实验性完全输尿管梗阻对肾发育影响的机制

先天性输尿管梗阻及其所引起的肾积水临床对手术治疗方面研究比较多 ,但梗阻对肾脏发育影响的机制报道甚少 ,今对实验性完全输尿管梗阻所致肾脏损害的有关研究作如下综述。一、肾脏血流动力学变化在新生鼠单侧输尿管梗阻 (ＵＵＯ)后 ,早期肾血流量 (ＲＢＦ)呈三相性变化。开始肾盂压力升高 ,入球小动脉扩张 ,ＲＢＦ增加。约 90ｍｉｎ后 ,出球小动脉开始收缩 ,ＲＢＦ逐渐降低。但由于入球小动脉尚未收缩 ,肾盂压力仍进一步升高。3～ 5ｈ后入球小动脉开始收缩 ,ＲＢＦ返回基线并进一步下降 ,肾盂压力开始下降[1,2 ] 。此反应在去肾脏交感神经 …     

逆行气囊扩张治疗婴幼儿肾盂输尿管交界处梗阻的早期结果

逆行气囊扩张治疗婴幼儿肾盂输尿管交界处梗阻的早期结果对肾盂输尿管交界处（ＵＰＪ）梗阻的治疗，开放性离断式肾盂成形术已得到广泛认可；肾盂内切开术在成人及儿童已有成功的报道，但却极少用于婴儿；顺行和逆行气囊扩张也成功地应用于成人输尿管狭窄和原发性ＵＰＪ梗...     

离断性肾盂成形术治疗先天性肾盂输尿管连接部梗阻

先天性肾盂输尿管连接部梗阻（UPJO）所致肾积水是小儿较常见的泌尿系畸形，发病原因包括肾盂输尿管连接部狭窄、瓣膜、息肉、高位输尿管口、迷走血管或副血管压迫等。临床症状无特异性，主要依靠影像学诊断，B超检查简便易行，可作为UPJO的初步筛选诊断。静脉尿路造影通过肾脏对特殊造影剂的摄取和经上尿路的排泄，     

先天性巨输尿管症二例

先天性巨输尿管症二例田洪孝例１男，１．５岁。因左腹部包块１０余天入院。体检：左腹部稍隆起，可触及包块，囊性感，不活动，无压痛。尿常规及培养（一），ＢＵＮ正常，Ｂ超提示左腹部１２ｃｍ×１１ｃｍ×８ｃｍ囊性包块。静脉肾盂造影（ＩＶＰ）：左肾及输尿管未显影...     

先天性肾盂输尿管连接部梗阻的诊断治疗

目的 探讨小儿先天性肾盂输尿管连接部梗阻（UPJO）致肾积水诊断和治疗的最佳方法。方法 对26例小儿UPJO致重度肾积水，采用B超与静脉尿路造影（IVU）相结合的诊断方法和离断式肾盂输尿管成形术（Anderson—Hynes手术）治疗，总结其效果。结果 所有病例经手术和病理检查证实诊断正确，手术经过顺利，术后恢复好。24例获随访6个月～2年，无血尿、尿频、腹部包块、腹胀等症状，尿常规正常，临床治愈。B超示扩张的肾较术前缩小、肾实质厚度增加；IVU示手术侧肾显影时间提前、杯口形态改善，其中20例输尿管显示良好。结论 B超和IVU相结合是诊断小儿UPJO极有效的方法；Anderson—Hynes手术是治疗UPJO的最佳术式。     

幼小婴儿肾盂输尿管连接部梗阻治疗体会

目的32例幼小婴儿肾盂输尿管连接部梗阻诊断和治疗方法。方法回顾性分析了32例平均年龄51．5d的幼小婴儿肾盂输尿管连接部梗阻的诊断和治疗。结果32例患儿术后恢复良好，但其中出现尿外渗2例；吻合口狭窄1例，因周围粘连和瘢痕压迫吻合口而被迫重新二次手术。于术后1个月、3个月、6个月、1年、2年分别行B超检查，均未再出现肾盂增大，未出现肾盂肾盏分离加大趋势，证实吻合口通畅。结论产前和产后的B超检查是比较可靠的辅助手段，对产前肾积水胎儿，建议产后7～10d首次常规B超检查，并于生后6周继续复查；如果B超观察肾盂未再继续扩张，可继续观察；反之，则应积极手术治疗，以防止影响肾功能；小儿肾积水一般均应考虑保留肾脏，肾皮质在2mm以上，肾脏无明显发育异常，应争取保存患肾；离断性肾盂输尿管成形术效果最好；同时应该积极进行术后随访。     

先天性肾积水转化生长因子转录水平的研究

目的：检测先天性肾积水患肾转化生长因子β1（TGF－β1）mRNA表达的改变，探讨TGF－β1在小儿先天性肾积水肾损伤中的作用及其临床意义。方法：采用RT－PCR技术检测15例先天性肾积水患儿患肾实质组织，肾盂组织，肾盂输尿管狭窄段组织TGF－β1mRNA的表达，经凝胶分析系统半定量，和10例正常肾，肾盂及输尿管对照.结果：患肾组织，肾盂及肾盂输尿管狭窄段中TGF－β1mRNA的表达增高，结论：先天性肾积水患儿患肾组织TGF－β1表达增高，TGF－β1表达的增高可能和先天性肾积水所致的慢性肾损伤及间质纤维化有关。     

小儿肾积水

肾积水是小儿泌尿外科的常见病。小儿肾积水绝大多数是因先天性肾盂输尿管联接部发育异常引起;其他原因引起的小儿肾积水比较少见。本文仅综述肾盂输尿管联接部先天性异常所引起的肾积水。过去治疗这类小儿巨大肾积水,多主张作肾盂切除术,近来提倡作肾盂成形术的作者日益增多。     

先天性肾积水实验研究进展

小儿先天性肾积水临床多见 ,约占新生儿的 1% [1] 。虽已进行大量临床和实验研究 ,其病理生理机制仍未完全阐明。现代影像学及肾功能测定方法的发展改变了过去“肾脏扩张即存在梗阻需要手术治疗”的观念。已发现许多肾积水患儿不必手术可长期保持良好稳定的肾功能[2 4] 。动物实验已显示 ,轻度输尿管不全梗阻虽能引起明显肾积水 ,肾功能并不一定进行性下降[3] 。但临床也确有不少肾盂输尿管交界处不全梗阻患儿如不及时手术解除梗阻 ,肾功能将进行性损害。然而 ,如何精确诊断输尿管梗阻程度及预测肾积水发展趋势 ,临床尚无可靠方法。Ｂ超、…     

先天性肾积水肾脏纤维化机制的研究进展

先天性肾积水是小儿泌尿外科常见疾病,发病原因主要是：肾盂输尿管连接处梗阻（UPJO）、输尿管末端狭窄、膀胱输尿管反流、后尿道瓣膜以及神经源性膀胱等。其中UPJO所致小儿肾积水最常见,发生率约1／600～1／800,肾盂输尿管连接部狭窄、息肉、瓣膜以及高位输尿管、迷走血管压迫和腔静脉后输尿管等是UPJO发病的主要病因。此外,肾盂输尿管连接部平滑肌细胞异常,大量胶原纤维沉积,导致蠕动功能障碍,也发挥着重要的作用。     

尿液AQP2在先天性肾积水患儿中的检测和意义

目的研究单侧先天性肾盂输尿管连接处梗阻（pyeloureteric junction obstruction，PUJO）肾积水患儿尿液水通道蛋白2（Aquaporins-2，AQP2）测定在肾脏浓缩功能评价中的意义。方法选取12例正常儿童，26例单侧先天性PUJO肾积水患儿，其中轻度14例，重度12例，均经彩超、静脉。肾盂造影证实，并排除其它泌尿系统疾病；在严格设定条件下，收集正常对照组术前和梗阻解除术后第3天患肾24h尿液，同时收集术中。肾盂尿液，测定尿渗透压，并用酶联免疫吸附法（ELISA）测定尿液中AQP2。结果与正常对照组比较，积水组术中尿液AQP2和尿渗透压明显下降（P〈0．05），且重度积水组术中尿液AQP2与轻度积水组相比，明显降低（P〈0．05），但重度积水组和轻度积水组术中尿渗透压相比无显著差异（P〉0．05）；梗阻解除后，轻、重度积水组术中尿液AQP2与术后第3天相比，无显著差异（P〉0．05），而术后第3天两组尿渗透压却明显下降／P〈0．05），术后第3天重度积水组与轻度积水组尿液AQP2和尿渗透压相比较，显著下降（P〈0．05）；正常对照组与轻、重度积水组术后第3天尿液AQP2和尿渗透压之间存在一定相关性。结论小儿单侧先天性PUJO尿液AQP2和尿渗透压较正常对照组显著降低，且二者之间存在一定相关性。尿液中AQP2下降可能和尿液浓缩功能改变有关。     

小儿肾积水肾盂成形术后尿理化性质和尿水通道蛋白2改变的研究

目的 探讨小儿单侧先天性肾盂输尿管连接处梗阻(pyeloureteric junction obstruction,PUJO)肾积水术后1周内尿理化性质和尿水通道蛋白2(aquaporins-2.AQP2)的变化.方法 选取14例符合Grignon法四、五级标准的小儿单侧先天性PUJO.肾积水患儿;经肾盂成形术后给予严格设定的补液标准,收集术后第一、三、五、七天积水侧肾脏和对侧无积水肾脏24 h尿液,分别测定排尿量、渗透压、Na+和肌酐,并用酶联免疫吸附法(ELISA)测定尿液中AQP2水平.结果 相对于对侧无积水肾脏,积水侧肾脏梗阻解除术后1周内尿液AQP2显著降低.尿量明显增多.尿渗透压显著降低,Na+排泄分数显著增加;同时术后1周内双侧肾脏尿液AQP2、排尿量和尿渗透压有升高趋势,而积水侧肾脏Na+排泄分数有减少的趋势;积水侧.肾脏术后第五、七天的尿液AQP2.排尿量和尿渗透压较第一天均显著增加(P<0.05).对侧无积水肾脏术后第五、七天的尿液AQP2和尿渗透压较第一天也显著增加(P<0.05).其余指标在术后小同天数之间差异无统计学意义(P>0.05).结论小儿单侧先天性PUJO解除术后1周内积水侧.肾脏的尿液AQP2和尿渗透压均较对侧尤积水肾脏尿液显著减少.提示梗阻解除后尿液中AQP2可能和尿液浓缩能力改变有关.     

Laparoscopic pyeloplasty is feasible for lower pole pelvi-ureteric obstruction in duplex systems

Exclusive lower pole pelvi-ureteric junction obstruction (PUJO) in double collecting systems in children is a rare condition requiring reconstructive surgery. We report on the feasibility of laparoscopic transabdominal dismembered pyeloplasty in two cases. Two children with duplicated collecting systems presented with hydronephrosis of lower pole moiety due to exclusive PUJO. Isotope renography revealed impaired drainage of affected lower kidney pole. A four-trocar transabdominal technique was used. No stent was used in one patient with bilateral duplication (male, 6 years) while a 4 Fr. double-J stent was placed laparoscopically in another with unilateral duplication (male, 9 months). Both patients had uneventful laparoscopic transabdominal dismembered pyeloplasty. The operation time was 115 and 155 min, respectively, and was comparable to our previously reported patient series undergoing laparoscopic pyeloplasty for singular collecting systems. After a mean follow-up of 21 months (range 12-42), both patients were asymptomatic and showed improved pelvi-ureteric drainage on isotope renography and improved hydronephrosis on ultrasound scan. Laparoscopic transabdominal dismembered pyeloplasty is safe and effective in children with rare lower pole PUJO in double collecting systems.     

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。     

Micturating cystourethrograms are not necessary for all cases of antenatally diagnosed hydronephrosis

ObjectiveSince 1995 we have, at our centre, adopted a selective approach to performing micturating cystourethrograms (MCUGs) on patients with antenatally diagnosed hydronephrosis. This study reviews the outcome of this policy.MethodsWe carry out MCUGs only if any of the following features are present on ultrasound: bilateral hydronephrosis, ureteric dilatation, renal scarring, bladder wall thickness greater than 5 mm, or presence of a duplex system or ureterocele. Patients with simple unilateral hydronephrosis are excluded, and are managed with 6 months’ trimethoprim prophylaxis and ultrasound surveillance with a minimum of 3 years’ follow up.ResultsFifty-five patients were referred with an antenatal diagnosis of hydronephrosis between 1999 and 2002; 26 (47%) did not have an MCUG. Of these, five had increasing hydronephrosis and required surgery for pelvi-ureteric junction obstruction, and three had a multicystic dysplastic kidney on postnatal scanning. In the remaining 18 patients, the hydronephrosis resolved spontaneously, with no renal scars or asymmetry. During follow up, none of these patients had a urinary tract infection.ConclusionWe believe that vesico-ureteric reflux in most antenatally diagnosed hydronephrotic kidneys is physiological rather than pathological, and resolves with time without causing long-term renal damage. This is a separate entity from, rather than a precursor of, the pathological symptomatic refluxing kidney in older, mainly female children. Taking a more conservative approach to the postnatal investigation of antenatally diagnosed hydronephrotic kidneys has not resulted in any missed damaged kidneys, but has reduced the number of invasive investigations performed. A careful protocol and detailed postnatal ultrasonography are important to prevent missed pathological cases.     

Congenital ureteric stenosis: a study of 17 children

Aim  To review cases of congenital ureteric stenosis treated in the period between 1999 and 2007. We propose to analyze the type of presentation, management and results. Material and methods  We report 17 children aged 20 days to 8 years with obstructive uropathy due to congenital stenosis of the ureter at one or more levels. This condition could be mistaken for the more common pelviureteric junction obstruction (PUJO) or primary megaureter, but it is a distinct and more serious anomaly. 13 of the 17 children had one or more associated anomalies, the most significant of which was a contralateral multicystic dysplastic kidney. Other associated anomalies included PUJO, megacalyx, vesicoureteric reflux, urogenital sinus, duplicate vagina, anorectal malformation and agenesis of the bladder. 16 children were symptomatic at presentation, with uremia (serum creatinine >1 mg/dl) in 5, while 1 was diagnosed antenatally. The correct preoperative diagnosis was made in only three children. Reconstruction included ureteroureteral anastomosis, ureteric reimplantation or ureteral substitution. Results  There is follow up for 15 of the 17 patients. Length of follow up ranges from 1 to 7 years (average 2.7 years). There was satisfactory urinary drainage established in all 17 cases and uremia has resolved 3 of the 5 children. The children with solitary functioning kidney are at risk of uremia in later life. Conclusion  Congenital ureteric stenosis is a rare condition, but distinct anomaly with possible grave consequence and has been distinguished from other causes of congenital ureteric obstruction.     

Can renal doppler sonography replace diuretic radionuclide renography in infants with hydronephrosis?

Reliable differentiation between obstructive and non-obstructive hydronephrosis is decisive for the further therapeutic management in infants. The results of renal Doppler sonography were compared with diuretic radionuclide renography and with the follow-up results in 33 patients (range: 21 to 98 days). In Doppler sonography, a resistive index (RI) of > 0.9 was considered to be abnormal in the sense of an obstruction. In diuretic renography, a T1/2 value (time until a 50% decrease in activity in the kidneys was observed after injection of furosemide) of >20 min was appraised as obstructive hydronephrosis. In six patients an obstructive (T1/2 >20 min) and in 27 patients a non-obstructive (T1/2 <20 min) hydronephrosis was found. All patients with obstruction in diuretic renography showed an abnormal RI (>0.9) in Doppler sonography. In addition, all patients with surgery and obstruction in diuretic radionuclide renography showed an improvement in hydronephrosis. However, seven patients had a false-positive result in Doppler sonography. All patients with non-obstruction in diuretic radionuclide renography showed no worsening of hydronephrosis without surgery in the follow-up. We found a RI of 0.84 +/- 0.07 in the non-obstructive group and of 0.96 +/- 0.05 (p = 0.018) in the obstructive group. Doppler sonography showed discrepant results compared to diuretic radionuclide renography and therefore cannot replace this method.     

Diagnostic accuracy of color Doppler sonographic study of the ureteric jets in evaluation of hydronephrosis

ObjectiveHydronephrosis (HN) and obstruction are closely associated, but upper urinary tract dilatation can occur without significant obstruction. Despite some pitfalls, conventional ultrasonography and diuretic renography (DR) are the main means of evaluation of HN in children. Recent reports have demonstrated color Doppler ultrasonography (CDUS) to reliably identify ureteric jets in the bladder. The aim of this study was to evaluate this method as a diagnostic tool to distinguish obstructive from non-obstructive dilatations of the upper tract.MethodsWe evaluated 51 patients (37 boys and 14 girls), aged 3 months to 14 years (median 4 years), who presented with unilateral grade III and IV hydronephrosis with suspicion of pyeloureteral junction obstruction. All patients underwent DR and evaluation of ureteric jets by transverse CDSG of the bladder within a maximum of 2 weeks. Obstruction was considered in the DR when the hydronephrotic unit showed a differential renal function of less than 40%, or when symptomatic intermittent renal colic was present in older children. The number of ureteric jets was counted over a 5-min period and the frequency calculated for each ureteral orifice. Relative jet frequency (RJF) was defined as frequency of the hydronephrotic side divided by total ureteric jet frequency. Receiver-operating characteristic (ROC) plots were constructed to determine the best cut-off for RJF, in order to identify renal units with obstructive hydronephrosis.ResultsTwenty-three (45.1%) hydronephrotic units were considered obstructed. The mean RJF differed between obstructive (0.09 ± 0.15) and non-obstructive hydronephrosis (0.42 ± 0.11) (p < 0.001). ROC analysis revealed that RJF <0.25 was the best threshold, and correctly discriminated obstruction in 91.2% of the children with a sensitivity of 87% (95% CI 78.6–98.2%) and specificity of 96.4% (95% CI 87.8–99%). The positive likelihood ratio was 24.3 and the area under the ROC curve was 0.92 (95% CI 0.86–0.98).ConclusionsRJF <25% was found to be a good indicator of obstruction in children with unilateral hydronephrosis. CDUS evaluation of ureteric jets is an easy and non-invasive method that can be used as an initial diagnostic tool, and in follow-up cases, to differentiate obstructed from non-obstructed hydronephrosis in the pediatric population.     

利尿性肾图在小儿肾盂输尿管连接部梗阻致肾积水诊断中的作用

目的探讨以利尿性肾图替代静脉尿路造影对小儿肾盂输尿管连接部梗阻引起的肾积水进行诊断的可能性.方法对1995年8月至2001年10月间的52例同时行静脉尿路造影和利尿性肾图检查并进行了肾盂成形术的病例进行回顾性分析,男47例,女5例,年龄0.2～13岁(平均6.5岁).结果本组52例静脉尿路造影对梗阻部位判断有52例(100%)正确;利尿性肾图检查后,52例患儿梗阻部位的判断也都(100%)正确.静脉尿路造影和利尿性肾图在梗阻部位诊断上差异没有显著性意义(P＞0.05).结论在诊断小儿肾盂输尿管连接部梗阻上,以利尿性肾图来替代静脉尿路造影是可行的.     

Treatment and outcome of prenatally detected newborn hydronephrosis

ObjectiveFor neonates with antenatally diagnosed primary ureteropelvic junction (UPJ)-type hydronephrosis, to attempt to clarify and refine criteria for establishing optimal follow-up and treatment guidelines.Patients and methodsA total of 162 newborns (228 hydronephrotic kidneys) with this condition were prospectively followed and treated by the same surgeon in 2001–2005 for a mean of 53 (13–72) months. Ultrasonography and diuretic renogram were used for diagnosis and follow up, and Onen's grading system to determine degree of hydronephrosis.ResultsOn first postnatal ultrasound, the severity of hydronephrosis was grade 1 in 152 kidneys (surgery, 0%), 2 in 41 (surgery, 19.5%), 3 in 19 (surgery, 42.1%), and 4 in 16 kidneys (surgery, 93.8%). All the grade 1 cases resolved spontaneously without renal deterioration (renal function >40%). Renal function ranged between 7% and 34% in grade 4 patients. Overall, 201 hydronephrotic kidneys (88.2%) resolved spontaneously, while 27 (11.8%) required pyeloplasty because of evidence of obstructive injury, including increased hydronephrosis in 14/27 (6/27 had less than 10% decrease in renal function), greater than 10% decrease of renal function in 9/27, and greater than 10% decrease of renal function as well as increased hydronephrosis in 4/27 patients (15%).ConclusionIn most cases, neonatal hydronephrosis is a relatively benign condition that can be followed safely by an initial non-operative approach unless there is evidence of obstructive injury. A follow-up protocol that permits early identification of a limited number of kidneys that may develop signs of obstruction and require pyeloplasty is crucial for a favorable outcome in patients with primary UPJ-type hydronephrosis. Onen's hydronephrosis grading system promises an easier and more appropriate follow up and timely treatment for children with this condition. A sufficient follow-up interval, especially during the first 3 years of life, is essential to help prevent permanent loss of renal function in kidneys that do develop signs of obstruction.