[Mineralocorticoid-induced hypertension].
Several important advances have been made in the pathogenesis of mineralocorticoid induced hypertension. A hybrid gene was found to be responsible for glucocorticoid remediable hypertension. This extra gene contains fragments of 11-beta-hydroxylase and aldosterone synthase. The hybrid gene is the result of an unequal crossing-over of the two genes located in close proximity on chromosome 8, and leads to the production of aldosterone and the hybrid steroids 18-hydroxycortisol and 18-oxocortisol. These hybrid steroids are also detected in patients with aldosterone producing adenoma but not in patients with hyperaldosteronism due to bilateral adrenal hyperplasia. In Apparent "Mineralocorticoid Excess", inherited as an autosomal recessive disorder, an increased ratio of urinary cortisol metabolite to cortisone is diagnostic. The syndrome is due to a deficiency of the renal enzyme 11-beta-hydroxysteroid dehydrogenase type II, which protects the mineralocorticoid receptor against cortisol that binds to the mineralocorticoid receptor like aldosterone. Liddle's syndrome is a rare entity and due to a constitute activation of an aldosterone dependent protein which triggers the amiloride sensitive sodium channel in the kidney. This results in hypokalemic hypertension with suppressed aldosterone and renin levels.
Hensen
J
J
Medizinische Klinik I, Friedrich-Alexander-Universität Erlangen-Nürnberg.
Oelkers
W
W
D015067:17-Hydroxysteroid Dehydrogenases / Q000172:deficiency; D000236:Adenoma / Q000378:metabolism; D000450:Aldosterone / Q000378:metabolism; D002898:Chromosomes, Human, Pair 8; D003434:Crossing Over, Genetic; D019405:Cytochrome P-450 CYP11B2 / Q000235:genetics; D003937:Diagnosis, Differential; D006801:Humans; D006929:Hyperaldosteronism / Q000378:metabolism; D006973:Hypertension / Q000139:chemically induced* / Q000175:diagnosis / Q000235:genetics / Q000628:therapy; D008901:Mineralocorticoids / Q000009:adverse effects*; D013577:Syndrome
D008901:Mineralocorticoids; D000450:Aldosterone; D015067:17-Hydroxysteroid Dehydrogenases; D019405:Cytochrome P-450 CYP11B2
Mineralokortikoidhochdruck.