Objective: Cancer stem cells are involved in chemotherapy resistance. Neuroblastoma (NB) is a common solid tumor in children, which is responsible for about 15% of pediatric cancer deaths. Jumonji domain-containing 6 (JMJD6), which is an arginine demethylase and lysine hydroxylase, regulates the progression of various tumors. However, the molecular mechanism underlying JMJD6's activity in NB stem cells remains unclear. Thus, this study aims to explore the role of JMJD6 in NB stem cells. Material and Methods: Side population and sphere formation assays were used to investigate the role of JMJD6 in NB stem cell expansion. Immunohistochemistry was performed to determine the expression level of JMJD6 in NB tissues. Luciferase reporter assays were used to discover the effect of JMJD6 on the wingless/integrated (WNT) pathway. Results: JMJD6 was upregulated in NB tissues and cells (P < 0.05). NB patients with high JMJD6 levels had poor outcomes. JMJD6 could serve as an independent prognostic factor for NB. JMJD6 overexpression promoted NB stem cell expansion, whereas JMJD6 knockdown had the opposite effect, as determined by cell and animal models. Mechanism Roche studies showed that JMJD6 promoted NB stem cell expansion by activating the WNT pathway. Conclusion: JMJD6 promotes NB stem cell expansion by activating the WNT pathway, thereby providing a new target for NB therapy and a prognostic factor for patients with NB.

PurposeComprehension of the anatomical characteristics of pediatric kidney tumors is crucial for making surgical decisions. Previous kidney tumor nephrometry systems failed to incorporate two significant factors: tumor thrombus and multifocality. We develop a refined nephrometry system based on a comprehensive understanding of the characteristics exhibited by pediatric kidney tumors.MethodsThe TUMORS nephrometry scoring system comprises 6 indicators, including tumor (T)hrombus, (U)rinary collecting system involvement, (M)ultiple tumors, (O)utward property, (R)adius, and (S)ite relative to the polar lines. Each renal unit was assessed and scored independently. The complexity characteristics of kidney tumors were summarized, and the correlation was compared with RENAL nephrometry system. Furthermore, the complexity of kidney tumors was compared across different surgical procedures.ResultsA total of 43 patients were enrolled, involving 70 kidney units. Radical nephrectomy (RN) was performed on 13 kidneys, while the remaining 57 kidneys underwent nephron sparing surgery. In the NSS group, tumors in 37 kidneys were resected in vivo, whereas 20 kidneys underwent tumor resection ex vivo followed by kidney autotransplantation. According to the TUMORS nephrometry scoring system, there were 13, 34 and 23 kidney units classified as low, moderate and high complexity, respectively. Tumors that underwent RN or ex vivo removal exhibited higher complexity. The complications and positive margins of NSS were not statistically significant in relation to tumor complexity.ConclusionThe TUMORS nephrometry scoring system holds significant guidance for the decision of surgical protocol and can be applied to the preoperative evaluation.

Neuroblastoma is the most common extracranial solid tumor of childhood, previous studies show synaptic protein neuroligin-3 (NLGN3) promotes glioma proliferation and growth, However, no investigation about the role of NLGN3 in neuroblastoma was reported. Here, we found NGLGN3 was significantly upregulated in neuroblastoma cells and tissues, its overexpression significantly promoted neuroblastoma cell proliferation and growth determined by MTT analysis, colony formation assay, cell cycle progression analysis, BrdU incorporation assay and animal model, while its knockdown inhibited cell proliferation and growth. Then we found NLGN3 could increase the phosphorylation level of AKT and the transcription activity of FOXO family, suggesting NLGN3 activated PI3K/AKT pathway, inhibition of PI3K/AKT pathway in NLGN3 overexpressing cells inhibited cell proliferation, confirming NLGN3 promoted neuroblastoma proliferation through activating PI3K/AKT pathway. In summary, we found NLGN3 promoted neuroblastoma cell proliferation and growth through activating PI3K/AKT pathway and providing a new target for neuroblastoma therapy.

Neuroblastoma is a childhood tumor, and high-stage neuroblastoma has a poor prognosis. The regulatory mechanisms for neuroblastoma progression are poorly understood. In present study, we found that GDNF family receptor alpha 2 (GFRA2) was upregulated in neuroblastoma cells and tissues, and its overexpression promoted neuroblastoma cell proliferation, as revealed using colony formation, soft agar growth, and 3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyltetrazolium bromide (MTT) assays Tumor suppressor phosphatase and tensin homolog (PTEN) is an inhibitor of the phosphatidylinositol-4,5-bisphosphate 3-kinase (PI3K)/AKT serine/threonine kinase (AKT) pathway that interacts with GFRA2. A luciferase activity assay showed GFRA2 inhibits the transcriptional activity of the forkhead box O (FOXO) family proteins, which suggested that GFRA2 activated the PI3K/AKT pathway. Inhibition of the PI3K/AKT pathway in GFRA2 overexpressing cells decreased cell proliferation, confirming that GFRA2 promoted neuroblastoma cell proliferation by activating the PI3K/AKT pathway. In summary, cell proliferation via the GFRA2-PTEN-PI3K/AKT axis may represent new target to develop treatments for neuroblastoma.

目的 探讨膀胱外翻合并尿道上裂男性患儿围手术期的护理路径.方法 中山大学附属第一医院小儿外科对膀胱外翻合并尿道上裂男性患儿3例实施手术治疗.对3例膀胱外翻合并尿道上裂男性患儿实施互利计划及心理干预.结果 围手术期的精心护理对手术效果及术后病情的恢复起到重要的作用.围手术期护理效果满意.经过12个月随访,1例患儿膀胱容量小,泌尿系反复感染,多次返院行膀胱镜探查,其余2例每日可自行排尿,生活质量提高.结论

目的：局麻组和静脉麻醉组两组对比在镇痛效果上，静脉麻组明显优于局麻组。而且不良反应可耐受。结论：冬眠三号半量静脉小壶给药+地西泮10毫克静脉缓注+2%利多卡因宫颈封闭用于人流手术麻醉效果可靠，安全性高，是基层医院开展无痛人流技术既安全又麻醉效果非常可靠的方法。

目的探讨突发性抽搐小儿的抢救配合方法与护理措施。方法分析总结2007-06-2008-05本科发生的6例小儿突发性抽搐的抢救配合过程及抢救后小儿的体位、安全、呼吸道、病情观察等方面的护理。结果 6例小儿中,5例小儿病情恢复良好出院,1例小儿放弃治疗出院,住院期间均无出现护理并发症。结论及时镇静、补充钙剂是缓解症状的关键,保持呼吸道通畅,预防外伤,密切病情观察,防止并发症是护理的重点。

Objective To investigate the clinical significance of the expression of sphingolipids kinase-1 ( SPHK1 ) in neuroblastoma and its relationship with the clinical staging and prognosis of neuroblastoma. Methods The expression of SPHK1 in consecutive 142 paraffin-embedded sections of neuroblastoma from Jan. 1995 to Oct. 2009 was observed by immunohistological staining. The relationship between the expression of SPHK1 with the clinical staging and prognosis of neuroblastoma was analyzed. Results Low expression of SPHK1 was found in 69 cases of neuroblastoma and high expression in 73 cases. The expression of SPHK1 had a significant relationship with INSS staging (P ＜0.01 ) and survival (P ＜0.01 ) of patients with neuroblastoma. Conclusion There is a significant relationship between the expression of SPHK1 with the clinical staging and prognosis of neuroblastoma. SPHK1 can be used as a tumor marker of neuroblastoma.

Objective To explore the treatment of giant hydronephrosis in children,and the methods to upgrade the curative effect.Methods The outcome of 37 children with giant hydronephrosis from Mar.2005 to Mar.2009 were analyzed.Twenty cases had been performed pyeloplasty(immediate operative group),and another 17 cases(19 kidneys) were performed pyeloplasty after nephrostomy(nephrostomy group).All cases were followed up for 6 months up to 4 years after pyeloplasty.The outcome of the 2 curative methods were compared.In nephrostomy group,the renal function were evaluated by Doppler ultrasound and urinary albumin(Alb),β2-microglobulin(β2-MG) were detected during nephrostomy.Results Of the immediate operative group,2 kidneys had been excised due to renal atrophy.There were abdominal distension in 5 cases and urinary infection in 2 cases.Two cases of them had renal calculi.The ultrasonic examination showed that 6 cases,9 cases and 5 cases had mild,moderate or severe hydronephrosis,respectively.Their parenchyma thickness was(5.35±2.00) mm(ranging from 1 to 8 mm).The children in nephrostomy group had no abdominal distension or urinary infection.Four cases had no hydronephrosis,11 cases had mild hydronephrosis and 2 cases had moderate hydronephrosis.No case had severe hydronephrosis,renal calculi and renal atrophy.The parenchyma thickness was(8.30±1.85) mm(rang from 6 to 13 mm).After nephrostomy,the parenchyma thickness increased mainly in 1 week,having no significant change 4 weeks later.Urinary β2-MG returned to normal after 4 weeks.While urinary Alb decreased gradually,it did not return to normal level 12 weeks later.Conclusions The outcome of pyeloplasty after nephrostomy was better,with less complications.By using this method,some giant hydronephrotic kidneys which are supposed to be excised can survive.The function of renal tubule returns to normal earlier than that of glomerulus after nephrostomy.Urinary β2-MG can be used as an indicator to monitor the renal function after nephrostomy.

通过对35例婴儿深静脉置管的护理,探讨在临床中如何针对婴儿年龄幼小、无自我保护意识等特点进行护理。预防感染、加强导管的护理、防止并发症以及健康宣教是护理的重点,为提高护理质量、有效利用深静脉置管的优点提供保障。

Objectives To investigate the clinical characteristics of diagnosis and treatment of congenital male urethral diverticulum.Methods Retrospective analysis was performed on clinical data of 4 congenital urethral diverticulum patients who were admitted in our hospital from Oct.1st 1996 to Sept.1st 2009.Results Age of the 4 patients ranged from 3 months to 3 years old,all of them presented with urinary obstruction symptoms after birth,including dysuria,postmicturition dribble,etc.A palpable mass at the root of penis or scrotum could be found in all patients.All patients underwent voiding urethrography,B untrasonography and urethral endoscopy,1 case got MRI,all patients got the accurate diagnosis preoperatively.Open diverticulectomy with urethroplasty was performed on these 4 cases,15 months to 99 months follow up showed good result of the operation.No urethral stricture and urethral diverticulum recurrence was found after operation.Conclusions The possibility of congenital anterior urethral diverticulum should be considered for the patients with symptoms of urinary obstruction after birth,accompanied malformation should be found simultaneously.Open diverticulectomy with urethroplasty is suggested for the patient with congenital anterior urethral diverticulum.

Objective To investigate the benefit of preserving ostomy bag in repeatedly children enema.Methods 26 infants undergoing closure after colostomy were equally divided into two groups randomly from January 2007 to December 2009.In the control group,the conventional methods of enema was used,while ostomy bag was preserved during the period of repeatedly enema in the experimental group.The two groups were compared in respect of time for enema and cost for care pad and ostomy bag.Results The time for enema in the experimental group was shorter than that in the control group.The cost for nursing pad and ostomy bag was less than that in the control group.Conclusions Preservation of ostomy bag is a good method for infants undergoing repeated enema.It can shorten the time for enema and reduce cost significantly.

目的 探讨小儿急性胆源性胰腺炎(ABP)的临床特点和护理经验.方法 对22例小儿ABP患儿进行严格的饮食、输液管理,加强病情观察,重视患儿的心理护理.结果 22例患儿中1例急诊行ERCP,胆道口括约肌切开,其余均平稳度过急性期.本组无一例死亡.结论 对小儿ABP患儿进行有针对性的护理,结合儿童心理护理特点,提高患儿护理质量.

Objective:To summarize the clinical characteristic and rational treatment of 46,XY DSD.Methods:A retrospective analysis was performed on 26 46,XY DSD patients which were admitted in our hospital from 1979.1 to 2005.2,in cluding sex chromosome,gonad,pudendum appearance,course of treatment,etc.Results:There were 14 cases of androgen insensitive syndrome,5 cases of 5α-reducase abnormality,4 cases of Muller's duct perpetuity syndrome,3 cases of testosterone dyssynthesis.The social sex were determined to be male in 15 cases,female in 11 cases which were performed or chiectomy.Conclusion:The diagnosis and typing of 46,XY DSD are based on careful physical examination,aggregate analysis of phenotype,sex chromosome,gonads,etc.The social sex should be determined before 3 years old.

[目的]探讨晨间护理查房在护理质量管理中的作用。[方法]对本科室晨间护理查房模式进行分析。[结果]通过规范的晨间护理查房使病房护理质量得到保障。[结论]晨间护理查房可提高护理服务质量,加强护患沟通,提高病人对护理服务的满意度。

回肠代膀胱术(Brieker术)的尿道改道.现将围手术期护理体会报道如下. 1 临床资料 1.1 对象患者男,78岁.1年前无明显诱因下出现左腰部酸胀,排尿次数较频繁,白天9～10次,夜尿4～6次,无明显肉眼血尿,期间症状有反复发作,长期服用坦索罗辛缓释胶囊控制症状,以后症状加重.

目的 缩短小儿术后连接管道与引流袋之间所消耗的时间,提高工作效率.方法 运用新设计制作的小胶管接头代替临床上常用的玻璃接头连接引流袋.患儿分别应用小胶管接头(观察组)和玻璃接头(对照组)连接引流袋,对2组患儿术后连接引流袋所消耗的时间进行测定、观察停留管道期间接头的松脱情况进行比较.结果 小胶管接头在小儿术后管道的连接中使用简单、方便、易于连接、耗时少.结论 小胶管接头更适合应用于小儿术后留置直径细小的引流管与引流袋之间的连接.

目的探讨小儿性别畸形存在的护理问题及护理心理学。方法对43例患儿进行性别确定和矫正。分析43例性别畸形患儿在手术前后的护理问题并强调护理心理学在本病护理工作中的作用。结果43例中1例放弃治疗自动出院,42例痊愈满意出院。结论小儿性别畸形手术前后存在护理问题。手术前做好患者及家属的心理护理,手术后积极预防感染,消除其角色冲突的心理问题是护理的关键。