True hermaphroditism

True hermaphroditism


Caption =
DiseasesDB = 29664
ICD10 =
ICD9 =
OMIM = 235600
MedlinePlus =
eMedicineSubj = med
eMedicineTopic = 1702
MeshID = D050090

True hermaphroditism is a medical term for an intersex condition in which a person is born with both ovarian and testicular tissue.

There may be an ovary on one side and a testis on the other, but more commonly one or both gonads is an ovotestis containing both types of tissue.

It is rare for both types of gonadal tissue to function. Encountered karyotypes are 47XXY, 46XX/46XY, or 46XX/47XXY.

Although similar in some ways to mixed gonadal dysgenesis, the conditions can be distinguished histologically.cite journal |author=Kim KR, Kwon Y, Joung JY, Kim KS, Ayala AG, Ro JY |title=True hermaphroditism and mixed gonadal dysgenesis in young children: a clinicopathologic study of 10 cases |journal=Mod. Pathol. |volume=15 |issue=10 |pages=1013–9 |year=2002 |month=October |pmid=12379746 |doi=10.1097/01.MP.0000027623.23885.0D |url=]


External genitalia are often ambiguous, the degree depending mainly on the amount of testosterone produced by the testicular tissue between 8 and 16 weeks of gestation.


This condition is very rare. There are several ways in which this may occur.
* It can be caused by the division of 1 ova, followed by fertilization of each haploid ova and fusion of the 2 zygotes early in development.
* Alternately, an ovum can be fertilized by two sperm followed by trisomic rescue in one or more daughter cell.

It can be associated with SRY.cite journal |author=Braun A, Kammerer S, Cleve H, Löhrs U, Schwarz HP, Kuhnle U |title=True hermaphroditism in a 46,XY individual, caused by a postzygotic somatic point mutation in the male gonadal sex-determining locus (SRY): molecular genetics and histological findings in a sporadic case |journal=Am. J. Hum. Genet. |volume=52 |issue=3 |pages=578–85 |year=1993 |month=March |pmid=8447323 |pmc=1682159 |doi= |url=]


ee also

* Ovotestis

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