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So I am confused two of the three Congential Adrenal Hyperplasias: 11B-hydroxylase deficiency and 17a-hydroxylase deficiency.
- Why do patients with 11B-hydroxylase deficiency have supranormal mineralocorticoid activity?
The books I've looked at site the increase in 11-Deoxycorticosterone (DOC) as the reason, while other sources say that DOC just has minor mineralocorticoid activity. If we don't have 11B-hydroxylase, we can't make corticosteron, which means that we can't make Aldosterone. If we are making little to no aldosterone (the major mineralocorticoid), how does an increase in a minor mineralocorticoid more than make up for it?
- Why do patients with 17a-hydroxylase deficiency have supranormal mineralocorticoid activity but decreased glucocorticoid activity?
If increased DOC is enough to make up for a lack of aldosterone, why isn't the increase corticosterone in this syndrome enough to make up for the lack of cortisol. Costanzo's physio text, Guyton, and Kaplan's lecture notes all mention Corticosterone as a weak glucocorticoid just as DOC is a weak mineralocorticoid. At one point in Cotanzo's text, she even mention that Corticosterone can substitute for cortisol when cortisol is deficient. But even though these patients make an excess of corticosterone, they still have reduced glucocorticoid activity!
So, is Costanzo wrong? Why do these sources bother mentioning the weak glucocorticoid activity of corticosterone if it can't make up for lost cortisol activity (as DOC can make up for lost Aldosterone activity)?
Last edited by CBG23; 03-18-2011 at 10:39 PM.
So I am confused two of the three Congential Adrenal Hyperplasias: 11B-hydroxylase deficiency and 17a-hydroxylase deficiency.
- Why do patients with 11B-hydroxylase deficiency have supranormal mineralocorticoid activity?
The books I've looked at site the increase in 11-Deoxycorticosterone (DOC) as the reason, while other sources say that DOC just has minor mineralocorticoid activity. If we don't have 11B-hydroxylase, we can't make corticosteron, which means that we can't make Aldosterone. If we are making little to no aldosterone (the major mineralocorticoid), how does an increase in a minor mineralocorticoid more than make up for it?
- Why do patients with 17a-hydroxylase deficiency have supranormal mineralocorticoid activity but decreased glucocorticoid activity?
If increased DOC is enough to make up for a lack of aldosterone, why isn't the increase corticosterone in this syndrome enough to make up for the lack of cortisol. Costanzo's physio text, Guyton, and Kaplan's lecture notes all mention Corticosterone as a weak glucocorticoid just as DOC is a weak mineralocorticoid. At one point in Cotanzo's text, she even mention that Corticosterone can substitute for cortisol when cortisol is deficient. But even though these patients make an excess of corticosterone, they still have reduced glucocorticoid activity!
So, is Costanzo wrong? Why do these sources bother mentioning the weak glucocorticoid activity of corticosterone if it can't make up for lost cortisol activity (as DOC can make up for lost Aldosterone activity)?
Last edited by CBG23; 03-18-2011 at 10:39 PM.