先天性小肠闭锁术后肠动力功能障碍的原因探讨

目的探讨小肠闭锁术后发生肠动力功能障碍的原因及手术切除病变肠管范围。方法对小肠闭锁手术切除小肠标本15例及对照组6例非肠道或神经系统疾病死亡的足月新生儿尸检小肠标本肠壁S-100蛋白、α-平滑肌肌动蛋白（α-SMA）和c-kit蛋白进行免疫组化研究。观察闭锁两端肠壁肠神经系统（ENS）、平滑肌和肠间质细胞（ICCs）病理改变及其分布范围，并行统计学处理。结果闭锁近端肠壁S-100、α-SMA和c—kit阳性表达明显低于对照组。随远离盲端，以上指标呈逐渐增加趋势。在闭锁近端16cm、远端4cm处，三者病变总体趋于正常。结论小肠闭锁两端肠壁与肠动力密切相关的ENS、平滑肌和ICCs均存在病变。是小肠闭锁术后发生肠道动力功能障碍的原因。在患儿小肠长度允许的情况下．切除闭锁近端肠管16cm以上，而远端切除4cm．可减少或避免术后肠动力功能障碍的发生。     

先天性肠闭锁、肠重复畸形一例报告

患儿,女,足月顺产。2002年3月16日出生,生后1d发现肛门无胎便排出,并出现明显腹胀、频繁呕吐。T:36.2℃,P:148次/min,R:48次/min,体重:3.8kg。腹部高度膨胀,可见胃肠型及蠕动波,以上腹部为著。腹软,无移动性浊音,肠鸣音亢进,肛诊直肠内空虚无物。腹部平片示:立位见上腹部胃肠大量积气,可见多个气液平面;倒立位见气体及液平未固定于上腹部而     

先天性肠闭锁肠电图和肠间质细胞的同步研究

目的 通过肠闭锁患儿肠电图和肠间质细胞的临床研究，探讨肠闭锁患儿手术前后肠动力的改变及与肠间质细胞发育情况的关系。方法 分别对正常新生儿和肠闭锁患儿手术前后进行肠电图检测；对先天性肠闭锁患儿标本，分别选取闭锁盲端远近端肠壁全层组织，采用Envision法免疫组织化学技术，观察肠间质细胞的发育情况。结果 肠闭锁患儿闭锁近端术前肠电图电压高于正常对照组，术后肠电压及术前术后的肠电图频率与对照组接近；在肠闭锁肠壁中，发现肠间质细胞在远近端的分布与正常对照组相比显著减少，肠间质细胞网络被破坏。近端改变范围在8cm左右，远端改变局限在1～2cm内。结论 肠电图(EEG)对肠闭锁的诊断和术后肠功能恢复的判断有一定的临床意义，肠电图表现可反映肠间质细胞的发育情况。     

小肠诱发电位检测技术的实验研究

利用带有银－氯化银不极化电极的双腔导管，通过胃造瘘置管于小肠，测定新西兰大白兔小肠吸收葡萄糖、乳糖、甘氨酸、亮氨酸的诱发电位（ＰＤ）值，最大诱发电位值（ＰＤ＿（ｍａｘ））及最大半饱和浓度（ＣＫ）。结果证明该装置可清晰地显示诱发电位图像，通过计算能计算出小肠单位面积最大的吸收功能及输入溶液的最佳浓度，作为临床评定小肠吸收功能的一种定性、定量指标。     

先天性肠闭锁与肠神经系统发育

先天性肠闭锁是新生儿常见的消化道畸形，肠闭锁的特征是肠管在发育过程中出现停滞，表现为一处或多处肠管发生闭锁，目前病因不清，空化不全和血液循环障碍是其两种主要学说。自从Louw和Barnard在1955年关于肠闭锁病因学的实验工作建立以后．胚胎期血液循环障碍被普遍接受为造成这种畸形的主要病因.     

小儿下消化道出血123例报告

为了提高对小儿下消化道出血的诊治水平。对１２３例１２岁以内下消化道出血患儿，按不同年龄分为五组，又按出血量的大小分为急性大出血和慢性间歇性出血两大类，从而找出不同年龄组及急慢性出血的常见原因。结果发现年龄越小病死率越高，不明原因的下消化道急性大出血也是致死的主要原因。对下消化道出血患儿详细地询问病史，按诊断程序全面进行检查，绝大多数病例可得到明确诊断。根据检查结果积极地进行治疗是减少病死率和并发症的主要环节。     

先天性肠闭锁病死率40年回顾性分析

目的 总结先天性肠闭锁病理分型及不同部位的发病率,分析影响各型肠闭锁病死率的因素.方法 回顾性分析本院近40年来302例先天性肠闭锁患儿的病例资料,对其病死率、手术方式和并发症进行统计学性分析.结论 闭锁在肠道的发病例数依次为回肠152例(50.3%)、空肠115例(38.1%)、十二指肠19例(6.3%)和结肠16例(5.3%),病理分型依次为Ⅰ型45例(14.9%)、Ⅱ型4例(1.3%)、Ⅲ-a型188例(62.3%)、Ⅲ-b型8例(2.6%)和Ⅳ型57例(18.9%).由宫内肠套或宫内肠扭转原因引起的闭锁发生率为16.8%.病死率由上个世纪70年代的50%显著降至近年的27.5%.闭锁发生的部位对于手术方式的选择和预后有显著影响,闭锁部位越高,其预后愈差.术后肠梗阻(包括功能性梗阻)和剩余肠管长度是影响其预后最为显著的因素.结论 肠闭锁的病死率随近年新生儿外科和重症监护的发展有显著下降,闭锁发生的部位和术后并发症(梗阻和短肠)是影响其治疗和预后的重要因素.

Abstract：

Objective To compare and analyze the factors affecting the morbidity and mortality among the different types of the congenital intestinal atresia.Methods Medical records of 302 cases of intestinal atresia during last 40 years were reviewed retrospectively.The statistic studies of morbidity and mortality,surgical procedures and complications were analyzed with SPSS11.5.Results The ileum was the most commonly location of the intestinal atresia followed by jejunum,duodenum and colon.The incidences of different types of atresia were: type Ⅰ(14.9%),Ⅱ(1.3%),Ⅲ-a(62.3%),Ⅲ-b(2.6%),and Ⅳ(18.9%).Intro-uterus intussusceptions and torsion of intestine were part of the causes of atresia with the incidence of 16.8%.The mortality was markedly decreased from 50% in 1970s to the recent 27.5%.The location of the atresia is an important factor that affects the surgical procedures and prognosis,indicating that the higher location,the poorer prognosis.Postoperative intestinal obstruction and length of the reserved intestine were another factors affecting prognosis.Conclusions The mortality is tremendously decreased due to the recent development of the neonatal surgery,anesthesia and NICU.The location of the atresia and postoperative complications such as obstruction and short bowel syndrome are the two important factors that affect prognosis.     

先天性肠旋转不良的诊治体会

目的总结小儿先天性肠旋转不良及合并畸形的临床表现特点,探讨合适的治疗方法。方法本组病人全部采用Ladd's手术方法,将压迫于十二指肠前方的腹膜带彻底松解,对合并十二指肠狭窄或闭锁者外观不容易看出,则采用从胃管向十二指肠内注入盐水的方法以确定诊断。结果41例中治愈38例,死亡3例,治愈率为92.7%。结论早期诊断、早期治疗、手术中勿遗漏合并畸形是提高治愈率,减少死亡率的关键。     

肠运动的肌电表现和肽物质控制

晚近肠运动生理及病理表现已逐步深入到研究其肌电和各种化学物质的变化,但在小儿领域中开展的尚不多,现就这二方面的进展情况作一简略介绍。     

早产儿视网膜病的危险因素分析及随访调查

研究早产儿视网膜病(retinopathy of prematurity,ROP)的发生率、高危因素、治疗与随访情况。方法对2005年7月-2007年12月温州医学院附属第一医院NICU收治的符合ROP筛查标准的早产儿,于生后2周开始由资深眼科医师开始行间接眼底镜检查眼底,并进行随访。结果434例早产儿中ROP的发生率为5.5%(24/434例),24例ROP中Ⅰ期19例,Ⅱ期3例,Ⅲ期2例。Ⅲ期阈值病变者行激光光凝治疗,全部患儿均恢复正常。对434例早产儿行单因素分析得出,胎龄、出生体重、住院时间、吸氧、吸氧浓度、吸氧时间、呼吸暂停、新生儿肺透明膜病(RDS)、肺表面活性剂(PS)的应用、机械通气、输血、光疗时间、感染与ROP的发生有相关性(P<0.05)。Logistic回归分析显示胎龄、出生体重、胎数、吸氧时间、光疗时间、代谢性酸中毒、母亲妊高症、颅内出血是影响ROP发生的主要因素。结论早产是ROP的根本原因,防治各种并发症、合理的氧疗是预防ROP的关键。建立完善有效的ROP筛查制度,早期发现、早期治疗ROP,可改善ROP的预后。     

Outcome of the Treatment of Inborn Errors of Metabolism

The use of specific dietary restrictions, cofactor administration, mobilisation of insoluble substances, environmental modifications, product replacement and selective enzyme inhibition are now established for the treatment of some inborn errors of metabolism. There is no generally accepted application for enzyme administration, cytopharmacology (manipulation of the cytoskeleton) or for cell transplantation except for bone marrow transplantation in disorders where the bone marrow is primarily at fault. The other uses of bone marrow transplantation which have been proposed require further evaluation. Results of recent research suggest that the scope of this approach is gradually being widened. There is also scope for development in the field of organ transplantation taking advantage of recent technical¹ and immunological progress. The treatment of inborn errors of metabolism by genetic modification is not yet on a practical clinical level; more laboratory and animal studies are needed before this can be attempted in man. Adenosine deaminase deficiency appears to be the disease in which this will be first attempted using a retroviral vector to insert the gene into the genome of pluripotential bone marrow cells.     

婴幼儿法乐四联症手术治疗近期疗效的影响因素

目的 探讨影响婴幼儿法乐四联症手术治疗近期疗效的各相关因素.方法 将2003年8月至2011年2月经作者一期手术纠治的117例年龄≤3岁的法乐四联症患儿分为疗效良好、疗效较差两组.分析手术时患儿年龄、体重、术前HCT值、McGoon指数、EDVI、主动脉阻断时间、转流时间、室间隔缺损大小、升主动脉与肺动脉干直径比、...     

术中判断肠活力三种方法的比较

术中准确地评价肠活力是外科医师面临的一个普遍问题。应用兔肠缺血模型，比较静脉荧光素、表面血氧测定和激光多普勒三种技术判断肠活力的精确性。结果：诊断效率静脉荧光素法为７８％，表面血氧测定为６８％，激光多普勒为９５％。后者精确度显著优于前二者，且操作简便、迅速，具有临床应用前景。     

Treatment of retinopathy of prematurity

Retinopathy of prematurity is a potentially blinding disorder of premature infants. Retinal ablation of the avascular retina originally described using cryotherapy but now most commonly undertaken with laser photocoagulation, reduces the unfavourable structural outcomes and improves the functional visual acuity outcome. The CRYO-ROP study showed the long-term benefit of treatment of threshold disease compared with no treatment, however even with cryoablation 44.4% of treated eyes had a visual acuity of 6/60 or worse at 10 year follow-up. The ETROP study of earlier treatment for high-risk pre-threshold disease, rather than treatment at threshold, has shown that pre-threshold treatment of type 1 disease produces a significantly improved outcome. Despite treatment some infants develop retinal detachment for which various surgical treatments have been described, although not always with a good functional outcome. Future treatment modalities may include the use of anti-VEGF therapies.     

主动脉缩窄的治疗研究进展

主动脉缩窄(coarctation of aorta,CoA)是主动脉的局限性狭窄,狭窄最常见于峡部,是一种常见的先天性心血管畸形,占所有先天性心脏病的5％ ～8％.缩窄导致上肢血压升高,下肢血压降低,并可引起心功能下降,未经治疗的CoA预后不佳.治疗方式包括外科治疗及经皮介入治疗,不同治疗方式的并发症发生率不同,术后长期监测其并发症、心功能是评估预后的重要指标.该文对主动脉缩窄的治疗方式及术后并发症、心功能情况的研究进展作一综述.     

Spectrum of clinical presentations of vein of Galen aneurysm

Aneurysm of the vein of Galen is a rare intracranial vascular malformation. It is known to have diverse manifestations and varying severity. Four cases with different modes of presentation and outcome are reported. A mortality of 50 per cent was encountered. Among the survivors, one had neurologic sequelae whereas the other had attained age-appropriate developmental milestones. The former was a rare case of spontaneous thrombosis of the aneurysm while the latter was a boy who underwent therapeutic embolization.     

Evaluation of the Efficacy of Treatment of Kawasaki Disease before Day 5 of Illness

We evaluated the efficacy of treating Kawasaki disease earlier than Day 5 of illness with a standard dose of immunoglobulin and aspirin. We performed a case–control study of patients with Kawasaki disease admitted to Princess Margaret Hospital from 1994 to 1999. Patients with pretreatment coronary aneurysm or those treated after day 10 of illness were excluded. All patients received immunoglobulin (2 g/kg) and aspirin (80–100 mg/kg/day) until fever subsided for 48 hours. Immunoglobulin retreatment was given for persistent fever 48 hours after the first dose of immunoglobulin or recrudescent fever. The case group consisted of 15 patients who received treatment earlier than day 5 of illness, and the control group consisted of 66 patients who were treated on or after day 5. Patients sex, age, duration of posttreatment fever, need for additional immunoglobulin, and coronary artery status were noted. Treatment efficacy was assessed by the duration of posttreatment fever and the prevalence of coronary artery aneurysms. Eighty-one patients were included in this study. There were 15 patients in the case group and 66 in the control group. No significant difference was noted in age and sex between the case and control groups. Thirty-three percent (5/15) and 8% (5/66) of the case and control groups, respectively, had persistent/ recrudescent fever 48 hours after the first dose of immunoglobulin that required retreatment (p = 0.017). Thirteen percent (2/15) and 5% (3/66) of the case and control groups, respectively, had coronary aneurysms (p = 0.158). Treatment of Kawasaki disease before day 5 of illness was associated with persistent/recrudescent fever that required retreatment. However, there was no significant increase in the prevalence of coronary aneurysm if retreatment was given. Poster presented at the third World Congress of Pediatric Cardiology and Cardiac Surgery, Toronto, Ontario, Canada, 2001     

小儿法洛四联症根治术106例临床分析

目的探讨小儿法洛四联症根治术的特点,以提高手术疗效。方法对1998-02—2005-07在河南省郑州市第七人民医院接受法洛四联症根治术的106例患儿的临床资料进行回顾性分析。结果治愈103例,死亡3例,病死率为2.9%。术后随访率100%,随访3～93个月,无晚期死亡。全部病例心功能皆恢复至Ⅰ或Ⅱ级(NYHA)。结论绝大部分法洛四联症患儿可施行一期根治术,提高手术成功率的关键在于合理的体外循环灌注、严密修补室间隔缺损和恰当疏通右室流出道。     

640������̱�������̽��

目的探讨脑性瘫痪的诊断以利防治。方法对南京市儿童医院早期干预门诊确诊的640例脑患儿[早产儿258例(早产儿组),足月儿382例(足月儿组)]分析其病因、早期临床表现及头颅CT征象,进行体格神经系统检查并智测。结果(1)高危因素,两组在颅内出血、肺部疾病早产儿组明显高于足月儿组,缺氧缺血性脑病组足月儿明显高于早产儿组,P均<0·05外余P均>0·05。(2)6个月以内确诊早产儿、足月儿两组各为28、90例的临床表现,反应差、嗜睡、少吃、少哭、少动,肌张力低下,早产儿组明显高于足月儿组,而不停啼哭,护理困难,肌张力增高,足月儿组明显高于早产儿组,P均<0·01及0·05余P均>0·05。(3)早产儿、足月儿两组的身长、体重、头围、MDI和PDI各为73·24±9·82、73·58±11·64(cm),9·12±2·31、9·23±2·80(kg),44·15±3·47、42·99±4·23(cm),44·42±6·09、41·99±7·28(分),43·81±4·95、40·80±4·51(分)。(4)头颅CT表现。早产儿、足月儿两组CT异常率各为95·4%及89·8%,P<0·05。(5)发育落后早产儿组言语落后明显高于足月儿组外,足月儿组身长<2SD明显高于早产儿组P均<0·05,余P均>0·05。结论当患儿具有高危因素、异常临床表现与神经症状、发育明显落后,应结合头颅CT密切随访可早期诊断脑性瘫痪及早干预。