Gynaecological endocrinology: practice question

A OBG question in the style of the NEET PG paper. Answer it first, then read why the right option is right and why each of the others is wrong.

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OBG · Gynaecological endocrinology

Sample item

An 18-year-old has primary amenorrhoea with normal breast development and normal pubic and axillary hair. Karyotype is 46,XX and ultrasound shows absence of the uterus and upper vagina, with normal ovaries. What is the diagnosis?

Original practice item, not a recalled exam question. Nothing here is recorded.

The answer and the reasoning

B. Mayer-Rokitansky-Kuster-Hauser syndrome

Mullerian agenesis causes primary amenorrhoea in a woman with normal ovarian function, so secondary sexual characteristics are normal. The karyotype is 46,XX. Renal and skeletal anomalies are associated, and the neovagina can be created by dilators or surgery.

Option by option

  • A. Kallmann syndrome

    Kallmann syndrome has hypogonadotropic hypogonadism with anosmia and absent breast development.

  • B. Mayer-Rokitansky-Kuster-Hauser syndrome (correct)

    Correct. Absent uterus and upper vagina with normal ovaries and 46,XX defines the condition.

  • C. Turner syndrome

    Turner syndrome has streak gonads, short stature and absent breast development.

  • D. Complete androgen insensitivity syndrome

    Complete androgen insensitivity is 46,XY with scant pubic and axillary hair.

Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.

More OBG questions

Questions about this OBG question

What is the answer to this OBG question?

The best answer is B, Mayer-Rokitansky-Kuster-Hauser syndrome. Mullerian agenesis causes primary amenorrhoea in a woman with normal ovarian function, so secondary sexual characteristics are normal.

Why is "Kallmann syndrome" not the answer?

Kallmann syndrome has hypogonadotropic hypogonadism with anosmia and absent breast development.

Why is "Turner syndrome" not the answer?

Turner syndrome has streak gonads, short stature and absent breast development.

Why is "Complete androgen insensitivity syndrome" not the answer?

Complete androgen insensitivity is 46,XY with scant pubic and axillary hair.

What concept does this question test?

It tests Gynaecological endocrinology in OBG. The karyotype is 46,XX. Renal and skeletal anomalies are associated, and the neovagina can be created by dilators or surgery.

How hard is this question?

We tag it as an application question: it asks you to use a fact on a patient or a finding. The tag is our own editorial judgement, not an official difficulty rating, so read the reason for every option, not only the answer.

How should I study Gynaecological endocrinology?

Learn management algorithms as steps: PPH, eclampsia, ectopic pregnancy. Then use the flashcards and the other OBG questions on this site to test what stuck.

Is this a real NEET PG question?

No. It is an original practice item (editorial status: sample, version 1). NextPrep does not publish recalled or leaked questions, and independent faculty review of this item has not been completed yet, so confirm important facts in a standard textbook.