pseudohermaphroditism


Also found in: Dictionary, Thesaurus, Encyclopedia, Wikipedia.
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

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.
References in periodicals archive ?
et al Male 40% 59% 52% 19% pseudohermaphroditism True 14% 6% 2% 6% pseudohermaphroditism Female 46% 26% 28% 60% pseudohermaphroditism Gonadal dysgenesis 0% 4% 8% 10% Others 0% 5% 12% 5% DISORDERS OF SEXUAL PRESENT DEVELOPMENT STUDY Male 27% pseudohermaphroditism True 27% pseudohermaphroditism Female 9% pseudohermaphroditism Gonadal dysgenesis 9% Others 27% CHART 1: Phenotypical proportions of disorders of sexual development reviewed in our study.
CAH is the most common cause of female pseudohermaphroditism. It can rarely be a result of virilizing adrenocortical tumours.4
Genetic sex determination, gender identification and pseudohermaphroditism in the knobbed whelk, Busycon carica (Mollusca: Melongenidae).
(21) Type 2 deficiency gives men pseudohermaphroditism with ambiguous external genitalia and no prostate gland.
porcellus when used as experimental models may be useful in those studies on sexual infantilism in women, polycystic ovary syndrome, ambiguous genitalia at birth, pseudohermaphroditism, female genital virilization (Nobrega et al., 2004; Martin et al., 2008; Costenaro et al., 2010; Kalfa et al., 2010) and other studies on genetic syndromes associated with female pseudohermaphroditism, with alterations in genitalia in the female fetus, as clitoromegaly and hypoplasia of labia minora (Arnold et al., 1999) and McKusick-Kaufman syndrome, in which occurs association with hydrometrocolpos and agenesis of the vagina with the presence of the female urogenital sinus (Slavotinek and Biesecker, 2000).
Guerra-Junior, "Idiopathic male pseudohermaphroditism is associated with prenatal growth retardation," European Journal of Pediatrics, vol.
DISCUSSION: Male pseudohermaphroditism (pmds) characterized by presence of uterus, cervix, fallopian tubes in normal 46xy male.
In this case report, our aim is to discuss how disorders with psychotic symptoms may affect different cultural life styles, circumstances, experience, delusion contents of identification and acceptance in a patient formerly diagnosed with DSD with male- pseudohermaphroditism and followed up with the diagnosis of bipolar affective disorder (BAD).
17-[beta]-HSD3 deficiency (OMIM: 264300) is a rare autosomal recessive disorder of male sex differentiation (pseudohermaphroditism) resulting from a defect in the last reversible step of steroidogenesis in testosterone biosynthesis in the testes in which androstenedione is converted into testosterone.
The TBT contributes to the development of antimicrobial tolerance and imposex or pseudohermaphroditism in marine invertebrates [12].
Type III: It is associated with genitourinary anomalies, other than persistent Mullerian duct (13-20%): hypospadias, pseudohermaphroditism, seminal vesicle cyst, scrotal abnormalities, common deferent duct, and horseshoe kidney.