http://www.medhelp.org/www/ais/21_OVERVIEW.HTM
or part of it is here....
The usual pattern of human foetal development results in a 3-part alignment, as follows
Either:
1 (sex chromosomes = XY) ---> 2 (gonads = testes) ---> 3 (external genitalia = male)
or:
1 (sex chromosomes = XX) ---> 2 (gonads = ovaries) ---> 3 (external genitalia = female)
Very rare...
... is a type of intersex condition in which the person actually has a male/female mix at the genetic (sex chromosome) level and at the gonadal level. This is extremely rare and very few members of our group are in this situation. The old term for this situation is a hermaphrodite. Note however that a hermaphrodite, in the sense understood by most of society, is a purely mythical creature from ancient literature, one that supposedly has a complete working set of both male and female internal and external organs (such that the individual can, in theory, impregnate itself). This is not humanly possible. Unfortunately medicine took over this literary term in the days before genetics was understood and employed it as a medical term, to refer to these individuals who have both ovarian and testicular tissue internally (an ovo-testis) and who, as a result, can have ambiguous external genitalia.
Not quite so rare...
... is the type of intersex condition in which the sex chromosomes are either XY or XX (i.e. not a mixture) and the gonads are either testes or ovaries (i.e. not a mixture) - as in the majority of the population - but there is a mismatch or distortion in the usual 3-part alignment of these two elements with the external genitalia.
This means that you can, for example, have an XY individual with testes but with an external appearance that is essentially female (i.e. an XY female: either completely female as in Complete AIS, or partly female as in Partial AIS) or an XX individual with ovaries but with some degree of male genital appearance (e.g. a woman with congenital adrenal hyperplasia CAH).
Note that the term 'intersex' refers to the elements of this entire alignment (the sex chromosomes, the gonads and the genitalia), and not just to the appearance of the external genitalia. A patient with the complete form of AIS (CAIS), or with Swyers Syndrome (XY gonadal dysgenesis), will always appear female externally (no ambiguity) but she is still intersexed, because she has XY chromosomes and internal testes (testicular streak gonads in the case of Swyers) that are at odds with her external femaleness.
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'Intersex' vs 'Ambiguous Genitalia'
It is important to emphasise that the term 'ambiguous genitalia' refers to one specific component of the intersex state, i.e. the form taken by the external genitalia. Sometimes even specialist clinicians use the term 'intersex' wrongly, to mean 'ambiguous genitalia', forgetting that in doing so they exclude the large proportion of intersexed patients who have a completely female phenotype (body form) and, usually, no gender or gender identity conflicts.
The problems this causes...
This misuse of terminology causes us a lot of problems because the media (magazines, newspapers, TV) pick up on this and assume that 'intersex = ambiguous genitalia = gender identity problem', then print/broadcast material that conveys to the general public the idea that gender identity is, of necessity, an issue in intersex... which it absolutely is not.
People may suffer various problems on finding out their diagnosis (especially if it has been previously hidden from them by clinicians and/or parents) - problems such as confusion, anger and shame, an existential type of identity crisis, low self-esteem, difficulty grasping how this biological phenomenon can come about, grief at being denied fertility and rites of passage (e.g. lack of menstruation), a feeling of freakishness and isolation compared to their peers, a fear that others might see them as 'male', a concern regarding their ability to function in a relationship, the burden of keeping a secret, a retreat from medical care leading to failure to take HRT with a risk of osteoporosis, etc., etc. These are the issues that are of major concern to most of our members, and none of these issues necessarily mean that their inner sense of being a man or a woman is compromised.
For example, we have CAIS women members who've told their story for magazine articles without even mentioning gender identity but the editor will, without consulting her (or us, if we have been involved), slip in a last minute headline that includes emotive terms like 'hermaphrodite', or perhaps a picture (e.g. the left half of a man's photo joined to the right half of a woman's photo) with a caption saying something like "<Name> doesn't know if they're a girl or a boy".... just because they have this fixed idea that if you're intersexed you must have a gender identity problem (and because this notion, of course, sells more magazines/newpapers).
Why is CAH classified as intersex then?
