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Primary Aldosteronism Differential Diagnoses

  • Author: Gabriel I Uwaifo, MD; Chief Editor: Romesh Khardori, MD, PhD, FACP  more...
 
Updated: Jun 30, 2016
 
 

Diagnostic Considerations

Consider the diagnosis of primary aldosteronism in all persons with hypertension (HTN) and hypokalemia. Making the correct diagnosis may be the only way to achieve adequate blood pressure control and thus, to prevent the sequelae of poorly controlled HTN.

Conditions to consider in the differential diagnosis of primary aldosteronism include the following:

  • HTN
  • Malignant HTN
  • Hypertensive encephalopathy
  • Hypokalemia
  • Metabolic alkalosis
  • Renal artery stenosis
  • Renovascular HTN
  • Low-renin essential HTN - Constitutes about 40% of essential HTN
  • Tobacco chewing
  • Carbenoxolone intoxication
  • Apparent mineralocorticoid excess (AME) syndrome
  • Various causes of secondary aldosteronism - Unlike primary aldosteronism, these causes are associated with elevated renin (plasma renin activity) levels
  • Chrétien syndrome - This rare syndrome is characterized by mineralocorticoid excess and adrenocortical HTN secondary to a pituitary adenoma producing pro-opiomelanocortin (POMC)[17]
  • Deoxycorticosterone (DOC)–secreting adrenal tumors
  • Renovascular ischemia
  • Preeclampsia (toxemia of pregnancy)
  • Renin-secreting tumor - These are rare tumors arising from the juxtaglomerular apparatus
  • Excessive licorice intake - In this situation, the glycyrrhizinic acid component inhibits 11beta-hydroxysteroid dehydrogenase, impairing conversion of cortisol to cortisone in the kidneys; hence, cortisol binds to mineralocorticoid receptors and acts as a mineralocorticoid

Additional select genetic/familial disorders and syndromes to consider include the following:

  • Gitelman syndrome - This is due to a defective sodium/chloride cotransporter (NCCT); it is basically a salt-losing tubulopathy with secondary aldosteronism
  • Barrter syndrome - This is a phenocopy of at least 3 distinct genetic defects (ie, hyperactivition of the sodium-potassium-dichloride cotransporter [NKCC2], the renal outer medullary potassium channel [ROMK1], or the renal epithelial chloride channel [ClCKb],the latter encoded by the barttin gene; this is also a salt-losing tubulopathy with secondary aldosteronism and is pathophysiologically similar to Gitelman syndrome
  • Gordon syndrome - This is due to inactivating mutations of the serine-threonine kinases WNK1 and WNK4 (“with no lysine [K]” kinases), leading to hypertension, hyperkalemia, mild hyperchloremia, acidosis, and suppressed plasma renin activity
  • Pseudoaldosteronism (Liddle syndrome) - This is a rare autosomal dominant disorder due to hyperactivating mutations of the renal epithelial sodium channel (ENaC), with excessive sodium reabsorption in the renal distal tubule; levels of renin and aldosterone are low
  • 11beta-hydroxysteroid dehydrogenase deficiency
  • Glucocorticoid resistance - This is due to inactivating mutations of the glucocorticoid receptor

Differential Diagnoses

 
 
Contributor Information and Disclosures
Author

Gabriel I Uwaifo, MD Associate Professor, Section of Endocrinology, Diabetes and Metabolism, Louisiana State University School of Medicine in New Orleans; Adjunct Professor, Joint Program on Diabetes, Endocrinology and Metabolism, Pennington Biomedical Research Center in Baton Rouge

Gabriel I Uwaifo, MD is a member of the following medical societies: American Association of Clinical Endocrinologists, American College of Physicians-American Society of Internal Medicine, American Diabetes Association, American Medical Association, American Society of Hypertension, Endocrine Society

Disclosure: Nothing to disclose.

Coauthor(s)

Nicholas J Sarlis, MD, PhD, FACP Vice President, Head of Medical Affairs, Incyte Corporation

Nicholas J Sarlis, MD, PhD, FACP is a member of the following medical societies: American Association for the Advancement of Science, American Association for Cancer Research, American Association of Clinical Endocrinologists, American College of Physicians, American Federation for Medical Research, American Head and Neck Society, American Medical Association, American Society for Radiation Oncology, American Thyroid Association, Endocrine Society, New York Academy of Sciences, Royal Society of Medicine, Association for Psychological Science, American College of Endocrinology, European Society for Medical Oncology, American Society of Clinical Oncology

Disclosure: Received salary from Incyte Corporation for employment; Received ownership interest from Sanofi-Aventis for previous employment; Received ownership interest/ stock & stock option (incl. rsu) holder from Incyte Corporation for employment.

Chief Editor

Romesh Khardori, MD, PhD, FACP Professor of Endocrinology, Director of Training Program, Division of Endocrinology, Diabetes and Metabolism, Strelitz Diabetes and Endocrine Disorders Institute, Department of Internal Medicine, Eastern Virginia Medical School

Romesh Khardori, MD, PhD, FACP is a member of the following medical societies: American Association of Clinical Endocrinologists, American College of Physicians, American Diabetes Association, Endocrine Society

Disclosure: Nothing to disclose.

Acknowledgements

Arthur B Chausmer, MD, PhD, FACP, FACE, FACN, CNS, Professor of Medicine (Endocrinology, Adj), Johns Hopkins School of Medicine; Affiliate Research Professor, Bioinformatics and Computational Biology Program, School of Computational Sciences, George Mason University; Principal, C/A Informatics, LLC

Arthur B Chausmer, MD, PhD, FACP, FACE, FACN, CNS is a member of the following medical societies: American Association of Clinical Endocrinologists, American College of Endocrinology, American College of Nutrition, American College of Physician Executives, American College of Physicians, American College of Physicians-American Society of Internal Medicine, American Medical Informatics Association, American Society for Bone and Mineral Research, American Society of Law, Medicine &Ethics, Endocrine Society, and International Society for Clinical Densitometry

Disclosure: Nothing to disclose.

Serge A Jabbour, MD Associate Professor, Department of Medicine, Division of Endocrinology, Jefferson Medical College of Thomas Jefferson University

Serge A Jabbour, MD is a member of the following medical societies: American Association of Clinical Endocrinologists, American College of Physicians-American Society of Internal Medicine, American Diabetes Association, American Medical Association, American Thyroid Association, Endocrine Society, and Pennsylvania Medical Society

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Frederick H Ziel, MD Associate Professor of Medicine, University of California, Los Angeles, David Geffen School of Medicine; Physician-In-Charge, Endocrinology/Diabetes Center, Director of Medical Education, Kaiser Permanente Woodland Hills; Chair of Endocrinology, Co-Chair of Diabetes Complete Care Program, Southern California Permanente Medical Group

Frederick H Ziel, MD is a member of the following medical societies: American Association of Clinical Endocrinologists, American College of Endocrinology, American College of Physicians, American College of Physicians-American Society of Internal Medicine, American Diabetes Association, American Federation for Medical Research, American Medical Association, American Society for Bone and Mineral Research, California Medical Association, Endocrine Society, andInternational Society for Clinical Densitometry

Disclosure: Nothing to disclose.

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Magnetic resonance imaging (MRI) scan in a patient with Conn syndrome showing a left adrenal adenoma.
Scintigram obtained by using iodine-131-beta-iodomethyl-norcholesterol (NP-59) in a 59-year-old man with hypertension shows fairly intense radionuclide uptake in the right adrenal tumor. At surgery, a Conn tumor was confirmed.
Effects of main antihypertensives on the renin-angiotensin system.
Potential causes of primary aldosteronism.
Transitional zone adrenocortical steroids.
Algorithm for screening for potential primary aldosteronism.
Algorithm for confirmation of primary aldosteronism.
Algorithm for distinguishing subtypes of primary aldosteronism.
 
 
 
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