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Biological Sex: part 2 Intersex & Disorder of Sex Development (DSD)


Hermaphrodites have both male and female reproductive organs during their lifespan Some of these animals can later change into the opposite sex others can self-fertilize A rough estimate of the number of hermaphroditic animal species is 65,000 So can humans be born as hermaphrodites? That’s what we’ll find out today, in this episode of Auau studio. The term “hermaphrodite” implies that a person has both male and female reproductive organs Not just parts In other words, they can produce both egg and sperm for reproduction It is impossible in human beings The word “hermaphrodite” is now only used for animals that have both natural male and female organs You may have heard Pseudo Hermaphrodite and True Hermaphrodite in humans They have been used to describe Ambiguous genitalia and gonadal Dysgenesis These words are out of date as well as misleading, stigmatizing, and “scientifically specious and clinically problematic.” These conditions are now called an intersex or a new medical term “Disorder of Sex Development” DSD DSD is a name given to a lot of different variations of sex development According to the United Nations, between 0.05% and 1.7% of the population is born with intersex traits the upper estimate is similar to the number of red-haired or twins people DSD people are born with sex characteristics that do not fit typical binary notions of male or female. Here are five Sex Characteristics present at birth Sex Chromosome Composition (XY or XX) Gonads (It can develop into testes or ovaries) Internal Genitalia (It can regress or develop into uterus) Sex Hormones (high or low level of androgens or estrogen) The appearance of the External Genitalia (It can develop into penis or clitoris) Typical males have things in the left column Typical females have things in the right column Intersex people can have things in between, or some are left and some are right However, they can’t have both So what causes DSD? We will go through every sex characteristic to see what causes DSD First, Sex Chromosome The most common sex chromosome compositions are XX and XY But there are several other possible compositions For example, X0, XXY, XXX, XYY,
XXYY, XXXY etc … If only next X is female and only XY is male. Then how do we account for it to be called chromosomes? Second, the gonads The proto-gonads will differentiate to become either ovaries or testes By default, the proto-gonads will differentiate into an ovary; The formation of testes was activated by the SRY gene on the Y chromosome. Swyer syndrome and 46 XX testicular DSD are forms of gonadal dysgenesis Swyer syndrome people were born with typically “male” XY chromosomes, but the SRY gene mutated 46 XX testicular DSD people were born with typically “female” XX chromosomes, but they have the Y chromosome gene SRY attached to one of their X chromosomes. The gonads can be any combination of ovary, testis or ovotestis (combined ovary and testis) A person might be born with ovary on one side, testis on the other, or one ovary and one ovotestis or some other combination Without fully functioning gonads, produced no hormones, the body did not develop in typical male or female path. Third, the Internal Genitalia Male sex differentiation is mainly driven by two types of hormones, testosterone and Anti-Müllerian Hormone (AMH) AMH is responsible for the regression of Müllerian ducts in male fetuses. When AMH is either not secreted or inactive Müllerian ducts do not regress It leads to Persistent Müllerian Duct Syndrome (PMDS) People with this syndrome have normal male structures But they have a uterus On the other hand, in Mayer-Rokitansky-Küster-Hauser (MRKH) MRKH have normal female structure But the Müllerian ducts failed to develop completely, Resulting in a missing uterus Forth, Sex Hormones & Fifth, External Genitalia The level of testosterone ultimately determines the appearance of the external genitalia, Androgen insensitivity syndrome (AIS) and Congenital Adrenal Hyperplasia (CAH) are the two most common cases. People with AIS have normal male structure But their bodies don’t respond in the normal way to male hormones And so the external genital cannot develop into penis People with CAH have normal female structure But the adrenal glands of people with CAH produce excess testosterone when they were in the womb And so the external genital cannot develop into clitoris As the level of testosterone are unusual with AIS and CAH both of them usually have ambiguous genitalia
(the genitalia are between male and female) We’ve looked at the five factors present at birth Because there are so many stages of sex development in human life, There are a lot of opportunities for a person to develop along a path that is not the average one for a boy or a girl Due to the existence of multiple forms of intersex conditions many view sex as existing along a spectrum, rather than as simply two mutually exclusive categories People usually have no doubt about their gender. But we don’t actually know our chromosomal make-up, we still believe we are typical male or female. In fact, If you were born with a penis, you would think you have XY, Likewise, if you were born without a penis, you would think you have XX Obviously, it’s not correct So, what made doctors decide our gender? That determination was the size of phallus. For the last fifty years, the size of the phallus has been the principal decision-making criteria for doctors In truth, surgeons can make a hole as vagina but they can’t build a pole as penis No one cares if they have XX or XY, have testes or ovaries, have uterus or not Maybe it’s time we need to listen what intersex people think It’s not what physicians can do

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