Mineralocorticoid Disorders and Endocrine Hypertension

The renin-angiotensin-aldosterone system regulates intravascular volume and serum potassium concentration through its regulation of sodium and potassium handling in the distal nephron of the kidneys. Isolated disorders of this system in children are rare but when present can cause significant morbidity from either deficient or excess mineralocorticoid action. This chapter describes the physiology of mineralocorticoid action followed by the mechanisms responsible for disordered mineralocorticoid action. For each disorder, the appropriate diagnostic evaluation and treatment recommendations are presented.