and dysregulated zonation of the glomerulosa as well as increased aldosterone levels and aldosterone target gene expression and reduced plasma potassium levels...
December 7, 2017: JCI Insight
https://www.readbyqxmd.com/read/29202495/disordered-cyp11b2-expression-in-primary-aldosteronism
#16
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
https://www.readbyqxmd.com/read/29157485/la-chirurgie-d%C3%A2-%C3%A3-pargne-surr%C3%A3-nalienne-du-cortex-%C3%A3-la-m%C3%A3-dulla-cortical-sparing-surgery-from-cortex-to-medulla
#17
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
https://www.readbyqxmd.com/read/29141372/-role-of-adrenal-vein-sampling-in-differential-diagnosis-of-primary-aldosteronism-subtypes
#18
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]
https://www.readbyqxmd.com/read/29095987/an-unusual-case-of-takotsubo-syndrome-with-hyperaldosteronism-as-the-potential-cause
#19
Yuanweixiang Ou, Zhengang Zhao, Jiayyu Tsauo, Licheng Jiang, Yong Yang, Mao Chen
Context: Catecholamine-related factors are the most popular explanation for the occurrence of Takotsubo syndrome. An aldosterone-related mechanism, however, has not been proposed. Case Description: A 45-year-old male patient presenting with ST-segment elevation myocardial infarction was diagnosed with primary aldosteronism, severe hypokalemia, and Takotsubo syndrome. After excluding the known conditions of apical ballooning and the factors of vasospasm, primary aldosteronism is considered as the major contributor to the development of Takotsubo syndrome...
January 1, 2018: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/29094913/secondary-hypertension-discovering-the-underlying-cause
#20
REVIEW
Lesley Charles, Jean Triscott, Bonnie Dobbs
Most patients with hypertension have no clear etiology and are classified as having primary hypertension. However, 5% to 10% of these patients may have secondary hypertension, which indicates an underlying and potentially reversible cause. The prevalence and potential etiologies of secondary hypertension vary by age. The most common causes in children are renal parenchymal disease and coarctation of the aorta. In adults 65 years and older, atherosclerotic renal artery stenosis, renal failure, and hypothyroidism are common causes...
October 1, 2017: American Family Physician
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