re: re: To Chris D.
Aug. 12th, 2002   11:24pm

With 17 Hydroxylase deficiency, the metabolic processes would get as far as making aldosterone no problem.  However, he would still make high levels of androgen if not having glucocorticoid replacement possibly.  If you look at the diagram below, you will see that the processes to make cortisol occur after  17 Hydroxylase and therefore 17 Hydroxylase deficient kids will not make it or will make it in a reduced amount.  It would all depend on the severity or block again like 21 hydrolase deficiency.  So whilst Jack does not need to take florinef, he needs cortisol replacement.  I think possibly when a salt waster, that  is where some kids seem to need extra cortisol for some reason.  I’ve noted a great deal that the salt wasters are seemingly on higher doses than the non-salt wasters.

Enzymatic pathways for Cortisol and Aldosterone:

 Desmolase 17-alpha-Hydroxylase C-17-Lyase 
Cholesterol--------------------->Pregnenolone----------------------->17-oh-Pregnenolone------------------->Dehydroepiandrosterone
  | | |
 3-beta-oh-steroid
dehydrogenase
|3-beta-oh-steroid
dehydrogenase
|3-beta-oh-steroid
dehydrogenase
|
  Progesterone 17-oh-Progesterone Androstenedione
 21-Hydoxylase|21-Hydroxylase|Peripheral tissues|
  Deoxycorticosterone 11-Deoxycortisol Testosterone
 11-beta-Hydroxylase|11-beta-Hydoxylase|  
  Corticosterone Cortisol  
 18-Hydroxylase|    
  18-oh-Corticosterone    
 18-Oxidase|    
  Aldosterone    
Anne-Marie
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