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Systematization of ambiguous genitalia.
Organogenesis ( IF 2.3 ) Pub Date : 2016-07-27 , DOI: 10.1080/15476278.2016.1210749
Zograb Makiyan 1
Affiliation  

Sex assignment in newborns depends on the anatomy of the external genitalia, despite this stage being the final in embryogenesis. According to the current view, the genital tubercle is the embryonic precursor of penis and clitoris. It originates from mesenchymal tissue, but mesenchymal cells are arranged across the embryonal body and do not have specific androgen receptors. The nature of the signal that initiates early derivation of the indifferent genital tubercle is unknown at present. The aims of this article are to improve surgical management of intersex disorders and investigate the development of the genital tubercle. Clinical examination of 114 females with various forms of DSD revealed ambiguous (bisexual) external genitalia in 73 patients, and 51 of them underwent feminizing surgery. Intersexuality (ambiguity) in 46,XY patients results from disruptors in the pathways of sex steroid hormones or receptors; in 46,XX females arises from excessive levels of androgens. Systematization of intersex disorders distinguishes the karyotype, gonadal morphology, and genital anatomy to provide a differential diagnosis and guide appropriate surgical management. Modified feminizing clitoroplasty with preservation of the dorsal and ventral neurovascular bundles to retain erogenous sensitivity was performed in females with severe virilization (Prader degree III-V). The outgrowth of the genital tubercle and the fusion of the urethral fold proceed in an ordered fashion; but in some cases of ambiguity, there was discordance due to different pathways. Speculation about the derivation of the genital tubercle have discussed with a literature review. The genital tubercle derives from the following 3 layers: the ectodermal glans of the tubercle, the mesodermal corpora cavernosa and the endodermal urogenital groove. According to the new hypothesis, during the indifferent stages, the 5 sacral somites have to recede from their segmentation and disintegrate: the sclerotomes form the pelvic bones, the fused myotomes follow with their genuine neurotomes and the angiotomes join to the corpora cavernosa of the genital tubercle. Sexual differentiation of external genitalia is final in gender embryogenesis, but surprisingly derivation of the indifferent genital tubercle from 5 somites occurs before gonadal and internal organs development.



中文翻译:

生殖器模棱两可的系统化。

新生儿的性别分配取决于外部生殖器的解剖结构,尽管这个阶段是胚胎发生的最后阶段。根据目前的观点,生殖器结节是阴茎和阴蒂的胚胎前体。它起源于间充质组织,但间充质细胞排列在整个胚胎体内,没有特定的雄激素受体。目前尚不清楚启动无差异生殖器结节的早期衍生的信号的性质。本文的目的是改善两性失调的外科治疗,并研究生殖器结节的发展。对114例患有各种DSD形式的女性进行的临床检查显示,有73例患者的外生殖器模棱两可(双性恋),其中51例进行了女性化手术。46岁时的两性恋(含糊不清),XY患者是由性类固醇激素或受体途径的破坏引起的;在46,XX名女性中,雄激素含量过多。两性疾病的系统化可区分核型,性腺形态和生殖器解剖结构,以提供鉴别诊断并指导适当的外科治疗。改良女性化阴蒂成形术保留了背侧和腹侧神经血管束,以保持性敏感性,在严重男性化的女性中进行(Prader等级为III-V)。生殖器结节的向外生长和尿道皱褶的融合以有序的方式进行。但在某些歧义情况下,由于途径不同而存在不一致。关于生殖器结节衍生的推测已与文献综述进行了讨论。生殖器结节来自以下3个层:结节的外胚层龟头,中胚层海绵体和内胚层泌尿生殖道。根据新的假设,在无差异的阶段中,五个节必须退出其节段并崩解:巩膜形成骨盆骨,融合的肌层紧随其后的是真正的神经切片,而血管切片则连接到生殖器的海绵体结节。外部生殖器的性别分化在性别胚胎发生中是最终的,但是令人惊讶的是,从5个母体中衍生出无差异的生殖器结核发生在性腺和内部器官发育之前。在不同的阶段,the骨的5个节必须从其节段中退缩并崩解:巩膜形成骨盆的骨骼,融合的肌变体随后是真正的神经切面,而血管切面则连接到生殖器结节的海绵体。外部生殖器的性别分化在性别胚胎发生中是最终的,但是令人惊讶的是,从5个母体中衍生出无差异的生殖器结核发生在性腺和内部器官发育之前。在不同的阶段,the骨的5个节必须从其节段中退缩并崩解:巩膜形成骨盆的骨骼,融合的肌变体随后是真正的神经切面,而血管切面则连接到生殖器结节的海绵体。外部生殖器的性别分化在性别胚胎发生中是最终的,但是令人惊讶的是,从5个母体中衍生出无差异的生殖器结核发生在性腺和内部器官发育之前。

更新日期:2016-07-27
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