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尾椎退化综合征(尾椎发育不全序列征)患儿的尿动力学评估

Urodynamic evaluation of children with the caudal regression syndrome (caudal dysplasia sequence).

作者信息

Boemers T M, van Gool J D, de Jong T P, Bax K M

机构信息

Department of Pediatric Urology, Wilhelmina Children's Hospital, Utrecht, The Netherlands.

出版信息

J Urol. 1994 Apr;151(4):1038-40. doi: 10.1016/s0022-5347(17)35171-6.

DOI:10.1016/s0022-5347(17)35171-6
PMID:8126785
Abstract

A total of 50 children with the caudal regression syndrome (caudal dysplasia sequence) underwent urodynamic evaluation of lower urinary tract function. Of the patients 15 had isolated sacral agenesis, 13 had sacral agenesis in combination with an anorectal malformation and 22 had an anorectal malformation without sacral anomaly. Neurogenic bladder-sphincter dysfunction was observed in 11 of the 15 children with isolated sacral agenesis (73%) and in 10 of the 13 children with combined sacral agenesis and anorectal malformation (77%). The pattern of neurogenic bladder-sphincter dysfunction in both groups did not differ significantly. In the group of 22 children with isolated anorectal malformation only 1 had signs of neurogenic bladder-sphincter dysfunction. These results indicate that anorectal malformation per se does not influence lower urinary tract function and that the sacral anomaly is the determining factor for neurogenic bladder-sphincter dysfunction in children with anorectal malformation.

摘要

共有50例患有尾椎退化综合征(尾部发育异常序列)的儿童接受了下尿路功能的尿动力学评估。其中,15例患儿为单纯骶骨发育不全,13例患儿骶骨发育不全合并肛门直肠畸形,22例患儿有肛门直肠畸形但无骶骨异常。在15例单纯骶骨发育不全的患儿中,11例(73%)存在神经源性膀胱括约肌功能障碍;在13例骶骨发育不全合并肛门直肠畸形的患儿中,10例(77%)存在神经源性膀胱括约肌功能障碍。两组神经源性膀胱括约肌功能障碍的模式无显著差异。在22例单纯肛门直肠畸形的患儿中,只有1例有神经源性膀胱括约肌功能障碍的迹象。这些结果表明,单纯肛门直肠畸形本身并不影响下尿路功能,骶骨异常是肛门直肠畸形患儿神经源性膀胱括约肌功能障碍的决定因素。

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