That’s ok Chris :)
Aug. 13th, 2002   4:26pm

With 17 Hydroxylase deficiency things would be a totally different kettle of fish where control was concerned.  For example:

With 21 Hydroxylase Deficiency, they measure the enzyme that precedes this in the metabolic pathway because that is the pre-cursor for all androgens being made by the adrenal glands.  If this is outside of the normal ranges (10-50nmol/L) then we can know that there is a possibility that everything else after it in that process is to.  

However, in a child or individual with 17 Hydroxylase deficiency measuring the 17 Hydroxylase would in effect be useless as the levels will always be lower than they otherwise would be anyway due to the deficiency.  Just like say measuring someone with 21 Hydroxylase deficiency.   However, all the cholesterol would be shunted down the pathway after it just the same as in 21 Hydroxylase.  The difference like I said would be that they can make corticosterone so they would to some degree beable to make some glucocorticoid.   If it needs for a child to exceed 50nmol/L where 17 OHP is concerned or if they were being controlled using the normal ranges for a 21 Hydroxylase deficient child where 17 OHP was concerned this would be nothing short of disastrous really.  That’s because if their levels had to reach 50nmol?L before anyone paid attention and measured androgen thereafter, those would be that much more elevated really than a child’s that has 21 Hydroxylase.  I think therefore you would have to measure another enzyme that preceded 17 Hydroxylase or hormone and keep that within the normal ranges by gauging that against the androstenedione and testosterone levels and the clinical assessments until you establish a good normal range of the other pre cursor. 

In a sense it is therefoe just as well that Jack is not 17 Hydroxylase deficiency as it would most certainly be a rough few months then trying to re establish all those things and what have you. 

I don’t mind about the misinterpretation because I learned something in the process. ;)

Take care,  

Cheers

Anne-Marie

Anne-Marie
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