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Pseudohermaphroditism

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Pseudohermaphroditism
Other namesPseudo-hermaphroditism
SpecialtyGynecology,endocrinology

Pseudohermaphroditismis an outdated[1]term for when an individual's gonads were mismatched with their internal reproductive system and/or external genitalia. The term was contrasted with "true hermaphroditism"(now known asovotesticular syndrome), a condition describing an individual with both female and male reproductive gonadal tissues. Associated conditions includesPersistent Müllerian duct syndromeand forms ofandrogen insensitivity syndrome.

Mechanism

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Sexual development is determined by chromosomes during fertilization. In the early stages of human development, a human embryo has theprecursors of female (paramesonephric or Müllerian ducts) and male (mesonephric ducts or Wolffian) gonads.[2]If aY chromosomeis lacking, or defective as seen inSwyer syndrome,the embryo will reabsorb the mesonephric ducts and proceed with paramesonephric ducts, which give rise to ovaries. The Y chromosome contains a sex-determining region called theSRYgene. Thus, the developmental plan of the embryo is altered only if this gene is present and functional.[3]

Mutations affecting the androgen receptor (AR) gene may cause either complete or partialandrogen insensitivity syndrome.Androgensare a group of hormones which regulate the development and maintenance of male characteristics. Between 8 and 12 weeks, human male fetuses become externally distinct as androgens enlarge the phallus and produce a penis with a urethra andscrotum.[4]

Female pseudohermaphroditism refers to an individual with ovaries and external genitalia resembling those of a male. Male pseudohermaphroditism refers to an individual with testicles and external genitalia resembling those of a female.[5][6]In some cases, externalsex organsassociated with pseudohermaphroditism appear intermediate between a typicalclitorisandpenis.Thus, pseudohermaphroditism is sometimes not identified untilpubertyor adulthood.

Persistent Müllerian duct syndromewas considered a form of pseudohermaphroditism, developed through Müllerian-inhibiting factor defects. In such instances, duct derivatives are present in males, including the uterus, fallopian tubes, and upper vagina.[7]

Management

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Main article:Intersex medical interventions

Surgery is sometimes performed to alter the appearance of the genitals. Sex-specific cancers present on the gonads may require surgical removal.[8][9][10][11]

History

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Main articles:Intersex in historyandHistory of intersex surgery

John Moneyis perhaps the best-known early researcher in this area. Hisdoctoralthesiswas titledHermaphroditism: An Inquiry into the Nature of a Human Paradox,and awarded byHarvard Universityin 1952.[12]

Other animals

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Narave pigs,which are native toMalo Island,Vanuatu,are pseudohermaphrodite male domestic pigs that are kept for ceremonial purposes.[13][14][15]

Terminology

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The term "Pseudohermaphroditimus" (pseudohermaphroditism) was coined in German byEdwin Klebsin 1876.[16][17]Klebs had included the term as a synonym for the earlier coined, "spurious hermaphroditism" (which he referred to asSchein-Zwitterin German).[17]"Spurious hermaphroditism" was coined in 1836 by J. Y. Simpson.[18]

Although "pseudohermaphroditism" persisted in the International Classification of Diseases, Versions 9 (ICD-9) and 10 (ICD-10) as 752.7 (Indeterminate sex and pseudohermaphroditism)[19]and Q56 (Interdeterminate sex and pseudohermaphroditism),[20]it has since been removed in the eleventh version (ICD-11), in favor of LD2A.Y (Other specified malformative disorders of sex development).[21]

Some experts have indicated that both pseudohermaphroditism (also called false hermaphroditism) and true hermaphroditism are outdated,[22][23][24][25][26][27]confusing,[24][28]and potentially pejorative terms,[24][27][28][29][30]indicating replacement with "disorders of sex development", "disorders of sexual development", "differences of sex development" (all abbreviated as DSD)[22][24][27]or "intersex".[23][30]

Additionally,intersex activistshave noted that: "The qualifiers 'pseudo' and 'true' are even more harmful [than hermaphrodite on its own], because they imply a sort of authenticity, or lack of same, that carry powerful emotional baggage".[31]Dregeret al had also noted that "division of many intersex types into true hermaphroditism, male pseudohermaphroditism, and female pseudohermaphroditism is scientifically specious and clinically problematic".[32]

See also

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References

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  1. ^Lee, Peter A.; Houk, Christopher P.; Ahmed, S. Faisal; Hughes, Ieuan A. (1 August 2006)."Consensus Statement on Management of Intersex Disorders".Pediatrics.118(2): e488–e500.doi:10.1542/peds.2006-0738.PMID16882788.S2CID11236329.Retrieved1 May2023.
  2. ^Wilson, Danielle; Bordoni, Bruno (2024)."Embryology, Mullerian Ducts (Paramesonephric Ducts)".StatPearls.StatPearls Publishing.PMID32491659.
  3. ^"SRY gene: MedlinePlus Genetics".medlineplus.gov.Retrieved2021-07-03.
  4. ^Celayir A, Moralioglu S, Cetiner H, Kir G, Celayir S (2019)."Expression of androgen, estrogen, and progesterone hormone receptors in the penile tissues of children with different types of hypospadias".Northern Clinics of Istanbul.6(2): 110–116.PMC6593914.PMID31297475.
  5. ^"Pseudohermaphroditism | pathology".Encyclopedia Britannica.Retrieved2021-07-03.
  6. ^Michiels I, Peperstraete L, De Wever I, Gruwez JA (July 1984). "Inguinal hernia repair leading to the diagnosis of internal male pseudohermaphroditism".Acta Chirurgica Belgica.84(4): 255–258.PMID6485686.
  7. ^Patil V, Muktinaini S, Patil R, Verma A (June 2013)."Persistent müllerian duct syndrome: a case report".The Indian Journal of Surgery.75(Suppl 1): 460–462.doi:10.1007/s12262-013-0831-6.PMC3693295.PMID24426648.
  8. ^Boyd, Theonia K. (September 2010). "Disorders of Sexual Differentiation".Surgical Pathology Clinics.3(3): 553–601.doi:10.1016/j.path.2010.06.003.PMID26839224.
  9. ^Quattrin T, Aronica S, Mazur T (December 1990). "Management of male pseudohermaphroditism: a case report spanning twenty-one years".Journal of Pediatric Psychology.15(6): 699–709.doi:10.1093/jpepsy/15.6.699.PMID2283575.
  10. ^Hughes IA, Williams DM, Batch JA, Patterson MN (1992). "Male pseudohermaphroditism: clinical management, diagnosis and treatment".Hormone Research.38(2): 77–81.doi:10.1159/000182604(inactive 2024-09-12).PMID1292987.{{cite journal}}:CS1 maint: DOI inactive as of September 2024 (link)
  11. ^Boczkowski, Krzysztof (1974). "Management and Treatment of Male Pseudohermaphrodites".The Endocrine Function of the Human Testis.pp. 247–256.doi:10.1016/B978-0-12-380102-9.50019-6.ISBN978-0-12-380102-9.
  12. ^Money JW(1952).Hermaphroditism: An inquiry into the nature of a human paradox(PhD thesis). Harvard University.OCLC81648824.[page needed]
  13. ^Lum JK, McIntyre JK, Greger DL, Huffman KW, Vilar MG (November 2006)."Recent Southeast Asian domestication and Lapita dispersal of sacred male pseudohermaphroditic" tuskers "and hairless pigs of Vanuatu".Proceedings of the National Academy of Sciences of the United States of America.103(46): 17190–17195.Bibcode:2006PNAS..10317190L.doi:10.1073/pnas.0608220103.PMC1859908.PMID17088556.
  14. ^"Intersex Pigs".Southwest Pacific Research Project.Retrieved2023-03-07.
  15. ^"Pigs in Paradise".Penn State University.Retrieved2023-03-07.
  16. ^Zucker KJ (1999). "Intersexuality and gender identity differentiation".Annual Review of Sex Research.10:1–69.doi:10.1080/10532528.1999.10559774(inactive 2024-09-12).PMID10895247.{{cite journal}}:CS1 maint: DOI inactive as of September 2024 (link)
  17. ^abKlebs, T. A. E. (1876). Handbuch der pathologischen Anatomie [Handbook of pathological anatomy]. Berlin: A. Hirschwald, p. 723.[1]
  18. ^Simpson, J. Y. (1836). "Hermaphroditism" inThe Cyclopaedia of Anatomy and Physiology.Vol. 2. p. 685.[2]
  19. ^"2012 ICD-9-CM Diagnosis Code 752.7: Indeterminate sex and pseudohermaphroditism".Retrieved2022-03-03.
  20. ^"2022 ICD-10-CM Diagnosis Code Q56*: Indeterminate sex and pseudohermaphroditism".Retrieved2022-03-03.
  21. ^"ICD-11 for Mortality and Morbidity Statistics (Version: 02/2022): LD2A.Y Other specified malformative disorders of sex development".Retrieved2022-03-03.
  22. ^abMehmood, Khawar T.; Rentea, Rebecca M. (2024)."Ambiguous Genitalia and Disorders of Sexual Differentiation".StatPearls.StatPearls Publishing.PMID32491367.
  23. ^abMedlinePlus Encyclopedia:Intersex
  24. ^abcdKim, Kun Suk; Kim, Jongwon (2012)."Disorders of Sex Development".Korean Journal of Urology.53(1): 1–8.doi:10.4111/kju.2012.53.1.1.PMC3272549.PMID22323966.
  25. ^Amato V (2016).Intersex Narratives: Shifts in the Representation of Intersex Lives in North American Literature and Popular Culture.Bielefeld: Transcript Verlag.ISBN978-3-8394-3419-2.[page needed]
  26. ^Reis, Elizabeth (September 2007). "Divergence or Disorder?: the politics of naming intersex".Perspectives in Biology and Medicine.50(4): 535–543.doi:10.1353/pbm.2007.0054.PMID17951887.
  27. ^abcHughes IA (February 2008). "Disorders of sex development: a new definition and classification".Best Practice & Research. Clinical Endocrinology & Metabolism.22(1): 119–134.doi:10.1016/j.beem.2007.11.001.PMID18279784.
  28. ^abHouk CP, Hughes IA, Ahmed SF, Lee PA (August 2006). "Summary of consensus statement on intersex disorders and their management. International Intersex Consensus Conference".Pediatrics.118(2): 753–757.doi:10.1542/peds.2006-0737.PMID16882833.S2CID46508895.
  29. ^Kovacs CS, Deal C (12 October 2019).Maternal-Fetal and Neonatal Endocrinology: Physiology, Pathophysiology, and Clinical Management, p. 841.Academic Press.ISBN9780128148242.Retrieved2022-03-03.
  30. ^ab"Affirming Primary Care for Intersex People, 2020"(PDF).Retrieved2022-03-03.
  31. ^"On the Word Hermaphrodite - Intersex Society of North America".Retrieved2022-03-03.
  32. ^Dreger AD, Chase C, Sousa A, Gruppuso PA, Frader J (August 2005). "Changing the nomenclature/taxonomy for intersex: a scientific and clinical rationale".Journal of Pediatric Endocrinology & Metabolism.18(8): 729–733.doi:10.1515/jpem.2005.18.8.729.PMID16200837.S2CID39459050.
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