Embryonic testicular regression. A clinical spectrum of XY agonadal individuals

C. D. Edman, A. J. Winters, J. C. Porter, J. Wilson, P. C. MacDonald

Research output: Contribution to journalArticle

38 Citations (Scopus)

Abstract

Aberrations of fetal sexual development were studied in 3 XY agonadal individuals. Two were phenotypic females with primary amenorrhea, sexual infantilism, and no internal genital structures. Plasma androstenedione and testosterone concentrations were similar to those of prepubertal children and increased after ACTH infusion. The daily production rates of estrone were low. The 3rd agonadal XY individual, a phenotypic male, developed gynecomastia in response to approx. 40 μg of estrone produced daily by the extraglandular aromatization of plasma androstenedione. All plasma testosterone was accounted for by the conversion of plasma androstenedione. The absence of internal genitalia, the prepubertal external genitalia in the phenotypic females, and the incomplete growth of both the internal and external genital structures in the phenotypic male subject suggest that testicular elaboration of mullerian regression substance occurred, but that regression of the testes occurred subsequently during varying stages of embryogenesis. In the females, testicular regression occurred prior to the initiation of fetal testicular production of androgen. On the other hand, in the male subject, testicular activity persisted longer; thus, both mullerian duct regression and embryonic virilization of wolffian ducts and genital tubercles occurred.

Original languageEnglish (US)
Pages (from-to)208-217
Number of pages10
JournalObstetrics and Gynecology
Volume49
Issue number2
StatePublished - 1977

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Androstenedione
Genitalia
Estrone
Sexual Infantilism
Testosterone
Wolffian Ducts
Mullerian Ducts
Virilism
Gynecomastia
Sexual Development
Amenorrhea
Fetal Development
Adrenocorticotropic Hormone
Androgens
Embryonic Development
Testis
Anorchia
Growth

ASJC Scopus subject areas

  • Obstetrics and Gynecology

Cite this

Edman, C. D., Winters, A. J., Porter, J. C., Wilson, J., & MacDonald, P. C. (1977). Embryonic testicular regression. A clinical spectrum of XY agonadal individuals. Obstetrics and Gynecology, 49(2), 208-217.

Embryonic testicular regression. A clinical spectrum of XY agonadal individuals. / Edman, C. D.; Winters, A. J.; Porter, J. C.; Wilson, J.; MacDonald, P. C.

In: Obstetrics and Gynecology, Vol. 49, No. 2, 1977, p. 208-217.

Research output: Contribution to journalArticle

Edman, CD, Winters, AJ, Porter, JC, Wilson, J & MacDonald, PC 1977, 'Embryonic testicular regression. A clinical spectrum of XY agonadal individuals', Obstetrics and Gynecology, vol. 49, no. 2, pp. 208-217.
Edman CD, Winters AJ, Porter JC, Wilson J, MacDonald PC. Embryonic testicular regression. A clinical spectrum of XY agonadal individuals. Obstetrics and Gynecology. 1977;49(2):208-217.
Edman, C. D. ; Winters, A. J. ; Porter, J. C. ; Wilson, J. ; MacDonald, P. C. / Embryonic testicular regression. A clinical spectrum of XY agonadal individuals. In: Obstetrics and Gynecology. 1977 ; Vol. 49, No. 2. pp. 208-217.
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