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新生儿先天畸形围术期的治疗探讨（作者:___________单位: ___________邮编: ___________）【摘要】目的回顾小儿外科自2002年1月至2009年5月收治的先天性畸形患儿104例治疗的临床资料，探讨新生儿先天性畸形的有效治疗方法。
方法先天性畸形患儿入院后，详细询问病史，母亲的孕期服药史，羊水情况，胎动情况，产前胎儿系统查体情况，以及患儿出生后的表现如呕吐、排便等，仔细全面体格检查，辅助检查，明确诊断。
经过完善的术前准备，良好的麻醉支持，精细的外科手术，完备的术后护理，患儿康复出院。
结果在收治的104例患儿中，其中87例达到一期治愈，占87.8%;12例出现腹部刀口感染，二期手术治愈;1例先天性胃壁肌层缺损并破裂者，合并硬肿症而死亡;3例回肠末端闭锁者，因就诊时间晚，出现电解质紊乱，肺部感染而死亡。
1例先天性十二指肠闭锁并小肠多端闭锁,放弃治疗。
随访发现安全渡过围术期患儿生长发育正常。
结论随着麻醉技术的提高，小儿外科医生操作技术水平的进步，护理人员耐心细致的护理，以及高新材料的临床应用，只要重视每一个临床细节并使之完善，新生儿先天畸形的治愈率将会越来越高。
【关键词】新生儿;先天畸形;围术期Perioperational therapy in newborn with congenital malformation WANG Shou-guo,ZHANG Zheng-mao.Department of Pediatric Surgery,Maternal and Child Health Hospital of Zibo City,Zibo 255029,China[Abstract] Objective This paper aims to explore the effective therapy for newborn with congenital malformation after 104 cases of neonatal abnormalities from January 2002 to May 2009 were reviewed and analyzed in department of pediatric surgery of our hospital.Methods After being hospitalized,the newborns with congenital malformation were checked from inquiry about their medical histories,their mothers’prenatal medication,conditions about amniotic fluid,fetal movement,systematic antenatal physical examination as well as manifestation like vomit and defecation after being born.Conducted double and overall physical check and accessory examination,clarified the diagnosis.Fully preparation before operation which is aided with good anaesthesia,subtle surgery and complete postoperative care can make these neonatal abnormalities recovere from the hospital.Results 104 newborns all received an operation.87 (87.8%) cases were cured after one-stage operation.12 cases got abdominal blade infection,and were cured after two-stage operation.1 case died because ofcongenital defect of muscular layer of stomach wall and mucous membrane got rupture after taking food,incorporated with scleredema.3 cases with distal ileum atresia died from paying a late visit to doctor,suffered from electrolyte disturbances and pulmonary infection.1 case with neonatal atresia of duodenum and multiports of small intestine gave up treatment and was found in the prognosis that this patient grew normally after safely lived through the perioperational period.Conclusion As the advancement of technique of anaesthesia and pediatric surgeons,patient and painstaking care from nursing staff,clinical application of the hi-tech stuff as well as enough attention are paid to every clinical details and make them perfect,curative ratio for newborn with congenital malformation will become higher in the future.[Key words] newborn;congenital malformation;perioperational period选择淄博市妇幼保健院的小儿外科自2002年1月至2009年5月,共收治先天性畸形患儿104例，经过完善的术前准备，良好的麻醉支持，精细的外科手术，完备的术后护理，使患先天性畸形的患儿康复出院，治愈率达95%以上，特对治疗过程进行探讨，以便使更多的患儿重获新生。
出⼊NICU的标准新⽣⼉期，可发⽣各系统外科疾病，其中以先天性发育畸形占⾸要地位。
新⽣⼉外科中相当⼤⼀部分即是对先天性畸形的诊治。
先天性畸形的病因由遗传造成的约占20%，染⾊体畸形引起的约占10%，环境因素所致者约占10%，另外60%原因未阐明。
新⽣⼉内外科及围产医学医师常需针对新⽣⼉患⼉去评价、鉴别、诊断和处理，并且提供引起的原因和预后。
感染性疾病在新⽣⼉外科中仍然具有特殊意义。
30多年的实践已经清楚地证明，新⽣⼉重症监护病房（NICU）在抢救危重新⽣⼉的治疗中已起到重要作⽤。
我国的⼤中城市⾃20世纪80年代起相继建⽴NICU，挽救了不少新⽣⼉⽣命。
出⼊NICU的标准：（⼀）⼊NICU的标准1．循环系统：①威胁⽣命的⼼率失常；②侵袭性⾎液动⼒学监测；③⼼脏⼿术后；④不稳定性⼼脏病。
2．呼吸系统：①辅助呼吸；②⽓管内呼吸；③FiO2>0.5。
3．神经系统：①颅内压监测；②颅内压增⾼；③颅内⼿术后；④不稳定的神经系统疾病；⑤闭合性头颅外伤。
4．消化系统：①活动性消化道出⾎；②严重腹泻和脱⽔；③⼤⼿术后。
5．其他：①⾎管性药物的应⽤；②严重电解质紊乱。
总之，在⼀切可能危及新⽣⼉⽣命，随时有发⽣恶化的疾病均应视具体条件进⼊NICU。
（⼆）出NICU的标准：1．循环系统：①⼼率稳定；②稳定的⾎液动⼒学评价。
2．呼吸系统：①FiO2<0.5;②没有⽓管插管。
3．神经系统：①正常的颅内压；②稳定的神经系统疾病。
4．消化系统：①消化道⼤出⾎已控制；②液体丧失已补偿。
5．其他：⽔电解质维持稳定。
NICU监护内容：最好的“监护仪”是精明能⼲并且关⼼患⼉的护⼠，她们经常守护在危重患⼉的⾝边。
护理⼈员的数量和质量直接关系到重症监护的效果。
在NICU，监护仪发出的报警⾄少有⼀半是由于婴⼉哭闹或活动等因素造成的误报警，所以中⼼监护系统对NICU并不实⽤。
NICU唯⼀的监护⽅法就是仪器和医务⼈员构成的“床旁监护”。
天津医药２００６年７月第３４卷第７期新生儿先天性梅毒的早期诊断及治疗李玉萍刘晶赵颖李世娟关键谓梅毒婴儿．瓶生诊断先天性梅毒又称胎传梅毒．是梅毒螺旋体由母体经过胎盘进入胎儿血循环中所致的梅毒感染．可导致新生儿全身多脏器的损害，并可遗留终身后遗症。
近年来随着性传播疾病的蔓延．该病的发病率呈上升趋势，对先天性梅毒早期诊断及时给予正确治疗，可大大减少后遣症。
提高远期生存质量。
我院２００５年收治了２例先天性梅毒新生儿，现将诊治经过报告如下。
１病例报告例１女．主因胎龄３６“周早产．生后４０ｍｉｎ，于２００５年４月２８日人住新生儿科病房。
体质量３１００ｇｏ查体可见呼吸急促，口唇青紫，全身皮肤轻度黄染，手、足、骶尾部皮肤散在大疱性表皮剥脱，疱内可见黏稠脓液．前囟１ｃｍｘｌｃｍ平坦，五官无畸形，颈软无抵抗，两肺可阉及细小水泡音，心音有力，律齐，未闻及杂音，腹膨隆．肝于右肋下５ｃｍ，质中等硬，脾于左季肋下３ｃｒｔｌ，质中等硬，四肢活动可。
急查外周血象：白细胞２８．３ｘｌ＆／Ｌ，中性粒细胞０．５１，血红蛋白１１３辱／Ｌ，红细胞压积０．３６，血小板１０５ｘ１０９／Ｌ，Ｃ一反应蛋白（ＣＲＰ）正常。
胸片提示两侧肺纹理增粗．伴散在点片状阴影。
人院后拟诊为：新生儿肺炎，早产儿，轻度贫血，可疑宫内感染。
给予优立欣７５ｍ晷／（ｋｓ・次），１次／１２ｈ，拉氧头孢钠２０ｍｇ／（ｋｓ・次）．１欢／１２ｈ．静脉浦注。
能量合剂、复方丹参液保护心肌细胞及脑活素改善脑细胞代谢等综合治疗。
同时做静脉血肝功能检查、乙肝病毒、ＴＯＲＣＨ感染及梅毒血清学试验等相关检查。
结果报告：乙肝病毒阴性．ＴＯＲＣＨ阴性，血清直接胆红素升高，谷氨酰转肽酶明显升高，梅毒快速反应索试验（ＲＰＲ）阳性，效价１：１６、梅毒快速血浆反应凝集试验（ＴＦＰＡ）阳性。
追问母病史，否认梅毒患病史。
化验母ＣＲＰ７５ｎｌｇ／Ｌ，ＲＰＲ阳性、鼓债１：１６，ＴＰＰＡ阳性。
诊断为先天性梅毒感染（Ⅱ期）。
PO-015例儿童外伤性胰腺炎非手术治疗的护理刘晓文, 刘晓文, 甘红霞武汉市儿童医院普外科 430016目的总结了5例儿童外伤性胰腺炎非手术治疗的护理要点。
方法对5例I、II级外伤性胰腺炎非手术治疗的患儿实施监测血尿淀粉酶，及时完成血标本的采集及运送，重视胃肠道管理，每班注意保持胃肠减压管通畅，保证有效的负压，妥善固定，防止扭曲、打折、受压，以免影响减压效果，所有患儿禁食期间给予全肠外营养，在减少胰、胃分泌的同时, 可保证机体的能量需求。
观察患儿腹部体征，护理人员要积极配合医生，耐心询问家属及患儿受伤部位及经过，对于上腹部挤压伤，撞击伤尤其应引起注意，以鼓励性语言减轻患儿对治疗的恐惧，增添患儿的治疗信心，对于可能发生的并发症，鼓励家长积极面对，取得他们的理解和配合，努力帮助减轻家属的的心理负担，加强心理护理，对患儿及家属做好出院指导和随访工作等护理措施。
结果经过精心治疗和护理，5例患儿住院7～20d均痊愈出院。
随访2个月至3年，4例患儿未出现并发症，1例出院1个月后出现胰腺假性囊肿，CT提示囊肿直径4cm，经对症治疗，囊肿自行吸收，随访1年未再复发。
结论I、II级胰腺外伤应首选非手术治疗,护理人员做好细致的观察和护理工作能有效掌握病情的动态发展，及时发现胰腺假性囊肿等并发症，可以有效提高本病的治愈率。
PO-02既往上腹部手术史行腹腔镜胆总管囊肿根治术体会尹强, 周小渔, 刘朝阳, 戴翼, 袁妙贤, 陈立健湖南省儿童医院 410007目的探讨腹腔镜胆总管囊肿根治术在既往有上腹部手术史患者中的可行性及特点。
方法收集2012年2月至2015年2月我院收治的既往有上腹部手术史且需行腹腔镜胆总管囊肿根治术的病例15例，四孔法手术，在腹腔镜的下进行分离粘连及腹腔探查，进而行胆总管囊肿彻底切除肝管空肠Roux-en-Y吻合术。
结果中转开腹手术4例，余患者均在腹腔镜下完成了手术，所有患者均康复出院。
结论采用腹腔镜技术治疗既往有上腹部手术史的患者是安全有效的，但需要外科医师有丰富的腔镜外科手术经验。
PO-03Comparison of different noninvasive diagnostic methods for biliaryatresia: a meta-analysisjinpeng He, Jie-Xiong Feng, Jing-Fan Shao, Xxiao-Lin Wang, Hong-Yi Zhang Department of Pediatric Surgery, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, HubeiBackground This study was undertaken to retrospectively analyze the accuracy of different methods in differentiating biliary atresia from neonatal jaundice.Methods A search was made in MEDLINE, and the Web of Science for relevant original articles published in English; methodological quality of the included studies was also assessed. Two reviewers extracted data independently. Studies were pooled, Summary receiver operating characteristics (SROC) curve and diagnostic odds ratio (DOR) with corresponding confidence intervals (CIs) were calculated.Results For diagnosis of biliary atresia, ultrasonography (US), hepatic scintigraphy (HBS), and magnetic resonance cholangiography (MRCP) had a pooled sensitivity of 74.9% (range 70.4%-79.1%), 93.4% (range 90.3%-95.7%) and 89.7% (range 84.8%-93.4%), a specificity of 93.4% (range 91.4%-95.1%), 69.2% (range 65.1%-73.1%) and 64.7% (range 58.0%-71.0%), LR+ of 12.16 (range 6.41-23.08), 3.01 (range 2.15-4.20) and 3.10 (range 1.59–6.06), LR of 0.23 (range 0.13-0.38), 0.13 (range 0.06-0.25) and 0.16 (range 0.06–0.44), DOR of 72.56 (range 27.34-192.58), 29.88(range 12.82-69.64) and 32.48 (range 8.22-128.29), with an AUC of 0.96, 0.91, and 0.92 and Q of 0.90, 0.85, and 0.85, respectively.Conclusions US, HBS and MRCP can be very useful for the diagnostic work-up of neonatal cholestasis. To improve the sensitivity and specificity, several measures can be used.PO-04胆道闭锁患儿肝内胆管γδT 细胞和调节性T细胞浸润及意义李康华中科技大学同济医学院附属协和医院小儿外科 430022目的研究胆道闭锁患儿肝组织中γδT细胞和调节性T细胞(Foxp3+ Treg)比例变化。
方法采用免疫组织化学方法和流式细胞术观察和检测23例胆道闭锁患儿组（BA组）和12例对照组患儿（CG组）肝组织中γδT细胞分布情况以及γδT细胞和Foxp3+ Treg细胞比例关系。
结果免疫组织化学染色显示BA肝脏汇管区胆管周围有大量的γδT细胞和一定程度的Foxp3+Treg 细胞浸润。
流式细胞术显示胆道闭锁肝组织中γδT细胞与Foxp3+ Treg细胞比例明显高于对照组（P<0.05），且γδT细胞与Foxp3+Treg细胞比例呈显著负相关(P<0.05)。
结论胆道闭锁患儿肝组织中γδT细胞增多，或抑制Foxp3+Treg细胞增殖，促进了胆管的进行性炎症损伤。
PO-05Identification Of Circulating MicroRNAs In Biliary Atresia ByIllumina Sequencingxiaofang peng, Li-Yuan Yang, Shu-Yin Pang, Yi-hao Chen, Jie Fu, Zhe We n, Le Li, Rui-zhong Zhang, Wei Zhong, Jia-Kang Yu, Yi Xu, Si-Tang Gong, Hui-Min Xia, Hai-Ying LiuGuangzhou Medical University, Affiliated to Guangzhou women and children Children's MedicalCenterObjective Biliary atresia (BA) is a fibroinflammatory obstruction of extrahepatic bile duct that leads to end-stage liver disease, reliable noninvasive diagnosis is still challenged. Recent studies have revealed that circulating microRNAs (miRNAs) with specific expression patterns can serve as new non-invasive biomarkers for numerous diseases, however, very little information is known in BA. In this study, we aimed to investigate the specific circulating microRNAs expression of BA by use of Illumina next-generation sequencing.Methods Two panels of BA patients and healthy controls (HCs), and a panel of disease controls with other cholestatic (DCs) were recruited in this study, we employed Illumina deep sequencing for the initial screening to display the differential expression of circulating miRNAs in plasma from BA patients and HCs. Differentially expressed miRNAs were validated by quantitative real-time polymerase chain reaction (qRT-PCR), the expression levels were presented as fold-changes (2-ΔΔCt). The diagnostic utility of BA-specific miRNAs were examined by using receiver oprating characteristic (ROC) analysis. Then the target genes and related signaling pathways of BA specific miRNAs were predicted using target gene prediction software.Results Using Illumina high-throughput sequencing, 146 differential miRNAs were identified. Additionally, 15 (7 up-regulated and 8 down-regulated ) miRNAs with higher read counts (>1000) were retained for further consideration. And 6 plasma miRNAs whose sequencing reads were more than 5000 were selected for qRT-PCR analysis on an independent set from 44 BA patients, 20 DCs and 20 HCs. Following qRT-PCR analysis, we were able to confirm the significant up-regulated of 2 miRNAs (miR-122-5p and miR100-5p) and significant down-regulated of 2 miRNAs (miR-140-3p and miR126-3p) in BA patients compared to HCs, only miR-140-3p was significantly decreased in BA patients in comparison to DCs and HCs. The area under the ROC curve (AUC) of miR-140-3p in the evaluation of 44 BA population and 40 no-BA population was 0.75, specificity was 79.1%, andsensitivity was 66.7%. Target gene prediction results showed that the target genes of miR-140-3p were involved in the development and differentiation of bile duct, formation of extracellular matrix and occurrence of liver fibrosis.Conclusions our results indicate that the plasma from patients with BA has a unique miRNA expression profile and miR-140-3p may be a promising diagnostic biomarker for BA. Further study contribute to understanding the pathogenesis of BA.PO-06完全腹腔镜下胆总管囊肿根治性切除2例报告何少华, 徐迪福建省立医院儿外科 350001目的探讨总结完全经腹腔镜途径根治性切除胆总管囊肿，重建胆道的手术方法。
