Tan T T, Khalid B A
Department of Medicine, Universiti Kebangsaan Malaysia, Kuala Lumpur.
Postgrad Med J. 1993 Apr;69(810):315-7. doi: 10.1136/pgmj.69.810.315.
The case of a 32 year old male with normal male adrenarchal hair pattern, bilateral gynaecomastia, a small phallus, hypospadias and bilateral poorly developed testes presenting with primary infertility secondary to azoospermia and a pelvic cyst is described. Repeated chromosomal analysis showed 46XX chromosomal constitution. Laparotomy revealed a simple cyst between the urinary bladder and the rectum. XX male syndrome is a rare cause of male infertility. The majority of cases is due to interchange of a fragment of the short arm of the Y chromosome containing the region that encodes the testes determining factor with the X chromosome. The presence of a simple cyst in the anatomical location of the uterus to our knowledge has not been reported in the literature.
本文描述了一名32岁男性病例，其具有正常男性肾上腺初现毛发模式、双侧乳腺增生、小阴茎、尿道下裂以及双侧发育不良的睾丸，因无精子症继发原发性不育并伴有盆腔囊肿。反复染色体分析显示染色体组成为46XX。剖腹探查发现膀胱与直肠之间有一个单纯囊肿。XX男性综合征是男性不育的罕见原因。大多数病例是由于Y染色体短臂上包含编码睾丸决定因子区域的片段与X染色体发生互换所致。据我们所知，文献中尚未报道过在子宫解剖位置出现单纯囊肿的情况。
