pseudohermaphroditism


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Related to pseudohermaphroditism: Male pseudohermaphroditism, Female pseudohermaphroditism

pseudohermaphroditism

 [soo″do-her-maf´ro-dit-izm″]
a congenital abnormality in which an individually is genetically and gonadally of one sex but has significant contradictions in the morphologic criteria of sex, often including ambiguous external genitalia. In female pseudohermaphroditism, the individual is a genetic and gonadal female with partial masculinization such as an enlarged clitoris resembling a penis and labia majora resembling a scrotum. In male pseudohermaphroditism, the individual is a genetic and gonadal male with feminization or incomplete masculinization, including a small penis, perineal hypospadias, and a scrotum that lacks testes. Pseudohermaphroditism is not to be confused with hermaphroditism, in which the individual has both ovarian and testicular tissue.

pseu·do·her·maph·ro·dit·ism

(sū'dō-hĕr-maf'rō-dīt-izm'),
A state in which the person is of an unambiguous gonadal sex (that is, possesses either testes or ovaries) but has ambiguous external genitalia. Compare: steroid 5α-reductase.

pseudohermaphroditism

/pseu·do·her·maph·ro·dit·ism/ (-her-maf´ro-dīt-izm″) a state in which the gonads are of one sex, but one or more contradictions exist in the morphologic criteria of sex. In female p., the individual is genetically female and has female gonads (ovaries) but has significant male secondary sex characters. In male p., the individual is genetically male and has male gonads (testes) but has significant female secondary sex characters.

pseudohermaphroditism

[-hərmaf′rəditiz′əm]
Etymology: Gk, pseudes + Hermaphroditos, son of Hermes and Aphrodite
a condition in which a person exhibits the somatic characteristics of both sexes though possessing the physical characteristics of either males (testes) or females (ovaries). Also spelled pseudohermaphrodism. See also ambiguous genitalia, feminization, def. 2, hermaphroditism. pseudohermaphroditic, adj.

pseudohermaphroditism

Endocrinology A state in which a person has the gonadal tissue of one sex, but the wiring, plumbing, and/or chassis of the opposite sex. See Female pseudohermaphroditism, Male pseudohermaphroditism. Cf Hermaphroditism, Intersex, Virilization.
Pseudohermaphroditism
Female pseudohermaphroditism A condition affecting a genotypic–46, XX female with ovaries, caused by a relative excess of androgen in utero, resulting in equivocal or masculinized genital duct derivatives, ie external genitalia and/or a male phenotype with genital ambiguity and/or virilization Etiology
1. Adrenogenital syndrome Defects of 21-hydroxylase, 11-β-hydroxylase or 3-β-hydroxysteroid dehydrogenase, or delta 5-4 isomerase deficiency, resulting in ↑ androgenic intermediates.
2. Maternal ingestion of progestins or androgens and three maternal virilizing tumors–eg, luteoma of pregnancy
Male pseudohermaphroditism, A condition affecting a genotypic–46, XY male with testes, caused by a relative deficiency of androgen in utero, resulting in a phenotypic female with ambiguous genitalia Etiology 1. Gonadal defects Testicular regression syndrome, persistent müllerian duct origin, Leydig cell agenesis and defects in testosterone synthesis 2. End-organ defects Testicular feminization or androgen insensitivity syndrome, incomplete androgen insensitivity syndrome and 5-α reductase deficiency. See Testicular feminization.
.

pseu·do·her·maph·ro·dit·ism

(sū'dō-hĕr-maf'rō-di-tizm)
A state in which the person is of an unambiguous gonadal sex (i.e., possesses either testes or ovaries) but has ambiguous external genitalia.
Synonym(s): false hermaphroditism.

pseudohermaphroditism

A congenital abnormality of the GENITALIA in which they resemble those of the opposite sex. The testes and ovaries (gonads) are, however, those of the genetically correct sex.

pseudohermaphroditism

a state in which the gonads are of one sex but one or more contradictions exist in the morphological criteria of sex. In female pseudohermaphroditism, the individual is a genetic and gonadal female with partial masculinization; in male pseudohermaphroditism, the individual is a genetic and gonadal male with incomplete masculinization. Pseudohermaphroditism is not to be confused with hermaphroditism, in which the individual possesses both ovarian and testicular tissue.
References in periodicals archive ?
A (R80Q) mutation in 17 beta-hydroxysteroid dehydrogenase type 3 gene among Arabs of Israel is associated with pseudohermaphroditism in males and normal asymptomatic females.
PMDS represents a small fraction of this broad spectrum of male pseudohermaphroditism, characterized by the presence of well developed or rudimentary uterus, cervix, vagina and fallopian tube in normal 46xy male.
Female pseudohermaphroditism occurs when the genital ducts, external genitalia, and other aspects of phenotypic sex virilize in XX females.
Thus, androgen insensitivity syndrome, for example, was understood more generally as a case of male pseudohermaphroditism.
Male Pseudohermaphroditism Caused by 5alpha-reductase Deficiency in the Simbari Anga of Papua New Guinea.
A syndrome of pseudohermaphroditism, Wilms' tumor, hypertension, and degenerative renal disease.
2004); observed phenotypic sex reversal or pseudohermaphroditism, may result from a variety of environmental factors (Jenner 1979).
On the basis of a study of Paracelsus's remains, we learn, "forensic specialists suggest that Paracelsus was either a genetic male afflicted with pseudohermaphroditism or a genetic female suffering from androecial syndrome" (197).
Intersex conditions such as chromosomal abnormalities, pseudohermaphroditism, and enzyme deficiencies should not be confused with gender identity disorders.
Enlarged clitoris in wild polar bears (Ursus maritimus) can be misdiagnosed as pseudohermaphroditism.
ca 1,2,3,5,6 French materials ACQUIRED IMMUNE DEFICIENCY SYNDROME See: AIDS ACROCEPHALOSYNDACTYLY, TYPE I See: Apert Syndrome ACROFACIAL DYSOSTOSIS, NAGER TYPE See: Nager & Miller Syndromes ACROMEGALY See: Multiple Endocrine Neoplasia I; Pituitary Disorders/Tumors ACTH DEFICIENCY See: Adrenal Disorders ACYL-COA DEHYDROGENASE DEFICIENCY, MEDIUM-CHAIN See: Fatty Oxidation Disorder ADDISON DISEASE See: Adrenal Disorders ADIPOGENITAL-RETINITIS PIGMENTOSA-POLYDACTYLY SYNDROME See: Laurence-Moon-Bardet-Biedl Syndrome ADRENAL CORTEX MALE PSEUDOHERMAPHRODITISM See: Adrenal Disorders ADRENAL DISORDERS National Adrenal Diseases Foundation 505 Northern Blvd.