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.

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. 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.
  2. "Definition of hermaphroditus". Numen: The Latin Lexicon. Retrieved 19 July 2013.
  3. Ovid, Metamorphoses, Book IV: The story of Hermaphroditus and Salmacis.
  4. Diodorus Siculus — Book IV Chapters 1–7
  5. Oxford English Dictionary, 1st edn, s.v. hermaphrodite, n. and adj.; "Online Etymology Dictionary". Retrieved 3 June 2012.
  6. 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 1682159Freely accessible. PMID 8447323.
  7. 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.
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