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Reproductive Physiology, Endocrinology, and Infertility

Question
47 out of 56
 

A girl has Primary amenorrhea with normal ovaries, absent internal genitalia but normal external genitalia. Most probable diagnosis? (AIPG 2010)



A Mayer-Rokitansky-Küster-Hauser Syndrome

B Turner syndrome

C Noonan’s

D androgen insensitivity syndrome

Ans. A

Mayer-Rokitansky-Küster-Hauser Syndrome

1. Müllerian agenesis is a congenital malformation in women characterised by a failure of the Müllerian ducts to develop, resulting in absent uterus and variable malformations of the vagina.

2. It is the second most common cause of primary amenorrhea. The condition is also called MRKH or Mayer-Rokitansky-Küster-Hauser Syndrome.

3. A woman with this condition is hormonally normal, that is she will enter puberty with development of secondary sexual characteristics including thelarche and adrenarche.

4. Her chromosome constellation will be 46,XX. Ovulation usually occurs. Typically, the vagina is shortened and intercourse may in some cases be difficult and painful.

5. Medical examination supported by gynecologic ultrasonography demonstrates a complete or partial absence of the cervix, uterus, and vagina. Ovaries are present and function normally.

6. This syndrome is characterised by congenital absence of vagina, primary amenorrhoea, rudimentary cornua uteri or absent uterus and morphologically normal ovaries and rudimentary Fallopian tubes (or fallopian tubes may also be absent) situated on the pelvic sidewall.

7. Normal ovulation; normal breast development; normal body and hair. The woman is amenorrhoeic and infertile.

8. In androgen insensitivity syndrome testes are present

Reproductive Physiology, Endocrinology, and Infertility Flashcard List

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