Coulam C B
Obstet Gynecol. 1979 Jan;53(1):44-9.
Recently, the testicular regression syndrome has been reported as being representative of a clinical range of XY agonadal persons. A patient with aberrant fetal sexual development is described. Of particular interest is that all of the tissues examined--the rudimentary uterine horn and the 2 fallopian tubes--displayed an XY chromosome complement. Correlation was made between the known temporal events of embryogenesis of the testes and the apparent time of regression of the testes. In this patient, the insult to testicular development, with subsequent testicular regression, occurred between 48 and 60 days after fertilization. This review supports the previously reported thesis that in certain XY persons the testes are irreparably damaged at a critical stage in fetal development, and that this critical stage is represented by a range of abnormalities of genital development. Possible causes and simplified nomenclature for such gonadal damage are presented.
最近，睾丸退化综合征被报道为XY性腺发育异常人群临床谱系的代表。本文描述了一名胎儿性发育异常的患者。特别值得关注的是，所有检查的组织——残留的子宫角和两条输卵管——均显示为XY染色体组成。研究将已知的睾丸胚胎发生时间事件与睾丸明显的退化时间进行了关联。在该患者中，对睾丸发育的损害以及随后的睾丸退化发生在受精后48至60天之间。本综述支持先前报道的论点，即在某些XY个体中，睾丸在胎儿发育的关键阶段受到不可修复的损害，而这一关键阶段表现为一系列生殖器发育异常。本文还介绍了这种性腺损害的可能原因及简化命名法。
