Steroid characteristics of mineralocorticoid adrenocortical hypertension.
Adrenocortical causes of hypertension are established by examining the mineralocorticoid hormones produced in the zona glomerulosa and zona fasciculata. In the zona glomerulosa, aldosterone excess leads to hypertension, hypokalemia, and suppressed plasma renin activity, with increased concentrations of urinary aldosterone (either as the 18-glucuronide or free aldosterone) as an index of its production. Identifying a tumor by computed tomography scan verifies the diagnosis of a correctable lesion. If no tumor is found, several maneuvers are used to identify primary adrenal hyperplasia, a disorder with autonomous aldosterone production, for which reduction of adrenal mass is curative. The zona fasciculata has two major pathways: the 17-deoxy pathway, where deoxycorticosterone (DOC) and corticosterone are the significant steroids, and the 17-hydroxy pathway, which leads to cortisol production. Tumors of the 17-deoxy pathway, DOC-producing adenomas, have increased concentrations of DOC and its precursor steroids, normal concentrations of cortisol, and suppression of aldosterone production secondary to suppression of the renin system. Two enzymatic defects in the zona fasciculata, 11 beta- and 17 alpha-hydroxylase deficiency, can be first readily identified by the virilization in the former, hypogonadal features in the latter. Steroid patterns are diagnostic. DOC is produced in excess in both deficiencies and is the cause of the hypertension. Deficient or impaired 11 beta-hydroxy steroid dehydrogenase in the apparent mineralocorticoid excess syndrome or after licorice ingestion retards the conversion of cortisol to inactive cortisone in the kidney, leading to mineralocorticoid hypertension; this leads to suppression of the renin system and subsequently of aldosterone.
Biglieri
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EG
San Francisco General Hospital, Clinical Study Center, CA 94110.
Kater
C E
CE
D000302:Adrenal Cortex / Q000378:metabolism*; D000303:Adrenal Cortex Diseases / Q000175:diagnosis / Q000378:metabolism*; D003937:Diagnosis, Differential; D006801:Humans; D006929:Hyperaldosteronism / Q000378:metabolism / Q000652:urine; D006973:Hypertension / Q000209:etiology / Q000378:metabolism*; D008901:Mineralocorticoids / Q000378:metabolism*; D012083:Renin / Q000097:blood
D008901:Mineralocorticoids; D012083:Renin