van Niekerk W A, Retief A E
Hum Genet. 1981;58(1):117-22. doi: 10.1007/BF00284158.
Gonadal distribution in 409 cases of human true hermaphroditism is reviewed. An ovary was found on the left side of the body in 62.8% of the cases and the testis on the right side in 59.5%. The ovotestis is the most common gonad of the true hermaphrodite; amongst 806 gonads in 406 cases it was found in 44.3%. In this paper we give a detailed description of the morphology of ovotestis, testis and ovary in the true hermaphrodite. In addition we discuss the effects of fetal androgens and Müllerian inhibiting factor on the Wolffian and Müllerian ducts. Correlations between chromosomal complement and gonadal distribution are presented. True hermaphrodites with a 46,XX karyotype most commonly have an ovary on one side and an ovotestis on the other side; those with a Y-chromosome have a testis in 61% of cases. An analysis of the ratio of ovarian and testicular tissue within ovotestes showed a continuum from very little ovarian tissue to a small portion of testicular tissue. Each type of tissue was clearly demarcated. Hypotheses for gonadal induction in the true hermaphrodite should take cognizance of these facts. True hermaphrodites with a 46,XX chromosomal complement were characterized by a male phenotype in 54% of cases. This group may suggest a greater testicular induction ability in the genome as compared to the 46% with a female phenotype.
回顾了409例人类真两性畸形患者的性腺分布情况。62.8%的病例在身体左侧发现卵巢,59.5%的病例在右侧发现睾丸。卵睾是真两性畸形最常见的性腺;在406例患者的806个性腺中,44.3%为卵睾。本文详细描述了真两性畸形患者卵睾、睾丸和卵巢的形态。此外,我们还讨论了胎儿雄激素和苗勒管抑制因子对中肾管和苗勒管的影响。呈现了染色体组成与性腺分布之间的相关性。核型为46,XX的真两性畸形患者最常见的情况是一侧为卵巢,另一侧为卵睾;有Y染色体的患者中,61%有睾丸。对卵睾内卵巢组织和睾丸组织比例的分析显示,从极少的卵巢组织到一小部分睾丸组织存在连续变化。每种组织都有明显的界限。关于真两性畸形患者性腺诱导的假说应考虑到这些事实。核型为46,XX的真两性畸形患者中,54%表现为男性表型。与46%表现为女性表型的患者相比,这一组可能表明基因组中睾丸诱导能力更强。