Kojima Yoshiyuki, Mizuno Kentaro, Nakane Akihiro, Kato Toshiki, Kohri Kenjiro, Hayashi Yutaro
Department of Nephro-urology, Nagoya City University Graduate School of Medical Sciences, Nagoya 467-8601, Japan.
J Pediatr Surg. 2009 Aug;44(8):1491-6. doi: 10.1016/j.jpedsurg.2008.10.111.
We investigated the long-term physical, hormonal, and sexual outcomes of males with disorders of sex development (DSD) and discussed the necessity of long-term follow-up for these patients after surgery.
Twelve DSD patients (average age, 21.0 +/- 3.6 years old) who had been designated as male in childhood (3 ovotesticular DSD, four 45,XO/46,XY mixed gonadal dysgenesis, four 46,XX testicular DSD, and one 46,XY DSD; androgen insensitivity syndrome) were enrolled. For these patients, height, penile length, and testicular volume were evaluated in adulthood. Serum levels of luteinizing hormone, follicle-stimulating hormone, and testosterone were also measured during follow-up. In addition, sexual function and romantic relationships were evaluated.
Development of the penis and testes was poor. According to the hormonal study, these patients were diagnosed with hypergonadotropic hypogonadism or normogonadism; 90% patients had experienced penile erection and masturbation at the time of participation, and 70% and 40% patients had experienced ejaculation and sexual intercourse with female partners, respectively. No patients preferred to avoid sexual contact with women.
Although DSD males had an undeveloped penis and testis and had hypergonadotropic hypogonadism or normogonadism, most had male sexual potential and male sex identity as long as testicular tissues were preserved.
我们研究了性发育障碍(DSD)男性的长期身体、激素和性方面的结局,并讨论了这些患者术后进行长期随访的必要性。
纳入12例童年时被指定为男性的DSD患者(平均年龄21.0±3.6岁)(3例卵睾性DSD、4例45,XO/46,XY混合性性腺发育不全、4例46,XX睾丸性DSD和1例46,XY DSD;雄激素不敏感综合征)。对这些患者在成年期评估身高、阴茎长度和睾丸体积。在随访期间还测量血清促黄体生成素、促卵泡生成素和睾酮水平。此外,评估性功能和恋爱关系。
阴茎和睾丸发育不良。根据激素研究,这些患者被诊断为高促性腺激素性性腺功能减退或性腺功能正常;90%的患者在参与研究时经历过阴茎勃起和手淫,70%和40%的患者分别经历过射精和与女性伴侣性交。没有患者倾向于避免与女性进行性接触。
尽管DSD男性阴茎和睾丸发育不全,患有高促性腺激素性性腺功能减退或性腺功能正常,但只要保留睾丸组织,大多数患者具有男性性潜能和男性性别认同。