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Reproduction > Embryology of the Genital System > Flashcards

Embryology of the Genital System Flashcards

1
Q

what weeks is the embryo in the undifferentiated state

A

weeks 1-6

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2
Q

when does the phenotypic sexual differentiation befin

A

week 7

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3
Q

what week can male and female external genitalia be recognized

A

week 12

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4
Q

when is the phenotypic differentiation complete

A

week 20

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5
Q

primordial germ cells from ____ invade ____ and migrate to ____

A

endoderm of yolk sac, invade dorsal mesentery, migrate to urogenital ridges

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6
Q

what is the migration of the primordial germ cells guided by what?
-failure of migration = what

A

c-kit receptor and stem cell factor (c-kit ligand) expressed on PGCs
-failure of migration leads to gonadal dysgenesis

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7
Q

what is the karyotype of people with pure gonadal dysgenesis

A

normal set of sex chroms 46, XX or 46, XY

-may have defects of a specific gene on a chromosome

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8
Q

what is the name of PGD, 46, XY

A

swyer syndrome

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9
Q

what is the name of PGD, 46, XX

A

XX gonadal dysgenesis

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10
Q

in a male the ___ part of the gonad develops

in the female the __ part of the gonad develops

A 
male = medulla
female = cortex
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11
Q

what kind of mesoderm is gonads

A

intermediate, problems with gonads can also be accompanied by problems with kidneys

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12
Q

what does the mesonephric duct turn into in male

A

ductus deferens and epididymis

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13
Q

paramesonephric or mesonephric duct develops in female?

A

paramesonephric (mullerian)

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14
Q

what is the default fetal sex and what triggers the other sex

A

female is default
SRY protein made from sex determining region of Y chromosome
–SRY protein triggers male devlopment

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15
Q

what is the transcriptional regulator for testes differentiation and via what

A

SOX9 via SF1

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16
Q

what is the signifigance of the sertoli cells in the male

A

sertoli cells secrete MIH or AMH which causes the paramesonephric ducts to regress

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17
Q

what is campomelic dysplasia

A

mutation in SOX9 required for proper sex diff and chondrogenesis

  • congenital short limbed dwarfism with bowing of long bones
  • malformations of lungs, heart, kidneys
  • male to femal sex reversal
  • almost always fatal
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18
Q

sox9 is required for the formation of what cells

A

sertoli and leydig

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19
Q

what is function of leydig cells in males

A

formation of testosterone

20
Q

what is the function of DHT

A

masculinization of the external genitalia and prostate
masculinization of the brain
masculiniztion of body tissues and systems

21
Q

what is required for the maculinization of the internal reproductive system

A

testosterone

22
Q

what enzyme converts testosterone into DHT (which type) and where is it made

A

5a-reductase II

-made in seminal vesicle, epididymis, prostate

23
Q

in male the following structures become what? and what is the signal

  • urogenital sinus
  • genital tubercle
  • labioscrotal swellings
A

DHT is signal

UG sinus = urethra prostate
genital tubercle = penis
labioscrotal swellings = scrotum

24
Q

in female the following become what

  • Urogenital sinus
  • genital tubercle
  • labioscrotal swellings
A
  • UG sinus = stays open, lower vagnia vestibule
  • genital tubercle = clitoris
  • labioscrotal swelling, remain open, vulva
25
Q

in the female what can be left over from degeneration of the mesonephric ducts

A

epoophoron and paroophoron

-can be called gartner’s duct

26
Q

what signal is found on the X chromosome in both sexes but repressed by __ in males
-what is it upregulated by

A

DAX1 and repressed by SRY protein

-upregulated by WTN4

27
Q

DAX1 duplicated in male prevents what

A

testis formation, resulting in 46XY female

28
Q

cranial end of paramesonephric duct opens where and distal end goes where

A

cranial end = peritoneal cavity
distal end = joins to form uterovaginal primordium—>epithelium and glands of the uterus, epithelium of upper 1/3 of vagina, and muscular wall of vagina

29
Q

the inferior portion of the vagina forms from what

A

sinovaginal bulbs

-posterior wall of UG sinus (urinary bladder and urethra)

30
Q

what is mayer-rokitansky-kuster-hauser syndrome

A

failure of the paramesonephric ducts to develop so no uterine tubes, uterus, and malformation of upper vagina

31
Q

what is the presentation of re-canalization abnormality

A

present with cyclic or persistent pelvic pain and primary amenorrhea
-retrogade passage of blood into uterine tubes can cause hematosalimx which can lead to endometriosis and adhesion formation

32
Q

what is hematometra

A

from imperforate hymen, blood from menstrual cycle cannot get out

33
Q

the urogenital fold becomes ____ in female

labioscrotal fold becomes ____ in female

A

labia minora

labia majora

34
Q

steps of male external genitalia

A

1) enlargement of genital tubercle forms the phallus
2) urehtral folds then fuse to form penile urethra
3) labioscrotal swellings become the scrotum

35
Q

cryptorchidism

A

undescended testes, can be unilateral or bilateral

-can be associated with increased incidence of testicular cancer

36
Q

what is hypospadias and its cause

A

incomplete fusion of the urethral folds

  • urethra opens onto the ventral aspect of the benis
  • can be from inadequate androgen production or inadequate receptor sites for DHT
37
Q

what are the four types of hypospadias and which is most severe

A

1) glandular
2) penile
3) penoscrotal
4) perineal

the closer to body the more severe

38
Q

what is epispadias

and what does it often occur with

A

urethral meatus opens on dorsum of penis

  • often occurs with bladder exstrophy
  • anterior abdominal wall doesn’t close
39
Q

what is klinefelter syndrome and treatment

A

47, XXY

  • small testes, low levels testosterone
  • poor developed secondary sex characteristics and gynecomastia
  • risk for osteoporosis
  • testosterone replacement therpay
40
Q

what is turner’s syndrome

and treatment

A

45, XO or 45, XO/46,XX

  • short stature, borad chest, webbed neck, CHD
  • secondary sex characterisitcs don’t develop
  • GH and estrogen replacement therapy
41
Q

pseudointersexuality of 46 XX DSD or 46 XY DSD and cause

A

external genitalia of one sex accopmanies the gonads of the other sex

  • abnormal levels of sex hormones or receptors
  • prenatal exposure to androgens
  • the earlier the exposure, the more severe
42
Q

classic virilizing adrenal hyperplasia

A

46, XX, DSD

  • females with severe forms of adrenal hyperplasia have ambiguous genitalia at birth
  • labioscrotal folds fuse and look like scrotum
  • clitoral hypertrophy
  • ovaries are normal
43
Q

androgen insensitivity syndrome

A

46, XY, DSD

  • genetic male does not respond to testosterone
  • X linked disorder
  • external genitalia feminine
  • internally they possess non functioning undescended testes
  • at puberty, secondary femal sex charact may appear due to estradiol from testosterone aromatization
44
Q

5 a reducatase deficiency

A
  • externally look more female
  • males born with ambiguous genitalia
  • first woman then man
    • at puberty, increase in testosterone and if even a slight amount of 5a reductase then start to look like a man
  • also a 46, XY, DSD
45
Q

true intersex or ovotesticular DSD

A

have both ovarian and testicular tissue or ovotestes

46
Q

what part of the urogenital sinus is taken to make the lower part of the vagina

A

sinovaginal bulbs

Reproduction (15 decks)

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