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Archives of Sexual Behavior

, Volume 38, Issue 5, pp 615–618 | Cite as

The Dilemma of Sex Reassignment in an Adolescent with 17β-HSD-3 Deficiency Raised as a Female: Ten-Year Follow-Up

  • Magda LiakopoulouEmail author
  • Dimitrios Keramydas
  • Maria Dracopoulou
  • Catherine Dacou-Voutetakis
Letter to the Editor

A number of patients with defective differentiation of the genital system, described in the literature by various terminologies (Dacou-Voutetakis, 2007; Hughes et al., 2006), wish to have sex reassignment in adolescence or adulthood.

One of the disorders prompting the request for sex reassignment after childhood is the deficiency of 17β-hydroxysteroid dehydrogenase-3 (17β-HSD-3) caused by mutations in the HSD17B3 gene. The enzyme 17β-HSD-3 is involved in the terminal phase of testicular steroidogenesis and specifically catalyzes the conversion of androstenedione (A) to testosterone (T). Persons with this defect have near-normal or ambiguous female external genitalia at birth due to the absence of testosterone and dihydrotestosterone when the external genitalia differentiate (Gooren, 2002). Individuals with 17β-HSD-3 deficiency are considered as females and are raised as girls. At puberty, however, they become virilized as a result of an increase in T and dihydrotestosterone levels due...

Keywords

Hypospadias Tanner Stage External Genitalia Gender Reassignment HSD17B3 Gene 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

Notes

Acknowledgment

We would like to express our appreciation to Professor H. Morel for the molecular study.

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Copyright information

© Springer Science+Business Media, LLC 2009

Authors and Affiliations

  • Magda Liakopoulou
    • 1
    Email author
  • Dimitrios Keramydas
    • 2
  • Maria Dracopoulou
    • 3
  • Catherine Dacou-Voutetakis
    • 4
  1. 1.Child Psychiatry Department, “Aghia Sophia” Children’s HospitalAthens University School of MedicineAthensGreece
  2. 2.Surgery Department, “Aghia Sophia” Children’s HospitalAthensGreece
  3. 3.Pediatric Endocrinology Department, “Aghia Sophia” Children’s HospitalAthens University School of MedicineAthensGreece
  4. 4.Department of Pediatric EndocrinologyAthens University School of MedicineAthensGreece

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