Re: Reily - Renin level - to Karen
12/12/00 10:29 AM

Hi Karen,

I'm sorry but I can't really comment on the specific values you gave for Reily's 17-Hydroxyprogesterone (17-OHP) or her plasma renin since I don't know what the units of measure are. But since you did include the normal levels for the plasma renin test it is obvious that hers are high. Have you asked her doctor why she isn't on Florinef?  Even if she can overcome her salt wasting by consuming extra salt that does not stop her adrenal glands from constantly trying to make aldosterone anyway.  Anytime a CAH child has a craving for salty food it is a good bet that the adrenal glands are being stimulated to produce aldosterone and this is an undesirable situation because progesterone accumulates whenever the adrenal glands have to make aldosterone.  Whatever progesterone doesn't go into aldosterone synthesis is converted into that good old 17-OHP which in turn feeds into the androgen synthetic pathway.

You really shouldn't have to know this stuff, Reily's doctor should already be aware of it or at least be willing to learn. This link is a nice diagram that shows what I'm talking about;
http://matweb.hcuge.ch/matweb/endo/Reproductive_health/Steroid_hormone_metabolism_Fig2.html
If you think it would do any good, why not print it out (landscape mode) and show it to Reily's doctor. 

Here are the details pertinant to CAH in that diagram:
The blocks in both Cortisol and Aldosterone synthesis are at the right pointing arrows labeled 21OH (located in the middle of rows 1 and 2). Everything to the right of a 21OH arrow is blocked to some extent (depending on the severity of the CAH) and there is a buildup of whatever is to the immediate left of the 21OH arrow. Notice that whatever Progesterone (middle of the top row) can't get by the 21OH block is converted to 17-Hydroxyprogesterone (immediately
below Progesterone on the 2nd row) and the only place for all that excess 17-OHP to go is into making androgens.

I don't know if salt wasting had anything to do with Reily's three emergencies but even if it didn't I still think she would benefit from Florinef.

I hope this helps,
Joan

JoanW
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