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Re: Interesting case From: ATFSOCCER@aol.com Wed Aug 27 13:24:55 1997 Messages sorted by: [ date ][ thread ][ subject ][ author ] Next message: rbraun@indyunix.iupui.edu: "Re: Puzzler?? 24 week ob with severe left flank pain" Previous message: ATFSOCCER@aol.com: "Re: Puzzler?? 24 week ob with severe left flank pain" Maybe in reply to: Milko Sirakov: "Interesting case" In a message dated 97-08-26 07:03:43 EDT, you write: << I have investgated a 19 years old girl with primary amenorrhoea. She have normal female fenotype, brest development and pubic hair - Tanner-4, very hypoplastic uterus, normal vagina. From the investigations - FSH - 135,6; LH - 26,5; Prl - norm.; Test - norm. (for female); Estr - 1,1. Surprise - kariogram 47 XY + mar. Her parents were also investigated - the mother is 46 XX, but the father - 47 XY + mar ! Sonography - Ut. - 10/16 mm., no ovaries were visualised. What do you mean - what I have to do ? >> Very interesting and very rare. It almost sounds like "testicular feminization" where she has the genotype of a male but has been insensitive to the virilizing effects of the androgens, ending up with female phenotype and primary amenorrhea. I would think that the gonads may be present although possibly hidden by the internal inguial ring or in the inguinal canal (as in failed descent of the testes) I am also curious to know if she has had a bilateral inguial hernia repair in early childhood?? The "y" containing gonads would have to be removed through laparoscopy or laparotomy and then start her on HRT with counseling that she will never have children naturally. This is my guess, see what the experts say. Next message: rbraun@indyunix.iupui.edu: "Re: Puzzler?? 24 week ob with severe left flank pain" Previous message: ATFSOCCER@aol.com: "Re: Puzzler?? 24 week ob with severe left flank pain" Maybe in reply to: Milko Sirakov: "Interesting case"

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