In which of the following conditions do the ovaries function normally :
Correct Answer: Rokitansky-Kuster-Hauser syndrome
Description: In Klinfelter's syndrome :Genetic analysis reveals their karyotype to be 47 XXY. Testicular biopsy usually reveals hyaline degeneration of the seminiferous tubules and overgrowth of Leydig cells. In Turner's syndrome has also been called ovarian agenesis or gonadal dysgenesis because at laparotomy the gonad is found to consist of undifferentiated stroma with absence of sex cells, a mere strip of fibrous tissue attached to the back of the broad ligament like a pale strip, the so-called streak gonad. The follicles grow up to 20th week of fetal life but become atretic due to absence of one X sex chromosome. In some, germ cells fail to migrate to the genital ridge from the yolk sac. These ovaries do not contain Graafian follicles, so oestrogen is not produced. In swyer's syndrome, a male pseudohermaphrodite, a pure 46 XY gonadal dysgenesis with presence of uterus and the cervix but with hypo-oestrogenism and poorly developed breasts. Undeveloped testes do not secrete testosterone and MIF resulting in the development of female genital organs and female phenotype. The woman presents with primary amenorrhoea, absence of secondary sex characters and female external genitalia. Cyclical oestrogen and progestogen can induce menstruation. Conception with in vitro feilization (IVF) using donor eggs is a possibility.The gonads (testis) have 30% risk to undergo malignancy and should be removed Shaw's Textbook of Gynaecology 16thnedition page no 146.147
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Gynaecology & Obstetrics
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