Question

Hypoplastic uterus with streak gonads is a feature of

• A. Testicular feminization syndrome
• B. Triple X syndrome
• C. Adrenogenital syndrome
• D. Turner syndrome

Hint:

Think about which condition has ovarian failure but no testes to block uterus formation.

Answer 1

The Correct Option is D

Step 1: Understanding the Question.
The question asks which condition shows both a small, underdeveloped uterus and streak gonads (thin fibrous bands instead of normal ovaries or testes).

Step 2: Key Concept.
Streak gonads with a hypoplastic uterus point to a genetic disorder where the gonads fail to develop properly (gonadal dysgenesis) in a person who still has a uterus, meaning no functioning testes were ever present to block Mullerian duct development.

Step 3: Detailed Explanation.
Turner syndrome (karyotype 45,X) is the classic cause. The single X chromosome cannot support normal ovarian development, so the ovaries degenerate into streak gonads before birth. Since there are no testes and no anti-Mullerian hormone, the uterus and fallopian tubes still form from the Mullerian ducts, but they stay small and underdeveloped because there is no estrogen to make them grow.
Testicular feminization syndrome (androgen insensitivity syndrome) has a 46,XY karyotype with intact testes that secrete anti-Mullerian hormone, so the uterus is absent, not hypoplastic; the gonads here are testes, not streak gonads.
Triple X syndrome (47,XXX) usually causes no gonadal or uterine problem at all; most affected women have normal periods and fertility.
Adrenogenital syndrome (congenital adrenal hyperplasia) affects a 46,XX person with normal ovaries and a normal uterus; the extra androgens only change the external genitalia, not the internal Mullerian structures.

Step 4: Final Answer.
Only Turner syndrome combines streak gonads with a small uterus, so the answer is Turner syndrome.