新生鼠输尿管不全梗阻后肾盂压力和肾脏形态变化的观察

目的 了解新生鼠输尿管不全梗阻后肾盂压力和肾脏形态的变化。方法 65只新生鼠用腰大肌包埋不同长度的左侧输尿管，制成轻（n=31)、重（n=34)度输尿管不全梗阻。对照组仅进行剖腹探查。术后8周和24周分别用核磁共振检查肾脏形态变化，术后分别于24周和30周进行肾盂测压和组织学检查。结果 梗阻肾脏均有不同程度积水。严重梗阻组除积水较严重外，发现4例肾脏肾发育不全，其平均肾实质重量仅是对照组的35％。轻度梗阻组和对照组未见发育不良的肾脏。严重梗阻组的肾脏灌注压明显高于轻度梗阻组和正常对照组。结论 新生鼠输尿管不全梗阻后均产生明显肾积水。严重梗阻组可产生肾脏发育不良，可能与严重梗阻组肾盏灌注压明显增加有关。     

先天性肾盂输尿管连接处梗阻性肾积水的神经性因素研究

目的探讨神经性因素在先天性肾盂输尿管连接处梗阻性肾积水发病中的作用。方法将31例患儿手术切除的肾盂输尿管连接处（实验组）、同一患儿输尿管切缘（切缘对照组）、产前排除泌尿系异常、孕32周以上引产并进行尸体解剖的胎儿30例（阴性对照组）进行病理分析。显微镜下观察神经节细胞及神经纤维的分布。分析神经节细胞和神经纤维在各组中的分布及差异。结果切缘对照组以及阴性对照组的神经纤维分布密度均多于实验组,而切缘对照组与实验对照组比较,差异无统计学意义。结论神经性因素是先天I生。肾盂输尿管连接处梗阻性肾积水的重要原因。临床应充分切除输尿管连接处狭窄段,以减少术后复发。     

核素利尿肾动态显像在小儿先天性肾盂积水肾造瘘术后的应用

目的探讨^99mTc-EC利尿肾动态显像（DR）对小儿先天性肾盂积水肾造瘘术后决策第二次手术方式的价值。方法用常规^99mTc-EC肾动态显像方法,15min后静脉注射速尿,共检查肾盂积水患儿33例。34只患肾行肾造瘘术,术后随访4～12个月,根据DR检查结果,28只患肾行离断性肾盂成形术,6只行肾切除术。10只患肾在肾盂成形术后5～15个月行DR检查进一步随访。结果定量评估,肾盂成形术组9只单肾盂积水患肾在肾造瘘术前后的分肾血流灌注率（BPR）变化值为好转7只,不变2只。该组BPR值在肾造瘘术前后的差异有统计学意义（P〈0.01）。肾切除术组6只患肾的BPR变化值为不变3只,恶化3只。肾造瘘术后肾盂成形术组患侧BPR及肾有效血浆流量（ERPF）明显高于肾切除术组,两组差异有统计学意义（P〈0.01）。定性评估,肾盂成形术组28只患肾肾盂积水程度变化值为明显好转3只,好转23只,无改变2只。肾切除术组6只患肾在肾造瘘术前后肾盂积水程度的变化值均为无改变。结论DR对于小儿先天性肾盂积水肾造瘘术后的评估具有独特的价值,有助于判定第二次的手术方式,并可对肾盂成形术后肾功能的恢复进行评估。     

肾盂前后径对胎儿肾积水出生后行肾盂成形术的预测价值分析

目的分析出生前后肾盂前后径(anteroposterior diameter of renal pelvis,APD)预测胎儿肾积水患儿在随访期间行肾盂成形术的临床价值。方法回顾性分析2017年6月至2019年3月湖南省儿童医院、湖南省妇幼保健院通过产前超声检出孕中期APD≥4 mm或孕晚期APD≥7 mm胎儿肾积水患者的临床资料。在随访过程中,胎儿肾积水患者出生后结局分为手术和非手术,在手术组与非手术组间分别比较孕中期、孕晚期及出生后1个月的APD值。结果本研究共纳入98例患儿(161肾),36例患儿(40肾)因出现临床手术指征而行肾盂成形术。多因素Logistic回归分析发现,孕中期、孕晚期及出生后1个月的APD值均是肾积水患儿行肾盂成形术的危险因素。孕中期、孕晚期及出生后1个月APD值分辨肾积水患儿出生后行肾盂成形术的最佳临界值分别为7.5 mm、16.4 mm和15.5 mm,灵敏度分别为75.7%、70.3%和83.8%,特异度分别为79.0%、90.0%和87.0%。当孕中期APD值为7.5 mm、孕晚期APD值为16.4 mm或出生后1个月APD值为15.5 mm时对预测行肾盂成形术的临床价值最佳(灵敏度为91.9%,特异度为85.0%)。结论孕中期、孕晚期及出生后1个月的APD值均能在一定程度上预测患儿出生后行肾盂成形术的概率。孕晚期及出生后1个月可以更好地预测UPJO患儿出生后行肾盂成形术的最佳临界值,但在妊娠中期就可以对手术风险较高的患儿进行筛查。结合胎儿期及出生后APD值,可以提高预测UPJO患儿行肾盂成形术的准确性。     

胎儿期解除单侧输尿管不完全梗阻的实验研究

目的 探讨胎儿期解除单侧输尿管不完全梗阻的效果.方法 取22只健康孕羊在妊娠第75至85天采用宫内手术的方法 造成胎羊左侧输尿管不完全梗阻.然后分为二组,第一组12只继续妊娠,第二组10只三周后再次手术去除胎羊输尿管上的硅胶环和丝线以解除梗阻.对羔羊进行影像、病理学研究.结果第一组12只孕羊中有3只流产,有9只孕羊顺产羔羊;第二组10只孕羊中有2只流产,有8只孕羊顺产羔羊.第一组羔羊左肾均可见增大、积水和肾功能损害;第二组羔羊左肾大小、肾功能正常,左肾盂无扩张,但表面不平滑、肾实质较薄.组织学上,第二组羔羊左肾肾小球数目无减少,肾小管扩张也较第一组羔羊左肾轻.结论 胎儿期解除单侧输尿管不完全梗阻,可以及时阻止病程进展,使梗阻解除肾在形态和功能上都可得到较好的恢复.     

重肾双输尿管合并肾盂输尿管交界部梗阻性肾积水的诊治分析

目的 探讨重肾双输尿管合并肾盂输尿管交界部梗阻性肾积水的诊断与处理方法。方法 回顾分析1986～2004年间收治重肾合并肾盂输尿管交界部梗阻肾积水8例的临床资料。男2例，女6例，年龄7个月～10岁，平均4．8岁。病变位于左侧3例，右侧5例；上肾积水2例，下肾积水5例，上下肾积水1例；3例为重肾完全型双输尿管，5例重肾Y型输尿管。结果 3例重肾完全型双输尿管中，上肾积水1例因肾实质薄无功能行上肾切除术，下肾积水伴上肾输尿管膨出症1例行上肾切除下肾离断性肾盂成形术，另1例下肾积水因临床症状轻微，IVP示积水半肾的肾盏变钝不明显，未行手术门诊随诊。5例重肾Y型输尿管中，上肾积水1例行上肾盂与下输尿管吻合，下肾积水3例行上肾输尿管下肾盂吻合 下肾离断性肾盂成形术，1例上下肾均积水行上下肾盂吻合 下肾盂成形术。术后3～6个月复查IVP肾积水明显好转。结论 重肾肾盂输尿管交界部梗阻肾积水发病率很低，术前不容易明确诊断或被误诊。腹部B超、IVP或MRU是有效的辅助检查手段。治疗应根据息肾功能、形态而定，如息肾功能严重受损，行患肾切除，反之，根据积水的部位、输尿管的形态选择手术方式。     

中国儿童先天性肾积水早期管理专家共识

正1疾病概况先天性肾积水(congenital hydronephrosis)是指在产前及生后早期通过超声等筛查发现的肾集合系统扩张,国外文献将其称为产前和生后尿路扩张,而国内通常称其为先天性肾积水。其病因可为暂时性或生理性的,也可为病理性的,如肾盂输尿管连接处梗阻(ureteropelvic junction obstruction,UPJO)、膀胱输尿管连接处梗阻(ureterovesical junction obstruction,UVJO)、膀胱输尿管反流(vesicouretericreflux,VUR)、后尿道瓣膜(posterior urethral valves,     

产前肾积水的生后评价

正先天性肾积水是产前超声筛查出来的常见胎儿疾病之一,发病率可达1%～5%~([1])。产前诊断的肾积水病因很多,包括暂时性肾积水、肾盂输尿管连接处梗阻(ureteropelvic junction obstruction,UPJO)、输尿管膀胱连接处梗阻、膀胱输尿管反流、多囊性肾发育不良、输尿管末端囊肿、重复肾以及后尿道瓣膜等~([2])。除去暂时性肾积水,先天性肾积水最常见病因是肾盂输尿管连接处梗阻。本文主要     

148例肾盂积水胎儿出生后的随访和治疗

目的 分析胎儿期发现肾盂积水148例患儿的出生后随访和治疗情况,初步探讨胎儿肾盂积水出生后诊断、随访方案以及手术治疗时机的选择.方法 按照美国胎儿泌尿外科协会(SFU)分级,将本院1999年1月至2006年1月收治的148例胎儿期发现肾盂积水的患儿分为4组,所有各组患儿于出生后继续B超观察随访,并辅以放射性核素扫描(ECT),磁共振水成像(MRU)等方法明确诊断,及时根据不同分级采取相应治疗方案.结果 41例1级肾盂积水的患儿持续随访1年.未出现肾盂积水加重和肾功能受损迹象,未予手术干预;49例2级患儿持续随访观察3～6个月后,其中12例因肾盂持续增大,达到3级或4级诊断标准而进行手术干预,其余37例仍持续随访观察中;31例3级和27例4级患儿均在随访观察3～6个月后出现持续肾盂增大,肾功能受损,明确诊断后进行手术干预;术后恢复良好,并分别于术后1个月、3个月、6个月、1年分别行B超随访和肾功能检查,均未再出现肾盂肾盏分离加大和肾功能继续受损趋势.结论 ①越来越多肾盂积水在胎儿期就被早期发现并被密切随访至出生后,区别于以往偶然发现,对此病诊断、治疗、随访等也应有所转变;②胎儿期肾盂积水需要区分生理性和病理性肾盂积水,进行出生后的严密随访观察和恰当治疗;③胎儿期肾盂积水出生后需根据积水级别、动态随访结果、肾功能受损情况等选择手术时机.     

先天性肾盂输尿管连接部梗阻

肾盂输尿管连接部梗阻（pelviureteric iunction obstruction，PUJO）是小儿肾积水的常见原因，可经产前B超检出。胎儿肾集合系统扩张中，PUJO约占48％，远多于多房性肾囊性变。先天性PUJO的发生率为1／600～1／800，可见于胎儿至出生后各年龄组，除经产前B超检出者外，约25％见于1岁以内，少数在青少年或成人期明确诊断。     

先天性畸形围产期处理的初步体会

目的 总结2年多来10例先天性畸形围产期管理的方法和效果。方法 对经产科B超检查拟诊为食管闭锁、右侧卵巢囊肿、左侧唇裂及右侧胸壁囊肿各1例和十二指肠闭锁、脐膨出及肾积水各2例共10例妊娠25周—39周的孕妇进行了产前咨询。并在分娩后及时转诊、手术治疗或定期随访服务。结果 10例中8例诊断正确，1例产后影像学检查与产前诊断相符，但因21—三体，家长放弃手术治疗，1例脐膨出妊娠27周时人工流产。8例中手术治疗5例(食管闭锁、十二指肠重复畸形、环状胰腺、脐膨出和唇裂)。1例胸壁淋巴管瘤穿刺注药治疗中。2例肾积水正在随访。结论 产前经B超诊断为先天性畸形的，应早期、有针对性地对家长进行有关疾病的诊断治疗、效果及预后的宣传解释。这样会有利于家长对未来子女出生后的手术治疗有思想准备，也会使孕妇心态平稳地度过妊娠中后期或及时下决心停止治疗或中止妊娠，还可明显提前转诊和治疗，有利于提高治愈率。在产前诊断先天性畸形工作中小儿外科医生可以更早地介入并发挥作用。     

Congenital hydronephrosis: prenatal diagnosis and epidemiology in Europe

ObjectiveTo describe prevalence, prenatal diagnosis and epidemiology of congenital hydronephrosis (CH) in Europe.Material and methodData from a large European database for surveillance of congenital malformations (EUROCAT). The 20 participating registries are all based on multiple sources of information and include information about livebirths, fetal deaths with gestational age ≥20 weeks and terminations of pregnancy after prenatal diagnosis of malformations. Included were all cases with CH and born 1995–2004.ResultsThere were 3648 cases with CH giving an overall prevalence of 11.5 cases per 10,000 births. The large majority of cases were livebirths (3506, 96% of total) and only 17 cases were fetal deaths and 120 were terminations of pregnancy. Almost all livebirths were alive 1 week after birth. Boys accounted for 72% of all cases. A high proportion of the cases (86%) had an isolated renal malformation. There were large regional differences in prevalence of CH ranging from 2 to 29 per 10,000 births. There was little regional variation in the prevalence of postnatally diagnosed cases while there were large regional differences in prevalence of prenatally diagnosed cases.ConclusionCases with CH are mainly livebirths, boys and survive the first week after birth. The large difference in prevalence seems to be related to the availability of prenatal screening in the region. The impact of over-diagnosis and potential over-treatment in regions with high prevalence or under-diagnosis with implications for renal function later in life in regions with low prevalence needs further investigation.     

Ultrasound diagnosis of fetal hydronephrosis: fetal renal pelvic size correlated to postnatal outcome

Ultrasound examinations during pregnancy are performed partly as a routine and partly on specific indications. Nineteen fetuses with intrauterine hydronephrosis were diagnosed during a 4-year period; 15 were inborns at the Department of Obstetrics in Lund and 4 were referred from other hospitals. Three cases were diagnosed at the first routine screening in the 17th week of gestation and the remaining 16 at a later screening examination at 32 – 36 weeks gestation. The size of the renal pelvis was measured at two perpendicular diameters in a transverse scan of the fetal abdomen. All infants were examined during the 1st week postpartum and re-examined within the 1st month of life with ultrasound and intravenous urography. Increasing dilatation of the renal pelvis was an indication for operation. There were no false-positive cases. A significant correlation between the measured mean diameter of the renal pelvis and postnatal treatment was found. When the antenatal pelvic diameter is more than 10 mm careful postpartum controls are recommended. All kidneys with a pelvic diameter of more than 20 mm were operated upon.Offprint requests to: C. Jörgensen     

Prenatal diagnosis and management of hydronephrosis

Congenital hydronephrosis is frequently amenable to prenatal diagnosis, often as early as the second trimester. Most clinicians use a renal pelvis anterior-posterior (AP) diameter of 4 mm or more prior to 20 weeks of gestation as a threshold for identifying pyelectasis. If mild dilation of the renal pelvis is an isolated finding in the second trimester, evaluation performed later in gestation is used to guide postnatal management. Since the normal renal pelvis dimensions may increase with advancing gestation, thresholds for the diagnosis are larger in the third trimester. Neonatal follow-up is typically recommended only if the fetal renal pelvis diameter exceeds a specified cut-off (e.g. 7 or 10 mm) at or beyond 34 weeks. If the measurement is less, most deem the pyelectasis physiologic or normal. However, it has been suggested that fetuses with early renal pelvis dilation that resolved during pregnancy might also benefit from postnatal surveillance. The newborn evaluation for hydronephrosis may be time consuming, invasive, and costly; however, it can often prevent sequelae from congenital uropathy.     

80例胎儿肾积水的诊断与产后转归

目的探讨胎儿肾积水的产前超声诊断及产后转归。方法对孕18～41周的胎儿进行产前超声检查，根据肾集合系统前后径情况选择分离〉0．5cm的胎儿80例（90只肾），分为3组：Ⅰ组肾集合系统前后径分离0．5～1．0cm（70例）；Ⅱ组：肾集合系统前后径分离1．0～1．5cm（16例）；Ⅲ组：肾集合系统前后径分离〉1．5cm（4例）。3组均出生后随访至2岁。结果80例（90只肾）中，14例引产，63例（73只肾）肾积水无变化或恢复正常，其中I组及Ⅱ组62例，Ⅲ组1例。Ⅲ组4例中，3例行手术治疗。结论胎儿期肾集合系统分离在1．5cm以内，且无进行性增宽者提示预后良好；〉1．5 cm伴。肾实质变薄者提示预后不良，需密切观察或手术治疗。     

槲皮素预防先天性肾积水的实验研究

目的研究槲皮素对先天性肾积水的预防作用。方法在第12个妊娠日（gestational day,GD）,将C57BL／6J孕鼠随机分为四组：对照组以玉米油灌胃,TCDD组以2,3,7,8-四氯二苯并二噁英（2,3,7,8-tetrachlorodibenzodioxin,TCDD,25μg/kg）灌胃,槲皮素组以槲皮素（100mg／kg）灌胃,TCDD＋槲皮素组以槲皮素（100mg／kg）及TCDD（25μg/kg）灌胃。于GD18,检查胎鼠畸形发生情况,并留取胎鼠泌尿系统做组织学观察及免疫组化检测。结果对照组及槲皮素组胎鼠元畸形发生。TCDD组胎鼠双侧肾盂及输尿管上段积水的发生率为100％（肾积水3级占84．1％,4级占15．9％）,TCDD＋槲皮素组肾积水的发生率为13．4％（均为3级）,差异有统计学意义（P〈0．05）。免疫组织化学检测结果显示,对照组与槲皮素组胎鼠输尿管黏膜上皮Prx1蛋白均呈阴性表达;TCDD组及TCDD＋槲皮素组存在肾积水的胎鼠输尿管黏膜上皮呈阳性表达。TCDD组胎鼠输尿管黏膜上皮Prx1蛋白阳性表达率（100％）高于TCDD＋槲皮素组（13．4％）,差异有统计学意义（P〈0．05）。结论槲皮素能有效降低TCDD所致胎鼠。肾积水的发生率,其对TCDD所致胎鼠肾积水的预防作用与抑制胎鼠输尿管黏膜上皮Prx1蛋白的表达有关。     

Clinical features of polyhydramnios associated with fetal anomalies

The purpose of this study was to examine the clinical features of pregnancy complicated by polyhydramnios associated with fetal anomalies. Sixty-nine patients with a singleton pregnancy complicated by polyhydramnios were retrospectively analyzed. Based on prenatal ultrasonographic findings, 13 cases were considered to have idiopathic polyhydramnios and the remaining 56 cases were associated with fetal anomalies. Between these two groups, no significant difference was found in the gestational weeks when polyhydramnios developed. However, significant difference was noted in the maximum amniotic fluid index (AFI) values during the pregnancy period; 25.4 +/- 2.7 cm in the former, and 30.6 +/- 8.9 cm in the latter (P = 0.0004). In all of 13 cases with idiopathic polyhydramnios, AFI values remained less than 30 cm until delivery. Twenty-two patients (39%) with fetal anomalies required a prenatal treatment such as amnioreduction and tocolysis, whereas only one patient (7.7%) with idiopathic polyhydramnios needed tocolysis therapy (P = 0.03). There was a significant risk of premature delivery with fetal anomalies (35.6 +/- 3.9 weeks' gestation vs. 38.8 +/- 1.5 weeks' gestation, P = 0.004) because of refractory polyhydramnios, rupture of membranes, non-reassuring fetal status, and intrauterine fetal death, and although most infants with idiopathic polyhydramnios were appropriate-for-dates, many of the infants with congenital anomalies were small-for-dates. Significant risk of fetal anomalies should be considered in pregnant women with severe polyhydramnios (AFI > or = 30 cm), an increased trend of amniotic fluid during the pregnancy period, polyhydramnios requiring a prenatal treatment, or fetal growth restriction. On the other hand, based on our experience, a fetus without these conditions seems to have a low risk of congenital anomalies even if polyhydramnios is noted.     

Evaluation of prenatally diagnosed hydronephrosis by morphometric measurements of the kidney

A large number of hydronephrotic kidneys (108) were diagnosed prenatally in 69 infants between 1987 and 1991 and subsequently confirmed postnatally. Prenatal morphometric measurements were done in order to find reliable parameters for the detection of a group at risk for surgical treatment. A second aim of the study was to describe the natural history and management of hydronephrosis detected prenatally. We devised a classification of postnatal obstructive uropathy using ultrasonography and the renal scan. Accordingly, we classified the patients as having mild, moderate or severe hydronephrosis. A renal pelvic antero-posterior diameter (APD) of 9 mm or more, and a pelvic-to-renal APD ratio of 0.45 before 32 weeks of gestation and 0.52 thereafter, were found to be useful for the detection of severe outcome. Our new parameter, a pelvic-to-renal volume ratio of greater than 0.08, can also be used for this purpose.     

99mTc双半胱氨酸利尿肾动态显像“超正常”现象在儿童单侧UPJO诊治中的价值

目的探讨单侧肾盂输尿管连接部狭窄(ureteropelvic junction obstruction,UPJO)患者出现99m Tc双半胱氨酸利尿肾动态显像“超正常”现象(患肾DRF≥55%)的临床意义,同时分析导致肾功能被高估的因素。方法收集上海交通大学医学院附属上海儿童医学中心2014年1月至2020年5月接受肾盂成形术且临床资料完整的107例单侧UPJO患者为研究对象。根据是否存在“超正常”现象(Supra-normal DRF,SNDRF)分为SNDRF组(n=14)与非SNDRF组(n=93),总结两组病例的临床症状及影像学等检查结果。结果在107例符合入组标准的患者中,共有14例术前肾动态显像提示存在SNDRF,两组术前APD值分别为(29.2±13.9)mm和(34.93±11.19)mm,差异有统计学意义(P=0.035)。SNDRF组术前平均DRF为56.5%。利用术前APD预测SNDRF时,其最佳阈值为26.5 mm。结论SNDRF与积水肾肾盂增大有关。术前APD≥26.5 mm是超正常功能的可靠预测因子。对于伴或不伴SNDRF严重积水的肾脏,肾功能都有被高估的可能,应谨慎解释DRF,手术适应证不应完全依赖DRF。