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II型梅耶尔-罗基坦斯基-库斯特-豪泽综合征合并交叉融合性肾异位:一例罕见病例报告

Mayer-Rokitansky-Kuster-Hauser syndrome type II with crossed fused renal ectopia: A rare case report.

作者信息

Soekersi Hari, Trihadrian Radi

机构信息

Department of Radiology, Faculty of Medicine Universitas Padjadjaran, Hasan Sadikin General Hospital, Jl. Pasteur No. 38, Pasteur, Kec. Sukajadi, Kota Bandung, Bandung, 40161, Indonesia.

出版信息

Radiol Case Rep. 2023 Mar 7;18(5):1877-1881. doi: 10.1016/j.radcr.2023.01.107. eCollection 2023 May.

Abstract

Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a congenital disorder characterized by agenesis or aplasia of the uterus. Patients usually present with primary amenorrhea. MRKH can be isolated (type I) or accompanied by other malformations (type II) involving the kidney, skeletal, and vascular systems. Magnetic resonance imaging (MRI) is the mainstay of imaging modality in evaluating this syndrome. A 20-year-old woman presented with cyclic abdominal pain and primary amenorrhea. Secondary sexual characteristics and hormone evaluation were normal. Ultrasound and MRI were conducted and revealed no normal uterus structure with ectopic ovarium and crossed fused ectopic renal.

摘要

迈耶-罗基坦斯基-库斯特-豪泽(MRKH)综合征是一种先天性疾病,其特征为子宫发育不全或发育异常。患者通常表现为原发性闭经。MRKH综合征可为孤立性(I型)或伴有涉及肾脏、骨骼和血管系统的其他畸形(II型)。磁共振成像(MRI)是评估该综合征的主要影像学检查方法。一名20岁女性因周期性腹痛和原发性闭经就诊。第二性征和激素评估均正常。进行了超声和MRI检查,结果显示未发现正常子宫结构,存在异位卵巢和交叉融合异位肾。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/587b/10017328/4ecc85f09e05/gr1.jpg

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