Bilateral adrenal hyperplasia, with or without nodule formation, is the most common cause of primary aldosteronism in childhood89 (see Chap. 80). Boys have a greater incidence of bilateral hyperplasia than girls do. Familial glucocorticoid-suppressible aldosteronism (Sutherland syndrome) arises from a mutation that causes a chimeric gene for aldosterone synthase, which contains the ACTH promoter from the homologous 11β-hydroxylase gene.22 Primary hyperaldosteronism may be due to familial hyperplasia or tumor.89a Solitary aldosteronoma or ectopic tumors are rare in children.

Excessive secretion of deoxycorticosterone, corticosterone, or cortisol itself may account for mineralocorticoid excess in cases of Cushing syndrome, 17-hydroxylation deficiency, nonendo-crine carcinomas, and hyperthyroidism.54,55 Primary cortisol resistance is an autosomal disease in which coexistent elevations in deoxycorticosterone levels may cause a mineralocorticoid excess syndrome.64,65 Resistance to multiple steroids may be found.65a The syndrome of apparent mineralocorticoid excess resembles pseudoaldosteronism. It appears to arise from heterogeneous defects in cortisol metabolism that allow filtered cortisol to escape inactivation by renal tubular 11β-hydroxysteroid dehy-drogenase.22,90 Licorice abuse produces the syndrome by competitively inhibiting this enzyme, thereby permitting filtered cortisol to reach renal mineralocorticoid receptors.91