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Androgen-insensitive male pseudohermaphroditism.
Birth Defects Orig Artic Ser. 1971 May; 7(6):179-84.BD

Abstract

Androgen insensitivity is a mendelian trait transmitted through phenotypically normal females. The affected males exhibit normal fetal testicular development and regression of the müllerian ducts. A luminized uterus is never present. External genitalia are feminine or malformed. The vagina, found in most cases, ends blindly. Female breasts are present in adults. The following three syndromes can be distinguished which differ in the character of the external genitalia: 1) Complete testicular feminization with normal female external genitalia: 2) Incomplete testicular feminization with ambiguous external genitalia showing an enlarged clitoris or phallus; 3) Testicular feminization with hypospadias (androgen-insensitive Reifenstein syndrome). External genitalia are hypospadic, exhibiting scrotal development. The presence of immature Sertoli cells in testicular tubules, in spite of hyperplastic and hypertrophic Leydig cells, is considered to be a pathognomonic histopathologic feature for androgen-insensitivity syndromes. The Leydig cells are able to produce androgens, but androgen-insensitive Sertoli cells are unable to mature.

Authors

Jirásek JE
No affiliation info available

MeSH

AdultAndrogen-Insensitivity SyndromeAndrogensChildDisorders of Sex DevelopmentFollicle Stimulating HormoneGenotypeGynecomastiaHumansHypospadiasLeydig CellsMalePhenotypeSyndromeTestis

Pub Type(s)

Journal Article

Language

eng

PubMed ID

5173159

Citation

Jirásek, J E.. "Androgen-insensitive Male Pseudohermaphroditism." Birth Defects Original Article Series, vol. 7, no. 6, 1971, pp. 179-84.
Jirásek JE. Androgen-insensitive male pseudohermaphroditism. Birth Defects Orig Artic Ser. 1971;7(6):179-84.
Jirásek, J. E. (1971). Androgen-insensitive male pseudohermaphroditism. Birth Defects Original Article Series, 7(6), 179-84.
Jirásek JE. Androgen-insensitive Male Pseudohermaphroditism. Birth Defects Orig Artic Ser. 1971;7(6):179-84. PubMed PMID: 5173159.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Androgen-insensitive male pseudohermaphroditism. A1 - Jirásek,J E, PY - 1971/5/1/pubmed PY - 1971/5/1/medline PY - 1971/5/1/entrez SP - 179 EP - 84 JF - Birth defects original article series JO - Birth Defects Orig Artic Ser VL - 7 IS - 6 N2 - Androgen insensitivity is a mendelian trait transmitted through phenotypically normal females. The affected males exhibit normal fetal testicular development and regression of the müllerian ducts. A luminized uterus is never present. External genitalia are feminine or malformed. The vagina, found in most cases, ends blindly. Female breasts are present in adults. The following three syndromes can be distinguished which differ in the character of the external genitalia: 1) Complete testicular feminization with normal female external genitalia: 2) Incomplete testicular feminization with ambiguous external genitalia showing an enlarged clitoris or phallus; 3) Testicular feminization with hypospadias (androgen-insensitive Reifenstein syndrome). External genitalia are hypospadic, exhibiting scrotal development. The presence of immature Sertoli cells in testicular tubules, in spite of hyperplastic and hypertrophic Leydig cells, is considered to be a pathognomonic histopathologic feature for androgen-insensitivity syndromes. The Leydig cells are able to produce androgens, but androgen-insensitive Sertoli cells are unable to mature. SN - 0547-6844 UR - https://www.unboundmedicine.com/medline/citation/5173159/Androgen_insensitive_male_pseudohermaphroditism_ L2 - http://www.diseaseinfosearch.org/result/6028 DB - PRIME DP - Unbound Medicine ER -