婴幼儿尿路感染61例影像学分析

目的 观察影像学检查在婴幼儿尿路感染诊断、治疗中的作用.方法 回顾性分析2008 - 2009年61例婴幼儿尿路感染的影像学检查,2008年尿路感染患儿21例,所有患儿完成尿路超声检查,其中23.8%行肾静态显像(DMSA)筛查,9.5%行排尿性膀胱尿道造影(MCU)检查;2009年尿路感染患儿40例,所有患儿均完成尿路超声、DMSA筛查,72.5%行MCU检查.结果 2008年未发现膀胱输尿管反流(VUR)患儿,不排除有漏诊可能;2009年发现VUR14例,其中Ⅱ级反流2支,Ⅲ级反流4支,Ⅳ级反流9支,Ⅴ级反流6支,以高级别反流为主,无Ⅰ级反流.结论 婴幼儿尿路感染的影像学检查,尿路超声仍应作为首选,DMSA应予常规检查,DMSA异常患儿行MCU检查,以早期诊断VUR给予合适的治疗而减少肾损伤发生.     

膀胱输尿管反流高危患儿的临床和影像学检查结果分析

目的 对膀胱输尿管反流(VUR)高危患儿的临床表现和排泄性膀胱尿道造影(VCUG)检查结果 进行分析,同时探讨应用无辐射的排泄性尿道超声检查(VUS)对VUR的诊断意义.方法 以2007年7月-2010年4月于首都儿科研究所住院的VUR高危患儿93例为研究对象,包括首次或反复尿路感染58例、无尿路感染但胎儿期或生后B超发现肾积水者35例,所有患儿均行VCUG检查,22例患儿同时接受VUS检查,反流分级采用五级法进行评价.结果 (1)58例尿路感染患儿中发现VUR 20例,检出率为34.5%.反复尿路感染患儿中VUR的检出率明显高于首次尿路感染患儿中VUR的检出率.VUR分布为:≤1岁13例,其中男10例,双侧反流6例,Ⅳ+Ⅴ级反流占57.9%;1～5岁4例,其中男2例,双侧反流1例,Ⅳ+Ⅴ级反流占40.0%;≥5岁3例,其中男1例,双侧反流1例,全部为Ⅲ级以下反流.35例无尿路感染的肾积水患儿中发现VUR 6例,检出率为17.1%.(2)22例患儿同时行VCUG和VUS两种检查,与VCUG相比,VUS诊断VUR的敏感度为100%,特异度为92.1%,阳性预测值66.7%,阴性预测值100%,VUS和VCUG的检出一致性为93.2%.结论 对儿童尤其是低年龄组男童的尿路感染、以及胎儿期或生后B超发现有肾积水的患儿应重视VUR的早期筛查.VUS是一种准确可靠的检查手段,且避免放射暴露,可作为儿童VUR的筛查或追踪观察的方法 之一.

Abstract：

Objective To analyze the clinical data and result of voiding cystourethrography (VCUG)in high-risk children with vesicoureteral reflux(VUR)for better awareness of VUR,and to assess the usefulness of non-radioactive voiding ultrasonography(VUS) in the diagnosis of VUR. Method Ninety-three high-risk children with VUR who were hospitalized from July 2007 to April 2010 were studied.The study included 58 cases of urinary tract infection(UTI)and 35 cases of fetal or postnatal hydronephrosis detected on a B ultrasound scan.The results of urinalysis,urine culture,renal function,B ultrasound and VCUG were evaluated.Part of patients underwent VUS followed by VCUG immediately.Result (1)Sixtytwo boys and 31 girls(aged 1 month to 11.5 years,mean age 2 years)were included.VUR was detected in 26 patients(28%)by VCUG.In terms of kidney-ureter units,VUR was detected in 36 of 186 kidney-ureter units,including 6 grade Ⅰ ,3 grade Ⅱ ,6 grade Ⅲ,15 grade Ⅳ and 6 grade Ⅴ.(2)VUR was detected in 20 of 58 UTI patients(34.5%)by VCUG.The proportion of VUR in recurrent UTI group was 61.1%,much higher than that in first UTI group(22.5%).Thirteen of 20 VUR(65%)occurred in UTI patients under 1 year of age(M/F 10/3),with more bilateral VUR and severe grades of VUR than the older group.VUR was detected in 6 of 35 fetal or postnatal hydronephrosis patients(17.1%)by VCUG.(3)Twentytwo patients underwent both VUS and VCUG.VUR was detected in 4 patients and 6 kidney-ureter units by VCUG,while in 6 patients and 9 kidney-ureter units by VUS.Taking VCUG as the reference standard,VUS     

输尿管远端直径比对儿童原发性膀胱输尿管反流早期突破性尿路感染的预测作用研究

目的探讨输尿管远端直径比在预测儿童原发性膀胱输尿管反流(vesicoureteral reflux, VUR)早期突破性尿路感染中的应用价值。方法回顾性分析2018年1月至2020年12月首都医科大学附属北京儿童医院泌尿外科经排尿性膀胱尿道造影(voiding cystourethrography, VCUG)诊断的VUR患儿临床资料, 总结其临床特点, 并对其进行门诊和电话随访, 了解患儿在开始预防性抗生素治疗的12个月内是否发生突破性尿路感染, 并对VUR患儿早期突破性尿路感染相关因素进行单因素及多因素Logistic回归分析。结果本研究中符合纳入与排除标准的患儿共102例, 其中男性75例, 女性27例, 年龄(25.30±26.57)个月;均于诊断VUR后予持续预防性抗生素治疗12个月, 期间31例(31/102, 30.3%)发生突破性尿路感染, 71例(69.7%)未发生。多因素Logistic回归分析发现, 性别、反流侧别、反流级别等因素与VUR患儿早期突破性尿路感染的发生无相关关系(P>0.05), 而输尿管远端直径比(P=0.042, OR=11.4, 95%C...     

超声造影对膀胱输尿管反流诊断价值的探讨

目的对可疑膀胱输尿管反流（vesicouretericreflux,VUR）患儿进行排尿性膀胱尿道超声造影检查（contrast—enhancedvoidingurosonography,VUS）和排尿性膀胱尿道造影检查（voidingcystoure．thrography,VCUG）,对其结果进行分析,探讨超声造影对膀胱输尿管反流的诊断价值。方法将2010年7月至2011年12月本院泌尿外科收治的可疑VUR患儿54例作为研究对象,包括反复尿路感染6例,可疑后尿道瓣膜12例,单侧或双侧。肾积水并输尿管扩张36例,对比脉冲序列超声造影技术对患儿进行超声造影检查,经膀胱注入SonoVue造影剂后,实时观察造影剂微泡在反流病例尿路中的流动过程,分析评价反流程度,用五级法分析评价,并与VUCG对照。结果（1）54例患儿（108个。肾输尿管单位）中,VUS检出VUR48个肾输尿管单位,VCUG检出45个肾输尿管单位,VUS诊断VUR的敏感性为91．1％,特异性为88．9％,阳性预测值85．4％,阴性预测值93．3％,VUS和VCUG对VUR的检查有较好的一致性（Kappa值=0．792）。（2）在16例患儿中,VUS诊断VUR的分级高于VCUG1～2级。（3）在5例（6个肾输尿管单位）中,VUS可显示出肾内反流微泡,提示对肾功能造成极大影响的肾内反流的存在。相比,VCUG只能检出V级反流,无法显示肾内反流。结论VUS是一种准确可靠的检查手段,对VUR检测的敏感性及特异性均较高,在VUR检出率及分级上优于VCUG。同时无放射性,重复性好,可作为VUR的筛查或追踪观察的首选方法之一。     

婴幼儿发热性尿路感染的影像学检查评价

目的 探讨肾脏超声检查(US)、放射性核素肾静态扫描(DMSA)和排泄性膀胱尿道造影(VCUG)检查在婴幼儿发热性尿路感染(UTI)中的应用价值.方法 发热性UTI患儿人院1周内进行US及DMSA检查,2周后进行VCUG检查,急性期DMSA异常者6个月复查,并对US、DMSA和VCUG检查结果进行回顾性分析.结果 婴幼儿发热性UTI患儿160例,急性期接受US、DMSA和VCUG三项检查的共75例,VCUG检出VUR患儿23例(35个VUR肾输尿管单位),检出率为30.7%;35个VUR肾输尿管单位中,Ⅰ级反流0个,Ⅱ级8个(22.9%).Ⅲ级11个(31.4%),Ⅳ级14个(40.0%),V级2个(5.7%).35个反流肾输尿管单位中,US提示反流的有15个;无反流的114个肾输尿管单位中US提示反流的有12个,US筛查VUR的敏感性为42.9%,特异性为89.5%,阳性预测率为55.6%,阴性预测率为83.6%.35个反流的肾输尿管单位中,DMSA提示异常的有31个;无反流的114个肾输尿管单位中,DMSA提示异常的有61个,DMSA筛查VUR的敏感性为88.6%,特异性为46.5%,阳性预测率为33.7%,阴性预测率为93.0%.24例6个月后DMSA复查发现肾瘢痕形成15例,占62.5%,其中VUR患儿有10例,反流程度均为Ⅲ级或Ⅲ级以上.结论 发热性UTI婴幼儿中VUR的发生率高,以严重VUR多见,且容易形成肾瘢痕,US、VCUG和DMSA均应作为常规评估检查.     

泌尿道感染患儿膀胱输尿管返流和肾瘢痕的发生情况

目的分析不同年龄泌尿道感染儿童膀胱输尿管返流（VUR）和肾瘢痕的发生情况，加强对VUR的认识，提高检出率。方法选择上海交通大学附属新华医院儿科2001年1月-2006年12月因泌尿道感染入院患儿90例，通过排泄性膀胱尿道造影（VCUG）和（或）直接放射性核索膀胱造影（DRNC）明确诊断VUR共40例。分别根据泌尿道感染发生次数及不同年龄，分为首次泌尿道感染和1次以上泌尿道感染组，〉5岁和≤5岁组；分析VUR在首次泌尿道感染和1次以上泌尿道感染组、不同年龄组发病率情况。通过肾静态皮质显像（DMSA）检测肾瘢痕，分析年龄与VUR的级别、肾瘢痕的关系。剖析胎儿超声检查异常、生后VUR和肾瘢痕的发生情况。结果VUR的发病率在首次泌尿道感染和1次以上泌尿道感染组、〉5岁和≤5岁组差异均有统计学意义（X^2=10．627，4．409Pa〈0．05）。年龄与VUR的级别、年龄与肾瘢痕均呈负相关（r=-0．342，-0．348Pa〈0．05）。反复泌尿道感染、≤5岁的泌尿道感染患儿VUR的发生率高。年龄越小发生肾疤痕和高级别VUR的可能性越大。胎儿B超检查显示肾积水患儿，发生高级别VUR和肾瘢痕的可能性大。结论反复泌尿道感染、≤5岁的泌尿道感染患儿、胎儿B超检查显示肾积水的患儿应及时行VCUG和DMSA，及时发现、监测VUR和肾瘢痕。     

Cohen再植术后反流影响因素的研究

目的 通过回顾性研究Cohen术围手术期各变量值,分析影响Cohen术后反流因素.方法 回顾性研究及追踪随访2008年1月至2013年12月我院收治的原发性膀胱输尿管反流(VUR)及原发性膀胱输尿管交界处梗阻(POM)患儿,排除继发反流及梗阻患儿,设置围手术期各个变量,对各变量进行统计学分析,找寻影响Cohen术后反流因素.结果 共随访218例原发VUR及POM,排除2例术中输尿管折叠再植术,1例术后出现对侧反流,1例术侧产生梗阻并发症.共纳入研究214例,其中VUR 74例,POM 140例.平均年龄3.7岁(7个月至17岁),平均手术时间72.3 min(60～95 min),平均出血5.5ml,随访时间6～53个月.19例患儿术后产生反流的并发症,其中11例通过保守治疗好转,8例通过再次Cohen术治愈,术后无反流率91.1％,最终手术成功率96.3％.经统计学检验发现在手术年龄7个月至17岁、输尿管黏膜下埋植长度与直径比2.5～7情况下,手术成功率与性别、年龄、病种、单双侧、反流度数、裁剪、埋植长度、埋植直径、埋植长度与直径比、支架管留置时间,导尿管留置时间、术前输尿管直径、术前泌尿系感染(UTI)无关(P＞0.05);输尿管黏膜下长度与直径比以≥5与＜5分成两组,两组手术成功率也无差异(P＞0.05).结论 在7个月至17岁、输尿管黏膜下埋植长度与直径比2.5～7情况下,Cohen术后反流与围手术期各个因素无关,即在此年龄段输尿管黏膜下埋植长度与直径比≥2.5即可起到抗反流作用.     

小儿遗尿与膀胱输尿管反流的相关性研究

目的 探讨小儿遗尿与膀胱输尿管反流(vesicoureteral reflux,VUR)的关系,筛选小儿VUR的高危因素为临床诊治提供依据.方法 选取郑州大学第一附属医院小儿尿动力学中心以遗尿为主诉就诊并且神经功能正常的儿童和青少年83例(男36例,女47例),年龄5～16岁,平均(9.42±3.21)岁.所有患儿进行排尿性膀胱尿道造影(VCUG)检查以评估VUR情况,并进行尿常规、尿培养、肾脏和膀胱超声检查.尿动力学检查包括尿流率、盆地肌电图和膀胱压力容积测定.泌尿系检查的准入标准包括泌尿系超声异常、白天尿失禁、尿动力学检查异常、尿路感染或者同胞兄妹中有VUR病史等.结果 48例(57.8％)出现单症状性夜遗尿(MNE),35例(42.2％)非单症状性夜遗尿(NMNE).13例(15.7％)出现VUR.t检验结果显示,白天尿失禁患儿、女性患儿和当前伴有尿路感染的患儿VUR发病率明显升高(P＜0.05);多自变量logistic回归分析结果显示,只有性别、白天尿失禁和当前尿尿路感染的回归系数检验有统计学意义(P＜0.05),VUR与患儿年龄、遗尿类型(原发性、继发性)、遗尿频率、有无遗尿家族史、尿路感染病史、有无排便异常和除白天尿失禁外的白天尿路症状无统计学意义(P＞0.05).结论 VUR明显常见于白天尿失禁的遗尿患儿,建议存在白天尿失禁的遗尿患儿进一步进行相关检查.当前尿路感染是遗尿患儿发生VUR的高危因素.     

肾盂输尿管连接部梗阻合并同侧膀胱输尿管连接部梗阻的诊断与治疗

目的 肾盂输尿管连接部梗阻(ureteropelvic junction obstruction,UPJO)和膀胱输尿管连接部梗阻(ureterovesical junction obstruction,UVJO)这两个最常见的儿童泌尿系统病理状态同时存在较少见.本文对单侧UPJO合并同侧UVJO的诊断与治疗进行探讨.方法 回顾性分析上海交通大学医学院附属新华医院2012年1月至2015年7月间手术治疗的UPJO合并UVJO患儿.术前常规行泌尿系超声、同位素利尿肾图(DR)及排泄性膀胱尿道造影(VCUG)等检查.结果 我院共诊治单侧UPJO合并UVJO的43例患儿,失访2例.11例患儿术前明确诊断;41例患儿先行肾盂成形术+肾造瘘术,其中10例术后复查发现输尿管末端狭窄自行缓解,28例再次接受输尿管膀胱再植术,3例患儿行肾盂成形术+肾造瘘术后复查核素提示分肾功能低于10％,给予患肾切除;38例患儿术复查肾积水程度和肾脏功能均得到显著改善.结论 单侧UPJO合并UVJO的术前诊断非常困难,术中应仔细检查,避免遗漏同时合并存在的病理改变.术前尽量通过超声、DR及MR等影像检查作出明确诊断,进而制定个体化的治疗方案,能够获得满意的治疗效果.如术前明确有UPJO的存在,应选择首先进行肾盂成形术,术后根据检查结果决定是否行输尿管膀胱再植术.     

139例小儿原发性膀胱输尿管反流临床分析

目的 分析小儿原发性膀胱输尿管反流(VUR)的发病情况、肾损害评价、预后等临床资料.方法 回顾性分析974例尿路感染患儿中的139例小儿原发性VUR患儿的反流发生情况、肾瘢痕形成、尿路B超、尿微量蛋白、预后等临床资料.结果 尿路感染患儿中VUR发生率为14.3%(139/974).婴幼儿期(＜2岁)发生率最高为17.2%(79/458),139例患儿中79例(56.8%)患儿年龄＜2岁,且男性所占比例高于女性(P=0.001).轻度(Ⅰ-Ⅱ级)、中度(Ⅲ级)、重度(Ⅳ-Ⅴ级)反流所占比例分别为19.7%(41/208)、35.6%(74/208)、44.7%(93/208).VUR患儿中肾瘢痕发生率为37.O%(50/135),在不同年龄段中婴儿期(＜1岁)发生率最高(42.4%).50例肾瘢痕患儿中30例(60.0%)年龄在2岁之内,随着反流级别加重,发生肾瘢痕危险性亦提高(P＜0.05).尿路B超诊断VUR的灵敏度与特异度分别为24.8%、94.3%.疾病急性感染期尿微量蛋白的升高与肾瘢痕形成无明显相关性(P＞0.05).31例随访患儿中有90%尿路感染控制,44.4%患儿反流消失,57.1%患儿肾瘢痕好转,无肾功能进行性恶化病例.结论 小儿原发性VUR需要及早诊断与治疗,并同时对.肾损害作出正确评估,只有坚持长期的正规治疗与长期随访,才能有效保护肾脏,防止肾瘢痕的形成.     

先天性肾脏和尿路畸形超声筛查三级转诊体系的高危儿肾盂扩张筛查和随访研究

目的 对0~3月龄高危儿肾盂扩张（RPD）筛查和随访,探讨先天性肾脏和尿路畸形泌尿系统超声筛查三级（社区卫生服务中心-妇幼保健院-儿童专科医院）转诊体系（简称三级转诊体系）实施的可行性。方法 在三级转诊体系中,社区卫生服务中心甄别高危儿并转诊至妇幼保健院,妇幼保健院泌尿系统超声筛查RPD、随访和转诊,儿童专科医院对转诊RPD患儿确诊和制定干预措施。 结果①筛查结果：2010年6月至2012年5月,3 743名0~3月龄高危儿泌尿系统超声筛查RPD阳性率9.0%（338例）,男婴在总的RPD以及轻、中和重度RPD的筛查阳性率均明显高于女婴,但男女婴轻、中、重度RPD构成比差异无统计学意义;②随访结果：285例在1岁时统计随访结果,诊断肾盂输尿管连接处梗阻 3例,巨输管症2例,重复肾、重复上肾积水伴输尿管异位开口1例,其中手术3例;余279例RPD患儿经(5.4±4.5)个月随访,241例RPD恢复正常,其中轻、中和重度比例分别为91.9%、76.3%和22.2%;RPD好转16例,持续17例,加重5例。③三级转诊体系：第2年度较第1年度自愿接受泌尿系统超声筛查高危儿的比例增加（83.0% vs 64.1%）,RPD筛查阳性率未见明显差异（9.5% vs 7.8%）;第1年度较第2年度转诊率及随访率差异均无统计学意义。结论 基于三级转诊体系的高危儿RPD筛查、随访和转诊运行实施效果良好,儿童专科医院需进一步完善培训和转诊机制,妇幼保健院在三级转诊体系中的作用关键,特别是随访和转诊是三级转诊体系的最重要环节。     

先天性前尿道瓣膜及憩室对上尿路损害的研究

目的 比较先天性前尿道瓣膜及憩室对上尿路的损害,分析二者是否同一疾病不同表现的可能,并了解其对上尿路的损害.方法 回顾性分析本院1990年1月至2009年12月收治的先天性前尿道瓣膜（52例）及憩室（26例）患儿临床资料,比较其临床表现、手术方式及术后恢复情况.两组间率的比较采用x2检验,P＜0.05为差异有统计学意义.结果 20例（38.5%）前尿道瓣膜患儿及38例（30.8%）前尿道憩室患儿存在不同程度膀胱输尿管返流,前者20例（38.5%）及后者12例（46.2%）存在上尿路积水.两组在输尿管反流发生率及程度、上尿路积水方面比较,差异无统计学意义（P＞0.05）.前尿道瓣膜患儿存在膀胱憩室和（或）成小梁改变者明显多于前尿道憩室患儿（P＜0.05）.11例前尿道瓣膜及6例前尿道憩室患儿接受了2次或2次以上手术,其中5例行膀胱输尿管再植抗反流.存在输尿管反流的11例前尿道瓣膜和4例憩室患儿获随访,随访时反流消失或不超过3级.结论 本研究中,前尿道瓣膜和憩窒患儿上尿路积水和膀胱输尿管反流情况无明显差异,支持二者是同一疾病不同表现形式的判断.对于存在膀胱输尿管反流的患儿,当下尿路梗阻解除后,若反流仍达4级以上,可以考虑采取抗反流手术.     

加强对儿童尿路感染和膀胱输尿管反流的认识

儿童尿路感染是婴儿和儿童中一种常见的细菌性感染,6岁以内儿童泌尿道感染(UTI)累计发病率女孩为6.6%,男孩为1.8%.UTI与膀胱输尿管反流(VUR)的关系复杂,共同存在是导致持续性的肾脏损害和疤痕化的重要因素.UTI诊断明确后最常应用的影像学检查有肾脏和尿路超声检查、排泄性膀胱尿道造影和核素肾静态显像(DMSA),其中DMSA为目前公认的诊断肾瘢痕的金标准.尽管对在不同年龄、性别和临床表现的患儿中如何正确选择相关的影像学检查有较大的争议,但多数推荐对所有2岁以下的儿童进行超声、VCUG和DMSA检查.研究还显示VUR有其一定的遗传基础,在同胞中的发病率显著高于健康儿童.随时间推移发育逐渐成熟,有部分反流可自行痊愈,大都不需要手术治疗.大多数VUR的病例,尤其是5岁以下的儿童建议使用低剂量持续性抗生素预防治疗.反流性肾病长期的并发症是发生终末期慢性肾功能衰竭.     

输尿管镜钬激光治疗儿童输尿管末端囊肿的疗效分析

目的：探讨输尿管镜钬激光治疗儿童输尿管末端囊肿的疗效。方法回顾性分析2010年3月至2014年3月本院22例儿童输尿管末端囊肿临床资料,均经B 超、泌尿系CT、静脉尿路造影、膀胱逆行造影检查确诊,并接受输尿管镜钬激光囊肿切开术。22例患儿年龄3个月至15岁,平均3.6岁,其中女性15例（68.2％）,男性7例（31.8％）;左侧9例（40.9％）,右侧11例（50.0％）,双侧2例（9.1％）;囊肿开口于膀胱内20例（90.9％）,开口于膀胱颈尿道内口2例（9.1％）;16例（72.7％）合并患侧肾积水或者输尿管扩张,7例（31.8％）合并重复肾畸形,且均为上下位肾输尿管Y形融合并以末端囊肿开口于膀胱内,4例（18.2％）伴有膀胱输尿管反流,1例（4.5％）合并输尿管末端结石,13例（59.1％）合并尿路感染;均行输尿管镜钬激光囊肿切开术,手术时间为14～46 min,平均（28±10）min,术中出血量2～10 mL,住院时间2～5 d,平均（3.1±1.0）d。结果术后21例随访,1例失访,随访时间为3～31个月,20例未见输尿管囊肿复发,10例尿路感染症状完全消失,12例肾积水或者输尿管扩张得到明显改善,1例合并结石的未见复发,1例出现膀胱输尿管反流加重而行输尿管膀胱再植术。结论输尿管镜钬激光治疗儿童输尿管末端囊肿操作简便,创伤小,手术时间短,恢复快,并发症少,可作为首选治疗方式;对于严重膀胱输尿管反流,异位输尿管开口的重复肾患儿,术前需要做充分评估,以减少再次手术的风险。     

超声筛查26989名儿童中先天性肾脏和尿路畸形的临床分析

目的 了解泌尿系超声筛查儿童先天性肾脏和尿路畸形(CAKUT)的发生率及其中各种畸形的发病诊疗情况,探讨超声在筛查诊断儿童CAKUT的重要性.方法 对2008年底北京儿童医院超声筛查26 989名儿童发现的489例儿童泌尿系异常病例进行随访,核实是否进一步明确筛查发现的泌尿系异常情况,对于未复查或者仍未确诊者统一者安排到北京儿童医院复查超声,进行总结分析.结果 ①本次筛查中(26 989例)估算CAKUT发生率约为1.67%,绝大部分无临床表现.②489例病例中随访到320例,失访169例,随访组(320例)中明确CAKUT 295例,其中16例确诊结果与超声筛查结果不完全一致.③肾积水137例(0.78%),输尿管膀胱连接部梗阻33例(0.19%),肾发育不良30例(0.17%),肾囊肿26例(0.15%);重复肾24例(0.14%);孤立肾22例(0.12%);输尿管囊肿16例(0.09%);融合肾10例(0.06%);肾发育不全9例(0.05%);异位肾5例(0.03%);多囊肾3例(0.02%);原发膀胱输尿管反流、后尿道瓣膜、肾旋转不良、分支肾盂、肾外型肾盂各1例.④114例畸形病例需手术治疗,占随访组中CAKUT患儿的38.6%(114/295),只有1例肾盂输尿管连接部梗阻病例手术失败.结论 儿童CAKUT的发生率较高,且早期并无明显临床表现而不易发现;超过三分之一的CAKUT需要外科干预;超声检查对发现儿童CAKUT具有明显优势,是筛查的首选方法,可以作为儿童的常规体检项目.

Abstract：

Objective To investigate the proportion of children with congenital abnormalities of the kidney and urinary tract(CAKUT)among those who received screening,analyze the rate, diagnosis and treatment of each abnormality, and evaluate the value of ultrasonogrphy in the CAKUT diagnosis.Method Totally 489 of 26 989 children who received screening for urinary tract abnormalities were found to have CAKUT and were followed up by telephone interviews and were asked whether they had defined diagnosis of CAKUT, had clinical symptoms and received treatment. Children who had been operated on were also asked about the operation time, surgical procedure, postoperative diagnosis, and outcome. Children who had never been reviewed or still had no diagnosis were arranged to Beijing Children's Hospital for ultrasound follow-up. The final data were summed up for a retrospective analysis. Result ① The proportion of children with CAKUT was 1.67%. Most children with CAKUT had no clinical manifestations. ② Of the 489 cases, 320 were followed up, and 169 were lost. Of the cases who were followed up, 16 out of 295 cases who had clear diagnosis showed inconsistent results in ultrasound screening.③Hydronephrosis was identified in 137 cases (0.78%),among whom 111 were males and 26 were females,in 98 cases the hydronephrosis was on the left, in 28 on the right and in 11 were bilateral. Ureterovesical junction obstruction occurred in 33 cases (0.19%),25 males and 8 females,in 22 cases on the left, in 10 on right and bilateral in 1 case. Renal dysplasia was shown in 30 cases(0.17%),22 males and 8 females;renal cysts in 26 cases(0.15%); duplex kidney in 24 cases (0.14%) ;solitary kidney in 22 cases(0.12%);ureterocele in 16 cases(0.09%);fused kidney in 10 cases(0.06%);renal hypoplasia in 9 cases(0.05%);ectopic kidney in 5 cases(0.03%);polycystic kidney in 3 cases(0.02%).Primary vesicoureteral reflux, posterior urethral valves, renal malrotation, branch renal pelvis, extrarenal pelvis in 1 case each.④Totally 114 of the 295 cases (38.6%)who were followed up for CAKUT in screening test required surgical treatment.Only one case of ureteropelvic junction obstruction(UPJO)failed in surgery.Conclusion The proportion of children with CAKUT was high.CAKUT is difficult to detect since there is no significant clinical manifestations in early ages.More than one third of patients with CAKUT needed surgical treatment.Ultrasonography showed obvious advantages in detecting children's CAKUT, proven to be the most preferred method of screening, thus could be recommended as a routine of children's physical examination.     

Study of the association between the BMP4 gene and congenital anomalies of the kidney and urinary tract

Objectiveto determine the frequency of different phenotypes for congenital anomalies of the kidney and urinary tract (CAKUT) in a Brazilian sample, and to evaluate the association between the CAKUT phenotypes and the BMP4 gene.Methodsin this study, 457 Brazilian individuals were analyzed in an attempt to establish the association between the BMP4 gene and the CAKUT diagnosis. A case-control sample was genotyped for three BMP4 gene polymorphisms.Resultsassociation data was established with CAKUT sample as a whole and with the three most important CAKUT phenotypes: multicystic dysplastic kidney disease (MDK), ureteropelvic junction obstruction (UPJO) and vesicoureteral reflux (VUR). When the sample was segregated in these three phenotypes, associations between the BMP4 gene were observed with UPJO and with MDK. Conversely, VUR was not associated to the polymorphisms of the BMP4 gene.Conclusionsthe present data suggest that Brazilian individuals with polymorphisms of the BMP4 gene have a higher risk to develop CAKUT, especially the malformations related to nephrogenesis and initial branching such as MDK and UPJO. Conversely, VUR appeared not to be related to BMP4 gene.     

Renal development in the fetus and premature infant

Congenital abnormalities of the kidney and urinary tract (CAKUT) are one of the leading congenital defects to be identified on prenatal ultrasound. CAKUT represent a broad spectrum of abnormalities, from transient hydronephrosis to severe bilateral renal agenesis. CAKUT are a major contributor to chronic and end stage kidney disease (CKD/ESKD) in children. Prenatal imaging is useful to identify CAKUT, but will not detect all defects. Both genetic abnormalities and the fetal environment contribute to CAKUT. Monogenic gene mutations identified in human CAKUT have advanced our understanding of molecular mechanisms of renal development. Low nephron number and solitary kidneys are associated with increased risk of adult onset CKD and ESKD. Premature and low birth weight infants represent a high risk population for low nephron number. Additional research is needed to identify biomarkers and appropriate follow-up of premature and low birth weight infants into adulthood.     

Prediction of vesicoureteral reflux after a first febrile urinary tract infection in children: validation of a clinical decision rule.

AIMS: To test the reproducibility of a highly sensitive clinical decision rule proposed to predict vesicoureteral reflux (VUR) after a first febrile urinary tract infection in children. This rule combines clinical (family history of uropathology, male gender, young age), biological (raised C reactive protein), and radiological (urinary tract dilation on renal ultrasound) predictors in a score, and provides 100% sensitivity. METHODS: A retrospective hospital based cohort study included all children, 1 month to 4 years old, with a first febrile urinary tract infection. The sensitivities and specificities of the rule at the two previously proposed score thresholds (< or =0 and < or =5) to predict respectively, all-grade or grade > or =3 VUR, were calculated. RESULTS: A total of 149 children were included. VUR prevalence was 25%. The rule yielded 100% sensitivity and 3% specificity for all-grade VUR, and 93% sensitivity and 13% specificity for grade > or =3 VUR. Some methodological weaknesses explain this lack of reproducibility. CONCLUSIONS: The reproducibility of the previously proposed decision rule was poor and its potential contribution to clinical management of children with febrile urinary tract infection seems to be modest.