Department of Geriatric Endocrinology, Shandong Provincial Hospital, Cheeloo College of Medicine, Shandong University, Shandong, Jinan, China.
Department of Ultrasound, Shandong Provincial Hospital, Cheeloo College of Medicine, Shandong University, Shandong, Jinan, China.
J Pediatr Adolesc Gynecol. 2021 Feb;34(1):77-79. doi: 10.1016/j.jpag.2020.05.008. Epub 2020 Jun 17.
To date, only 23 cases of Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome with duplex kidney have been reported. We present the first reported case of MRKH syndrome with solitary duplex kidney and anal stenosis.
A 17-year-old Chinese girl presented with primary amenorrhea and fully developed secondary sex characteristics. Ultrasonography of the abdomen and pelvis revealed the absence of the right kidney, a left duplex kidney, and a primordial uterus. Surgery for anal stenosis was performed when she was 1 year of age. The patient had a normal 46, XX karyotype.
迄今为止，仅有 23 例 Mayer-Rokitansky-Küster-Hauser（MRKH）综合征合并双肾盂的病例报道。我们报告首例 MRKH 综合征合并孤立性双肾盂和肛门狭窄的病例。
一名 17 岁的中国女孩因原发性闭经和完全发育的第二性征就诊。腹部和盆腔的超声检查显示右侧肾脏缺如，左侧双肾盂，以及原始子宫。1 岁时因肛门狭窄行手术治疗。患者核型为正常 46，XX。
