True hermaphroditism
| True hermaphroditism | |
|---|---|
| Classification and external resources | |
| OMIM | 400045 |
| DiseasesDB | 29664 |
| eMedicine | med/1702 |
| MeSH | D050090 |
True hermaphroditism, also known as ovotesticular disorder of sex development, is a medical term for an intersex condition in which an individual is born with ovarian and testicular tissue. There may be an ovary underneath one testicle or the other, but more commonly one or both gonads is an ovotestis containing both types of tissue.
Although similar in some ways to mixed gonadal dysgenesis, the conditions can be distinguished histologically.[1]
Etymology
The term derives from the Latin: hermaphroditus, from Ancient Greek: ἑρμαφρόδιτος hermaphroditos,[2] which derives from Hermaphroditos ( Ἑρμαϕρόδιτος), the son of Hermes and Aphrodite in Greek mythology. According to Ovid, he fused with the nymph Salmacis resulting in one individual possessing physical traits of both sexes;[3] according to the earlier Diodorus Siculus, he was born with a physical body combining both sexes.[4] The word hermaphrodite entered the English lexicon in the late fourteenth century.[5]
Causes
This condition is very rare. There are several ways in which this may occur.
- It can be caused by the division of one ovum, followed by fertilization of each haploid ovum and fusion of the two zygotes early in development.
- Alternately, an ovum can be fertilized by two sperm followed by trisomic rescue in one or more daughter cells.
- Two ova fertilized by two sperm will occasionally fuse to form a tetragametic chimera. If one male zygote and one female zygote fuse, a hermaphroditic individual may result.
- It can be associated with mutation in the SRY gene.[6]
Human prevalence
There are no documented cases in which both types of gonadal tissue function. Encountered karyotypes are 47XXY, 46XX/46XY, or 46XX/47XXY, and various degrees of mosaicism.[7]
Although fertility is possible in true hermaphrodites, there has yet to be a documented case where both gonadal tissues function, contrary to the misconception that hermaphrodites can impregnate themselves. As of 2010, there have been at least 11 reported cases of fertility in true hermaphrodite humans in the scientific literature,[1] with one case of a person with XY-predominant (96%) mosaic giving birth.[7]
Presentation
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.
References
- 1 2 Kim, Kyu-Rae; Kwon, Youngmee; Joung, Jae Young; Kim, Kun Suk; Ayala, Alberto G.; Ro, Jae Y. (2002). "True Hermaphroditism and Mixed Gonadal Dysgenesis in Young Children: A Clinicopathologic Study of 10 Cases". Modern Pathology. 15 (10): 1013–9. doi:10.1097/01.MP.0000027623.23885.0D. PMID 12379746.
- ↑ "Definition of hermaphroditus". Numen: The Latin Lexicon. Retrieved 19 July 2013.
- ↑ Ovid, Metamorphoses, Book IV: The story of Hermaphroditus and Salmacis.
- ↑ Diodorus Siculus — Book IV Chapters 1–7
- ↑ Oxford English Dictionary, 1st edn, s.v. hermaphrodite, n. and adj.; "Online Etymology Dictionary". Retrieved 3 June 2012.
- ↑ Braun, A; Kammerer, S; Cleve, H; Löhrs, U; Schwarz, H. P.; Kuhnle, U (1993). "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". American Journal of Human Genetics. 52 (3): 578–85. PMC 1682159
. PMID 8447323. - 1 2 Schoenhaus, S. A.; Lentz, S. E.; Saber, P; Munro, M. G.; Kivnick, S (2008). "Pregnancy in a hermaphrodite with a male-predominant mosaic karyotype". Fertility and Sterility. 90 (5): 2016.e7–10. doi:10.1016/j.fertnstert.2008.01.104. PMID 18394621.
External links
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Media related to Intersex at Wikimedia Commons