10,411 results match your criteria Conn Syndrome


[Hypokalemia with pseudo-hyperaldosteronism: Is it Lidl® syndrome?]

Nephrol Ther 2020 Jul 3. Epub 2020 Jul 3.

Division of nephrology, Rennes University Hospital, 2, rue Henri-Le-Guilloux, 35000 Rennes, France; Irset (Institut de Recherche en Santé, Environnement et Travail)-UMR 1085, 9, avenue du Professeur-Léon-Bernard, 35000 Rennes, France.

Introduction: Intoxication induced by glycyrrhizin is a common cause of hypokalaemia by pseudo-hyperaldosteronism.

Observation: We hereby present the observation of a 68-year old patient hospitalised following a full hip-prosthesis operation after a deep hypokalaemia at 2.5mM was observed, with ECG signs (flat T waves and appearance of U waves). Read More

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http://dx.doi.org/10.1016/j.nephro.2020.03.008DOI Listing

Markers of potassium homeostasis in salt losing tubulopathies- associations with hyperaldosteronism and hypomagnesemia.

BMC Nephrol 2020 Jul 6;21(1):256. Epub 2020 Jul 6.

Division of Nephrology and Dialysis, Department of Medicine III, Medical University Vienna, Währinger Gürtel 18-20, A-1090, Vienna, Austria.

Background: Renal loss of potassium (K) and magnesium (Mg) in salt losing tubulopathies (SLT) leads to significantly reduced Quality of Life (QoL) and higher risks of cardiac arrhythmia. The normalization of K is currently the most widely accepted treatment target, however in even excellently designed RCTs the increase of K was only mild and rarely normalized. These findings question the role of K as the ideal marker of potassium homeostasis in SLT. Read More

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http://dx.doi.org/10.1186/s12882-020-01905-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7336449PMC

Two novel mutations in the gene leading to classic Bartter syndrome presenting as syncope and hypertension in a 13-year-old boy.

BMJ Case Rep 2020 Jul 5;13(7). Epub 2020 Jul 5.

School of Medicine, Vietnam National University, Ho Chi Minh City, Vietnam.

Classic Bartter syndrome is a rare condition caused by mutations in the gene and characterised by metabolic alkalosis, hypokalaemia, hyper-reninaemia and hyperaldosteronism. Early signs and symptoms usually occur before a child's sixth birthday and include polyuria and developmental delay. We treated a 13-year-old Vietnamese boy with this syndrome presenting with atypical presentations including syncope and hypertension, but normal growth and development. Read More

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http://dx.doi.org/10.1136/bcr-2019-233872DOI Listing

Dopamine-producing adrenal medulla hyperplasia associated with Conn syndrome. An unusual coexistence of refractory hypertension.

Med Clin (Barc) 2020 Jul 2. Epub 2020 Jul 2.

Unidad de Cirugía Endocrina, Servicio de Cirugía General, Instituto Murciano de Investigación Biomédica (IMIB), Hospital Clínico Universitario Virgen de la Arrixaca, El Palmar, Murcia, España.

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http://dx.doi.org/10.1016/j.medcli.2020.04.040DOI Listing

[Bartter-Gitelman syndromes].

Nephrol Ther 2020 Jul 1. Epub 2020 Jul 1.

Département de génétique, hôpital européen Georges-Pompidou, 20, rue Leblanc, 75015 Paris, France; Centre de Référence de maladies rénales rares de l'enfant et de l'adulte (MARHEA), Paris, France.

Bartter-Gitelman syndromes are rare inherited autosomal recessive salt-losing tubulopathies characterized by severe and chronic hypokalemia associated with metabolic alkalosis and secondary hyperaldosteronism. Bartter syndrome results from a furosemide-like defect in sodium reabsorption in the Henle's loop leading to hypercalciuria and defect in urinary concentration capacity. The antenatal Bartter syndrome is defined by polyhydramnios and an infantile polyuria with severe dehydration whereas classic Bartter syndrome appears during childhood or adulthood. Read More

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http://dx.doi.org/10.1016/j.nephro.2020.06.001DOI Listing

A speckle tracking echocardiographic study on right ventricular function in primary aldosteronism.

J Hypertens 2020 Jun 25. Epub 2020 Jun 25.

Centre for Cardiovascular Evaluations, The Shanghai Institute of Hypertension, Department of Hypertension, Shanghai Key Lab of Hypertension, Ruijin Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, China.

Objective: We investigated right ventricular function using speckle tracking echocardiography (STE) in patients with primary aldosteronism.

Methods: Our study included 51 primary aldosteronism patients and 50 age and sex-matched primary hypertensive patients. We performed two-dimensional echocardiography to measure cardiac structure and function. Read More

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http://dx.doi.org/10.1097/HJH.0000000000002527DOI Listing

[Primary hyperaldosteronism: difficulties in diagnosis].

Ter Arkh 2019 Oct 15;91(10):91-99. Epub 2019 Oct 15.

Endocrinology Research Centre.

Primary hyperaldosteronism (PA) - is the clinical syndrome, results from autonomous of the major regulators of secretion, aldosterone overproduction by a tumorous or hyperplastic tissue in adrenal cortex. Being the most frequent cause of secondary hypertension, PA may be represented by disorders with unilateral or bilateral aldosterone overproduction and differential diagnosis between them is crucial for choosing a right therapeutic approache: lifelong medical therapy with mineralocorticoid receptor antagonists or unilateral adrenalectomy. Adrenal venous sampling (AVS) is currently the «gold standard» test for identifying laterality of excess hormone production, unlike imaging tests, sensitivity and specificity of which is not enough, due to inability to evaluate functional activity with confidence, and also to limitations in detecting tiny abnormalities of adrenals, such as microadenoma or hyperplasia. Read More

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http://dx.doi.org/10.26442/00403660.2019.10.000138DOI Listing
October 2019

Treatment of primary hyperaldosteronism.

Med Clin (Barc) 2020 Jun 22. Epub 2020 Jun 22.

Unidad de Neuroendocrinología, Servicio de Endocrinología y Nutrición, Hospital Universitario Ramón y Cajal, Madrid, España. Electronic address:

Primary aldosteronism is associated with higher cardiovascular and renal morbidity and mortality than essential hypertension in age- and sex-matched patients with the same degree of blood pressure elevation. Therefore, it is essential to establish a specific treatment to avoid the deleterious effects of aldosterone excess. Although adrenalectomy is generally considered the treatment of choice in cases of primary aldosteronism due to unilateral disease, several aspects and circumstances should be taken into account that may make medical treatment more appropriate. Read More

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http://dx.doi.org/10.1016/j.medcli.2020.04.029DOI Listing

Impact of primary aldosteronism on renal function in patients with type 2 diabetes.

J Diabetes Investig 2020 Jun 24. Epub 2020 Jun 24.

Endocrinology and Diabetes Center, Yokohama Rosai Hospital, 3211 Kozukue-cho, Kouhoku-ku, Yokohama, Kanagawa, 222-0036, Japan.

Aims/introduction: Renal dysfunction may quickly progress in patients with type 2 diabetes (T2DM), when accompanied by hypertension. However, whether primary aldosteronism (PA), which autonomously over-secretes aldosterone, causes additional renal damage in patients with T2DM is unclear. We evaluated the impact of PA on renal function in patients with T2DM. Read More

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http://dx.doi.org/10.1111/jdi.13332DOI Listing

Nicotine-induced adrenal beta-arrestin1 upregulation mediates tobacco-related hyperaldosteronism leading to cardiac dysfunction.

World J Cardiol 2020 May;12(5):192-202

Laboratory for the Study of Neurohormonal Control of the Circulation, Department of Pharmaceutical Sciences (Pharmacology), College of Pharmacy, Nova Southeastern University, Fort Lauderdale, FL 33328-2018, United States.

Background: Tobacco-related products, containing the highly addictive nicotine together with numerous other harmful toxicants and carcinogens, have been clearly associated with coronary artery disease, heart failure, stroke, and other heart diseases. Among the mechanisms by which nicotine contributes to heart disease is elevation of the renin-angiotensin-aldosterone system (RAAS) activity. Nicotine, and its major metabolite in humans cotinine, have been reported to induce RAAS activation, resulting in aldosterone elevation in smokers. Read More

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http://dx.doi.org/10.4330/wjc.v12.i5.192DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7283997PMC

[Clinical Characteristics of Aldosterone Producing Adenoma and Idiopathic Hyperaldosteronism with Obstructive Sleep Apnea Hypopnea Syndrome].

Sichuan Da Xue Xue Bao Yi Xue Ban 2020 May;51(3):298-303

Adrenal Center, Department of Endocrinology and Metabolism, West China Hospital, Sichuan University, Chengdu 610041, China.

Objective: To investigate the clinical characteristics of aldosterone producing adenoma (APA) and idiopathic hyperaldosteronism (IHA) complicated with obstructive sleep apnea hypopnea syndrome (OSAHS) and the effect of OSAHS on renin-angiotensin-aldosterone system (RAAS) in APA and IHA patients.

Methods: The clinical data of 127 patients with primary aldosteronism (PA) diagnosed from May 2010 to Aug. 2019 were retrospectively analyzed. Read More

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http://dx.doi.org/10.12182/20200560602DOI Listing

[The Value of Serum Potassium and Ratio of Sodium to Potassium after Saline Infusion Test in Differential Diagnosis of Primary Hyperaldosteronism].

Sichuan Da Xue Xue Bao Yi Xue Ban 2020 May;51(3):287-291

Adrenal Center, Department of Endocrinology and Metabolism, West China Hospital, Sichuan University, Chengdu 610041, China.

Objective: To explore the electrolyte characteristics between different types of primary aldosteronism (PA), especially the value of serum potassium and the ratio of sodium to potassium after saline infusion test (SIT) in differential diagnosis of PA.

Methods: The clinical data was collected from 135 patients who received screening for the causes of hypertension from Jan. 2009 to Dec. Read More

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http://dx.doi.org/10.12182/20200560101DOI Listing

Hemodynamics of primary aldosteronism associated with adrenocortical adenoma: Insights from bioimpedance cardiography measurements.

J Intern Med 2020 Jun 13. Epub 2020 Jun 13.

Unit of Nephrology and Hypertension, European Society of Hypertension Excellence Centre, Dipartimento Di Promozione Della Salute, Materno Infantile, Medicina Interna e Specialistica Di Eccellenza (PROMISE), Università degli studi di Palermo, Italy.

In mid 1950s, Dr Jerome Conn described a patient with hypertension, and renal potassium wasting associated with adrenocortical adenoma and increased urinary excretion of a sodium-retaining hormone, initially termed electrocortin, which was subsequently shown to be aldosterone. This was the first full report of primary hyperaldosteronism associated with an aldosterone-producing adenoma (APA). It subsequently became apparent that similar abnormalities can occur in the absence of an adrenocortical tumor, and it is now recognized that the APA is just one of many subtypes of primary aldosteronism (PA). Read More

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http://dx.doi.org/10.1111/joim.13127DOI Listing

Vasculometabolic and Inflammatory Effects of Aldosterone in Obesity.

J Clin Endocrinol Metab 2020 Aug;105(8)

Department of Internal Medicine, Radboud University Medical Center, Nijmegen, GA, the Netherlands.

Context: Not all obese individuals develop cardiovascular disease (CVD). Hyperaldosteronism is suggested to cause inflammation and metabolic dysregulation, and might contribute to CVD development in obese individuals.

Objective: We aimed to investigate the association of aldosterone concentrations with inflammation, metabolic disturbances, and atherosclerosis in overweight and obese individuals. Read More

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http://dx.doi.org/10.1210/clinem/dgaa356DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7320834PMC

Hypertension resolution after adrenalectomy for primary hyperaldosteronism: Which is the best predictive model?

Surgery 2020 Jun 2. Epub 2020 Jun 2.

Department of Surgery, Weill Cornell Medical College, New York-Presbyterian Hospital, New York, NY, USA. Electronic address:

Background: We aimed to compare the predictive performance of three distinct clinical models purported to predict the resolution of aldosteronoma-associated hypertension after adrenalectomy.

Methods: A tri-institutional database of aldosteronoma patients who underwent adrenalectomy between 2004 and 2019 was retrospectively reviewed. The three models of interest incorporate various preoperative clinical factors, such as age and sex. Read More

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http://dx.doi.org/10.1016/j.surg.2020.04.017DOI Listing
June 2020
3.380 Impact Factor

Co-Existence of Congenital Adrenal Hyperplasia and Bartter Syndrome due to Maternal Uniparental Isodisomy of HSD3B2 and CLCNKB Mutations.

Horm Res Paediatr 2020 Jun 5;93(1):66-71. Epub 2020 Jun 5.

Guy's and St Thomas' Hospital NHS Foundation Trust, London, United Kingdom.

Introduction: We present a patient with co-existence of 3β-hydroxysteroid dehydrogenase type 2 (HSD3B2) deficiency and Bartter syndrome, a unique dual combination of opposing pathologies that has not been reported previously in the literature.

Case: A female infant (46,XX) born at 34/40 weeks' gestation, weighing 2.67 kg (-1. Read More

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http://dx.doi.org/10.1159/000507577DOI Listing
June 2020
1.713 Impact Factor

Different pathogenesis of glucose intolerance in two subtypes of primary aldosteronism: Aldosterone-producing adenoma and idiopathic hyperaldosteronism.

J Diabetes Investig 2020 May 29. Epub 2020 May 29.

Department of Endocrinology, Diabetes and Metabolism, Graduate School of Medicine, Nippon Medical School, Tokyo, Japan.

Aims/introduction: An increased risk of diabetes mellitus has been reported in primary aldosteronism, but the pathogenesis of glucose intolerance between the primary aldosteronism subtypes remains unclear. This study aimed to evaluate glucose metabolism in oral glucose tolerance test between aldosterone-producing adenoma and idiopathic hyperaldosteronism, and characterize patients with improved glucose intolerance after primary aldosteronism treatment.

Materials And Methods: Oral glucose tolerance test was carried out in 116 patients who were diagnosed with primary aldosteronism and received adrenal venous sampling for subtyping. Read More

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http://dx.doi.org/10.1111/jdi.13312DOI Listing

Prospective evaluation of aldosterone LC-MS/MS-specific cutoffs for the saline infusion test.

Eur J Endocrinol 2020 Aug;183(2):191-201

Medical Department III, Endocrinology, Nephrology, Rheumatology, University Hospital of Leipzig, Leipzig, Germany.

Objective: Liquid chromatography coupled to tandem mass spectrometry (LC-MS/MS) has become state of the art for the quantitative analysis of steroid hormones. Although method comparisons show that aldosterone measurement using LC-MS/MS yields considerably lower levels than immunoassays (IAs), method-specific cutoff values for primary aldosteronism (PA) are largely missing. Objective of this study was to analyze the diagnostic accuracy of proposed LC-MS/MS-specific cutoff values for the saline infusion test (SIT). Read More

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http://dx.doi.org/10.1530/EJE-20-0030DOI Listing

Aldosterone and Mineralocorticoid Receptor Antagonists on Pulmonary Hypertension and Right Ventricular Failure: A Review.

Curr Pharm Des 2020 May 23. Epub 2020 May 23.

Halal Research Center of IRI, FDA, Tehran. Iran.

There is an increasing number of therapeutic agents being developed for the treatment of pulmonary artery hypertension (PAH) which is a condition characterized by raised pulmonary artery pressure and right heart failure. Despite our better understanding of the pathophysiology of PAH, the treatment outcomes are still suboptimal. There is growing evidence suggesting the role of increases in the levels of aldosterone, which is a mineralocorticoid hormone, in the pathophysiology of PAH; however, the extent to which hyperaldosteronism is associated with PAH in patients is unclear. Read More

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http://dx.doi.org/10.2174/1381612826666200523171137DOI Listing
May 2020
3.452 Impact Factor

Temporal estimates of case-fatality rate for COVID-19 outbreaks in Canada and the United States.

CMAJ 2020 06 22;192(25):E666-E670. Epub 2020 May 22.

Agent-Based Modelling Laboratory (Abdollahi, Moghadas), York University, Toronto, Ont.; Department of Pathology and Laboratory Medicine (Champredon), Western University, London, Ont.; Canadian Center for Vaccinology (Langley), Dalhousie University, IWK Health Centre and Nova Scotia Health Authority (Langley), Halifax, NS; Center for Infectious Disease Modeling and Analysis ( Galvani), Yale School of Public Health, New Haven, Conn.

Background: Estimates of the case-fatality rate (CFR) associated with coronavirus disease 2019 (COVID-19) vary widely in different population settings. We sought to estimate and compare the COVID-19 CFR in Canada and the United States while adjusting for 2 potential biases in crude CFR.

Methods: We used the daily incidence of confirmed COVID-19 cases and deaths in Canada and the US from Jan. Read More

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http://dx.doi.org/10.1503/cmaj.200711DOI Listing

Characterization and Management of Juxtaglomerular Cell Tumor: Analysis of 9 Cases and Literature Review.

Balkan Med J 2020 May 22. Epub 2020 May 22.

Department of Urology, the First Affiliated Hospital of Sun Yat-Sen University, Guangzhou, China.

Aims: Juxtaglomerular cell tumor (JGCT) is a rare kidney tumor. This study aimed to report the clinic features of JGCT and our treatment experience.

Methods: The medical records of 9 JGCT patients treated in our hospital from 1997 to 2017 were retrospectively reviewed. Read More

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http://dx.doi.org/10.4274/balkanmedj.galenos.2020.2019.12.79DOI Listing

Sensitivity of total body electrical resistance measurements in detecting extracellular volume expansion induced by infusion of NaCl 0.9.

Eur J Clin Nutr 2020 May 18. Epub 2020 May 18.

Department of Internal Medicine, Rijnstate Hospital, Arnhem, The Netherlands.

Background: Fluid balance management in hospitalized patients is hampered by the limited sensitivity of currently available tools. The aim of this study was to assess the sensitivity of total body electrical resistance (TBER) measurements for the detection of extracellular volume (ECV) expansion.

Methods: TBER and plasma resistivity (ρ) were measured during a 4-h infusion of NaCl 0. Read More

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http://dx.doi.org/10.1038/s41430-020-0655-yDOI Listing

Adrenal Vein Sampling to Distinguish Between Unilateral and Bilateral Primary Hyperaldosteronism: To ACTH Stimulate or Not?

J Clin Med 2020 May 13;9(5). Epub 2020 May 13.

Department of Surgery and Stanford Cancer Institute, Stanford University School of Medicine, Stanford, CA 94305, USA.

The aim of this study is to determine the accuracy of adrenal vein sampling (AVS) with and without adrenocorticotropic hormone (ACTH) stimulation to distinguish between unilateral and bilateral primary hyperaldosteronism (PA). Retrospective analysis of a prospective database from a referral center between 1984 and 2009, 76 patients had simultaneous cannulation of bilateral adrenal veins and AVS with and without ACTH stimulation. All patients had adrenalectomies. Read More

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http://dx.doi.org/10.3390/jcm9051447DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7291054PMC

Safety of outpatient adrenalectomy across 3 minimally invasive approaches at 2 academic medical centers.

Surgery 2020 May 11. Epub 2020 May 11.

Department of Surgery, University of Alabama at Birmingham, AL.

Background: Outpatient adrenalectomy has the potential to decrease costs, improve inpatient capacity, and decrease patient exposure to hospital-acquired conditions. Still, the practice has yet to be widely adopted and current studies demonstrating the safety of outpatient adrenalectomy are limited by sample size, extensive exclusion criteria, and no comparison to inpatient cases. We aimed to study the characteristics and safety of outpatient adrenalectomy using the largest such sample to date across 2 academic medical centers and 3 minimally invasive approaches. Read More

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http://dx.doi.org/10.1016/j.surg.2020.03.026DOI Listing

Clinical efficacy of potassium canreonate-canrenone in sinus rhythm restoration among patients with atrial fibrillation - a protocol of a pilot, randomized, double -blind, placebo-controlled study (CANREN-AF trial).

Trials 2020 May 12;21(1):397. Epub 2020 May 12.

National Institute of Cardiology, ul. Alpejska 42, Warsaw, 04-628, Poland.

Background: Atrial fibrillation (AF) is the most frequent cardiac arrhythmia which increases the risk of thromboembolic complications and impairs quality of life. An important part of a therapeutic approach for AF is sinus rhythm restoration. Antiarrhythmic agents used in pharmacological cardioversion have limited efficacy and potential risk of proarrhythmia. Read More

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http://dx.doi.org/10.1186/s13063-020-04277-3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7218584PMC

Preoperative non-stimulated adrenal venous sampling index for predicting outcomes of adrenalectomy for unilateral primary aldosteronism.

J Formos Med Assoc 2020 Jul 5;119(7):1185-1192. Epub 2020 May 5.

Department of Medical Imaging, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, Taiwan; Department and Graduate Institute of Forensic Medicine, National Taiwan University College of Medicine, Taipei, Taiwan. Electronic address:

Background/purpose: The aim of this study was to predict outcomes of adrenalectomy for unilateral primary aldosteronism (PA) using non-stimulated adrenal venous sampling (AVS) indices and the standardized Primary Aldosteronism Surgical Outcome (PASO) criteria.

Methods: Patients with unilateral PA who underwent adrenalectomy based on non-stimulated AVS and had follow-up data regarding surgical outcomes between 2011 and 2016 were enrolled. Demographic data and non-stimulated AVS indices, including lateralization index (LI) and contralateral suppression, were collected for analysis. Read More

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http://dx.doi.org/10.1016/j.jfma.2020.04.016DOI Listing
July 2020
1.695 Impact Factor

Primary Aldosteronism: Where Are We Now? Where to from Here?

Horm Metab Res 2020 Jun 4;52(6):459-466. Epub 2020 May 4.

Hudson Institute of Medical Research and Monash University, Clayton, Victoria, Australia.

The past nine years have seen major advances in establishing the etiology of unilateral primary aldosteronism, and very possibly that of bilateral hyperaldosteronism, in response to somatic mutations in aldosterone synthase expressing cells. Though there have been important advances in the management of primary aldosteronism, in small but convincing studies, they represent minor changes to current guidelines. What has been totally absent is consideration of the public health issue that primary aldosterone represents, and the public policy issues that would be involved in addressing the disorder. Read More

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http://dx.doi.org/10.1055/a-1120-8623DOI Listing

Pseudohypoaldosteronism Type 1 Newborn Patient with a Novel Mutation in .

J Pediatr Intensive Care 2020 Jun 7;9(2):145-148. Epub 2020 Jan 7.

Department of Pediatrics, Faculty of Medicine, University of Harran, Sanliurfa, Turkey.

Pseudohypoaldosteronism is a rare disease characterized by resistance to aldosterone-targeted organs, hyponatremia, hyperkalemia, metabolic acidosis, and severe salt loss in hyperaldosteronism. Homozygous mutations in , and genes were found to be responsible for the etiology. About 80 cases with molecular basis have been reported to date. Read More

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http://dx.doi.org/10.1055/s-0039-1700950DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7186019PMC

Performance of the unilateral AV/IVC index in primary hyperaldosteronism subtype prediction: A validation study in a single tertiary centre.

Clin Endocrinol (Oxf) 2020 Apr 29. Epub 2020 Apr 29.

Endocrinology and Metabolism Unit, Division of Internal Medicine, Faculty of Medicine, Prince of Songkla University, Hat Yai, Thailand.

Background: Failure of cannulation of the right adrenal vein is frequent during AVS for investigation of primary aldosteronism (PA). The aldosterone:cortisol ratio of either adrenal vein compared with the inferior vena cava (AV/IVC index) has been proposed to differentiate between unilateral and bilateral disease, and aid in lateralization of unilateral disease.

Methods: Sixty-two patients with unilateral or bilateral PA identified by either successful bilateral (45 patients) or unilateral (17 patients) adrenal vein cannulation, and with biochemical remission following surgery were enrolled into the analysis. Read More

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http://dx.doi.org/10.1111/cen.14210DOI Listing

EXPLORATION OF THE SEATED SALINE SUPPRESSION TEST FOR THE DIAGNOSIS OF PRIMARY ALDOSTERONISM IN CHINESE POPULATION.

Endocr Pract 2020 Apr 27. Epub 2020 Apr 27.

From: Department of Endocrinology and Metabolism, Adrenal Center, West China Hospital, Sichuan University, Chengdu, Sichuan, 610041, China.

We prospectively investigated the accuracy of the seated saline suppression test (SSST) in 113 patients with hypertension (including 93 PA and 20 essential hypertension (EH)) in the Department of Endocrinology and Metabolism. Each patient underwent an recumbent saline suppression test (RSST) and SSST. The accuracy of the SSST for a confirmative primary aldosteronism (PA) diagnosis and subtype classification was evaluated and compared with the RSST. Read More

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http://dx.doi.org/10.4158/EP-2020-0064DOI Listing
April 2020
2.811 Impact Factor

Disease monitoring of Primary Aldosteronism.

Best Pract Res Clin Endocrinol Metab 2020 Mar 10;34(2):101417. Epub 2020 Mar 10.

Clinica dell'Ipertensione Arteriosa, Department of Medicine - DIMED, University of Padua, Italy. Electronic address:

Primary aldosteronism (PA) is a highly prevalent cause of arterial hypertension featuring excess cardiovascular events. A timely diagnosis and treatment of PA cures hyperaldosteronism and can provide resolution or improvement of arterial hypertension, even when the latter is resistant to drug treatment. Accordingly, strategies to screen early and widely the hypertensive patients for PA by means of simplified diagnostic algorithms are justified. Read More

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http://dx.doi.org/10.1016/j.beem.2020.101417DOI Listing

A de novo CACNA1D missense mutation in a patient with congenital hyperinsulinism, primary hyperaldosteronism and hypotonia.

Channels (Austin) 2020 Dec;14(1):175-180

Pediatric Endocrinology Unit, Hospital Universitario la Fe, Valencia, Spain.

Congenital hyperinsulinemic hypoglycemia is the most frequent cause of persistent and recurrent hypoglycemia in the first years of life and in many patients rare genetic variants can be identified. Recently a case of congenital hyperinsulinemic hypoglycemia and a severe neurodevelopmental syndrome due to a mutation in the voltage-gated Cav1.3 Ca channel gene has been reported which required long-term treatment with diazoxide. Read More

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http://dx.doi.org/10.1080/19336950.2020.1761171DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7219433PMC
December 2020

[Progress of research on the role of CLCNKB gene in classical Bartter syndrome].

Zhonghua Yi Xue Yi Chuan Xue Za Zhi 2020 May;37(5):573-577

Department of Nephrology, Children's Hospital of Nanjing Medical University, Nanjing, Jiangsu 210000, China.

Bartter syndrome is an inherited metabolic disorder characterized by hypokalemic alkalosis and high rennin-angiotensin-aldosteronism which can occur at all ages but mainly in childhood. Classical Bartter syndrome is caused by loss-of-function variants in the gene encoding basolateral chloride channel ClC-Kb (CLCNKB), which is a common type of Bartter syndrome characterized with diverse clinical manifestations ranging from severe to very mild. This article reviews the function and mechanism of CLCNKB variants in Chinese population and the genotype-phenotype correlation of CLCNKB variants in classical Bartter syndrome. Read More

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http://dx.doi.org/10.3760/cma.j.issn.1003-9406.2020.05.020DOI Listing

Preeclampsia: A Diagnosis-Nondiagnosis That Is Too Easily Made: The Case of Primary Hyperaldosteronism.

Kidney Blood Press Res 2020 23;45(3):363-367. Epub 2020 Apr 23.

Néphrologie, Centre Hospitalier Le Mans, Le Mans, France.

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http://dx.doi.org/10.1159/000507116DOI Listing

Tetraparesis with Major Hypokalaemia and Rhabdomyolysis Induced by Chronic Liquorice Ingestion.

Eur J Case Rep Intern Med 2020 9;7(4):001375. Epub 2020 Mar 9.

Chronic ingestion of liquorice induces a syndrome with findings similar to those for primary hyperaldosteronism. This is characterized by hypokalaemia, hypertension, metabolic alkalosis and suppression of the renin-aldosterone system. We describe a 30-year-old woman who, with a plasma potassium level of 1. Read More

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http://dx.doi.org/10.12890/2020_001375DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7162572PMC

Age- and sex-specific reference ranges are needed for the aldosterone/renin ratio.

Clin Endocrinol (Oxf) 2020 Apr 19. Epub 2020 Apr 19.

Department of Endocrinology, Monash Health, Clayton, Vic., Australia.

Objective: Current Endocrine Society Clinical Practice Guidelines use a specific aldosterone/renin ratio (ARR) threshold to screen for primary aldosteronism (a treatable disease causing up to 15% of hypertension in primary care) in all patients. We sought to characterize demographic variations in the ARR, hypothesizing a need for age- and sex-specific reference ranges to improve the accuracy of the test.

Design: Retrospective cross-sectional analysis of ARR measurements at a single tertiary hospital from December 2016 to June 2018. Read More

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http://dx.doi.org/10.1111/cen.14199DOI Listing

Immunohistochemistry of the Human Adrenal CYP11B2 in Normal Individuals and in Patients with Primary Aldosteronism.

Horm Metab Res 2020 Jun 14;52(6):421-426. Epub 2020 Apr 14.

Department of Uro-Oncology, Saitama Medical University International Medical Center, Saitama, Japan.

The CYP11B2 enzyme is the terminal enzyme in the biosynthesis of aldosterone. Immunohistochemistry using antibodies against CYP11B2 defines cells of the adrenal ZG that synthesize aldosterone. CYP11B2 expression is normally stimulated by angiotensin II, but becomes autonomous in primary hyperaldosteronism, in most cases driven by recently discovered somatic mutations of ion channels or pumps. Read More

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http://dx.doi.org/10.1055/a-1139-2079DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7299743PMC

Bilateral Aldosterone Suppression in Patients With Right Unilateral Primary Aldosteronism and Review of the Literature.

J Endocr Soc 2020 Apr 12;4(4):bvaa033. Epub 2020 Mar 12.

Department of Endocrinology, Changi General Hospital, Singapore.

Introduction: Adrenal vein sampling (AVS) identifies unilateral primary aldosteronism but may occasionally show paradoxically low aldosterone-cortisol ratios bilaterally. Postulated reasons include venous anomalies, fluctuating aldosterone secretion, or superselective cannulation. We report our findings in patients who underwent repeat AVS and reviewed the current literature. Read More

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http://dx.doi.org/10.1210/jendso/bvaa033DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7138278PMC

Mineralocorticoid Receptor Antagonists in ESKD.

Clin J Am Soc Nephrol 2020 Jul 8;15(7):1047-1049. Epub 2020 Apr 8.

Division of Nephrology and Hypertension, Department of Internal Medicine, University of Utah Health Center, Salt Lake City, Utah

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http://dx.doi.org/10.2215/CJN.13221019DOI Listing

Salt Appetite and its Effects on Cardiovascular Risk in Primary Aldosteronism.

Horm Metab Res 2020 Jun 6;52(6):386-393. Epub 2020 Apr 6.

Medizinische Klinik und Poliklinik IV, LMU München, Munich, Germany.

First described in 1955 by Jerome W. Conn, primary aldosteronism (PA) today is well established as a relevant cause of secondary hypertension and accounts for about 5-10 % of hypertensives. The importance of considering PA is based on its deleterious target organ damage far beyond the effect of elevated blood pressure and on PA being a potentially curable form of hypertension. Read More

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http://dx.doi.org/10.1055/a-1116-2407DOI Listing

Fatal neonatal nephrocutaneous syndrome in 18 Roma children with EGFR deficiency.

J Dermatol 2020 Jun 6;47(6):663-668. Epub 2020 Apr 6.

Department of Pediatrics and Adolescent Medicine, General University Hospital, First Medical Faculty, Charles University, Prague, Czech Republic.

Epidermal growth factor receptor (EGFR) is a transmembrane glycoprotein with tyrosine-kinase signaling activity, involved in many cellular functions including cell growth and differentiation. Germ line loss-of-function mutations in EGFR lead to a severe neonatal skin disorder (Online Mendelian Inheritance in Man #131550). We report 18 premature Roma children from 16 families with birthweights ranging 440-1470 g and multisystem diseases due to the homozygous mutation c. Read More

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http://dx.doi.org/10.1111/1346-8138.15317DOI Listing

Nadir Aldosterone Levels After Confirmatory Tests Are Correlated With Left Ventricular Hypertrophy in Primary Aldosteronism.

Hypertension 2020 Jun 6;75(6):1475-1482. Epub 2020 Apr 6.

Clinical Researh Institute of Endocrinology and Metabolism, National Hospital Organization Kyoto Medical Center and Endocrine Center, Ijinkai Takeda General Hospital, Kyoto, Japan (M.N.).

Left ventricular hypertrophy (LVH) is often seen in patients with primary aldosteronism (PA), and the prevalence of LVH is reportedly higher among patients with PA than patients with essential hypertension. However, the correlation between aldosterone levels and LVH is undefined, and how aldosterone affects LVH in patients with PA remains unclear. We, therefore, retrospectively assessed a large PA database established by the multicenter JPAS (Japan Primary Aldosteronism Study) to reveal the factors associated with LVH in patients with PA without suspected autonomous cortisol secretion. Read More

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http://dx.doi.org/10.1161/HYPERTENSIONAHA.119.14601DOI Listing

Phenotypic profiling of mGlu knockout mice reveals new implications for neurodevelopmental disorders.

Genes Brain Behav 2020 Apr 4. Epub 2020 Apr 4.

Department of Pharmacology, Vanderbilt University, Nashville, Tennessee, USA.

Neurodevelopmental disorders are characterized by deficits in communication, cognition, attention, social behavior and/or motor control. Previous studies have pointed to the involvement of genes that regulate synaptic structure and function in the pathogenesis of these disorders. One such gene, GRM7, encodes the metabotropic glutamate receptor 7 (mGlu ), a G protein-coupled receptor that regulates presynaptic neurotransmitter release. Read More

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http://dx.doi.org/10.1111/gbb.12654DOI Listing

The Potential Role of Aldosterone-Producing Cell Clusters in Adrenal Disease.

Horm Metab Res 2020 Jun 30;52(6):427-434. Epub 2020 Mar 30.

Departments of Molecular, Integrative Physiology and Internal Medicine, University of Michigan, Ann Arbor, MI, USA.

Primary aldosteronism (PA) is the most common cause of secondary hypertension. The hallmark of PA is adrenal production of aldosterone under suppressed renin conditions. PA subtypes include adrenal unilateral and bilateral hyperaldosteronism. Read More

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http://dx.doi.org/10.1055/a-1128-0421DOI Listing

Cranial Fossa Volume and Morphology Development in Apert Syndrome.

Plast Reconstr Surg 2020 04;145(4):790e-802e

Beijing, People's Republic of China; New Haven, Conn.; Jacksonville, Fla.; and São Paulo, Brazil From the Chinese Academy of Medical Sciences, Peking Union Medical College, Plastic Surgery Hospital; Division of Plastic and Reconstructive Surgery, Yale School of Medicine; Division of Plastic and Reconstructive Surgery, Mayo Clinic Florida; and the Department of Plastic Surgery, University of São Paulo.

Background: Apert syndrome causes normal or enlarged intracranial volume overall as patients grow. This study aimed to trace the segmental anterior, middle, and posterior cranial fossae volume and structural morphology in these patients, to help discern a more focused and individualized surgical treatment plan for patients with Apert syndrome.

Methods: This study included 82 preoperative computed tomographic scans (Apert, n = 32; control, n = 50) divided into five age-related subgroups. Read More

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http://dx.doi.org/10.1097/PRS.0000000000006679DOI Listing

Primary Aldosteronism and Obstructive Sleep Apnea: Casual Association or Pathophysiological Link?

Horm Metab Res 2020 Jun 27;52(6):366-372. Epub 2020 Mar 27.

Division of Internal Medicine 4 and Hypertension Unit, Department of Medical Sciences, University of Torino, Torino, Italy.

The coexistence of aldosterone oversecretion and obstructive sleep apnea is frequently observed, especially in patients with resistant hypertension, obesity, and metabolic syndrome. Since aldosterone excess and sleep apnea are both independently associated with an increased risk of cardiovascular disease, to investigate whether their coexistence might be attributed to common predisposing conditions, such as metabolic disorders, or to an actual pathophysiological interconnection appears of great importance. Fluid overload and metabolic abnormalities relating to aldosterone oversecretion may be implicated in obstructive sleep apnea development. Read More

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http://dx.doi.org/10.1055/a-1133-7255DOI Listing

The risk factors for hepatic steatosis in patients with primary aldosteronism.

Endocr J 2020 Jun 24;67(6):623-629. Epub 2020 Mar 24.

Department of Rheumatology, Endocrinology and Nephrology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, 060-8648 Sapporo, Japan.

Patients with primary aldosteronism (PA) are complicated by metabolic syndrome more frequently than those without PA. Hyperaldosteronism has been reported to be associated with a higher prevalence of non-alcoholic fatty liver disease (NAFLD). We aimed to clarify the risk factors for hepatic steatosis in the two subtypes of PA, comparing the status of hepatic steatosis in each of these subtypes. Read More

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http://dx.doi.org/10.1507/endocrj.EJ19-0600DOI Listing

Imaging CXCR4 expression in patients with suspected primary hyperaldosteronism.

Eur J Nucl Med Mol Imaging 2020 Mar 23. Epub 2020 Mar 23.

Division of Nuclear Medicine, Department of Biomedical Imaging and Image-guided Therapy, Medical University of Vienna, Vienna, Austria.

Purpose: It is challenging to differentiate unilateral aldosterone-producing adenoma (APA) from bilateral idiopathic adrenal hyperplasia (IAH) and nonfunctional adrenal adenoma (NFA) in primary aldosteronism (PA). In a first primarily ex vivo study detection, CXC chemokine receptor type 4 (CXCR4) expression has been shown to be a valuable tool for the detection of APA. In this study, we aimed to clinically evaluate CXCR4 imaging with Ga-pentixafor PET/CT for detecting APA. Read More

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http://dx.doi.org/10.1007/s00259-020-04722-0DOI Listing

Surgical approach to patients with primary aldosteronism.

Gland Surg 2020 Feb;9(1):25-31

Department of Surgery, Section of Endocrine Surgery, Columbia University Medical Center, New York, NY, USA.

Primary hyperaldosteronism (PA) is one of the most common causes of hypertension that is amenable to surgical cure. Once a patient has a biochemical diagnosis of PA, workup should proceed with tumor lateralization to determine whether the patient has unilateral or bilateral disease. Tumor lateralization can be done with noninvasive imaging such as a CT or MRI. Read More

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http://dx.doi.org/10.21037/gs.2019.10.19DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7082277PMC
February 2020