Primary hyperaldosteronism (PAL), first described by Conn in 1954 in association with an aldosterone-producing adrenal adenoma (36), is rare in children. Other causes of PAL, which are also rare in children, include familial hyperaldosteronism type I (described as GRA above) and type II, idiopathic bilateral hyperplasia, and aldosterone-producing adrenal carcinoma. Once, PAL was thought to be a rare condition in adults, accounting for less than 1% of patients with hypertension. Improvement in assay techniques of aldosterone and renin values and screening approaches has changed this view. The prevalence rate ranges from 8-13% in nonselected essential hypertensives across studies in many different countries. (37)

It can present with hypertension, and symptoms of hypokalemia that include tiredness, muscle weakness, thirst, polyuria and nocturia. Normal potassium levels are often associated with PAL, so that past use of hypokalemia as a screening test may have led to underestimation of hyperaldosteronism as a cause of hypertension. The recent use of the ratio of plasma aldosterone to PRA is a more sensitive test and has increased the detection of hyperaldosteronism in hypertensive patients.

PAL in childhood is more likely to be caused by bilateral adrenal hyperplasia in which non-autonomous overproduction of aldosterone occurs. This can be differentiated from other causes of PAL by increased aldosterone secretion in response to upright posture and angiotensin II infusion.

Another rare cause is familial hyperaldosteronism type II (FHII), the first cases being described by Gordon et al. in 1991 in three families with a familial variety of PAL. (38) It is distinguished from type I (GRA) by failure of suppression of aldosterone by dexamethasone and no hybrid gene mutation. Patients with FH II are older than those with FH I, perhaps owing to diagnosis of FH I at a younger age, made possible by genetic testing. No significant in age, sex, biochemical parameters, or aldosterone and renin levels were seen between patients with FH II and those with apparently sporadic PAL. (10) It has been described both in families and in sporadic cases worldwide, with the range in age starting at 14 years and equal gender distribution. (39) Although the inheritance in many families appears to be autosomal dominant, in sporadic cases it is still uncertain. Surgical treatment in the case of unilateral adrenal mass and medical treatment with mineralocorticoid receptor antagonists can be effective.(10)