泌尿生殖窦畸形:从发育到治疗。

IF 1.1 Q2 MEDICINE, GENERAL & INTERNAL
Yu Ding, Yaping Wang, Yiqing Lyu, Hua Xie, Yichen Huang, Min Wu, Fang Chen, Zhongzhong Chen
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引用次数: 0

摘要

泌尿生殖系统窦畸形(UGS),又称持续性泌尿生殖系统窦畸形(PUGS),是一种罕见的先天性泌尿生殖系统畸形。当尿道和阴道口在外阴内不能正确形成并不正确融合时,就会出现这种情况。PUGS可以作为孤立的异常或复杂综合征的一部分发生,并且经常与先天性肾上腺增生(CAH)相关。PUGS的管理还没有完善,对于何时进行手术或如何长期随访患者没有标准化的指导方针。本文就PUGS的胚胎发育、临床评价、诊断和治疗作一综述。我们还回顾了病例报告和研究结果,以探索手术和后续护理的最佳实践,希望提高对PUGS的认识,改善患者的预后。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Urogenital sinus malformation: From development to management.

Urogenital sinus (UGS) malformation, also known as persistent urogenital sinus (PUGS), is a rare congenital malformation of the urogenital system. It arises when the urethra and vaginal opening fail to form properly in the vulva and fuse incorrectly. PUGS can occur as an isolated abnormality or as part of a complex syndrome, and is frequently associated with congenital adrenal hyperplasia (CAH). The management of PUGS is not well-established, and there are no standardized guidelines on when to perform surgery or how to follow up with patients over the long term. In this review, we discuss the embryonic development, clinical evaluation, diagnosis, and management of PUGS. We also review case reports and research findings to explore best practices for surgery and follow-up care, in hopes of increasing awareness of PUGS and improving patient outcomes.

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来源期刊
Intractable & rare diseases research
Intractable & rare diseases research MEDICINE, GENERAL & INTERNAL-
CiteScore
2.10
自引率
0.00%
发文量
29
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