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      Psychological Long-Term Outcome in Intersex Conditions

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          Abstract

          For decades, sex assignment in children with intersex conditions has depended more on surgical possibilities than on other criteria, since it was assumed that children are psychosexually neutral at birth. Adults with intersex conditions and professionals in the field have increasingly criticized this policy after the publication of studies suggesting that prenatal brain exposure to sex hormones determines gender development. Although prenatal brain exposure to androgens plays some part in the development of gender role behaviour, the current evidence is not in line with the idea of determination of gender identity through prenatal sex steroid exposure. Recent reviews on gender dysphoria and gender change in patients with intersex conditions show that initial gender assignment still seems to be the best predictor of adult gender identity.

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          Most cited references6

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          Gender change in 46,XY persons with 5alpha-reductase-2 deficiency and 17beta-hydroxysteroid dehydrogenase-3 deficiency.

          Individuals with 5alpha-reductase-2 deficiency (5alpha-RD-2) and 17beta-hydroxysteroid dehydrogenase-3 deficiency (17beta-HSD-3) are often raised as girls. Over the past number of years, this policy has been challenged because many individuals with these conditions develop a male gender identity and make a gender role change after puberty. The findings also raised doubts regarding the hypothesis that children are psychosexually neutral at birth and emphasized the potential role of prenatal brain exposure to androgens in gender development. If prenatal exposure to androgens is a major contributor to gender identity development, one would expect that all, or nearly all, affected individuals, even when raised as girls, would develop a male gender identity and make a gender role switch later in life. However, an estimation of the prevalence of gender role changes, based on the current literature, shows that gender role changes occur frequently, but not invariably. Gender role changes were reported in 56-63% of cases with 5alpha-RD-2 and 39-64% of cases with 17beta-HSD-3 who were raised as girls. The changes were usually made in adolescence and early adulthood. In these two syndromes, the degree of external genital masculinization at birth does not seem to be related to gender role changes in a systematic way.
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            Gender dysphoria and gender change in chromosomal females with congenital adrenal hyperplasia.

            This article reviews the literature on studies and case reports on gender identity and gender identity problems, gender dysphoria, and gender change in chromosomal females with congenital adrenal hyperplasia, raised male or female. The large majority (94.8%) of the patients raised female (N= 250) later developed a gender identity as girls and women and did not feel gender dysphoric. But 13 (5.2%) patients had serious problems with their gender identity. This percentage is higher than the prevalence of female-to-male transsexuals in the general population of chromosomal females. Among patients raised male, serious gender identity problems were reported in 4 (12.1%) out of 33 patients. From these observations, we conclude that the assignment to the female gender as a general policy for 46,XX patients with CAH appears justified, even in severely masculinized 46,XX newborns with CAH (Prader stage IV or V).
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              Gender identity outcome in female-raised 46,XY persons with penile agenesis, cloacal exstrophy of the bladder, or penile ablation.

              This review addresses the long-term gender outcome of gender assignment of persons with intersexuality and related conditions. The gender assignment to female of 46,XY newborns with severe genital abnormalities despite a presumably normal-male prenatal sex-hormone milieu is highly controversial because of variations in assumptions about the role of biological factors in gender identity formation. This article presents a literature review of gender outcome in three pertinent conditions (penile agenesis, cloacal exstrophy of the bladder, and penile ablation) in infancy or early childhood. The findings clearly indicate an increased risk of later patient-initiated gender re-assignment to male after female assignment in infancy or early childhood, but are nevertheless incompatible with the notion of a full determination of core gender identity by prenatal androgens.
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                Author and article information

                Journal
                HRE
                Horm Res Paediatr
                10.1159/issn.1663-2818
                Hormone Research in Paediatrics
                S. Karger AG
                978-3-8055-8009-0
                978-3-318-01274-3
                1663-2818
                1663-2826
                2005
                November 2005
                15 November 2005
                : 64
                : Suppl 2
                : 27-30
                Affiliations
                Department of Medical Psychology, VU University Medical Center, Amsterdam, The Netherlands
                Article
                87750 Horm Res 2005;64:27–30
                10.1159/000087750
                16286767
                6093a6b7-63e7-42e5-88b1-14f9fed53bb7
                © 2005 S. Karger AG, Basel

                Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher. Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug. Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.

                History
                Page count
                Figures: 1, References: 11, Pages: 4
                Categories
                Gender Identity

                Endocrinology & Diabetes,Neurology,Nutrition & Dietetics,Sexual medicine,Internal medicine,Pharmacology & Pharmaceutical medicine
                5α-Reductase-2 deficiency,17β-Hydroxysteroid dehydrogenase-3 deficiency,Intersexuality,Gender dysphoria,Gender identity,Gender change,Gender role change,Androgen insensitivity syndrome,Congenital adrenal hyperplasia

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