Sano K, Terashima K, Tanaka Y, Sasaki Y
Department of Urology, Kanagawa Childrens' Medical Center.
Hinyokika Kiyo. 1995 Jan;41(1):73-7.
We report four cases of true hermaphroditism. The first patient (legal sex female, karyotype of 46, XX/46, XY, bilateral ovotestis) was changed to a male at 2 years old. The second patient (karyotype of 46,XX/47, XXY/48, XXYY, hypospadia, and right undescended testis) was referred to us at 6 months old, and turned out to have a uterus, an ovary on the left side and a testis on the right side. The third patient (6 months old, karyotype of 46, XX/46, XY, hypospadia) had an ovotestis on the right side and a testis on the left side. The fourth patient (1 year and 6 months old, a karyotype of 46,XX, hypospadia) had bilateral ovotestis. All four patients showed a variety of responses of testosterone to human chorionic gonadotropin stimulation. They were grown as males. It is difficult to treat a case of true hermaphroditism including determination of sex. We should consider the results of chromosomal analysis, hormone values, imaging studies and histology of gonads, and determine appropriate sex.
我们报告了4例真两性畸形病例。首例患者(法定性别为女性,核型为46,XX/46,XY,双侧卵睾)在2岁时转变为男性。第二例患者(核型为46,XX/47,XXY/48,XXYY,尿道下裂,右侧睾丸未降)6个月大时转诊至我们处,结果发现有子宫、左侧卵巢和右侧睾丸。第三例患者(6个月大,核型为46,XX/46,XY,尿道下裂)右侧有一个卵睾,左侧有一个睾丸。第四例患者(1岁6个月,核型为46,XX,尿道下裂)有双侧卵睾。所有4例患者对人绒毛膜促性腺激素刺激的睾酮反应各不相同。他们均按男性抚养。治疗真两性畸形病例,包括性别确定,都很困难。我们应综合考虑染色体分析结果、激素值、影像学检查和性腺组织学检查结果,然后确定合适的性别。