17 hydroxylase deficiency

[ENThopeful]

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quick question: to clarify, in 17 hydroxylase deficiency, there is no internal male genitalia, because testosterone is required for wolffian duct development, which is lacking. MIF is still present from the sertoli cells, hence the

im reading different sources that say it "may" be present which serves to confuse me even more.

 

[NCF145]

quick question: to clarify, in 17 hydroxylase deficiency, there is no internal male genitalia, because testosterone is required for wolffian duct development, which is lacking. MIF is still present from the sertoli cells, hence the

im reading different sources that say it "may" be present which serves to confuse me even more.

They will have gonads, but will lack other male sexual structures like, prostate, seminal vesicles, etc.

 

[Sislo]

I read this from the biochem book goljan was talking about in his lectire and it was very helpful...

#Out of the three ( 11, 17 and 21) if you see salt wasting or hypotension, stop thinking: it 21 alpha hydroxylase, the other two are associated with hypertension

#11 or 21 hydroxylase : male with precocious puberty and female with ambigous genitalia( they will tell you the sex by the result of karyotyping, xx or xy)

# 17 hydroxylase: Male with female genitalia and female with hypogonadism( undevelopped sec sexual characteristics).Once again how will you know if male or female? karyotype xx or xy

Hope this help.I was missing all my questions on adrenal until I found that out in rapid review biochem and copied it on my board

 

[ENThopeful]

They will have gonads, but will lack other male sexual structures like, prostate, seminal vesicles, etc.

I think I figured it out. If I am completely off base, someone please let me know!

Testis - seminferous tubules, rete testis, etc - are primary sex cords, come from the primordial germ cells of the XY genotype from the wall of the yolk sac.

Accessory testicular structures - wolffian ducts, seminal vesical, epididymis, ejaculatory ducts - require testosterone to develop, which is produced by leydig cells induced by SRY gene/testis determining factor

External structures - prostate, penis, etc. - require DHT to develop, 5 alpha reductase at the respective tissues

Paramesonephric ducts - under the influence of MIF by sertoli induced by SRY gene/testis determining factor, involute, explaining why 5 alpha reductase/17 hydroxylase deficiency XY do not have mullerian structures

 

[cpants]

I read this from the biochem book goljan was talking about in his lectire and it was very helpful...

#Out of the three ( 11, 17 and 21) if you see salt wasting or hypotension, stop thinking: it 21 alpha hydroxylase, the other two are associated with hypertension

#11 or 21 hydroxylase : male with precocious puberty and female with ambigous genitalia( they will tell you the sex by the result of karyotyping, xx or xy)

# 17 hydroxylase: Male with female genitalia and female with hypogonadism( undevelopped sec sexual characteristics).Once again how will you know if male or female? karyotype xx or xy

Hope this help.I was missing all my questions on adrenal until I found that out in rapid review biochem and copied it on my board

Barone (Kaplan) taught us the best mnemonic. Write 11, 17, and 21 in a column. You use the 1's. On top of the ones on the left side draw an up arrowhead for hypertension. On top of the ones on the right side put a mushroom cap (get it?). Now the numbers look like what they do.

 

[ENThopeful]

Barone (Kaplan) taught us the best mnemonic. Write 11, 17, and 21 in a column. You use the 1's. On top of the ones on the left side draw an up arrowhead for hypertension. On top of the ones on the right side put a mushroom cap (get it?). Now the numbers look like what they do.

👍

 

[FutureDoc4]

alright, to much studying or im just so tired... but i dont get the column thing? ...stupid finals

 

[cpants]

alright, to much studying or im just so tired... but i dont get the column thing? ...stupid finals

11
17
21

^--Column, as in stacked on top of each other.

Left sided 1's get up arrow, right sided 1's get mushroom cap.

 

[docmo83]

what does the mushroom cap imply?

 

[docmo83]

like masculinization?

 

[cpants]

what does the mushroom cap imply?

It turns the 1 into a fallus to symbolize ambiguous genitalia. I didn't think this was that complex.

 

[Brucelaa67]

Females, XX, in 17 hydroxylase, show lack of sec sexual characters, as they can't produce estrogen to the enzyme deficiency. But how does external female genitalia develop in the first place, why not ambugus gentilai for 46, XX too?

 

[TheOther]

Females, XX, in 17 hydroxylase, show lack of sec sexual characters, as they can't produce estrogen to the enzyme deficiency. But how does external female genitalia develop in the first place, why not ambugus gentilai for 46, XX too?

Estrogen is also produced peripherally.

 

[Brucelaa67]

There are two kind of explanations here. One states that female external genitalia develops ''by default'', when no androgens are produced. @nd is as you state above, the role of peripheral or placental aromatse. But in that case, the maternal androgens can cross the placenta too. So, whats their role in sex differentiation?

Estrogen is also produced peripherally.

 

[TheOther]

There are two kind of explanations here. One states that female external genitalia develops ''by default'', when no androgens are produced. @nd is as you state above, the role of peripheral or placental aromatse. But in that case, the maternal androgens can cross the placenta too. So, whats their role in sex differentiation?

Androgens can cross, but overall DHT levels are insufficient to influence the "default" external female genitalia to differentiate into male counterparts.