True hermaphroditism: Geographical distribution, clinical findings, chromosomes and gonadal histology

  title={True hermaphroditism: Geographical distribution, clinical findings, chromosomes and gonadal histology},
  author={G. Krob and Andreas Braun and Ursula Kuhnle},
  journal={European Journal of Pediatrics},
Key MethodWe reviewed 283 cases of human true hermaphroditism published from 1980 to 1992. Of the 96 cases described in Africa 96.9% showed a 46,XX karyotype. In Europe 40.5% of 74 cases and 21.0% of the patients in North America had chromosomal mosaicism. The 46,XY karyotype is extremely rare (7%) and equally distributed through Asia, Europe and North America. Of 283 cases 87 were of black or black mixed origin with a 46,XX chromosomal constellation. The most common gonad in patients with true…

The aetiology of XX true hermaphroditism in the southern African black population

Four different hypotheses were tested in an attempt to unravel the molecular basis of XXTH in South African blacks and it is believed that mosaicism or chimerism with a Y bearing cell line in the gonads of true hermaphrodites may be responsible for the presence of testicular tissue.

Genetic analysis in a true hermaphrodite: A case report

Though phallic size is the single most important criteria for gender assessment, a multidisciplinary team of specialists in consultation with parents must assign sex of the child on basis of genitalia, gonads and genetic factors.

Ovotesticular disorder of sexual development and a rare 46,XX/47,XXY karyotype

The case of a boy with a 46,XX/47,XXY karyotype diagnosed as ovotesticular DSD by gonadal biopsy is reported, highlighting the importance of histological and cytogenetic investigation in DSD.

True hermaphroditism in southern Africa: the clinical picture

  • R. Wiersma
  • Medicine
    Pediatric Surgery International
  • 2004
The previously reported high incidence (51%) of this condition, as well as some of the unusual features of true hermaphroditism in this region are highlighted.

True Hermaphroditism in a Phenotypic Male without Ambiguous Genitalia: An Unusual Presentation at Puberty

The case of an adolescent boy with true hermaphroditism who presented with normal external genitalia and no sexual ambiguity and was referred due to progressive gynecomastia and arrest of puberty is reported.

Ovotesticular disorder of sex development with unusual karyotype: patient report

A thorough revision of literature revealed a single case of OT-DSD with the same chromosome constitution, which is a rare cause of sex ambiguity and Gonadal biopsy is necessary in cases of sex chromosome mosaicism.

[True hermaphroditism: experience with 36 patients].

The female sex of rearing seems to be the most adequate, and it is important to try to preserve, in these cases, the ovarian portion of the ovotestis, allowing the patient to have normal puberty and fertility.

True Hermaphroditism with Characteristics of Klinefelter's Syndrome: A Rare Presentation

True hermaphroditism, a very rare cause of intersex, is usually diagnosed during the newborn period in the course of evaluating ambiguous genitalia. As an exception we report an unusual case of a



Is true hermaphroditism a primary germ cell disorder?

Eleven cases of true hermaphroditism, 6 raised as female and 5 as male, were seen in the 20-year period from 1965 to 1985, with a marked variation in macroscopic and microscopic appearances with five patterns of distribution of gonadal tissue distribution.

A possible common origin of “Y-negative” human XX males and XX true hermaphrodites

It is postulate that 46, XX males with AG and 46,XX TH may represent altenative manifestations of the same genetic defect and an autosomally (or pseudoautosomally) determined mechanism is suggested.

Early gender assignment in true hermaphroditism.

This classification, based on the appearance of the external genitalia, allows a simple screening and prompt gender assignment in infants identified as true hermaphrodites.

True Hermaphroditism with XX Chromosomes

The aim of this paper is to present the clinical features, chromosomal analysis, hormonal assays and histopathological results in an adolescent hermaphrodite who chose the male sex.

XX true hermaphroditism in southern African blacks: an enigma of primary sexual differentiation.

The aim of the study on 11 families with histologically proven XX true hermaphroditism was to determine whether a common genetic or environmental etiology could be identified and whether a constant environmental factors could be implicated.


A true hermaphrodite with ambiguous genitalia and 46XX karyotype was studied from 18 months of age to 17 years, finding the relative peripheral insensitivity to testosterone, is, in some ways, inconsistent with the presence of receptors for androgens.

True hermaphrodite with bilateral ovotestes, bilateral gonadoblastomas and dysgerminomas, 46, XX/46, XY karyotype, and a successful pregnancy

The first case (to the authors' knowledge) is reported of a true hermaphrodite with bilateral ovotestes, bilateral gonadoblastomas and dysgerminomas, a 46, XX/46,XY karyotype, and a successful

[True hermaphroditism. Late diagnosis. Surgical treatment and a 15-year follow-up].

The benefice of a such therapeutic option in the true hermaphroditism lately diagnosed recording to organic and psychological data is discussed and the difficulty in therapeutic choice is pointed out, mainly when the patient has raised as a boy.

The gonads of human true hermaphrodites

A detailed description of the morphology of ovotestis, testis and ovary in the true hermaphrodite is given and a continuum from very little ovarian tissue to a small portíon of testicular tissue is shown.

[46, XX true hermaphroditism: a case report with a review of 37 cases reported in Japan].

The patient was a 17-year-old male who had undergone chordectomy at the age of 5 years and received gonadectomy, hysterectomy and implantation of thesis prosthesis into his scrotum with successful results concerning his secondary sexual characters.