梅尔-罗基坦斯基-库斯特-豪泽综合征 II 型伴骨盆腔肾脏融合：病例报告

IF 0.5 4区医学 Q3 Medicine

Journal of Nepal Medical Association Pub Date : 2024-03-31 DOI:10.31729/jnma.8532

Suman Paudel, Prerana Singh Rokaha, Pankaj Kafle

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引用次数: 0

摘要

摘要：Mayer-Rokitansky-Küster-Hauser 综合征（MRKH）又称穆勒氏管发育不全，是由于胚胎期穆勒氏管发育不全，导致阴道、子宫或两者均发育不全或闭锁。患者通常表现为原发性闭经，发育和青春期发育正常。这里我们要介绍的是一例 29 岁女性的原发性闭经病例。第二性征和激素评估均正常。超声波和核磁共振检查显示她完全没有子宫，阴道口很小。双侧肾窝空虚，双肾位于盆腔内，相互融合，单肾盂呈薄饼状。

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Mayer-Rokitansky-Kuster-Hauser Syndrome Type II with Fused Kidneys in Pelvic Cavity: A Case Report.

Abstract: Mayer-Rokitansky-Küster-Hauser syndrome (MRKH) also known as Müllerian agenesis, is caused by embryologic underdevelopment of the Mullerian duct, with resultant agenesis or atresia of the vagina, uterus, or both. Patients usually present with primary amenorrhea with normal growth and pubertal development. Here we present a case of a 29-year-old woman presented with primary amenorrhea. Secondary sexual characteristics and hormone evaluation were normal. Ultrasound and MRI were conducted and revealed complete absence of uterus, small vaginal canal. Bilateral renal fossa were empty and both the kidneys were located in the pelvic cavity fused to one-another with single renal pelvis giving pancake appearance.

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来源期刊

Journal of Nepal Medical Association PUBLIC, ENVIRONMENTAL & OCCUPATIONAL HEALTH-MEDICINE, GENERAL & INTERNAL

CiteScore

1.00

自引率

0.00%

发文量

233

审稿时长

>12 weeks

期刊介绍： Journal of Nepal Medical Association is an internationally peer-reviewed, MedLine/PubMed indexed, a monthly general medical journal published by Nepal Medical Association. JNMA is the first and oldest medical journal from Nepal since 1963 AD.