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真两性畸形。41例病例回顾及睾丸组织学与功能观察

True hermaphroditism. A review of 41 cases with observations on testicular histology and function.

作者信息

Aaronson I A

出版信息

Br J Urol. 1985 Dec;57(6):775-9. doi: 10.1111/j.1464-410x.1985.tb07052.x.

DOI:10.1111/j.1464-410x.1985.tb07052.x
PMID:4084741
Abstract

Thirty-six children and five adults with true hermaphroditism have been reviewed. Twenty-five were raised as males and 16 as females. Those assigned as males had all ovarian tissue excised, leaving either a single testis or the testicular portion of one or both ovotestes. Post-operative testicular function was studied in 15 males. Basal testosterone levels were low in all four post-pubertal patients. Eleven children had plasma testosterone measured before and after stimulation with HCG. Response was poor in eight and normal in only three. Although social factors in South Africa usually result in true hermaphrodites being raised as males, the functional outcome of this decision is often disappointing.

摘要

对36名儿童和5名真性两性畸形的成年人进行了回顾性研究。其中25人被抚养为男性,16人被抚养为女性。那些被指定为男性的患者均切除了所有卵巢组织,仅保留单个睾丸或一个或两个卵睾的睾丸部分。对15名男性患者术后的睾丸功能进行了研究。所有4名青春期后的患者基础睾酮水平均较低。11名儿童在接受人绒毛膜促性腺激素(HCG)刺激前后检测了血浆睾酮水平。其中8人反应较差,只有3人反应正常。尽管在南非社会因素通常导致真性两性畸形患者被抚养为男性,但这一决定的功能结果往往令人失望。

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引用本文的文献

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Ann Pediatr Endocrinol Metab. 2019 Dec;24(4):231-236. doi: 10.6065/apem.2019.24.4.231. Epub 2019 Dec 31.
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True hermaphrodite presenting as primary amenorrhea.表现为原发性闭经的真两性畸形。
Indian J Endocrinol Metab. 2012 Jul;16(4):640-2. doi: 10.4103/2230-8210.98031.
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True hermaphroditism in southern Africa: the clinical picture.
Pediatr Surg Int. 2004 May;20(5):363-8. doi: 10.1007/s00383-004-1200-0. Epub 2004 May 26.
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Ambiguous genitalia--an Indian perspective.两性畸形——印度视角
Indian J Pediatr. 1997 Mar-Apr;64(2):189-94. doi: 10.1007/BF02752442.
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True hermaphroditism in a 46,XY individual, caused by a postzygotic somatic point mutation in the male gonadal sex-determining locus (SRY): molecular genetics and histological findings in a sporadic case.一名46,XY个体中的真两性畸形,由雄性性腺性别决定位点(SRY)的合子后体细胞点突变引起:一例散发病例的分子遗传学和组织学发现
Am J Hum Genet. 1993 Mar;52(3):578-85.
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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
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