Question

In which of the following is the Mullerian system present?

• A. Mayer-Rokitansky-Kuster-Hauser syndrome
• B. Turner syndrome
• C. Testicular feminization syndrome
• D. Klinefelter syndrome

Hint:

Ask which option has no testes and therefore no anti-Mullerian hormone.

Answer 1

The Correct Option is B

Step 1: Understanding the Question.
We need to find the condition in which the Mullerian system (the uterus, fallopian tubes, and upper vagina) actually develops, even if it later stays small or abnormal.

Step 2: Key Concept.
The Mullerian ducts develop into the uterus and tubes unless a fetus has functioning testes that release anti-Mullerian hormone (AMH), which makes these ducts disappear. So the presence or absence of working testes decides whether the Mullerian system survives.

Step 3: Detailed Explanation.
Mayer-Rokitansky-Kuster-Hauser syndrome is defined by Mullerian agenesis itself; the uterus and upper vagina fail to form even though the ovaries and karyotype (46,XX) are normal, so the Mullerian system is absent here, not present.
Turner syndrome (45,X) has no testes at all, only streak gonads, so no AMH is made. The Mullerian ducts are never blocked and go on to form a uterus and tubes, though they stay small from lack of estrogen. This means the Mullerian system is present, just underdeveloped.
Testicular feminization syndrome (androgen insensitivity, 46,XY) has fully formed testes that secrete normal AMH, so the Mullerian ducts regress completely and no uterus forms.
Klinefelter syndrome (47,XXY) is a male with testes that also make AMH, so the Mullerian ducts regress in the same way and there is no uterus or tubes.

Step 4: Final Answer.
Since only Turner syndrome lacks functioning testes and AMH, it is the one condition where the Mullerian system is present, so the answer is Turner syndrome.