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探讨先天性多发性关节挛缩症中的神经性膀胱。

Investigation into neurogenic bladder in arthrogryposis multiplex congenita.

机构信息

Center for Children's Rehabilitation of SARAH Hospital, Salvador, Brazil.

出版信息

J Pediatr Urol. 2013 Dec;9(6 Pt A):895-9. doi: 10.1016/j.jpurol.2012.12.011. Epub 2013 Mar 13.

Abstract

OBJECTIVE

During the follow-up of children who had been diagnosed with arthrogryposis multiplex congenita (AMC), it was noted that some were experiencing dysfunctional voiding. Further investigation into these cases led to a diagnosis of neurogenic bladder. Few studies have investigated the relationship between AMC and neurogenic bladder, this being the first to describe the clinical characteristics of neurogenic bladder among these patients.

METHODS

A series of 26 cases were obtained from the electronic medical records of patients with AMC who were admitted to Hospital Sarah in Salvador between 1994 and 2007. The patients had all been diagnosed with neurogenic bladder through clinical symptoms, lower urinary tract exams, and urodynamic findings.

RESULTS

There was urinary incontinence in 21 patients (81%), and 50% had a history of urinary tract infections. Renal function was altered in 4 patients (15%) and normal in 22 (85%). In the urodynamic study, 14 patients (64%) had detrusor overactivity and 6 (27%) had underactivity.

CONCLUSION

Patients with AMC may show changes in the urinary tract, including neurogenic bladder. It is mandatory to study these symptomatic children with urinary disorders.

摘要

目的

在对先天性多发性关节挛缩症(AMC)患儿进行随访时,发现部分患儿存在排尿功能障碍。进一步对这些病例进行调查,得出了神经源性膀胱的诊断。鲜有研究探讨 AMC 与神经源性膀胱之间的关系,本研究首次描述了这些患者中神经源性膀胱的临床特征。

方法

从 1994 年至 2007 年期间在萨尔瓦多的 Sarah 医院住院的 AMC 患者的电子病历中获取了一系列 26 例病例。所有患者均通过临床症状、下尿路检查和尿动力学发现诊断为神经源性膀胱。

结果

21 例患者(81%)存在尿失禁,50%有尿路感染史。4 例患者(15%)肾功能改变,22 例(85%)正常。在尿动力学研究中,14 例患者(64%)存在逼尿肌过度活动,6 例(27%)存在逼尿肌活动不足。

结论

AMC 患儿可能出现包括神经源性膀胱在内的泌尿道变化。有必要对有泌尿道症状的这些有症状的儿童进行研究。

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