True hermaphroditism with 46, XX/46, XY chromosome complement: Report of a case

In Joo Park, Howard W. Jones, Wilma B. Bias

Research output: Contribution to journalArticle

Abstract

A true hermaphrodite with 46, XX/46, XY chromosome complement and with evidence of haptoglobin chinierism suggesting double fertilization is reported. Ten additional case reports of true hermaphroditism with 46, XX/46, XY genotypes from the literature are reviewed. The phenotypic features of such patients indicate that embryonic testes, or ovotcstes, arc less competent than normal testes in miillcrian duct suppression and masculinization of external genitalia. The uterus and vagina arc present in a majority of such patients and external genitalia arc ambiguous in all of them. The ovary or ovotestis in these patients behaves like a normal ovary as far as development of tubes and breasts is concerned.

Original languageEnglish (US)
Pages (from-to)377-387
Number of pages11
JournalObstetrics and gynecology
Volume36
Issue number3
StatePublished - Sep 1970

ASJC Scopus subject areas

  • Obstetrics and Gynecology

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