Table 4.

Rare, Recently Discovered Identifiable Hypertension

Disorder	Approximate Prevalence or Gene Frequency	Genetic Origin: Pattern	Clinical Features	Specific Treatment
Congenital adrenal hyperplasia with hypertension 11‐OH dehydroxylase deficiency		Yes	Virilization, precocious puberty	Glucocorticoid management
17‐OH hydroxylase deficiency	Very rare	Yes	Women: lack of feminizing at puberty	Glucocorticoid management
Apparent mineralocorticoid excess 11 steroid dehydrogenase deficiency	Very rare	Yes	Early onset, hypokalemia, low renin, low aldosterone, normal cortisol	Glucocorticoid management
Glucocorticoid remediable hypertension. Type I familial aldosteronism	Very rare	Yes	Early onset, low renin, elevated aldosterone corrects with low dose glucocorticoids	Glucocorticoid management
Familial aldosteronism, not glucocorticoid remediable hypertension. Type II familial aldosteronism	Very rare	Yes	Hypertension, low potassium, low renin, high aldosterone	Mineralocorticoid receptor blockade. In rare cases, adrenalectomy
Pseudohypoaldosteronism type 2, Gordon's syndrome	Very rare	Yes	Hypertension with high potassium, low renin, low aldosterone	Thiazide‐type diuretic
Liddle syndrome	Very rare	Noa	Hypertension, low potassium, low renin and normal‐low aldosterone	Amiloride monotherapy
Renin‐secreting juxtaglomerlar tumor27	Unknown	No	High renins, secondary aldosteronism. Respond well to anti‐renin therapy. CT, MRI, renal sonography reveal intra‐renal tumor	Surgery, partial nephrectomy
Extrarenal renin secreting tumor	Very rare	No	May occur in renal cancers, germ cell tumors	Surgery to remove causative tumor

Abbreviations: CT, computed tomography; MRI, magnetic resonance imaging.

^aNormalization by monotherapy target to specific genetic phenotype.