Background: Pediatric non-rhabdomyosarcoma bladder tumors (NRBTs) encompass a diverse spectrum of tumors with low incidence rates. This study aims to improve understanding and contribute to the management of pediatric NRBTs. Methods: We retrospectively enrolled patients with primary pediatric NRBTs diagnosed and treated at our center between 2003 and 2023. Clinicodemographic and clinicopathological data were collected. NRBTs were classified into four categories: urothelial tumors (UTs), mesenchymal tumors (MTs), neuroendocrine tumors (NTs), and rare types of bladder tumors (rBTs). Intergroup comparative analyses were conducted. Results: We identified 51 cases of NRBTs. Four categories encompassed: UTs (n=25, 49.0%), MTs (n=17, 33.3%) and NTs (n=5, 9.8%), and rBTs (n=4, 7.8%). No cases of UTs were identified below the age of 3 years. Comparative analyses demonstrated significant intergroup differences in preoperative anemia prevalence (P=0.02) and tumor size (P<0.001). Total 45 patients (88.2%) completed follow-up, with a median duration of 64 months [interquartile range (IQR), 28-113 months]. Local recurrences were identified in 5 cases (11.1%), among whom 1 case (2.0%) succumbed to local recurrence and disease progression. Conclusions: Pediatric NRBTs demonstrate generally favorable clinical outcomes. Age under 3 years, preoperative anemia or a maximum tumor diameter exceeding 3 cm should raise a strong suspicion for non-UTs bladder tumors. Postoperative surveillance protocols can incorporate urinary ultrasonography (US) as a non-invasive monitoring tool.

This study aims to evaluate the feasibility, safety and efficacy of robotic assisted laparoscopic pyeloplasty (RALP) with the novel SHURUI single-port (SR-SP) robotic surgical platform in children. Between November 2023 and April 2024, 20 pediatric patients diagnosed with ureteropelvic junction obstruction (UPJO) underwent transperitoneal RALP with the SR-SP surgical platform. Baseline characteristics, perioperative parameters and follow-up data were collected and analyzed. All surgical procedures were successfully performed without conversion to open or laparoscopic surgery. The median docking time, console time, and operative time were 3.5 min (range 3.5–6.0), 144.5 min (range 88.0–290.0), and 221.5 min (range 136.0–450.0), respectively. The median estimated blood loss was 5 ml (range 2–20) and the median length of hospital stays was 6 days (range 4–24). Through trans-umbilical incision, RALP was feasible in patients with a height of no less than 114 cm. The minimum incision length was 2 cm. No intraoperative complication was identified. Two cases were identified with the Clavien–Dindo Grade II postoperative complications and one with the Clavien–Dindo Grade III complications. The surgical success rate was 95

目的 探讨尿动力学(urodynamics,UDS)检查评估无神经系统及下尿路器质性病变的膀胱输尿管反流(vesicoureteral reflux,VUR)患儿下尿路功能障碍(lower urinary tract dysfunction,LUTD)的临床意义.方法 回顾性分析2017年1月至2021年12月首都医科大学附属北京儿童医院收治的106例VUR患儿UDS检查结果,按照有无LUTD将患儿分为有LUTD组(77例)和无LUTD组(29例),比较两组患儿膀胱容量、残余尿量(post voiding residual urine volume,PVR)以及最大尿流率等尿动力学指标.结果 有LUTD组残余尿量97.71±93.17 mL,明显高于无LUTD组11.81±9.17 mL(t=5.564,P=0.008);有 LUTD 组最大尿流率 8.82±5.01 mL/s,明显低于无 LUTD 组 15.04±7.75 mL/s(t=6.314,P=0.030).有LUTD组中,29例为低级别VUR(Ⅰ～Ⅲ级),48例为高级别VUR(Ⅳ～Ⅴ级).低级别VUR患儿中3例膀胱顺应性减低,高级别VUR患儿中19例顺应性减低,两者比较(x2=7.537,P=0.006)差异有统计学意义;低级别VUR患儿中7例PVR增加,高级别VUR患儿中23例PVR增加,两者比较(x2=4.298,t=0.038)差异有统计学意义.特发性逼尿肌过度活动(idiopathic detrusor overactivity disorder,IDOD)35例(35/77,45.5％),其中19例为低级别VUR,占低级别VUR总数的65.5％(19/29);逼尿肌括约肌协同失调(dysfunctional voiding,DV)33例,其中27例为高级别VUR,占高级别VUR总数的56.3％(27/48).结论 PVR增多、最大尿流率降低提示VUR患儿存在LUTD的可能.高级别VUR患儿中,PVR增加更为明显、且膀胱顺应性更差.IDOD在低级别VUR中常见,DV在高级别VUR中更为常见.

目的 探讨儿童肾盏憩室(calyceal diverticulum,CD)的临床特点、诊断及手术治疗方式.方法 回顾性分析2010年1月至2021年10月首都医科大学附属北京儿童医院泌尿外科与包头市第四医院小儿外科收治的7例肾盏憩室患儿临床资料,其中男5例,女2例;就诊年龄8个月至11岁,平均年龄3岁11个月.憩室位于左侧3例,右侧4例;肾上盏4例,肾中盏3例.憩室长径3～9cm,平均6.2 cm.产前超声发现肾脏囊性变3例,腹痛2例,无症状腹部包块1例,体检发现肾脏囊性病变合并高血压1例.3例术前经泌尿系超声检查确诊,4例术中确诊.7例均行手术治疗,其中开放肾盏憩室切除术2例,肾盏憩室剪裁+盏颈扩大术3例,开放和腹腔镜憩室壁翻瓣卷管输尿管端侧吻合术各1例.结果 7例均获随访,随访时间3个月至10年,患儿术后CD相关症状均消失,术前扩张的肾盏憩室均明显缩小,无一例复发;1例合并肾积水患儿术后积水明显减轻,但肾功能恢复较差,肾核素扫描显示分肾功能为20％,目前仍在随访中.结论 肾盏憩室易发生反复泌尿系感染、慢性腹痛、肉眼血尿、结石等,易误诊为单纯性肾囊肿,两者处理方式不同,需加以区分.应根据憩室位置、盏颈狭窄程度采取个体化治疗,包括肾盏憩室切除、盏颈扩大部分憩室切除、憩室壁翻瓣卷管输尿管端侧吻合术.如处理得当通常预后较好,少有复发.

Background Hypospadias is a congenital anomaly of the male urogenital system. Genetics factors play an important role in its pathogenesis. To search for potential causal genes/variants for hypospadias, we performed exome sequencing in a pedigree with three patients across two generations and a cohort of 49 sporadic patients with hypospadias. Results A novel BRAF variant (NM_004333.6: c.362C > A) was found to co-segregate with the hypospadias phenotype in the disease pedigree. In cells overexpressing the BRAF mutant, the phosphorylation level of p38 MAPK was significantly increased as compared with the cells overexpressing the wild-type BRAF or RASopathy-related BRAF mutant. This variant further led to a reduced transcription level of the SRY gene, which is essential for the normal development of the male reproductive system. In the cohort of sporadic patients, we identified two additional variants in p38 MAPK signaling-related genes ( TRIM67 and DAB2IP ) potentially associated with hypospadias. Conclusion Our study expands the phenotypic spectrum of variants affecting p38 MAPK signaling toward the involvement of hypospadias.

目的 了解单侧肾盂输尿管连接部梗阻(ureteropelvic junction obstruction,UPJO)手术后短期患侧分肾功能(differential renal function,DRF)缓解效果. 方法 选择2019年3月至2019年10月首都医科大学附属北京儿童医院收治的单侧UPJO行肾盂成形术患者50例为研究对象,其中男37例,女13例,手术前后均行利尿性肾动态显像检查.根据术前DRF情况分为术前DRF降低组(DRF＜40％)、术前DRF正常组(40％ ＜DRF＜55％)和术前DRF超常组(DRF＞ 55％).分别对比各组手术前后患侧DRF及肾实质厚度(renal parenchymal thickness,PT)变化情况,进一步对比组间DRF缓解情况及术后DRF水平. 结果 三组患者手术年龄、方式及术后DRF差异具有统计学意义(P＜0.05);在术前DRF降低组及超常组患者中,手术前后DRF差异存在统计学意义(P＜0.05),术前DRF正常组患者手术前后DRF无明显变化,但肾实质厚度存在明显差异(P＜0.05).以术后DRF增加≥5％作为术后肾功能缓解标准(超常肾功能患者以术后DRF减少≥5％作为术后肾功能缓解标准),术前DRF不同组缓解率差异存在统计学意义(P＜0.05),术前DRF降低组患者术后肾功能水平仍低于术前DRF正常组及术前DRF超常组患者(P＜0.05). 结论 术前DRF＜ 40％的单侧UPJO患者术后短期DRF缓解显著,但其术后DRF低于术前DRF正常及超常患者.

Inflammatory myofibroblastic tumor (IMT) is a rare tumor with an indolent course. It is less often reported as a second tumor that occurs after treatment of malignant tumors in pediatric patients. Here, we report a case of IMT following Wilms tumor (WT), and conduct a literature review concerning IMTs and WT to evaluate the diagnostic possibility of IMT as a second tumor. The coexistence of the 2 tumors may cause confusion as to whether they share genetic links or that IMTs may appear as late effects of the treatment of WT.

Urethral fistula caused by anterior urethral valve combined with penile curvature is a rare clinical disease, which is rarely reported at home and abroad. We diagnosed 2 cases, treated with urethral diverticulum resection + urethral valve resection + dorsal albuginea of the penis + Duplay one-stage urethroplasty and urethral diverticulum resection + urethral valve resection + penis Dorsal albuginea fold + Duckett one-stage urethroplasty respectively. There were no surgical complications such as penile recurvation, urinary fistula, urethral stricture or urethral diverticulum, during the follow-up period of 10 and 15 months.

Background: Contrast-enhanced ultrasonography (CEUS) is a new potential modality for the quantitative evaluation of the microvascular perfusion of renal parenchyma.Objective: To prospectively evaluate the diagnostic value of CEUS in assessing renal function in patients with ureteropelvic junction obstruction (UPJO).Methods: The study protocol was approved by the ethics committee of Beijing Children’s Hospital (Beijing,China), and written informed consent was obtained from the patients’ parents or guardians. Ultrasonography (US), CEUS, and radioisotope renography were performed in 51 children (42 boys, 9 girls; mean age, 6.75 ± 4.14 years) with unilateral UPJO. The slope of the ascending curve (A), time to peak (TTP), peak intensity (PI), and area under the curve (AUC) were recorded during CEUS; the quantitative data were calculated by the QLAB system (semiautomated border tracking, Philips Healthcare) software. Sensitivity and specificity values were determined for CEUS and compared with radioisotope renography.Results: A total of 102 kidneys in 51 patients were depicted by CEUS and the perfusion time-intensity curve (TIC).CEUS depicted 102 kidneys in 51 patients, in whom the perfusion time-intensity curve（TIC）was determined. The TIC of renal cortical perfusion in all groups showed an asymmetrical single-peak curve, which could be clearly distinguished between the experimental group and the control group. Compared to the control group, the TTP was markedly prolonged but A was significantly decreased in the experimental group (P < 0.05). There was no significant correlation between AUC, PI and DFR，but the correlation coefficient between TTP, A and DFR remained significant (p<0.001).The receiver operating characteristic （ROC）curve drawn to differentiate the differential renal function (DRF) using the TTP value provided an area under the ROC curve (AUROC) of 0.86. The diagnostic performance of contrast-enhanced US was better than that of US, as the sensitivity and specificity values were 92.86% and 76.14%, respectively.Conclusions: This preliminary experience represents the first report of evaluating the diagnostic value of CEUS in assessing renal function in children with UPJO. CEUS is a highly sensitive, rapid, and diagnostic imaging modality for detecting and monitoring renal function noninvasively.

Objective:To summarize the characteristics and experience of diagnosing and treating pediatric paratesticle rhabdomyosarcoma(RMS), to enhance the understanding of pediatric paratesticle rhabdomyosarcoma and to emphasize the importance of accurate preoperative recognition of the disease and correct surgical approaches.Methods:From January 2008 to December 2018, clinical data were retrospectively reviewed for 21 RMS children at both hospitals.The involved side was left(n=13, 61.9%)and right(n=8, 38.1%). According to whether or not there was a previous surgical history, they were divided into two groups of A(no previous surgical history, n=12)and B(previous surgical history at other medical institutions, n=9). The mean ages of groups A and B were(77.19±56.38)and(99.4±17.4)months respectively.In group A, the involved side was left(n=7)and right(n=5); In group B, the involved side was left(n=5)and right(n=4). Clinical characteristics, pathological types, diagnosis and treatment methods and therapeutic outcomes of two groups were analyzed.SPSS statistical software was utilized for data processing.The measurement data were expressed as mean±standard deviation.Chi-square test was employed for counting data, Kaplan-Meier curve for survival analysis and Log-rank test for comparing survival difference between two groups.Results:Group A underwent a high inguinal approach.Nine cases in group B were initially operated via a scrotal approach and followed by extended resection via a high inguinal approach.The mean follow-up periods of groups A and B were(99.33±7.34)(14-122)and(67.75±11.81)(6-87)months respectively.The overall survival rate was 90.9%(10/11)in group A and 75.0%(6/8)in group B without significant inter-group statistical difference( P=0.28). The progression-free survival rate was 90.9%(10/11)in group A and 50%(4/8)in group B with significant inter-group statistical significance( P=0.028). Conclusions:Preoperative diagnosis and selecting correct surgical approaches for pediatric RMS are still challenging for pediatric urologists.As an initial treatment option, incomplete scrotal resection significantly elevates the risks of local tumor recurrence and lymph node metastasis.Therefore remedial surgery should be actively performed for expanded resection.It is also imperative to strengthen the management of high-risk children and improve the survival rate as much as possible.

INTRODUCTION:Laparoscopic pyeloplasty (LP) has been widely used in the treatment of pediatric ureteropelvic junction obstruction (UPJO). However, no prior reports with a large pediatric series have focused on the analysis of complications and impact factors of the outcomes. We hypothesized there were risk factors of higher Clavien grade postoperative complications. OBJECTIVE:To analyze the characteristics of complications and risk factors of high Clavien grade postoperative complications. PATIENTS AND METHODS:All children with UPJO treated with primary transperitoneal LP between July 2016 and July 2018 were retrospectively reviewed. The Clavien complication grades in groups with different weight, intraoperative complication (drainage methods), anteroposterior pelvic diameters (APPD), side, gender, title of surgeon, preoperative presentation and obstruction reason were compared. RESULTS:Of the 279 children, intraoperative complications in which the placement of double-J stents was not accomplished and conversion to open surgery (Satava grade II) occurred in 17 (6.09%) and 2 (0.72%) patients, respectively. A total of 270 patients (277 kidneys) were included in the analysis of postoperative complications. Postoperative complications occurred in 51 (18.89%) patients. The most frequent postoperative incident was febrile UTI in 27 patients. Of the 13 patients who required reoperations, 6 patients had kidney restenosis and were considered as failure of surgery. All complications with an exact onset time occurred within 10 months after surgery. In the univariate and multivariate analysis, weight <10 kg and having intraoperative complication with nephrostomy tube were risk factors of higher Clavien postoperative complication grade (P＜0.05). DISCUSSION:Patients who were <10 kg in weight and having intraoperative complication with nephrostomy tube had a greater risk of a higher Clavien grade postoperative complication. To reduce high Clavien grade postoperative complications, asymptomatic patients under 10 kg in weight and having intraoperative complication with nephrostomy tube need close monitoring. In the 26 patients who had an exact time of the postoperative complications, the longest time we found was 10 months. Thus, we recommend the follow-up time required to observe postoperative complications in patients should be at least 10 months after surgery. CONCLUSIONS:LP has been proven to be safe and effective in children with a low rate of complications. Weight <10 kg and having intraoperative complications with nephrostomy tube were risk factors of higher Clavien grade postoperative complications. Children with low weight and intraoperative complications need more attention in terms of the occurrence of complications.

Background: Ptch1 and Ptch2 are expressed in tubular epithelium and stromal cells adjacent to the UPJ. They mediate inhibition of Smoothened, a transmembrane protein expressed on the cell surface. If the pathway is disturbed, UPJOcan occur. This aim study aimed to determine the expression of Ptch1 (P1) and Ptch2 (P2) in stenotic segments in children with congenital ureteropelvic junction obstruction (UPJO) compared with normal control subjects. Methods: Stenotic segments of ureter tissues were obtained from 20 UPJO patients.UPJO caused by other pathogenies, such as vessel and ureteral polyps, were excluded. The control ureter specimens were obtained from 10 patients with Wilm’s tumor, and the tissues were confirmed histologically to be unaffected. Immunofluorescence, western blot and real-time PCR were used to investigate the expression of P1 and P2. Statistical methods were used to find the differences between the two groupsResults: P1 and P2 were identified in the cytoplasm of smooth muscle in two groups through immunohistochemistry. However, there were no statistical differences between the two groups in P1 and P2 with immunohistochemistry (P=0.31 and P=0.3, respectively). There were also no statistical differences with western blot (P=0.75 and P=0.9, respectively) and real-time PCR (P=0.52 and P=0.45, respectively). However, with the immunofluorescence it was found that red-stained P1 were diffused in the controls group, but were mainly located in the intracellular perinuclear compartment of smooth muscle cells in UPJO. Conclusions: The expression of P1 and P2 between the two groups had no statistical significant. P1 were mainly located in the intracellular perinuclear compartment of smooth muscle cells in UPJO. The P1 pathway might be disturbed by the abnormal distribution rather than the quantity, which might be one probable pathogenesis of UPJO.

Objective:To explore the risk factors of postoperative SRF failure to recover at Month 6 after Anderson-Hynes procedure in children with unilateral SFU(Society of Fetal Urology)Ⅳ ureteropelvic junction obstruction (UPJO) of preoperative SRF (split renal function) <40%.Methods:Retrospective reviews were performed for 146 children with unilateral SFU IV UPJO of preoperative SRF<40% undergoing Anderson-Hynes pyeloplasty at four children's clinical centers from January 1, 2016 to December 31, 2018. There were 126 boys and 20 girls. And the invoved side was left (82.2%, 120/146) and right (17.8%, 26/146); the median operative age 5.5(2.0-38.0) months; the median preoperative anteroposterior diameter (APD) 32.4(24.5-43.3) mm; the median preoperative SRF 31.4%(22.7%-36.2%). And 29(19.9%) of them had clinical preoperative symptoms. Personal demographics, preoperative and surgical approaches data were recorded.Results:In 94(64.4%) of them, postoperative SRF failed to recovery at Month 6 post-pyeloplasty. The independent risk factors for postoperative SRF failure to recovery at Month 6 were operatve age ( OR=1.014, P=0.035) and preoperative SRF ( OR=0.958, P=0.035). The OR values of operative age (≥8.1 vs <8.1 months) and preoperative SRF (<22.1% vs ≥22.1%) were 2.619 ( P=0.012) and 4.907( P=0.006) respectively. Conclusions:Preoperative SRF and operative age are independent risk factors for postoperative SRF failure to recover post-pyeloplasty. The risk of SRF failure to recover post-pyeloplasty is higher in children with operative age (≥8.1 months) and preoperative SRF (<22.1%).

目的 通过多中心回顾性队列研究,对不同术前分肾功能(split renal function,SRF)的单侧美国胎儿泌尿外科学会(Society of Fetal Urology,SFU)Ⅳ度肾盂输尿管交界处梗阻患儿术后疗效进行分析. 方法 以2016年1月1日至2018年12月31日于我国四个儿童临床中心接受Anderson-Hynes手术治疗的单侧SFUⅣ度肾盂输尿管交界处梗阻患儿281例(进行倾向性匹配后最终纳入214例)为研究对象,并将入选患儿分为术前SRF≥40％组与术前SRF＜ 40％组.对患儿人口学资料,术前检查、手术方式、术后半年肾脏形态恢复、术后半年肾脏功能恢复以及术后并发症发生情况等资料进行统计学分析. 结果 术前SRF≥40％组与术前SRF＜ 40％组的患儿相比,术后半年SRF更好(48.8％vs.42.1％,P＜0.001),术后SRF恢复正常的比例更高(75.7％ vs.35.5％,P＜0.001),但术后半年SRF与术前相比恢复程度较低(1.6％ vs.9.0％,P＜0.001). 结论 本次多中心回顾性队列研究发现,虽然术前SRF≥40％患儿术后半年SRF与术前相比恢复程度较低,但术后半年SRF更好,且术后半年SRF恢复正常患儿的比例更高,建议单侧SFUⅣ度肾盂输尿管交界处梗阻患儿SRF≥40％且出现临床症状或积水加重时应行手术治疗,而不要待SRF降至40％以下后再行手术治疗.

Objective:To explore the pathogenesis and clinical managements of intermittent hydronephrosis secondary to ureteropelvic junction obstruction (UPJO).Methods:The retrospective study was based upon medical records from January 2006 to August 2018. Intermittent hydronephrosis was defined as intermittent UPJO presenting with abdominal pain. UPJO with renal dysplasia, multiple ureteral strictures, urinary reflux, urinary calculi and iatrogenic cause were excluded. Their clinical manifestations, attack preventions and imaging findings were analyzed.Results:The clinical data of 79 children were recorded. The mean operative age was 5.4(2.5-16) years. There were 58 boys and 21 girls. The causes were intrinsic stenosis (n=37), extrinsic vessel (n=29), horseshoe kidney (n=13) and ureteral polyp (n=7). All presented with acute abdominal pain of perinephric (n=53, 67.1%), navel (n=15, 19.0%) and indescribable (n=11, 13.9%). The history of inducement was acquired in 34 cases (43.0%). The major clinical manifestations were nausea & vomiting (n=66, 83.5%), abdominal mass (n=8, 10.1%) and hematuria (n=5, 6.3%). Ureter lumens were stenotic (n=44, 55.7%) and non-stenotic (n=35, 44.3%). Thirty-one cases (39.2%) stayed in remission. There were 48 cases (60.8%) in remission and 79 cases (100%) in the period of onset with SFU III~IV. The anteroposterior diameters of pelvis were significantly higher in the period of onset ( P<0.05). However, no inter-group difference exited in renal parenchyma thickness between two periods ( P>0.05). The cases of T 1/2 >20 min were significantly higher in the period of onset ( P<0.05). Open and laparoscopic surgeries were performed in 19 and 60 cases respectively. The follow-up period was 6-18 months and the success rate reached 94.7%(71/75). Pathology revealed intraluminal stenosis (n=44, 55.7%) and non-stenosis (n=35, 44.3%). Conclusions:Intermittent UPJO should be diagnosed in conjunctions with disease history. Immediate or serial ultrasonography is valuable during two periods. Diagnosis is difficult in remission. During the period of onset, UPJO may be diagnosed with IVP and diuretic nucleotide scan.

AIM:By observing the expression and distribution of platelet-derived growth factor receptor α-positive (PDGFRα+) cells in ureteropelvic junction obstruction (UPJO), to explore their role in the pathogenesis of children with congenital hydronephrosis. MATERIALS AND METHODS:The control group involved specimens of the normal ureter (nephrectomy for tumor; n = 10), and the UPJO group contained specimens of ureteropelvic junction (UPJ) segment excised during pyeloplasty (n = 30). The specimens were investigated using immunofluorescence for the expression and distribution of PDGFRα+ cells in each group by light microscopy with computerized image analysis. Real-time PCR (RT-PCR) was used to study PDGFRα gene expression levels. In addition, small conductance calcium-activated potassium channel 3 (SK3) and closely associated cells consisting of smooth muscle cells (SMCs), interstitial cells of Cajal (ICCs), and nerve fibers were investigated. RESULTS:PDGFRα+ cells were in close proximity to SMCs, ICCs, and nerve fibers. PDGFRα+ cells expressed SK3 channels, which are found to regulate purinergic inhibitory neurotransmission in SMCs. Regarding the expression of PDGFRα+ cells no significant difference was seen between the two groups, while the expression of SK3 channels in PDGFRα+ cells was significantly decreased in the UPJO group versus the control group. CONCLUSION:This study identified the expression of PDGFRα+ cells in the human UPJ. Our results demonstrate the expression of SK3 channels in PDGFRα+ cells was decreased in UPJO, and SK3 channels may be involved in the pathogenesis of UPJO by perturbing the UPJ peristalsis.

PURPOSE To present the clinicopathological characteristics and outcome of children with bladder and ureteral inflammatory myofibroblastic tumors (IMTs) in our center. METHODS We reviewed the medical records of patients with bladder and ureteral IMTs from 2010 to 2018. We recorded patients' demographic data, presentation, hemoglobin level, presence of hydronephrosis, tumor size, treatment, and outcomes. RESULTS Eight patients with bladder IMTs and 3 with ureteral IMTs were treated at our center during this period. The mean age was 7.1 years. Four patients presented with anemia at diagnosis with the mean hemoglobin level 84.5 g/L. Among patients with bladder IMTs, 5 were male and 3 were female. The most common symptom was lower urinary symptoms in 6 patients, followed by hematuria in 4 patients. 2 patients had complications of hydronephrosis and hydroureter. Among patients with ureteral IMTs, 2 were male and one was female. The most common symptom was abdominal pain, and 3 patients presented with upper urinary tract dilation. All patients underwent surgery. A total of 81.8% were positive for anaplastic lymphoma kinase. Cytokeratin (CK) expression was present in all patients with bladder IMTs, while it was negative in 2 patients with ureteral IMTs. During mean follow-up of 43.4 months, all patients survived event-free. CONCLUSION The presence of hydronephrosis and hydroureter is rare in patients with bladder IMTs. Anemia caused by hematuria should be raised the index of suspicion for IMTs. Children with bladder and ureteral IMTs had excellent prognosis. The expression pattern of CK varied between bladder and ureteral IMTs. (C) 2020 Elsevier Inc.

明遗民画家石涛北游京城三年,以精湛的画艺流连于达官贵人之间,却再未得到皇帝垂青,终南返扬州.这样的结局是北游前已受到康熙礼遇的石涛始料未及的.石涛北游受挫,惨淡南归的结局是多种因素导致的,当时京城紧张的政治文化环境造成遗民士绅处境艰难,石涛作为明宗室遗民的贵胄心理也使其难以进一步屈从于清初的文化正统,而他狂傲的艺术个性又为官方画派所不容.石涛"欲问皇家问赏心,好从宝绘通知遇"希冀的破灭,看似是时运不济所致,其实偶然中早已蕴含着必然.

ABSTRACT Introduction: Transverse preputial island flap urethroplasty (TPIFU) is one of the most frequently performed technique for single-stage repair in proximal hypospadias. It was reported that the subepithelial urethroplasty would obviously decrease urethrocutaneous fistula (UF) complication after proximal TIP. But in the process of TPIFU, it had not been reported yet. Objective: We reviewed our experience to evaluate and compare the effect of continuous eversion suture (CES) versus continuous inversion subepithelial suture (CIS) on complication rates in the TPIFU. Material and methods: A retrospective review of all patients operated with CES and CIS in our institution between January 2017 and Jun 2017 was performed. Results: A total of 161 patients were enrolled in the research. Patients were followed up for 12~17 months. Total success rate was 73.9% (119/161). No statistically difference was found between the two groups with regard to age of patients (P=0.097), catheter size (P=0.52), time of catheterization (P=0.47), length of neourethra (P=0.20), non-urethral comorbidity (P=0.44) and post-operative infection (P=1.0). The overall postoperative complications had no statistically difference between the two groups (P=0.067). There were no statistically significant differences in the incidence of urethra-cutaneous fistula (UF) (OR=0.07, 95% CI: -0.24~0.037, P=0.22), urethral diverticulum (UD) (OR=0.026, 95% CI: -0.16~-0.056, P=0.323), urethral stricture (US) (OR=0.081, 95% CI: -0.15~0.15, P=1.0) and breakdown of urethral repair (BU) (OR=0.02, 95% CI: -0.118~-0.044, P=1.0). Discussion: The comparison of two group’s postoperative complications was feasible because there were no statistically differences among perioperative variables. It seemed as if continuous inversion subepithelial suture would promote healing. However, it indicated that the overall success rate and the incidences of UF, UD, US and BU complications had no statistically difference between groups. It might be accounted for the subtle differences of techniques changing the process of establishing prime and side branches vascularization. Conclusions: The CIS technique had no significantly different effect on the four complications rates when compared with CES in TPIFU. Thus, CES and CIS could be randomly adopted in TPIFU as personal preference.

目的 探讨前尿道瓣膜切除术后造成上尿路持续损害的尿动力学危险因素. 方法 回顾性分析2007年1月至2020年1月26例前尿道瓣膜切除术后患儿的临床资料,平均年龄3.4岁(5个月至14岁).瓣膜切除术后4个月至12.5年,平均5.5年.患儿术后均进行尿动力学检查.手术前后均进行血生化(包括尿素氮、肌酐)检查、泌尿系统B超检查、静脉肾脏造影(intravenous pyelography,IVP)和排尿性膀胱尿道造影(voiding cystourethrogram,VCUG).比较瓣膜切除前后肾和输尿管积水以及膀胱输尿管反流情况,分析造成前尿道瓣膜术后上尿路持续损害的危险因素. 结果 前尿道瓣膜切除手术前肾和输尿管积水患儿共15例24侧,占57.7％ (15/26);膀胱输尿管反流8例11侧,占30.8％ (8/26).瓣膜切除术后有5例7侧肾和输尿管积水消失,占19.2％ (5/26);2例3侧膀胱输尿管反流消失.瓣膜切除术后肾和输尿管积水患儿共10例17侧,占38.5％ (10/26);膀胱输尿管反流6例8侧,占23.1％ (6/26).有7例11侧肾和输尿管积水较术前加重,占26.9％ (7/26),其中4例6侧膀胱输尿管反流较术前加重.根据术后肾和输尿管积水以及膀胱输尿管反流恢复情况,分为上尿路损害加重组(7例)和上尿路损害减轻或消失组(19例).行瓣膜切除术后尿动力学检查发现,在上尿路损害加重组7例患儿中,压力流率图显示5例依然存在下尿路梗阻或可疑梗阻.上尿路损害减轻或消失组19例患儿压力流率图均显示无梗阻(P＜0.05);两组最大尿流率平均值、膀胱顺应性、排尿期最大逼尿肌压力值差异均存在统计学意义(P＜0.05);上尿路损害加重组中5例动态VUCG显示排尿时膀胱颈全程开放不全,行膀胱尿道镜检查发现3例膀胱壁增厚,呈小梁样改变和膀胱假性憩室形成,尤其膀胱基底以及膀胱内口附近组织明显增厚. 结论 前尿道瓣膜是一种罕见的下尿路梗阻性疾病,瓣膜切除之后上尿路损害仍然会持续存在或加重,可能与患儿异常的膀胱功能有关.排尿期最大逼尿肌压力升高、最大尿流率低、膀胱顺应性低以及压力流率图显示梗阻仍存在是导致膀胱功能异常的尿动力学危险因素,可能与前尿道瓣膜患儿同时存在膀胱颈部功能与结构的异常有关.