先天性性别异常的治疗

Treatment of Disorders of Sex Development

先天性性别异常(DSD)包括先天性染色体和性腺发育异常,或解剖性别不典型的疾病,这一名称取代了各种两性畸形的称谓。本研究主要为医务人员对DSD患儿及其家属的治疗、训练和支持提供帮助。DSD治疗的目标是维护患者长期生理、心理及性健康。其中包括为患者提供内外科治疗方法,处理威胁健康的并发症,尽量减少患儿不良感受,如羞耻、被蔑视或过度的为生殖器外观困扰,最好将可以等待的内外科治疗延迟至患儿本人可以积极参与决定的时候,如实告知病情,并缓解患儿及其家属的心理社会压力,从而建立"以病人为中心"的综合治疗。

Disorders of sex development（DSD） include anomalies of sex chromosomes,gonads,reproductive ducts,and genitalia.Here,the term ＂intersex＂ is avoided because of its imprecision.The purpose of this review is to assist health care professionals in the provision of treatment,education,and support to children born with DSD and to their families.The goal of DSD treatment is to achieve the long-term physical,psychological,and sexual well-being of the patients.In the case of DSD it involves several principles.Providing medical and surgical care to deal with a complication that threatens to the patient＇s physical well-being;minimizing the potential for the patient and family to feel ashamed,stigmatized,or overly obsessed with genital appearance;delaying elective surgical and hormonal treatments until the patient can actively participate in decision-making;telling the truth to the family and the child and addressing psychosocial distress of the children and their parents.This approach is termed as ＂patient-centered＂.