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True hermaphroditism and mixed gonadal dysgenesis in young children: a clinicopathologic study of 10 cases.
幼儿真两性畸形和混合性性腺发育不全: 10 例临床病理研究。

摘要

True hermaphroditism (TH) refers to individuals who have both unequivocal ovarian tissue and testicular elements regardless of their karyotypes; whereas mixed gonadal dysgenesis (MGD) refers to individuals who usually have a differentiated gonad on one side and a streak gonad or streak testis on the other side. A differential diagnosis between the TH and MGD has important clinical implications for gender assignment and the decision for early gonadectomy; however, variable clinical and histological features frequently lead to the confusion of TH with MGD. We reviewed the clinicopathological features of TH (n = 4) and MGD (n = 6) in young children to identify which morphological features are important for a differential diagnosis between the two conditions. In both conditions, the testicular compartment was composed of immature seminiferous tubules lined by immature Sertoli cells and primitive germ cells; this finding was not helpful for a differential diagnosis. The ovarian compartment in TH cases showed numerous primordial follicles containing primary oocytes with a few primary or antral follicles; however, ovarian compartments in patients with MGD were characterized by primitive sex-cordlike structures with or without germ cell components within the ovarian-type stroma, mimicking gonadoblastomas in two cases and granulosa cell or Sertoli cell tumors in three cases. Hormonal profiles, cytogenetic results, and an internal duct system were not helpful in a differential diagnosis. In conclusion, a differential diagnosis between TH and MGD is largely dependent on the histological features of the gonads. Therefore, examination of all resected or biopsied tissue and the application of strict histological criteria are important.

译文

真正的两性畸形 (TH) 是指具有明确的卵巢组织和睾丸成分的个体,无论其染色体组型如何; 而混合性腺发育不全 (MGD) 指通常一侧有分化的性腺,另一侧有条纹性腺或条纹睾丸的个体。TH 和 MGD 之间的鉴别诊断对性别分配和早期性腺切除术的决定有重要的临床意义; 然而,不同的临床和组织学特征经常导致 TH 和 MGD 的混淆。我们回顾了儿童 TH (n = 4) 和 MGD (n = 6) 的临床病理特征,以确定哪些形态学特征对这两种情况的鉴别诊断很重要。在这两种情况下,睾丸隔室由不成熟的生精小管组成,这些生精小管由不成熟的支持细胞和原始生殖细胞排列; 这一发现对鉴别诊断没有帮助。TH 病例的卵巢隔间显示有许多原始卵泡,其中包含初级卵母细胞和少数初级或窦状卵泡; 然而, MGD 患者的卵巢隔间的特征是在卵巢类型基质中有或没有生殖细胞成分的原始性索样结构,模拟性腺母细胞瘤 2 例,颗粒细胞或支持细胞肿瘤 3 例。激素谱、细胞遗传学结果和内部导管系统对鉴别诊断没有帮助。总之,TH 和 MGD 之间的鉴别诊断很大程度上取决于性腺的组织学特征。因此,所有切除或活检组织的检查和严格的组织学标准的应用是重要的。

hermaphroditism

妇产 生殖器官发育异常 疾病
概述  :  

两性畸形为胚胎和胎儿暴露于过高或不足雄激素刺激所致。男女生物学性别可根据性染色体、生殖腺结构、外生殖器形态以及第二性征加以区分。但有些患者生殖器官同时具有某些男女两性特征,称为两性畸形(hermaphroditism)。其为先天性生殖器发育畸形的一种特殊类型,可能对患儿的抚育、心理及未来的生活、工作和婚姻等带来诸多困扰,必须及早诊断和处理。矫正治疗方案取决于社会性别、本人性别自认和畸形程度。对于未分化或异常嵌合的生殖腺应尽早切除,以防癌变。   分

hermaphroditism    [hɝ'mæfrədaɪ,tɪzm] 

释    义    n. [动] 雌雄同体性  [遗] 两性畸形

同根词    hermaphrodite 

adj. hermaphrodite [动] 雌雄同体的 hermaphroditic 两性的;雌雄同体的 

n. hermaphrodite [动] 雌雄同体;两性体;[植] 雌雄同株

例    句    About 2% of the same year self-crossing crucian carp are hermaphroditism. 同一年自交繁殖的鱼群中发现约有2%的雌雄同体。

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