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Ovotesticular DSD

Ovotesticular DSD

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Published by Ahmed Rizk Ahmed

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Published by: Ahmed Rizk Ahmed on Jun 08, 2011
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05/21/2012

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Ovotesticular DSD - Ovarian and testicular tissues are present - Ambiguous genitals - Varying from normal female with

only slight enlargement of the clitoris - To almost normal male external genitals - 70% 46,XX SRY translocated on X chromosome - 20% 46,XX/46,XY mosaicism o Derived from more than one zygote o Chimeras (chi 46,XX/46,XY). o Presence of paternal and both maternal alleles for some blood groups is demonstrated. o An ovotesticular DSD chimera, 46,XX/46,XY, embryo amalgamation after in vitro fertilization. Each embryo was derived from an independent, separately fertilized ovum. - 10% portion of the Y chromosome including the SRY gene. - Most encountered gonad is ovotestis - Bilateral - If unilateral, the contralateral gonad is usually an ovary but may be a testis. - Ovarian tissue is normal, but testicular tissue is dysgenetic. - Basal and hCG-stimulated testosterone levels as well as AMH levels. - Patients who are highly virilized, have good testicular function, and have no uterus are usually reared as males. - If uterus exists, virilization is mild, testicular function minimal; assignment of female sex may be indicated. - Selective removal of gonadal tissue inconsistent with sex of rearing may be indicated. DIAGNOSIS AND MANAGEMENT. - Neonatal ambiguity o KARYOTYPING o to decide sex of rearing o . Management of the potential psychologic upheaval o Pelvic ultrasonography  Uterus +NO gonads= virilized XX female(search source of virilization  NO uterus±palpable gonads= undervirilized male o Measure: gonadotropins, testosterone, AMH, DHT

particularly when a vagina is present. controversy exists regarding the timing of the . a course of 3 monthly IM of testosterone enanthate (25 50 mg) o It has been thought that more feasible to reconstruct the external genitals to create a functional female. than to create a functional male phallus. o Similarly.o When receptor disorders in XY male with small phallus (micropenis).

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