Pseudohermaphroditism

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Pseudohermaphroditism
ICD-10 Q56.1-Q56.3
ICD-9 752.7
DiseasesDB 14836 14839
MeSH D011545

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List of terms related to Pseudohermaphroditism

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Pseudohermaphroditism or pseudo-hermaphroditism, is a name used to describe the fact that some people are born with external sex organs that look intermediate between the typical vagina or penis. The term was adopted to distinguish between these people, who have typical ovaries or testes, and "true" hermaphrodites, who do not. It is also used for a range of other rare human physicalities. The term pseudohermaphroditism is now restricted to the technical literature, having been replaced by intersex in most public discussion, though this term is also challenged (see External links below).

The inadequency of the term outside technical descriptions can be demonstrated by many examples, but a simple one is the case of women with Complete Androgen Insensitivity Syndrome (CAIS). These women typically have primary and secondary sexual characteristics typical of other women, however they are genetically XY and have internal testes, rather than ovaries. They often marry but cannot have children. However scientifically precise the description "male" pseudohermaphrodite may be for such women, it is clearly socially inappropriate. CAIS is little better. Most intersex conditions are normal (if rare) human physicalities. The S for syndrome in CAIS does not accord with the "normality" CAIS women feel about their bodies.

Historical Perspective

Use of the term "pseudohermaphroditism" can be problematic. The term "pseudohermaphroditism" was created by Edwin Klebs in 1876, [1][2] long before the genetic roles of the X chromosome and Y chromosome and the social components of gender identity were well characterized, which is why the term is usually used to describe the dissonance between gonadal histology and external genital appearance.

The term "intersexuality" was introduced by Richard Goldschmidt in 1923. [1][3] However, the term "intersex" has also been challenged; the Lawson Wilkins Pediatric Endocrine Society and the European Society for Pediatric Endocrinology have adopted a nomenclature system based on disorders of sex development which covers "congenital conditions in which development of chromosomal, gonadal, or anatomical sex is atypical" and thus replaces many disparate terms, including but not limited to those based on "hermaphrodite."[4][5]

One example of the challenges involved in the use of the term is the case of women with Complete Androgen Insensitivity Syndrome (CAIS). These women typically have primary and secondary sexual characteristics typical of other women; however, they are genetically XY and have internal testes, rather than ovaries. They often marry but cannot have children. However scientifically precise the description "male" pseudohermaphrodite may be for such women, it is clearly socially inappropriate. CAIS is little better. The S for syndrome in CAIS does not accord with the "normality" CAIS women feel about their bodies.

Treatment

Surgery

Surgery has sometimes been performed to alter the appearance of the genitals.[6][7] Modern approaches often involve delaying surgery when possible until the child matures enough to express a preference.

References

  1. 1.0 1.1 "Intersexuality and gender identity differentiation Annual Review of Sex Research - Find Articles". Retrieved 2007-12-07.
  2. Klebs, T. A. E. (1876). Handbuch der pathologischen Anatomie [Handbook of pathological anatomy]. Berlin: A. Hirschwald,
  3. Goldschmidt, R. (1923). The Mechanism and Physiology of Sex Determination, Methuen & Co., London.
  4. Lee, P. A., C. P. Houk, S. F. Ahmed, and I. A. Hughes. 2006. Consensus statement on management of intersex disorders. Pediatrics 118 (2):e488-500. http://pediatrics.aappublications.org/cgi/reprint/118/2/e488
  5. "Why is ISNA using "DSD"?". Retrieved 2007-12-07. Text " Intersex Society of North America " ignored (help)
  6. Piró C, Asensio M, Barceló C, Martín JA, Chicaiza E (2004). "[Good results with Passerini's technique in severely masculinised female pseudohermaphroditism]". Cir Pediatr (in Spanish; Castilian). 17 (3): 118–21. PMID 15503946.
  7. Nihoul-Fékété C, Thibaud E, Lortat-Jacob S, Josso N (2006). "Long-term surgical results and patient satisfaction with male pseudohermaphroditism or true hermaphroditism: a cohort of 63 patients". J. Urol. 175 (5): 1878–84. doi:10.1016/S0022-5347(05)00934-1. PMID 16600787.

Acknowledgements

The content on this page was first contributed by: C. Michael Gibson, M.S., M.D.

it:Pseudoermafroditismo


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