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男性假两性畸形:临床处理、诊断与治疗

Male pseudohermaphroditism: clinical management, diagnosis and treatment.

作者信息

Hughes I A, Williams D M, Batch J A, Patterson M N

机构信息

Department of Paediatrics, University of Cambridge, UK.

出版信息

Horm Res. 1992;38 Suppl 2:77-81. doi: 10.1159/000182604.

DOI:10.1159/000182604
PMID:1292987
Abstract

Male pseudohermaphroditism (MPH) is a disorder of sexual differentiation whereby the external genitalia are at variance with a male karyotype and the presence of testes. Abnormalities of Leydig cell function are an important cause of MPH, but postnatal age must be considered when interpreting the testosterone response when HCG stimulation is used as a diagnostic test. Androgen insensitivity is also a common cause of MPH; the specific defect in androgen receptor function is currently the subject of intense study, using a combination of biochemical assays and molecular analysis of the androgen receptor gene. The treatment of MPH is influenced by genital tissue responsiveness to androgens and the technical complexity of reconstructive surgical procedures. There is a need for information on the outcome of MPH treatment regarding pubertal development, sexual performance and fertility.

摘要

男性假两性畸形(MPH)是一种性分化障碍,其外生殖器与男性核型及睾丸的存在不一致。莱迪希细胞功能异常是MPH的一个重要原因,但在将人绒毛膜促性腺激素(HCG)刺激用作诊断试验来解释睾酮反应时,必须考虑出生后的年龄。雄激素不敏感也是MPH的常见原因;雄激素受体功能的具体缺陷目前是深入研究的主题,采用了生化分析和雄激素受体基因分子分析相结合的方法。MPH的治疗受生殖器组织对雄激素的反应性以及重建外科手术技术复杂性的影响。需要有关MPH治疗在青春期发育、性功能和生育能力方面结果的信息。

相似文献

1
Male pseudohermaphroditism: clinical management, diagnosis and treatment.男性假两性畸形:临床处理、诊断与治疗
Horm Res. 1992;38 Suppl 2:77-81. doi: 10.1159/000182604.
2
Case Report of Male Pseudohermaphroditism (Androgen Insensitivity Syndrome): Congenital Disorder of Sexual Differentiation.男性假两性畸形(雄激素不敏感综合征)病例报告:先天性性分化障碍
J Assoc Physicians India. 2023 Jan;71(1):1.
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[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.
4
Leydig cell hypofunction resulting in male pseudohermaphroditism.
Fertil Steril. 1982 May;37(5):675-9.
5
[Human sex skin fibroblast cultures : a useful tool for diagnosis of male pseudohermaphroditism due to target organ unresponsiveness to androgens (author's transl)].[人类性皮肤成纤维细胞培养:诊断因靶器官对雄激素无反应所致男性假两性畸形的有用工具(作者译)]
Ann Endocrinol (Paris). 1980 Jul-Aug;41(4):305-10.
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[Male pseudohermaphroditism].[男性假两性畸形]
Nihon Hinyokika Gakkai Zasshi. 1994 Aug;85(8):1189-212. doi: 10.5980/jpnjurol1989.85.1189.
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Male pseudohermaphroditism.男性假两性畸形
Ann Endocrinol (Paris). 1980 Jul-Aug;41(4):311-43.
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Eur J Pediatr. 1993;152 Suppl 2:S91-2. doi: 10.1007/BF02125449.
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Ambiguous genitalia in the newborn.新生儿生殖器模糊。
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引用本文的文献

1
Pituitary-gonadal axis in male undermasculinisation.男性垂体-性腺轴发育不全。
Arch Dis Child. 2000 Jan;82(1):54-8. doi: 10.1136/adc.82.1.54.
2
Assessment of the gonadotrophin-gonadal axis in androgen insensitivity syndrome.雄激素不敏感综合征中促性腺激素 - 性腺轴的评估
Arch Dis Child. 1999 Apr;80(4):324-9. doi: 10.1136/adc.80.4.324.