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睾丸女性化综合征的一种完整形式;光镜和电镜研究

A complete form of testicular feminisation syndrome; a light and electron microscopy study.

作者信息

Justrabo E, Cabanne F, Michiels R, Bastien H, Dusserre P, Pansiot F, Cayot F

出版信息

J Pathol. 1978 Nov;126(3):165-71. doi: 10.1002/path.1711260305.

Abstract

A case is described of testicular feminisation syndrome in a 19-yr-old patient with a normal female appearance. A laparotomy with removal of the gonads was performed. The latter looked like testes with intraparenchymal nodules. Light and electron microscopy study of these nodules revealed the presence of immature seminiferous-like tubules and of Leydig cell clusters. The tubules were filled with clear Sertoli cells, few degenerative dark cells and scanty spermatogonia. Thin collagen bundles surrounded the tubules and the foci of Leydig cells without any crystals of Reinke. The immaturity of all these cellular components could be connected with a mutation at a repressive locus which could be responsible for the abnormalities observed in the different steps of androgen action at target cell level.

摘要

描述了一名19岁外表正常女性的睾丸女性化综合征病例。进行了剖腹手术并切除性腺。性腺看起来像睾丸,有实质内结节。对这些结节进行光镜和电镜研究发现存在未成熟的生精样小管和Leydig细胞簇。小管内充满清亮的支持细胞、少量变性的暗细胞和稀少的精原细胞。细胶原束围绕着小管和Leydig细胞灶,无任何Reinke晶体。所有这些细胞成分的不成熟可能与抑制位点的突变有关,该突变可能是靶细胞水平雄激素作用不同步骤中观察到的异常的原因。

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