经导管同期介入治疗儿童复合型先天性心脏病8例分析及随访

目的：儿童先天性心脏病(congenital heart disease,CHD)复合畸形(复合型CHD)既往外科手术是唯一的治疗方法,随着介入治疗技术的日渐成熟和新型封堵器械的不断出现,经导管同期介入治疗儿童复合型CHD引起人们的关注,目前,国内外报道尚不多。该研究对儿童复合型CHD经导管介入治疗的方法及疗效进行探讨。方法：应用经导管介入治疗方法同期封堵治疗8例儿童复合型CHD,其中男4例、女4例,平均年龄(6.1±2.9)岁,其中房间隔缺损(ASD)伴室间隔缺损(VSD)、动脉导管未闭(PDA)1例,ASD伴PDA、肺动脉瓣狭窄(PS)1例,ASD伴PDA 1例,卵原孔未闭(PFO)伴PS 1例,ASD伴PS 4例。经导管介入治疗的原则是:ASD伴VSD、PDA者,先行VSD封堵术,再行PDA封堵术,最后行ASD封堵术;ASD伴PDA、PS者,先行经皮球囊肺动脉瓣成形术(PBPV)纠正PS,再行PDA封堵术,最后行ASD封堵术;PFO伴PS和ASD伴PS者,先行PBPV纠正PS,其次行PFO或ASD封堵术。结果：8例复合型CHD均1次治疗成功,术中未发生任何严重并发症。术后即刻超声和造影检测显示VSD、ASD及PDA均无残余分流,6例合并PS者,跨肺动脉瓣压差由术前的75.3±15.6 mmHg下降至术后的14.0±5.6 mmHg,经3.4±1.2年随访证实无残余分流,跨瓣压差均在通常标准良好的范围内,无任何并发症发生。结论：经导管介入治疗儿童复合型CHD技术要求较单纯型CHD相对高,宜遵循先难后易、后期操作不影响前面治疗的原则,规范操作,可获良好的治疗效果。     

先天性心脏病复合畸形的介入治疗

目的探讨介入治疗小儿先天性心脏病(CHD)复合畸形的可行性和疗效。方法对CHD复合畸形患儿10例施行联合介入治疗,其中房间隔缺损(ASD)并肺动脉瓣狭窄(PS)2例,室间隔缺损(VSD)并动脉导管未闭(PDA)4例,PDA并PS 2例,ASD并PDA 2例。结果7例1次治疗成功,余3例中2例分2次治疗,1例8个月患儿,因其在最初0.5年随访中发现VSD明显缩小,故先封堵了PDA,目前正在随访中。成功率100%,无严重并发症。经1个月-3年随访,患儿心脏缩小,心脏功能改善。结论在病例选择恰当情况下,对小儿CHD复合畸形进行介入治疗是安全、可行的。     

先天性心脏病介入治疗与传统外科手术治疗的临床比较

目的对房间隔缺损(ASD)、室间隔缺损(VSD)、动脉导管未闭(PDA)的介入治疗(Amplatzer封堵器)和外科手术治疗的临床疗效进行比较。 方法研究对象为2004 05—2005 05在北京安贞医院小儿心脏科住院患儿80例,符合单一左向右分流畸形ASD、VSD、PDA,根据治疗方法不同分为介入组(40例)和手术组(40例);比较2组在疗效、费用、并发症、输血量及住院时间等方面的异同;通过放免法测定2组ASD及VSD治疗前后的心钠素(ANP)水平。 结果2组手术成功率均为100%;介入组均未输血,手术组均输血治疗;术后住院时间介入组少于手术组(P<0.01);治疗费用介入组高于手术组(P<0.01);ANP水平,外科手术及介入治疗后均较治疗前降低。 结论单一ASD、VSD、PDA的介入治疗与手术治疗临床效果相同;介入组不需输血,术后住院时间短,但治疗费用较昂贵。     

���������ಡ���������봫ͳ����������Ƶ��ٴ��Ƚ�

目的对房间隔缺损(ASD)、室间隔缺损(VSD)、动脉导管未闭(PDA)的介入治疗(Amplatzer封堵器)和外科手术治疗的临床疗效进行比较。方法研究对象为2004-05—2005-05在北京安贞医院小儿心脏科住院患儿80例,符合单一左向右分流畸形ASD、VSD、PDA,根据治疗方法不同分为介入组(40例)和手术组(40例);比较2组在疗效、费用、并发症、输血量及住院时间等方面的异同;通过放免法测定2组ASD及VSD治疗前后的心钠素(ANP)水平。结果2组手术成功率均为100%;介入组均未输血,手术组均输血治疗;术后住院时间介入组少于手术组(P<0.01);治疗费用介入组高于手术组(P<0.01);ANP水平,外科手术及介入治疗后均较治疗前降低。结论单一ASD、VSD、PDA的介入治疗与手术治疗临床效果相同;介入组不需输血,术后住院时间短,但治疗费用较昂贵。     

小儿先天性心脏病介入治疗后心肌损伤和炎症反应的研究

目的：研究先天性心脏病（CHD）介入治疗后7 d心肌损伤和炎症反应。方法：选择77例CHD患儿,其中室间隔缺损（VSD）12例,肺动脉瓣狭窄(PS)14例,房间隔缺损（ASD）14例,动脉导管未闭（PDA）37例。检测术后即刻、术毕、术后6 h、24 h、72 h及7 d 的血清心肌酶（AST、CK、CKMB）、肌钙蛋白I(cTnI)和CRP值的变化。结果：VSD组AST水平于术后即刻,术后6 h和术后24 h明显高于其他3种类型CHD组（P<0.05）;术后即刻及术后6 h各组间CK及CKMB水平差异有统计学意义,以VSD组最高;术后72 h和术后7 d PDA组CRP水平明显高于其他3种类型CHD组（P<0.05）。与术前相比,术后6 h和24 h,4种类型CHD组AST均明显升高（P<0.05）;术后即刻和术后6 h,VSD组CK和CKMB水平较术前明显升高（P<0.05）;术后即刻和术后6 h,VSD、PDA和PS组cTnI水平较术前明显升高（P<0.05）;PDA组CRP水平术后24 h、72 h和7 d较术前明显升高（P<0.05）。结论：介入治疗CHD 7 d内可引起轻微心肌损伤,以VSD明显,主要集中在术毕至术后24 h较短时间内,未引起明显炎症反应。     

常州市103例新生儿先天性心脏病随访分析

目的探讨建立符合中国国情的新生儿先天性心脏病(CHD)筛查、评估、干预体系。方法 2011年5月1日至2013年4月30日期间,对常州市妇幼保健院出生的16 070名新生儿进行CHD筛查及诊断,并对CHD新生儿定期随访至1岁。结果发现CHD 103例,发生率0.641%。CHD构成类型前三位是室间隔缺损(VSD,0.205%)、动脉导管未闭(PDA,0.174%)、房间隔缺损(ASD,0.10%)。103例CHD完成1年随访94例、失访9例,其中小型VSD、ASD及早产儿PDA闭合率高,部分严重CHD及有并发症患儿已手术治疗,其余未愈者仍在随访中。结论常州地区新生儿CHD发病率处于中国平均水平,对CHD患儿进行系统随访能有助于提高CHD患儿的生存质量。     

儿童先天性心脏病介入治疗362例效果分析

目的总结分析儿童先天性心脏病介入治疗的效果及并发症。方法本研究收集2014年1月至2016年12月在本院治疗的362例先心病患儿为研究对象,针对PDA患儿采用蘑菇伞封堵法、弹簧圈封堵法、国产ADOⅡ封堵法,VSD、ASD患儿采用蘑菇伞封堵法,PS患儿采用肺动脉瓣球囊扩张术单球囊法和双球囊法。结果 352例患儿成功完成手术,手术成功率为97.24%,术中死亡病例为1例重度PS合并冠状窦间隙开放患儿;转外科治疗5例,包括术中行心导管检查发现PDA合并主动脉弓离断畸形2例,PDA合并主动脉缩窄1例,ASD合并肺静脉异位引流2例;中止手术4例,包括术中发现VSD已快愈合而不需手术的3例,VSD术中出现Ⅲ度房室传导阻滞而中止手术的1例。352例成功完成手术的患儿中7例术后出现并发症,发生率为1.99%,3例残余分流,术后6个月随访时,残余分流均消失;3例心律失常,术后6个月随访时1例VSD封堵术的患儿仍存在散在室性早搏,其余患儿心电图均恢复正常;1例股动脉血栓经溶栓治疗痊愈。结论儿童先心病介入治疗的成功率高,并发症的发生率较低,但应严格掌握适应证与禁忌症,熟练掌握操作技术,术后需长期随访。     

婴幼儿先天性心脏病并心内膜弹力纤维增生症的临床特点

目的研究婴幼儿先天性心脏病(CHD)并心内膜弹力纤维增生症(EFE)的临床及实验室特点,诊断和治疗。方法对本院收治婴幼儿CHD并EFE患儿的临床及实验室检查资料进行回顾性分析、总结。结果3166例CHD中21例(0.66%)并EFE,其中动脉导管未闭(PDA)5例,房间隔缺损(ASD)6例,室间隔缺损(VSD)6例,PDA VSD 1例,ASD VSD 2例.法洛四联症1例。临床有不同程度心力衰竭,多伴肺炎,心脏增大较明显,起病绝大多数较急,病情多较重.预后较差。结论CHD是引起EFE的原因之一,但不除外其他原因尤其是病毒感染导致EFF;疾病预后可能与心脏扩大程度、心力衰竭程度、并发症严重程度及治疗及时与否有关。     

左向右分流型先天性心脏病患儿心脏结构和功能改变与骨龄指数的相关性

目的探讨左向右分流型先天性心脏病(CHD),包括房间隔缺损(ASD)、室间隔缺损(VSD)、动脉导管未闭(PDA)患儿的骨龄变化以及心脏结构和功能改变与骨龄指数(BAI)的相关性。方法收集确诊左向右分流型CHD共130例,其中ASD 52例、VSD 46例、PDA 32例,超声检测各项心脏结构和功能指标,摄左手及腕关节正位片,应用最新《中国儿童骨龄评分法》图谱进行骨龄评定并计算BAI,对比分析各组骨龄以及CHD各项心脏结构和功能指标与BAI的直线相关性。结果 ASD、VSD与PDA患儿BAI的差异有统计学意义(P0.05);PDA组的BAI分别高于ASD组和VSD组,差异均有统计学意义(P均0.05)。无PAH、轻度PAH以及中重度PAH三组的BAI分别为(0.84±0.09)、(0.75±0.07)和(0.65±0.08),差异有统计学意义(F=27.77,P=0.000);小缺损组的BAI为(0.82±0.09),高于中大缺损组的(0.73±0.10),差异有统计学意义(t=3.54,P=0.002)。ASD、VSD患儿缺损大小(DS)与BAI均呈负相关(r=-0.48、-0.54,P均0.05);ASD、VSD、PDA患儿肺动脉收缩压(PASP)以及肺循环血流量/体循环血流量比值(Qp/Qs)均与BAI呈负相关(r=-0.64~-0.38,P均0.05)。结论左向右分流型CHD患儿的骨龄及BAI均显著落后于正常同龄儿童;左向右分流型CHD患儿骨龄落后程度与PASP、DS及分流量大小相关。     

超声心动图在新生儿先天性心脏病筛查中的应用

目的探讨超声心动图对新生儿先天性心脏病(CHD)临床筛查的应用价值。方法对本院2010年1月-2011年12月出生的330例疑似CHD新生儿的心脏超声心动图筛查结果进行分析,应用心脏彩超对新生儿经胸行多切面扫查,了解心脏及大血管位置关系、内径比例,测量房室大小、室壁厚度、运动幅度,测量心功能。观察瓣膜厚度、活动度、房室间隔是否完整。测量瓣口血流速度,观察瓣口有无返流,心腔及大血管间是否存在分流。结果 330例新生儿中单纯卵圆孔未闭96例(29.0%),单纯PDA 45例(13.6%)。CHD 116例(35.1%),其中单纯VSD 16例(13.8%),VSD并卵圆孔未闭15例(12.9%),单纯ASD 13例(11.2%),ASD并卵圆孔未闭13例(11.2%),ASD并VSD 8例(6.9%),ASD并VSD并卵圆孔未闭7例(6.0%),ASD并PDA 17例(14.7%),法洛四联症14例(4.2%),完全性心内膜垫缺损6例(1.8%),右心室双出口3例(0.9%),大动脉转位、三尖瓣下移畸形各2例(各占0.6%)。结论新生儿期较为多见的心内分流为卵圆孔未闭,其与PDA在未来均有闭合的可能。目前绝大多数常见的CHD可通过超声检查得到确诊,可取代大部分心导管检查和心血管造影对于CHD的诊断。     

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.     

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     

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。     

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.     

�����뼤���ٴ�Ӧ��ע���

??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.