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单侧多囊性形脏中中管异常:一个双中心的回顾性研究

Hana Flogelova¹, Jan Langer², Jana Volejnikova¹

¹Department of Pediatrics, Faculty of Medicine and Dentistry, Palacky University Olomouc and University Hospital Olomouc, Czech Republic.

Journal of pediatric urology

|November 30, 2025

概括

与单边多囊性形 (UMCDK) 相关的中管 (MND) 异常在儿童中很少见,通常是良性的. 然而,有些病例需要干预,例如下尿道功能障碍等并发症,需要终身监测.

科学领域:

儿科泌尿外科儿科泌尿外科
遗传异常是一种先天性异常.
脏发育脏发育

背景情况:

单侧多囊性形 (UMCDK) 是一种先天性异常,其中受影响的脏是非功能性的,很少与中管 (MND) 遗留物有关.
这些残留物可以表现为尿管或尿管茎,代表UMCDK的不太常见呈现.

研究的目的:

调查诊断为UMCDK的儿童中ipsilateral MND异常的频率,临床意义和管理策略.
为这些相关异常建立全面的产前和产后监测协议.

主要方法:

对349名患有UMCDK的儿童 (年龄为0-19岁) 进行了回顾性分析,这些儿童在2005年至2024年期间在两个高等中心接受治疗.
根据它们的解剖特征,包括尿道和尿道残留物,MND异常被分为四种类型 (Ia,Ib,II,III).

主要成果:

在7.2%的UMCDK儿童中发现了MND异常,中位数诊断年龄为2个月,中位数随访时间为10.7年.
虽然大多数病例是无症状的,但16%的受影响男性患有下尿道功能障碍,尿路感染或脑膜炎等并发症,这些并发症通常与Ib型和III型异常有关.
两名患者在随访期间出现了2期慢性病 (CKD2),尽管最初的GFR正常. 15名患者的UMCDK自发消失,只有3名患者的MND异常在保守管理下回归.

结论:

患有UMCDK的儿童中管异常不常见,但可能导致显著的晚发性并发症,主要是下泌尿道功能障碍.
虽然许多病例是良性的,但可能需要进行内镜或手术干预,这凸显了这些患者终身监测的重要性.

关键词:

异常异常的情况中管 (MND) 是一个中管.单侧多囊性形 (UMCDK) 的病例尿路细胞结核.

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