Messages

Date: Tue, 20 Feb 2001 09:17:32 -0600 From: St.Aubyn, Ronald A > How then does someone with XXY/XX get the 'XX'? I'm not a geneticist, so I don't know the answer to that question. I do know that in true mosaics, the initial split of the egg contains the proper number of sex chromosomes and the extra Xs and/or Ys are added later due to some dysjunctional meiosis. From what I have read, any XX cells with XXY cells, the XX was the initial cell division. The Y is sometimes on the arm of one of the Xs, and materializes in the cells sometime down the road. Any Y chromosome in a female phenotype will affect the genitalia internally and externally by the 12th week gestation and will affect the growth and development of the infant/child/adolescent/teenager/adult. Ron St. Aubyn Louisiana State University Health Sciences Center School of Medicine & Allied Health Sciences Shreveport, Louisiana

Subject: Growth and development

Date: Tue, 13 Mar 2001 09:47:45 -0600

From: "St.Aubyn, Ronald "

We know that the brain is affected due to learning difficulties and problems dealing with stress. We already know what it does to the testes and skeletal development. There are also problems with the cardiovascular system and immune system. A problem that we haven't seen in Klinefelter syndrome individuals is overall growth retardation.

I want to emphasize that the pituitary gland is probably one of the most important of the endocrine organs that regulate growth and development. The pituitary gland may be considered a unisex type of gland, because the hormones that are secreted are targeted to stimulate another organ. Such as LH and FSH.

In females, the target organs are the ovaries, in males the testes. LH and FSH are the same hormone for both male and female. It is the target organ that is different, but it does the same. The thyroid gland is probably second in importance to the pituitary gland is regulating growth and development. Thyroid hormone promotes body growth and development, enhances skeletal growth and development, promotes sexual maturation, influences mental development, increases metabolic rate and is associated with skin texture, including hair growth.

The gonads are apparently activated during puberty, as reflected by increased secretion of androgens, estrogens and their metabolites. Insulin from the pancreas complements the action of growth hormone, promoting the accumulation of protein within the cell. In addition, insulin enhances the building of protein from amino acids in the absence of glucose. The adrenal glands are important to growth and development. The adrenal cortex produces hormones that are important in the control of water and electrolyte balance (aldosterone - which acts on the kidneys), and carbohydrate-protein balance (corticoids - all body systems are effected).

They also produce androgens and estrogens. The androgens have a marked influence on skeletal maturation and various aspects of secondary sexual development.

Hormones do not work in isolation, I cannot stress this enough.

Ron St. Aubyn

Subject:
Re: [XXY+ADULTS] Questions for Ron St. Aubyn re LH, the adrenals, and estrogen
Date:
Thu, 24 May 2001 13:12:55 -0500
From:
"St.Aubyn, Ronald A

> estrogen
>
> These questions will probably be best answered by the incomparable Ron
> St. Aubyn, but I believe they may be of general interest to the group.
> Some of these questions may just be restatements or versions of the same
> question, and if this is true, feel free to combine them.
>
> Ron, if you would care to take a stab at any of these, I and, I'm sure,
> the community as a whole, would be very grateful:
>
> 1. Klinefelter males tend to have high estradiol levels. Are these
> levels higher, on average, than other hypogonadal men? Or is this
> known?
>
1. I don't really know about this. Some articles claim that extra X
males have higher estradiol levels due to the aromatase enzyme. Not all
docs get estrogen levels routinely on XXY males. They rely on the physical
appearances such as skin pigmentation and texture, fat distribution, hair
distribution, quantity and quality. However, there are XYs who produce
higher levels of estrogen, that may be genetic or hereditary.

> 2. Since Klinefelter males don't, as a rule, have ovaries, where does
> our estrogen come from? Is it the same as in other men?
>
2. See answer to # 3. Estradiol is converted by P450 aromatase from
testosterone. The adrenal glands and the testes will both produce estrogens
through a series of enzyme mediated actions.

> 3. If the adrenal glands are also responsible for making testosterone
> and estrogen, in men as well as women, do the cells in the adrenals that
> make testosterone suffer the same damage from Klinefelter syndrome as do
> the Sertoli cells in the testes?
>
3. First of all, Sertoli cells are responsible in part for
spermatogenesis, under the influence of FSH. Leydig cells in the testes
produce the 17 carbon testosterone under the influence of LH. In the
adrenal cortex, by means of a series of enzyme-mediated changes, cholesterol
is modified to produce the 21 carbon steroids classified as progestogens,
glucocorticoids and mineralocorticoids and the 19 carbon androgens and the
18 carbon estrogens. Of the androgens produced by the adrenals,
dehydro-3-epiandrosterone (DHEA) is produced in abundance, and is a weak
androgenic steroid. Conversion to testosterone occurs primarily in the
testes and also in the presence of ACTH or adrenocorticotropic hormone.

There are no Leydig cells in the adrenal glands. Testosterone is
not produced in the adrenal gland as a primary hormone. It is converted to
testosterone by series of specific enzymatic actions beginning with
cholesterol. You have to understand that the adrenal glands are not gender
specific organs such as the testes or the ovaries. The adrenal glands alone
cannot sustain the secondary sexual characteristics in either the male or
the female. They work in conjunction with the gonads, and produce both
androgens and estrogens in the male and the female to mature and sustain
sexual function and reproduction.

Estrogens are not produced by mistake in XY or XXY men. Nor are
androgens produced by mistake in women. Both hormones are essential for
linear growth and skeletal and sexual maturation. If you are looking for
specific short answers, there are none. Each organ has a specific set of
enzymes used to modify or convert a particular substance. They all work
together and not separately.

> 4. Hypogonadal men, as a class, not just Klinefelter males, seem to
> have general problems with a distorted ratio of estrogen to
> testosterone. Why would this be?
>
4. Don't know. Could be that the testosterone being low, is more in
line with the estrogen. Giving a distorted ratio for males.

Ron