儿童原发性膀胱毛细血管扩张症1例报告并文献复习

发布时间：2018-11-18 12:45

【摘要】：目的分析原发性膀胱毛细血管扩张症的诊断思路。方法回顾1例原发性膀胱毛细血管扩张症患儿的临床资料。结果患儿,女,9岁。近3岁时无明显诱因出现肉眼血尿,以后多次呼吸道感染后诱发的间断肉眼血尿,血凝块,持续性镜下血尿,曾伴失血性休克1次。尿常规蛋白+~++,红细胞满视野;24 h尿蛋白定量0.96 g;腹部、泌尿系超声、泌尿系增强CT扫描未见异常;肾动脉造影未见动静脉畸形或瘘;膀胱镜检查提示毛细血管扩张症。患儿无相关家族史,无其他部位毛细血管扩张。遗传性毛细血管扩张症和共济失调-毛细血管扩张症相关基因突变分析未见异常。结论儿童原发性膀胱毛细血管扩张症临床较为罕见,对于表现为早发、长期、间断肉眼血尿伴血凝块,特别有失血性休克者,应首先考虑血管因素病变可能,常规行泌尿系统各种影像学,包括血管造影检查,必要时膀胱镜检查。
[Abstract]:Objective to analyze the diagnosis of primary bladder capillary dilatation. Methods A case of primary bladder capillary dilatation was reviewed. Results Children, female, 9 years old. There was no obvious inducement to appear naked hematuria at the age of 3 years. Discontinuous hematuria, blood clot and hematuria under persistent microscope were induced by respiratory tract infection for many times, once accompanied by hemorrhagic shock. Urine routine protein ~, red blood cell full visual field, 24 h urinary protein quantity 0.96g, abdominal, urinary system ultrasound, urinary system enhanced CT scan no abnormality, renal arteriography no arteriovenous malformation or fistula; Cystoscopy suggests capillary dilatation. There was no family history and no capillary dilatation. Genetic telangiectasia and ataxia-telangiectasia-related gene mutations were not abnormal. Conclusion Primary bladder capillary dilatation in children is rare in clinic. For those with premature, long-term, intermittent hematuria with blood clots, especially with hemorrhagic shock, the possibility of vascular disease should be considered first. Routine imaging of the urinary system, including angiography and cystoscopy if necessary.
【作者单位】：北京大学第一医院儿科;河北省儿童医院肾脏免疫科;
【分类号】：R726.9

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