21OH hydroxylase deficiency question

[troopersmama]

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hi everyone

i was wondering if someone could explain to me the need to treat with insulin sensitizer in adult-onset 21OH hyroxylase deficiency.

Thank you!

 

[gschl1234]

hi everyone

i was wondering if someone could explain to me the need to treat with insulin sensitizer in adult-onset 21OH hyroxylase deficiency.

Thank you!

This is a really great question! I couldn't find an exact answer to this but I have an idea. I took a look at the biochemical pathway. It looks like 21-OH-ase deficiency causes the aldosterone and cortisol pathways to get backed up. With regards to cortisol pathway, it means thht at ACTH will be very high (since there is now loss of negative feedback from cortisol). This means that the pathway will actually be constantly stimulated. So then you get excess intermediates from the cortisol pathway. If I recall correctly, these intermediates are weak glucocorticoids; however, since you have a ton of it (due to too much ACTH for which you've lost negative feedback), you now have excess glucocorticoids, which cause decreased insulin sensitivity, increased gluconeogensis, etc....Don't know if this is the right answer but it's my best guess.