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Related Experiment Videos

[True hermaphroditism: experience with 36 patients].

Durval Damiani1, Dulce Rondina Guedes, Daniel Damiani

  • 1Unidade de Endocrinologia Pediátrica, Instituto da Criança, Faculdade de Medicina, Universidade de São Paulo, SP. durvald@iconet.com.br

Arquivos Brasileiros De Endocrinologia E Metabologia
|March 18, 2006
PubMed
Summary

True hermaphrodites (TH) often present with ambiguous genitalia, with most 46,XX individuals being SRY-negative. Female sex of rearing is generally recommended, preserving ovarian tissue for potential normal puberty and fertility.

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Area of Science:

  • Pediatric Endocrinology
  • Genetics
  • Reproductive Medicine

Context:

  • True hermaphroditism (TH) is a rare disorder of sex development.
  • Data from two reference centers in São Paulo, Brazil, were analyzed retrospectively.
  • Patients were followed from 1975 to 2004.

Purpose:

  • To present data on true hermaphrodites (TH) followed in two reference centers.
  • To analyze clinical and pathological data and sex of rearing decisions.

Summary:

  • Genital ambiguity was the most common presentation in 36 TH patients, with a median age of first appointment at 20 months.
  • The 46,XX karyotype was most frequent (47.2%), with SRY negativity in these cases. Ovotestis was the prevalent gonad (47%).
  • Sex of rearing decisions were influenced by urethral meatus location, with female rearing favored for perineal urethras.

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Impact:

  • Findings challenge current understanding of TH, particularly the development of testicular and ovarian tissues in 46,XX SRY-negative individuals.
  • The study suggests female sex of rearing is often most adequate.
  • Preserving ovarian tissue in TH patients may allow for normal puberty and fertility.