Systematization of ambiguous genitalia.

IF 1.6 4区 生物学 Q4 BIOCHEMISTRY & MOLECULAR BIOLOGY Organogenesis Pub Date : 2016-10-01 Epub Date: 2016-07-08 DOI:10.1080/15476278.2016.1210749
Zograb Makiyan
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引用次数: 13

Abstract

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.

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模糊生殖器的系统化。
新生儿的性别分配取决于外生殖器的解剖结构,尽管这一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,但间充质细胞分布在整个胚体中,没有特异性的雄激素受体。目前尚不清楚引起生殖器结节早期分化的信号的性质。本文的目的是提高对阴阳人疾病的外科治疗和探讨生殖器结节的发展。114例不同形式DSD女性患者的临床检查发现73例患者外生殖器模糊(双性),其中51例接受了女性化手术。46例XY患者的中间性(不明确)是由于性类固醇激素或受体通路的干扰所致;46例中,XX例女性因雄激素水平过高而发病。系统化的双性疾病区分核型,性腺形态和生殖器解剖,以提供鉴别诊断和指导适当的手术处理。改良女性化阴蒂成形术,保留背侧和腹侧神经血管束,以保留性敏感,适用于严重男性化的女性(Prader度III-V)。生殖器结节的生长和尿道褶皱的融合以有序的方式进行;但在某些模棱两可的情况下,由于不同的路径,存在不一致。关于生殖器结节起源的推测已与文献综述进行了讨论。生殖器结节由以下三层组成:结节的外胚层龟头、中胚层海绵体和内胚层泌尿生殖沟。根据新的假设,在不同的阶段,5个骶节必须从它们的分割中退缩并解体:硬核组形成骨盆骨,融合肌组与它们真正的神经组一起,血管组与生殖器结节的海绵体连接。外生殖器的性别分化是性别胚胎发生的最后阶段,但令人惊讶的是,在性腺和内脏器官发育之前,就已经从5个小体中衍生出了冷漠的生殖器结节。
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来源期刊
Organogenesis
Organogenesis BIOCHEMISTRY & MOLECULAR BIOLOGY-DEVELOPMENTAL BIOLOGY
CiteScore
4.10
自引率
4.30%
发文量
6
审稿时长
>12 weeks
期刊介绍: Organogenesis is a peer-reviewed journal, available in print and online, that publishes significant advances on all aspects of organ development. The journal covers organogenesis in all multi-cellular organisms and also includes research into tissue engineering, artificial organs and organ substitutes. The overriding criteria for publication in Organogenesis are originality, scientific merit and general interest. The audience of the journal consists primarily of researchers and advanced students of anatomy, developmental biology and tissue engineering. The emphasis of the journal is on experimental papers (full-length and brief communications), but it will also publish reviews, hypotheses and commentaries. The Editors encourage the submission of addenda, which are essentially auto-commentaries on significant research recently published elsewhere with additional insights, new interpretations or speculations on a relevant topic. If you have interesting data or an original hypothesis about organ development or artificial organs, please send a pre-submission inquiry to the Editor-in-Chief. You will normally receive a reply within days. All manuscripts will be subjected to peer review, and accepted manuscripts will be posted to the electronic site of the journal immediately and will appear in print at the earliest opportunity thereafter.
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