Post-pubertal female psychosexual orientation in incomplete male pseudohermaphroditism type 2 (5 alpha-reductase deficiency).
Post-pubertal female psychosexual orientation in incomplete male pseudohermaphroditism type 2 (5 alpha-reductase deficiency).
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不完全男性假两性畸形 2 型（5 α 还原酶缺乏症）中青春期后女性性心理取向。
DOI：
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发表时间：
1980
期刊：
影响因子：
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通讯作者：
A. Hernández
中科院分区：
文献类型：
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作者：
J. Cantú;E. Corona‐Rivera;M. Díaz;C. Medina;E. Esquinca;V. Cortes;G. Vaca;A. Hernández
研究了一名18岁46，XY女性抚养的患有不完全男性假两性畸形2型（5 α-还原酶缺乏症）的患者。她有男性的体型，沃尔夫管衍生物，正常的睾丸和小阴茎;没有苗勒管衍生物，也没有男性乳房。临床和遗传学数据是诊断的典型依据，内分泌学研究证实了这一点。hCG给药前后LH、FSH、睾酮（T）和雌二醇均正常，双氢睾酮（DHT）血浆水平降低; T：DHT比值显著升高。睾丸活检的组织病理学研究显示正常的成年男性模式，减数分裂染色体被解释为正常。在评估她的性心理取向后，成功地进行了手术和药物治疗，以维持和改善她的女性性。青春期后的性别角色转换通常观察到这些女性抚养的患者进行了讨论。
An 18 year-old 46,XY female-reared patient with incomplete male pseudohermaphroditism type 2 (5 alpha-reductase deficiency) was studied. She had a male habitus, Wolffian ducts derivatives, normal testes and small phallus; there were no Mullerian duct derivatives nor gynaecomastia. Clinical and genetic data were typical of the diagnosis which was corroborated by endocrinological studies. Normal LH, FSH, testosterone (T) and oestradiol and decreased dihydrotestosterone (DHT) plasma levels before and after hCG administration were found; the T:DHT ratio was highly increased. The histopathological studies of a testis biopsy showed a normal adult male pattern, and the meiotic chromosomes were interpreted as normal. After assessment of her psychosexual orientation, successful surgical and medical therapy to maintain and improve her femaleness was effectuated. The post-pubertal gender role switch commonly observed in these female-reared patients is discussed.