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。     

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。     

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、...     

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.     

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     

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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。     

早产儿脑病研究进展

早产儿脑病是复杂的原发性脑损伤和继发性脑发育异常疾病.过去几年对该病有了较为深刻的认识,该文就最新的有关早产儿脑病概念演变,脑损伤后的细胞分子机制和损伤后发育障碍,尤其是活化小胶质细胞介导的少突胶质细胞损伤机制、神经/髓鞘受损和丘脑、板层下神经元及大脑皮层的受损机制或发育成熟障碍进行综述,以便对该病有更深刻的认识.     

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??Inhaled corticosteroids??ICS?? are the most effective medicine for chronic airway inflammation nowdays. Atomization inhalation has been widely applied in clinics because of its efficacy??fewer side-effects and convenience. Here??we focused on some points which should be paid attention to??including how to choose appropriate patients??how to ensure the effectiveness of inhaled steroids and how to reduce possible side-effects.     

毛细支气管炎演变为哮喘的相关因素研究

哮喘是威胁儿童健康最常见的慢性气道炎症性疾病之一.毛细支气管炎显著增加了儿童哮 喘的发病风险,研究毛细支气管炎向哮喘发展的相关因素是近年研究热点,它有助于深入认识哮喘发生、发展的机制,为哮喘提供新的防治措施.目前研究认为,毛细支气管炎在遗传学、免疫学、环境因素(如病毒感染)等方面影响着哮喘的发展,而毛细支气管炎是否直接导致儿童哮喘的发展,通过预防毛细支气管炎能否降低儿童哮喘的发病率仍有待进一步研究.     

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.     

深入认识早产儿脑病

早产儿脑病是近十年来提出的新概念,主要针对早产儿脑损伤。新生儿神经病学、神经病理学技术以及磁共振研究发现,在早产儿弥散性白质损伤同时,存在灰质损伤,并强调损伤影响进一步脑发育,其对远期神经预后的作用远重于简单的组织丢失。早产儿脑病涉及多方面问题,在疾病早期难以作出独立的疾病诊断。影像学检查是发现早产儿脑病的重要手段,对这些早产儿应实施长期随访,予以个体化治疗,以改善预后。     

Predictors of Outcome of Myocarditis

Heart failure from myocarditis may be transient or may progress to unremitting severe cardiac failure. This study was performed to determine the outcomes and prognostic features of pediatric patients with myocarditis. Patients with the diagnosis of myocarditis between 1990 and 2001 were identified through the coding system of Yale–New Haven Hospital. A total of 28 patients were included, with ages ranging from 1 day to 20 years. Before discharge, 11 patients developed unremitting severe cardiac failure. Of the remaining 17 patients, at the time of discharge 10 had normal systolic function and 7 had decreased systolic function. Unremitting cardiac failure developed in 9 of 14 patients (64%) with an ejection fraction <30% and in only 2 of 14 (14%) of those with an ejection fraction 30% on admission (p < 0.01). Furthermore, shortening fraction <15%, left ventricular dilatation, and moderate to severe mitral regurgitation on admission as well as arrhythmia were significantly associated with development of unremitting severe cardiac failure. In this series of patients with myocarditis, by the time of discharge 39% of the patients had developed unremitting severe cardiac failure, 25% had depressed systolic function, and 36% had normal systolic function. Predictive factors at admission for poor outcome were ejection fraction <30%, shortening fraction <15%, left ventricular dilatation, and moderate to severe mitral regurgitation.Data from this study was presented as a poster at the 2002 annual meeting of the Society for Pediatric Research and Pediatric Academic Societies.     

小儿孟氏骨折脱位扣眼畸形的病理认识以及环状韧带重建术的探讨

目的 对小儿盂氏骨折脱位及陈旧性盂氏骨折的传统治疗方法、创伤病理解剖、治疗结果及发生漏、误诊、误治的病例进行研究,以找到解决问题的方法,提高疗效。方法 对170例盂氏骨折脱位患儿的致病原因、治疗方法、术中所见、手术方式以及治疗效果等进行研究、分析。结果 83例中,优59例,良15例,一般9例,差5例,结论 （1）盂氏骨折脱位的定义应为：尺骨干骨折合并桡-肱及桡-尺脱位,合并/或不合并桡骨上1/3骨折、桡骨颈骨折、桡骨头骨折脱位;（2）尺骨鹰嘴骨折合并肘关节脱位不应诊断为盂氏骨折;（3）提出“超伸直型”盂氏骨折的概念;（4）尺骨骨折的稳妥治疗及坚强内固定是治疗盂氏骨折的关键;（5）桡骨颈骨折既要对位好,更要对线好,以免以后发生旋转障碍;（6）急诊患儿可行环状带修补术及关节囊紧缩术,晚期患儿应行关节囊紧缩术,不主张行环状韧带重建术。     

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。     

Reliability of subjective estimates of exercise capacity after total repair of tetralogy of Fallot

Sixty-nine patients (age 10 × 3.5 years, median 9.7 years) operated on for tetralogy of Fallot, underwent excercise testing 5.1 × 2.5 years after total correction. Aerobic capacity was assessed by the ventilatory threshold method. After the exercise test, parents completed a validated standardized questionnaire to evaluate the habitual level of physical activity of their child. They were then asked to place their child into one of three groups, describing their child's activity level, compared to normals (below average, average and above average). In the average and above-average groups, 73% and 65% of the patients were misclassified when compared to objective exercise testing. However, in the below-average group, no misclassifications were found. Subjective estimates of activity level, and consequently exercise tolerance, were poorly associated with objective measurements of exercise performance in these patient groups. Our results question the reliability of self-reported estimates of physical activity level and consequently exercise capacity in children after surgical correction of tetralogy of Fallot.     

160例儿童急性白血病免疫表型分析

目的　免疫表型可以确定细胞的来源和分化阶段 ,是急性白血病的重要生物学特征。该文了解儿童急性白血病 (AL)抗原表达规律、分布情况并探讨其临床意义。方法　采用流式细胞术直接或间接免疫荧光法检测 1 6 0例儿童AL的免疫表型。结果　 1 6 0例AL中 ,急性淋巴细胞白血病 (ALL) 1 1 2例 ,急性髓细胞白血病(AML) 4 8例。 1 1 2例ALL中 ,T系ALL 1 2例 (1 0 .7% ) ;B系ALL(不包括成熟B ALL) 93例 (83.0 % )。ALL中 ,1 3.8%有髓系抗原表达 ,以CD33阳性最常见 ;AML中 ,4 0 %有淋系抗原表达 ,以CD7阳性率最高。儿童AML淋系抗原表达阳性率高于儿童ALL髓系抗原表达 (P <0 .0 1 )。CD1 4在儿童M 4和M 5的表达频率为 77.8%。B系ALL中CD34阳性率 5 8.5 %。AML中 ,CD34的表达频率为 5 5 .3% ,其中M1和M 2的CD34阳性率为 76 .5 % ,高于其它AML病人的CD34阳性率 (4 3.3% ) ,P <0 .0 5。HLA DR抗原在M 3的表达频率为 1 1 .1 % ,低于其它类型AML病人的HLA DR阳性率 (76 .3% ) ,P <0 .0 1。B系ALL中 ,CD34表达与FAB分型、起病时白细胞数、髓外侵润情况均无关 (P >0 .0 5 ) ;CD34-组婴幼白血病发生率 (1 7.6 % )高于CD34+ 组 (4 .2 % ) ,P <0 .0 5。结论　HLA DR阴性对于M3的诊断具有一定参考价值。     

Epidemiology of respiratory distress of newborns

The present prospective study was conducted to find out the incidence, etiology and outcome of respiratory distress (RD) in newborns. All newborns (n=4505), delivered at this hospital over a period of 13 months, were observed for respiratory problems. Relevant antenatal, intranatal and neonatal information was noted. Cases were investigated for the cause of respiratory distress and followed up for the outcome. The overall incidence of RD was 6.7% Preterm babies had the highest incidence (30.0%) followed by post-term (20.9%) and term babies (4.2%). Transient tachypnea of newborn (TTN) was found to be the commonest (42.7%) cause of RD followed by infection (17.0%), meconium aspiration syndrome (10.7%), hyaline membrane disease (9.3%) and birth asphyxia (3.3%). TTN was found to be common among both term and preterm babies. While Hyaline membrane disease (HMD) was seen mostly among preterms, and Meconium aspiration syndrome (MAS) among term and post-term babies. Overall case fatality ration for RD was found to be 19%, being highest for HMD (57.1%), followed by MAS (21.8%) and infection (15.6%). Our results indicate that RD is a common neonatal problem. TTN accounts for a large proportion of thhese cases. MAS and infection also contribute significantly and are largely preventable. Without adequate ventilatory support HMD and MAS carry high mortality.