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1. Full Text Somatic HIF2A Gain-of-Function Mutations in Paraganglioma with Polycythemia
by Zhuang, Zhengping and Yang, Chunzhang and Lorenzo, Felipe and Merino, Maria and Fojo, Tito and Kebebew, Electron and Popovic, Vera and Stratakis, Constantine A and Prchal, Josef T and Pacak, Karel
The New England Journal of Medicine, ISSN 0028-4793, 09/2012, Volume 367, Issue 10, pp. 922 - 930
Two patients with paraganglioma (one of whom also had somatostatinoma) were found to have mutations in HIF2A that altered HIF-2α turnover and led to excess... 
MEDICINE, GENERAL & INTERNAL | HIF-2-ALPHA | INHIBITION | PHEOCHROMOCYTOMA | GENE | HIF-1-ALPHA | CANCER | ERYTHROCYTOSIS | TUMORIGENESIS | HYPOXIA-INDUCIBLE FACTORS | Up-Regulation | Basic Helix-Loop-Helix Transcription Factors - genetics | Hydroxylation | Paraganglioma - genetics | Humans | Abdominal Neoplasms - complications | Pancreatic Neoplasms - genetics | Paraganglioma - complications | Somatostatinoma - complications | Paraganglioma - blood | Somatostatinoma - genetics | Basic Helix-Loop-Helix Transcription Factors - metabolism | Polycythemia - etiology | DNA Mutational Analysis | Abdominal Neoplasms - genetics | Adolescent | Adrenal Gland Neoplasms - complications | Adult | Female | Transcription, Genetic | Mutation | Adrenal Gland Neoplasms - genetics | Causes of | Genetic aspects | Research | Gene mutations | Polycythemia | Risk factors | Hypoxia-inducible factors | Erythropoiesis | Energy metabolism | Transcription factors | Nuclear magnetic resonance--NMR | Genes | Childrens health | Paraganglioma | Metastasis | Patients | Abdomen | Proteins | Mutagenesis | Plasmids | Hypoxia | Tumorigenesis | Age | Deoxyribonucleic acid--DNA | Tumors | Cancer | Index Medicus | Abridged Index Medicus
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2. Full Text Constitutive Activation of PKA Catalytic Subunit in Adrenal Cushing's Syndrome
by Beuschlein, Felix and Fassnacht, Martin and Assié, Guillaume and Calebiro, Davide and Stratakis, Constantine A and Osswald, Andrea and Ronchi, Cristina L and Wieland, Thomas and Sbiera, Silviu and Faucz, Fabio R and Schaak, Katrin and Schmittfull, Anett and Schwarzmayr, Thomas and Barreau, Olivia and Vezzosi, Delphine and Rizk-Rabin, Marthe and Zabel, Ulrike and Szarek, Eva and Salpea, Paraskevi and Forlino, Antonella and Vetro, Annalisa and Zuffardi, Orsetta and Kisker, Caroline and Diener, Susanne and Meitinger, Thomas and Lohse, Martin J and Reincke, Martin and Bertherat, Jérome and Strom, Tim M and Allolio, Bruno
The New England Journal of Medicine, ISSN 0028-4793, 03/2014, Volume 370, Issue 11, pp. 1019 - 1028
Corticotropin-independent Cushing's syndrome occurs with adrenocortical tumors or hyperplasia. The authors report that germline duplications of PRKACA lead to... 
PROTEIN-KINASE-A | REGULATORY SUBUNIT | MEDICINE, GENERAL & INTERNAL | MANAGEMENT | GENE | RECEPTOR | CARNEY COMPLEX | HYPERPLASIA | ADRENOCORTICAL TUMORS | EXPRESSION | SOMATIC MUTATIONS | Cyclic AMP-Dependent Protein Kinases - metabolism | Catalytic Domain | Adenoma - genetics | Humans | Middle Aged | Cyclic AMP-Dependent Protein Kinases - chemistry | Cushing Syndrome - etiology | Adrenal Hyperplasia, Congenital - genetics | Sequence Analysis, DNA | Exome | Adenoma - enzymology | Adenoma - complications | Adrenal Gland Neoplasms - enzymology | Cyclic AMP-Dependent Protein Kinases - genetics | Cushing Syndrome - enzymology | Adrenal Gland Neoplasms - complications | Germ-Line Mutation | Adult | Protein Conformation | Hydrocortisone - biosynthesis | Mutation | Adrenal Gland Neoplasms - genetics | Influence | Research | Protein kinases | Risk factors | Cushing syndrome | Protein kinase A | Chromosome 19 | Nervous system diseases | AMP | Adrenal glands | Genes | Hyperplasia | Adrenal cortex | Cyclic AMP | Cushing's syndrome | Hydrocortisone | Kinases | Adrenocorticotropic hormone | Pituitary | Tumors | Index Medicus | Abridged Index Medicus | Biochemistry, Molecular Biology | Adenoma | Life Sciences | Adrenal Hyperplasia, Congenital | Cushing Syndrome | Adrenal Gland Neoplasms | Cyclic AMP-Dependent Protein Kinases
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3. Full Text Novel insights into the polycythemia-paraganglioma-somatostatinoma syndrome
by Därr, Roland and Nambuba, Joan and Del Rivero, Jaydira and Janssen, Ingo and Merino, Maria and Todorovic, Milena and Balint, Bela and Jochmanova, Ivana and Prchal, Josef T and Lechan, Ronald M and Tischler, Arthur S and Popovic, Vera and Miljic, Dragana and Adams, Karen T and Prall, F Ryan and Ling, Alexander and Golomb, Meredith R and Ferguson, Michael and Nilubol, Naris and Chen, Clara C and Chew, Emily and Taïeb, David and Stratakis, Constantine A and Fojo, Tito and Yang, Chunzhang and Kebebew, Electron and Zhuang, Zhengping and Pacak, Karel
Endocrine-Related Cancer, ISSN 1351-0088, 12/2016, Volume 23, Issue 12, pp. 899 - 908
Worldwide, the syndromes of paraganglioma (PGL), somatostatinoma (SOM) and early childhood polycythemia are described in only a few patients with somatic... 
Paraganglioma | HIF2A mutation | Pheochromocytoma | Polycythemia | Somatostatinoma | polycythemia | somatostatinoma | HIPPEL-LINDAU-DISEASE | pheochromocytoma | FAMILIAL ERYTHROCYTOSIS | OF-FUNCTION MUTATIONS | HYPOXIA | TUMORS | paraganglioma | SUPERIORITY | GENE | ONCOLOGY | HIF2A MUTATIONS | ENDOCRINOLOGY & METABOLISM | HYPERTENSION | Polycythemia - diagnosis | Pancreatic Neoplasms - diagnosis | Humans | Middle Aged | Somatostatinoma - diagnosis | Male | Paraganglioma - complications | Paraganglioma - diagnosis | Polycythemia - complications | Adrenal Gland Neoplasms - diagnosis | Somatostatinoma - therapy | Young Adult | Neoplasms, Multiple Primary - therapy | Somatostatinoma - pathology | Adult | Female | Polycythemia - pathology | Retrospective Studies | Child | Adrenal Gland Neoplasms - pathology | Neoplasms, Multiple Primary - diagnosis | Paraganglioma - pathology | Paraganglioma - therapy | Pancreatic Neoplasms - pathology | Somatostatinoma - complications | Neoplasms, Multiple Primary - pathology | Disease Progression | Syndrome | Adolescent | Adrenal Gland Neoplasms - complications | Polycythemia - therapy | Neoplasms, Multiple Primary - complications | Pancreatic Neoplasms - complications | Adrenal Gland Neoplasms - therapy | Cohort Studies | Pancreatic Neoplasms - therapy | Index Medicus
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4. Full Text Somatic Mutations Affecting the Selectivity Filter of KCNJ5 Are Frequent in 2 Large Unselected Collections of Adrenal Aldosteronomas
by Azizan, Elena A.B and Murthy, Meena and Stowasser, Michael and Gordon, Richard and Kowalski, Bartosz and Xu, Shengxin and Brown, Morris J and OʼShaughnessy, Kevin M
Hypertension, ISSN 0194-911X, 03/2012, Volume 59, Issue 3, pp. 587 - 591
Primary hyperaldosteronism, one cause of which is aldosterone-producing adenomas (APAs), may account for ≤5% to 10% of cases of essential hypertension.... 
hyperaldosteronism | aldosterone-producing adenoma | KCNJ5 | posture response | hypertension | potassium channels | DIAGNOSIS | HYPERALDOSTERONISM TYPE-II | ANGIOTENSIN-II | GLOMERULOSA CELLS | CHROMOSOME 7P22 | LOW RENIN HYPERTENSION | PERIPHERAL VASCULAR DISEASE | K+ CHANNEL | ADENOMAS | Adrenal Glands - pathology | Genetic Predisposition to Disease | Prognosis | Adenoma - genetics | Aldosterone - biosynthesis | Humans | Middle Aged | Adrenal Gland Neoplasms - blood | Hyperaldosteronism - genetics | Male | G Protein-Coupled Inwardly-Rectifying Potassium Channels - genetics | Hyperaldosteronism - blood | Adenoma - blood | Phenotype | Adenoma - complications | Hyperaldosteronism - etiology | Adrenal Glands - metabolism | Polymerase Chain Reaction | Adrenal Gland Neoplasms - complications | Germ-Line Mutation | Female | DNA, Neoplasm - genetics | Adrenal Gland Neoplasms - genetics | Index Medicus
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5. Full Text Bone Metastases and Skeletal-Related Events in Patients With Malignant Pheochromocytoma and Sympathetic Paraganglioma
by Ayala-Ramirez, Montserrat and Palmer, J. Lynn and Hofmann, Marie-Claude and de la Cruz, Maxine and Moon, Bryan S and Waguespack, Steven G and Habra, Mouhammed Amir and Jimenez, Camilo
The Journal of Clinical Endocrinology & Metabolism, ISSN 0021-972X, 04/2013, Volume 98, Issue 4, pp. 1492 - 1497
Context: Bone metastases (BM) can cause severe pain, spinal cord compression, pathological fractures, and/or hypercalcemia. These skeletal-related events... 
BREAST-CANCER | CELLS | POSITRON-EMISSION-TOMOGRAPHY | PAIN | PAMIDRONATE | EFFICACY | SCINTIGRAPHY | ENDOCRINOLOGY & METABOLISM | B GENE-MUTATIONS | EXPRESSION | CARCINOMA | Bone Diseases - epidemiology | Bone Neoplasms - mortality | Humans | Middle Aged | Bone Neoplasms - secondary | Fractures, Bone - epidemiology | Bone Neoplasms - epidemiology | Male | Paraganglioma - complications | Pheochromocytoma - mortality | Young Adult | Bone Diseases - etiology | Chronic Pain - epidemiology | Adrenal Gland Neoplasms - mortality | Nervous System Neoplasms - epidemiology | Adrenal Gland Neoplasms - epidemiology | Fractures, Bone - etiology | Paraganglioma - mortality | Aged, 80 and over | Pheochromocytoma - pathology | Adult | Female | Paraganglioma - epidemiology | Nervous System Neoplasms - complications | Retrospective Studies | Sympathetic Nervous System - pathology | Child | Chronic Pain - etiology | Adrenal Gland Neoplasms - pathology | Pheochromocytoma - epidemiology | Paraganglioma - pathology | Nervous System Neoplasms - mortality | Spinal Cord Compression - epidemiology | Spinal Cord Compression - etiology | Bone Neoplasms - complications | Bone Diseases - mortality | Pheochromocytoma - complications | Adolescent | Adrenal Gland Neoplasms - complications | Aged | Nervous System Neoplasms - pathology | Index Medicus | Abridged Index Medicus | Endocrine Care
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6. Full Text "Nonfunctional" adrenal Tumors and the risk for incident diabetes and cardiovascular outcomes: A cohort study
by Lopez, Diana and Luque-Fernandez, Miguel Angel and Steele, Amy and Adler, Gail K and Turchin, Alexander and Vaidya, Anand
Annals of Internal Medicine, ISSN 0003-4819, 10/2016, Volume 165, Issue 8, pp. 533 - 542
Background: Benign adrenal tumors are commonly discovered on abdominal imaging. Most are classified as nonfunctional and are considered to pose no health risk,... 
MORTALITY | DIAGNOSIS | MEDICINE, GENERAL & INTERNAL | CLINICAL-PRACTICE GUIDELINE | ADRENOCORTICAL ADENOMAS | SUBCLINICAL CUSHINGS-SYNDROME | PREVALENCE | CORTISOL | Prevalence | Follow-Up Studies | Humans | Middle Aged | Risk Factors | Adrenal Gland Neoplasms - blood | Male | Adrenal Gland Neoplasms - diagnostic imaging | Prediabetic State - complications | Renal Insufficiency, Chronic - complications | Diabetes Mellitus, Type 2 - epidemiology | Renal Insufficiency, Chronic - epidemiology | Cardiovascular Diseases - complications | Adrenal Gland Neoplasms - complications | Cardiovascular Diseases - epidemiology | Female | Retrospective Studies | Hydrocortisone - blood | Prediabetic State - epidemiology | Adrenocortical Hyperfunction - complications | Diabetes Mellitus, Type 2 - complications | Development and progression | Metabolic diseases | Cardiovascular diseases | Risk factors | Diabetes | Adrenal glands | Clinical outcomes | Tumors | Index Medicus | Abridged Index Medicus
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7. Full Text Cardiovascular events and mortality in patients with adrenal incidentalomas that are either non-secreting or associated with intermediate phenotype or subclinical Cushing's syndrome: a 15-year retrospective study
by Di Dalmazi, Guido, MD|Vicennati, Valentina, MD|Garelli, Silvia, MD|Casadio, Elena, MD|Rinaldi, Eleonora, MD|Giampalma, Emanuela, MD|Mosconi, Cristina, MD|Golfieri, Rita, MD|Paccapelo, Alexandro|Pagotto, Uberto, MD|Pasquali, Renato, Prof
Lancet Diabetes & Endocrinology, The, ISSN 2213-8587, 2014, Volume 2, Issue 5, pp. 396 - 405
Summary Background Incidental discovery of adrenal masses has increased over the past few years. Mild alterations in cortisol secretion without clinical signs... 
Endocrinology & Metabolism | Other | FOLLOW-UP | RISK | ENDOCRINOLOGY & METABOLISM | CORTISOL | Cardiovascular Diseases - etiology | Adrenal Gland Neoplasms - secretion | Follow-Up Studies | Humans | Middle Aged | Cushing Syndrome - etiology | Male | Adrenal Gland Neoplasms - mortality | Phenotype | Adrenal Gland Neoplasms - complications | Cardiovascular Diseases - epidemiology | Female | Aged | Retrospective Studies | Index Medicus
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8. Full Text Androgen Excess in Women: Experience with Over 1000 Consecutive Patients
by Azziz, R and Sanchez, L. A and Knochenhauer, E. S and Moran, C and Lazenby, J and Stephens, K. C and Taylor, K and Boots, L. R
The Journal of Clinical Endocrinology & Metabolism, ISSN 0021-972X, 02/2004, Volume 89, Issue 2, pp. 453 - 462
The objective of the present study was to estimate the prevalence of the different pathological conditions causing clinically evident androgen excess and to... 
POLYCYSTIC-OVARY-SYNDROME | 20 MU-G | ETHINYL ESTRADIOL | THYROID-DISORDERS | CYPROTERONE ACETATE | INSULIN-RESISTANCE | IDIOPATHIC HIRSUTISM | ENDOCRINOLOGY & METABOLISM | PLACEBO-CONTROLLED TRIAL | RANDOMIZED CONTROLLED-TRIAL | ACNE-VULGARIS | Endocrine Gland Neoplasms - secretion | Prevalence | Androgens - secretion | Humans | Acanthosis Nigricans - complications | Treatment Outcome | Acanthosis Nigricans - epidemiology | Adrenal Hyperplasia, Congenital - complications | Hyperandrogenism - etiology | Hyperandrogenism - drug therapy | Endocrine Gland Neoplasms - complications | Time Factors | Adrenal Hyperplasia, Congenital - epidemiology | Endocrine Gland Neoplasms - epidemiology | Androgen Antagonists - therapeutic use | Adult | Female | Retrospective Studies | Androgen Antagonists - adverse effects | Index Medicus | Abridged Index Medicus
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9. Full Text The European Registry on Cushing's syndrome: 2-year experience. Baseline demographic and clinical characteristics
by Elena Valassi and Alicia Santos and Maria Yaneva and Miklós Tóth and Christian J Strasburger and Philippe Chanson and John A H Wass and Olivier Chabre and Marija Pfeifer and Richard A Feelders and Stylianos Tsagarakis and Peter J Trainer and Holger Franz and Kathrin Zopf and Sabina Zacharieva and Steven W J Lamberts and Antoine Tabarin and Susan M Webb and ERCUSYN Study Grp and ERCUSYN Study Group
European Journal of Endocrinology, ISSN 0804-4643, 09/2011, Volume 165, Issue 3, pp. 383 - 392
Objective: The European Registry on Cushing's syndrome (ERCUSYN) is designed to collect prospective and follow-up data at EU level on Cushing's syndrome (CS).... 
DIAGNOSIS | MANAGEMENT | TESTOSTERONE | DISEASE | ENDOCRINOLOGY & METABOLISM | RISK | FRACTURES | OLDER MEN | QUALITY-OF-LIFE | HEALTH | OSTEOPOROSIS | Cushing Syndrome - epidemiology | Prevalence | Prospective Studies | Humans | Middle Aged | Bone Diseases, Metabolic - etiology | Europe - epidemiology | Cushing Syndrome - etiology | Male | Pituitary Neoplasms - complications | Cushing Syndrome - complications | Fractures, Bone - etiology | Adolescent | Adrenal Gland Neoplasms - complications | Aged, 80 and over | Quality of Life | Adult | Female | Registries | Aged | Retrospective Studies | Cohort Studies | Index Medicus
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10. Full Text Surgical versus conservative management for subclinical cushing syndrome in adrenal incidentalomas: A prospective randomized study
by Toniato, Antonio and Merante-Boschin, Isabella and Opocher, Giuseppe and Pelizzo, Maria R and Schiavi, Francesca and Ballotta, Enzo
Annals of Surgery, ISSN 0003-4932, 03/2009, Volume 249, Issue 3, pp. 388 - 391
Objective: To compare the clinical outcome of patients with subclinical Cushing syndrome (SCS) due to an adrenal incidentaloma (the autonomous hypersecretion... 
SURGERY | LAPAROSCOPIC ADRENALECTOMY | DISEASE | Cushing Syndrome - surgery | Prospective Studies | Humans | Middle Aged | Incidental Findings | Cushing Syndrome - etiology | Male | Laparoscopy | Adrenalectomy | Cushing Syndrome - therapy | Adrenal Gland Neoplasms - complications | Female | Aged | Adrenal Gland Neoplasms - therapy | Index Medicus | Abridged Index Medicus
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11. Full Text Unusual Techniques for Preserving Surgical and Oncologic Safety in Hepatectomy of Advanced Adrenal Malignancy with Vena Cava and Liver Invasion
by Lee, Jeong-Moo and Lee, Kwang-Woong and Hong, Suk Kyun and Yoon, Kyung Chul and Cho, Jae-Hyung and Yi, Nam-Joon and Suh, Kyung-Suk
Annals of Surgical Oncology, ISSN 1068-9265, 10/2018, Volume 25, Issue 11, pp. 3324 - 3325
Status in terms of major vascular structure invasion is a crucial factor for successful major hepatic resection. In particular, surgery for advanced tumors... 
Oncology | Medicine & Public Health | Surgical Oncology | Surgery | SURGERY | ONCOLOGY | RESECTION | CARCINOMA | Adrenal Gland Neoplasms - surgery | Vena Cava, Inferior - surgery | Adrenal Cortex Neoplasms - complications | Prognosis | Humans | Middle Aged | Liver Neoplasms - complications | Carcinoma, Hepatocellular - complications | Hepatectomy - instrumentation | Vena Cava, Inferior - pathology | Adult | Female | Liver Neoplasms - pathology | Adrenalectomy - instrumentation | Venous Thrombosis - surgery | Adrenal Gland Neoplasms - pathology | Hepatectomy - methods | Neoplasm Invasiveness | Adrenal Cortex Neoplasms - surgery | Carcinoma, Hepatocellular - surgery | Liver Neoplasms - surgery | Adrenal Cortex Neoplasms - pathology | Venous Thrombosis - etiology | Adrenalectomy - methods | Carcinoma, Hepatocellular - pathology | Adrenal Gland Neoplasms - complications | Venous Thrombosis - pathology | Index Medicus
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12. Full Text Case of parotid mucoepidermoid carcinoma: Expanding the spectrum of von Hippel‐Lindau–related neoplasms
by Berger, Michael H and Kerr, Darcy A and Rangel Filho, Artur E and Sargi, Zoukaa B and Irish, Jonathan
Head & Neck, ISSN 1043-3074, 03/2017, Volume 39, Issue 3, pp. E51 - E54
Background. von Hippel-Lindau (VHL)-related tumors occurring outside the spectrum of VHL-defining tumors are rare, and mucoepidermoid carcinoma (MEC) in the... 
parotid | mucoepidermoid carcinoma | fine‐needle aspiration | von Hippel–Lindau (VHL) disease | fine-needle aspiration | SURGERY | TRANSLOCATION | OTORHINOLARYNGOLOGY | DISEASE | von Hippel-Lindau (VHL) disease | TUMORS | RENAL-CELL CARCINOMA | GLAND | Immunohistochemistry | Parotid Gland - surgery | Follow-Up Studies | Humans | Brain Neoplasms - pathology | von Hippel-Lindau Disease - complications | Brain Neoplasms - complications | von Hippel-Lindau Disease - pathology | In Situ Hybridization | Hemangioblastoma - complications | Pheochromocytoma - pathology | Adult | Female | Von Hippel-Lindau Tumor Suppressor Protein - genetics | Adrenal Gland Neoplasms - pathology | Genetic Predisposition to Disease | Carcinoma, Mucoepidermoid - complications | Risk Assessment | Brain Neoplasms - genetics | Treatment Outcome | Carcinoma, Mucoepidermoid - surgery | von Hippel-Lindau Disease - genetics | Carcinoma, Mucoepidermoid - pathology | Biopsy, Fine-Needle | Parotid Neoplasms - complications | Pheochromocytoma - complications | Hemangioblastoma - pathology | Hemangioblastoma - genetics | Adrenal Gland Neoplasms - complications | Parotid Neoplasms - pathology | Pheochromocytoma - genetics | Mutation | Adrenal Gland Neoplasms - genetics | Parotid Neoplasms - genetics | Gene mutations | Carcinoma | Cancer | Index Medicus
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13. Full Text Evidence for an increased rate of cardiovascular events in patients with primary aldosteronism
by Milliez, Paul and Girerd, Xavier and Plouin, Pierre-François and Blacher, Jacques and Safar, Michel E and Mourad, Jean-Jacques
Journal of the American College of Cardiology, ISSN 0735-1097, 04/2005, Volume 45, Issue 8, pp. 1243 - 1248
The aim of this report was to show that the rate of cardiovascular events is increased in patients with either subtype of primary aldosteronism (PA). Primary... 
FIBROSIS | DIAGNOSIS | CARDIAC & CARDIOVASCULAR SYSTEMS | RENOVASCULAR HYPERTENSION | HYPERALDOSTERONISM | SPIRONOLACTONE | CARDIOMYOPATHY | ADENOMA | CONGESTIVE-HEART-FAILURE | HYPERTENSIVE PATIENTS | ATRIAL-FIBRILLATION | Blood Pressure | Cardiovascular Diseases - etiology | Humans | Middle Aged | Hyperaldosteronism - complications | Male | Adrenal Hyperplasia, Congenital - complications | Renin - blood | Stroke - etiology | Adenoma - complications | Hyperaldosteronism - etiology | Adrenal Gland Neoplasms - complications | Atrial Fibrillation - etiology | Hypertension - complications | Female | Hypokalemia - complications | Myocardial Infarction - etiology | Hypertension | Care and treatment | Cardiac patients | Hyperaldosteronism | Cardiology | Confidence intervals | Plasma | Heart attacks | Rodents | Values | Cardiovascular disease | Blood pressure | Drug therapy | Potassium | Patients | Tumors | Index Medicus | Abridged Index Medicus
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