Okay, but doesn't that sound like the selection was arbitrary? Indeed, in the case of "Partial Androgen Insensitivity Syndrome" the label is "Male" whereas for "Complete Androgen Insensitivity Syndrome" the label is female.
PAIS is probably one of the most controversial ones.
CAIS is SRY positive, but the receptors don't work. So during fetal development, the signal to do down the wolffian pathway is *sent*, but it's never *received*. In the absence of that reception, the reproductive anatomy develops to be phenotypically female. CAIS results in the fetus developing a uterus, fallopian tubes, cervix, and vagina, with the urethral opening separated from the vaginal opening. They do NOT develop a penis, vas deferens, prostate, or scrotal sac. All of the elements of a female reproductive system are present, but the tissue in their gonads is testicular and sterile.
PAIS, on the other hand, ends up with the signal being *sent* and *partially received*. Most PAIS will end up with a definitive male reproductive phenotype, although it's often not fully developed, and sometimes they end up with vestigial bits left over from the early fetal stage.
It can be controversial because depending on how garbled the signal, you end up with more or less development of the male phenotype. But they will end up with at least SOME male development.
I'm not going to get on board any train here, but the idea that someone slides from male to female depending upon hormonal insensitivity does suggest in this case that there is something "spectrum-y" going on here.
It's the insensitivity during early fetal development that is critical - as that's what prompts the mullerian or the wolffian pathway. It's not spectrumy, because there's no "in between" pathway.
In addition, I see the words "Mosaic" on two of the labels. I don't know much about this, and am willing to know more, but it seems that theoretically this could result in someone being able to produce both large and small gametes (even if they are unlikely to be functional). That again suggests some form of genuine intersex condition, no?
So when we get to mosaics and chimeras, things get weird - and I don't understand the distinction between them well enough to explain it. One has to do with the genes being mixed up within the same egg, and I think the other has to do with two separated eggs merging and the genetic material ending up mixed after that. But it's very confusing to me.
It's hypothetically possible for someone with whichever comes from the merging to end up with one ovary and one testis... but the likelihood of both of those being functional is vanishingly small. IIRC, there's been one single documented case of a person with ovotesticular disorder having been autopsied and finding that there was some suggesting that they may have ovulated at some point during their life - but they lacked a uterus or fallopian tubes or anything else that is related to a female reproductive phenotype, and the individual had fathered children with their active sperm - which definitively makes them male.
At this point, however, we're way into the realm of hypotheticals... like, it's hypothetically possible for conjoined twins to share a brain and have two predominantly separate and independent bodies. Sure, it could maybe happen given extraordinarily unlikely circumstances, but I'm not taking bets on it, and I'm definitely not going to support policies that define humans as having a spectrum of torsos because of that vanishingly small possibility.
In addition, that one labelled "Turner syndrome". Could someone walk me through why that person is female?
Turner is female because... there is no SRY gene present at all. There's not even a second X chromosome! Turner females develop entirely female reproductive systems.
As a general note... there are many conditions that fall under the grouping of Disorders of Sexual Development. That grouping really just means "something went wonky in the development of your reproductive system". But the overwhelming majority of people with DSDs are unambiguously male or female - every bit of their reproductive anatomy phenotypical for their sex. Within humans, something like 0.2% have a DSD... and within those with DSDs, it's like 0.1% that have anatomies that are ambiguous enough to even need more than a cursory glance at birth. 5-ARD is one of those, ovotesticular disorder is another, and I forget which others. The reality is that less than 0.002% of humans need any kind of testing to determine if they're male or female. Most DSDs end up presenting at puberty or later. Some get caught at puberty, because the person's body doesn't develop as expected - females don't menstruate or develop breasts, males don't see elongation of the penis or begin ejaculating. In a lot of cases, however, it doesn't get caught until they're entirely adult and find that they're sterile.
My godchild has a condition that doesn't usually get talked about in this context, but is considered a DSD. They have Kallman syndrome. Their karyotype and phenotype are completely normal for a female - but their pituitary gland is malformed. The pituitary plays a critical role in puberty and in hormone production post-puberty. In their case, their pituitary just completely failed to send any signal to start hormone production. Their adrenal worked just fine, so their long bones grew rapidly, and they grew fine leg, armpit, and pubic hair... but that was it. Their endocrinologist put them on standard issue birth control pills, and that provides enough hormones to allow for reproductive maturation as well as bone density accretion... but they're unlikely to ever have children. Interestingly - their condition was discovered because they had congenital anosmia - complete inability to smell anything. And it turns out that during early fetal development, the lump of goo that turns into the pituitary makes a pit stop on its way to the brain to drop off nasal bulbs - and theirs skipped that stop. It's one of the only indicators for Kallman that can show up prior to puberty.
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