Hyperaldosteronism familial type 2
Overview
Type 2 familial hyperaldosteronism, inherited as an autosomal dominant trait, causes hypertension secondary to primary aldosteronism that is not suppressed with dexamethasone. Patients present with an adrenal adenoma that secretes aldosterone. The observation of several families affected suggests that the molecular mechanism may be different than in type I familial hyperaldosteronism, but remains to be identified. Diagnostic suspicion should lead to the screening of patients by determining the aldosterone/renin ratio.
Causes
* Primary hyperaldosteronism o APA - High aldosterone, low PRA o IHA - Responds to posture (bilateral adrenal hyperplasia) o Primary adrenal hyperplasia - Responds to posture (unilateral disease) o GRA - Sustained suppression of aldosterone ( 2 ng/mL) o Renovascular hypertension o Renin-producing tumors o Pregnancy * Conditions that mimic aldosterone excess o Congenital adrenal hyperplasia (11beta-hydroxylase deficiency, 17alpha-hydroxlyase deficiency) - Low aldosterone, low PRA, elevated steroid intermediates o Primary glucocorticoid resistance - High glucocorticoid secretion unsuppressed by dexamethasone o Deoxycorticosterone-secreting tumors - Elevated deoxycorticosterone levels o Syndrome of apparent mineralocorticoid excess o Liddle syndrome o Licorice ingestion o Carbenoxolone