Endocrinology, Department of Translational Medicine, Università del Piemonte Orientale, Novara, Italy.
Endocrinology, Department of Translational Medicine, Università del Piemonte Orientale, Novara, Italy.
Urology. 2019 Jul;129:68-70. doi: 10.1016/j.urology.2019.04.008. Epub 2019 Apr 18.
A 53-year-old male referred to our centre because of hypergonadotropic hypogonadism detected during urological follow-up for urethral lithiasis. Physical examination showed short stature, micropenis, ambiguous external genitalia, and normal secondary sexual characteristics. Karyotype: 45 × 0/46XY. Abdominal MRI revealed the presence of uterus-like structure, right annex, and left testes without prostate. He underwent laparoscopic removal of dysgenetic tissues; histologic examination confirmed the presence of little uterus, fallopian tubes, little atrophic ovary, and vaginal tract; left testes was atrophic with sclero-jalinosis of seminal tubes and Leydig's cells hyperplasia. Testosterone replacement therapy was started after surgery and prostate became MRI visible after 2 years.
一位 53 岁男性因尿道结石的泌尿科随访中发现促性腺激素性性腺功能减退症而被转至我中心。体格检查显示身材矮小、阴茎短小、外生殖器模糊不清，且第二性征正常。染色体核型：45×0/46XY。腹部 MRI 显示存在子宫样结构、右侧附件和左侧睾丸而无前列腺。他接受了腹腔镜下发育不良组织切除术；组织学检查证实存在小子宫、输卵管、小萎缩卵巢和阴道；左侧睾丸萎缩，伴有精管硬化和莱迪希细胞增生。手术后开始进行睾酮替代治疗，2 年后前列腺在 MRI 上可见。
