46,XY性别发育异常的性腺处理  

Gonadal management in 46,XY disorders of sexual development

作　　者：杨屹[1] 殷晓鸣[1] Yang Yi;Yin Xiaoming(Department of Pediatric Urology,Shengjing Hospital,Affiliated to China Medical University,Shenyang 110004,China)

机构地区：[1]中国医科大学附属盛京医院小儿泌尿外科

出　　处：《临床小儿外科杂志》2019年第3期167-171,共5页Journal of Clinical Pediatric Surgery

基　　金：国家自然基金面上项目(编号:81571514)

摘　　要：性分化异常是指先天性染色体、性腺和表型性别的发育异常或不匹配。根据芝加哥会议分型,46,XYDSD是病因最复杂的一类性分化异常。目前46,XYDSD患者的性腺处理仍存在一定争议。本文通过文献复习结合笔者临床经验,从性腺活检及探查、性腺恶变风险、性腺随访策略、性腺切除时机及指征等方面讨论46,XYDSD的性腺处理。Disorders of sex development(DSD)are a generic definition including any problem noted at birth where the genitalia are atypical in relation to chromosomes or gonads.According to the DSD classification of Chicago Conference,46,XY DSD is the most complex disorder of sexual development.Until now,optimal protocol has remained controversial in the gonad management of 46,XY DSD.Based upon a literature review and personal clinical experiences,gonadal management in 46,XY DSD was addressed from the aspects of gonadal biopsy and exploration,gonadal malignancy risks,follow-up strategies,timing and indication of gonadectomy.

关 键 词：性腺发育不全 46 XY/胚胎 性别特征 

分 类 号：R726.9[医药卫生—儿科] R691.1[医药卫生—临床医学]