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一例伴有严重急性肾衰竭的小儿欣曼综合征病例。

An infantile case of Hinman syndrome with severe acute renal failure.

作者信息

Chaichanamongkol Vorapong, Ikeda Masahiro, Ishikura Kenji, Hamasaki Yuko, Hataya Hiroshi, Satoh Hiroyuki, Asanuma Hiroshi, Shishido Seiichiro, Honda Masataka

机构信息

Department of Pediatric Nephrology, Tokyo Metropolitan Kiyose Children's Hospital, Tokyo, 204-8567, Japan.

Department of Pediatric Urology, Tokyo Metropolitan Kiyose Children's Hospital, Tokyo, Japan.

出版信息

Clin Exp Nephrol. 2008 Aug;12(4):309-311. doi: 10.1007/s10157-008-0048-3. Epub 2008 Apr 15.

Abstract

A 1-year-6-month-old Japanese girl with Hinman syndrome manifested urosepsis and severe obstructive nephropathy. Her voiding cystourethrogram (VCUG) revealed high-grade vesicoureteral reflux with hydronephrosis; urodynamic study was compatible with detrusor-sphincter dyssynergia. She was treated conservatively, including clean intermittent catheterization. At 3 years old, bladder function had not improved, and estimated creatinine clearance was in the subnormal range. Hinman syndrome is a potential cause of acute and chronic renal failure in infancy. Taking account of the possibility of this condition in any neonates or infants who present urinary tract infection (UTI) appears to be necessary, since early recognition and proper management of this syndrome may prevent serious complications.

摘要

一名1岁6个月大的日本女孩患有欣曼综合征,出现了泌尿道感染和严重的梗阻性肾病。她的排尿性膀胱尿道造影(VCUG)显示重度膀胱输尿管反流伴肾积水;尿动力学研究结果符合逼尿肌-括约肌协同失调。她接受了保守治疗,包括清洁间歇性导尿。3岁时,膀胱功能仍未改善,估计肌酐清除率处于低于正常范围。欣曼综合征是婴儿期急性和慢性肾衰竭的一个潜在原因。鉴于任何出现尿路感染(UTI)的新生儿或婴儿都有可能患这种疾病,考虑到这种可能性似乎是必要的,因为早期识别和妥善管理该综合征可能预防严重并发症。

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