Primary Aldosteronism

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Chapter 572 Primary Aldosteronism

Perrin C. White

Primary aldosteronism encompasses disorders caused by excessive aldosterone secretion independent of the renin-angiotensin system. These disorders are characterized by hypertension, hypokalemia, and suppression of the renin-angiotensin system.

Etiology

Aldosterone-secreting adenomas are unilateral and have been reported in children as young as yr of age; they mainly affect girls. Bilateral micronodular adrenocortical hyperplasia tends to occur in older children and is more frequent in males. Primary aldosteronism due to unilateral adrenal hyperplasia may also occur. Glucocorticoid-suppressible hyperaldosteronism is discussed in Chapter 570.

Epidemiology

These conditions are thought to be rare in children, but they may account for 5-10% of cases of hypertension in adults. Although usually sporadic, kindreds with several affected members have been reported. Genetic linkage to chromosome 7p22 has been identified in some of these kindreds, but the involved gene has not yet been identified.

Clinical Manifestations

Some affected children have no symptoms, the diagnosis being established after incidental discovery of moderate hypertension. Others have severe hypertension (up to 240/150 mm Hg), with headache, dizziness, and visual disturbances. Chronic hypokalemia, if present, may lead to polyuria, nocturia, enuresis, and polydipsia. Muscle weakness and discomfort, tetany, intermittent paralysis, fatigue, and growth failure affect children with severe hypokalemia.

Laboratory Findings

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