Normal ovaries are seen in which of the following condition –
Correct Answer: MRKH syndrome
Description: Ans-A i.e. MRKH syndrome Mayer Rokitansky - Kuster Hauser syndromeIt refers to congenital absence of the vagina with variable uterine development - It results from agenesis or hypoplasia of the mullerian duct system although the underlying etiology remain unknown.These women have normal female karyotype with normal ovaries and ovarian function thus they develop normal secondary sexual characteristics (e.g. breast development axillary hair, and pubic hair) except menarche does not occur.Meyer Rokitansky kuster Hauser Syndrome These patients often present with primary amenorrhea at 15-17 years of age.Most patients have "rudimentary non functioning ironing uterus" but 2-7 percent have uterus with functioning endometrium and may present with cyclic or chronic abdominopelvic pain.* On physical examination the external genitalia are normal * In addition, these females exhibit extrarenal abnormalities- Approximately 20-50% have urologic abnormalities such as unilateral renal agenesis, pelvic or horseshoe kidney or irregularities of the collecting system and 10-15% have skeletal anomalies involving the spine ribs and extremities.
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