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唐氏综合征患儿的膀胱肠道功能障碍

Bladder bowel dysfunction in children with Down's syndrome.

作者信息

Bhatt Nikita R, Murchison Louise, Yardy George, Kulkarni Milind, Mathur Azad B

机构信息

Department of Urology, Ipswich Hospital, East Suffolk and North Essex NHS Foundation Trust (ESNEFT), Heath Rd, Ipswich, IP4 5PD, UK.

Department of Paediatric Urology, Norfolk and Norwich University Hospital (NNUH), Norwich, UK.

出版信息

Pediatr Surg Int. 2020 Jul;36(7):763-772. doi: 10.1007/s00383-020-04682-4. Epub 2020 May 26.

Abstract

INTRODUCTION

Bladder Bowel Dysfunction (BBD) has been described in patients with Down's Syndrome (DS). Our aim was to report the incidence, demographics, presentation, complications and management of the bladder in DS patients with BBD.

METHODS

A systematic review was performed using PRISMA guidelines and search terms "{[(trisomy 21) OR down's syndrome]} AND [("non-neurogenic") OR voiding dysfunction]" in the search engines MEDLINE and SCOPUS. We also include a case series from two paediatric urology centres.

RESULTS

A total of 38 patients with BBD and DS were included. Mean age was 12 years (newborn to 21 years), the male:female ratio was 2:1. Functional constipation (90%), recurrent urinary tract infections (38%) and enuresis were common at presentation (56%), while over 56% patients required surgical intervention. Medical treatment and behavioral modification were less successful while intermittent catheterisation did not work.

CONCLUSION

This study reviews the largest cohort of patients with BBD in DS. It is common with serious consequences requiring operative intervention. Usual interventions are unreliable due to poor compliance. Early identification and management protect the renal tract. Regular screening for urogenital anomalies in DS is currently not performed. We recommend a thorough history of bladder function in DS patients to identify these cases early.

摘要

引言

唐氏综合征(DS)患者中存在膀胱肠道功能障碍(BBD)的情况已有相关描述。我们的目的是报告患有BBD的DS患者膀胱功能障碍的发病率、人口统计学特征、临床表现、并发症及治疗情况。

方法

按照PRISMA指南进行系统综述,并在MEDLINE和SCOPUS搜索引擎中使用检索词“{[(21三体综合征)或唐氏综合征]} AND [(‘非神经源性’)或排尿功能障碍]”进行检索。我们还纳入了来自两个儿科泌尿外科中心的病例系列。

结果

共纳入38例患有BBD的DS患者。平均年龄为12岁(从新生儿到21岁),男女比例为2:1。就诊时常见的症状有功能性便秘(90%)、复发性尿路感染(38%)和遗尿(56%),超过56%的患者需要手术干预。药物治疗和行为矫正效果欠佳,间歇性导尿也不起作用。

结论

本研究回顾了患有BBD的DS患者的最大队列。这种情况很常见,会产生严重后果,需要手术干预。由于依从性差,常规干预措施不可靠。早期识别和管理可保护尿路。目前未对DS患者进行泌尿生殖系统异常的定期筛查。我们建议详细了解DS患者的膀胱功能病史,以便早期识别这些病例。

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