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Primary hyperaldosteronism


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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Syed Hassan A. Kazmi BSc, MD [2]

Synonyms and keywords: Conn’s syndrome, Primary hayperaldosteronism.

Overview

Overview

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Syed Hassan A. Kazmi BSc, MD [2]

Overview

In 1955, Dr Jerome W. Conn, the American endocrinologist, first described the condition and named it Conn’s syndrome. Primary hyperaldosteronism may be classified into five groups, include adrenal carcinoma, familial hyperaldosteronism type I and II, Idiopathic hyperaldosteronism (IH), primary adrenal hyperplasia, and aldosterone producing adenoma (APA), which is either renin-responsive or renin-unresponsive. Conn’s syndrome (primary hyperaldosteronism) features overproduction of aldosterone despite suppressed plasma renin activity (PRA). The resulting sodium retention produces hypertension, and elevated potassium excretion may cause hypokalemia. Patients with Conn’s syndrome due to primary hyperaldosteronism may have an aldosterone producing adrenocortical adenoma (APA); classically referred to as Conn’s syndrome, a unilateral hyperplasia, idiopathic hyperaldosteronism (IHA, also known as bilateral adrenal hyperplasia), familial forms (familial hyperaldosteronism types I, II, and III) have also been described, ectopic secretion of aldosterone. Common causes of primary hyperaldosteronism are aldosterone-secreting adenoma, bilateral hyperplasia of the adrenal glands, and ectopic secretion of aldosterone from ovaries and kidneys. Primary hyperaldosteronism must be differentiated from other diseases that cause hypertension and hypokalemia, such as renal artery stenosis, cushing’s syndrome, congenital adrenal hyperplasia, Liddle’s syndrome, diuretic use, licorice ingestion, and renin-secreting tumors. Patients with primary hyperaldosteronism usually appear well. Physical examination of patients with primary hyperaldosteronism is usually remarkable for high blood pressure, tachycardia, and an S4 maybe heard on auscultation of the precordium suggesting left ventricular hypertrophy secondary to increased afterload due to hypertension. Laboratory findings consistent with the diagnosis of primary hyperaldosteronism include plasma aldosterone to renin activity ratio (PAC/PRA) > 30, serum aldosterone value > 6 ng/dl, and simultaneous plasma renin activity levels < 1.0 ng/ml/hour after fludrocortisone suppression test or a plasma aldosterone > 10 ng/dl on saline infusion test or on oral sodium loading test, the post-test 24-hour urinary aldosterone excretion < 12 μg/day and a urinary sodium excretion of more than 200 mMol/day. The adrenal venous sampling test is gold standard for subtype classification of primary hyperaldosteronism. The optimal therapy for primary hyperaldosteronism depends on the etiology of hyperaldosteronism. Medical therapy is indicated for bilateral adrenal hyperplasia, and all ambiguous causes of primary hyperaldosteronism. Surgery is the mainstay of treatment for unilateral adrenal hyperplasia, aldosterone producing adenomas (APAs), adrenal carcinoma, ectopic ACTH, renin, and deoxycorticosterone secreting tumors.

Historical Perspective

Primary hyperaldosteronism (Conn’s syndrome) was described for the first time by the Polish internist, Michał Lityński. In 1955, Dr Jerome W. Conn, the American endocrinologist, first described the condition and named it Conn’s syndrome. Over the last few decades other more rare type of primary hyperaldsoteronism have also been described. From 1960s to early 1970s, techniques of diagnosis and treatment were greatly improved by the availability of spironolactone, realization of the renin-angiotensin-aldosterone system, and progress in laboratory tests and adrenal venous sampling.

Classification

Primary hyperaldosteronism may be classified into five groups, including adrenal carcinoma, familial hyperaldosteronism type I and II, Idiopathic hyperaldosteronism (IH), primary adrenal hyperplasia, and aldosterone producing adenoma (APA), which is either renin-responsive or renin-unresponsive.

Pathophysiology

Conn’s syndrome (primary hyperaldosteronism) features overproduction of aldosterone despite suppressed plasma renin activity (PRA). The resulting sodium retention produces hypertension, and elevated potassium excretion may cause hypokalemia. Patients with Conn’s syndrome due to primary hyperaldosteronism may have an aldosterone producing adrenocortical adenoma (APA); classically referred to as Conn’s syndrome, a unilateral hyperplasia, idiopathic hyperaldosteronism (IHA, also known as bilateral adrenal hyperplasia), familial forms (familial hyperaldosteronism types I, II, and III) have also been described, ectopic secretion of aldosterone (the ovaries and kidneys are the 2 organs described in the literature that, in the setting of neoplastic disease, can be ectopic sources of aldosterone, but this is a rare occurrence).

Causes

Common causes of primary hyperaldosteronism are aldosterone-secreting adenoma, bilateral hyperplasia of the adrenal glands, and ectopic secretion of aldosterone from ovaries and kidneys. Less common causes of primary hyperaldosteronism are familial hyperaldosteronism types I-III, pure aldosterone-producing adrenocortical carcinomas, and unilateral hyperplasia of the adrenal gland.

Differentiating Primary Hyperaldosteronism from other Diseases

Primary hyperaldosteronism must be differentiated from other diseases that cause hypertension and hypokalemia, such as renal artery stenosis, cushing’s syndrome, congenital adrenal hyperplasia, Liddle’s syndrome, diuretic use, licorice ingestion, and renin-secreting tumors.

Epidemiology and Demographics

Prevalence of primary hyperaldosteronism is from 1,400-32,000 (median 8,800) per 100,000 individuals around the world. Primary hyperaldosteronism incidence among patients with newly diagnosed hypertension is 11,200 of per 100,000 individuals. The case-fatality rate of primary hyperaldosteronism is approximately 23.4 per 1,000 individual-years. Patients of all age groups may develop primary hyperaldosteronism. There is no racial predilection for primary hyperaldosteronism.

Risk Factors

The only risk factors in the development of primary hyperaldosteronism are age of hypertension onset and serum potassium level.

Screening

There is insufficient evidence to recommend routine screening for primary hyperaldosteronism but according to the Endocrine Society Clinical Practice Guideline, screening for hyperaldosteronism is recommended for resistant hypertension by checking the plasma aldosterone to renin ratio (PAC/PRA).

Natural History, Complications and Prognosis

If left untreated, patients with primary hyperaldosteronism may progress to develop stroke, coronary artery disease, and renal insufficiency with associated proteinuria. Aldosterone producing adenomas (APAs) continue to grow slowly over time. The aldosterone production likely correlates with the size of the adenoma. It is a progressive disease and its common complications include left ventricular hypertrophy due to chronic hypertension, atrial fibrillation, myocardial infarction, stroke, proteinuria, and metabolic syndrome. The prognosis of primary hyperaldosteronism is good with treatment. Without treatment, primary hyperaldosteronism will result in hypertension with resultant hypertension-related complications, which may be a major cause of morbidity and mortality among patients. 

Diagnosis

History and Symptoms

The hallmark of primary hyperaldosteronism is resistant hypertension. A positive history of spontaneous or unprovoked hypokalemia and treatment-resistant (refractory) hypertension are suggestive of primary hyperaldosteronism. The most common symptoms of primary hyperaldosteronism include headaches, facial flushing, vision changes, and weakness.

Physical Examination

Patients with primary hyperaldosteronism usually appear well. Physical examination of patients with primary hyperaldosteronism is usually remarkable for high blood pressure, tachycardia, and an S4 maybe heard on auscultation of the precordium suggesting left ventricular hypertrophy secondary to increased afterload due to hypertension.

Laboratory Findings

Laboratory findings consistent with the diagnosis of primary hyperaldosteronism include plasma aldosterone to renin activity ratio (PAC/PRA) > 30, serum aldosterone value > 6 ng/dl, and simultaneous plasma renin activity levels < 1.0 ng/ml/hour after fludrocortisone suppression test or a plasma aldosterone > 10 ng/dl on saline infusion test or on oral sodium loading test, the post-test 24-hour urinary aldosterone excretion < 12 μg/day and a urinary sodium excretion of more than 200 mMol/day. The adrenal venous sampling test is gold standard for subtype classification of primary hyperaldosteronism.

CT scan

Adrenal CT scan may be helpful in the diagnosis of primary hyperaldosteronism. Findings on CT scan suggestive of primary hyperaldosteronism are signal intensity near to <10 HU, no contrast enhancement, and non-calcified lesion in adrenal gland.

MRI

Adrenal MRI may be helpful in the diagnosis of primary hyperaldosteronism when the attenuation on CT is < 30 HU. 

Other Imaging Findings

Iodocholesterol scintigraphy (NP-59 scan) may be helpful in the diagnosis of primary aldosteronism. Findings on an Iodocholesterol scintigraphy (NP-59 scan) suggestive of primary aldosteronism is increased uptake of the adrenal glands.

Other Diagnostic Studies

There are no other diagnostic studies associated with primary hyperaldosteronism.

Treatment

Medical Therapy

The optimal therapy for primary hyperaldosteronism depends on the etiology of hyperaldosteronism. Medical therapy is indicated for bilateral adrenal hyperplasia, and all ambiguous causes of primary hyperaldosteronism.

Surgery

Surgery is the mainstay of treatment for unilateral adrenal hyperplasia, aldosterone producing adenomas (APAs), adrenal carcinoma, ectopic ACTH, renin, and deoxycorticosterone secreting tumors.

Primary Prevention

There is no primary prevention for primary hyperaldosteronism.

Secondary Prevention

There is no secondary prevention for primary hyperaldosteronism.

References

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Historical Perspective

Historical Perspective

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Syed Hassan A. Kazmi BSc, MD [2]

Overview

Primary hyperaldosteronism (Conn’s syndrome) was described for the first time by the Polish internist, Michał Lityński. In 1955, Dr Jerome W. Conn, the American endocrinologist, first described the condition and named it Conn’s syndrome. Over the last few decades other more rare type of primary hyperaldosteronism have also been described. From 1960s to early 1970s, techniques of diagnosis and treatment were greatly improved by the availability of spironolactone, realization of the renin-angiotensin-aldosterone system, and progress in laboratory tests and adrenal venous sampling.

Historical Perspective

  • Primary hyperaldosteronism (Conn’s syndrome) was described for the first time by the Polish internist, Michał Lityński.[1]
  • In 1955, Dr Jerome W. Conn, the American endocrinologist, first described the condition and named it Conn’s syndrome.[2][3]
  • By 1964, Conn had collected 145 cases, and he postulated that up to 20% of patients with essential hypertension might have primary aldosteronism and later stated that the actual prevalence may be less than he previously thought. He predicted prevalence of primary aldosteronism to be 10% of hypertensives patients, a prediction that became true nearly 40 years later.[4]
  • From 1960s to early 1970s, techniques of diagnosis and treatment were greatly improved by the availability of spironolactone, realization of the renin-angiotensin-aldosterone system, and progress in laboratory tests and adrenal venous sampling.
  • On October 19, 1964, Jerome W. Conn, Edwin L. Cohen, and David R. Rovner differentiated between primary and secondary aldosteronism in hypertensive disease.[5]
  • In 1970s, there was an extensive application of modern imaging modalities, such as CT scanning, adrenal venous sampling and steroid analysis.[6]
  • From 1980s, more and more patients with primary aldosteronism were screened out from the hypertensive population by plasma renin activity/plasma aldosterone concentration ratio and cured by surgical interventions; laparoscopic unilateral adrenalectomy has become the generally accepted gold standard of operation.[7]
  • In the subsequent decades, besides aldosterone-producing adenoma (APA) described by Conn, six other subtypes of primary aldosteronism have been described. APA and bilateral idiopathic hyperaldosteronism (IHA) are the most common subtypes of primary aldosteronism. Other types that were discovered are unilateral hyperplasia or primary adrenal hyperplasia (PAH).[8]
  • Familial hyperaldosteronism (FH) is also rare and three types of that have been described.[9]

References

  1. ↑ Kucharz EJ (2007). “[Michał Lityński–a forgotten author of the first description on primary hyperaldosteronism]”. Pol. Arch. Med. Wewn. (in Polish). 117 (1–2): 57–8. PMID 17642209.
  2. ↑ Conn JW, Louis LH. Primary aldosteronism: a new clinical entity. Trans Assoc Am Physicians 1955;68:215-31; discussion, 231-3. PMID 13299331.
  3. ↑ “Grand Rounds: Primary Aldosteronism, Beyond Conn’s Syndrome | Clinical Correlations”.
  4. ↑ CONN JW, KNOPF RF, NESBIT RM (1964). “CLINICAL CHARACTERISTICS OF PRIMARY ALDOSTERONISM FROM AN ANALYSIS OF 145 CASES”. Am. J. Surg. 107: 159–72. PMID 14099489.
  5. ↑ Conn JW, Cohen EL, Rovner DR (1985). “Landmark article Oct 19, 1964: Suppression of plasma renin activity in primary aldosteronism. Distinguishing primary from secondary aldosteronism in hypertensive disease. By Jerome W. Conn, Edwin L. Cohen and David R. Rovner”. JAMA. 253 (4): 558–66. PMID 3881606.
  6. ↑ Lingam RK, Sohaib SA, Vlahos I, Rockall AG, Isidori AM, Monson JP, Grossman A, Reznek RH (2003). “CT of primary hyperaldosteronism (Conn’s syndrome): the value of measuring the adrenal gland”. AJR Am J Roentgenol. 181 (3): 843–9. doi:10.2214/ajr.181.3.1810843. PMID 12933492.
  7. ↑ Rayner BL, Opie LH, Davidson JS (2000). “The aldosterone/renin ratio as a screening test for primary aldosteronism”. S. Afr. Med. J. 90 (4): 394–400. PMID 10957926.
  8. ↑ “Aldosterone-producing adenoma and other surgically correctable forms of primary aldosteronism”.
  9. ↑ Quack I, Vonend O, Rump LC (2010). “Familial hyperaldosteronism I-III”. Horm. Metab. Res. 42 (6): 424–8. doi:10.1055/s-0029-1246187. PMID 20131203.

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Pathophysiology

Pathophysiology


Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Syed Hassan A. Kazmi BSc, MD [2], Mehrian Jafarizade, M.D [3]

Overview

Conn’s syndrome (primary hyperaldosteronism) features overproduction of aldosterone despite suppressed plasma renin activity (PRA). The resulting sodium retention produces hypertension, and elevated potassium excretion may cause hypokalemia. Patients with Conn’s syndrome due to primary hyperaldosteronism may have an aldosterone producing adrenocortical adenoma (APA); classically referred to as Conn’s syndrome, a unilateral hyperplasia, idiopathic hyperaldosteronism (IHA, also known as bilateral adrenal hyperplasia), familial forms (familial hyperaldosteronism types I, II, and III) have also been described, ectopic secretion of aldosterone (The ovaries and kidneys are the 2 organs described in the literature that, in the setting of neoplastic disease, can be ectopic sources of aldosterone, but this is a rare occurrence).

Pathophysiology

Renin

Basic physiology of aldosterone

Circulating aldosterone is principally made in the zona glomerulosa of the adrenal cortex (outer layer of the cortex) by a cascade of enzyme steps leading to the conversion of cholesterol to aldosterone.  

Renin angiotensin system – by Mikael Häggström, Via: Wikimedia.org[2]


Adrenal steroid synthesis pathways in adrenal cortex and related enzymes, Via: Wikimedia.org[3]

Pathogenesis

Primary hyperaldosteronism (PA) features overproduction of aldosterone despite suppressed plasma renin activity (PRA). The resulting sodium retention may lead to hypertension, and elevated potassium excretion may cause hypokalemia.

Genetics

1. Aldosterone Producing Adenoma (APA)

APAs are typically solitary, well circumscribed tumors which can cause aldosterone hypersecretion.

Somatic mutations
Gain of function mutations (KCNJ5, CACNA1D, CTNNB1 mutations)

Loss of function mutations (ATP1A1 and ATP2A3)

2. Familial hyperaldosteronism Type I (FH-I)

3. Familial hyperaldosteronism Type II (FH-II)

4. Familial hyperaldosteronism Type III

Associated Conditions

The following conditions may be found in association with primary hyperaldosteronism:

Gross Pathology

  • An aldosterone producing adenoma is usually, a unilateral, yellow, lipid-laden adenoma ranging in diameter from 5 to 35 mm.

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Microscopic Pathology

Microscopically, on hematoxylin and eosin section, the following findings can be observed for aldosterone producing adenomas:[23]

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References

  1. ↑ Williams GH (2005). “Aldosterone biosynthesis, regulation, and classical mechanism of action”. Heart Fail Rev. 10 (1): 7–13. doi:10.1007/s10741-005-2343-3. PMID 15947886.
  2. ↑ <https://commons.wikimedia.org/w/index.php?curid=8458370>
  3. ↑ “File:Adrenal Steroids Pathways.svg – Wikimedia Commons”.
  4. ↑ Young WF (2007). “Primary aldosteronism: renaissance of a syndrome”. Clin. Endocrinol. (Oxf). 66 (5): 607–18. doi:10.1111/j.1365-2265.2007.02775.x. PMID 17492946.
  5. ↑ Scholl UI, Healy JM, Thiel A, Fonseca AL, Brown TC, Kunstman JW, Horne MJ, Dietrich D, Riemer J, Kücükköylü S, Reimer EN, Reis AC, Goh G, Kristiansen G, Mahajan A, Korah R, Lifton RP, Prasad ML, Carling T (2015). “Novel somatic mutations in primary hyperaldosteronism are related to the clinical, radiological and pathological phenotype”. Clin. Endocrinol. (Oxf). 83 (6): 779–89. doi:10.1111/cen.12873. PMC 4995792. PMID 26252618.
  6. ↑ Choi M, Scholl UI, Yue P, Björklund P, Zhao B, Nelson-Williams C, Ji W, Cho Y, Patel A, Men CJ, Lolis E, Wisgerhof MV, Geller DS, Mane S, Hellman P, Westin G, Åkerström G, Wang W, Carling T, Lifton RP (2011). “K+ channel mutations in adrenal aldosterone-producing adenomas and hereditary hypertension”. Science. 331 (6018): 768–72. doi:10.1126/science.1198785. PMC 3371087. PMID 21311022.
  7. ↑ Scholl UI, Healy JM, Thiel A, Fonseca AL, Brown TC, Kunstman JW, Horne MJ, Dietrich D, Riemer J, Kücükköylü S, Reimer EN, Reis AC, Goh G, Kristiansen G, Mahajan A, Korah R, Lifton RP, Prasad ML, Carling T (2015). “Novel somatic mutations in primary hyperaldosteronism are related to the clinical, radiological and pathological phenotype”. Clin. Endocrinol. (Oxf). 83 (6): 779–89. doi:10.1111/cen.12873. PMC 4995792. PMID 26252618.
  8. ↑ Fernandes-Rosa FL, Williams TA, Riester A, Steichen O, Beuschlein F, Boulkroun S, Strom TM, Monticone S, Amar L, Meatchi T, Mantero F, Cicala MV, Quinkler M, Fallo F, Allolio B, Bernini G, Maccario M, Giacchetti G, Jeunemaitre X, Mulatero P, Reincke M, Zennaro MC (2014). “Genetic spectrum and clinical correlates of somatic mutations in aldosterone-producing adenoma”. Hypertension. 64 (2): 354–61. doi:10.1161/HYPERTENSIONAHA.114.03419. PMID 24866132.
  9. ↑ Monticone S, Hattangady NG, Nishimoto K, Mantero F, Rubin B, Cicala MV, Pezzani R, Auchus RJ, Ghayee HK, Shibata H, Kurihara I, Williams TA, Giri JG, Bollag RJ, Edwards MA, Isales CM, Rainey WE (2012). “Effect of KCNJ5 mutations on gene expression in aldosterone-producing adenomas and adrenocortical cells”. J. Clin. Endocrinol. Metab. 97 (8): E1567–72. doi:10.1210/jc.2011-3132. PMC 3410264. PMID 22628608.
  10. ↑ 10.0 10.1 Monticone S, Hattangady NG, Penton D, Isales CM, Edwards MA, Williams TA, Sterner C, Warth R, Mulatero P, Rainey WE (2013). “a Novel Y152C KCNJ5 mutation responsible for familial hyperaldosteronism type III”. J. Clin. Endocrinol. Metab. 98 (11): E1861–5. doi:10.1210/jc.2013-2428. PMC 3816265. PMID 24037882.
  11. ↑ Oki K, Plonczynski MW, Luis Lam M, Gomez-Sanchez EP, Gomez-Sanchez CE (2012). “Potassium channel mutant KCNJ5 T158A expression in HAC-15 cells increases aldosterone synthesis”. Endocrinology. 153 (4): 1774–82. doi:10.1210/en.2011-1733. PMC 3320257. PMID 22315453.
  12. ↑ Åkerström T, Maharjan R, Sven Willenberg H, Cupisti K, Ip J, Moser A, Stålberg P, Robinson B, Alexander Iwen K, Dralle H, Walz MK, Lehnert H, Sidhu S, Gomez-Sanchez C, Hellman P, Björklund P (2016). “Activating mutations in CTNNB1 in aldosterone producing adenomas”. Sci Rep. 6: 19546. doi:10.1038/srep19546. PMC 4728393. PMID 26815163.
  13. ↑ Scholl UI, Healy JM, Thiel A, Fonseca AL, Brown TC, Kunstman JW, Horne MJ, Dietrich D, Riemer J, Kücükköylü S, Reimer EN, Reis AC, Goh G, Kristiansen G, Mahajan A, Korah R, Lifton RP, Prasad ML, Carling T (2015). “Novel somatic mutations in primary hyperaldosteronism are related to the clinical, radiological and pathological phenotype”. Clin. Endocrinol. (Oxf). 83 (6): 779–89. doi:10.1111/cen.12873. PMC 4995792. PMID 26252618.
  14. ↑ 14.0 14.1 Stowasser M, Gordon RD (2001). “Familial hyperaldosteronism”. J. Steroid Biochem. Mol. Biol. 78 (3): 215–29. PMID 11595502.
  15. ↑ 15.0 15.1 Jackson RV, Lafferty A, Torpy DJ, Stratakis C (2002). “New genetic insights in familial hyperaldosteronism”. Ann. N. Y. Acad. Sci. 970: 77–88. PMID 12381543.
  16. ↑ Lafferty AR, Torpy DJ, Stowasser M, Taymans SE, Lin JP, Huggard P, Gordon RD, Stratakis CA (2000). “A novel genetic locus for low renin hypertension: familial hyperaldosteronism type II maps to chromosome 7 (7p22)”. J. Med. Genet. 37 (11): 831–5. PMC 1734468. PMID 11073536.
  17. ↑ Torpy DJ, Gordon RD, Lin JP, Huggard PR, Taymans SE, Stowasser M, Chrousos GP, Stratakis CA (1998). “Familial hyperaldosteronism type II: description of a large kindred and exclusion of the aldosterone synthase (CYP11B2) gene”. J. Clin. Endocrinol. Metab. 83 (9): 3214–8. doi:10.1210/jcem.83.9.5086. PMID 9745430.
  18. ↑ “Genetics of primary hyperaldosteronism”.
  19. ↑ Malinow KC, Lion JR (1979). “Hyperaldosteronism (Conn’s disease) presenting as depression”. J Clin Psychiatry. 40 (8): 358–9. PMID 468762.
  20. ↑ Apostolopoulou K, Künzel HE, Gerum S, Merkle K, Schulz S, Fischer E, Pallauf A, Brand V, Bidlingmaier M, Endres S, Beuschlein F, Reincke M (2014). “Gender differences in anxiety and depressive symptoms in patients with primary hyperaldosteronism: a cross-sectional study”. World J. Biol. Psychiatry. 15 (1): 26–35. doi:10.3109/15622975.2012.665480. PMID 22568586.
  21. ↑ Astegiano M, Bresso F, Demarchi B, Sapone N, Novero D, Palestro G, Resegotti A, Pellicano R, Rizzetto M (2005). “Association between Crohn’s disease and Conn’s syndrome. A report of two cases”. Panminerva Med. 47 (1): 61–4. PMID 15985978.
  22. ↑ Kim YA, Lee SS (2003). “Conn’s syndrome associated with Behcet’s disease”. J. Korean Med. Sci. 18 (1): 145–7. doi:10.3346/jkms.2003.18.1.145. PMC 3054990. PMID 12589107.
  23. ↑ “Aldosterone-producing adenoma and other surgically correctable forms of primary aldosteronism”.

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Causes

Causes

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Syed Hassan A. Kazmi BSc, MD [2]

Overview

Common causes of primary hyperaldosteronism are aldosterone-secreting adenoma, bilateral hyperplasia of the adrenal glands, and ectopic secretion of aldosterone from ovaries and kidneys. Less common causes of primary hyperaldosteronism are familial hyperaldosteronism types I-III, pure aldosterone-producing adrenocortical carcinomas, and unilateral hyperplasia of the adrenal gland.

Causes

Common Causes

Common causes of primary hyperaldosteronism (PA) may be divided into:[1][2]

Less Common Causes

Less common causes of primary hyperladosteronism include:[3][4][5]


References

  1. ↑ “Primary aldosteronism: renaissance of a syndrome – Young – 2007 – Clinical Endocrinology – Wiley Online Library”.
  2. ↑ Aronova A, Iii TJ, Zarnegar R (2014). “Management of hypertension in primary aldosteronism”. World J Cardiol. 6 (5): 227–33. doi:10.4330/wjc.v6.i5.227. PMC 4062125. PMID 24944753.
  3. ↑ “Primary aldosteronism: renaissance of a syndrome – Young – 2007 – Clinical Endocrinology – Wiley Online Library”.
  4. ↑ So A, Duffy DL, Gordon RD, Jeske YW, Lin-Su K, New MI, Stowasser M (2005). “Familial hyperaldosteronism type II is linked to the chromosome 7p22 region but also shows predicted heterogeneity”. J. Hypertens. 23 (8): 1477–84. PMID 16003173.
  5. ↑ Song MS, Seo SW, Bae SB, Kim YJ, Kim SJ (2012). “Aldosterone-producing adrenocortical carcinoma without hypertension”. Korean J. Intern. Med. 27 (2): 221–3. doi:10.3904/kjim.2012.27.2.221. PMC 3372808. PMID 22707896.

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Differentiating Conn syndrome from other Diseases

Differentiating Conn syndrome from other Diseases

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Syed Hassan A. Kazmi BSc, MD [2]

Overview

Primary hyperaldosteronism must be differentiated from other diseases that cause hypertension and hypokalemia, such as renal artery stenosis, cushing’s syndrome, congenital adrenal hyperplasia, Liddle’s syndrome, diuretic use, licorice ingestion, and renin-secreting tumors.

Differentiating Primary Hyperaldosteronism from other Diseases

Primary hyperaldosteronism (PA) should be differentiated from other diseases causing hypertension and hypokalemia for example:[1][1][2][3][4][5][6][7][8][9][10][11][12][13][14][15]

 
 
 
 
 
 
 
 
Hypertension and Hypokalemia
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Plasma renin activity
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Normal or High (Plasma Renin/Aldosterone ratio <10)
 
 
 
 
 
 
 
 
 
 
 
Suppressed (Plasma Renin/Aldosterone ratio > 20)
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
• Renin-secreting tumors
• Diuretic use
• Renovascular hypertension
• Coarctation of aorta
• Malignant hypertension
 
 
 
 
 
 
 
 
 
 
 
Urinary aldosterone
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Elevated
 
Normal
 
 
Low
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Conn’s syndrome (Primary aldosteronism)
 
Profound K depletion
 
 
• 17 alpha hydroxylase deficiency
• 11 beta hydroxylase deficiency
• Liddle’s syndrome
• Licorice ingestion
• Deoxycortisone producing tumor
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Add Mineralocrticoid antagonist for 8 weeks
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
BP response
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
No BP response
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
• Deoxycorticosterone excess (Tumor, 17 alpha hydroxylase, and 11 beta hydroxylase deficiency)
• Licorice ingestion
• Glucocorticoid resistance
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Liddle’s syndrome
Differential Diagnoses Clinical features History Findings Laboratory Findings
Headache and hypertension Nausea and vomiting Palpitations Shortness of breath Diminished pulses Fatigue Constipation Pruritis Ambiguous genitalia
Renin-Secreting tumors ✔ ✔ ✔ ✔ – – – – –
Coarctation of aorta ✔ ✔ ✔ ✔ ✔ ✔ – – –
11-beta hydroxylase deficiency ✔ (Hypertensive crisis due to increased 11-deoxycorticosterone-11-DOC) ✔ ✔ – – ✔ – – ✔
17-alpha hydroxylase deficiency ✔ ✔ ✔ – – – – – ✔
Uremia ✔ ✔ ✔ – ✔ ✔ ✔ –
Liddle’s syndrome ✔ ✔ ✔ – – – ✔ – –

References

  1. ↑ 1.0 1.1 Wada N, Jin S, Hui SP, Yanagisawa K, Kurosawa T, Chiba H (2014). “[Differential diagnosis of primary aldosteronism by measurement of hybrid steroids using mass spectrometry]”. Rinsho Byori (in Japanese). 62 (3): 276–82. PMID 24800505.
  2. ↑ Nielsen ML, Pareek M, Andersen I (2012). “[Liquorice-induced hypertension and hypokalaemia]”. Ugeskr. Laeg. (in Danish). 174 (15): 1024–5. PMID 22487411.
  3. ↑ Chow KM, Ma RC, Szeto CC, Li PK (2012). “Polycystic kidney disease presenting with hypertension and hypokalemia”. Am. J. Kidney Dis. 59 (2): 270–2. doi:10.1053/j.ajkd.2011.08.020. PMID 21962616.
  4. ↑ Sarafidis PA, Georgianos PI, Germanidis G, Giavroglou C, Nikolaidis P, Lasaridis AN, Madias NE (2012). “Hypertension and symptomatic hypokalemia in a patient with simultaneous unilateral stenoses of intrarenal arteries and mesangioproliferative glomerulonephritis”. Am. J. Kidney Dis. 59 (3): 434–8. doi:10.1053/j.ajkd.2011.11.001. PMID 22154539.
  5. ↑ Khosla N, Hogan D (2006). “Mineralocorticoid hypertension and hypokalemia”. Semin. Nephrol. 26 (6): 434–40. doi:10.1016/j.semnephrol.2006.10.004. PMID 17275580.
  6. ↑ Weiner ID (2013). “Endocrine and hypertensive disorders of potassium regulation: primary aldosteronism”. Semin. Nephrol. 33 (3): 265–76. doi:10.1016/j.semnephrol.2013.04.007. PMC 3748390. PMID 23953804.
  7. ↑ Martell-Claros N, Abad-Cardiel M, Alvarez-Alvarez B, García-Donaire JA, Pérez CF (2015). “Primary aldosteronism and its various clinical scenarios”. J. Hypertens. 33 (6): 1226–32. doi:10.1097/HJH.0000000000000546. PMID 25715092.
  8. ↑ Franse LV, Pahor M, Di Bari M, Somes GW, Cushman WC, Applegate WB (2000). “Hypokalemia associated with diuretic use and cardiovascular events in the Systolic Hypertension in the Elderly Program”. Hypertension. 35 (5): 1025–30. PMID 10818057.
  9. ↑ Rossi E, Farnetti E, Nicoli D, Sazzini M, Perazzoli F, Regolisti G, Grasselli C, Santi R, Negro A, Mazzeo V, Mantero F, Luiselli D, Casali B (2011). “A clinical phenotype mimicking essential hypertension in a newly discovered family with Liddle’s syndrome”. Am. J. Hypertens. 24 (8): 930–5. doi:10.1038/ajh.2011.76. PMID 21525970.
  10. ↑ Ruecker B, Lang-Muritano M, Spanaus K, Welzel M, l’Allemand D, Phan-Hug F, Katschnig C, Konrad D, Holterhus PM, Schoenle EJ (2015). “The Aldosterone/Renin Ratio as a Diagnostic Tool for the Diagnosis of Primary Hypoaldosteronism in Newborns and Infants”. Horm Res Paediatr. 84 (1): 43–8. doi:10.1159/000381852. PMID 25968592.
  11. ↑ Ardhanari S, Kannuswamy R, Chaudhary K, Lockette W, Whaley-Connell A (2015). “Mineralocorticoid and apparent mineralocorticoid syndromes of secondary hypertension”. Adv Chronic Kidney Dis. 22 (3): 185–95. doi:10.1053/j.ackd.2015.03.002. PMID 25908467.
  12. ↑ Iglesias P, Tajada P, Martínez I, Díez JJ (2009). “[Salt-wasting congenital adrenal hyperplasia associated to hyperreninemic hyperaldosteronism]”. Med Clin (Barc) (in Spanish; Castilian). 132 (2): 80–1. doi:10.1016/j.medcli.2008.09.002. PMID 19174076.
  13. ↑ Kikuta Y, Sanjo K, Nakajima K, Ashizawa I, Ojima M (1988). “Primary aldosteronism in childhood due to primary adrenal hyperplasia”. Tohoku J. Exp. Med. 155 (1): 57–70. PMID 3413779.
  14. ↑ Hassan-Smith Z, Stewart PM (2011). “Inherited forms of mineralocorticoid hypertension”. Curr Opin Endocrinol Diabetes Obes. 18 (3): 177–85. doi:10.1097/MED.0b013e3283469444. PMID 21494136.
  15. ↑ Bartter FC, Henkin RI, Bryan GT (1968). “Aldosterone hypersecretion in “non-salt-losing” congenital adrenal hyperplasia”. J. Clin. Invest. 47 (8): 1742–52. doi:10.1172/JCI105864. PMC 297334. PMID 4299011.

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Epidemiology and Demographics

Epidemiology and Demographics


Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Syed Hassan A. Kazmi BSc, MD [2]

Overview

Prevalence of primary hyperaldosteronism is from 1,400-32,000 (median, 8,800) per 100,000 individuals around the world. Primary hyperaldosteronism incidence among patients with newly diagnosed hypertension is 11,200 of per 100,000 individuals. The case-fatality rate of primary hyperaldosteronism is approximately 23.4 per 1,000 individual-years. Patients of all age groups may develop primary hyperaldosteronism. There is no racial predilection for primary hyperaldosteronism.

Epidemiology and Demographics

Prevalence

  • Prevalence of primary hyperaldosteronism varies from 1,400 to 32,000 (median, 8,800) per 100,000 individuals around the world.[1][2]
  • In patients with resistant hypertension, the prevalence of primary hyperaldosteronism is reported to be even higher, ranging from a low of 17,000 per 100,000 patients to a high of 23,000 per 100,000 patients.[3]
  • The prevalence of familial hyperaldosteronism type II ranges from 1.2 to 6% in adult populations of primary hyperaldosteronism.[4]

Incidence

  • Primary hyperaldosteronism incidence among patients with newly diagnosed hypertension is 11,200 of per 100,000 individuals.[5]

Case-Fatality rate

  • The case-fatality rate of primary hyperaldosteronism is approximately 23.4 per 1,000 individual-years.[6]

Age

  • Patients of all age groups may develop primary hyperaldosteronism.

Gender

  • Familial hyperaldosteronism type I accounts for 0.5 to 1.0% of primary hyperaldosteronism and occurs equally among women and men.[7]
  • Males are more commonly affected by unilateral adrenal hyperplasia than females. The male to female ratio is approximately 4 to 1.
  • Females are more commonly affected by adrenal adenomas.
  • Bilateral adrenal hyperplasia affects men and women equally.[8]

Race

  • There is no racial predilection for primary hyperaldosteronism.[9]
  • Blacks have been found to have lower plasma renin activity than other populations.[10]

References

  1. ↑ Rossi GP, Pessina AC, Heagerty AM (2008). “Primary aldosteronism: an update on screening, diagnosis and treatment”. J. Hypertens. 26 (4): 613–21. doi:10.1097/HJH.0b013e3282f4b3e6. PMID 18327065.
  2. ↑ Rossi GP, Seccia TM, Pessina AC (2007). “Clinical use of laboratory tests for the identification of secondary forms of arterial hypertension”. Crit Rev Clin Lab Sci. 44 (1): 1–85. doi:10.1080/10408360600931831. PMID 17175520.
  3. ↑ Stowasser M, Taylor PJ, Pimenta E, Ahmed AH, Gordon RD (2010). “Laboratory investigation of primary aldosteronism”. Clin Biochem Rev. 31 (2): 39–56. PMC 2874431. PMID 20498828.
  4. ↑ Stowasser M, Gordon RD (2000). “Primary aldosteronism: learning from the study of familial varieties”. J. Hypertens. 18 (9): 1165–76. PMID 10994747.
  5. ↑ Rossi GP (2010). “Prevalence and diagnosis of primary aldosteronism”. Curr. Hypertens. Rep. 12 (5): 342–8. doi:10.1007/s11906-010-0134-2. PMID 20665130.
  6. ↑ Wu, Vin-Cent; Wang, Shuo-Meng; Chang, Chia-Hui; Hu, Ya-Hui; Lin, Lian-Yu; Lin, Yen-Hung; Chueh, Shih-Chieh Jeff; Chen, Likwang; Wu, Kwan-Dun (2016). “Long term outcome of Aldosteronism after target treatments”. Scientific Reports. 6 (1). doi:10.1038/srep32103. ISSN 2045-2322.
  7. ↑ “Evidence for Abnormal Left Ventricular Structure and Function in Normotensive Individuals with Familial Hyperaldosteronism Type I | The Journal of Clinical Endocrinology & Metabolism | Oxford Academic”.
  8. ↑ Woo K, Waisman J, Melamed J, Lepor H (2000). “Primary aldosteronism caused by unilateral adrenal hyperplasia”. Rev Urol. 2 (2): 100–4. PMC 1476104. PMID 16985748.
  9. ↑ Calhoun DA, Nishizaka MK, Zaman MA, Thakkar RB, Weissmann P (2002). “Hyperaldosteronism among black and white subjects with resistant hypertension”. Hypertension. 40 (6): 892–6. PMID 12468575.
  10. ↑ Lee MR, Critchley JA, Gordon CJ, Makarananda K, Sriwatanakul K, Balali-Mood M, Boye GL (1990). “Ethnic differences in the renal sodium dopamine relationship. A possible explanation for regional variations in the prevalence of hypertension?”. Am. J. Hypertens. 3 (6 Pt 2): 100S–103S. PMID 2383374.

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Risk Factors

Risk Factors

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Syed Hassan A. Kazmi BSc, MD [2]

Overview

The only risk factors in the development of primary hyperaldosteronism are age of hypertension onset and serum potassium level.

Risk Factors

The only risk factors in the development of primary hyperaldosteronism are age of onset of hypertension and serum potassium level.[1]


References

  1. ↑ Sang X, Jiang Y, Wang W, Yan L, Zhao J, Peng Y, Gu W, Chen G, Liu W, Ning G (2013). “Prevalence of and risk factors for primary aldosteronism among patients with resistant hypertension in China”. J. Hypertens. 31 (7): 1465–71, discussion 1471–2. doi:10.1097/HJH.0b013e328360ddf6. PMID 24006040.

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Screening

Screening

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Syed Hassan A. Kazmi BSc, MD [2]

Overview

There is insufficient evidence to recommend routine screening for primary hyperaldosteronism but according to the Endocrine Society Clinical Practice Guideline, screening for hyperaldosteronism is recommended for resistant hypertension by checking the plasma aldosterone to renin ratio (PAC/PRA).

Screening

Screening population

The following individuals should be screened for primary hyperaldosteronism:[1]

Plasma Aldosterone to Renin Ratio (PAC/PRA)

The plasma aldosterone to renin ratio is widely used as a screening test for hyperaldosteronism.[2][3][4]

Protocol

Interpretation

References

  1. ↑ Horsley MG, Bailie GR (1988). “Effectiveness of theophylline monitoring by the use of serum assays”. J Clin Pharm Ther. 13 (5): 359–64. PMID 3230101.
  2. ↑ Ríos MC, Izquierdo A, Sotelo M, Honnorat E, Rodríguez Cuimbra S, Catay E, Popescu BM (2011). “[Aldosterone/renin ratio in the diagnosis of primary aldosteronism]”. Medicina (B Aires) (in Spanish; Castilian). 71 (6): 525–30. PMID 22167725.
  3. ↑ Pilz S, Kienreich K, Gaksch M, Grübler M, Verheyen N, Bersuch LA, Schmid J, Drechsler C, Ritz E, Moosbrugger A, Stepan V, Pieber TR, Meinitzer A, März W, Tomaschitz A (2014). “Aldosterone to active Renin ratio as screening test for primary aldosteronism: reproducibility and influence of orthostasis and salt loading”. Horm. Metab. Res. 46 (6): 427–32. doi:10.1055/s-0034-1367033. PMID 24526370.
  4. ↑ “The Management of Primary Aldosteronism: Case Detection, Diagnosis, and Treatment: An Endocrine Society Clinical Practice Guideline: The Journal of Clinical Endocrinology & Metabolism: Vol 101, No 5”.
  5. ↑ Doi SA, Abalkhail S, Al-Qudhaiby MM, Al-Humood K, Hafez MF, Al-Shoumer KA (2006). “Optimal use and interpretation of the aldosterone renin ratio to detect aldosterone excess in hypertension”. J Hum Hypertens. 20 (7): 482–9. doi:10.1038/sj.jhh.1002024. PMID 16617310.
  6. ↑ Pilz S, Kienreich K, Gaksch M, Grübler M, Verheyen N, Bersuch LA, Schmid J, Drechsler C, Ritz E, Moosbrugger A, Stepan V, Pieber TR, Meinitzer A, März W, Tomaschitz A (2014). “Aldosterone to active Renin ratio as screening test for primary aldosteronism: reproducibility and influence of orthostasis and salt loading”. Horm. Metab. Res. 46 (6): 427–32. doi:10.1055/s-0034-1367033. PMID 24526370.

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Natural history, Complications and Prognosis

Natural history, Complications and Prognosis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Syed Hassan A. Kazmi BSc, MD [2]

Overview

If left untreated, patients with primary hyperaldosteronism may progress to develop stroke, coronary artery disease, and renal insufficiency with associated proteinuria. Aldosterone producing adenomas (APAs) continue to grow slowly over time. The aldosterone production likely correlates with the size of the adenoma. It is a progressive disease and its common complications include left ventricular hypertrophy due to chronic hypertension, atrial fibrillation, myocardial infarction, stroke, proteinuria, and metabolic syndrome. The prognosis of primary hyperaldosteronism is good with treatment. Without treatment, primary hyperaldosteronism will result in hypertension with resultant hypertension-related complications, which may be a major cause of morbidity and mortality among patients.

Natural History, Complications, and Prognosis

Natural History

Complications

Primary aldosteronism is characterized by the development of the following complications:[3][4][5][6][7][8]

Cardiovascular complications

Neurological complications

Renal complications

Metabolic complications

Prognosis

  • The prognosis of primary hyperaldosteronism is good with treatment. Without treatment, primary hyperaldosteronism will result in hypertension with resultant hypertension-related complications, which may be a major cause of morbidity and mortality among patients.
  • Adrenalectomy lowers long-term all-cause mortality from primary hyperaldosteronism.[14][15][16]

Patients undergoing unilateral adrenalectomy for unilateral adenoma

Patients receiving aldosterone antagonist medications

Patients with FH-I undergoing treatment with glucocorticoid medications

References

  1. ↑ Gordon RD (1997). “Primary aldosteronism: a new understanding”. Clin. Exp. Hypertens. 19 (5–6): 857–70. PMID 9247760.
  2. ↑ “Cardiovascular complications in patients with primary aldosteronism – ScienceDirect”.
  3. ↑ Nishimura M, Uzu T, Fujii T, Kuroda S, Nakamura S, Inenaga T, Kimura G (1999). “Cardiovascular complications in patients with primary aldosteronism”. Am. J. Kidney Dis. 33 (2): 261–6. PMID 10023636.
  4. ↑ Giacchetti G, Turchi F, Boscaro M, Ronconi V (2009). “Management of primary aldosteronism: its complications and their outcomes after treatment”. Curr Vasc Pharmacol. 7 (2): 244–49. PMID 19356005.
  5. ↑ 5.0 5.1 Reincke M, Meisinger C, Holle R, Quinkler M, Hahner S, Beuschlein F, Bidlingmaier M, Seissler J, Endres S (2010). “Is primary aldosteronism associated with diabetes mellitus? Results of the German Conn’s Registry”. Horm. Metab. Res. 42 (6): 435–9. doi:10.1055/s-0029-1246189. PMID 20119885.
  6. ↑ Hanslik G, Wallaschofski H, Dietz A, Riester A, Reincke M, Allolio B, Lang K, Quack I, Rump LC, Willenberg HS, Beuschlein F, Quinkler M, Hannemann A (2015). “Increased prevalence of diabetes mellitus and the metabolic syndrome in patients with primary aldosteronism of the German Conn’s Registry”. Eur. J. Endocrinol. 173 (5): 665–75. doi:10.1530/EJE-15-0450. PMID 26311088.
  7. ↑ 7.0 7.1 Gordon RD (1995). “Primary aldosteronism”. J. Endocrinol. Invest. 18 (7): 495–511. doi:10.1007/BF03349761. PMID 9221268.
  8. ↑ 8.0 8.1 “Prevalence and Characteristics of the Metabolic Syndrome in Primary Aldosteronism | The Journal of Clinical Endocrinology & Metabolism | Oxford Academic”.
  9. ↑ Wu VC, Kuo CC, Wang SM, Liu KL, Huang KH, Lin YH, Chu TS, Chang HW, Lin CY, Tsai CT, Lin LY, Chueh SC, Kao TW, Chen YM, Chiang WC, Tsai TJ, Ho YL, Lin SL, Wang WJ, Wu KD (2011). “Primary aldosteronism: changes in cystatin C-based kidney filtration, proteinuria, and renal duplex indices with treatment”. J. Hypertens. 29 (9): 1778–86. doi:10.1097/HJH.0b013e3283495cbb. PMID 21738054.
  10. ↑ Novello M, Catena C, Nadalini E, Colussi GL, Baroselli S, Chiuch A, Lapenna R, Bazzocchi M, Sechi LA (2007). “Renal cysts and hypokalemia in primary aldosteronism: results of long-term follow-up after treatment”. J. Hypertens. 25 (7): 1443–50. doi:10.1097/HJH.0b013e328126855b. PMID 17563567.
  11. ↑ Fallo F, Federspil G, Veglio F, Mulatero P (2007). “The metabolic syndrome in primary aldosteronism”. Curr. Hypertens. Rep. 9 (2): 106–11. PMID 17442220.
  12. ↑ “Metabolic syndrome in primary aldosteronism and essential hypertension: Relationship to adiponectin gene variants – ScienceDirect”.
  13. ↑ Ronconi V, Turchi F, Rilli S, Di Mattia D, Agostinelli L, Boscaro M, Giacchetti G (2010). “Metabolic syndrome in primary aldosteronism and essential hypertension: relationship to adiponectin gene variants”. Nutr Metab Cardiovasc Dis. 20 (2): 93–100. doi:10.1016/j.numecd.2009.03.007. PMID 19481913.
  14. ↑ “Long term outcome of Aldosteronism after target treatments | Scientific Reports”.
  15. ↑ “Treatment strategy and outcome with primary aldosteronism: a nationwide longitudinal cohort based study”.
  16. ↑ Celen O, O’Brien MJ, Melby JC, Beazley RM (1996). “Factors influencing outcome of surgery for primary aldosteronism”. Arch Surg. 131 (6): 646–50. PMID 8645073.
  17. ↑ 17.0 17.1 Stowasser M, Gordon RD, Gunasekera TG, Cowley DC, Ward G, Archibald C, Smithers BM (2003). “High rate of detection of primary aldosteronism, including surgically treatable forms, after ‘non-selective’ screening of hypertensive patients”. J. Hypertens. 21 (11): 2149–57. doi:10.1097/01.hjh.0000098141.70956.53. PMID 14597859.
  18. ↑ 18.0 18.1 Stowasser M, Gordon RD (2004). “Primary aldosteronism–careful investigation is essential and rewarding”. Mol. Cell. Endocrinol. 217 (1–2): 33–9. doi:10.1016/j.mce.2003.10.006. PMID 15134798.
  19. ↑ Stowasser M, Gordon RD, Rutherford JC, Nikwan NZ, Daunt N, Slater GJ (2001). “Diagnosis and management of primary aldosteronism”. J Renin Angiotensin Aldosterone Syst. 2 (3): 156–69. doi:10.3317/jraas.2001.022. PMID 11881117.
  20. ↑ Stowasser M, Gartside MG, Gordon RD (1997). “A PCR-based method of screening individuals of all ages, from neonates to the elderly, for familial hyperaldosteronism type I”. Aust N Z J Med. 27 (6): 685–90. PMID 9483237.

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Diagnosis

Diagnosis

History and Symptoms | Physical Examination | Laboratory Findings | Other Imaging Findings | Other Diagnostic Studies

Treatment

Treatment

Medical Therapy | Surgery | Cost-Effectiveness of Therapy

Case Studies

Case Studies

Case #1


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