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      Congenital Adrenal Hyperplasia: Review from a Surgeon’s Perspective in the Beginning of the Twenty-First Century

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          Abstract

          Congenital adrenal hyperplasia (CAH) most commonly due to 21-hydroxylase deficiency is the most common type of disorder of sex development. This review will focus on CAH addressing historical and current surgical techniques with their anatomical foundations, with special attention to long-term results and outcomes on sexual function, patient satisfaction, patient attitude toward surgery, and ongoing controversies in management of these patients.

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          Most cited references 52

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          Consensus statement on management of intersex disorders.

           P Lee,  James Houk,   (2006)
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            Psychosexual development of women with congenital adrenal hyperplasia.

            Women with congenital adrenal hyperplasia (CAH) (N = 31) and their unaffected sisters or female cousins (N = 15) participated in a study of psychosexual development. All participants were > or = 18 years of age (mean age, 25 years; range, 18-40). Comparisons were also made between the CAH women with the salt-wasting (SW) form of the disorder and those with simple virilization (SV). A psychosexual assessment protocol examined six variables: (1) sex assignment at birth (probands only); (2) recalled sex-typed behavior during childhood; (3) gender identity and gender role identification in adulthood; (4) relationship status; (5) sexual orientation in fantasy; and (6) sexual orientation in behavior. Salt-wasting status and sex assignment at birth were also ascertained for the CAH women who either refused to participate in the study (N = 10) or could not be traced (N = 13). Compared to the controls, the women with CAH recalled more cross-gender role behavior and less comfort with their sense of "femininity" during childhood. The two groups did not differ in degree of gender dysphoria in adulthood, although the probands showed more cross-gender role identification. Three of the nonparticipant probands were living, as adults, in the male social role (2 reared from birth as boys and 1 who changed from the female to the male social role during adolescence). The CAH women and the controls did not differ in relationship status (married/cohabiting vs. single). The CAH women had lower rates of exclusive heterosexual fantasy and fewer sexual experiences with men than the controls; however, the CAH women did not have more sexual experiences with women than the controls. Comparisons between the SW and SV revealed several differences: the SW were less likely to be assigned to the female sex at birth, recalled more cross-gender role behavior during childhood, were less likely to be married or cohabiting, and had lower rates of sexual experiences with men. The results were discussed in relation to the effects of prenatal androgens on psychosexual differentiation.
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              The effect of clitoral surgery on sexual outcome in individuals who have intersex conditions with ambiguous genitalia: a cross-sectional study.

              The effects on sexual function of surgical removal of parts of the clitoris are unknown. For infants with intersex conditions and ambiguous genitalia being raised female, this surgery is often undertaken in early childhood. Our aim was to assess the effects of surgery on sexual outcome in this population. We did a cross-sectional study to which we recruited 39 adults who had intersex conditions with ambiguous genitalia who were living as female from clinical (n=15) and peer-support (n=24) settings. We obtained data by use of a postal questionnaire, incorporating a validated sexual function assessment inventory. We also obtained hospital notes of 36 respondents who did not want to remain anonymous, and did genital examinations of 19 women. We assessed sexual problems in relation to surgical history and compared the results for our population to those of a healthy control group. Of the 39 individuals enrolled, 28 had been sexually active and all had sexual difficulties. The 18 women who had undergone clitoral surgery had higher rates of non-sensuality (78%) and of inability to achieve orgasm (39%) than did the ten who had not had surgery (20% [p=0.002] and 0% [p=0.03], respectively). Sexual function could be compromised by clitoral surgery. Debate on the ethics of the use of this surgery in children should be promoted and further multicentre research is needed to ensure representative samples and comprehensive outcome assessment. Meanwhile, parents and patients who consent to clitoral surgery should be fully informed of the potential risks to sexual function.
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                Author and article information

                Contributors
                Journal
                Front Pediatr
                Front Pediatr
                Front. Pediatr.
                Frontiers in Pediatrics
                Frontiers Media S.A.
                2296-2360
                23 October 2013
                02 January 2014
                2013
                : 1
                Affiliations
                1Universidad Nacional Del Sur, Abordaje Quirúrgico de las Enfermedades, Cirugía y Urologia Infantil , Bahía Blanca, Argentina
                2Hospital IGA Dr. J. Penna, Pediatría, Cirugía Infantil , Bahía Blanca, Argentina
                Author notes

                Edited by: Qiangsong Tong, Union Hospital of Tongji Medical College, China

                Reviewed by: Yuk Him Tam, The Chinese University of Hong Kong, China; Chaohui Gu, Zhengzhou University, China

                *Correspondence: Lisandro Ariel Piaggio, Departamento de Cirugía, Facultad De Medicina, Universidad Nacional del Sur. Belgrano 323 4°A. CP 8000, Bahía Blanca, BA, Argentina e-mail: lpiaggio@ 123456yahoo.com

                This article was submitted to Pediatric Urology, a section of the journal Frontiers in Pediatrics.

                Article
                10.3389/fped.2013.00050
                3877834
                Copyright © 2014 Piaggio.

                This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

                Page count
                Figures: 0, Tables: 1, Equations: 0, References: 55, Pages: 7, Words: 6346
                Categories
                Pediatrics
                Review Article

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