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Primary hyperaldosteronism

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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
#1
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
#2
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
#3
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
#4
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
#5
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
#6
Yuanweixiang Ou, Zhengang Zhao, Jiayyu Tsauo, Licheng Jiang, Yong Yang, Mao Chen
Context: Catecholamine-related factors are currently the most popular explanation for the occurrence of Takotsubo syndrome. The aldosterone-related mechanism, however, has not been proposed. Case Description: A 45-year old male, presenting with ST-segment elevation myocardial infarction (STEMI), was finally 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 the Takotsubo syndrome...
October 31, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/29094913/secondary-hypertension-discovering-the-underlying-cause
#7
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
https://www.readbyqxmd.com/read/29093236/primary-hyperaldosteronism-due-to-adrenocortical-adenoma-a-case-report
#8
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
https://www.readbyqxmd.com/read/29092077/significance-of-computed-tomography-and-serum-potassium-in-predicting-subtype-diagnosis-of-primary-aldosteronism
#9
Hironobu Umakoshi, Mika Tsuiki, Yoshiyu Takeda, Isao Kurihara, Hiroshi Itoh, Takuyuki Katabami, Takamasa Ichijo, Norio Wada, Takanobu Yoshimoto, Yoshihiro Ogawa, Junji Kawashima, Masakatsu Sone, Nobuya Inagaki, Katsutoshi Takahashi, Minemori Watanabe, Yuichi Matsuda, Hiroki Kobayashi, Hirotaka Shibata, Kohei Kamemura, Michio Otsuki, Yuichi Fujii, Koichi Yamamto, Atsushi Ogo, Toshihiko Yanase, Tomoko Suzuki, Mitsuhide Naruse
Context: The number of centers with established adrenal venous sampling (AVS) programs for subtype diagnosis of primary aldosteronism (PA) is limited. Objective: Aim was to develop an algorithm for AVS based on subtype prediction by computed tomography (CT) and serum potassium. Design: A multi-institutional retrospective cohort study in Japan. Patients: 1591 PA patients were classified into four groups according to CT findings and potassium status...
October 6, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/29069360/mouse-models-of-primary-aldosteronism-from-physiology-to-pathophysiology
#10
Leticia Aragao-Santiago, Celso E Gomez-Sanchez, Paolo Mulatero, Ariadni Spyroglou, Martin Reincke, Tracy Ann Williams
Primary aldosteronism (PA) is a common form of endocrine hypertension that is characterized by the excessive production of aldosterone relative to suppressed plasma renin levels. PA is usually caused by either a unilateral aldosterone-producing adenoma or bilateral adrenal hyperplasia. Somatic mutations have been identified in several genes that encode ion pumps and channels that may explain the aldosterone excess in over half of aldosterone-producing adenomas, whereas the pathophysiology of bilateral adrenal hyperplasia is largely unknown...
October 23, 2017: Endocrinology
https://www.readbyqxmd.com/read/29068510/characteristics-of-adrenal-incidentalomas-in-a-new-zealand-centre
#11
Z Goh, I Phillips, P J Hunt, S Soule, T J Cawood
BACKGROUND: Management of adrenal incidentalomas (AI) is becoming more conservative, based on international data showing a low incidence of functional or malignant lesions. The clinical characteristics of AI in New Zealand are unknown. Therefore, whether the AI guidelines apply to the New Zealand population is also unknown. AIMS: To investigate the clinical characteristics of patients with AI presenting to a tertiary-care centre in New Zealand. METHOD: This study prospectively evaluated consecutive patients aged 18 or older with AI, 1 cm or larger, diagnosed in Canterbury, New Zealand...
October 25, 2017: Internal Medicine Journal
https://www.readbyqxmd.com/read/29065434/unanswered-questions-in-the-genetic-basis-of-primary-aldosteronism
#12
Ute I Scholl
Over the past six years, the genetic basis of a significant fraction of primary aldosteronism (PA) cases has been solved. Breakthrough discoveries include the role of somatic variants in the KCNJ5, CACNA1D, ATP1A1, and ATP2B3 genes as causes of aldosterone-producing adenomas (APAs), and the recognition of three novel hyperaldosteronism syndromes with germline variants in the KCNJ5, CACNA1D, and CACNA1H genes. The description of somatic variants in CACNA1D and ATP1A1 in aldosterone-producing cell clusters (APCCs) suggests that these clusters are precursors of some aldosterone-producing adenomas...
October 24, 2017: Hormone and Metabolic Research, Hormon- und Stoffwechselforschung, Hormones et Métabolisme
https://www.readbyqxmd.com/read/29030398/on-the-effect-of-hyperaldosteronism-inducing-mutations-in-na-k-pumps
#13
Dylan J Meyer, Craig Gatto, Pablo Artigas
Primary aldosteronism, a condition in which too much aldosterone is produced and that leads to hypertension, is often initiated by an aldosterone-producing adenoma within the zona glomerulosa of the adrenal cortex. Somatic mutations of ATP1A1, encoding the Na/K pump α1 subunit, have been found in these adenomas. It has been proposed that a passive inward current transported by several of these mutant pumps is a "gain-of-function" activity that produces membrane depolarization and concomitant increases in aldosterone production...
November 6, 2017: Journal of General Physiology
https://www.readbyqxmd.com/read/28994759/a-novel-method-super-selective-adrenal-venous-sampling
#14
Kohzoh Makita, Koshiro Nishimoto, Kanako Kiriyama-Kitamoto, Shigehiro Karashima, Tsugio Seki, Masanori Yasuda, Seishi Matsui, Masao Omura, Tetsuo Nishikawa
Primary aldosteronism (PA) and subclinical Cushing's syndrome (SCS) are conditions in which the adrenal glands autonomously produce excessive amounts of aldosterone and cortisol, respectively. The conventional adrenal venous sampling (cAVS) method collects blood samples from both adrenal central veins and is useful for identifying the laterality of excess hormone production in a unilateral lesion(s), as documented in PA cases. In cAVS, plasma cortisol concentrations (PCCs) are used to normalize plasma aldosterone concentrations (PACs)...
September 15, 2017: Journal of Visualized Experiments: JoVE
https://www.readbyqxmd.com/read/28993452/macrolides-blunt-aldosterone-biosynthesis-a-proof-of-concept-study-in-kcnj5-mutated-adenoma-cells-ex-vivo
#15
Brasilina Caroccia, Selene Prisco, Teresa Maria Seccia, Maria Piazza, Giuseppe Maiolino, Gian Paolo Rossi
Aldosterone-producing adenoma (APA), a major subtype of primary hyperaldosteronism, the main curable cause of human endocrine hypertension, involves somatic mutations in the potassium channel Kir3.4 (KCNJ5) in 30% to 70% of cases, typically the more florid phenotypes. Because KCNJ5 mutated channels were reported to be specifically sensitive to inhibition by macrolide antibiotics, which concentration dependently blunts aldosterone production in HAC15 transfected with the G151R and L168R mutated channel, we herein tested the effect of clarithromycin on aldosterone synthesis and secretion in a pure population of aldosterone-secreting cells obtained by immunoseparation (CD56(+) cells) from APA tissues with/without the 2 most common KCNJ5 mutations...
October 9, 2017: Hypertension
https://www.readbyqxmd.com/read/28918446/favorable-surgical-outcomes-of-aldosterone-producing-adenoma-based-on-lateralization-by-ct-imaging-and-hypokalemia-a-non-avs-based-strategy
#16
Hai Li, Jianbin Liu, Xiujuan Feng, Liehua Liu, Guohong Wei, Xiaopei Cao, Yanbing Li
PURPOSE: To test the efficacy of a strategy based on CT imaging and clinical characteristics on lateralizing origin of excess aldosterone secretion in primary aldosteronism. PATIENTS AND METHODS: Consecutive patients with diagnosed primary hyperaldosteronism from June 2006 to July 2012 in our center underwent adrenal surgeries without pre-operational adrenal venous sampling (AVS) if all the three criteria were met: (1) round- or oval-shaped occupational lesion of low density after contrast enhancement with diameter >1 cm on CT scan was located in one adrenal gland; (2) unequivocally normal contralateral adrenal gland; (3) serum potassium level lower than 3...
December 2017: International Urology and Nephrology
https://www.readbyqxmd.com/read/28904009/diagnosis-of-endocrine-disease-18-oxocortisol-and-18-hydroxycortisol-is-there-clinical-utility-of-these-steroids
#17
Jacques Lenders, Tracy A Williams, Martin Reincke, Celso E Gomez-Sanchez
Since the early nineteen eighties 18-hydroxycortisol and 18-oxocortisol have attracted attention when it was shown that the urinary excretion of these hybrid steroids was increased in primary aldosteronism. The development and more widespread use of specific assays has improved the understanding of their role in the (patho)physiology of adrenal disorders. The adrenal site of synthesis is not fully understood although it is clear that for the synthesis of 18-hydroxycortisol and 18-oxocortisol the action of both aldosterone synthase (zona glomerulosa) and 17α-hydroxylase (zona fasciculata) is required with cortisol as main substrate...
September 13, 2017: European Journal of Endocrinology
https://www.readbyqxmd.com/read/28870047/-expert-consensus-for-the-diagnosis-and-treatment-of-patients-with-gitelman-syndrome
#18
(no author information available yet)
Gitelman syndrome (GS) is an autosomal recessive, salt-losing tubulopathy caused by inactivating mutations in the SLC12A3 gene that encodes the thiazide-sensitive sodium-chloride cotransporter (NCC). GS is characterized by hypokalemic metabolic alkalosis, hypomagnesemia and hypocalciuria. GS is one of the most common inherited renal tubulopathy with a prevalence estimated at about one to ten per 40 000 people. The prevalence of GS is even higher in Asia than other countries. The majority of GS patients present mild and nonspecific symptoms during adolescence or adulthood...
September 1, 2017: Zhonghua Nei Ke za Zhi [Chinese Journal of Internal Medicine]
https://www.readbyqxmd.com/read/28810583/kidney-dysfunction-following-adrenalectomy-in-autosomal-dominant-polycystic-kidney-disease-complicated-with-primary-aldosteronism-a-case-report
#19
Hiroyuki Hirai, Makoto Kanno, Tsuyoshi Watanabe, Hiroaki Satoh
The present study reported a case of autosomal dominant polycystic kidney disease complicated with primary aldosteronism in a 49-year-old woman. The patient was referred for refractory hypertension. Laboratory examinations revealed low potassium and renin levels. Computed tomography indicated a right adrenal tumor and multiple renal cysts. Adrenal vein sampling revealed a high aldosterone level on the side of the tumor. The patient was diagnosed with autosomal dominant polycystic kidney disease complicated by primary aldosteronism and adrenalectomy was performed...
August 2017: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/28758009/11-deoxycortisol-may-be-superior-to-cortisol-in-confirming-a-successful-adrenal-vein-catheterization-without-cosyntropin-a-pilot-study
#20
Naris Nilubol, Steven J Soldin, Dhaval Patel, Muthoni Rwenji, Jianghong Gu, Likhona S Masika, Richard Chang, Constantine A Stratakis, Electron Kebebew
AIM: We aimed to compare the performance of nine adrenal steroids in confirming the correct catheter position during adrenal venous sampling (AVS) without cosyntropin in patients with primary hyperaldosteronism. MATERIALS & METHODS: A successful adrenal vein catheterization without cosyntropin was defined as the ratio of steroids from adrenal to peripheral veins being >3:1. AVS samples from four patients with primary hyperaldosteronism were analyzed. RESULTS: Compared with the mean ratio of cortisol without cosyntropin, the ratios of 11-deoxycortisol (p = 0...
May 2017: International Journal of Endocrine Oncology
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