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

@article{Krob2005TrueHG,
  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},
  year={2005},
  volume={153},
  pages={2-10}
}
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

TLDR
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

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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

TLDR
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
TLDR
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

TLDR
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

TLDR
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].

TLDR
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
...

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