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Management of disorders of sex development - With a focus on development of the child and adolescent through the pubertal years.
Best Practice & Research Clinical Endocrinology & Metabolism ( IF 7.4 ) Pub Date : 2019-07-27 , DOI: 10.1016/j.beem.2019.101297
Jamal Raza 1 , Syed Zafar Zaidi 2 , Garry L Warne 3
Affiliation  

Disorders of sex development, congenital conditions in which chromosomal, gonadal or anatomic sex is atypical at birth, require urgent assessment by a multidisciplinary team, to define whether there is a life threatening disorder of congenital adrenal hyperplasia or a healthy child with a complex condition. Uncertainty, stigma and taboo complicate counselling which must be knowledgeable, comprehensive and sensitive to different circumstances, religions and cultures. This articles will discuss clinical and genetic diagnosis, decisions regarding sex of rearing, ethical dilemmas, medical management of the infant and of the child or adolescent presenting for the first time with a DSD. Surgical options, timing and management are outlined.

中文翻译:

性发育障碍的管理-重点关注青春期之前儿童和青少年的发育。

性发育障碍,出生时染色体,性腺或解剖性别不典型的先天性疾病,需要多学科小组紧急评估,以确定是否存在危及生命的先天性肾上腺增生性疾病或健康状况复杂的儿童。不确定性,污名和禁忌使咨询变得复杂,咨询必须知识丰富,全面并且对不同的情况,宗教和文化敏感。本文将讨论首次出现DSD的临床和遗传学诊断,有关育性的决定,道德困境,婴儿以及儿童或青少年的医疗管理。概述了手术选择,时机和管理。
更新日期:2019-07-27
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