Pseudohermaphroditism

Pseudohermaphroditism
Pseudohermaphroditism
Other names	Pseudo-hermaphroditism
Specialty	Gynecology, endocrinology

Pseudohermaphroditism is an outdated^[1] term for when an individual's gonads were mismatched with their internal reproductive system and/or external genitalia.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 as ovotesticular syndrome), a condition describing an individual with both female and male reproductive gonadal tissues. Associated conditions includes Persistent Müllerian duct syndrome and forms of androgen insensitivity syndrome.

Mechanism

Sexual development is determined by chromosomes during fertilization. In the early stages of human development, a human embryo has the precursors of female (paramesonephric or Müllerian ducts) and male (mesonephric ducts or Wolffian) gonads.^[2] If a Y chromosome is lacking, or defective as seen in Swyer 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 the SRY gene. 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 partial androgen insensitivity syndrome. Androgens are 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 and scrotum.^[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, external sex organs associated with pseudohermaphroditism appear intermediate between a typical clitoris and penis. Thus, pseudohermaphroditism is sometimes not identified until puberty or adulthood.

Persistent Müllerian duct syndrome was 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

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

John Money is perhaps the best-known early researcher in this area. His doctoral thesis was titled Hermaphroditism: An Inquiry into the Nature of a Human Paradox, and awarded by Harvard University in 1952.^[12]

Other animals

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

Terminology

The term "Pseudohermaphroditimus" (pseudohermaphroditism) was coined in German by Edwin Klebs in 1876.^[16]^[17] Klebs had included the term as a synonym for the earlier coined, "spurious hermaphroditism" (which he referred to as Schein-Zwitter in 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 activists have 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] Dreger et 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]

References

^ 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. PMID 16882788. S2CID 11236329. Retrieved 1 May 2023.
^ Wilson, Danielle; Bordoni, Bruno (2024). "Embryology, Mullerian Ducts (Paramesonephric Ducts)". StatPearls. StatPearls Publishing. PMID 32491659.
^ "SRY gene: MedlinePlus Genetics". medlineplus.gov. Retrieved 2021-07-03.
^ 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. PMC 6593914. PMID 31297475.
^ "Pseudohermaphroditism | pathology". Encyclopedia Britannica. Retrieved 2021-07-03.
^ 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. PMID 6485686.
^ 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. PMC 3693295. PMID 24426648.
^ Boyd, Theonia K. (September 2010). "Disorders of Sexual Differentiation". Surgical Pathology Clinics. 3 (3): 553–601. doi:10.1016/j.path.2010.06.003. PMID 26839224.
^ 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. PMID 2283575.
^ 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). PMID 1292987.{{cite journal}}: CS1 maint: DOI inactive as of September 2024 (link)
^ 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. ISBN 978-0-12-380102-9.
^ Money JW (1952). Hermaphroditism: An inquiry into the nature of a human paradox (PhD thesis). Harvard University. OCLC 81648824.^{[page needed]}
^ 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. PMC 1859908. PMID 17088556.
^ "Intersex Pigs". Southwest Pacific Research Project. Retrieved 2023-03-07.
^ "Pigs in Paradise". Penn State University. Retrieved 2023-03-07.
^ 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). PMID 10895247.{{cite journal}}: CS1 maint: DOI inactive as of September 2024 (link)
^ ^a ^b Klebs, T. A. E. (1876). Handbuch der pathologischen Anatomie [Handbook of pathological anatomy]. Berlin: A. Hirschwald, p. 723. [1]
^ Simpson, J. Y. (1836). "Hermaphroditism" in The Cyclopaedia of Anatomy and Physiology. Vol. 2. p. 685. [2]
^ "2012 ICD-9-CM Diagnosis Code 752.7 : Indeterminate sex and pseudohermaphroditism". Retrieved 2022-03-03.
^ "2022 ICD-10-CM Diagnosis Code Q56* : Indeterminate sex and pseudohermaphroditism". Retrieved 2022-03-03.
^ "ICD-11 for Mortality and Morbidity Statistics (Version: 02/2022) : LD2A.Y Other specified malformative disorders of sex development". Retrieved 2022-03-03.
^ ^a ^b Mehmood, Khawar T.; Rentea, Rebecca M. (2024). "Ambiguous Genitalia and Disorders of Sexual Differentiation". StatPearls. StatPearls Publishing. PMID 32491367.
^ ^a ^b MedlinePlus Encyclopedia: Intersex
^ ^a ^b ^c ^d Kim, 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. PMC 3272549. PMID 22323966.
^ Amato V (2016). Intersex Narratives: Shifts in the Representation of Intersex Lives in North American Literature and Popular Culture. Bielefeld: Transcript Verlag. ISBN 978-3-8394-3419-2.^{[page needed]}
^ 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. PMID 17951887.
^ ^a ^b ^c Hughes 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. PMID 18279784.
^ ^a ^b Houk 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. PMID 16882833. S2CID 46508895.
^ Kovacs CS, Deal C (12 October 2019). Maternal-Fetal and Neonatal Endocrinology: Physiology, Pathophysiology, and Clinical Management, p. 841. Academic Press. ISBN 9780128148242. Retrieved 2022-03-03.
^ ^a ^b "Affirming Primary Care for Intersex People, 2020" (PDF). Retrieved 2022-03-03.
^ "On the Word Hermaphrodite - Intersex Society of North America". Retrieved 2022-03-03.
^ 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. PMID 16200837. S2CID 39459050.

External links

[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. PMID 16882788. S2CID 11236329. Retrieved 1 May 2023.

[2] Wilson, Danielle; Bordoni, Bruno (2024). "Embryology, Mullerian Ducts (Paramesonephric Ducts)". StatPearls. StatPearls Publishing. PMID 32491659.

[3] "SRY gene: MedlinePlus Genetics". medlineplus.gov. Retrieved 2021-07-03.

[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. PMC 6593914. PMID 31297475.

[5] "Pseudohermaphroditism | pathology". Encyclopedia Britannica. Retrieved 2021-07-03.

[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. PMID 6485686.

[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. PMC 3693295. PMID 24426648.

[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. PMID 26839224.

[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. PMID 2283575.

[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). PMID 1292987.{{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. ISBN 978-0-12-380102-9.

[12] Money JW (1952). Hermaphroditism: An inquiry into the nature of a human paradox (PhD thesis). Harvard University. OCLC 81648824.^{[page needed]}

[PMID_17088556-13] 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. PMC 1859908. PMID 17088556.

[14] "Intersex Pigs". Southwest Pacific Research Project. Retrieved 2023-03-07.

[15] "Pigs in Paradise". Penn State University. Retrieved 2023-03-07.

[Intersexuality_and_gender_identity_differentiation_Annual_Review_of_Sex_Research_-_Find_Articles-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). PMID 10895247.{{cite journal}}: CS1 maint: DOI inactive as of September 2024 (link)

[klebs-17] Klebs, T. A. E. (1876). Handbuch der pathologischen Anatomie [Handbook of pathological anatomy]. Berlin: A. Hirschwald, p. 723. [1]

[simpson-18] Simpson, J. Y. (1836). "Hermaphroditism" in The Cyclopaedia of Anatomy and Physiology. Vol. 2. p. 685. [2]

[icd9-19] "2012 ICD-9-CM Diagnosis Code 752.7 : Indeterminate sex and pseudohermaphroditism". Retrieved 2022-03-03.

[icd10-20] "2022 ICD-10-CM Diagnosis Code Q56* : Indeterminate sex and pseudohermaphroditism". Retrieved 2022-03-03.

[icd11-21] "ICD-11 for Mortality and Morbidity Statistics (Version: 02/2022) : LD2A.Y Other specified malformative disorders of sex development". Retrieved 2022-03-03.

[mehmood_and_rentea-22] Mehmood, Khawar T.; Rentea, Rebecca M. (2024). "Ambiguous Genitalia and Disorders of Sexual Differentiation". StatPearls. StatPearls Publishing. PMID 32491367.

[medline_plus-23] MedlinePlus Encyclopedia: Intersex

[kim_and_kim-24] Kim, 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. PMC 3272549. PMID 22323966.

[intersex_narratives-25] Amato V (2016). Intersex Narratives: Shifts in the Representation of Intersex Lives in North American Literature and Popular Culture. Bielefeld: Transcript Verlag. ISBN 978-3-8394-3419-2.^{[page needed]}

[divergence_or_disorder-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. PMID 17951887.

[hughes-27] Hughes 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. PMID 18279784.

[houk_et_al-28] Houk 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. PMID 16882833. S2CID 46508895.

[kovacs_and_deal-29] Kovacs CS, Deal C (12 October 2019). Maternal-Fetal and Neonatal Endocrinology: Physiology, Pathophysiology, and Clinical Management, p. 841. Academic Press. ISBN 9780128148242. Retrieved 2022-03-03.

[affirming_care-30] "Affirming Primary Care for Intersex People, 2020" (PDF). Retrieved 2022-03-03.

[isna-31] "On the Word Hermaphrodite - Intersex Society of North America". Retrieved 2022-03-03.

[dreger_et_Al-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. PMID 16200837. S2CID 39459050.

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Pseudohermaphroditism

Mechanism

Management

History

Other animals

Terminology

See also

References

External links

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