Development Of The Genital System Flashcards

1
Q

At what week can the male and female external genitalia be recognized?

A

Week 12

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2
Q

By what week should all the phenotypic differentiation be complete?

A

Week 20

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3
Q

Where do the primordial germ cells reside in the developing fetus?

A

Endoderm of yolk sac

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4
Q

What guides migration of the primordial germ cells?

A

C-kit receptor and stem-cell factor

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5
Q

What is the karyotype of an individual that has pure gonadal dysgenesis?

A

Normal, just problem with specific gene on a chromosome due to failure of PGC migration.

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6
Q

Where do the PGCs migrate too?

A

Primitive sex cords

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7
Q

The ductus deferens is a remnant of what?

A

Mesonephric duct

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8
Q

Gonads, kidneys, and supra renal glands are derived from what tissue?

A

Intermediate mesoderm

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9
Q

What ducts are kept by the male and female?

A

Male- mesonephric duct

Female- paramesonephric duct

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10
Q

What determines the sex of a male?

A

SRY on the Y chromosome. Synthesis will trigger male development.

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11
Q

What two effects does testosterone have in the normal developing male fetus?

A

Descent of testes into the scrotum.

Mesonephric duct-> epididymis, seminal vesicles, ductus deferens.

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12
Q

What could be the cause of a genitic male with female secondary sex characterists?

A

Defect in the 5 alpha reductase.

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13
Q

What does SRY/ TDF act upon?

A

Sertoli cells

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14
Q

What are the two molecules that are required for Sertoli cell differentiation and normal male sexual development?

A

SOX9, SF1

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15
Q

What is the result of stimulation of the Sertoli cells in males?

A

Production of Mullerian inhibitory substance and regression of the paramesonephric ducts.

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16
Q

What is the result when there is a mutation in SOX9?

A

Campomelic dysplasia. Can have female sex characteristics.

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17
Q

What does the urogenital sinus become in males and females?

A

Male- urethra, prostate. Closes in response to DHT.

Female- lower vagina vestibule.

18
Q

What does the genital tubercle become in the male and female?

A

Male- penis

Female- clitoris

19
Q

What does the labioscrotal swellings become in the male and female?

A

Male- scrotum.

Female- vulva.

20
Q

What is lost in the developing ovary?

A

Medulla and mesonephric duct.

21
Q

What are the remnants of the mesonephric duct in the female and where can they be found?

A

Epoophoron and paroophoron. Found in the mesentary of the ovary.

22
Q

What signaling molecule in the female blocks the action of SOX9 and AMH expression?

A

DAX 1

23
Q

The uterus and upper portion of the vagina are formed via what structures coming together?

A

Paramesonephric duct.

24
Q

What tissue contributes to the formation of the lower 1/3 of the vagina?

A

Posterior wall of the urogenital sinus. AKA tissue of sinovaginal bulbs

25
Q

How can a 46, XY have persistence of the paramesonephric ducts and female sexual characteristics?

A

Circulating levels of AMH are low or there is an abnormal response to normal AMH.

26
Q

What is the failure of paramesonephric ducts to develop called in a female?

A

MRKH- Mayer-Rokitansky-Kuster-Hauser Syndrome.

27
Q

What divides the cloacal membrane and anal membranes?

A

Urorectal septum

28
Q

What is the term for undescended testes?

A

Cryptrochidism

29
Q

what is the name of the incomplete fusion of the urethral folds?

A

Hypospadias.

30
Q

What are the four types of hypospadias?

A

Glandular, penile, penoscrotal, perineal.

31
Q

What is it called when the urethral meatus opens on the dorsum of the penis?

A

Epispadias

32
Q

What is klinefelters syndrome?

A
  • 47 XXY.
  • characterized by small testes, low levels of testosterone.
  • poorly developed secondary sexual characteristics, gynecomastia.
33
Q

What is turners syndrome?

A
  • 45X or 45X/46XX.

- short stature, ovary development is abortive, secondary sexual characteristics do not develop.

34
Q

What is 46XX DSD or 46XY DSD?

A
  • external genitalia of one sex accompanies the other sex.
35
Q

Early exposure to androgens or excess amounts of androgens can result in what in females?

A
  • 46, XX, DSD.

- clitoral hypertrophy and labial fold fusion.

36
Q

What are the characteristics of Androgen Insensitivity Syndrome?

A
  • 46, XY, DSD.
  • will not respond to testosterone.
  • Will show female secondary sexual characteristics.
37
Q

What is 5 alpha reductase deficiency?

A
  • genetic males that are born with ambiguous genitalia.

- Cannot convert testosterone into DHT.

38
Q

At what week does the phenotypic sexual differentiation begin?

A

Week 7.

39
Q

What cells releases AMH?

A

Sertoli cells in the testes

40
Q

What is the driving force for development of the male external genetalia?

A

DHT