通过系统文献回顾解读梅耶-罗金丹斯基-库斯特-豪泽综合征

Nancy Yolanda Urbina Romo, Nicole Ahily Serrano Escobar, María Ilusión Solís Sánchez
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引用次数: 0

摘要

Mayer-Rokitansky-Küster-Hauser(MRKH)综合征又称缪勒氏腺增生症,是一种罕见的影响女性生殖道的先天性畸形,其特征是外观正常、核型为 46 XX 且内分泌功能正常的女性无子宫、宫颈和上阴道。本研究旨在通过文献综述解读 MRKH 综合征的主要风险因素、临床标准和诊断及其对社会心理的影响。研究人员进行了描述性文献综述,分析了2019年至2023年期间发表在高影响力期刊上的文章。共发现 16 篇文章,包括病例研究和理论信息来源。研究发现,约每 4000 到 5000 名女性中就有 1 人患有该综合征,并可能存在遗传基础。该综合征分为两种类型:I 型,无子宫和阴道;II 型,肾脏和骨骼畸形。诊断可通过超声波和核磁共振成像进行,治疗方法包括通过手术或非手术程序制造新阴道。由于不育和性生活困难,该综合征会给患者带来很大的精神和心理负担。结论是,MRKH 涉及家族史、原发性闭经和正常发育等风险因素,需要采取多学科方法,包括治疗方案。在厄瓜多尔,协议和认识的缺乏给受影响者带来了挑战。必须提高对这一疾病的认识,并支持相关研究,以改善患者的生活质量。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Interpretation of mayer-rokintansky-küster-hauser syndrome by systematic literature review
Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome, also known as Müllerian aplasia, is a rare congenital anomaly affecting the female genital tract, characterized by the absence of the uterus, cervix and upper vagina in women with normal appearance, karyotype 46 XX and normal endocrine function. The aim of the study was to interpret the main risk factors, clinical criteria and diagnosis of MRKH syndrome and its psychosocial impact through a literature review. A descriptive documentary literature review was conducted, analyzing articles published between 2019 and 2023 in high-impact journals. Sixteen articles were identified, including case studies and theoretical sources of information. The syndrome was found to affect approximately 1 in 4,000 to 5,000 women, with possible genetic basis. Two types are distinguished: type I, with absence of the uterus and vagina, and type II, with additional kidney and skeletal malformations. Diagnosis is made by ultrasound and MRI, and treatment includes the creation of a neovagina through surgical or non-surgical procedures. The syndrome carries a significant emotional and psychological burden due to infertility and sexual difficulties. It was concluded that MRKH involves risk factors such as family history, primary amenorrhea and normal development, requiring a multidisciplinary approach and including treatment options. In Ecuador, the lack of protocols and awareness presents challenges for those affected. It is vital to raise awareness of this condition and support research to improve the quality of life of those affected
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