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dhddmods · 10 months ago

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Intersex Guide!

(UPDATED: April 4th, 2025. Please reblog again if you only saw our old version. Please also reblog if you've never seen this before, to spread awareness.)

Hello and happy pride! We wished to share a passion project we have been working on for months - a guide to intersex traits and variations!

Now, a question that many ask - what is intersex? Well, we will be answering that question for you here! Anything on this post that is written in red is NOT intersex, so if you wish to skip over any of it, you can. And if you wish to get straight into the intersex types, scroll down to the read-more and start from there.

Intersex, also known as the intersex spectrum, is a term used to describe when someone's biological sex - as in the sex they are born with/what they naturally develop during puberty - is not clearly defined as the typical male or female sex traits.

(This does not include someone that was born male or female, and later chose to have their sex traits changed due to being transgender, transsex, or altersex. It also does not include males that experienced circumcision/dorsal slits or penis splitting, females that experienced genital mutilation, or males & females that indulged in modifications such as piercings and beading.)

This only applies to primary sex traits - chromosomes, genitals, reproductive organs, hormones, and hormone responsiveness. Atypical secondary sex traits (breasts, muscle tone, body/facial hair, deepness of voice) do not make someone intersex unless it is paired with "abnormalities" in primary sex traits.

Before you can understand what it means to be intersex, first we must clarify what it means to not be intersex.

A typical male has XY chromosomes, a penis, two testicles within the scrotum, a prostate, and more androgens (mostly testosterone) than females. Upon puberty, they usually (but not always) develop more facial hair & muscle tone than females, and a deeper voice than females.

(Note: A penis has a phallus, a scrotum beneath the phallus, foreskin protecting the head of the phallus, and a urethra on the head of the penis. It is straight or slightly curved when erect.)

A typical female has XX chromosomes, a vulva, two ovaries, a single uterus, and more estrogen than males. Upon puberty, they usually (but not always) develop larger breasts and wider hips than males.

(Note: A vulva has two labia, a single pea-sized clitoris, a single vaginal entry, and a urethra above the vaginal entry and under the clitoris.)

Here is a list of non-typical sex traits that, by themselves, are not intersex.

Accessory Breasts (Polymastia): Having more than two breasts. Accessory Nipples (Polythelia): Having more than two nipples. Athelia: Having only one nipple, or no nipples at all. Amastia: Having only one breast & nipple, or no breasts & nipples at all. Breast Hypertrophy/Macromastia/Gigantomastia: Having extremely large breasts Gynecomostia: Breasts on a male. The reason this is not considered intersex is because all sexes (except for people with amastia) have breast tissue, which can vary in size regardless of sex. Females can have small breasts, and males can have larger breasts than is expected. Hypotonia: Low muscle tone. Bicornuate Uterus: A heart-shaped uterus. Septate Uterus: A uterus that internally has a partition down the middle. Macropenis: A penis that is 7 inches/17.78 centimeters or larger. Macroorchidism: Testicles that are 4 milliliters or above pre-puberty, and above 30 milliliters as an adult. Macrovagina: A vagina that is deeper than 5 inches/13 centimeters. Labial Hypertrophy: Labia that is longer than average (above 2 inches/5 centimeters)

Now, onto the intersex spectrum! First, some notes.

-An intersex trait is a singular atypical trait. For example, someone with ambiguous genitals, but no other "abnormality" has an intersex trait. -An intersex variation is when multiple atypical traits are present, with at least one of them being an intersex trait. For example, someone with ambiguous genitals and fused kidneys has an intersex variation. Equally, someone with ambiguous genitals and cryptorchidism also has an intersex variation. -CTF stands for "close to female." CTF traits are characteristics that are closely associated with females (vulvas, uteruses, ovaries, estrogen as the main sex hormone, breasts, widened hips, XX chromosomes, etc.) CTF people are intersex people who call their body as a whole CTF, due to a majority (but not necessarily all) of their sex traits being CTF (ie; a person with breasts, a uterus, ovaries, and a penis might call themself CTF, even though not all of their traits are CTF in nature.) -CTM stands for "close to male." CTM traits are characteristics that are closely associated with males (a penis, testicles, androgens as the main sex hormones, increased hair growth, higher muscle mass, a deepened voice, XY chromosomes, etc.) CTM people are intersex people who call their body as a whole CTM, due to a majority (but not necessarily all) of their sex traits being CTM. -CTA stands for "close to androgynous." CTA traits are characteristics that are predominantly "androgynous", or an equal mix of "feminine" and "masculine" (ie; ambiguous genitals, ovotestes, XXYY chromosomes, etc.) CTA people are intersex people who call their body as a whole CTA, due to a majority (but not necessarily all) of their sex traits being CTA, or they have a near-equal mix of CTF and CTM sex traits. -CTN stands for "close to neutral." CTN traits are characteristics that are predominantly "neutral" (ie; small/absent/blocked off genitalia or reproductive organs, a lack of hormone production, 0X chromosomes, etc.) CTN people are intersex people who call themself CTN, due to a majority (but not necessarily all) of their sex traits being CTN.

Also, when we state that an intersex trait/variation is "fairly common", we mean that it is fairly common amongst the intersex population, not that it is fairly common in the general population. Being intersex is still classified as "rare" statistically speaking (as statistics define "rare" as 1 in 1,000 people.)

So for the sake of this post, here is how we are classifying the following:

"Fairly common" = 1 in every 5,000 (or less)

"Rare" = above 1 in every 5,000, up to 1 in every 100,000

"Extremely rare" = above 1 in every 100,000

Keep in mind that "may co-occur" means that not all of the features will be present on every single person with that variation; in fact, none of the extra features could be present. However, for chromosomal variations specifically, it is highly likely that at least 1-5 (or more) of the listed extra features will be present.

And finally, when we say that "fertility is average", what we mean is that the gonads are fully capable of producing healthy average numbers of sperm/eggs, and/or the uterus is capable of carrying healthy babies. Struggles with the sperm reaching the eggs still might occur, but if direct insemination is done (as in the sperm is directly injected), then pregnancy should occur perfectly fine.

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Penile Traits/Variations (not including those on the agenital spectrum)

These are traits that affect the development of the penis.

Congenital Chordee: An trait in which an individual is born with a curved penis. This curve may be on its own, or it may be curved due to webbed-skin on the scrotum connecting to the penis and holding it in a curved manner. As a lone variation, those with congenital chordee have XY chromosomes, testicles, and a prostate. They may also have hypospadias. During puberty (without the influence of HRT), they will have testosterone as the main sex hormone, and will likely have increased body/facial hair, higher muscle mass, and a deepened voice. They can produce semen, but it may not be able to exit the urethra depending on its placement. Fertility is average. This trait is extremely common, occurring in 1 of every 200 AMAB births.

Penoscrotal Transposition (PST): A trait in which an individual is born with a scrotum that is in front of the penis, rather than beneath it. A Shawl Scrotum/Donut Scrotum is a form of PST where the scrotum completely surrounds the penis like a shawl. As a lone variation, those with PST have XY chromosomes, testicles (possibly cryptorchidism, in which case the scrotum surrounding the penis is full of empty tissue), and a prostate. They may also have hypospadias. During puberty (without the influence of HRT), they will have testosterone as the main sex hormone, and will likely have increased body/facial hair, higher muscle mass, and a deepened voice. They can produce semen, but it may not be able to exit the urethra efficiently, depending on the urethra's placement. This trait is extremely rare. Due to the rarity, little is known about the fertility status, however it appears to be average. Common traits and disabilities that may co-occur include absent kneecaps, ureter abnormalities, an absent or underdeveloped kidney, heart disease, overgrown/protruding ribs, widely-spaced nipples, an incurved pinkie finger, a small lower jaw, a chin dimple, folded eyelids, a large/protruding back of the skull, cerebral atrophy, and intellectual disability.

Diphallia/Bifid Penis: A trait in which an individual has two penises (either next to each other or one on top the other.) These penises could be of the same size, or have a size difference. A urethra may be present on only one penis (possibly with epispadias or hypospadias), both penises, or they may have a single urethra that is in-between both the two penises. Erections may be possible in both penises or only in one. As a lone trait or variation, those with diphallia have XY chromosomes, testicles (possibly with cryptorchidism), and a prostate. During puberty (without the influence of HRT), they will have testosterone as the main sex hormone, and will likely have increased body/facial hair, higher muscle mass, and a deepened voice. They can produce semen, but it may not be able to exit the urethra efficiently, depending on the urethra's placement. This trait is extremely rare. Due to the rarity, little is known about the fertility status, however it appears to be average. Common traits and disabilities that may co-occur include pubic bone abnormalities, an extra hole (not an anus) that leads into the rectum, an absent anus (which will need surgical intervention in order to pass waste), two colons, an extra ureter, fused kidneys, an extra kidney, a rotated kidney, an inguinal hernia, gastrointestinal abnormalities, an extra gastrointestinal tract, exposed organs on the lower abdomen, heart abnormalities, spinal abnormalities, and an absent thumb.

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Urethral Traits/Variations (not including those on the agenital spectrum)

These are traits that affect the development of the urethra.

Urethral Duplication/Double Urethra: A trait in which an individual with a singular set of genitals has two urethras. One of the urethras may not fully reach between the bladder and genitals, either cutting off before reaching the bladder (making it visible from the outside, but without a use), cutting off before reaching the genitals (making it connected to the bladder, but not visible or accessible from the outside), or cutting off in the middle (making it connected to the bladder, and visible from the outside, but a blockage existing somewhere in the middle internally.) As a lone variation, this comes in both an XX form and an XY form. Those with the XX form have XX chromosomes, a vulva (usually with epispadias for one or both of the urethras, however the urethras could be side-by-side as well) or clitoromegaly & fused labia, two ovaries, and a uterus. During puberty (without the influence of HRT), they will have estrogen as the main sex hormone, and will likely develop breasts and widened hips. Those with the XY form have XY chromosomes, a penis (usually with hypospadias or epispadias for one of the urethras, though both can be side-by-side on the penis head), two testicles, and a prostate. During puberty (without the influence of HRT), they will have testosterone as the main sex hormone, and likely have increased body/facial hair, higher muscle mass, and a deepened voice. This trait is extremely rare. Due to the rarity, little is known about the fertility status of this variation, however it appears to be average (implying those with testes can produce sperm, and those with a uterus can get pregnant.) Common traits and disabilities that may co-occur include an extra ureter, an extra bladder, urinary tract infections, an extra rectum, an extra colon, an absent kidney, an abnormally-located kidney, kidney cysts, spinal abnormalities, and an opening between the windpipe and the esophagus.

Hypospadias: A trait in which an individual with a penis is born with a urethra that is located lower than typical. This could be lower on the head of the phallus than typical, or it could be on the shaft, the scrotum, or even underneath the phallus. As a lone variation, they have XY chromosomes, a penis/micropenis (possibly with chordee or PST), testicles, and a prostate. During puberty (without the influence of HRT), they will have testosterone as the main sex hormone, and will likely have increased body/facial hair, higher muscle mass, and a deepened voice. They can produce semen, but it may not be able to exit the urethra depending on its placement. Fertility is average. This trait is extremely common, occurring in 1 of every 250 AMAB births. Vulval Hypospadias/Hypospadias Feminis is an extremely rare form of hypospadias that occurs on vulvas, in which a urethral opens on the vaginal wall. As a lone variation, those with vulval hypospadias have XX chromosomes, two ovaries, and a uterus. During puberty (without the influence of HRT), they will have estrogen as the main sex hormone, and will likely develop breasts and widened hips. Due to the rarity, little is known about the fertility status of vulval hypospadias.

Epispadias: A trait in which an individual is born with a urethra that is located higher than typical. On a penis, this refers to a urethra that is located above the typical spot on the penis head. Ona vulva, the urethra is on the stomach or above/in the middle of the clitoris (thus splitting the clitoris into two.) As a lone variation, this comes in both an XX form and an XY form. Those with the XX form have XX chromosomes, a vulva, two ovaries, and a uterus. During puberty (without the influence of HRT), they will have estrogen as the main sex hormone, and will likely develop breasts and widened hips. Those with the XY form have XY chromosomes, a penis, two testicles, and a prostate. During puberty (without the influence of HRT), they will have testosterone as the main sex hormone, and likely have increased body/facial hair, higher muscle mass, and a deepened voice. Fertility is average (implying those with testes can produce sperm, and those with a uterus can get pregnant.) This trait is extremely rare. A common trait/disability that may co-occur is exposed organs on the lower abdomen.

Urogenital Sinus Anomaly (UGS)/Persistent Urogenital Sinus Anomaly (PUGS): A trait in which an individual has a single opening, which is a combination of both urethral and vaginal tissue, and connects to both the urinary tract and the reproductive tract. This opening could be the size of a typical vaginal entry, the size of a urethra, or somewhere in-between. As a lone variation, those with UGS have XX chromosomes, a vulva or clitoromegaly & fused labia, two ovaries/an ovary (unilateral ovarian agenesis) or ovarian agenesis, and a uterus (possibly with uterine hypoplasia) or MRKH syndrome. Puberty may be absent or delayed. If they start puberty naturally (and don't use HRT), they will have estrogen as the main sex hormone, and will likely develop breasts and widened hips. Pregnancy is possible if they have a uterus that is present and large enough. If they have no ovaries, an embryo implant will be necessary. Alternatively, if they have no uterus or a uterus that is too small for pregnancy, but have ovaries, their eggs can be used to implant an embryo into another carrier. This trait is rare.

Persistent Cloaca: A trait in which an individual has a single opening for urination, menstruation/sexual output, and feces (being a combination of the urethra, vaginal entry, and anus.) This opening could be the size of a typical vaginal entry, the size of a urethra, the size of an anus, or somewhere in-between. It could be placed anywhere from the crotch to between the buttocks, or somewhere in the middle. Partial cloacas may occur as well, in which the anus and the vagina are combined but the urethra is still separate, or the anus and the urethra are combined but the vagina is still separate. As a lone variation, those with persistent cloacas have XX chromosomes, a vulva or clitoromegaly & fused labia, two ovaries/one ovary (unilateral ovarian agenesis) or ovarian agenesis, and a uterus (possibly with uterine hypoplasia) or MRKH syndrome. If they have a uterus and a partial cloaca, they may have a double cervix, with one cervix in each opening. If they start puberty naturally (and don't use HRT), they will have estrogen as the main sex hormone, and will likely develop breasts and widened hips. Pregnancy is possible if they have a uterus that is present and large enough. If they have no ovaries, an embryo implant will be necessary. Alternatively, if they have no uterus or a uterus that is too small for pregnancy, but have ovaries, their eggs can be used to implant an embryo into another carrier. This trait is rare. Common traits and disabilities that may co-occur include a dilated colon, a dilated vaginal entry, urine buildup (which may cause a stretched/swollen kidneys or ureter), an absent kidney, an oddly-placed kidney, heart defects, a tethered spinal cord, and an underdeveloped esophagus.

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Ambiguous Genitals

These are traits in which a person was born with a combination of vulval and penile characteristics. Micropenises are often considered a part of this spectrum.

As a lone trait, this can come in a CTA, CTF, or CTM form.

The CTA form can have any mix of gonads, reproductive organs, hormone production, and puberty. Typically they either have XX or XY chromosomes, however occasionally they may have a chromosomal variation as well.

Those with the CTF version have XX chromosomes, two ovaries (or ovotestes), and a uterus. During puberty (without the influence of HRT), they will have estrogen as the main sex hormone, and will likely develop breasts and widened hips.

Those with the CTM version have XY chromosomes, testicles (or ovotestes) and a prostate. During puberty (without the influence of HRT), they will have testosterone as the main sex hormone, and likely have increased body/facial hair, higher muscle mass, and a deepened voice.

Ambiguous genitals are fairly common, occurring in 1 of every 5,000 births.

Bifid Scotum: A form of ambiguous genitalia where a penis is present, however the scrotum is separated in a labia-like fashion around the phallus. Fused Labia: A form of ambiguous genitalia, where a vulva is present, however the labia is fused in a scrotum-like fashion. The fused labia either covers the entirety of the vulva (leaving only the clitoris) exposed, or half of the vulva (leaving the clitoris & urethra exposed, but the vaginal opening mostly or fully hidden.) There may be tissue within the labia that appears like small testicles. Clitoromegaly: A form of ambiguous genitalia, where a vulva is present, however the clitoris is enlarged, being over 1 inch (2.54 centimeters) in diameter. In some cases, it looks like a small penis. Combined Clitoromegaly & Fused Labia: A form of ambiguous genitalia, where a vulva is present, however the labia fuses to resemble a scrotum and the clitoris is enlarged, creating a structure almost identical to a small penis. The vaginal opening is fully hidden under the labia. Pseudophallus: A form of ambiguous genitalia, where a vulva is present, however the clitoris is enlarged significantly (beyond the typical clitoromegaly expectations) and is merged with labia, creating a significantly-sized penis-like structure. Penis & Vulva Combo 1: A form of ambiguous genitalia where a vulva is present, however the labia stretches up and merges with a penis, becoming the outer skin & foreskin of the phallus. The penis is located where the clitoris usually is (thus replacing the clitoris.) No scrotum or descended testes are present. The urethra is on the vulva, below the penis. Penis & Vulva Combo 2: A form of ambiguous genitalia where there is a penis (with no scrotum or descended testicles) that has a vulva underneath it. The vulva has labia (which is not merged with the penis) and a vaginal opening. It may also have a clitoris beneath the penis as well. The urethra is on the vulva, below the penis. Penis & Vulva Combo 3: A form of ambiguous genitalia where there is a penis with a scrotum (possibly with descended testicles) that has labia (which is not merged with the penis) and a vaginal opening, but no clitoris. The urethra can either be on the tip of the penis or on vulva beneath it. Penis & Vagina Combo: A form of ambiguous genitalia where there is a penis with a scrotum (possibly with descended testicles) that a vaginal entry tucked beneath the scrotum.

It is important to note that the term Clitorophallus is often used as an umbrella term to describe micropenises, clitoromegaly, combined clitoromegaly & fused labia, and pseudophalluses.

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The Agenital Spectrum/Agenital/Agenitalia

These are traits in which a person was born with absent, small, or closed off genitals. Anorchia & Monoorchidism fall under this umbrella as well.

Urethral Agenesis: A trait or variation in which an individual is born without a urethra. It may be visibly absent, or it may appear to be present from the outside, with a blockage of skin being within the first few centimeters or inches internally. A urethra is necessary to be surgically added, as to avoid urine buildup. As a lone variation, this comes in both an XX form and an XY form. Those with the XX form have XX chromosomes, a vulva, two ovaries, and a uterus. During puberty (without the influence of HRT), they will have estrogen as the main sex hormone, and will likely develop breasts and widened hips. Those with the XY form have XY chromosomes, a penis, testicles, and a prostate. During puberty (without the influence of HRT), they will have testosterone as the main sex hormone, and likely have increased body/facial hair, higher muscle mass, and a deepened voice. This trait is extremely rare. Due to the rarity, little is known about the fertility status, however it appears to be average (implying those with testes can produce sperm, and those with a uterus can get pregnant.) Common traits and disabilities that may co-occur include limb deformities, an anus covered by skin (which will need surgical intervention in order to pass waste), urine buildup (which may cause a stretched/swollen kidneys or ureter), an abnormal connection between the bowel and bladder, an absent bladder (which will need surgical intervention in order for urine to have somewhere to be collected), a small kidney, kidney cysts, an underdeveloped lung, an enlarged chamber of the heart, and heart issues.

Urethral Hypoplasia: A trait in which an individual is born with a narrow or underdeveloped urethra. As a lone variation, this comes in both an XX form and an XY form. Those with the XX form have XX chromosomes, a vulva, two ovaries, and a uterus. During puberty (without the influence of HRT), they will have estrogen as the main sex hormone, and will likely develop breasts and widened hips. Those with the XY form have XY chromosomes, a penis, two testicles, and a prostate. During puberty (without the influence of HRT), they will have testosterone as the main sex hormone, and likely have increased body/facial hair, higher muscle mass, and a deepened voice. Fertility is average (implying those with testes can produce sperm, and those with a uterus can get pregnant.) This trait is rare.

Vaginal Hypoplasia: A trait in which an individual has a vaginal opening that is small/tight or not very deep. As a lone variation, those with vaginal hypoplasia have XX chromosomes, two ovaries, and a uterus (possibly with uterine hypoplasia) or MRKH syndrome. Puberty may be absent or delayed. If they start puberty naturally (and don't use HRT), they will have estrogen as the main sex hormone, and will likely develop breasts and widened hips. Pregnancy is possible if they have a uterus that is present and large enough. If they have no uterus or a uterus that is too small for pregnancy, their eggs can be used to implant an embryo into another carrier. This trait is fairly common, occurring in 1 of every 5,000 AFAB births.

Vaginal Agenesis/Vaginal Aplasia: A trait in which an individual has some or all of the external vulva (clitoris, labia, & urethra), but no vaginal opening. As a lone variation, those with vaginal agenesis have XX chromosomes, two ovaries, and a uterus (possibly with uterine hypoplasia) or MRKH syndrome. Puberty may be absent or delayed. If they start puberty naturally (and don't use HRT), they will have estrogen as the main sex hormone, and will likely develop breasts and widened hips. If menstruation occurs, birth control will be necessary in order to prevent internal buildup (as there is no exit for the uterine shedding), or surgery could be chosen (ideally by the intersex person themself, not forced upon them) to create a vaginal entry. Pregnancy is possible if they have a uterus that is present and large enough, however sperm will need to be medically injected (unless they have a vaginal entry created surgically.). If they have no uterus or a uterus that is too small for pregnancy, their eggs can be used to implant an embryo into another carrier. This trait is fairly common, occurring in 1 of every 4,000 AFAB births.

Imperforate Hymen: A trait in which an individual has a hymen that completely covers the vaginal opening. As a lone variation, those with imperforate hymens have XX chromosomes, a vulva, two ovaries, and a uterus. During puberty (without the influence of HRT), they will have estrogen as the main sex hormone, and will likely develop breasts and widened hips. If menstruation occurs, birth control will be necessary in order to prevent internal buildup (as there is no exit for the uterine shedding), or surgery could be chosen (ideally by the intersex person themself, not forced upon them) to remove the hymen. Pregnancy is possible, however embryos will need to be medically implanted (unless they have the hymen removed surgically.) This trait is fairly common, occurring in 1 of every 1,000 AFAB births.

Microperforate Hymen: A trait in which an individual has a hymen that fully covers the vaginal opening, with only one tiny hole in the hymen. Cribriform Hymen: A trait in which an individual has a hymen that fully covers the vaginal opening, with only multiple tiny holes scattered across the hymen. Septate Hymen: A trait in which an individual has a hymen that stretches across the middle of the vaginal opening, making it appear as though there are two (or more) vaginal entries (when in reality, every hole leads to the same vagina.) As lone variations, those with microperforate hymens, cribriform hymens, or septate hymens have XX chromosomes, a vulva, two ovaries, and a uterus. During puberty (without the influence of HRT), they will have estrogen as the main sex hormone, and will likely develop breasts and widened hips. Pregnancy is possible, and menstruation may occur without any complication, however if menstrual fluids fail to exit due to the holes being too small, birth control will be necessary in order to prevent internal buildup, or surgery could be chosen (ideally by the intersex person themself, not forced upon them) to remove the hymen. All three of these variations are fairly common, with each (separately) occurring in 1 of every 1,000 AFAB births.

Vaginal Septum: A trait in which an individual has a vaginal opening that is partially or fully blocked by skin tissue (which is not a hymen.) A Transverse Vaginal Septum (TVS) is when the tissue runs horizontally across or within the vaginal cavity, dividing or blocking it. Depending on how deep or wide this tissue is within the vagina, it may cause the vagina to seem short/small (due to blocking the vagina mid-way), may make the cervix seem absent (due to covering the cervix), could split the vagina into two entryways (creating a double vagina, which may be obvious externally or only noticeable internally), or could be covering the entrance to the vagina altogether. An Obstructed Hemivagina is when the tissue runs vertically across/within the vaginal cavity, and curves to block part (but not all) of the vaginal entrance, leaving one side open. This might cause the vagina to seem short/small, and might cover the cervix. It may be obvious externally or only noticeable internally. This trait almost always partners uterus didelphys, blocking menstrual output from one of the two uteruses. A Longitudinal Vaginal Septum (LVS) is when the tissue runs vertically across/within the vaginal cavity, splitting it into two entryways (creating a double vagina, which may be obvious externally or only noticeable internally.) As a lone variation, those with vaginal septums have XX chromosomes, a vulva, two ovaries, and a uterus. During puberty (without the influence of HRT), they will have estrogen as the main sex hormone, and will likely develop breasts and widened hips. Pregnancy is possible, and menstruation may occur without any complication, however if menstrual fluids fail to exit due to the holes being too small or absent, birth control will be necessary in order to prevent internal buildup, or surgery could be chosen (ideally by the intersex person themself, not forced upon them) to remove the tissue. This trait is rare.

Labial Hypoplasia: A trait in which an individual with a vulva is born without one or both labia. As a lone variation, those with labial hypoplasia have XX chromosomes, a vulva, two ovaries, and a uterus. During puberty (without the influence of HRT), they will have estrogen as the main sex hormone, and will likely develop breasts and widened hips. Fertility is average. This trait is rare.

Clitoral Hypoplasia: A trait in which an individual has a vulva with a small clitoris (under 0.75 inches or 1.90 centimeters.) As a lone variation, those with clitoral hypoplasia have XX chromosomes, a vulva, two ovaries, and a uterus. During puberty (without the influence of HRT), they will have estrogen as the main sex hormone, and will likely develop breasts and widened hips. Fertility is average. This trait is rare.

Penile Agenesis/Penile Aplasia: A trait in which an individual who is expected to have a penis is born without one. They either have urethral agenesis, a lone urethra, or a persistent cloaca. As a lone variation, those with penile agenesis have XY chromosomes, two testicles/one testicle (monorchidism) or anorchia, and a prostate. If they have testes, cryptorchidism is common. Puberty may be absent or delayed. If they start puberty naturally (and don't use HRT), they will have testosterone as the main sex hormone, and will likely have increased body/facial hair, higher muscle mass, and a deepened voice. If testes are present, they can produce semen, but it may not be able to exit the urethra/cloaca depending on its placement and how the testes connect. Fertility is average. This trait is extremely rare. Common traits and disabilities that may co-occur include twisted feet, an absent anus (which will need surgical intervention in order to pass waste), anal abnormalities, bladder abnormalities, small kidneys, kidney cysts, urine buildup (which may cause a stretched/swollen kidneys or ureter), a hole between the heart chambers, small lungs, a connection between the windpipe and esophagus, a flat nose, and rotated ears.

Congenital Buried Penis/Congenital Hidden Penis: A trait in which an individual is born with a penis that is mostly or entirely hidden within the skin of the abdomen, thigh, or scrotum. As a lone variation, those with congenital buried penises have XY chromosomes, a penis, two testicles, and a prostate. During puberty (without the influence of HRT), they will have testosterone as the main sex hormone, and likely have increased body/facial hair, higher muscle mass, and a deepened voice. Fertility is average. This trait is fairly common, with the exact estimates unknown.

Aposthia: A trait in which an individual is born with a penis that has very little or no foreskin. As a lone variation, those with aposthia have XY chromosomes, a penis, two testicles, and a prostate. During puberty (without the influence of HRT), they will have testosterone as the main sex hormone, and likely have increased body/facial hair, higher muscle mass, and a deepened voice. Fertility is average. This trait is extremely rare.

Microorchidism/Testicular Hypoplasia: A trait in which an individual is born with one or multiple testicles that are smaller than average. Microtestes are 14 milliliters or less in adults, 3 milliliters or less during puberty, and under 1 milliliter pre-puberty. As a lone variation, those with microorchidism XY chromosomes, two testicles (possibly with cryptorchidism), a penis/micropenis or ambiguous genitals, and a prostate. During puberty (without the influence of HRT), they will have testosterone as the main sex hormone, and likely have increased body/facial hair, higher muscle mass, and a deepened voice. They may have hypogonadism, so their testosterone levels might be lower than males. They may be capable of producing semen, but are at a higher risk of infertility. This trait is rare on its own, but fairly common when partnered with Klinefelter Syndrome.

Cryptorchidism: A trait in which an individual is born with one or multiple testicles that are undescended (within the body, rather than hanging in the genital area.) This trait only counts as intersex if the testicles do not descend within the first 3-6 months after birth. As a lone variation, those with cryptorchidism have XY chromosomes, a penis, two testicles, and a prostate. During puberty (without the influence of HRT), they will have testosterone as the main sex hormone, and likely have increased body/facial hair, higher muscle mass, and a deepened voice. They may be capable of producing semen, but are at a higher risk of infertility. This trait is extremely common, occurring in 1 of every 100 AMAB births, however it can also occur in AFAB and AXAB/UAB individuals too. A common trait/disability that may co-occur is testicular cancer.

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Gonadal Agenesis

These are traits that cause one or both gonads to be absent.

Common traits and disabilities that may co-occur with gonadal agenesis include an absent kidney, kidney swelling, and kidney stones.

Anorchia/Bilateral Congenital Anorchia/Testicular Agenesis: A trait in which an individual that is expected to have testicles instead has no gonads. As a lone variation, those with cryptorchidism have XY chromosomes, a penis/micropenis or ambiguous genitals (which may have a urogenital sinus anomaly or a hole between the urethra & vagina) or penile agenesis, and a prostate. They will not begin puberty unless they are on HRT. Due to the lack of gonads, they are infertile. This trait is rare.

Monorchidism/Monorchism/Unilateral Congenital Anorchia: A trait in which an individual is born with a single testicle. As a lone variation, those with monorchidism have XY chromosomes, a penis, possibly cryptorchidism, and a prostate. During puberty (without the influence of HRT), they will have testosterone as the main sex hormone (though hypoandrogenism is common, so their testosterone may be low), and likely have increased body/facial hair, higher muscle mass, and a deepened voice. They may be capable of producing semen, but are at a higher risk of infertility. This trait is fairly common, occurring in 1 of every 5,000 AMAB births.

Unilateral Ovarian Agenesis: A trait in which an individual is born with a single ovary. As a lone variation, those with unilateral ovarian agenesis have XX chromosomes, a vulva, and a uterus/unicornuate uterus or MRKH syndrome. During puberty (without the influence of HRT), they will have estrogen as the main sex hormone (though hypoestrogenism is common, so their estrogen may be low), and will likely develop breasts and widened hips. They are at a higher risk of infertility. If they are fertile enough, and have a uterus that is large enough, pregnancy is possible. If they are fertile enough, and have no uterus or a uterus that is too small for pregnancy, their eggs can be used to implant an embryo into another carrier.

Ovarian Agenesis/Bilateral Ovarian Agenesis: A trait in which an individual that is expected to have ovaries instead has no gonads. As a lone variation, those with ovarian agenesis have XX chromosomes, a vulva, and a uterus (possibly with uterine hypoplasia) or MRKH syndrome. They will not begin puberty unless they are on HRT. Due to the lack of gonads, they are infertile, however if they have a uterus (and it is large enough), they can carry a (genetically unrelated) embryo if it is medically implanted. This trait is rare.

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Other Reproductive Traits

Gondal Dysgenesis: A trait in which an individual is born with a gonad (ovary, testicle, or ovoteste) that is full of empty tissue, rather than reproductive tissue. These are known as "streak gonads." If a person has two streak gonads, they will not begin puberty unless they are on HRT, and will have no eggs or sperm. XX Gonadal Dysgenesis/Pure Gonadal Dysgenesis (PGD) is a form of gonadal dysgenesis, in which an individual with XX chromosomes is born with two streak ovaries. They have a vulva or clitoromegaly and a uterus. They will not begin puberty unless they are on HRT. Due to the lack of eggs, they are infertile, however they can carry a (genetically unrelated) embryo if it is medically implanted. Swyer Syndrome and Mixed Gonadal Dysgenesis fall under this umbrella, and Turner Syndrome often does as well. All forms of gonadal dysgenesis are rare, except for when it occurs in Turner Syndrome.

Ovarian Hypoplasia/Hypoplastic Ovaries: A trait in which an individual is born with one or multiple ovaries that are smaller than typical. Unilateral Ovarian Hypoplasia is when an individual is born with one ovary that is smaller than typical. Bilateral Ovarian Hypoplasia is when an individual is born with both ovaries being smaller than typical. As a lone variation, those with ovarian hypoplasia have XX chromosomes, a vulva, and a uterus (possibly with fallopian tube agenesis or uterine hypoplasia.) Their menstruation may be irregular or absent. Pregnancy is possible if they have a uterus that is large enough, however they are at a higher risk of infertility and early menopause. If they have eggs and a large enough uterus, they may become pregnant naturally. If they have no fallopian tubes, they may need medical intervention in order to access their eggs. If their uterus is too small, then their eggs can be used to implant an embryo into another carrier. This trait is rare on its own, but fairly common when partnered with Turner Syndrome.

Fallopian Tube Agenesis: A trait in which an individual with ovaries and a uterus is born without one or both fallopian tubes. Unilateral Fallopian Tube Agenesis is when an individual is born with only one fallopian tube. Bilateral Fallopian Tube Agenesis is when an individual is born with no fallopian tubes. As a lone variation, those with fallopian tube agenesis have XX chromosomes, a vulva, two ovaries, and a uterus. During puberty (without the influence of HRT), they will have estrogen as the main sex hormone, and will likely develop breasts and widened hips. Those with fallopian tube agenesis are capable of producing eggs, however only people with unilateral fallopian tube agenesis are capable of being impregnated (as they still have one fallopian tube to transport eggs) meanwhile people with bilateral fallopian tube agenesis are incapable of transporting eggs (as they have no fallopian tubes) and will need medical intervention to access their eggs. This trait is rare.

Vas Deferens Aplasia/Congenital Absence of the Vas Deferens (CAVD): A trait in which an individual with testes is born without one or both vas deferens. Unilateral Vas Deferens Aplasia/Congenital Unilateral Absence of Vas Deferens (CUAVD) is when an individual is born with only one vas deferens. Bilateral Vas Deferens Aplasia/Congenital Bilateral Absence of Vas Deferens (CBAVD) is when an individual is born with no vas deferens. As a lone variation, those with vas deferens aplasia have XY chromosomes, a penis, two testicles, and a prostate. During puberty (without the influence of HRT), they will have testosterone as the main sex hormone, and likely have increased body/facial hair, higher muscle mass, and a deepened voice. Those with vas deferens aplasia are capable of producing sperm, however only people with CUAVD are capable of releasing it (as they still have one vas deferens to deliver the sperm to the urethra), meanwhile people with CBAVD are incapable of releasing it (as they have no vas deferens) and will need medical intervention to access their sperm. This trait is fairly common, occurring in 1 of ever 1,000 AMAB births.

Seminal Vesicle Hypoplasia: A trait in which an individual has a seminal vesicle that is smaller than typical. Unilateral Seminal Vesicle Hypoplasia is when an individual is born with one seminal vesicle that is smaller than typical. Bilateral Seminal Vesicle Hypoplasia is when an individual is born with two seminal vesicles that are smaller than typical. Seminal Vesicle Agenesis: A trait in which an individual that is expected to have seminal vesicles has none, or only one. Unilateral Seminal Vesicle Agenesis is when an individual is born with only one seminal vesicle. Bilateral Seminal Vesicle Agenesis is when an individual is born with no seminal vesicles. As lone variations, those with seminal vesicle hypoplasia or seminal vesicle agenesis have XY chromosomes, a penis, two testicles (possibly with vas deferens aplasisa), and a prostate. During puberty (without the influence of HRT), they will have testosterone as the main sex hormone, and likely have increased body/facial hair, higher muscle mass, and a deepened voice. They may or may not be capable of producing semen. If they are incapable of producing semen (or have vas deferens aplasia), then they will need medical intervention to access their sperm. These traits are rare. Common traits and disabilities that may co-occur include kidney abnormalities and an absent kidney.

Prostate Hypoplasia: A trait in which an individual has a prostate that is smaller than typical. For adults, the mass of a hypoplastic prostate is 10 cubic centimeters or less. As a lone variation, those with prostate hypoplasia have XY chromosomes, a penis or micropenis, and testes (possibly with microorchidism or cryptorchidism). During puberty (without the influence of HRT), they will have testosterone as the main sex hormone, and likely have increased body/facial hair, higher muscle mass, and a deepened voice. They may have hypogonadism, so their testosterone levels might be lower than males. They may be capable of producing semen, but are at a higher risk of infertility. The rarity of this variation is unknown. Common traits and disabilities that may co-occur include ureter abnormalities, kidney abnormalities, kidney cysts, and prune belly syndrome (a condition in which the abdominal muscles above the stomach are underdeveloped or absent.)

Prostate Agenesis: A trait in which an individual that is expected to have a prostate instead has none. As a lone variation, those with prostate hypoplasia have XY chromosomes, a penis or micropenis, and testes (possibly with microorchidism or cryptorchidism). During puberty (without the influence of HRT), they will have testosterone as the main sex hormone, and likely have increased body/facial hair, higher muscle mass, and a deepened voice. They may have hypogonadism, so their testosterone levels might be lower than males. Their testes typically produce sperm, however the sperm is incapable of leaving the body due to the lack of prostate, and thus medical intervention to extract the sperm would be necessary in order to create a biological offspring. The rarity of this variation is unknown. Common traits and disabilities that may co-occur include ureter abnormalities, kidney abnormalities, kidney cysts, and prune belly syndrome (a condition in which the abdominal muscles above the stomach are underdeveloped or absent.)

WNT4 Deficiency: A variation in which an individual with XX chromosomes has a mutation in the WNT4 gene, which causes them to be born with an absent uterus, a vulva with vaginal agenesis or vaginal hypoplasia, and two ovaries. During puberty (without the influence of HRT), they will have hyperandrogenism, leading them to have testosterone as their main sex hormone, and will likely develop clitoromegaly, increased facial/body hair, higher muscle mass, and breasts. Due to the lack of uterus, they are unable to get pregnant, however their eggs can be used to implant an embryo into another carrier. This variation is rare.

Mayer–Rokitansky–Küster–Hauser Syndrome (MRKH Syndrome)/Müllerian Agenesis/Müllerian Aplasia: A trait in which an individual that is expected to have a uterus is born with a completely or partially absent uterus. As a lone variation, those with MRKH syndrome have XX chromosomes, a vulva (possibly with vaginal agenesis or vaginal hypoplasia) or a urogenital sinus anomaly, and two ovaries (which may be on an atypical spot of the fallopian tubes.) During puberty (without the influence of HRT), they will have estrogen as the main sex hormone, and will likely develop breasts and widened hips. Due to the lack of uterus, they are unable to get pregnant, however their eggs can be used to implant an embryo into another carrier. This trait is fairly common, occurring 1 in every 4,500 AFAB births. Common traits and disabilities that may co-occur include a shorter height than average, abnormally located kidneys, an absent or underdeveloped kidney, heart complications, rib abnormalities, spinal abnormalities (possibly scoliosis), a short neck, a low hairline on the back of the neck, and hearing disabilities/deafness.

Unicornuate Uterus: A trait in which an individual is born with a uterus that has one side fully developed, while the other side of the uterus is underdeveloped (known as a Hemi-Uterus) or absent. If a hemi-uterus is present, it may or may not be functional. If it is functional, menstrual fluids will be released from it. The hemi-uterus might have a large enough opening that allows the menstrual fluids to leak into the main uterus - however, if the hemi-uterus does not have a wide enough opening, the menstrual fluids may get trapped and buildup could occur, or menstruation could take longer to end. As a lone variation, those with a unicornuate uterus will have XX chromosomes, a vulva, and two ovaries/an ovary (unilateral ovarian agenesis). The ovaries may be on an atypical spot of the fallopian tube(s). During puberty (without the influence of HRT), they will have estrogen as the main sex hormone, and will likely develop breasts and widened hips. Pregnancy is possible, however they are at a higher risk of premature birth, miscarriage, and ectopic pregnancy. This trait is fairly common, occurring in 1 of every 4,000 AFAB births. Common traits and disabilities that may co-occur include kidney abnormalities and kidney cysts.

Uterine Hypoplasia/Hypoplastic Uterus: A trait in which an individual is born with a uterus that is smaller than typical. For adults, the length (from top to bottom) of a hypoplastic uterus is 2 inches/5 centimeters or smaller. As a lone variation, those with uterine hypoplasia have XX chromosomes, a vulva (possibly with vaginal agenesis or vaginal hypoplasia), and two ovaries/an ovary (unilateral ovarian agenesis) or ovarian agenesis. Puberty may be absent or delayed. If they start puberty naturally (and don't use HRT), they will have estrogen as the main sex hormone, and will likely develop breasts and widened hips. They may have hypogonadism, so their estrogen levels might be lower than females. If the uterus is smaller than 1.60 inches/4 centimeters, pregnancy is not possible, however their eggs (if they have an ovary) can be used to implant an embryo into another carrier. If the uterus is 1.60 inches/4 centimeters or larger, pregnancy is possible. however they are at a higher risk of miscarriage and premature birth, and if they have no ovaries, an embryo implant may be the only way to become pregnant. This trait is rare.

Cervical Agenesis/Cervical Dysgenesis/Cervical Atresia: A trait in which an individual with a uterus is born without a cervix, leaving the uterus closed off from the genitalia. As a lone variation, those with cervical agenesis have XX chromosomes, a vulva, two ovaries, and a uterus. During puberty (without the influence of HRT), they will have estrogen as the main sex hormone, and will likely develop breasts and widened hips. If menstruation occurs, birth control will be necessary in order to prevent internal buildup (as there is no exit for the uterine shedding), or surgery could be chosen (ideally by the intersex person themself, not forced upon them) to create a cervix. Pregnancy is possible, however embryos will need to be medically implanted (unless they have a cervix created surgically.) This trait is extremely rare.

Cervical Hypoplasia: A trait in which an individual with a uterus is born with a cervix that is smaller than typical. As a lone variation, those with cervical agenesis have XX chromosomes, a vulva, two ovaries, and a uterus. During puberty (without the influence of HRT), they will have estrogen as the main sex hormone, and will likely develop breasts and widened hips. Pregnancy is possible, and menstruation may occur without any complication, however if menstrual fluids fail to exit due to the cervix being too small, birth control will be necessary in order to prevent internal buildup, or surgery could be chosen (ideally by the intersex person themself, not forced upon them) to expand the cervix. This trait is extremely rare.

Cervical Duplication/Double Cervix: A trait in which an individual with a uterus is born with 2 cervixes. As a lone variation, those with cervical duplication have XX chromosomes, a vulva (possibly with an obstructed hemivagina or longitudinal vaginal septum), two ovaries, and a uterus or uterus didelphys. During puberty (without the influence of HRT), they will have estrogen as the main sex hormone, and will likely develop breasts and widened hips. Fertility is average. This trait is rare on its own, but fairly common when partnered with uterus didelphys.

Uterus Didelphys: A variation in which an individual is born with two uteruses. As a lone variation, those with uterus didelphys have XX chromosomes, possibly cervical duplication, a vulva (possibly with an obstructed hemivagina or longitudinal vaginal septum), and two ovaries (typically one fallopian tube & ovary for each uterus). During puberty (without the influence of HRT), they will have estrogen as the main sex hormone, and will likely develop breasts and widened hips. Fertility is average, however there is a higher risk of miscarriage and premature birth. Oftentimes each uterus can be impregnated separately. This trait is fairly common, occurring in 1 of every 3,000 AFAB births. Common traits and disabilities that may co-occur include severe menstrual cramps, heavy bleeding, extra menstruation, sexual dysfunction, and an absent kidney.

Fallopian Tube Duplication/Accessory Fallopian Tube: A variation in which an individual is born with three (or more) fallopian tubes. The extra tubes may or may not be attached to the uterus itself. As a lone variation, those with fallopian tube duplication have XX chromosomes, a vulva, two ovaries, and a uterus. During puberty (without the influence of HRT), they will have estrogen as the main sex hormone, and will likely develop breasts and widened hips. Pregnancy is possible, however there may be difficulty with eggs reaching the uterus, due to going through one of the extra fallopian tubes, which may not have a connection with the uterus. This gives a higher risk of ectopic pregnancy and miscarriage. This trait is fairly common, with the exact estimates unknown. A common trait/disability that may co-occur is endometriosis.

Vas Deferens Duplication/Accessory Vas Deferens: A variation in which an individual is born with three (or more) vas deferens. The extra vas deferens may or may not be attached to the testes or prostate. As a lone variation, those with vas deferens aplasia have XY chromosomes, a penis, testes, and a prostate. During puberty (without the influence of HRT), they will have testosterone as the main sex hormone, and likely have increased body/facial hair, higher muscle mass, and a deepened voice. Fertility is average. This trait is extremely rare. Common traits and disabilities that may co-occur include inguinal hernias, an absent kidney, and cystic fibrosis.

Polyorchidism: A trait in which an individual is born with three (or more) testicles. These testicles could be descended, or they could have cryptorchidism. As a lone variation, those with polyorchidism have XY chromosomes, a penis, testes, and a prostate. During puberty (without the influence of HRT), they will have testosterone as the main sex hormone, and likely have increased body/facial hair, higher muscle mass, and a deepened voice. Fertility is average. This trait is extremely rare.

Accessory Ovary: A trait in which a person has three (or more) ovaries, with the extra ovaries being connected to the other ovaries, the fallopian tubes, or the uterus. Supernummary Ovary: A trait in which a person has three (or more) ovaries (and possibly extra fallopian tubes), with the extra ovaries (& fallopian tubes) being detached from the reproductive system, located away from the other ovaries. If they are latched onto/pressed against a different organ (ie; the intestines), they may cause abdominal pain. As lone variations, those with accessory ovaries or supernummary ovaries have XX chromosomes, a vulva, and a uterus (possibly a bicornuate one) or unicornuate uterus. During puberty (without the influence of HRT), they will have estrogen as the main sex hormone, and will likely develop breasts and widened hips. This trait is extremely rare. Due to the rarity, little is known about the fertility status, however it appears to be average. Common traits and disabilities that may co-occur include extra adrenal glands, an absent kidney, an absent ureter, an extra ureter, and pouches in the bladder.

Ovotesticular Syndrome/Bigonadal/Ambigonadal: A variation in which an individual is born with a combination of ovaries and testicles. This could be ovaries and testes as separate gonads, or it could be a unique gonads (Ovotestes) that has both testicular and ovarian tissue within it. If they have a testicle or ovotestes structured in a similar manner, it is common to have cryptorchidism. People with ovotestes have a uterus 90% of the time (possibly with uterine hypoplasia), and they may or may not have a prostate. They may have fallopian tubes, vas deferens, or both. As a lone trait, this comes in both an XX form and an XY form. Those with the XX form have XX chromosomes and a vulva or ambiguous genitals. During puberty (without the influence of HRT), depending on the function of their gonads, they could have any combination of secondary sex traits (widened hips, breasts, increased body/facial hair, higher muscle mass, a deepened voice, etc), and they could have estrogen or androgen as a main sex hormone, or a combination of both. Those with the XY form have XY chromosomes, a penis (possibly with chordee or hypospadias) or ambiguous genitals. During puberty (without the influence of HRT), depending on the function of their gonads, they could have any combination of secondary sex traits (widened hips, breasts, increased body/facial hair, higher muscle mass, a deepened voice, etc), and they could have estrogen or androgen as a main sex hormone, or a combination of both. People with both the XX and XY forms are at a higher risk of infertility. However, for those who are fertile, most commonly, the semen is infertile, while the eggs are fertile. However in rarer cases it is the other way around, with the eggs being infertile and the sperm being fertile. This trait is rare. Common traits and disabilities that may co-occur include germ cell tumors and inguinal hernias.

Persistent Müllerian Duct Syndrome (PMDS): A variation in which an individual with XY chromosomes is born with a uterus (possibly a hypoplastic uterus or an incomplete one) and possibly fallopian tubes. They usually have a prostate. In some cases, they may have an ovary or ovoteste, however most often they have testicles. If they have a testicle or ovotestes structured in a similar manner, it is common to have cryptorchidism. They will have a penis, micropenis, or ambiguous genitals. During puberty (without the influence of HRT), they will have testosterone as the main sex hormone, and likely have increased body/facial hair, higher muscle mass, and a deepened voice. They may have hypogonadism, so their testosterone levels might be lower than males. They may or may not be fertile, depending on the presence (or lack thereof) of a prostate, the function of their gonads, and the size of their uterus. If they have a prostate and functional testes, they are capable of producing sperm. If they have a large enough uterus, they are capable of pregnancy (however it may need to be medically implanted, depending on whether or not their genitals have a vaginal entry.) If the uterus is large enough to menstruate, the shedding will either exit through the genitals (if there is a vaginal entry or the urethra is connected to the uterus), or it will have nowhere to go. If it has nowhere to go, birth control will be necessary in order to prevent internal buildup (as there is no exit for the uterine shedding), or surgery could be chosen (ideally by the intersex person themself, not forced upon them) to create a vaginal entry or to remove the uterus. This variation is extremely rare.

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Hypergonadism

These are traits that cause a person's gonads to produce a higher level of hormones than in males and females.

Hyperestrogenism: A trait in which an individual has high levels of estrogen. If they have ovaries, this describes having higher levels of estrogen than females do. If they have testicles, this describes having higher levels of estrogen than males do. As a lone variation, this comes in both an XX form and an XY form. Those with the XX form have XX chromosomes, a vulva (possibly with labial hypertrophy), two ovaries, and a uterus. During puberty (without the influence of HRT), they will have estrogen as the main sex hormone (though it will be high), and will likely develop breasts (possibly with breast hypertrophy) and widened hips. Their menstruation could be heavy or irregular. They may also have hypoandrogenism, meaning their testosterone levels might be lower than females. Pregnancy is possible, however they are at a higher risk of infertility. Those with the XY form have XY chromosomes, a penis or micropenis, and two testicles (possibly with microorchidism). During puberty (without the influence of HRT), they will either have estrogen and testosterone at near-equal levels, or have estrogen as the main sex hormone, and will likely develop breasts and widened hips, with the possibility of increased body/facial hair, higher muscle mass, and a deepened voice. If estrogen is their main sex hormone, they may also have hypoandrogenism, meaning their testosterone levels might be lower than males. They may be capable of producing semen, but are at a higher risk of infertility. This trait is rare. Common traits and disabilities that may co-occur include an early puberty (which may lead to a shorter height than average), cold hands/cold feet, weight gain, swollen or tender breasts, bumpy breasts, a higher risk of breast cancer, a low libido, thinning hair, chronic migraines, memory difficulty, sleep disorders, and mood disorders. Aromatase Excess Syndrome (AEXS/AES)/Familial Hyperestrogenism is an extremely rare form of hyperestrogenism, caused by genetic mutations in the CYP19A1 gene, which makes the body convert more androgens into estrogen than is typical.

Hyperandrogenism: A trait in which an individual has high levels of androgens. If they have ovaries, this describes having higher levels of androgens than females do. If they have testicles, this describes having higher levels of androgens than males do. As a lone variation, this comes in both an XX form and an XY form. In 80% of cases the XX form is caused by Polycystic Ovarian Syndrome (PCOS), a genetic condition that causes the ovaries to overproduce hormones, which may cause cysts to develop on the ovaries. Those with the XX form have XX chromosomes, a vulva or clitoromegaly, two ovaries, and a uterus. During puberty (without the influence of HRT), they will either have estrogen and androgens at near-equal levels, or have androgens as the main sex hormones, and will likely develop increased body/facial hair, higher muscle mass, and a deepened voice, with the possibility of breasts and widened hips. If androgens are their main sex hormones, they may also have hypoestrogenism, meaning their estrogen levels might be lower than females. Pregnancy is possible, however they are at a higher risk of infertility. Those with the XY form have XY chromosomes, a penis (possibly a macropenis), two testicles (possible with macroorchidism), and a prostate. During puberty (without the influence of HRT), they will have testosterone as their main sex hormone (though it will be high), and will likely develop increased body/facial hair, higher muscle mass, and a deepened voice. They may have hypoestrogenism, meaning their estrogen levels might be lower than males. They may be capable of producing sperm, but are at a higher risk of infertility. The XX form is extremely common, occurring in 5-10% of AFAB people. The XY form is rare. Common traits and disabilities that may co-occur include an early puberty (which may lead to a shorter height than average), oily skin, higher levels of acne, a high libido, bald patches, high blood cholesterol, diabetes, behavioral disorders, mood disorders, and anxiety disorders. Familial Male-Limited Precocious Puberty (FMPP)/Testotoxicosis is an extremely rare form of the XY form of hyperandrogenism, where puberty-influencing androgen production begins extremely early, causing puberty to begin between the ages of 1-5 years old.

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Hypogonadism

These are traits that cause a person's gonads to produce a lower level of hormones than in males and females.

Primary Hypogonadism/Hypergonadotropic Hypogonadism describes when the gonads themselves have low production levels. The brain is still communicating to produce the average male/female levels of hormones, but the gonads are failing to keep up with the brains-signals.

Secondary Hypogonadism/Hypogonadtropic Hypogonadism/ Central Hypogonadism describes when the brain has low levels of communication with the gonads. The brain is failing to send out typical levels of signals to the gonads, and the gonads only produce hormones when a signal is received.

Hypoestrogenism/Estrogen Deficiency: A trait in which an individual has low levels of estrogen. If they have ovaries, this describes having lower levels of estrogen than females do. If they have testicles, this describes having lower levels of estrogen than males do. As a lone variation, this comes in both an XX form and an XY form. Those with the XX form have XX chromosomes, a vulva, two ovaries, and a uterus. During puberty (without the influence of HRT), they will have estrogen as their main sex hormone (though it will be low) and have the possibility of developing breasts and widened hips. Pregnancy is possible, however they are at a higher risk of infertility. Those with the XY form have XY chromosomes, a penis, two testicles, and a prostate. During puberty (without the influence of HRT), they will have testosterone as their main sex hormone, and will likely develop increased body/facial hair, higher muscle mass, and a deepened voice. They may be capable of producing sperm, but are at a higher risk of infertility. This trait is rare on its own, but fairly common when partnered with Turner Syndrome or PCOS. Common traits and disabilities that may co-occur include a delayed puberty, a low libido, genital dryness, a higher risk of urinary tract infections, less body/facial hair than average, low bone density, heart disease, sleep disorders, depressive disorders, anxiety disorders, and frequent headaches.

Hypoandrogenism/Androgen Deficiency: A trait in which an individual has low levels of androgens. If they have ovaries, this describes having lower levels of androgens than females do. If they have testicles, this describes having lower levels of androgens than males do. As a lone variation, this comes in both an XX form and an XY form. Those with the XX form have XX chromosomes, a vulva, two ovaries, and a uterus. During puberty (without the influence of HRT), they will have estrogen as their main sex hormone and will likely develop breasts and widened hips. Pregnancy is possible, however they are at a higher risk of infertility. Those with the XY form have XY chromosomes, a penis, two testicles (possibly with microorchidism), and a prostate. During puberty (without the influence of HRT), they will have testosterone as their main sex hormone (though it will be low), and have the possibility of developing increased body/facial hair, higher muscle mass, and a deepened voice. They may be capable of producing sperm, but are at a higher risk of infertility. This trait is rare on its own, but fairly common when partnered with Klinefelter Syndrome. Common traits and disabilities that may co-occur include a delayed puberty, a low libido, less body/facial hair than average, low bone density, heart disease, sleep disorders, depressive disorders, anxiety disorders, and frequent headaches.

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Other Hormonal Variations

Congenital Adrenal Hyperplasia (CAH): A variation in which an individual has adrenal glands that produce low levels of cortisol and/or aldosterone, and as a result, produce extra androgens to make up for it (leading to hyperandrogenism.) Classic CAH is when the symptoms are obvious at infancy, while Nonclassic CAH is when the symptoms occur in later childhood/teen years or adulthood. Classic CAH can be simple-virilizing or salt-wasting. Simple-virilizing CAH is when the aldosterone levels are low, but not dangerous. Salt-wasting CAH is when the aldosterone levels are too low to regulate the salt within the blood, leading to high levels of salt in the urine, which will need supplements and medications to manage. If people with salt-wasting CAH don't get treatment, it can result in low blood sugar, high levels of acid in the blood, dehydration, vomiting, diarrhea, irregular heartbeat, shock, coma, and death. Classic CAH has both an XX form and an XY form. Those with the XX form of classic CAH have XX chromosomes, a vulva (possibly with vaginal hypoplasia or a urogenital sinus anomaly) or ambiguous genitals, two ovaries, and a uterus. During puberty (without the influence of HRT), they will have estrogen as their main sex hormone and will likely develop breasts and widened hips, with the possibility of increased body/facial hair, higher muscle mass, and a deepened voice. Their menstruation may be irregular. Pregnancy is possible, however they are at a higher risk of infertility. Those with the XY form of Classic CAH have XY chromosomes, a penis (possibly a macropenis) two testicles (possibly with macroorchidism), and a prostate. During puberty (without the influence of HRT), they will have testosterone as their main sex hormone (though it will be high) and will likely develop increased body/facial hair, higher muscle mass, and a deepened voice. They may be capable of producing sperm, but are at a higher risk of infertility. Nonclassic CAH has both an XX and an XY form as well. Those with the XX form of nonclassic CAH have XX chromosomes, a vulva (though it may develop into clitoromegaly), two ovaries, and a uterus. During puberty (without the influence of HRT), they will have estrogen as their main sex hormone and will likely develop breasts and widened hips, with the possibility of increased body/facial hair, higher muscle mass, and a deepened voice. They might also develop PCOS. Their menstruation may be irregular. Pregnancy is possible, however they are at a higher risk of infertility. Those with the XY form of Nonclassic CAH have XY chromosomes, a penis, two testicles , and a prostate. During puberty (without the influence of HRT), they will have testosterone as their main sex hormone (though it will be high) and will likely develop increased body/facial hair, higher muscle mass, and a deepened voice. They may be capable of producing sperm, but are at a higher risk of infertility. Classic CAH is rare. Nonclassic CAH is extremely common, occurring in 1 of every 200-1,000 births. Common traits and disabilities that may co-occur with all forms of CAH include an early puberty (which may lead to a shorter height than average), oily skin, and higher levels of acne.

Leydig Cell Hypoplasia (LCH): A variation in which an individual with XY chromosomes is born with testicles (possibly with microorchidism or cryptorchidism), which has underdeveloped or absent leydig cells, resulting in hypoandrogenism and delayed or absent puberty. They have a penis or micropenis (possibly with hypospadias), bifid scrotum, or a vulva. They also have a prostate. If they start puberty naturally (and don't use HRT), they will have testosterone as the main sex hormone (though it will be low), and have the possibility of developing increased body/facial hair, higher muscle mass, and a deepened voice. They may be capable of producing sperm, but are at a higher risk of infertility (and if a vulva is present, the semen may have no way to exit, depending on how the testes are connected to the genitals.) This variation is extremely rare. Common traits and disabilities that may co-occur include a taller height than average, low bone density, and delayed or absent bone maturation.

17-Ketosteroid Reductase Deficiency (17 KSR Deficiency): A variation in which an individual with XY chromosomes has a genetic mutation that causes the hormone androstenedione not to convert into testosterone as frequently as it down within males, resulting in hypoandrogenism. They have a penis or micropenis (possibly with hypospadias), ambiguous genitals, or a vulva (though it may develop into clitoromegaly). They also have two testicles (possibly with microorchidism or cryptorchidism) and a prostate. During puberty (without the influence of HRT), they will have testosterone as their main sex hormone (though it will be high) and will likely develop increased body/facial hair, higher muscle mass, and a deepened voice, with the possibility of breast growth. They may be capable of producing sperm, but are at a higher risk of infertility (and if a vulva is present, the semen may have no way to exit, depending on how the testes are connected to the genitals.) This variation is rare.

5α-Reductase 2 Deficiency (5αR2D)/Pseudovaginal Perineoscrotal Hypospadias/PPSH: A variation in which an individual with XY chromosomes has a genetic mutation that causes a lower production of dihydrotestosterone compared to males, resulting in hypoandrogenism. They have a penis or micropenis (possibly with hypospadias), ambiguous genitals, or a vulva (though it may develop into clitoromegaly.) They also have two testicles (possibly with cryptorchidism) and a prostate (possibly with prostate hypoplasia.) During puberty (without the influence of HRT), they will have testosterone as their main sex hormone (though it will be high) and will likely develop increased body/facial hair, higher muscle mass, and a deepened voice. They may be capable of producing sperm, but are at a higher risk of infertility (and if a vulva is present, the semen may have no way to exit, depending on how the testes are connected to the genitals.) This variation is rare.

Aromatase Deficiency: A variation in which an individual's body is incapable of converting androgens into estrogen, resulting in hyperandrogenism and hypoestrogenism. This begins during fetal development, and comes in both an XX form and an XY form. Those with the XX form have XX chromosomes, a clitorophallus or fused labia, two ovaries (possibly hypoplastic ovaries), and a uterus (though menstruation does not occur.) During puberty (without the influence of HRT), they will have testosterone as their main sex hormone (though it will be high), and will likely develop increased body/facial hair, higher muscle mass, and a deepened voice. Those with the XY form of aromatase deficiency have XY chromosomes, a penis, two testicles (possibly with microorchidism or cryptorchidism), and a prostate. During puberty (without the influence of HRT), they will have testosterone as their main sex hormone (though it will be high) and will likely develop increased body/facial hair, higher muscle mass, and a deepened voice. This variation is extremely rare. Due to the rarity, little is known about the fertility status. Common traits and disabilities that may co-occur include patches of darker/lighter skin, a taller height than average, low bone density, long limbs, inturned knees, high blood sugar, type 2 diabetes, weight gain, and a fatty liver. A person who is pregnant with a baby that has aromatase deficiency will get secondary effects of the androgens from the fetus within them. They may develop phallus growth, increased body/facial hair, and a deepened voice. These changes typically end once the baby is born.

Estrogen Insensitivity Syndrome (EIS)/Estrogen Resistance: A variation in which an individual's estrogen receptors do not respond to estrogen being produced. The estrogen is present, but the body is not fully using it. This may result in high levels of estrogen in the blood (due to estrogen being unused and building up), with the body only using minimal amounts of it. This comes in both an XX and an XY form. Those with the XX form have XX chromosomes, a vulva, two ovaries, and a uterus (possibly with uterine hypoplasia.) Menstruation is absent or irregular. They may also have hyperandrogenism and experience ovarian cysts. During puberty (without the influence of HRT), if they don't have hyperandrogenism, then they will have estrogen as the main sex hormone, however it will have very little effect on their physical development. If they have hyperandrogenism, then testosterone will be their main sex hormone (or even just equal to estrogen, though the estrogen is being unused), however its effects will be minimal, likely only resulting in severe acne and pubic hair. Those with the XY form have XY chromosomes, a penis, two testicles (possibly with microorchidism or cryptorchidism), and a prostate. During puberty (without the influence of HRT), they will have testosterone as their main sex hormone, and will likely develop increased body/facial hair, higher muscle mass, and a deepened voice. This variation is extremely rare. Due to the rarity, little is known about the fertility status, though it seems there's a higher risk of infertility. Common traits and disabilities that may co-occur include a delayed puberty, a taller height than average, and low bone density.

Androgen Insensitivity Syndrome (AIS)/Androgen Resistance: A variation in which an individual with XY chromosomes has androgen receptors that do not respond to androgens being produced. The androgens are present, but the body is not fully using it. This may result in high levels of estrogen in the blood (due to estrogen being unused and building up), with the body only using minimal amounts of it. This comes in 3 forms - mild, partial, and complete. Those with Mild Androgen Insensitivity Syndrome (MAIS) have a body that is only mildly unresponsive to androgens, meaning that most of it is still in use. They have a penis (possibly with hypospadias), two testicles, and a prostate. During puberty (without the influence of HRT), they will have testosterone as their main sex hormone, and will likely develop increased body/facial hair, higher muscle mass, and a deepened voice. They may be capable of producing semen, but are at a higher risk of infertility. Those with Partial Androgen Insensitivity Syndrome (PAIS) have a body that is partially unresponsive to androgens, meaning that a significant amount of it is not in use. They have a penis or micropenis (possibly with chordee or hypospadias), ambiguous genitals, or a vulva with a urogenital sinus anomaly. They have two testicles (possibly with cryptorchidism), and may have a prostate (possibly with prostate hypoplasia) or no prostate. During puberty (without the influence of HRT), they will have testosterone as their main sex hormone, and will likely develop increased body/facial hair, higher muscle mass, and a deepened voice, with the possibility of breast growth and widened hips. They may be capable of producing sperm, but are at a higher risk of infertility (and if they lack a prostate, the sperm will be incapable of leaving, and thus medical intervention to extract the sperm would be necessary in order to create a biological offspring.) Those with Complete Androgen Insensitivity Syndrome (CAIS) have a body that is completely unresponsive to androgens. They have a vulva (possibly with vaginal hypoplasia, labial hypoplasia, and clitoral hypoplasia,) and two testicles with cryptorchidism. They have no prostate and no uterus. During puberty (without the influence of HRT), they will have estrogen as their main sex hormone, and will likely develop breasts and widened hips. They are infertile. All forms AIS are rare. Common traits and disabilities that may co-occur include a taller height than average, large teeth, dry skin, drier eyes than typical, light sensitivity in the eyes, and dry hair.

Chromosomal Variations

These are variations affect a person's chromosomes.

Swyer Syndrome/XY Gonadal Dysgenesis: A variation in which an individual has XY chromosomes, but due to a genetic mutation, they develop a CTF appearance. They have a vulva or clitoromegaly, a uterus (possibly with uterus hypoplasia), and two streak ovaries. They will not begin puberty unless they are on HRT. Due to the streak ovaries, they are infertile, however if their uterus is large enough, they can carry a (genetically unrelated) embryo if it is medically implanted. This variation is rare. A common trait/disability that may co-occur is low bone density.

Mixed Gonadal Dysgenesis (MGD)/45X-46XY Mosaicism: A variation in which an individual is born with some cells having X chromosomes, and other cells having XY chromosomes. This comes in two forms. Those with the first form have a vulva (possibly with a urogenital sinus anomaly or vaginal aplasia) or ambiguous genitals and ovaries (though one or both will be streak ovaries). They may in some cases have a uterus. If both of their gonads are streak ovaries, they will not start puberty without HRT. If they start puberty naturally (and don't use HRT), they will have estrogen as the main sex hormone (though hypoestrogenism is common, meaning their estrogen levels may be low), and will likely develop breasts and widened hips. If they have a non-streak ovary, but no uterus, their eggs can create an embryo to be implanted into another carrier. If they have two streak ovaries, but have a uterus, then they can carry a (genetically unrelated) embryo if it is medically implanted. If they have two streak ovaries, and no uterus, then they are infertile. Those with the second form have a penis/micropenis or ambiguous genitals, testicles (though one or both will be streak), and a prostate (possibly with prostate hypoplasia). If both of their gonads are streak testes, they will not start puberty without HRT. If they start puberty naturally (and don't use HRT), they will have testosterone as the main sex hormone (though hypoandrogenism is common, meaning their testosterone levels may be low), and will likely develop increased body/facial hair, higher muscle mass, and a deepened voice. If they have a non-streak testicle, they can produce semen - though, if they also have prostate hypoplasia, they are at a higher risk of infertility. If both of their testes are streak, then they are infertile. This variation is rare.

XYY Syndrome/Jacob's Syndrome: A variation in which an individual is born with XYY chromosomes. They have a penis/micropenis (possibly with hypospadias), two testicles (possibly with macroorchidism or cryptorchidism), and a prostate. During puberty (without the influence of HRT), they will have testosterone as their main sex hormone (though hypoandrogenism or hyperandrogenism is common, so their testosterone may be high or low), and will likely develop increased body/facial hair, higher muscle mass, and a deepened voice. They may be capable of producing semen, but are at a higher risk of infertility. This variation is fairly common, occurring in 1 of every 1,000 AMAB births. Common traits and disabilities that may co-occur include being taller than average, hypotonia, flat feet, abnormal feet patterns, asthma, a large head, low-set ears, flattened cheekbones, dental abnormalities, widely-spaced eyes, night-blindness, tic disorders, seizure disorders, neurodevelopmental disabilities, learning disabilities, and communication disabilities.

XYYY Syndrome: A variation in which an individual is born with XYYY chromosomes. They have a penis, two testicles, and a prostate. During puberty (without the influence of HRT), they will have testosterone as their main sex hormone (though hypoandrogenism is common, so their testosterone may be low), and will likely develop increased body/facial hair, higher muscle mass, and a deepened voice. This variation is extremely rare. Due to the rarity, little is known about the fertility status, though it seems there's a higher risk of infertility. Common traits and disabilities that may co-occur include being taller than average, hypotonia, fused forearm bones, incurved fingers/toes, radial head dislocation, a short neck, dental abnormalities, a thick lower lip, a flat bridge of the nose, a longer space between the upper lip and nose, folded eyelids, widely spaced eyes, higher levels of acne, and intellectual disability.

XYYYY Syndrome: A variation in which an individual is born with XYYYY chromosomes. They have a penis, two testicles, and a prostate. During puberty (without the influence of HRT), they will have testosterone as their main sex hormone (though hypoandrogenism is common, so their testosterone may be low), and will likely develop increased body/facial hair, higher muscle mass, and a deepened voice. This variation is extremely rare. Due to the rarity, little is known about the fertility status, though it seems there's a higher risk of infertility. Common traits and disabilities that may co-occur include fused forearm bones, an incurved pinkie finger, facial asymmetry, a smaller than typical jaw, widely-spaced eyes, low set ears, hypertelorism, neurodevelopmental disabilities, and communication disabilities.

XXYYY Syndrome: A variation in which an individual is born with XXYYY chromosomes. They have a penis, two testicles, and a prostate. During puberty (without the influence of HRT), they will have testosterone as their main sex hormone, and will likely develop increased body/facial hair, higher muscle mass, and a deepened voice. This variation is extremely rare. Due to the rarity, little is known about the fertility status. Common traits and disabilities that may co-occur include delayed bone development, abnormal facial features, a small head, and intellectual disability.

XXXYY Syndrome: A variation in which an individual is born with XXXYY chromosomes. They have a penis/micropenis or ambiguous genitals, two testicles (possibly with microorchidism or cryptorchidism), and a prostate. During puberty (without the influence of HRT), they will have testosterone as their main sex hormone (though hypoandrogenism is common, so their testosterone may be low), and will likely develop breasts and widened hips. This variation is extremely rare. Due to the rarity, little is known about the fertility status. Common traits and disabilities that may co-occur include being taller than average, delayed bone development, twisted feet, abnormal feet patterns, long legs, long arms, incurved fingers, a slim or underweight body, frequent upper respiratory infections, a narrow chest, a prominent jaw, a small jaw, a wide bridge of the nose, widely spaced eyes, folded eyelids, a prominent forehead, an abnormal face shape, low-set rotated ears, neurodevelopmental disabilities, communication disabilities, and behavioral disorders.

XXYY Syndrome: A variation in which an individual is born with XXYY chromosomes. They have a penis or micropenis, two testicles (possibly with microorchidism or cryptorchidism), and a prostate. During puberty (without the influence of HRT), they will have testosterone as their main sex hormone (though hypoandrogenism is common, so their testosterone may be low), and will likely develop increased body/facial hair, higher muscle mass, a deepened voice, breast growth, and widened hips. They may be capable of producing semen, but are at a higher risk of infertility. This variation is rare. Common traits and disabilities that may co-occur include being taller than average, hypotonia, hyperflexible joints, flat feet, hip dislocation, inguinal hernias, cardiovascular abnormalities, gastrointestinal disabilities, weight gain , asthma, apnea, frequent upper respiratory tract infections, lymphatic cancer, scoliosis, an incurved pinkie finger, elbow dislocation, fused forearm bones, shoulder abnormalities, a flat back of the skull, a long face, facial asymmetry, a broad jaw, dental abnormalities, a cleft lip, a thick lower lip, widely spaced eyes, upslanting eyelids, folded eyelids, drooping eyelids, movement disorders, neurodevelopmental disabilities, communication disabilities, depressive disorders, anxiety disorders, and psychotic disorders.

Klinefelter Syndrome/XXY Syndrome: A variation in which an individual has XXY chromosomes. They have a penis or micropenis, two testicles (possibly with cryptorchidism), and a prostate. During puberty (without the influence of HRT), they will have testosterone as their main sex hormone (though hypoandrogenism is common, so their testosterone may be low), and will likely develop increased body/facial hair, higher muscle mass, a deepened voice, breast growth, and widened hips. They may be capable of producing semen, but are at a higher risk of infertility. This variation is extremely common, occurring in 1 of every 500 AMAB births. Common traits and disabilities that may co-occur include a low libido, being taller than average, weak bones, hypotonia, breast cancer, increased body fat, type 2 diabetes, cardiovascular abnormalities, autoimmune disorders, lung disease, narrow shoulders, dental abnormalities, neurodevelopmental disabilities, communication disabilities, learning disabilities, behavioral disorders, depressive disorders, and anxiety disorders.

XXXY Syndrome: A variation in which an individual is born with XXXY chromosomes. They have a penis or micropenis, two testicles (possibly with microorchidism or cryptorchidism), and a prostate. During puberty (without the influence of HRT), they will have testosterone as their main sex hormone (though hypoandrogenism is common, so their testosterone may be low), and will likely develop increased body/facial hair, higher muscle mass, a deepened voice, breast growth, and widened hips. They may be capable of producing semen, but are at a higher risk of infertility. This variation is rare. Common traits and disabilities that may co-occur include being taller than average, hypotonia, hyperflexible joints, abnormal shaping at the end of bones, flat feet, twisted feet, hip dislocation, abnormally angled hips, an underdeveloped kidney, inguinal hernias, gastrointestinal disabilities, increased body fat, frequent respiratory tract infections, asthma, cardiovascular abnormalities, scoliosis, elbow dislocation, fused forearm bones, an incurved pinkie finger, low-set sloped shoulders, a short neck, a flat back of the skull, dental abnormalities, a large jaw, a cleft palate, facial asymmetry, a flat bridge of the nose, widely-spaced eyes, upslanting eyelids, folded eyelids, drooping eyelids, crossed eyes, frequent ear infections, tremors, seizure disorders, neurodevelopmental disabilities, communication disabilities, anxiety disorders, and psychotic disorders.

XXXXY Syndrome: A variation in which an individual has XXXXY chromosomes. They have a penis or micropenis, two testicles (possibly with microorchidism or cryptorchidism), and a prostate. During puberty (without the influence of HRT), they will have testosterone as their main sex hormone (though hypoandrogenism is common, so their testosterone may be low), and will likely develop increased body/facial hair, higher muscle mass, a deepened voice, breast growth, and widened hips. They are infertile. This variation is extremely rare. Common traits and disabilities that may co-occur include being shorter than average, hypotonia, hyperflexible joints, abnormal shaping for the end of the bones, flat feet, a twisted foot, abnormally angled hips, hip dislocation, an absent or underdeveloped kidney, cardiovascular abnormalities, gastrointestinal disabilities, asthma, frequent respiratory infections, scoliosis, an incurved pinkie finger, fused forearm bones, low-set sloped shoulders, a short neck, a flat back of the skull, a prominent jaw, dental abnormalities, a cleft palate, a wide nose, a flat nose, absent olfactory bulbs (resulting in an inability to smell), upslanted eyelids, drooping eyelids, widely spaced eyes, crossed eyes, near-sightedness, frequent ear infections, tremors, seizure disorders, neurodevelopmental disabilities, and communication disabilities.

XXXXX Syndrome/Pentasomy X: A variation in which an individual has XXXXX chromosomes. They have a vulva, two ovaries, and a uterus. During puberty (without the influence of HRT), they will have estrogen as the main sex hormone, and will likely develop breasts and widened hips. This variation is extremely rare. Due to the rarity, little is known about the fertility status, however it appears to be average. Common traits and disabilities that may co-occur include delayed puberty, being shorter than average, short feet, hip dislocation, immune system disorders, cardiovascular abnormalities, a small head, a flat back of the head, a small lower jaw, a flat front face, dental abnormalities, a wide nasal bridge, upslanted eyelids, widely spaced eyes, crossed eyes, low-set rotated ears, and intellectual disability.

XXXX Syndrome/Tetrasomy X: A variation in which an individual has XXXX chromosomes. They have a vulva, two ovaries, and a uterus. During puberty (without the influence of HRT), they will have estrogen as the main sex hormone, and will likely develop breasts and widened hips. Their menstruation may be irregular. Pregnancy is possible, however they are at a higher risk of infertility and early menopause. This variation is extremely rare. Common traits and disabilities that may co-occur include delayed puberty, hypotonia, hyperflexible joints, hip dislocation, kidney disorders, bladder disabilities, immune system abnormalities, autoimmune disorders, cardiovascular abnormalities, short fingers/toes, an incurved pinkie finger, dental abnormalities, widely spaced eyes, upslanting eyelids, crossed eyes, seizure disorders, learning disabilities, and communication disabilities.

Triple X Syndrome/Trisomy X/XXX Syndrome: A variation in which an individual has XXX chromosomes. They have a vulva, two ovaries, and a uterus. During puberty (without the influence of HRT), they will have estrogen as the main sex hormone, and will likely develop breasts and widened hips. Their menstruation may be irregular. Pregnancy is possible, however they are at a higher risk of infertility and early menopause. This variation is fairly common, occurring in 1 of every 1,000 AFAB births. Common traits and disabilities that may co-occur include delayed puberty, being taller than average, hypotonia, hyperflexible joints, flat feet, an absent or underdeveloped kidney, kidney defects, frequent urinary tract infections, autoimmune disorders, a caved-in chest, fused forearm bones, incurved fingers, widely-spaced eyes, folded eyelids, drooping eyelids, tremors, seizure disorders, neurodevelopmental disabilities, communication disabilities, learning disorders, mood disorders, anxiety disorders, and psychotic disorders.

SERKAL Syndrome: A variation in which an individual has XX chromosomes, but due to a genetic mutation in the WNT4 gene, they develop a CTM appearance. They have a penis or micropenis (possibly with hypospadias or penoscrotal transposition), two testicles or ovotestes (possibly with cryptorchidism), and a prostate. This variation is extremely rare. There is no information on fertility or puberty, as the only three recorded cases of this variation were terminated as fetuses. Common traits and disabilities that may co-occur include a small/underdeveloped bladder, an absent kidney, adrenal abnormalities, non-coiled intestines, small/underdeveloped lungs, heart abnormalities, a diaphragmatic hernia, and an oral cleft.

XX Male Syndrome/De La Chapelle Syndrome: A variation in which an individual has XX chromosomes, but due to one of their X chromosomes containing an SRY gene, they develop a CTM appearance. They have a penis/micropenis (possibly with hypospadias) or ambiguous genitals, two testicles (possibly with microorchidism or cryptorchidism), and a prostate. During puberty (without the influence of HRT), they will have testosterone as their main sex hormone (though hypoandrogenism is common, so their testosterone may be low), and will likely develop increased body/facial hair, higher muscle mass, a deepened voice, breast growth, and widened hips. They are infertile. This variation is rare. Common traits and disabilities that may co-occur include a shorter height than average and a low libido.

Turner Syndrome/Monosomy X: A variation in which an individual has only one X chromosome and no Y chromosome (0X.) They have a vulva, ovaries (usually hypoplastic ovaries or streak ovaries), and a uterus. Puberty may be absent or delayed. If they start puberty naturally (and don't use HRT), they will have estrogen as the main sex hormone (though hypoestrogenism is common, so their estrogen may be low), and will likely develop breasts and widened hips. If one of their ovaries is non-streak, then pregnancy is possible, however they are at a higher risk of infertility and early menopause. If they have two streak ovaries, then they can carry a (genetically unrelated) embryo if it is medically implanted. This variation is fairly common, occurring in 1 of every 2,500 AFAB births. Common traits and disabilities that may co-occur include a delayed puberty, a shorter height than average, vitiligo, moles, birthmarks, skin scarring, low bone density, outturned forearms, nail abnormalities, abnormal feet/hand patterns, flat feet, flexible toe joints, short toes, splayed toes, abnormal shape for the end of the tibia bones, inturned knees, enlarged outer thighbones, hip dislocation, an absent or underdeveloped kidney, fused kidneys, abnormally located kidneys, increased body fat, liver defects, heart defects, autoimmune disorders, thyroid gland inflammation, cystic hygroma, scoliosis, a hunched back, a short sternum, a wide rib cage, a caved-in chest, a broad chest, a broad neck, a webbed neck, a short neck, a low hairline on the back of the head, a small/underdeveloped or absent lower jaw, dental abnormalities, drooping eyelids, folded eyelids, crossed eyes, near-sightedness, prominent ears, frequent ear infections, hearing disabilities, neurodevelopmental disabilities, communication disabilities, depressive disorders, anxiety disorders, and psychotic disorders.

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And thats all!

Again, please reblog to spread awareness. Intersex people are highly discriminated against. Their bodies are still regularly mutilated at birth or during puberty, and they are still forced into HRT, in order to make them "look right" or "function right."

This medical abuse can cause complete infertility, a loss of sensation in genital areas (making sex unsatisfactory), chronic pain, gender or sex incongruence, and body dysmorphia.

Additionally, intersex children are often bullied at school for looking or sounding "abnormal" for their age/gender. And as they grow up, they face the same difficulties transgender individuals do - judgement for not being a "real man" or "real woman" (or for being non-binary), difficulty dating, struggles finding jobs, complications in receiving proper healthcare, and they are at an increased risk of being abused and assaulted. Many are also left out of sports or kicked out of public bathrooms as well.

This is all due to the lack of education. Tolerance and acceptance needs to be taught to children. Many doctors have no idea how to treat intersex patients, as they didn't learn about their bodies, even in advanced schooling. We need to put a stop to this.

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answersfromzestual · 2 years ago

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Information on Testosterone Hormone Therapy:

As you prepare to begin treatment, now is a great time to think through what your goals are, as the approach to hormone therapy is definitely not one-size-fits-all.

Do you want to get started right away on a path to the maximum safe effects? Or, do you want to begin at a lower dose and allow things to progress more slowly? Perhaps your long term goal is to seek less-than-maximal effects and you would like to remain on a low dose for the long term. Thinking about your goals will help you communicate more effectively with your medical provider (an endocrinologist is the best way) as you work together to map out your care plan.

Many people are eager for hormonal changes to take place rapidly- understandably so. But it's very important to remember that the extent of, and rate at which your changes take place, depend on many factors. These factors include your genetics, the age at which you start taking hormones, and your overall state of health.

Consider the effects of hormone therapy as a second puberty, and puberty normally takes years for the full effects to be seen. Taking higher doses of hormones will not necessarily bring about faster changes, but it could endanger your health. And because everyone is different, your medicines or dosages may vary widely from those of your friends, or what you may have read in books or in online forums. Use caution when reading about hormone regimens that promise specific, rapid, or drastic effects. While it is possible to make adjustments in medications and dosing to achieve certain specific goals, in large part the way your body changes in response to hormones is more dependent on genetics and the age at which you start, rather than the specific dose, route, frequency, or types of medications you are taking.

While I will speak about the approach to hormone therapy in transgender men, my comments are also applicable to non-binary people who were assigned female at birth and are seeking masculinizing hormone therapy.

There are four areas where you can expect changes to occur as your hormone therapy progresses: Physical, emotional, sexual, and reproductive.

Physical

The first physical changes you will probably notice are that your skin will become a bit thicker and more oily. Your pores will become larger and there will be more oil production. You’ll also notice that the odors of your sweat and urine will change and that you may sweat more overall. You may develop acne, which in some cases can be bothersome or severe, but usually can be managed with good skin care practices and common acne treatments. Some people may require prescription medications to manage acne, please discuss this with your provider. Generally, acne severity peaks during the first year of treatment, and then gradually improves. Acne may be minimized by using an appropriate dosing of testosterone that avoids excessively high levels.

Your chest will not change much in response to testosterone therapy. That said, surgeons often recommend waiting at least 6-12 months after the start of testosterone therapy before having masculinizing chest surgery, otherwise known as top surgery, in order to first allow the contours of the muscles and soft tissues of your chest wall to settle in to their new pattern.

Your body will begin to redistribute your weight. Fat will diminish somewhat around your hips and thighs. Your arms and legs will develop more muscle definition, with more prominent veins and a slightly rougher appearance, as the fat just beneath the skin becomes a bit thinner. You may also gain fat around your abdomen.

Your eyes and face will begin to develop a more angular, male appearance as facial fat decreases and shifts. Please note that it’s not likely your bone structure will change, though some people in their late teens or early twenties may see some subtle bone changes. It may take 2 or more years to see the final result of the facial changes.

Your muscle mass will increase, as will your strength, although this will depend on a variety of factors including diet and exercise. Overall, you may gain or lose weight once you begin hormone therapy, depending on your diet, lifestyle, genetics and muscle mass.

Testosterone will cause a thickening of the vocal chords, which will result in a more male-sounding voice. Not all trans men will experience a full deepening of the pitch of their voice with testosterone, however. Some may find that practicing various vocal techniques or working with a speech therapist may help them develop a voice that feels more comfortable and fitting. Voice changes may begin within just a few weeks of beginning testosterone, first with a scratchy sensation in the throat or feeling like you are hoarse. Next your voice may break a bit as it finds its new tone and quality.

The hair on your body, including your chest, back and arms will increase in thickness, become darker and will grow at a faster rate. You may expect to develop a pattern of body hair similar to other men in your family—just remember, though, that everyone is different and it can take 5 or more years to see the final results.

Regarding the hair on your head: most trans men notice some degree of frontal scalp hair thinning, especially in the area of your temples. Depending on your age and family history, you may develop thinning hair, male pattern baldness or even complete hair loss. Approaches to managing hair loss in trans men is the same as with cisgender men; treatments can include the partial testosterone blocker finasteride, minoxidil, which is also known as Rogaine, applied to the scalp, and hair transplantation. As with cis men, unfortunately there is no way to completely prevent male pattern baldness in those predisposed to develop this condition. Ask your provider for more information on strategies for managing hair loss.

Regarding facial hair, beards vary from person to person. Some people develop a thick beard quite rapidly, others take several years, while some never develop a full, thick beard. Just as with cisgender men, trans men may have varying degrees of facial hair thickness and develop it at varying ages. Those who start testosterone later in life may experience less overall facial hair development than those who start at younger ages.

Lastly, you may notice changes in your perception of the senses. For example, when you touch things, they may “feel different” and you may perceive pain and temperature differently. Your tastes in foods or scents may change.

Emotional state changes

The second area of impact of hormone therapy is on your emotional state.

Puberty is a roller coaster of emotions and the second puberty that you will experience during your transition is no exception. You may find that you have access to a narrower range of emotions or feelings, or have different interests, tastes or pastimes, or behave differently in relationships with other people. For most people, things usually settle down after a period time. Some people experience little or no change in their emotional state. I encourage you to take the time to learn new things about yourself, and sit with new or unfamiliar feelings and emotions while you explore and familiarize yourself with them. While psychotherapy is not for everyone, many people find that working with a therapist while in transition can help you to explore these new thoughts and feelings, get to know your new body and self, and help you with things like coming out to family, friends, or coworkers, and developing a greater level of self-love and acceptance.

Sexual changes

The third area of impact of hormone therapy is on your sexuality

Soon after beginning hormone treatment, you will likely notice a change in your libido. Quite rapidly, your genitals, especially your clitoris, will begin to grow and become even larger when you are aroused. You may find that different sex acts or different parts of your body bring you erotic pleasure. Your orgasms will feel different, with perhaps more peak intensity and a greater focus on your genitals rather than a whole body experience. Some people find that their sexual interests, attractions, or orientation may change when taking testosterone; it is best to explore these new feelings rather than keep them bottled up.

Don’t be afraid to explore and experiment with your new sexuality through masturbation and with sex toys. If you have a sex partner or partners, involve them in your explorations..

Reproductive system changes

The fourth area of impact of hormone therapy is on the reproductive system.

You may notice at first that your periods become lighter, arrive later, or are shorter in duration, though some may notice heavier or longer lasting periods for a few cycles before they stop altogether.

Testosterone may reduce your ability to become pregnant but it does not completely eliminate the risk of pregnancy. Transgender men can become pregnant while on testosterone, so if you remain sexually active with someone who is capable of producing sperm, you should always use a method of birth control to prevent unwanted pregnancy. Transgender men may use any form of contraception, including the numerous options available that do not contain estrogen, and some that contain no hormones at all. There are many contraception options that are long acting and do not require taking a daily pill. Transgender men may also use emergency contraception, also known as the “morning after pill”. Ask your medical provider for more information on the contraceptive and family planning options available to you.

If you suspect you may have become pregnant or have a positive pregnancy test while taking testosterone, speak with you provider as soon as possible, as testosterone can endanger the fetus.

If you do want to have a pregnancy, you’ll have to stop testosterone treatment and wait until your provider tells you that it’s okay to begin trying to conceive.

It’s also important to know that, depending on how long you’ve been on testosterone therapy, it may become difficult for your ovaries to release eggs, and you may need to consult with a fertility specialist and use special medications or techniques, such as in vitro fertilization, to become pregnant. These treatments are not always covered by insurance, and can be expensive. Uncommonly, testosterone therapy may cause you to completely lose the ability to create fertile eggs or become pregnant.

Risks

While cisgender men do have higher rates of cholesterol related disorders and heart disease than cisgender women, the available research on transgender men taking testosterone has generally not found these differences. Most of the research on risk of heart disease and strokes in transgender men suggests that risk does not increase once testosterone is begun. However, longer term, definitive studies are lacking. It has been suggested that the risk of other conditions such as diabetes or being overweight is increased by masculinizing testosterone therapy, however actual research supporting these claims are limited.

One known risk is that testosterone can make your blood become too thick, otherwise known as a high hematocrit count, which can cause a stroke, heart attack or other conditions. This can be a particular problem if you are taking a dose that is too high for your body’s metabolism. This can be prevented by maintaining an appropriate dose and through blood tests to monitor blood and hormone levels.

While available data are limited, it does not appear that testosterone increases the risk of cancer to the uterus, ovaries, or breasts. Because not all breast tissue is removed during masculinizing chest surgery, otherwise known as top surgery, there is a theoretical risk that breast cancer could develop in the remaining tissue. However, it can be difficult to screen for breast cancer in this tissue, and there are risks of a false positive test result. Your provider can give you more information about breast cancer screening after top surgery.

Cervical cancer is caused by an infection with the human papillomavirus, or HPV. HPV is transmitted sexually, more commonly by having sexual contact with someone who has a penis. However, people who have never had sexual contact with a penis may still contract an HPV infection. The HPV vaccine can greatly reduce your risk of cervical cancer, and you may want to discuss this with your provider. Pap smears are used to detect cervical cancer or precancer conditions, as well as an HPV infection. Your provider will make a recommendation as to how often you should have a pap smear. It is unclear if testosterone therapy plays any role in HPV infection or cervical cancer.

If your periods have stopped because of testosterone treatment, be sure to report any return of bleeding or spotting to your provider, who may request an ultrasound or other tests to be certain the bleeding isn’t a symptom of an imbalance of the lining of the uterus. Sometimes such an imbalance could lead to a precancerous condition, although this is rare in transgender men. Missing a dose or changing your dose can sometimes result in return of bleeding or spotting. Some men may experience a return of spotting or heavier bleeding after months or even years of testosterone treatment. In most cases this represents changes in the body’s metabolism over time. To be safe, always discuss any new or changes to bleeding patterns with your doctor.

Fortunately, since you do not have a prostate, you have no risk of prostate cancer and there is no need to screen for this condition.

If you have had your ovaries removed, it is important to remain on at least a low dose of hormones post-op until at minimum age 50. This will help prevent a weakening of the bones, otherwise known as osteoporosis, , which can result in serious and disabling bone fractures.

Most people using masculinizing testosterone therapy will experience at least a small amount of acne. Some may experience more advanced acne. Often this acne responds to typical over-the-counter treatments, but in some cases prescription medication may be required. Acne usually peaks within the first year of treatment and then begins to improve.

While gender affirming hormone therapy usually results in an improvement in mood, some people may experience mood swings or a worsening of anxiety, depression, or other mental health conditions as a result of the shifts associated with starting a second puberty. If you have any mental health conditions it is recommended you remain in discussion with a mental health providers as you begin hormone therapy.

Other medical conditions may be impacted by gender affirming hormone therapy, though research is lacking. These include autoimmune conditions, which can sometimes improve or worsen with hormone shifts, and migraines, which often have a hormonal component. Ask your medical provider if you have further questions about the risks, health monitoring needs, and other long term considerations when taking hormone therapy.

Some of the effects of hormone therapy are reversible, if you stop taking them. The degree to which they can be reversed depends on how long you have been taking testosterone. Clitoral growth, facial hair growth, voice changes and male-pattern baldness are not reversible.

Testosterone treatment approaches

Testosterone comes in several forms. Injections are usually best given weekly to maintain even levels of testosterone in the blood. Studies have shown that using a smaller needle and injection by the subcutaneous, or under the skin, approach, is just as effective as the intramuscular approach, which involves a larger needle injecting deeper into the muscle. In addition to injections, there are gel and patches that can be applied to the skin daily. The gel is applied to skin and once dry, you can swim, shower, and have contact with others. The patch also allows swimming, showering, exercise, and contact with others. All of these forms work equally well when the dosing is adjusted to achieve the desired hormone levels, and the decision about which form to use should be based mostly on your preference.

Another option for testosterone is the use of pellets under the skin. These are inserted every few months via a minor in-office procedure. Ask your medical provider for more information about this approach.

Recently, an oral form of testosterone, taken as a pill twice daily, has been approved for use. There are potential risks of high blood pressure when taking this medication, so extra steps need to be taken to monitor your health if you choose to use this form of your testosterone. Ask your medical provider for more information about this approach.

Regardless of the type of testosterone you are taking, it’s important to know that taking more testosterone will not make your changes progress more quickly, but could cause serious side effects or complications. Excess testosterone can result in mood symptoms or irritability, bloating, pelvic cramping, or even a return of menstruation. High levels of testosterone also result in increased estrogen levels, as a percentage of all testosterone in the body is converted to estrogen. In general estrogen blocking medicines are not used as a part of masculinizing hormone therapy.

Other medications that may be used include progestagens, which are hormones similar to or identical to those made by the body to maintain a balance in the lining of the uterus. These hormones can be used in cases where periods continue after testosterone levels have been optimized. These hormones can cause mood swings, bloating, and other side effects, so it is recommended that you discuss these medications further with you provider if they are to be used.

Final thoughts

And finally, please remember that all of the changes associated with the puberty you’re about to experience can take years to develop. Starting hormone therapy in your 40s, 50s, or beyond may bring less drastic changes than one might see when beginning transition at a younger age, due to the accumulated lifetime exposure to estrogen, and declining responsiveness to hormone effects as one approaches the age of menopause. Once you have achieved male-range testosterone levels, taking higher doses won’t result in faster or more dramatic changes, however they can result in more side effects or complications.

Now that you have learned about the effects of masculinizing hormone therapy, as well as risks and specific medication options, the next step will be to speak with your provider about what approach is best for you.

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