Review article
Clinical manifestation of aldosteronoma

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Primary hyperaldosteronism

Aldosteronoma is an important cause of hypertension that is surgically curable. In 1954, Jerome Conn described a patient with hypertension who was cured by the removal of a benign aldosterone-producing adrenal adenoma, and predicted the cure [1]. The hallmarks of Conn's syndrome are elevated aldosterone production and a suppressed renin. Resent reports suggest that primary hyperaldosteronism is frequently overlooked in normokalemic patients.

Why should we screen for and treat primary hyperaldosteronism?

Aldosterone may cause cardiovascular complications, such as cardiac myopathy, independent of its effect on increased blood pressure [2]. Mineralocorticoid receptors are present in the heart, brain, blood vessels, kidney, and colon. Patients with primary hyperaldosteronism may be at higher risk than other hypertensive patients for damage to the heart and the kidney. The left-ventricular hypertrophy (LVH) in these patients is disproportionately greater than for the same level of elevated blood

Etiology and pathophysiology

Aldosterone is a mineralocorticoid hormone produced by the zona glomerulosa. Primary hyperaldosteronism results from autonomous aldosterone production, and is defined as hypertension associated with increased aldosterone production and suppressed renin concentration or activity. In primary hyperaldosteronism, the excessive aldosterone production is no longer under the control of the renin-angiotensin system, and is not necessarily responsive to the inhibitory effects of low serum potassium

Clinical and biochemical features

Primary hyperaldosteronism is usually diagnosed in patients in the third to sixth decades, and very rarely in children. Patients with aldosteronoma tend to be younger and have more pronounced biochemical changes than those with IHA.

Most patients are relatively asymptomatic. When they occur, symptoms may be related to the effect of hypertension (eg, headache) or hypokalemia (eg, polyuria, nocturia, muscle cramps, paresthesia, muscle weakness, or paralysis). Women with primary hyperaldosteronism

Prevalence

Conn et al described ā€œnormokalemic primary hyperaldosteronism,ā€ and suggested that primary hyperaldosteronism may exist for many years before hypokalemia becomes demonstrable [26]. Conn suggested using a combination of high aldosterone and low renin as a screening test [27], but until recently only patients with hypertension and hypokalemia were tested for primary hyperaldosteronism. Because hypokalemia is only variably present in patients with primary hyperaldosteronism, its true prevalence

Who should be screened?

The widespread use of PAC/PRA ratio for screening, imaging studies that incidentally detected adrenal tumors, and advances in laparoscopic adrenalectomy have all contributed to more adrenalectomies being performed for aldosteronoma.

It is controversial whether routine screening of all hypertensive patients by plasma aldosterone level, or renin activity, or both, is cost-effective. Some argue that wider screening is likely to find more patients with primary hyperaldosteronism due to IHA, which is

Confirmation

In some patients, especially those with borderline results for the screening test, the diagnosis of primary hyperaldosteronism can be confirmed by physiologic maneuvers to inhibit or to stimulate aldosterone and renin secretion, using sodium loading or depletion respectively. Primary hyperaldosteronism causes autonomous aldosterone production that is not suppressed by saline loading, oral salt loading, or fludrocortisone.

Differential diagnosis between aldosteronoma and IHA

Once the biochemical diagnosis of primary hyperaldosteronism is established, aldosteronoma and IHA can be distinguished by a variety of tests.

Treatment

Spironolactone, 400 mg/day for 3 to 6 weeks before adrenalectomy, is useful to control hypertension, restore potassium store, and allow the contralateral adrenal gland to recover. Laparoscopic adrenalectomy is the treatment of choice for aldosteronoma, because of fewer complications and shorter hospitalization and recovery time compared with open adrenalectomy [37], [38], [39], [40], [41], [42], [43]. The patient should be advised that blood pressure might not become completely normal. Some

Summary

Formerly, fewer than 1% of patients with hypertension were believed to have primary hyperaldosteronism; however, recent studies have suggested a higher prevalence, in 5% to 10% of patients with hypertension. Hypokalemia is not necessary for the diagnosis and is probably a sign of more advanced disease. The best diagnostic test is the PAC/PRA ratio. Excess aldosterone level has a deleterious effect on the cardiovascular system. Aldosteronomas should be differentiated from IHA, because they are

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References (75)

  • E Rossi et al.

    High prevalence of primary aldosteronism using postcaptopril plasma aldosterone to renin ratio as a screening test among Italian hypertensives

    Am J Hypertens

    (2002)
  • W.F Young et al.

    Primary aldosteronism: diagnosis and treatment

    Mayo Clin Proc

    (1990)
  • C.A Proye et al.

    Essential hypertension: first reason for persistent hypertension after unilateral adrenalectomy for primary aldosteronism?

    Surgery

    (1998)
  • Y Horita et al.

    Cause of residual hypertension after adrenalectomy in patients with primary aldosteronism

    Am J Kidney Dis

    (2001)
  • D Clarke et al.

    Severe hypertension in primary aldosteronism and good response to surgery

    Lancet

    (1979)
  • K Jeschke et al.

    Laparoscopic partial adrenalectomy in patients with aldosterone-producing adenomas: indications, technique, and results

    Urology

    (2003)
  • J.W Conn

    Primary aldosteronism

    J Lab Clin Med

    (1955)
  • G.P Rossi et al.

    Left ventricular systolic function in primary aldosteronism and hypertension

    J Hypertens

    (1998)
  • G.P Rossi et al.

    Remodeling of the left ventricle in primary aldosteronism due to Conn's adenoma

    Circulation

    (1997)
  • C.G Brilla et al.

    Remodeling of the rat right and left ventricles in experimental hypertension

    Circ Res

    (1990)
  • V.E Torres et al.

    Association of hypokalemia, aldosteronism, and renal cysts

    N Engl J Med

    (1990)
  • J.M Halimi et al.

    Albuminuria in untreated patients with primary aldosteronism or essential hypertension

    J Hypertens

    (1995)
  • M Ogasawara et al.

    Clinical implications of renal cyst in primary aldosteronism

    Endocr J

    (1996)
  • M Fritsch Neves et al.

    Aldosterone: a risk factor for vascular disease

    Curr Hypertens Rep

    (2003)
  • M Sywak et al.

    Long-term follow-up and cost benefit of adrenalectomy in patients with primary hyperaldosteronism

    Br J Surg

    (2002)
  • W.F Young

    Minireview: primary aldosteronismā€”changing concepts in diagnosis and treatment

    Endocrinology

    (2003)
  • Y Ito et al.

    Clinical significance of associated nodular lesions of the adrenal in patients with aldosteronoma

    World J Surg

    (1990)
  • B Strauch et al.

    Prevalence of primary hyperaldosteronism in moderate to severe hypertension in the Central Europe region

    J Hum Hypertens

    (2003)
  • R.P Lifton et al.

    A chimaeric 11 beta-hydroxylase/aldosterone synthase gene causes glucocorticoid-remediable aldosteronism and human hypertension

    Nature

    (1992)
  • R.P Lifton et al.

    The molecular basis of glucocorticoid-remediable aldosteronism, a Mendelian cause of human hypertension

    Trans Assoc Am Physicians

    (1992)
  • M Stowasser et al.

    Primary aldosteronism: learning from the study of familial varieties

    J Hypertens

    (2000)
  • S Todesco et al.

    Primary aldosteronism due to a malignant ovarian tumor

    J Clin Endocrinol Metab

    (1975)
  • B Tetu et al.

    Renin-producing ovarian tumor. A case report with immunohistochemical and electron-microscopic study

    Am J Surg Pathol

    (1988)
  • B Jackson et al.

    Primary aldosteronism due to a malignant ovarian tumour

    Aust N Z J Med

    (1986)
  • C.E Fardella et al.

    Prevalence of primary aldosteronism in unselected hypertensive populations: screening and definitive diagnosis

    J Endocrinol Metab

    (2001)
  • P Mulatero et al.

    Diagnosis of glucocorticoid-remediable aldosteronism in primary aldosteronism: aldosterone response to dexamethasone and long polymerase chain reaction for chimeric gene

    J Clin Endocrinol Metab

    (1998)
  • J.W Conn et al.

    Suppression of plasma renin activity in primary aldosteronism

    JAMA

    (1964)

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