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      Controversies on Timing of Sex Assignment and Surgery in Individuals With Disorders of Sex Development: A Perspective

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          Abstract

          Appropriate management of disorders of sex development (DSD) has been a matter of discussion since the first guidelines were published in the 1950s. In the last decade, with the advent of the 2006 consensus, the classical methods, especially regarding timing of surgery and sex of rearing, are being questioned. In our culture, parents of DSD newborns usually want their children to undergo genital surgery as soon as possible after sexual assignment, as surgery helps them to confirm the assigned sex. Developmental psychology theories back this hypothesis. They state that anatomic differences between sexes initiate the very important process of identification with the parent of the same sex. Sex-related endocrinological issues also demand early care. For example, using dihydrotestosterone cream to increase penile length or growth hormone treatment to improve final height require intervention at young ages to obtain better results. Although the timing of surgery remains controversial, recent evidence suggests that male reconstruction should be performed between 6 and 18 months of age. Feminizing surgery is still somewhat controversial. Most guidelines agree that severe virilization requires surgical intervention, while no consensus exists regarding mild cases. Our perspective is that precocious binary sex assignment and early surgery is a better management method. There is no strong evidence for delays and the consequences can be catastrophic in adulthood.

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          Most cited references43

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          Global Disorders of Sex Development Update since 2006: Perceptions, Approach and Care

          The goal of this update regarding the diagnosis and care of persons with disorders of sex development (DSDs) is to address changes in the clinical approach since the 2005 Consensus Conference, since knowledge and viewpoints change. An effort was made to include representatives from a broad perspective including support and advocacy groups. The goal of patient care is focused upon the best possible quality of life (QoL). The field of DSD is continuously developing. An update on the clinical evaluation of infants and older individuals with ambiguous genitalia including perceptions regarding male or female assignment is discussed. Topics include biochemical and genetic assessment, the risk of germ cell tumor development, approaches to psychosocial and psychosexual well-being and an update on support groups. Open and on-going communication with patients and parents must involve full disclosure, with the recognition that, while DSD conditions are life-long, enhancement of the best possible outcome improves QoL. The evolution of diagnosis and care continues, while it is still impossible to predict gender development in an individual case with certainty. Such decisions and decisions regarding surgery during infancy that alters external genital anatomy or removes germ cells continue to carry risk.
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            Caring for individuals with a difference of sex development (DSD): a Consensus Statement

            The term differences of sex development (DSDs; also known as disorders of sex development) refers to a heterogeneous group of congenital conditions affecting human sex determination and differentiation. Several reports highlighting suboptimal physical and psychosexual outcomes in individuals who have a DSD led to a radical revision of nomenclature and management a decade ago. Whereas the resulting recommendations for holistic, multidisciplinary care seem to have been implemented rapidly in specialized paediatric services around the world, adolescents often experience difficulties in finding access to expert adult care and gradually or abruptly cease medical follow-up. Many adults with a DSD have health-related questions that remain unanswered owing to a lack of evidence pertaining to the natural evolution of the various conditions in later life stages. This Consensus Statement, developed by a European multidisciplinary group of experts, including patient representatives, summarizes evidence-based and experience-based recommendations for lifelong care and data collection in individuals with a DSD across ages and highlights clinical research priorities. By doing so, we hope to contribute to improving understanding and management of these conditions by involved medical professionals. In addition, we hope to give impetus to multicentre studies that will shed light on outcomes and comorbidities of DSD conditions across the lifespan.
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              Imprinting and the establishment of gender role.

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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
                10 January 2019
                2018
                : 6
                : 419
                Affiliations
                [1] 1PADS DSD Program, Hospital de Clínicas de Porto Alegre (HCPA) , Porto Alegre, Brazil
                [2] 2Psycology Service, Hospital de Clínicas de Porto Alegre (HCPA) , Porto Alegre, Brazil
                [3] 3Endocrinology Service, Hospital de Clínicas de Porto Alegre (HCPA) , Porto Alegre, Brazil
                [4] 4Pediatrics Department, School of Medicine, Universidade Federal do Rio Grande do Sul (UFRGS) , Porto Alegre, Brazil
                [5] 5Medical Genetics Service, School of Medicine, Universidade Federal do Rio Grande do Sul (UFRGS) , Porto Alegre, Brazil
                [6] 6Pediatric Surgery Service, Hospital de Clínicas de Porto Alegre (HCPA) , Porto Alegre, Brazil
                Author notes

                Edited by: Luis Henrique Braga, McMaster University, Canada

                Reviewed by: Gundela Holmdahl, Queen Silvia Children's Hospital, Sweden; Maximilian Stehr, Klinik Hallerwiese und Cnopfsche Kinderkrankenhaus, Germany

                *Correspondence: Eduardo Corrêa Costa eccosta@ 123456hcpa.edu.br

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

                Article
                10.3389/fped.2018.00419
                6335325
                ee66b79c-b71f-4cab-b4f4-2ba2cdc6cbe4
                Copyright © 2019 Hemesath, de Paula, Carvalho, Leite, Guaragna-Filho and Costa.

                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) and the copyright owner(s) 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.

                History
                : 10 September 2018
                : 18 December 2018
                Page count
                Figures: 0, Tables: 0, Equations: 0, References: 48, Pages: 6, Words: 4921
                Categories
                Pediatrics
                Perspective

                disorders of sex development,sex assignment,surgery,timing,psychosocial care,gender identity

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