Primary Amenorrhea
Primary amenorrhea:menstruation should have started in the preteen and have not yet started.
Primary amenorrhea is considered if the girl is:
• Up to 14 years without development of secondary sexual characters (breast and fur)
• Up to 16 years without menstruating, but with secondary sexual characteristics (this is the
physiological deadline for this child to menstruate)
• After 3 years from thelarche without menstruating (breast development), as the interval is usually
2 years.
Beauty, what can cause this then? when we receive this teenager, we have to think about: congenital
malformations and genetic syndromes.
Primary amenorrhea without developed sexual characteristics
It is the case ofTurner syndrome: in this syndrome, there is a chromosomal alteration where the gene is
expressed by45X0. Therefore, this individual does not have the Y to develop the testis and does not have
the X to develop the ovary and thegonad remains primitive. She may even have fur, but it's because
ofhormone production from the hypothalamic-pituitary axis rather than the ovarian axis. She has other
associated defects such as cardiac anomalies, webbed neck, shield chest.We ask for US and karyotype.She is
infertile and has no hormones of her own..
Primary amenorrhea with developed sexual characteristics
We have 3 possible reasons for the young woman who has hair and breasts, but has not yet had her period
at age 16.
It is the case ofimperforate hymen: Ohymen is imperforateand does not allow menstruation to come out
through the vagina, which starts to accumulate blood until there is no more and this amenorrhea is therefore
accompanied byprogressive pain and the existence of a pelvic mass,as your sexual organs will increase in size
to accumulate this blood.
It is the case ofRokitansky Syndrome or Mullerian Agenesis: when we are mere cells, the fact that we are
XX theoretically guarantees us an ovary and, from there, it will command the development of the mullerian
duct that, without the stimulation of testosterone, will form the rest (uterus, tubes and vagina). In this
syndrome,the duct has not developedand even though she ovulates, she doesn't menstruate because she
doesn't have a uterus. this woman isfertile and has its hormone production, will only need a surrogate.
It is the case ofMorris syndrome: in this syndrome she has an XY karyotype, therefore,have testicleand it
was meant to be a man butshe has no testosterone receptor. With this, the male duct (wolff) does not receive
the signal to develop, because it does not have a receptor for it. With this, as the signal did not come, it is
the muller duct that develops. Isfemale, with a testicle (which can even stick out of the body in the inguinal
region), but with a uterus, tube and vagina. Her chest and fur are developed through adrenal hormone
production. In this woman,we can do hormone replacement. is infertile.