9,984 results match your criteria Conn Syndrome


Sex-hormone producing adrenal tumors causing behavioral changes as the sole clinical sign in 3 cats.

Can Vet J 2019 Mar;60(3):305-310

Department of Clinical Sciences, College of Veterinary Medicine, Cornell University, Ithaca, New York 14853, USA (Sumner, Miller, Hayes); Department of Medicine and Epidemiology, School of Veterinary Medicine, University of California-Davis, Davis, California 95616, USA (Hulsebosch); Medvet Columbus, Medical and Cancer Centers for Pets, Worthington, Ohio 43085, USA (Dudley).

Three neutered cats with adrenocortical tumors that were presented with behavioral changes but no evidence of hyperaldosteronism or hypercortisolism are described. All 3 cats had resolution of their clinical signs following adrenalectomy. For neutered cats presenting with behavior changes, a sex-hormone secreting adrenal tumor should be considered as a differential diagnosis. Read More

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Gitelman Syndrome: A Rare Cause of Seizure Disorder and a Systematic Review.

Case Rep Med 2019 5;2019:4204907. Epub 2019 Feb 5.

Resident Physician, Abington Hospital-Jefferson Health, Abington, PA, USA.

Gitelman syndrome is one of the few inherited causes of metabolic alkalosis due to salt losing tubulopathy. It is caused by tubular defects at the level of distal convoluted tubules, mimicking a thiazide-like tumor. It usually presents in late childhood or in teenage as nonspecific weakness, fatigability, polyuria, and polydipsia but very rarely with seizures. Read More

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http://dx.doi.org/10.1155/2019/4204907DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6379858PMC
February 2019

PTH Modulation by Aldosterone and Angiotensin II is Blunted in Hyperaldosteronism and Rescued by Adrenalectomy.

J Clin Endocrinol Metab 2019 Mar 13. Epub 2019 Mar 13.

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

Context: Accumulating evidences suggest a link between adrenocortical zona glomerulosa and parathyroid gland through mechanisms that remain unexplored.

Objectives: To test the hypothesis that in vivo Angiotensin (Ang) II blockade affects parathyroid hormone (PTH) secretion in hypertensive patients and that aldosterone and Ang II directly stimulate PTH secretion ex vivo.

Design And Setting: We investigated the changes of serum PTH levels induced by oral captopril (50 mg) administration in patients with primary essential hypertension (EH) and with primary aldosteronism (PA) due to bilateral adrenal hyperplasia (BAH) or to aldosterone-producing adenoma (APA), the latter before and after adrenalectomy. Read More

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https://academic.oup.com/jcem/advance-article/doi/10.1210/jc
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http://dx.doi.org/10.1210/jc.2019-00143DOI Listing
March 2019
1 Read

The Key Role of CT for Success of Adrenal Venous Sampling Illustrated by a Unique Clinical Case.

High Blood Press Cardiovasc Prev 2019 Mar 11. Epub 2019 Mar 11.

Institute of Radiology, Department of Medicine, DIMED, University of Padua, Padua, Italy.

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http://link.springer.com/10.1007/s40292-019-00310-y
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http://dx.doi.org/10.1007/s40292-019-00310-yDOI Listing
March 2019
1 Read

Screening in adrenal tumors.

Curr Opin Oncol 2019 Mar 5. Epub 2019 Mar 5.

Department of Medicine, Division of Endocrinology.

Purpose Of Review: Adrenal tumors are mostly encountered as incidentalomas in patients undergoing imaging not performed for suspected adrenal disease; although the majority are benign and nonfunctioning, malignant tumors and functioning tumors need to be excluded. The purpose of this review is to highlight recent advances in the evaluation of adrenal tumors.

Recent Findings: As a consequence of increased use of technologically improved imaging techniques, the detection of adrenal incidentalomas has continued to increase. Read More

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http://dx.doi.org/10.1097/CCO.0000000000000528DOI Listing
March 2019
2 Reads

Links between aldosterone excess and metabolic complications: A comprehensive review.

Diabetes Metab 2019 Feb 27. Epub 2019 Feb 27.

Klinik für Endokrinologie, Diabetologie und Klinische Ernährung, UniversitätsSpital Zürich, Zürich, Switzerland.

Shortly after the first description of primary aldosteronism (PA) appeared in the 1950s by Jerome Conn, an association of the condition with diabetes mellitus was documented. However, a clear pathophysiological interrelationship linking the two entities has yet to be established. Nevertheless, so far, many mechanisms contributing to insulin resistance and dysregulation of glucose uptake have been described. Read More

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http://dx.doi.org/10.1016/j.diabet.2019.02.003DOI Listing
February 2019
1 Read

A Novel Clinical Nomogram to Predict Bilateral Hyperaldosteronism in Chinese Patients with Primary Aldosteronism.

Clin Endocrinol (Oxf) 2019 Feb 28. Epub 2019 Feb 28.

Shanghai Key Laboratory for Endocrine Tumors, Shanghai Clinical Center for Endocrine and Metabolic Diseases, Key Laboratory for Endocrine and Metabolic Diseases of Chinese Health Ministry, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, 197 Ruijin 2nd Road, Shanghai, 200025, P.R.

Context: Adrenal venous sampling (AVS) is recommended as the gold standard for subtype classification in primary aldosteronism (PA); however, this approach has limited availability.

Objective: We aimed to develop a novel clinical nomogram to predict PA subtype based on routine variables, thereby reducing the number of candidates for AVS.

Patients And Method: Patients were randomly divided into a training set (n=185) and a validation set (n=79). Read More

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http://dx.doi.org/10.1111/cen.13962DOI Listing
February 2019
3 Reads

Interrogating the Genetic Determinants of Tourette's Syndrome and Other Tic Disorders Through Genome-Wide Association Studies.

Am J Psychiatry 2019 Mar;176(3):217-227

The Psychiatric and Neurodevelopmental Genetics Unit, Center for Genomic Medicine, Department of Psychiatry, Massachusetts General Hospital, Boston (Yu, Illmann, Osiecki, Smoller, Pauls, Neale, Scharf); the Stanley Center for Psychiatric Research, Broad Institute of MIT and Harvard, Cambridge, Mass. (Yu, Neale, Scharf); the Semel Institute for Neuroscience and Human Behavior, David Geffen School of Medicine, University of California, Los Angeles (Sul, Huang, Zelaya, Ophoff, Freimer, Coppola); the Department of Psychiatry and Biobehavioral Sciences, University of California, Los Angeles (Sul, Huang, Zelaya, Freimer, Coppola); the Department of Molecular Biology and Genetics, Democritus University of Thrace, Xanthi, Greece (Tsetsos); the Department of Biological Sciences, Purdue University, West Lafayette, Ind. (Tsetsos, Paschou); deCODE Genetics/Amgen, Reykjavik, Iceland (Nawaz, H. Stefansson, K. Stefansson); the Bioinformatics Interdepartmental Program, University of California, Los Angeles (Huang, Zelaya); the Department of Psychiatry, University of California, San Francisco (Darrow); the Department of Psychiatry, UCSF Weill Institute for Neurosciences, University of California, San Francisco (Hirschtritt, Willsey); the Department of Psychiatry, Massachusetts General Hospital, Boston (Greenberg, Roffman, Buckner); the Clinic of Psychiatry, Social Psychiatry, and Psychotherapy, Hannover Medical School, Hannover, Germany (Muller-Vahl); the Institute of Human Genetics, Hannover Medical School, Hannover, Germany (Stuhrmann); McGill University Health Center, University of Montreal, McGill University Health Centre, Montreal (Dion); the Montreal Neurological Institute, Department of Neurology and Neurosurgery, McGill University, Montreal (Rouleau); the Department of Psychiatry and Psychotherapy, Medical University Vienna, Vienna (Aschauer, Stamenkovic); Biopsychosocial Corporation, Vienna (Aschauer, Schlögelhofer); University Health Network, Youthdale Treatment Centres, and University of Toronto, Toronto (Sandor); the Krembil Research Institute, University Health Network, Hospital for Sick Children, and University of Toronto, Toronto (Barr); Johns Hopkins University School of Medicine, Baltimore (Grados, Singer); the Institute of Human Genetics, University Hospital Bonn, University of Bonn Medical School, Bonn, Germany (Nöthen); the Department of Child and Adolescent Psychiatry, Psychosomatics, and Psychotherapy, University Hospital Essen, University of Duisburg-Essen, Essen, Germany (Hebebrand, Hinney); the Yale Child Study Center and the Department of Psychiatry, Yale University School of Medicine, New Haven, Conn. (King, Fernandez); the Institute of Medical Chemistry, Molecular Biology, and Pathobiochemistry, Semmelweis University, Budapest, Hungary (Barta); Vadaskert Child and Adolescent Psychiatric Hospital, Budapest, Hungary (Tarnok, Nagy); the Institute of Human Genetics, University Hospital Essen, University Duisburg-Essen, Essen, Germany (Depienne); Sorbonne Universités, UPMC Université Paris 06, UMR S 1127, CNRS UMR 7225, ICM, Paris (Depienne, Worbe, Hartmann); French Reference Centre for Gilles de la Tourette Syndrome, Groupe Hospitalier Pitié-Salpêtrière, Paris (Worbe, Hartmann); Assistance Publique-Hôpitaux de Paris, Department of Neurology, Groupe Hospitalier Pitié-Salpêtrière, Paris (Worbe, Hartmann); Zucker School of Medicine at Hofstra/Northwell, Hempstead, New York (Budman); Child Neuropsychiatry, Department of Clinical and Experimental Medicine, University of Catania, Catania, Italy (Rizzo); the Stanley Institute for Cognitive Genomics, Cold Spring Harbor Laboratory, Cold Spring Harbor, New York (Lyon); the Department of Psychiatry, University of Utah, Salt Lake City (McMahon); Children's Mercy Hospital, Kansas City, Mo. (Batterson); the Department of Psychiatry, University Medical Center Groningen and Rijksuniversity Groningen, and Drenthe Mental Health Center, Groningen, the Netherlands (Cath); the Department of Neurology, Fixel Center for Neurological Diseases, McKnight Brain Institute, University of Florida, Gainesville (Malaty, Okun); Pennsylvania State University College of Medicine, Hershey (Berlin); Marquette University and University of Wisconsin-Milwaukee, Milwaukee (Woods); Tripler Army Medical Center and University of Hawaii John A. Burns School of Medicine, Honolulu (Lee); Parkinson's Disease Center and Movement Disorders Clinic, Department of Neurology, Baylor College of Medicine, Houston (Jankovic); the Division of Psychiatry, Department of Neuropsychiatry, University College London (Robertson); the Department of Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati (Gilbert); Children's Hospital of Philadelphia, Philadelphia (Brown); the Department of Psychiatry and Behavioral Sciences, University of Miami Miller School of Medicine, Miami (Coffey); the Department of Child and Adolescent Psychiatry, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands (Dietrich, Hoekstra); University of Iowa Carver College of Medicine, Iowa City (Kuperman); the Department of Pediatrics, University of Washington, Seattle (Zinner); the Department of Pediatrics, Landspitalinn University Hospital, Reykjavik, Iceland (Luðvigsson, Thorarensen); the Faculty of Medicine, University of Iceland, Reykjavík, Iceland (Sæmundsen, Stefansson); the State Diagnostic and Counselling Centre, Kópavogur, Iceland (Sæmundsen); the Department of Genetics and the Department of Medicine, Albert Einstein College of Medicine, Bronx, New York (Atzmon, Barzilai); the Department of Human Biology, Haifa University, Haifa, Israel (Atzmon); the Department of Psychiatry and Psychotherapy, University of Bonn, Bonn, Germany (Wagner); the Department of Psychiatry and Psychotherapy, University of Tübingen, Tübingen, Germany (Moessner); SUNY Downstate Medical Center Brooklyn, New York (C.M. Pato, M.T. Pato, Knowles); the Athinoula A. Martinos Center for Biomedical Research, Department of Radiology, Massachusetts General Hospital, Charlestown (Roffman, Buckner); the Department of Epidemiology, Harvard T.H. Chan School of Public Health, Boston (Smoller); the Center for Brain Science and Department of Psychology, Harvard University, Cambridge, Mass. (Buckner); the Institute for Neurodegenerative Diseases, UCSF Weill Institute for Neurosciences, University of California San Francisco, San Francisco (Willsey); the Department of Genetics and the Human Genetics Institute of New Jersey, Rutgers, the State University of New Jersey, Piscataway (Tischfield, Heiman); the Department of Complex Trait Genetics, Center for Neurogenomics and Cognitive Research, VU University Amsterdam, Amsterdam (Posthuma); the Division of Genetic Medicine, Vanderbilt Genetics Institute, Vanderbilt University Medical Center, Nashville, Tenn. (Cox, Davis); the Analytic and Translational Genetics Unit, Department of Medicine, Massachusetts General Hospital, Boston (Neale); the Department of Psychiatry, Genetics Institute, University of Florida, Gainesville (Mathews); and the Department of Neurology, Brigham and Women's Hospital, and the Department of Neurology, Massachusetts General Hospital, Boston (Scharf).

Objective:: Tourette's syndrome is polygenic and highly heritable. Genome-wide association study (GWAS) approaches are useful for interrogating the genetic architecture and determinants of Tourette's syndrome and other tic disorders. The authors conducted a GWAS meta-analysis and probed aggregated Tourette's syndrome polygenic risk to test whether Tourette's and related tic disorders have an underlying shared genetic etiology and whether Tourette's polygenic risk scores correlate with worst-ever tic severity and may represent a potential predictor of disease severity. Read More

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http://dx.doi.org/10.1176/appi.ajp.2018.18070857DOI Listing
March 2019
2 Reads
12.295 Impact Factor

What is the role of cannabidiol in refractory epilepsy?

JAAPA 2019 Mar;32(3):16-18

At the time this article was written, Kathryn M. LaFleur was a student in the PA program at Sacred Heart University in Fairfield, Conn. Eric C. Nemec II is director of research and assessment and a clinical associate professor in the PA program at Sacred Heart University. The authors have disclosed no potential conflicts of interest, financial or otherwise.

The FDA recently approved the first cannabidiol oral medication to treat refractory epilepsy in patients with Dravet syndrome and Lennox-Gastaut syndrome. This article describes the safety and efficacy of cannabidiol treatment in patients with refractory epilepsy. Read More

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http://dx.doi.org/10.1097/01.JAA.0000553393.36249.dfDOI Listing

Na/K Pump Mutations Associated with Primary Hyperaldosteronism Cause Loss of Function.

Biochemistry 2019 Mar 14. Epub 2019 Mar 14.

Department of Cell Physiology and Molecular Biophysics, Center for Membrane Protein Research , Texas Tech University Health Sciences Center , Lubbock , Texas 79430 , United States.

Primary hyperaldosteronism (Conn's syndrome), a common cause of secondary hypertension, is frequently produced by unilateral aldosterone-producing adenomas that carry mutations in ion-transporting genes, including ATP1A1, encoding the Na/K pump's α1 subunit. Whether Na/K pump mutant-mediated inward currents are required to depolarize the cell and increase aldosterone production remains unclear, as such currents were observed in four out of five mutants described so far. Here, we use electrophysiology and uptake of the K congener Rb, to characterize the effects of eight additional Na/K pump mutations in transmembrane segments TM1 (delM102-L103, delL103-L104, and delM102-I106), TM4 (delI322-I325 and I327S), and TM9 (delF956-E961, delF959-E961, and delE960-L964), expressed in Xenopus oocytes. Read More

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http://dx.doi.org/10.1021/acs.biochem.9b00051DOI Listing
March 2019
2 Reads

1-adrenoceptor polymorphisms and blood pressure: 49S variant increases plasma renin but not blood pressure in hypertensive patients.

Am J Hypertens 2019 Feb 11. Epub 2019 Feb 11.

Division of Experimental Medicine and Immunotherapeutics, University of Cambridge, Cambridge, United Kingdom.

Background: Activation of beta-1 adrenoreceptors (β1-AR) in the kidney releases renin that plays a major role in the maintenance of blood pressure. Genetic variation in the β1-AR could therefore alter the physiological and clinical effects of this hormone. We tested this hypothesis in patients from a primary care cohort being screened for primary hyperaldosteronism, PHA (n=465). Read More

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http://dx.doi.org/10.1093/ajh/hpz019DOI Listing
February 2019
1 Read

Vamorolone targets dual nuclear receptors to treat inflammation and dystrophic cardiomyopathy.

Life Sci Alliance 2019 Feb 11;2(1). Epub 2019 Feb 11.

Department of Genomics and Precision Medicine, George Washington University School of Medicine and Health Sciences, Washington, DC, USA.

Cardiomyopathy is a leading cause of death for Duchenne muscular dystrophy. Here, we find that the mineralocorticoid receptor (MR) and glucocorticoid receptor (GR) can share common ligands but play distinct roles in dystrophic heart and skeletal muscle pathophysiology. Comparisons of their ligand structures indicate that the Δ9,11 modification of the first-in-class drug vamorolone enables it to avoid interaction with a conserved receptor residue (N770/N564), which would otherwise activate transcription factor properties of both receptors. Read More

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http://dx.doi.org/10.26508/lsa.201800186DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6371196PMC
February 2019
3 Reads

Genetic Characteristics of Aldosterone-Producing Adenomas in Blacks.

Hypertension 2019 Apr;73(4):885-892

From the Department of Molecular and Integrative Physiology (K.N., W.E.R.), University of Michigan, Ann Arbor.

Somatic mutations have been identified in aldosterone-producing adenomas (APAs) in genes that include KCNJ5, ATP1A1, ATP2B3, and CACNA1D. Based on independent studies, there appears to be racial differences in the prevalence of somatic KCNJ5 mutations, particularly between East Asians and Europeans. Despite the high cardiovascular disease mortality of blacks, there have been no studies focusing on somatic mutations in APAs in this population. Read More

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http://dx.doi.org/10.1161/HYPERTENSIONAHA.118.12070DOI Listing
April 2019
2 Reads

[Two case reports on resistant hypertension].

Internist (Berl) 2019 Feb 1. Epub 2019 Feb 1.

Klinik für Nieren- und Hochdruckerkrankungen, Medizinische Hochschule Hannover, Carl-Neuberg-Str. 1, 30625, Hannover, Deutschland.

Primary aldosteronism (PA) is a frequent cause of resistant hypertension. The clinical presentation is heterogeneous, but a suppressed or low normal renin (especially with ACE inhibitors or sartans) should raise suspicion for primary aldosteronism, even when aldosterone levels are in the normal range. Diagnosis of unilateral hormone production from an adrenal adenoma (Conn syndrome), which is curable by surgery, requires adrenal vein sampling, which should be performed in experienced centers. Read More

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http://dx.doi.org/10.1007/s00108-019-0563-1DOI Listing
February 2019
1 Read

Family hyperaldosteronism type I: a clinical case and review of literature.

Ter Arkh 2018 Sep;90(9):115-122

National Medical Cardiology Research Center of the Ministry of Healthcare of the Russian Federation, Moscow, Russia.

Family hyperaldosteronism type I (glucocorticoids-remediable hyperaldosteronism) is a rare form of symptomatic arterial hypertension (AH), which often leads to the development of cerebrovascular complications. The disease is caused by the formation of the chimeric gene CYP11B2/CYP11B1.  Expression of the chimeric gene is regulated by adrenocorticotropic hormone, and glucocorticoid therapy leads to a decrease in aldosterone secretion and normalization of blood pressure. Read More

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http://dx.doi.org/10.26442/terarkh2018909115-122DOI Listing
September 2018
7 Reads

[The third case report a patient with primary aldosteronism, seizures, and neurologic abnormalities (PASNA) syndrome de novo variant mutations in the CACNA1D gene].

Zh Nevrol Psikhiatr Im S S Korsakova 2018 ;118(12):49-52

Federal Research Centre of Nutrition and Biotechnology.

Germline mutations in CACNA1D cause the primary aldosteronism, seizures, and neurologic abnormalities (PASNA) syndrome (OMIM# 615474) characterized by primary aldosteronism, seizures and neurological abnormalities. The authors present a case-report of a 1-year 3-month male patient with neurological symptoms such as seizures and global developmental delay with primary hyperaldosteronism. The heterozygosis disease-causing variant c. Read More

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http://dx.doi.org/10.17116/jnevro201811812149DOI Listing
January 2018
1 Read

A simple and efficient automated cGMP-compliant radiosynthesis of [ C]metomidate using solid phase extraction cartridge purification.

J Labelled Comp Radiopharm 2019 Jan 28. Epub 2019 Jan 28.

Wolfson Brain Imaging Centre, Department of Clinical Neurosciences, University of Cambridge, Addenbrooke's Hospital, Cambridge, CB2 0QQ, UK.

[ C]metomidate ([ C]MTO) is a radiotracer widely used to detect disorders of adrenocortical origin by positron emission tomography (PET) imaging. [ C]MTO PET/computed tomography (PET/CT) is considered a sensitive and specific noninvasive alternative to adrenal vein sampling (AVS) in the management of primary hyperaldosteronism (PHA). Herein, we report a reliable automated procedure for the routine manufacturing of [ C]MTO in current good manufacturing practice (cGMP) conditions on the commercial Synthra MeI Loop Vessel synthesizer. Read More

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http://dx.doi.org/10.1002/jlcr.3711DOI Listing
January 2019
2 Reads
1.187 Impact Factor

Primary Aldosteronism: Present and Future.

Authors:
John W Funder

Vitam Horm 2019 23;109:285-302. Epub 2018 Dec 23.

Hudson Institute of Medical Research, Clayton, VIC, Australia; Monash University, Clayton, VIC, Australia. Electronic address:

Primary aldosteronism (PA), currently recognized to be 5-10% of hypertension, has a cardiovascular risk profile double that in age-, sex-, and blood pressure-matched essential hypertensives. Screening for PA is by determining the plasma aldosterone to renin ratio (ARR), followed by one of half a dozen confirmatory/exclusion tests. Unilateral hyperaldosteronism normally reflects an aldosterone producing adenoma; bilateral disease is the more common form, and termed idiopathic hyperaldosteronism (IHA). Read More

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http://dx.doi.org/10.1016/bs.vh.2018.10.006DOI Listing
December 2018
1 Read

Adipocyte Mineralocorticoid Receptor.

Vitam Horm 2019 21;109:189-209. Epub 2018 Dec 21.

Laboratory of Cardiovascular Endocrinology, IRCCS San Raffaele Pisana, Rome, Italy; Department of Human Sciences and Promotion of the Quality of Life, San Raffaele Roma Open University, Rome, Italy. Electronic address:

Mineralocorticoid receptor (MR) has been recently identified in adipose tissue, where its excessive activation contributes to several metabolic derangements often observed in obesity and metabolic syndrome. Recent findings support the existence of a bidirectional cross-talk between adipose tissue and adrenal glands, contributing to obesity-related hyperaldosteronism and subsequent adipocyte MR excessive activation. In this regard, MR pharmacological blockade has led to prevention of weight gain and metabolic benefits in murine models of genetic or diet-induced obesity. Read More

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http://dx.doi.org/10.1016/bs.vh.2018.10.005DOI Listing
December 2018
1 Read

Intervention for arch obstruction after the Norwood procedure: Prevalence, associated factors, and practice variability.

J Thorac Cardiovasc Surg 2019 Feb 22;157(2):684-695.e8. Epub 2018 Nov 22.

Division of Pediatric Cardiac Surgery, Rady Children's Hospital, San Diego, Calif.

Objective: Arch obstruction after the Norwood procedure is common and contributes to mortality. We determined the prevalence, associated factors, and practice variability of arch reintervention and assessed whether arch reintervention is associated with mortality.

Methods: From 2005 to 2017, 593 neonates in the Congenital Heart Surgeons' Society Critical Left Heart Obstruction cohort underwent a Norwood procedure. Read More

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http://dx.doi.org/10.1016/j.jtcvs.2018.09.130DOI Listing
February 2019
4 Reads

Classification of microadenomas in patients with primary aldosteronism by steroid profiling.

J Steroid Biochem Mol Biol 2019 Jan 14. Epub 2019 Jan 14.

Medizinische Klinik und Poliklinik IV, Klinikum der Universität, Ludwig-Maximilians-Universität München, Munich, Germany; Division of Internal Medicine and Hypertension, Department of Medical Sciences, University of Turin, Turin, Italy. Electronic address:

In primary aldosteronism (PA) the differentiation of unilateral aldosterone-producing adenomas (APA) from bilateral adrenal hyperplasia (BAH) is usually performed by adrenal venous sampling (AVS) and/or computed tomography (CT). CT alone often lacks the sensitivity to identify micro-APAs. Our objectives were to establish if steroid profiling could be useful for the identification of patients with micro-APAs and for the development of an online tool to differentiate micro-APAs, macro-APAs and BAH. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S09600760183047
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http://dx.doi.org/10.1016/j.jsbmb.2019.01.008DOI Listing
January 2019
8 Reads

Fate of distal aorta after frozen elephant trunk and total arch replacement for type A aortic dissection in Marfan syndrome.

J Thorac Cardiovasc Surg 2018 Aug 24. Epub 2018 Aug 24.

Department of Cardiovascular Surgery, Beijing Anzhen Hospital, Capital Medical University, and Beijing Institute of Heart Lung and Blood Vessel Diseases, Beijing, China; Fu Wai Hospital and Cardiovascular Institute, Chinese Academy of Medical Sciences, Beijing, China. Electronic address:

Objective: The use of the frozen elephant trunk technique for type A aortic dissection in Marfan syndrome is limited by the lack of imaging evidence for long-term aortic remodeling. We seek to evaluate the changes of the distal aorta and late outcomes after frozen elephant trunk and total arch replacement for type A aortic dissection in patients with Marfan syndrome.

Methods: Between 2003 and 2015, we performed frozen elephant trunk + total arch replacement for 172 patients with Marfan syndrome suffering from type A aortic dissection (94 acute; 78 chronic). Read More

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http://dx.doi.org/10.1016/j.jtcvs.2018.07.096DOI Listing
August 2018
3 Reads
4.168 Impact Factor

Frequency of Primary Hyperaldosteronism in Young Hypertensives in a Tertiary Care Setting of Rawalpindi.

J Coll Physicians Surg Pak 2019 Jan;29(1):58-61

Department of Medicine, Military Hospital, Rawalpindi, Pakistan.

Objective: To determine the frequency of primary hyperaldosteronism in young hypertensives in hospital settings of Rawalpindi.

Study Design: Cross-sectional study.

Place And Duration Of Study: Department of Chemical Pathology and Endocrinology, Armed Forces Institute of Pathology (AFIP), Rawalpindi, from June 2016 to May 2017. Read More

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http://dx.doi.org/10.29271/jcpsp.2019.01.58DOI Listing
January 2019
1 Read

Efficient screening of patients with aldosterone-producing adenoma using the ACTH stimulation test.

Hypertens Res 2019 Jan 8. Epub 2019 Jan 8.

Division of Circulatory and Body Fluid Regulation, Department of Internal Medicine, Faculty of Medicine, University of Miyazaki, Miyazaki, Japan.

Adrenal venous sampling (AVS) is the gold standard test for distinguishing between unilateral and bilateral primary aldosteronism (PA); however, AVS requires advanced and time consuming technique. The needs for AVS have been increasing due to the increased utilization of screening for PA. An efficient selection of unilateral PA, such as aldosterone-producing adenoma (APA), before AVS is useful to avoid undesirable AVS in bilateral PA, such as idiopathic hyperaldosteronism. Read More

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http://dx.doi.org/10.1038/s41440-018-0191-5DOI Listing
January 2019
2 Reads

Adrenal gland disorders, active component, U.S. Armed Forces, 2002-2017.

MSMR 2018 Dec;25(12):10-19

During 2002-2017, the most common incident adrenal gland disorder among male and female service members was adrenal insufficiency and the least common was adrenomedullary hyperfunction. Adrenal insufficiency was diagnosed among 267 females (crude overall incidence rate: 8.2 cases per 100,000 person-years [p-yrs]) and 729 males (3. Read More

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December 2018
6 Reads

Immunohistochemistry for aldosterone synthase CYP11B2 and matrix-assisted laser desorption ionization imaging mass spectrometry for in-situ aldosterone detection.

Curr Opin Nephrol Hypertens 2019 Mar;28(2):105-112

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

Purpose Of Review: Immunohistochemistry for aldosterone synthase (CYP11B2) has markedly provided a comprehensive picture of the adrenocortical diseases, particularly primary aldosteronism. The findings from CYP11B2-immunohistochemistry are consistent with the clinical courses of most patients with primary aldosteronism. We herein review the updated pathophysiology and usefulness of the method for understanding individual patients with different subtypes of primary aldosteronism. Read More

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http://dx.doi.org/10.1097/MNH.0000000000000487DOI Listing
March 2019
10 Reads

Proteomic Landscape of Aldosterone-Producing Adenoma.

Hypertension 2019 Feb;73(2):469-480

From the Biozentrum, University of Basel, Switzerland (M.M.S., M.C., E.D., P.J., S.M., C.P., M.N.H.), University Hospital Basel, Switzerland.

Primary aldosteronism is a disease of excessive production of adrenal steroid hormones and the most common cause of endocrine hypertension. Primary aldosteronism results mainly from bilateral adrenal hyperplasia or unilateral aldosterone-producing adenoma (APA). Primary aldosteronism cause at the molecular level is incompletely understood and a targeted treatment preventing excessive adrenal steroid production is not available. Read More

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http://dx.doi.org/10.1161/HYPERTENSIONAHA.118.11733DOI Listing
February 2019
4 Reads

Adrenal Tissue-Specific Deletion of TASK Channels Causes Aldosterone-Driven Angiotensin II-Independent Hypertension.

Hypertension 2019 Feb;73(2):407-414

From the Department of Pharmacology (N.A.G., J.Y., E.J.S., D.A.B, P.Q.B.), University of Virginia School of Medicine, Charlottesville.

The renin-angiotensin system tightly controls aldosterone synthesis. Dysregulation is evident in hypertension (primary aldosteronism), low renin, and resistant hypertension) but also can exist in normotension. Whether chronic, mild aldosterone autonomy can elicit hypertension remains untested. Read More

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https://www.ahajournals.org/doi/10.1161/HYPERTENSIONAHA.118.
Publisher Site
http://dx.doi.org/10.1161/HYPERTENSIONAHA.118.11962DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6326871PMC
February 2019
2 Reads

Rates of abnormal aldosterone/renin ratio in African-origin compared to European-origin patients: A retrospective study.

Clin Endocrinol (Oxf) 2019 Apr 25;90(4):528-533. Epub 2019 Jan 25.

Department of Endocrinology, The Adelaide and Meath Hospital, Incorporating the National Children's Hospital, Tallaght, Dublin, Ireland.

Introduction: The aldosterone/renin ratio is the initial screening test for primary hyperaldosteronism (PHA), but little data exists regarding ethnic variations in this.

Methods: Following clinical observation of a high prevalence of abnormal aldosterone/renin ratio (ARR) in patients of African-origin, we retrospectively reviewed all ARR measurements in a single centre over 10 years. Rates of hypokalaemia, intraventricular septal thickness (IVS, by echocardiography) and adrenal imaging were recorded when available. Read More

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http://dx.doi.org/10.1111/cen.13920DOI Listing
April 2019
4 Reads

Familial hyperaldosteronism type III a novel case and review of literature.

Rev Endocr Metab Disord 2018 Dec 19. Epub 2018 Dec 19.

Department of Pediatrics, Hospital Lluís Alcanyís de Xàtiva, Ctra. Xàtiva a Silla km 2, 46800, Xàtiva, Valencia, Spain.

Less than 15% of hypertension cases in children are secondary to a primary hyperaldosteronism. This is idiopathic in 60% of the cases, secondary to a unilateral adenoma in 30% and 10% remaining by primary adrenal hyperplasia, familial hyperaldosteronism, ectopic aldosterone production or adrenocortical carcinoma.To date, four types of familial hyperaldosteronism (FH I to FH IV) have been reported. Read More

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http://link.springer.com/10.1007/s11154-018-9481-0
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http://dx.doi.org/10.1007/s11154-018-9481-0DOI Listing
December 2018
14 Reads

Villus Growth, Increased Intestinal Epithelial Sodium Selectivity, and Hyperaldosteronism Are Mechanisms of Adaptation in a Murine Model of Short Bowel Syndrome.

Dig Dis Sci 2018 Dec 20. Epub 2018 Dec 20.

Department of General, Thoracic, Vascular and Transplantation Surgery, Rostock University Medical Center, Schillingallee 35, 18057, Rostock, Germany.

Background: Short bowel syndrome results from extensive small bowel resection and induces adaptation of the remaining intestine. Ileocecal resection (ICR) is the most frequent situation in humans. Villus hypertrophy is one hallmark of mucosal adaptation, but the functional mechanisms of mucosal adaptation are incompletely understood. Read More

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http://dx.doi.org/10.1007/s10620-018-5420-xDOI Listing
December 2018
3 Reads

A novel SLC12A3 homozygous c2039delG mutation in Gitelman syndrome with hypocalcemia.

BMC Nephrol 2018 Dec 17;19(1):362. Epub 2018 Dec 17.

The Endocrinology Department of the Third Xiangya Hospital, Central South University, Tongzipo Road, Changsha, 410013, China.

Background: Gitelman syndrome (GS) is a rare autosomal recessive renal tubular disease, caused by mutations in the SLC12A3 gene, which encodes the renal thiazide-sensitive Na/Cl cotransporter (NCCT) in the distal renal tubule.

Case Presentation: A 23-year-old woman was admitted with limb numbness, recurrent tetany and palpitation. Laboratory tests showed hypokalemic alkalosis, hypomagnesemia, hypocalcemia and secondary hyperaldosteronism, as well as hypocalciuria and transient decreased PTH. Read More

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http://dx.doi.org/10.1186/s12882-018-1163-3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6296056PMC
December 2018
3 Reads

Aldosteronism with mild hypokalemia presenting as life-threatening ventricular arrhythmias: A case report.

Medicine (Baltimore) 2018 Dec;97(50):e13608

Department of Cardiology, the First Hospital of Jilin University, Changchun, Jilin Province, China.

Rationale: Primary aldosteronism (PA) with hypokalemia increases the risk of life-threatening ventricular arrhythmias. Cases of PA with malignant arrhythmia as the first symptom have been reported. The role of severe hypokalemia in triggering malignant ventricular arrhythmia is well documented. Read More

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http://dx.doi.org/10.1097/MD.0000000000013608DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6319998PMC
December 2018
3 Reads

Simultaneous occurrence of primary aldosteronism due to aldosteronoma and ectopic meningioma in the adrenal gland: A case report.

Medicine (Baltimore) 2018 Dec;97(50):e13591

Department of Endocrinology, First Affiliated Hospital of Nanjing Medical University and Jiangsu Province Hospital, Gulou District, Nanjing City, Jiangsu Province, PR China.

Rationale: Primary aldosteronism due to aldosteronoma is the most common form of secondary hypertension, with an estimated prevalence of 4% of hypertensive patients in primary care and around 10% of referred patients. Diagnosis is a clinical challenge with simultaneous occurrence of primary ectopic meningioma in the adrenal gland. To our knowledge this is the first reported case of simultaneous occurrence of aldosteronomas and ectopic meningioma in the adrenal gland based on literatures. Read More

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http://dx.doi.org/10.1097/MD.0000000000013591DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6319975PMC
December 2018
1 Read

Resistant and refractory hypertension: two sides of the same disease?

J Bras Nefrol 2018 Dec 6. Epub 2018 Dec 6.

Departamento de Medicina, Faculdade de Ciências Médicas e da Saúde, Pontifícia Universidade Católica de São Paulo, Rio de Janeiro, RJ, Brasil.

Refractory hypertension (RfH) is an extreme phenotype of resistant hypertension (RH), being considered an uncontrolled blood pressure besides the use of 5 or more antihypertensive medications, including a long-acting thiazide diuretic and a mineralocorticoid antagonist. RH is common, with 10-20% of the general hypertensives, and its associated with renin angiotensin aldosterone system hyperactivity and excess fluid retention. RfH comprises 5-8% of the RH and seems to be influenced by increased sympathetic activity. Read More

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http://dx.doi.org/10.1590/2175-8239-jbn-2018-0108DOI Listing
December 2018

Facial Malformation in Crouzon's Syndrome Is Consistent with Cranial Base Development in Time and Space.

Plast Reconstr Surg Glob Open 2018 Oct 1;6(10):e1963. Epub 2018 Oct 1.

Section of Plastic and Reconstructive Surgery, Yale School of Medicine, New Haven, Conn.

Background: In Crouzon's syndrome, cranial base deformities begin sequentially in the anterior cranial fossa initially, and later to the posterior cranial base. Facial characteristics are likely related to cranial base development. The temporal correlation between cranial base development and facial features is in need of clarification in Crouzon's patients, to clarify initial sites of deformity, which may impact surgical decision making. Read More

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http://dx.doi.org/10.1097/GOX.0000000000001963DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6250456PMC
October 2018
2 Reads

Telehealth in Plastic Surgery: A Veterans Affairs Hospital Perspective.

Plast Reconstr Surg Glob Open 2018 Oct 2;6(10):e1840. Epub 2018 Oct 2.

Department of Plastic Surgery, Yale University, Cedar Street, New Haven, Conn.

Background: Telemedicine is a rapidly growing tool since its invention in the 1950s. Recently, it has expanded to the field of plastic surgery. In the Connecticut VA System, there is 1 plastic surgeon at a central location for the state of Connecticut and southern Massachusetts. Read More

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http://dx.doi.org/10.1097/GOX.0000000000001840DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6250470PMC
October 2018
3 Reads

Usefulness of Magnetic Resonance Imaging in the Diagnosis of Juxtaglomerular Cell Tumors: A Report of 10 Cases and Review of the Literature.

Am J Kidney Dis 2018 Dec 4. Epub 2018 Dec 4.

Hypertension Unit, AP-HP, Hôpital Européen Georges Pompidou, Paris, France; Faculty of Medicine, Paris-Descartes University, Paris, France. Electronic address:

Juxtaglomerular cell tumors (JCTs), a rare but potentially curable cause of hypertension, are difficult to diagnose because they may be missed or misidentified as a cyst by computed tomography (CT). Their magnetic resonance imaging (MRI) pattern has not been well described. We report the clinical, biological, and radiologic features of 10 patients with JCTs. Read More

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http://dx.doi.org/10.1053/j.ajkd.2018.09.005DOI Listing
December 2018
2 Reads

Separate and interacting effects of the endogenous circadian system and behaviors on plasma aldosterone in humans.

Am J Physiol Regul Integr Comp Physiol 2019 Feb 6;316(2):R157-R164. Epub 2018 Dec 6.

Oregon Institute of Occupational Health Sciences, Oregon Health & Science University , Portland, Oregon.

Measurements of aldosterone for diagnosis of primary aldosteronism are usually made from blood sampled in the morning when aldosterone typically peaks. We tested the relative contributions and interacting influences of the circadian system, ongoing behaviors, and prior sleep to this morning peak in aldosterone. To determine circadian rhythmicity and separate effects of behaviors on aldosterone, 16 healthy participants completed a 5-day protocol in dim light while all behaviors ranging from sleep to exercise were standardized and scheduled evenly across the 24-h circadian period. Read More

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http://dx.doi.org/10.1152/ajpregu.00314.2018DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6397357PMC
February 2019
2 Reads

Effects of Altered Calcium Metabolism on Cardiac Parameters in Primary Aldosteronism.

Endocrinol Metab (Seoul) 2018 Dec;33(4):485-492

Department of Internal Medicine, Severance Hospital, Endocrine Research Institute, Yonsei University College of Medicine, Seoul, Korea.

Background: Increasing evidence supports interplay between aldosterone and parathyroid hormone (PTH), which may aggravate cardiovascular complications in various heart diseases. Negative structural cardiovascular remodeling by primary aldosteronism (PA) is also suspected to be associated with changes in calcium levels. However, to date, few clinical studies have examined how changes in calcium and PTH levels influence cardiovascular outcomes in PA patients. Read More

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http://dx.doi.org/10.3803/EnM.2018.33.4.485DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6279903PMC
December 2018
3 Reads

Discussion: Early Mandibular Distraction in Craniofacial Microsomia and Need for Orthognathic Correction at Skeletal Maturity: A Comparative Long-Term Follow-Up Study.

Plast Reconstr Surg 2018 11;142(5):1294-1298

New Haven, Conn. From Plastic and Reconstructive Surgery, Craniomaxillofacial Surgery, Yale School of Medicine.

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http://dx.doi.org/10.1097/PRS.0000000000004954DOI Listing
November 2018
2 Reads

Bone health among patients with primary aldosteronism: a systematic review and meta-analysis.

Minerva Endocrinol 2018 Nov 26. Epub 2018 Nov 26.

Clinical Academic Unit, Newcastle University Medicine Malaysia, Johor, Malaysia.

Background: Recent studies showed a possible association between hyperaldosteronism and secondary hyperparathyroidism leading to reduced bone health, however results are conflicting.

Methods: We conducted a meta-analysis to evaluate the relationship between primary aldosteronism (PA) with bone biochemical markers and to assess bone mineral density in patients with primary aldosteronism.

Results: A total of 939 subjects were examined (37. Read More

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http://dx.doi.org/10.23736/S0391-1977.18.02867-5DOI Listing
November 2018
3 Reads

MANAGEMENT OF ENDOCRINE DISEASE: The role of confirmatory tests in the diagnosis of primary aldosteronism.

Eur J Endocrinol 2019 Feb;180(2):R45-R58

Department of Endocrinology and Diabetology, Côte de Nacre Regional Hospital Center, Caen, France.

The strategy for diagnosis of primary aldosteronism (PA) in the hypertensive population includes firstly a screening step, based on the measurement of plasma aldosterone-to-renin ratio (ARR), a test which must have high sensitivity, and secondly a confirmatory step based on the demonstration of excessive aldosterone production independent of the renin-angiotensin-aldosterone system (RAAS) activity. The high proportion of false-positive ARR results and conversely of actual PA without a persistent elevation in baseline plasma aldosterone concentration necessitates the addition of a confirmatory step in the work-up of PA diagnosis. The present review focuses on the description of the different dynamic tests available for demonstrating autonomy of aldosterone secretion, on the performance and limitations of confirmatory tests and on possible strategies for PA diagnosis which may either include or avoid the confirmatory step for PA diagnosis. Read More

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http://dx.doi.org/10.1530/EJE-18-0704DOI Listing
February 2019

Somatic mutations in adrenocortical carcinoma with hyperaldosteronism.

Endocr Relat Cancer 2018 Nov 1. Epub 2018 Nov 1.

T Else, Metabolism, Endocrinology and Diabetes/Internal Medicine, University of Michigan, Ann Arbor, United States.

Several somatic mutations specific to aldosterone-producing adenomas (APA) have been described. A small proportion of adrenocortical carcinomas (ACC) are associated with hyperaldosteronism, either primary aldosteronism or hyperreninemic hyperaldosteronism. However, it is unknown, whether they harbor mutations of the same spectrum as APAs. Read More

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https://erc.bioscientifica.com/view/journals/erc/aop/erc-18-
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http://dx.doi.org/10.1530/ERC-18-0385DOI Listing
November 2018
15 Reads

Risk of severe erectile dysfunction in primary hyperaldosteronism: A population-based propensity score matching cohort study.

Surgery 2019 Mar 23;165(3):622-628. Epub 2018 Nov 23.

Taiwan Primary Aldosteronism Investigation (TAIPAI) Study Group, Taipei; Division of Urology, Department of Surgery, Taipei Tzu Chi Hospital, The Buddhist Medical Foundation, New Taipei City, Taiwan. Electronic address:

Background: An elevated plasma aldosterone level has been reported as an independent risk factor for severe erectile dysfunction in men. The aim of this study was to explore whether primary hyperaldosteronism patients experience erectile dysfunction after targeted treatment.

Methods: We conducted a population-based cohort study of men with newly identified primary hyperaldosteronism/aldosterone-producing adenoma from January 1, 1997, to December 31, 2009. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00396060183055
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http://dx.doi.org/10.1016/j.surg.2018.08.020DOI Listing
March 2019
9 Reads
3.380 Impact Factor

Risk of sepsis in patients with primary aldosteronism.

Crit Care 2018 11 21;22(1):313. Epub 2018 Nov 21.

Department of Internal Medicine, National Taiwan University Hospital, Taipei, Taiwan.

Background: The interaction between hyperaldosteronism and immune dysfunction has been reported and glucocorticoid co-secretion is frequently found in primary aldosteronism (PA). The aforementioned conditions raise the possibility of the infection risk; however, clinical episodes of sepsis have not been reported in PA.

Methods: Using Taiwan's National Health Insurance Research Database between 1997 and 2009, we identified PA and aldosterone-producing adenoma (APA) matched with essential hypertension (EH) at a 1:1 ratio by propensity scores. Read More

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http://dx.doi.org/10.1186/s13054-018-2239-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6249889PMC
November 2018
17 Reads

Speckle-Tracking Echocardiographic Layer-Specific Strain Analysis on Subclinical Left Ventricular Dysfunction in Patients With Primary Aldosteronism.

Am J Hypertens 2019 Jan;32(2):155-162

The Shanghai Institute of Hypertension, Department of Hypertension, Ruijin Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, China.

Background: Primary aldosteronism (PA) may cause myocardial injury. We investigated myocardial dysfunction using speckle-tracking echocardiographic (STE) layer-specific strain in patients with PA.

Methods: Our study included 62 patients with PA (33 aldosterone-producing adenoma [APA] and 29 idiopathic hyperaldosteronism [IHA]) and 30 patients with primary hypertension. Read More

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http://dx.doi.org/10.1093/ajh/hpy175DOI Listing
January 2019
5 Reads
2.852 Impact Factor

Diagnostic challenges and good treatment outcomes in pediatric paraganglioma of the abdomen: A case report.

Medicine (Baltimore) 2018 Nov;97(47):e13268

Pathology Department, Central South University Xiangya School of Medicine Affiliated Haikou Hospital, Haikou, Hainan, PR China.

Rationale: Paraganglioma is a catecholamine-producing neuroendocrine tumor. Management of paraganglioma including its diagnosis is difficult, because it has no characteristic symptoms and many diseases can manifest as headache and high blood pressure. Herein, we report a rare case of paraganglioma of the abdomen with headache and initial normal blood pressure. Read More

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http://dx.doi.org/10.1097/MD.0000000000013268DOI Listing
November 2018
11 Reads

Aging-associated perifollicular changes and calcium deposition in poodles.

Vet Dermatol 2019 Feb 21;30(1):56-e15. Epub 2018 Nov 21.

Department of Veterinary Sciences, University of Pisa, Viale delle Piagge 2, 56124, Pisa, Italy.

Background: It is commonly accepted that canine dystrophic mineralization of the hair follicle glassy membrane can be seen in hyperadrenocorticism and as a senile change in poodles. Pathology textbooks define this change as deposition of calcium salts in the form of basophilic, amorphous, granular material along collagen fibrils.

Hypothesis/objectives: The aim was to evaluate whether the incidence of the lesion is specific to poodles and if it is always associated with calcium deposition. Read More

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http://dx.doi.org/10.1111/vde.12700DOI Listing
February 2019
2 Reads

Bartter Syndrome and Gitelman Syndrome.

Pediatr Clin North Am 2019 02;66(1):121-134

Department of Pediatrics, Inova Children's Hospital, 3300 Gallows Road, Falls Church, VA 22042, USA; Division of Nephrology and Hypertension, Pediatric Specialists of Virginia, 3023 Hamaker Court, Suite 600, Fairfax, VA 22031, USA; Virginia Commonwealth School of Medicine, Richmond, VA, USA. Electronic address:

Bartter and Gitelman syndromes are conditions characterized by renal salt-wasting. Clinical presentations range from severe antenatal disease to asymptomatic with incidental diagnosis. Hypokalemic hypochloremic metabolic alkalosis is the common feature. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00313955183013
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http://dx.doi.org/10.1016/j.pcl.2018.08.010DOI Listing
February 2019
13 Reads