"True agonadism": A misnomer?

Gary A. Parks, Kenneth W. Dumars, George A. Limbeck, W. Leslie Quinlivan, Maria I. New

Research output: Contribution to journalArticlepeer-review

22 Scopus citations

Abstract

A 3 1/2-year-old "girl" whose condition had been diagnosed as "true agonadism" underwent endocrine evaluation, which suggested the presence of Leydig cells. The child was considered to be female, although born with mild phallic enlargement and posterior labial fusion. At one year of age, evaluation revealed a, 46,XY karyotype; at exploratory laparotomy, no gonads, uterus, Fallopian tubes, or Wolffian remnants were observed. Base-line gonadotropins (FSH, 0.72 ng.per milliliter; LH, 1.9 ng. per milliliter), were not elevated and a significant rise in plasma testosterone (63 to 169 ng. per 100 ml.) followed stimulation with HCG; both features suggested the presence of testosterone-secreting Leydig cells. The obsence of Müllerian anlagen suggests that at some time during fetal development Müllerian-inhibiting factor was secreted by testes. "True agonadism" is probably a misnomer and actually represents that form of male pseudohermaphroditism with a scant amount of functioning testicular tissue present.

Original languageEnglish
Pages (from-to)375-380
Number of pages6
JournalJournal of Pediatrics
Volume84
Issue number3
DOIs
StatePublished - Mar 1974
Externally publishedYes

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