Intersex by AHMED ABDULWAHAB

Intersex
AHMED ABDULWAHAB
Intersex is a group of conditions where there
is a discrepancy between the external
genitals and the internal genitals (the testes
and ovaries).
 46 XX is the female genotype
 46 XY is the male genotype
 We need an X chromosome for human being to develop
 No X chromosome no life
 Y chromosome is to make a testis
 In a male fetus testis produce testosterone and Mullerian inhibitory factor MIF .
 Testosterone promote wolffian system development
 Dihydrotestosterone causes masculinization of the cloaca
 MIF suppresses the Mullerian system
 In female XX chromosome
 No Y chromosome no testis no testosterone no MIF
 Mularrian system will develop in the absence of the testis
 Making the fallopian tube, uterus, cervix, and upper two third of the vagina.
 Ovaries have nothing to do internal sexual organs
Causes
Intersex can be divided into four categories:

• 46, XX Intersex
• 46, XY Intersex
• True Gonadal Intersex
• Complex or Undetermined Intersex
46, XX Intersex
The person has the chromosomes of a woman, the ovaries of a woman,

but external genitals that appear male. This usually is the result of a
female fetus having been exposed to excess male hormones before birth.
The labia get fuse, and the clitoris enlarges to appear like a penis. Usually
this person has a normal uterus and Fallopian tubes. This condition is also
called 46, XX with virilization. It used to be called female
pseudohermaphroditism.
46, XX Intersex
There are several possible causes:

• Congenital adrenal hyperplasia (the most common cause).
• 21 hydroxylase deficiency.
• Male hormones (such as testosterone) taken by the mother during pregnancy.
• Male hormone-producing tumors in the mother.
• These are most often ovarian tumors.
• Mothers who have children with 46, XX intersex should be checked unless there is another clear
cause.
• Aromatase deficiency,. this one may not be noticeable until puberty.
• Aromatase is an enzyme that normally converts androgen to female hormones estrogen. Too
much aromatase activity can lead to excess estrogen.
46, XY Intersex
The person has the chromosomes of a man, but the external genitals are incompletely
formed, ambiguous, or clearly female. Internally, testes may be normal, malformed, or
absent. Formation of normal male external genitals depend on the adequate production and
function of male hormones dihydrotestosterone. Formation of normal male external genitals
depends on the appropriate balance between male and female hormones. Therefore, it
requires the adequate production and function of male hormones.
46, XY Intersex
46, XY intersex has many possible causes:

•Problems with the testes: The testes normally produce testosterone and if it failed it will
lead to under virilization. There are a number of possible causes for this, including XY
pure gonadal dysgenesis.
•Problems with testosterone formation:
•Testosterone is formed through a series of steps, where each requires a different enzyme.
Deficiencies in any of these enzymes can result in inadequate testosterone and produce a
different syndrome of 46, XY intersex. Different types of congenital adrenal hyperplasia
can fall in this category.
46, XY Intersex
• Some people have normal testes and make adequate amounts of testosterone, but still
have 46, XY intersex because of .5-alpha-reductase deficiency.
• People with 5-alpha-reductase deficiency lack the enzyme needed to convert
testosterone to dihydrotestosterone (DHT). That is essential for male external
genitalia, some have normal female genitalia, and many have something in between.
Most change to external male genitalia around the time of puberty.
• AIS is the most common cause of 46, XY intersex. It has also been called testicular
feminization. Here, the hormones are all normal, but the receptors to male hormones
don't function properly. There are over 150 different defects that have been identified
so far, and each causes a different type of AIS.
True Gonadal Intersex
Here the person must have both ovarian and testicular tissue. This may be in the same
gonad (an ovotestis), or the person might have one ovary and one testis. The person may
have XX chromosomes, XY chromosomes, or both. The external genitals may be
ambiguous or may appear to be female or male. This condition used to be called true
hermaphroditism. In most people with true gonadal intersex, the underlying cause is
unknown, although in some animal studies it has been linked to exposure to common
agricultural pesticides.
Complex or Undetermined Intersex
Many chromosome configurations other than simple 46, XX or 46, XY can result in
disorders of sex development. These include 45, XO (only one X chromosome), and 47,
XXY, 47, XXX -- both cases have an extra sex chromosome, either an X or a Y. These
disorders do not result in a condition where there is discrepancy between internal and
external genitalia. However, there may be problems with sex hormone levels, overall
sexual development, and altered numbers of sex chromosomes.
Symptoms
The symptoms associated with intersex will depend on the underlying cause, but may include:
•Ambiguous genitalia at birth
•Micropenis
•Clitoromegaly (an enlarged clitoris)
•Partial labial fusion
•Apparently undescended testes Labial or inguinal (groin) masses -- which may turn out to be
testes -- in girls
•Hypospadias [the opening of the penis is somewhere other than at the tip; in females, the
urethra (urine canal) opens into the vagina]
•Otherwise unusual-appearing genitalia at birth
•Electrolyte abnormalities
•Delayed or absent puberty
•Unexpected changes at puberty
Exams and Tests
•
Chromosome analysis
•Hormone levels
•Electrolyte tests
•Laparoscopy
•Ultrasound or MRI to evaluate whether internal sex organs are
present
Treatment
Ideally, a team of health care professionals with expertise in intersex should work together
to understand and treat the child with intersex -- and to understand, counsel, and support
the entire family.
Many experts now urge delaying definitive surgery for as long as healthy, and
ideally involving the child in the gender decision.
Clearly, intersex is a complex issue, and its treatment has short- and long-term
consequences.
The best answer will depend on many factors, including the specific cause of the intersex.
It is best to take the time to understand the issues before rushing into a decision. An intersex
support group may help acquaint families with the latest research, and may provide a
community of other families, children, and adult individuals who have faced the same
issues.

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Intersex

Intersex can be divided into four categories:

The person has the chromosomes of a woman, the ovaries of a woman,

There are several possible causes:

46, XY intersex has many possible causes:

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