Jamieson A, Ingran MC, Fraser R, White PC, Connell JMC
Impaired 11-beta-hydroxylase activity in glucocorticoid- suppressible hyperaldosteronism
AHA Council for High Blood Pressure Research
Hypertension (Sep) 26:540 (abst) 1995

Glucocorticoid suppressible hyperaldosteronism is due to expression of a chimeric aldosterone synthase in the z. fasciculata which is (abnormally) stimulated by ACTH. Unexplained high 11- deoxycorticosterone (DOC) levels in this syntrome are unexplained, but might be due to impaired 11 beta hydroxylation. 11 beta hydroxylation (of DOC to corticosterone and of 11- deoxycortisol [S] to cortisol) was assessed in pts with GSH, Conn's syndrome, and in normotensive controls before and after administration of ACTH (to stimulate aldosterone synthase in the GSH patients). 11 beta hydroxylation of both DOC and S was impaired in GSH. Similar impairment of S hydroxylation in GSH and Conn's patients suggests inhibition by 18-OH steroids while selective improvement in the ratio of DOC to corticosterone by ACTH in GSH but not Conn's patients suggests a mechanism which depends on the ectopic aldosterone synthase. (J. Umans)

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AHA Council for High Blood Pressure Research
H: Special problems : Endocrine hypertension