目的 评估生物反馈训练联合神经电刺激对严重功能性排尿障碍儿童的治疗效果.方法 2016年12月至2018年12月,中国医科大学附属盛京医院小儿泌尿外科对收治的28例6～15岁传统治疗无效的功能性排尿障碍儿童进行生物反馈联合电刺激治疗,其中女童12例,男童16例.主要症状为日间遗尿(89.2％),尿急迫(82.1％),夜间遗尿(39.4％).尿流动力学检查结果显示,膀胱过度活动和逼尿肌括约肌协调障碍分别占92.9％和71％.在治疗前教育的基础上,采用无创会阴表面电极进行生物反馈结合神经电刺激疗法对上述患儿进行治疗,一个疗程2周,共20次.在治疗期间和治疗后,指导患儿进行如厕和盆底训练. 结果 末次治疗6个月后随访结果:日遗尿患儿治愈率40％,好转率46.4％;夜间遗尿患儿治愈率18％,好转率54.5％.治疗12个月后随访结果:日遗尿患儿治愈率56.5％,好转率39.2％,夜间遗尿患儿治愈率和好转率均为36.4％. 结论 短期高频度针对盆底功能训练的生物反馈联合电刺激治疗学龄期重症DV无创、有效,可考虑对该疗法的疗效进行大规模随机对照研究,并制定相应的规范治疗程序.

Objective To screen the differential proteins in neonatal rat kidney at different timepoint with partial unilateral ureteral obstruction (PUUO) and verify the expression of PRDX1 in animal model and clinical hydronephrosis.Methods Two-dimensional gel electrophoresis (2-DE) and MALDI-TOF/TOF MS were employed for separating and identifying differential proteins in groups of PUUO and corresponding sham kidney tissues.Western blot and enzyme-linked immunosorbent assay (ELISA) were used for verifying the protein expression of PRDX1 in rat kidney and clinical urine samples.Results Forty-three differential proteins whose expression levels increased or decreased ＞2-fold identified by peptide mass fingerprinting were involved in regulation of actin,energy metabolism and injuries of mitochondrion,oxidative stress and endoplasmic reticulum stress,et al.In neonatal rat kidney,higher expression of PRDX1 protein in PUUO1d,PUUO 2 d and PUUO 5 d (0.836 1 ± 0.017,0.541 9 ± 0.035,0.548 8 ± 0.021 respectively) were detected by Western blot.In clinical urine samples,the expressions of PRDX1/cr.were up-regulated in hydronephrosis and decreased significantly within 1 month postoperatively (0.119 ± 0.056,0.072 ± 0.034 respectively).Conclusions Forty-three differentially expressed proteins associated with genesis and development of obstructive nephropathy have been successfully screened from PUUO and corresponding sham kidney tissues.And PRDX1 might be involved in the development and progression of obstructive nephropathy.

Objective To improve the understanding of the implications of testicular microlithiasis(TM)in paediatric patients by summarizing the clinical characteristics of TM. Methods One hundred and eighteen boys diag-nosed as TM based on ultrasound between March 2006 and December 2014 at Shengjing Hospital Affiliated to China Medical University were retrospectively reviewed. Demographic data,imaging data,indications for ultrasound,associated inguinoscrotal pathologies and follow - up data were collected. Results There were 118 cases of patients aged from 0. 25 to 14. 00 years(averaging 7. 98 years). The indication for ultrasound included health check(22 cases),testicular discomfort(28 cases),small testes(6 cases),scrotal mass(10 cases),with history of cryptorchidism(36 cases),with history of hydrocele(15 cases). Concomitant diseases included congenital anomaly of testis in 44 cases[cryptorchidism (34 cases),testicular absence(4 cases),testicular hypoplasia(6 cases)],hydrocele of tunica vaginalis or hernia(15 cases),testicular epididymitis(12 cases),varicocele(6 cases),epididymal cyst(5 cases),testicular teratomas(2 ca-ses),adrenal cortical hyperplasia(1 case),testicular torsion(1 case),and none associated disease(32 cases). Bilateral TM was in 87 cases(the typical TM in 38 cases,limited TM in 49 cases),while unilateral TM was in 31 cases(the typi-cal TM in 8 cases,limited TM in 23 cases). Fifty - three cases were followed up for 1 month to 36 months(averaging 12. 5 months),and the condition was stable,no testicular malignancy was observed during follow - up. Conclusions The TM in children is not a rare disease. The etiology of TM is not clear and there are no special clinical symptoms. TM is often associated with testicular abnormality in pediatric patients and the correlation between children′s TM and adult testicular tumor can not be determined yet. The distribution and involving degree of TM is stable in the short term with infrequent testiculoma,but an appropriate follow - up scheme is required,especially during and after puberty.

Partial deletions on the long arm of chromosome 13 lead to a number of different phenotypes depending on the size and position of the deleted region. The present study investigated 2 patients with 13q terminal (13qter) deletion syndrome, which manifested as anal atresia with rectoperineal fistula, complex type congenital heart disease, esophageal hiatus hernia with gastroesophageal reflux, facial anomalies and developmental and mental retardation. Array comparative genomic hybridization identified 2 regions of deletion on chromosome 13q31-qter; 20.38 Mb in 13q31.3-qter and 12.99 Mb in 13q33.1-qter in patients 1 and 2, respectively. Comparisons between the results observed in the present study and those obtained from patients in previous studies indicate that the gene encoding ephrin B2 (EFNB2) located in the 13q33.3-q34 region, and the gene coding for endothelin receptor type B, in the 13q22.1–31.3 region, may be suitable candidate genes for the observed urogenital/anorectal anomalies. In addition, the microRNA-17-92a-1 cluster host gene and the glypican 6 gene in the 13q31.3 region, as well as EFNB2 and the collagen type IV a1 chain (COL4A1) and COL4A2 genes in the 13q33.1-q34 region may together contribute to cardiovascular disease development. It is therefore possible that these genes may be involved in the pathogenesis of complex type congenital heart disease in patients with 13q deletion syndrome.

目的 总结分析新生儿睾丸扭转(neonatal testicular torsion,NTT)的临床特点及治疗措施.方法 回顾性分析2009年1月至2014年12月中国医科大学附属盛京医院收治的13例NTT病例.通过医院的医疗信息管理系统,收集这些病例的临床资料和随访情况等,对这些NTT患儿的临床特点和治疗情况进行描述性分析.结果(1)13例日龄为1~28 d,平均8.2 d;左侧8例,右侧4例,双侧1例;急症型7例,非急症型6例.(2)急症型7例存在阴囊急症表现,彩超显示患侧睾丸血运异常,术前均诊断为NTT;5例手术(探查6睾,切除5睾,其中1例为双侧),2例拒绝手术.4例单侧睾丸扭转证实已发生睾丸坏死,切除后,对侧睾丸行预防性固定;1例为双侧睾丸扭转,但扭转程度不同,保留相对病变较轻侧睾丸.所有切除睾丸术后病理检查显示睾丸组织结构基本消失,大部分呈现出血伴坏死改变,其间可见少量残存的生精小管和输精管.(3)非急症型6例,无阴囊急症表现,彩超显示患侧阴囊内异常包块,3例MRI可见混杂信号包块.术前3例诊断为睾丸肿瘤,3例诊断为NTT.6例均行手术治疗.术中见患侧阴囊内无正常睾丸组织,为质硬、均一的肿物,与周围组织可有粘连,扭转方向及程度已分辨不清,术后病理检查可见大部分出血坏死及钙化灶,睾丸结构基本不见,术后均诊断为NTT.(4)术后随访3~12个月.所有手术病例患侧阴囊切口均愈合良好,阴囊无出血肿胀,2例保守治疗的患儿及1例双侧NTT的患儿分别在出院2和3个月复查时发现患病睾丸明显萎缩,查体可触及小而硬的包块,术后6个月随访超声显示病变睾丸演变为无血流信号的瘢痕结构.所有单侧NTT患儿随访超声检查,均未发现对侧睾丸异常改变.结论 彩超结合临床表现可准确诊断急症型NTT,但非急症型NTT的超声影像需与睾丸畸胎瘤鉴别.对于急症型NTT,及时手术干预对挽救病变睾丸仍有意义.

Objective To analyze the factors associated with the outcome of pyeloplasty in term of renal function and morphology improvement in children with unilateral ureteropelvic junction obstruction,in order to provide clinical evidence for the treatment of hydronephrosis in children.Methods Clinical data of 174 children who underwent unilateral dismembered pyeloplasty from January 2009 to June 2014 were retrospectively studied.Differential changes in renal function and renal morphology after pyeloplasty were assessed by serial renal scan and ultrasound.On the basis of preoperative split DRF,these patients were divided into three groups:group Ⅰ with DRF ≥40％ (n =99),group Ⅱ with DRF 30％-40％ (n =29) and group Ⅲwith DRF ＜ 30％ (n =46).According to their age at surgery,the children were divided into four groups,including group A aged 1-3 months (n =52),group B aged 3 months-3 years (n =44),groupCaged3-6years (n =37),and group D aged more than 6 years (n =41).Results Inall 174 children,postoperative complication occurred in 7 cases,including urinary tract infection in 6 cases and renal atrophy in 1 case.A significant improvement of both function and morphology was confirmed in most patients (P ＜ 0.01).Patients in group Ⅰ showed stable renal function after operation(DRF 48.46％ ±4.80％ vs.50.78％ ± 5.45％,P ＜ 0.01),of them who underwent pyeloplasty at 1-3 months of age showed the best obvious recovery of renal morphology.Renal function of patients in group Ⅱ recovered obviously and most of them reached to the initial values (DRF 35.18％ ± 2.95％ vs.43.91％ ± 6.89％,P ＜ 0.01).While renal function of patients in group Ⅲ recovered significantly after surgery,most of them failed to restore the initial values(DRF 20.70％ ± 6.90％ vs.33.78％ ± 12.49％,P ＜ 0.01),and among them,the aged 1-3 months group possessed the best recovery.Moreover,the morphological improvement was similar to the functional improvement.The time for hydronephrosis recovered to less than Grade 2 of Society for Fetal Urology(SFU)was 6,24 and over 24 months respectively in group Ⅰ,Ⅱ and Ⅲll,and the renal morphology gradually improved with the increasing duration of follow-up.Conclusions The renal function and morphology of most patients improved significantly after pyeloplasty.Recovery of renal function and morphology after surgery was significantly correlated with the preoperative DRF.Early surgical intervention may improve the function and morphology recovery of the involved renal unit.

We report an unusual case with synchronous occurrence of ganglioneuroma (GN) and multiple urogenital malformations (including ipsilateral renal dysplasia, cryptorchidism and contralateral congenital hydronephrosis). The lesions may be a syndrome associated with embryogenetic disorders. Imaging examinations are helpful for preoperative diagnosis and choose of surgical approach. Staging surgical interventions which taking emphasis on the resection of the tumor was recommended for this case and postoperative histological confirmation, anticipation of malignant transformation of the tumor is also important to ensure a final cure. GN coexisting with multiple urogenital malformations is extremely rare and there is no previous similar reported case.

Objective To investigate the diagnosis and management of severe hydronephrosis induced by bilateral ureteropel -vic junction obstruction (UPJO) in children.Methods We retrospectively evaluated the records of 19 patients with severe hydrone-phrosis treated n from November 2011 to February 2015 in our institution.The Anderson-Hynes operation was performed in all patients with staged approaches .The patients were followed up .During the period of follow-up,ultrasonography and radionuclide renal scans were used to evaluate the recovery of renal function .Results Of 38 sides of kidneys ,30 sides received operation treatment .Of these pa-tients,9 cases had bilateral operations .However,the bilateral operation was not performed at the same time .The surgery interval time was 33 to 181 days.There were 8 children without further operation when their opposite hydronephrosis was reduced after one side oper -ation.Two children had to suffer the second operation because their hydronephrosis was aggravated again and differential renal function ( DRF) was decreased after opposite hydronephrosis was transiently reduced after one side operation .Two children underwent conserva-tive treatment in view that their DRF was not decreased although their one side hydronephrosis was not changed after opposite opera -tion.We found that the improved hydronephrosis was graded 3 according to the Society for Fetal Urology ( SFU) while the non-improved was grade 4 after comparison of corresponding data .The ratio between anterior/posterior diameter of renal pelvis and thickness of renal parenchyma in the improved cases was lower than that in non-improved patients ( P<0.05 ) .Conclusion In children with severe bi-lateral UPJO,the non-operated renal units with grade 3 and some with grade 4 hydronephrosis are improved spontaneously after unilater-al pyeloplasty.Therefore,delayed pyeloplasty of the opposite side should be considered for 2 month following initial pyeloplasty .

Renal fibrosis, considered to be a common consequence of progressive renal disease, involves glomerulosclerosis and/or tubulointerstitial fibrosis. Currently, research is focused on investigating potential mechanisms to prevent or reverse the damage caused by fibrosis. Under the influence of cytokines, chemokines and other signaling molecules, the cellular interactions that regulate the development of interstitial fibrosis are complex. Epithelial-mesenchymal transition (EMT) has emerged as an important pathway leading to the generation of matrix-producing fibroblasts and myofibroblasts in diseased kidneys. The proteomics study compared the protein profiles between the time points of podocyte EMT and tubular cell EMT in a partial unilateral ureteral obstruction (PUUO) model in rats. Proteins isolated from the PUUO group and corresponding sham rat kidney tissues were subjected to 2-D gel electrophoresis and were then identified by mass spectrometry. In total, 43 proteins with differential expression were identified, which were reported to be involved in the regulation of the cytoskeleton and actin, glucose metabolism, cell apoptosis, mitochondrial energy metabolism, oxidative stress and endoplasmic reticulum stress. Electron transfer flavoprotein, β polypeptide was detected by immunoblot analysis and its mRNA levels were determined in renal tissues. The results demonstrate protein alterations that reflect the pathology of the obstructed kidneys, and thus may aid in understanding the pathogenesis of obstructive nephropathy.

儿童尿道外口囊肿(parameatal urethral cyst)为小儿泌尿外科少见疾病之一,临床相关文献报道较少.为了探讨尿道外口囊肿的病因、临床特点、诊断要点、治疗方法以及治疗效果,现将我院小儿泌尿外科从2006年2月至2013年1月共收治的52例尿道外口囊肿患儿进行回顾性总结与分析,并将结果报告如下。

Objetive To determine the effect of age at operation and SFU degree to the recovery of renal function and morphology in children with congenital hydronephrosis after pyeloplasty and to provide clinical guidance for the treatment of hydronephrosis in children. Methods 82 patients with ureteropelvic junction ob-struction who underwent pyeloplasty at our hospital between 2007 and 201 3 were retrospectively evaluated.Pre-operative and Postoperative ultrasound findings of parenchymal thickness and ratio of parenchymal area to pelvic area were recorded.glomerular filtration rate (GFR)and differential renal function (DRF)were collected from renal nuclide imaging.Patients were divided into 3 groups (groupA:33 patients with age of 0 ~1 years, groupB:22 patients with age of 2~5 years,groupC:27 patients with age older than 5 years)based on opera-tion age,and based on the Society of Fetal Urology (SFU )degree respectively,patients were divided into 2 groups (group A:28 cases with SFU 3,group B:54 cases with SFU 4 ).Mean follow-up was 20 ± 1.5 months ranged from 6 months to 70 months.Data were analyzed using SPSS,version 1 9 with t-tests and correlation analysis. Results On ultrasound parenchymal thickness and Parenchymal area/pelvic area were significantly improved in all groups,and the latter had positive correlation with the follow-up period,r=0.381 , p=0.001 .The improved GFR was seen in group A and group B significantly (group A:p=0.001 ,group B:p=0.01 4 )and no seen in the group C (p=0.053).The improved DRF was seen in all groups.The improv-ment of DRF,parenchymal thickness and parenchymal area/pelvic area have no difference between the groups based on age,but SFU degree 4 had more significant differences than degree 3 in DRF,p=0.02. Conclusion The renal function and form have been improved significantly after pyeloplasty.Patients younger than 5 years had postoperative improvement in GFR significantly.Recovery of renal function in the affected renal unit after pyeloplasty has insignificant correlation with the operation age.SFU degree is the factor of DRF.Parenchymal area/pelvic area shows higher correlation with follow-up period.

Objective To explore the urodynamic characteristics of spina bifida occulta (SBO) children with urinal dysfunction.Methods A total of 113 patients with urinal dysfunction,48 with SBO diagnosed radiologically and 65 negative controls were recruited.The major symptoms were incontinence,increased voiding frequency and dysuria and nocturnal enuresis.Uroflowmetry was conducted for all cases.And 112 of them received invasive urodynamic examinations for analyzing the correlation between main items of urodynamics and symptoms.Results Most (46/48) of them had various abnormalities on urodynamic test.There were detrusor instability (DI,n =22),detrusor underactivity (n =21),decreased maximum flow rate (n =18,Qmax),decreased bladder capacity (n =15),increased post-void residual urine (n =12,PVR),decreased bladder compliance (BC,n =7),detrusor-external sphincter dyssynergia (n=4) and decreased urethral pressure (n=4).DI,detrusor underactivity,increased PVR and decreased bladder compliance were more often present in patients with SBO.According to the symptoms,correlation existed between incontinence and decreased Qmax and detrusor underactivity; increased voiding frequency and increased PVR; dysuria and detrusor underactivity; nocturnal enuresis and DI.Conclusions SBO children with urinal dysfunction may have various urodynamic changes.And the same clinical manifestation may have different abnormalities.And the symptoms are probably correlated with urodynamic changes.

T-box 1 (Tbx1) gene is closely involved in embryonic kidney development. To explore the role of Tbx1 in acute kidney injury (AKI) and the underlying mechanism, we detected the expression of Tbx1 and components of transforming growth factor-beta (TGF-β) signaling pathways including TGF-β, phosphorylated Smad2/3 (p-Smad2/3) and phosphorylated Smad1/5/8 (p-Smad1/5/8) in kidney tissues derived from a rat model for AKI induced by gentamicin (GM). Apoptosis of renal cells was assessed by terminal deoxynucleotidyl transferase-mediated dUTP nick end-labeling (TUNEL), along with the expression of two essential genes involved in apoptosis, caspase-3 and Bcl-2. Correlation between Tbx1 expression and the number of TUNEL-positive cells was analyzed by a Spearman test. Expression of TGF-β, p-Smad2/3 and p-Smad1/5/8 in Tbx1-knockdown NRK cells was also analyzed by real-time RT-PCR and Western blotting. Markedly increased Tbx1 expression was found in the injured kidney tissues, which has activated the TGFβ-Smad2/3 pathway whilst suppressed Smad1/5/8 expression. Conversely, decreased TGF-β and p-Smad2/3 levels, and elevated p-Smad1/5/8 levels were detected in Tbx1-knockdown NRK cells. More apoptotic cells were detected in the injured kidneys, which has well correlated with the expression of Tbx1. Expression of caspase-3 was markedly increased, while Bcl-2 was decreased in the injured kidney tissues. Above findings suggested that activation of Tbx1 is involved in AKI through the TGFβ-Smad2/3 pathway. Tbx1 expression may therefore serve as a marker for AKI, and Tbx1-blocking therapies may provide an option for treating GM-induced nephropathy.

儿童尿道外口囊肿(parameatal urethral cyst)为小儿泌尿外科少见疾病之一,临床相关文献报道较少.为了探讨尿道外口囊肿的病因、临床特点、诊断要点、治疗方法以及治疗效果,现将我院小儿泌尿外科从2006年2月至2013年1月共收治的52例尿道外口囊肿患儿进行回顾性总结与分析,并将结果报告如下。

Objective To detect the serum TNF-α and glomerular TNF-α mRNA and protein expressions in obesity-related glome-rulopathy(ORG) mice,in order to explore the pathogenesis of ORG. Methods Clean grade 35 d age C57BL/6 male mice were randomly divided into obesity group(20 cases) and control group(20 cases).The obesity group was fed with a high-fat high-energy diet,while the control group was fed with a normal diet.Eight weeks later,the 2 groups were assayed with enzyme linked immunosorbent assay(ELISA) for urinary microalbumin series proteins including urinary albumin(Alb),transferrin(TRF),retinol binding protein(RBP),and β2-microglobulin(β2-MG).Blood samples were also assayed with ELISA for serum TNF-α.Kidney tissue was fixed,sectioned and stained,and examined under both light and electron microscopy for histopathological changes.Renal tissue RNA was extracted and real-time quantitative polymerase chain reaction(RT-PCR) was carried out to measure the expression of TNF-α mRNA.TNF-α protein expression was assayed with Western blot.The difference between the 2 groups was assessed with SPSS 13.0 software. Results Compared with the control group,the obesity group had significantly higher urinary Alb,TRF,RBP and β2-MG and serum TNF-α levels(Pa 0.01).TNF-α mRNA and protein expressions of the renal tissue of the obesity group were significantly higher(Pa0.01).Histopathological examination found that the obesity group had glomerular hypertrophy.Electron microscope revealed epithelial cell foot process fusion,more lipid droplets in the cytoplasm of renal tubular epithelial cells,thickening of basement membrane,loss of the 3-layer structure,with sections insert into the segment mesangial. Conclusions ORG mice appear abnormal urine microalbumin;expression of TNF-α in serum and renal tissue abnormalities may play an important role in the occurrence and development of ORG.

The present report describes a 6-year-old boy with leiomyoma of the bladder and the imaging characteristics of the lesion, including images from ultrasonography, computed tomography, and the histopathologic examination. Leiomyoma of bladder is extremely rare in children. The present case, to our knowledge, represents the second reported case of bladder leiomyoma in pediatric patients since 1966. Although preoperative imaging can be useful to assess the disease extent, the final diagnosis depends on the histopathologic examination findings. Surgical excision is the ideal option to confirm the diagnosis and to treat the disorder. The prognosis is good after complete resection.

Objective To investigate the outcome and treatment of neonates bom with isolated unilateral hydronephrosis diagnosed antenatally.Methods We retrospectively reviewed the casenotes of 692 children (541males and 151 females) between january 1988 and January 2010,who had been diagnosed antenatally with isolated,unilateral hydronephrosis and the diagnosis was subsequently confirmed posmatally.The follow up time was 12 months - 22 years (mean 142 months).All patients were followed up with ultrasonography and dynamic renal imaging.Hydronephrosis was assessed and classified according to the Society of Fetal Urology (SFU) grading system.Results Stabilization occurred in all children with grade 1 hydronephrosis,in 87％ of children (155) with grade 2 hydronephrosis,and in 30％ of children (39) with grade 3 hydronephrosis.However,13％ of children (23) with grade 2 hydronephrosis,70％ of children (92) with grade 3 hydronephrosis,and 100％ of children with grade 4 hydronephrosis received surgical intervention according to our pre - detemfined criteria.107 patients (late pyeloplasty group) were treated due to a reduction in the differential renal function (DRF) to ＜40％,and 93 children (early pyeloplasty group) underwent surgery because of worsening hydronephrosis grades or failure to improve and had poor radiotracer clearance.In the early pyeloplasty groups,significant improvements were noted between postoperation and preoperation with respect to the DRF (45.8 ± 1.7％; 42.7 ± 2/％) and the ratio of the depth of the calyces to the thickness of the parenchyrna (C/P ratio) (0.7 ± 0.3; 2.0 ± 0.8,P＜0.05).The same results were seen in the late pyeloplasty group with respect to the DRF (38.4 ± 2.6％ ; 36.4 ± 3.3％ ) and C/P ratio ( 1.3 ±0.5; 2.4 ± 0.8,P＜0.05).The DRF was better in the early pyeloplasty group than the late pyeloplasty group.The improvement in DRF was significant during the first year post- operatively(48.8 ±4.3％) and became stable thereafter.The C/P ratio was inversely correlated with the DRF in the patients before and after pyeloplasty (r =- 0.26,P =0.01 ; and r =- 0.62,P =0.001,respectively).Conclusions All infants with SFU- 1 and most infants with SFU - 2 hydronephrosis do not need an invasive procedure.Although greater improvement occurred in patients with an initial DRF＜40％,the reduced DRF did not recover to the pre- deterioration level post- operatively.Earlier surgical intervention after a short period of strict clinical surveillance is beneficial for preserving renal function in patients with persistent SFU - 3 or SFU - 4 hydronephrosis.