Pseudohermaphroditism is a condition in which an individual has a matching chromosomal and

gonad A gonad, sex gland, or reproductive gland is a mixed gland that produces the gametes and sex hormones of an organism. Female reproductive cells are egg cells, and male reproductive cells are sperm. The male gonad, the testicle, produces sper ...

al tissue (

ovary The ovary is an organ in the female reproductive system that produces an ovum. When released, this travels down the fallopian tube into the uterus, where it may become fertilized by a sperm. There is an ovary () found on each side of the body. ...

or testis) sex, but mismatching external genitalia. Female pseudohermaphroditism refers to an individual with ovaries and external genitalia resembling those of a male. Male pseudohermaphroditism refers to an individual with testes and external genitalia resembling those of a female. In some cases, external sex organs associated with pseudohermaphroditism appear intermediate between a typical

clitoris The clitoris ( or ) is a female sex organ present in mammals, ostriches and a limited number of other animals. In humans, the visible portion – the glans – is at the front junction of the labia minora (inner lips), above the ope ...

and penis. Thus, pseudohermaphroditism is sometimes not identified until puberty or adulthood. The term contrasts with true hermaphroditism, a condition describing an individual with both female and male reproductive organs. Associated conditions include 5-α-reductase deficiency and androgen insensitivity syndrome.

Mechanism

Sex 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. 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. 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. Persistent Müllerian duct syndrome is 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.

Management

Surgery is sometimes performed to alter the appearance of the genitals. Sex-specific cancers present on the gonads may require surgical removal.

History

John Money is perhaps the best-known early researcher in this area. His

doctoral A doctorate (from Latin ''docere'', "to teach"), doctor's degree (from Latin ''doctor'', "teacher"), or doctoral degree is an academic degree awarded by universities and some other educational institutions, derived from the ancient formalism ''li ...

thesis was titled ''Hermaphroditism: An Inquiry into the Nature of a Human Paradox'', and awarded by Harvard University in 1952.

Terminology

The term "Pseudohermaphroditimus" (pseudohermaphroditism) was coined in German by Edwin Klebs in 1876.Klebs, T. A. E. (1876). Handbuch der pathologischen Anatomie andbook of pathological anatomy Berlin: A. Hirschwald, p. 723

/ref> Klebs had included the term as a synonym for the earlier coined, "spurious hermaphroditism" (which he referred to as ''Schein-Zwitter'' in German). "Spurious hermaphroditism" was coined in 1836 by J. Y. Simpson.Simpson, J. Y. (1836). "Hermaphroditism" in ''The Cyclopaedia of Anatomy and Physiology''. Vol. 2. p. 685

/ref> Although "pseudohermaphroditism" persisted in the International Classification of Diseases, Versions 9 (ICD-9) and 10 (ICD-10) as 752.7 (Indeterminate sex and pseudohermaphroditism) and Q56 (Interdeterminate sex and pseudohermaphroditism), it has since been removed in the eleventh version (ICD-11), in favor of LD2A.Y (Other specified malformative disorders of sex development). Some experts have indicated that both pseudohermaphroditism (also called false hermaphroditism) and true hermaphroditism are outdated, confusing, and potentially pejorative, indicating replacement with "disorders of sex development", "disorders of sexual development", "differences of sex development" (all abbreviated as DSD) or " intersex". Additionally, intersex activists have noted that: "The qualifiers 'pseudo' and 'true' are even more harmful

han hermaphrodite on its own Han may refer to: Ethnic groups * Han Chinese, or Han People (): the name for the largest ethnic group in China, which also constitutes the world's largest ethnic group. ** Han Taiwanese (): the name for the ethnic group of the Taiwanese p ...

because they imply a sort of authenticity, or lack of same, that carry powerful emotional baggage".

Dreger Dreger is a surname. Notable people with the surname include: * Alice Dreger, American historian, bioethicist and former professor of clinical medical humanities and bioethics at the Feinberg School of Medicine, Illinois * Darren Dreger (born 1968) ...

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".

References

External links