Mineralocorticoids in congenital adrenal hyperplasia.
While hypertension is observed in only two of the three major subtypes of congenital adrenal hyperplasia (CAH), 11 beta- and 17 alpha-hydroxylase deficiencies, deoxycorticosterone (DOC) production is increased in all. The elevated zona fasciculata (ZF) DOC produces mineralocorticoid hypertension with suppressed renin and reduced potassium concentrations. The DOC levels in 21-hydroxylase deficiency are in part produced by renin stimulation of the Zona glomerulosa (ZG) along with aldosterone. Assessment of the mineralocorticoid hormones of the ZF and ZF (17-deoxy steroids) provides additional unique characteristics of each subtype. Dissociation of DOC from cortisol is not unique to CAH. This dissociation is seen in other disorders and contrived conditions. There is a strong suggestion of a non-ACTH regulator of 17-deoxy steroids (DOC) that may contribute significantly to DOC production in general and effect DOC levels in CAH.
Biglieri
E G
EG
San Francisco General Hospital Medical Center, CA 94110.
Kater
C E
CE
D000312:Adrenal Hyperplasia, Congenital / Q000503:physiopathology*; D006801:Humans; D006973:Hypertension / Q000209:etiology*; D008901:Mineralocorticoids / Q000502:physiology*; D015383:Zona Fasciculata / Q000378:metabolism; D015384:Zona Glomerulosa / Q000378:metabolism
D008901:Mineralocorticoids