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混合型性腺发育不全的定义:一侧性腺为发育不全的睾丸,一侧为条索状,染色体核型45,X/46,XY。本组9例嵌合体核型45,X/46,XY有多种表型与性腺病理。社会性别女8例,男1例;9例均有Turner综合征表现,5例合并阴蒂增大,2例有泌尿生殖窦。术中见双侧条索状性腺6例;一侧发育不全睾丸,一侧条索状性腺2例;与一侧为发育不全卵睾,一侧条索状性腺1例。病理检查一侧发育不全睾丸5例,对侧卵巢间质3例,Leydig与门细胞各1例;3例双侧为卵巢间质,其中1例一侧有原始卵泡,2例性腺染色体均为45,X;1例一侧性腺为卵睾,另一侧为卵巢间质。XO/XY嵌合型性腺发育不全从表型、性腺与病理表现均为多样化,唯有染色体核型一致为45,X/46,XY,因而采用XO/XY嵌合体性腺发育不全为此类患者的名称。本组3例已发生性腺母细胞与支持细胞肿瘤。全部行性腺与子宫切除术。
The definition of mixed gonadal dysgenesis: one side of the gonads of hypoplasia testes, one side of the cord-like, karyotype 45, X / 46, XY. The group of 9 chimera karyotype 45, X / 46, XY has a variety of phenotype and gonadal pathology. 8 were female and 1 was male. All of them had Turner syndrome in 9 cases, 5 cases had clitoris increased, and 2 cases had urogenital sinus. Intraoperative see bilateral bilateral cord-like gonads in 6 cases; one side of the hypoplasia testis, one side of the cord-like gonads in 2 cases; and one side of the development of incomplete testis, one side of the cord-like gonads in 1 case. Five cases of hypoplastic testes on one side of the pathological examination, three cases of contralateral ovary interstitial, and one case of Leydig and gate cells. Three of them were ovarian stroma on both sides, of which one had primordial follicles on one side and two had gonadal chromosomes 45, X; 1 case of gonads on one side of the egg testis, the other side of the ovarian stroma. XO / XY chimeric gonadal dysgenesis from the phenotype, gonadal and pathological manifestations are diverse, only the chromosome karyotype consistent 45, X / 46, XY, and therefore XO / XY chimera gonadal hypoplasia for such Patient’s name. 3 cases of this group have occurred in gonadal cells and supporting cell tumors. All gonads and hysterectomy.