Fisher A D, Ristori J, Fanni E, Castellini G, Forti G, Maggi M
Sexual Medicine and Andrology Unit, Department of Experimental, Clinical and Biomedical Sciences, University of Florence, Viale Pieraccini 6, 50139, Florence, Italy.
Psychiatric Unit, Department of Neuroscience, Psychology, Drug Research and Child Health, University of Florence, Florence, Italy.
J Endocrinol Invest. 2016 Nov;39(11):1207-1224. doi: 10.1007/s40618-016-0482-0. Epub 2016 Jun 10.
Disorders of Sex Development (DSD) are a wide range of congenital conditions characterized by an incongruence of components involved in sexual differentiation, including gender psychosexual development. The management of such disorders is complex, and one of the most crucial decision is represented by gender assignment. In fact, the primary goal in DSD is to have a gender assignment consistent with the underlying gender identity in order to prevent the distress related to a forthcoming Gender Dysphoria. Historically, gender assignment was based essentially on surgical outcomes, assuming the neutrality of gender identity at birth. This policy has been challenged in the past decade refocusing on the importance of prenatal and postnatal hormonal and genetic influences on psychosexual development.
(1) to update the main psychological and medical issues that surround DSD, in particular regarding gender identity and gender assignment; (2) to report specific clinical recommendations according to the different diagnosis.
A systematic search of published evidence was performed using Medline (from 1972 to March 2016). Review of the relevant literature and recommendations was based on authors' expertise.
A review of gender identity and assignment in DSD is provided as well as clinical recommendations for the management of individuals with DSD.
Given the complexity of this management, DSD individuals and their families need to be supported by a specialized multidisciplinary team, which has been universally recognized as the best practice for intersexual conditions. In case of juvenile GD in DSD, the prescription of gonadotropin-releasing hormone analogues, following the World Professional Association for Transgender Health and the Endocrine Society guidelines, should be considered. It should always be taken into account that every DSD person is unique and has to be treated with individualized care. In this perspective, international registries are crucial to improve the understanding of these challenging conditions and clinical practice, in providing a better prediction of gender identity.
性发育障碍(DSD)是一系列先天性疾病,其特征是参与性分化的各个组成部分之间存在不一致,包括性别心理性发育。此类疾病的管理很复杂,其中最关键的决策之一是性别指定。事实上,DSD的首要目标是进行与潜在性别认同相符的性别指定,以防止因即将出现的性别焦虑症而产生的痛苦。从历史上看,性别指定主要基于手术结果,假定出生时性别认同是中性的。在过去十年中,这一政策受到了挑战,重新关注产前和产后激素及基因对心理性发育的影响。
(1)更新围绕DSD的主要心理和医学问题,特别是关于性别认同和性别指定的问题;(2)根据不同诊断报告具体的临床建议。
使用Medline(从1972年至2016年3月)对已发表的证据进行系统检索。基于作者的专业知识对相关文献和建议进行综述。
提供了对DSD中性别认同和指定的综述以及对DSD个体管理的临床建议。
鉴于这种管理的复杂性,DSD个体及其家庭需要由一个专门的多学科团队提供支持,这已被普遍认为是处理两性畸形情况的最佳做法。对于DSD中的青少年性别焦虑症,应按照世界跨性别健康专业协会和内分泌学会的指南考虑使用促性腺激素释放激素类似物。应始终牢记,每个DSD患者都是独特的,必须给予个性化护理。从这个角度来看,国际登记处对于增进对这些具有挑战性的疾病的了解以及临床实践、更好地预测性别认同至关重要。