10,829 results match your criteria Conn Syndrome

A systematic review of pathophysiology and management of familial hyperaldosteronism type 1 in pregnancy.

Endocrine 2021 May 27. Epub 2021 May 27.

Hypertension and Emergency Unit, Department of Medicine - DIMED, University of Padua, Padova, Italy.

Purpose: Familial hyperaldosteronism type 1 (FH-1) is a rare autosomal dominant form of primary aldosteronism, which features a marked phenotypic heterogeneity, ranging from mild to severe forms of arterial hypertension that can be complicated by stroke and cardiovascular events at a young age. As affected patients usually reach the fertile age, transmission of the disease to offspring is common. Notwithstanding this, reports of FH-1 in pregnancy are limited and there is a lack of treatment guidelines. Read More

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Homeobox A5 activates p53 pathway to inhibit proliferation and promote apoptosis of adrenocortical carcinoma cells by inducing Aldo-Keto reductase family 1 member B10 expression.

Bioengineered 2021 12;12(1):1964-1975

Departments of Endocrinology, Chongqing General Hospital, University of Chinese Academy of Sciences, Chongqing, China.

Aldo-Keto Reductase Family 1 Member B10 (AKR1B10) and Homeobox A5 (HOXA5) are both down-regulated in adrenocortical carcinoma (ACC), and HOXA5 is predicted to bind to the promoter of AKR1B10. We aimed to investigate whether HOXA5 could bind to AKR1B10 to regulate ACC cells proliferation and apoptosis. The expression of AKR1B10 and HOXA5 in ACC patients and the relationship of their expression between ACC prognosis were evaluated by searching database. Read More

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December 2021

Single-Center Prospective Cohort Study on the Histopathology, Genotype, and Postsurgical Outcomes of Patients With Primary Aldosteronism.

Hypertension 2021 May 24:HYPERTENSIONAHA12117348. Epub 2021 May 24.

Medizinische Klinik und Poliklinik IV, Klinikum der Universitaät Muünchen, Ludwig-Maximilians-Universität München, Germany. (L.S.M., L.H., I.K., M.B., M.R., T.A.W.).

Unilateral forms of primary aldosteronism are usually surgically treated to remove the source of aldosterone excess. After adrenalectomy, aldosteronism persists in some patients indicating abnormal aldosterone production from the unresected gland. Our objective was to investigate histopathology, genotype, and postsurgical outcomes in a 3-year prospective cohort of surgically treated patients for primary aldosteronism (from 2016 to 2018). Read More

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[Performance Verification of Plasma Renin and Aldosterone Examination with Chemiluminescence Immunoassay and Its Screening Efficacy for Primary Aldosteronism].

Sichuan Da Xue Xue Bao Yi Xue Ban 2021 May;52(3):472-476

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

Objective: To evaluate the performance of chemiluminescence immunoassay (CLIA) in examining renin and aldosterone and to determine its value for screening for primary aldosteronism (PA).

Methods: According to the relevant documents of Clinical and Laboratory Standards Institute (CLSI), we verified the precision, linear range and carryover rate of examining renin and aldosterone with CLIA. The study included 91 suspected PA patients, using two methods, CLIA and radioimmunoassay (RIA), to examine renin and aldosterone levels in order to compare the correlation between the two methods and their value for PA screening. Read More

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Pathology of Aldosterone Biosynthesis and its Action.

Tohoku J Exp Med 2021 ;254(1):1-15

Department of Pathology, Tohoku University, Graduate School of Medicine.

Aldosterone plays pivotal roles in renin-angiotensin-aldosterone system in order to maintain the equilibrium of liquid volume and electrolyte metabolism. Aldosterone action is mediated by both mineralocorticoid receptor and 11β-hydroxysteroid dehydrogenase type 2 (11β-HSD2). Its excessive actions directly induced tissue injuries in its target organs such as myocardial and vascular fibrosis in addition to chronic kidney diseases. Read More

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January 2021

Performance of the Aldosterone to Renin Ratio as a Screening Test for Primary Aldosteronism.

J Clin Endocrinol Metab 2021 Jul;106(8):2423-2435

Department of Medicine (Division of Nephrology) and the Ottawa Hospital Research Institute, University of Ottawa, Ottawa, ON,Canada.

Context: The aldosterone to renin ratio (ARR) is the guideline-recommended screening test for primary aldosteronism. However, there are limited data in regard to the diagnostic performance of the ARR.

Objective: To evaluate the sensitivity and specificity of the ARR as a screening test for primary aldosteronism. Read More

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Profiling beneficial and potential adverse effects of MeCP2 overexpression in a hypomorphic Rett syndrome mouse model.

Genes Brain Behav 2021 May 18:e12752. Epub 2021 May 18.

Department of Pharmacology and Warren Center for Neuroscience Drug Discovery, Vanderbilt University, Nashville, Tennessee, USA.

De novo loss-of-function mutations in methyl-CpG-binding protein 2 (MeCP2) lead to the neurodevelopmental disorder Rett syndrome (RTT). Despite promising results from strategies aimed at increasing MeCP2 levels, additional studies exploring how hypomorphic MeCP2 mutations impact the therapeutic window are needed. Here, we investigated the consequences of genetically introducing a wild-type MECP2 transgene in the Mecp2 R133C mouse model of RTT. Read More

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Urinary sodium/potassium ratio as a screening tool for hyperaldosteronism in men with hypertension.

Hypertens Res 2021 May 17. Epub 2021 May 17.

Center for Epidemiologic Research in Asia, Shiga University of Medical Science, Shiga, Japan.

Among individuals with hypertension, the prevalence of secondary hypertension has been reported to be ≈10%. More than half of individuals with secondary hypertension have associated hyperaldosteronism. However, given the current clinical environment, these patients often remain undiagnosed. Read More

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Clinical and Genetic Features in 31 Serial Chinese Children With Gitelman Syndrome.

Front Pediatr 2021 29;9:544925. Epub 2021 Apr 29.

Department of Nephrology, The Children's Hospital of Zhejiang University School of Medicine, Hangzhou, China.

Gitelman syndrome (GS, OMIM 263800) is a genetic congenital tubulopathy associated with salt loss, which is characterized by hypokalemic metabolic toxicity, hypocalciuria, and hypomagnesemia. GS, which is typically detected in adolescence or adulthood, has long been considered a benign tubular lesion; however, the disease is associated with a significant decrease in the quality of life. In this study, we assessed the genotype-phenotype correlations based on the medical histories, clinical symptoms, laboratory test results, and whole-exome sequencing profiles from pediatric patients with GS. Read More

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Eplerenone as a treatment for resistant hypertension in pregnancy.

Obstet Med 2021 Mar 24;14(1):35-38. Epub 2019 Mar 24.

Obstetric Medicine and Endocrinology, Mater Health, Brisbane, Queensland, Australia.

Mineralocorticoid receptor antagonists are highly effective in the management of resistant hypertension and primary hyperaldosteronism. Recent studies demonstrate that mineralocorticoid receptor antagonists significantly reduce blood pressure, severity of obstructive sleep apnoea and arterial stiffness in patients with resistant hypertension and moderate-severe obstructive sleep apnoea. Eplerenone is a selective mineralocorticoid receptor antagonist that does not act as an androgen receptor blocker, thus reducing the risk of fetal anti-androgenic effects. Read More

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Risk factors for post-nephrectomy hypotension in pediatric patients.

Pediatr Nephrol 2021 May 14. Epub 2021 May 14.

Division of Nephrology and Rheumatology, National Center for Child Health and Development, 2-10-1, Okura, Setagaya-ku, Tokyo, 157-8535, Japan.

Background: Although hypotension is a life-threatening complication of nephrectomy in children, risk factors for its development remain unknown. We evaluated the incidence, clinical course, and associated risk factors of pediatric post-nephrectomy hypotension in an observational study.

Methods: This retrospective observational study included the clinical data of children who underwent nephrectomy in our center between 2002 and 2020. Read More

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Diagnosis and outpatient management of Gitelman syndrome from the first trimester of pregnancy.

BMJ Case Rep 2021 May 12;14(5). Epub 2021 May 12.

Emergency Admission Unit, Colchester General Hospital, Colchester, UK.

A 32-year-old woman presented with an incidental finding of hypokalaemia on routine bloods at 9 weeks of a second pregnancy, on a background of lifelong salt craving. Her previous pregnancy was uncomplicated. She had no previous significant medical or family history. Read More

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Clinical Evaluation of Assays for Plasma Renin Activity and Aldosterone Measurement by Liquid Chromatography-Tandem Mass Spectrometry.

J Appl Lab Med 2021 Apr;6(3):668-678

SAS Laboratory Endocrinology, St James University Hospital, Leeds, UK.

Background: Aldosterone and renin are pivotal hormones in the regulation of salt and water homeostasis and blood pressure. Measurement of renin and aldosterone in serum/plasma is essential for the investigation of primary hyperaldosteronism (PA) and monitoring of glucocorticoid replacement therapy.

Methods: We report 2 LC-MS/MS methods developed to measure aldosterone and plasma renin activity (PRA). Read More

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Mineralocorticoid Receptor Antagonists Eplerenone and Spironolactone Modify Adrenal Cortex Morphology and Physiology.

Biomedicines 2021 Apr 20;9(4). Epub 2021 Apr 20.

Department of Experimental Biology, Faculty of Medicine, University of Porto, 4200-319 Porto, Portugal.

Mineralocorticoid receptor antagonists (MRAs) are a class of anti-hypertensive drugs that act by blocking aldosterone action. The aim of this study was to evaluate whether the MRAs spironolactone and eplerenone influence adrenal cortical physiology and morphology. Spontaneous hypertensive rats (SHR, = 18) and normotensive rats (WKY, = 18) were randomly exposed to a daily dose of spironolactone ( = 6), eplerenone ( = 6), or no drug ( = 6) over 28 days. Read More

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Update on Genetics of Primary Aldosteronism.

Biomedicines 2021 Apr 10;9(4). Epub 2021 Apr 10.

Department of Molecular and Internal Medicine, Graduate School of Biomedical and Health Sciences, Hiroshima University, Hiroshima 734-8551, Japan.

Primary aldosteronism (PA) is the most common form of secondary hypertension, with a prevalence of 5-10% among patients with hypertension. PA is mainly classified into two subtypes: aldosterone-producing adenoma (APA) and bilateral idiopathic hyperaldosteronism. Recent developments in genetic analysis have facilitated the discovery of mutations in , , , , , , and in sporadic or familial forms of PA in the last decade. Read More

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Renal function in patients with intestinal failure receiving home parenteral support.

JPEN J Parenter Enteral Nutr 2021 Apr 29. Epub 2021 Apr 29.

Department of Gastroenterology and Hepatology, Rigshospitalet, Copenhagen, Denmark.

Background: Progressive renal impairment, given by an annual decline in estimated glomerular filtration rate (eGFR), has been described in patients with intestinal failure (IF) receiving home parenteral support (HPS). The objective of this study was to examine changes in eGFR over 5 years following initiation of HPS treatment and to identify potential risk factors for loss of renal function.

Method: This retrospective database study investigates eGFR changes in nonmalignant IF patients discharged with HPS from Rigshospitalet, Copenhagen, in an 8-year period. Read More

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Should Adrenal Venous Sampling Be Performed in PA Patients Without Apparent Adrenal Tumors?

Front Endocrinol (Lausanne) 2021 12;12:645395. Epub 2021 Apr 12.

Department of Endocrinology and Metabolism, National Hospital Organization Kyoto Medical Center, Kyoto, Japan.

Introduction: Some aldosterone-producing micro-adenomas cannot be detected through image inspection. Therefore, adrenal venous sampling (AVS) is often performed, even in primary aldosteronism (PA) patients who have no apparent adrenal tumors (ATs) on imaging. In most of these cases, however, the PA is bilateral. Read More

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Aldosterone up-regulates basolateral Na -K -2Cl cotransporter-1 to support enhanced large-conductance K channel-mediated K secretion in rat distal colon.

FASEB J 2021 05;35(5):e21606

Department of Biochemistry, West Virginia University School of Medicine, Morgantown, WV, USA.

Na -K -2Cl cotransporter-1 (NKCC1) facilitates basolateral K and Cl uptake, supporting their efflux across mucosal membranes of colonic epithelial cells. NKCC1 activity has also been shown to be critical for electrogenic K secretion induced by aldosterone, which is known to stimulate large-conductance K (BK) channel expression in mucosal membranes. This study was aimed to (1) identify whether aldosterone enhances NKCC1 expression specifically to support BK-mediated K secretion and (2) to determine whether increased NKCC1 supports electrogenic Cl secretion in parallel to K secretion. Read More

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GENETICS IN ENDOCRINOLOGY: Impact of race and sex on genetic causes of aldosterone-producing adenomas.

Eur J Endocrinol 2021 May 21;185(1):R1-R11. Epub 2021 May 21.

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

Primary aldosteronism (PA) is a common cause of secondary hypertension. Recent technological advances in genetic analysis have provided a better understanding of the molecular pathogenesis of this disease. The application of next-generation sequencing has resulted in the identification of somatic mutations in aldosterone-producing adenoma (APA), a major subtype of PA. Read More

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Harefuah 2021 Apr;160(4):221-225

Nephrology and Hypertension Institute, "Samson" Assuta University Hospital, Ashdod, Israel.

Introduction: This is a case study of a thirty-five year old woman with a past medical history of anxiety disorder and hypertension which has been elevated up to 180/100 mmHg during the previous year. She had no cardiovascular risk factors or family history of hypertension. Her high blood pressure was initially attributed to emotional stress, however, she was later referred for additional evaluation for secondary causes of hypertension. Read More

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Acute Severe Renal Artery Stenosis Presenting as Acute Kidney Injury with Severe Hypertension, and Active Urine Sediment.

Can J Cardiol 2021 Apr 20. Epub 2021 Apr 20.

Division of Nephrology, Department of Medicine, The Ottawa Hospital, University of Ottawa. Electronic address:

We present a case of severe renal artery stenosis that mimicked a rapidly progressive glomerulonephritis with acute kidney injury, active urine sediments and severe hypertension. Simultaneous presence of secondary hyperaldosteronism and hypokalemia prompted renal angiogram and subsequent renal artery angioplasty and stenting, thus leading to rapid resolution of acute kidney injury, proteinuria, and hypertension. Our case emphasizes the importance and benefits of prompt diagnosis and revascularization of acute severe renal artery stenosis in a patient with history of atherosclerotic renovascular disease presenting with sudden onset of severe hypertension and acute kidney injury with active urine sediment mimicking rapidly progressive glomerulonephritis. Read More

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Adrenal Incidentaloma.

Electron Kebebew

N Engl J Med 2021 Apr;384(16):1542-1551

From the Division of General Surgery, Department of Surgery, Stanford University School of Medicine, Stanford, CA.

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Enhanced Ca signaling, mild primary aldosteronism, and hypertension in a familial hyperaldosteronism mouse model ( ).

Proc Natl Acad Sci U S A 2021 Apr;118(17)

Department of Nephrology, Charité-Universitätsmedizin Berlin, 10115 Berlin, Germany;

Gain-of-function mutations in the gene (encoding the T-type calcium channel Ca3.2) cause autosomal-dominant familial hyperaldosteronism type IV (FH-IV) and early-onset hypertension in humans. We used CRISPR/Cas9 to generate knockin mice as a model of the most common FH-IV mutation, along with corresponding knockout mice ( ). Read More

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Effect of Increased Potassium Intake on Adrenal Cortical and Cardiovascular Responses to Angiotensin II: A Randomized Crossover Study.

J Am Heart Assoc 2021 May 19;10(9):e018716. Epub 2021 Apr 19.

Department of Medicine Amager Hvidovre Hospital in Glostrup University of Copenhagen Glostrup Denmark.

Background Increased potassium intake lowers blood pressure in patients with hypertension, but increased potassium intake also elevates plasma concentrations of the blood pressure-raising hormone aldosterone. Besides its well-described renal effects, aldosterone is also believed to have vascular effects, acting through mineralocorticoid receptors present in endothelial and vascular smooth muscle cells, although mineralocorticoid receptors-independent actions are also thought to be involved. Methods and Results To gain further insight into the effect of increased potassium intake and potassium-stimulated hyperaldosteronism on the human cardiovascular system, we conducted a randomized placebo-controlled double-blind crossover study in 25 healthy normotensive men, where 4 weeks treatment with a potassium supplement (90 mmol/day) was compared with 4 weeks on placebo. Read More

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Time-Dependent Risk of Atrial Fibrillation in Patients With Primary Aldosteronism After Medical or Surgical Treatment Initiation.

Hypertension 2021 Jun 19;77(6):1964-1973. Epub 2021 Apr 19.

Department of Internal Medicine (N.H., H.L., S.L., Y.R.), Yonsei University College of Medicine, Seoul.

[Figure: see text]. Read More

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Small intestinal immunopathology and GI-associated antibody formation in hereditary alpha-tryptasemia.

J Allergy Clin Immunol 2021 Apr 15. Epub 2021 Apr 15.

Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, Md. Electronic address:

Background: Hereditary alpha-tryptasemia (HαT) is characterized by elevated basal serum tryptase due to increased copies of the TPSAB1 gene. Individuals with HαT frequently present with multisystem complaints, including anaphylaxis and seemingly functional gastrointestinal (GI) symptoms.

Objective: We sought to determine the prevalence of HαT in an irritable bowel syndrome cohort and associated immunologic characteristics that may distinguish patients with HαT from patients without HαT. Read More

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Prevalence, Subtype Classification, and Outcomes of Treatment of Primary Aldosteronism: A Prospective Study in China.

Endocr Pract 2021 May 14;27(5):478-483. Epub 2020 Dec 14.

Center of Laboratory Medicine, State Key Laboratory of Cardiovascular Disease, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, People's Republic of China. Electronic address:

Objective: To investigate the prevalence of primary aldosteronism (PA) among participants with hypertension, evaluate the concordance of PA classification between adrenal computed tomography and adrenal venous sampling, and compare the outcomes of surgery and medication for unilateral PA.

Methods: A prospective study was conducted among all inpatients with hypertension (n = 7594) at the National Center for Cardiovascular Diseases, China, from May 2016 to April 2018.

Results: Of the 7594 participants, 8. Read More

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Masking by hypokalemia-primary aldosteronism with undetectable aldosterone.

Clin Kidney J 2021 Apr 7;14(4):1269-1271. Epub 2020 Oct 7.

Stanford Hypertension Center, Department of Medicine, Stanford University School of Medicine, Stanford, CA, USA.

Primary aldosteronism is the most common cause of secondary hypertension; however, the dynamic regulation of aldosterone by potassium is less well studied and current diagnostic recommendations are imprecise. We describe a young man who presented with resistant hypertension and severe hypokalemia. The workup initially revealed undetectable aldosterone despite acute potassium repletion. Read More

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Hypokalemia-Induced Rhabdomyolysis Caused by Adrenal Tumor-Related Primary Aldosteronism: A Report of 2 Cases.

Am J Case Rep 2021 Apr 10;22:e929758. Epub 2021 Apr 10.

Division of Urology, Department of Surgery, Cathay General Hospital, Taipei, Taiwan.

BACKGROUND Primary aldosteronism, also known as Conn's syndrome, is a clinical condition caused by excessive production of aldosterone. The classic presenting signs of primary aldosteronism are hypertension and hypokalemia. However, rhabdomyolysis induced by severe hypokalemia is a rare manifestation of primary aldosteronism. Read More

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Association of aldosterone and blood pressure with the risk for cardiovascular events after treatments in primary aldosteronism.

Atherosclerosis 2021 05 29;324:84-90. Epub 2021 Mar 29.

Clinical Research Institute of Endocrinology and Metabolism, Kyoto Medical Center, National Hospital Organization, Endocrine Center, Ijinkai Takeda General Hospital, Kyoto, Japan.

Background And Aims: We used a dataset from a Japanese nationwide registry of patients with primary aldosteronism, to determine which of the parameters of hyperaldosteronism and blood pressure before or after treatments for primary aldosteronism (i.e., surgical adrenalectomy or a medication treatment) are important in terms of cardiovascular prognosis. Read More

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