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真两性畸形:诊断与外科治疗

True hermaphroditism: diagnosis and surgical treatment.

作者信息

Guaschino S, Stola E, Spinillo A, Marchetti M, Aguzzi A

机构信息

Institute of Obstetrics and Gynecology, School of Medicine, University of Pavia, Italy.

出版信息

Clin Exp Obstet Gynecol. 1988;15(3):74-9.

PMID:3402087
Abstract

We documented a new case of 46,XY true hermaphroditism in an 18-year-old patient, reared as a female at birth, with ambiguous genitalia and primary amenorrhea. At laparotomy, bilateral dysgenetic ovotestis containing gonadoblastoma were found. Karyotypes of peripheral lymphocytes and cells from tissue of both gonads were all 46,XY. Plastic surgery was conducted to transform ambiguous genitalia to the full female phenotype. Clitoreduction by glans resection and fastening of the corpora cavernosa in the labia major with enlargement of the vaginal orifice were performed. The results was a small eretcile organ with preserved sensitivity and a female capable of normal sexual activity.

摘要

我们记录了一例18岁46,XY真两性畸形的新病例,该患者出生时被当作女性抚养,存在生殖器模糊和原发性闭经。剖腹探查时,发现双侧发育不全的卵睾伴有性腺母细胞瘤。外周血淋巴细胞以及双侧性腺组织细胞的核型均为46,XY。进行了整形手术,将模糊的生殖器转变为完全女性表型。通过切除阴蒂头进行阴蒂缩小,并将阴茎海绵体固定于大阴唇,同时扩大阴道口。结果形成了一个具有保留感觉的小勃起器官,并且该女性能够进行正常的性活动。

相似文献

1
True hermaphroditism: diagnosis and surgical treatment.真两性畸形:诊断与外科治疗
Clin Exp Obstet Gynecol. 1988;15(3):74-9.
2
Production of testosterone and estrogen in vitro by gonadal tissue from a 46,XY true hermaphrodite with gonadal failure and gonadoblastoma.一名患有性腺功能衰竭和性腺母细胞瘤的46,XY真两性畸形患者的性腺组织在体外产生睾酮和雌激素。
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3
[Causes of ambiguous external genitalia in neonates].[新生儿外生殖器模糊的原因]
Srp Arh Celok Lek. 2001 Mar-Apr;129(3-4):57-60.
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Familial 46,XY pure gonadal dysgenesis and gonadoblastoma/dysgerminoma: case report.
Gynecol Oncol. 1989 Feb;32(2):261-7. doi: 10.1016/s0090-8258(89)80046-0.
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Clinical and pathologic spectrum of 46,XY gonadal dysgenesis: its relevance to the understanding of sex differentiation.46,XY性腺发育不全的临床和病理谱:其与性别分化理解的相关性。
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[True hermaphroditism with 46, XX karyotype: report of a case].
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Importance of early diagnosis and gonadectomy in 46, XY females.46,XY女性早期诊断及性腺切除术的重要性
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Unusual dual genital duct remnants in true hermaphroditism.真两性畸形中不寻常的双生殖管道残留
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引用本文的文献

1
True hermaphroditism: geographical distribution, clinical findings, chromosomes and gonadal histology.真两性畸形:地理分布、临床发现、染色体及性腺组织学
Eur J Pediatr. 1994 Jan;153(1):2-10. doi: 10.1007/BF02000779.