Objective:To summarize the characteristics of hypertension and target organ damage in hospitalized children, so as to provide scientific basis for comprehensive prevention and management of hypertension children.Methods:The clinical data of 165 hospitalized children diagnosed with hypertension at Beijing Children′s Hospital from January 2017 to December 2019 were analyzed retrospectively.Data including medical history, clinical symptoms, signs, auxiliary examination and treatment strategy were collected and analyzed statistically.Results:Of 165 admitted hypertensive children who met the inclusion criteria, 35(21.2%)were classified as having primary hypertension, and 130(78.8%)were secondary hypertension.The body mass index(BMI)and the rate of family history of children with primary hypertension were higher than those of children with secondary hypertension, the differences were statistically significant( t=2.052, P=0.026; χ2=3.321, P=0.009). Among 165 children with hypertension, 137 had varying degrees of clinical symptoms(83.0%), only nine primary hypertension cases showed symptoms.The original etiologies of secondary hypertension included 78 cases of renal disease(60.0%), 23 cases of cardiogenic disease(17.7%), six cases of endocrine disease(4.6%)and five cases of rheumatic disease(3.8%). Of all admitted patients, the blood pressure of 158 cases(95.8%)decreased to normal with weight control, low-fat and low-salt diet, antihypertensive drugs and treatment of primary disease.Besides, the parents of seven cases gave up any treatment and left hospital. Conclusion:Secondary hypertension is the main type of hypertension in children, among which renal parenchymal disease is the most common etiology.The clinical symptoms also vary by its original etiology.The overall goal of this study is to reduce the risk of target organ damage, and get blood pressure under control relatively early and effectively.

Objective:To analyze the clinical characteristics of Kawasaki disease(KD)with complications of acute abdominal disease and to improve the diagnosis, treatment and prognosis of patients.Methods:A total of 2 931 cases with KD hospitalized from January 2016 to December 2019 in our department were retrospectively analyzed, including 14 children with acute abdominal disease(acute abdomen group). And 62 patients with KD but without acute abdominal disease were randomly selected as the control group.The clinical characteristics, laboratory examination and treatment between two groups were compared and analyzed.Results:There were 8 males and 6 females.The average age of patients was(4.46±0.74) years.Compared with control group, there was no significant difference in age, sex and proportion of coronary artery injury in acute abdomen group( P>0.05). Acute abdominal disease group were more likely to have gastrointestinal symptoms, intravenous immunoglobulin(IVIG) resistance and longer fever duration( P<0.05). C-reactive protein(CRP), alanine aminotransferase(ALT), aspartate aminotransferase(AST), γ-glutamyltranspeptidase(GGT) and total bilirubin(TBIL) in acute abdominal disease group were significantly higher( P<0.05). There was no significant difference in white blood cell, erythroayte sedimentation rate, hemoglobin, platelet, Na + , albumin between two groups( P>0.05). All of the 14 patients used IVIG, 3 of them were treated with hormone(methylprednisolone)and 1 patient accepted infliximab.All patients were discharged from hospital.After follow-up for 6 months to 3 years, all patients had no sequelae of digestive system. Conclusion:KD can be complicated with acute abdominal disease, some of which as the first symptom.Gastrointestinal manifestations such as abdominal pain are common.For children with obvious gastrointestinal symptoms, IVIG resistance, and long duration of fever, attention should be paid to the possibility of acute abdominal disease.For children with significantly elevated CRP, ALT, AST, GGT, and TBIL should to be alert to the possibility of acute abdominal disease.KD complicated with acute abdominal disease generally has good prognosis.It is important to treat KD in the first place.

Objective:To analyze the changes of myocardial damage markers during ventricular tachycardia (SVT) onset and efficacy of various therapeutic approaches.Methods:The clinical data of 256 children with SVT admitted to Beijing Children′s Hospital Affiliated to Capital Medical University from October 2018 to October 2019 were retrospectively analyzed.According to the age of onset, children were divided into groups of 0~3 years old, >3-6 years old, and >6 years old.According to the different heart rates at the time of attack, patients were divided into the groups of <180 times/min, 180~260 times /min, and >260 times /min.According to the treatment methods, children were divided into physical therapy group, drug therapy group and radiofrequency ablation group.Frequency of SVT attacks, heart rate at the onset, serum creatine kinase isoenzyme (CK-MB), troponin (cTn) I, and brain natiuretic peptide(BNP) were collected within 2 h after the onset stop.The recovery rate of each treatment group was counted.Results:Among the 256 cases, there were 135 males and 121 females, which aged from 1 month to 18 years, mean age was (10.2±2.2) years and SVT attacked from 1 to 8 times.The faster the heart rate during SVT attack, the higher CK-MB, cTnI and BNP ( P<0.05). The recovery rate was 27.6% in the mechanical stimulation group, 98.0% in the drug treatment group and 100% in the radiofrequency ablation group, respectively.Successful recovery rate was 47.2%(34 cases)with ATP, 71.7%(109 cases)with Propafenone, 67.3%(64 cases)with Cediland, 59.6%(31 cases)with Verapamil, 61.5%(24 cases)with Sotalol and 54.7%(29 cases)with Amiodarone.Eithty-two cases were successfully converted by two drugs(32.7%) and 38 cases were successfully converted by three drugs(15.1%). Conclusion:When SVT attacks, most children have myocardial damage.The faster the heart rate, the greater the damage in the heart muscle; The treatment of SVT in children should still be the first choice of physical therapy in older children, and if it is not effective, drug therapy should be selected.Radiofrequency ablation is the most effective method to cure tachyarrhythmia.

目的:总结川崎病(KD)伴休克及心肾综合征5型(CRS-5)患儿的临床特点。方法:回顾性分析首都医科大学附属北京儿童医院2016年6月至2020年2月收治的4例KD伴休克及CRS-5患儿的临床表现、实验室检查、影像学特点、治疗及预后情况。结果:4例患儿中男3例，女1例，发病年龄2～12岁。3例患儿在KD经典症状的基础上于发病第5天出现休克表现，1例患儿以休克起病。所有患儿均同时伴有精神萎靡、呼吸心率增快、心音低钝、奔马律及尿少。4例患儿外周血白细胞计数、中性粒细胞比例、C反应蛋白、降钙素原、肾功能及N末端脑利钠肽前体均显著升高，并伴有低白蛋白血症及蛋白尿，3例患儿肌钙蛋白I升高。超声心动图提示4例患儿左心室射血分数值降低，波动在41%～55%，仅1例出现冠状动脉扩张。2例患儿泌尿系超声提示双肾增大，回声增强。4例患儿予液体复苏、应用血管活性药物、静脉用丙种球蛋白冲击、糖皮质激素抗炎、强心及营养脏器等支持治疗后，均好转出院。随访期间，所有患儿均未遗留脏器功能损害。结论:儿童KD急性期可出现休克及CRS-5表现，常伴强烈的炎症反应，心肾损伤标记物异常升高，及时正确治疗，预后良好。

目的 总结风湿性心脏炎的临床特点及用心脏彩超评价治疗前后心脏结构及功能情况.方法 回顾性分析2007年10月到2019年4月首都医科大学附属北京儿童医院收治的136例风湿性心脏炎患儿的临床特点,用心脏彩超评价治疗前与治疗后6个月心脏结构变化,分析抗风湿治疗的效果.结果 风湿性心脏炎患儿临床症状部分不典型.心脏彩超表现以瓣膜返流最为常见(86.0％),其次为心脏增大(34.6％),极少存在心包积液(0.7％).治疗前后二尖瓣、主动脉瓣、三尖瓣瓣膜返流程度差异有统计学意义(P＜0.05).左心室增大患儿治疗前左心室舒张末期前后径差异有统计学意义(P＜0.05).主动脉瓣狭窄及主动脉瓣脱垂者治疗前后均无明显改变.结论 风湿性心脏炎患儿经积极早期抗风湿及对症治疗后,瓣膜返流可较前减少,扩大的心脏可较前缩小,大部分射血分数减低患儿的射血分数升高,心包积液消失,但瓣膜狭窄及脱垂多无明显改善.

Objective:To summarize the changes of serum cytokines in 301 children with Kawasaki disease(KD) and analyze their clinical significance.Methods:Three hundred and one pediatic patients with KD admitted to Beijing Children′s Hospital, Capital Medical University between June 2018 and July 2019 were enrolled and divided into the immunoglobulin sensitive group and immunoglobulin non-response group according to the therapeutic effect.The levels of interferon gamma (IFN-γ), tumor necrosis factor-alpha (TNF-α), interleukin-10 (IL-10), interleukin-6 (IL-6), interleukin-4 (IL-4), and interleukin-2 (IL-2) were compared between the 2 groups.Results:(1)Fifty (16.6%) children had no-response to immunoglobulin.Before treatment, the level of serum cytokines in the immunoglobulin non-response group and the immunoglobulin sensitive group were as follows: IFN-γ[11.32(10.88) ng/L vs. 4.52(5.09) ng/L], TNF-α[7.43(11.25) ng/L vs. 4.42(6.04) ng/L], IL-10[14.44(20.3) ng/L vs. 8.32(7.99) ng/L], IL-6[100.28(126.39) ng/L vs. 41.34(49.32) ng/L], IL-4[1.06(1.79) ng/L vs. 0.98(1.42) ng/L], IL-2[0.48(0.90) ng/L vs. 0.40(0.62) ng/L]; there were significant differences in IFN - γ, TNF - α, IL-10 and IL-6 between the 2 groups (all P<0.05); there was no significant difference of IL-4 and IL-2 between the 2 groups (all P>0.05). (2)Before treatment and after the temperature was normal, the levels of cyto-kines in the immunoglobulin non-response group were as follows: IFN-γ[11.32(10.88) ng/L vs. 1.26(2.10) ng/L], TNF-α[7.43(11.25) ng/L vs. 1.52(3.46) ng/L], IL-10[14.44(20.3) ng/L vs. 4.63(7.00) ng/L], IL-6[100.28(126.39) ng/L vs. 22.85(39.95) ng/L], IL-4[1.06(1.79) ng/L vs. 0(0.25) ng/L], IL-2[0.48(0.90) ng/L vs. 0(0.02) ng/L]. Before treatment and after the temperature was normal, the levels of cytokines in the immunoglobulin sensitive group were as follows: IFN-γ[4.52(5.09) ng/L vs. 0.79(1.53) ng/L], TNF-α[4.42(6.04) ng/L vs. 1.60(3.4) ng/L], IL-10[8.32(7.99) ng/L vs. 3.59(3.74) ng/L], IL-6[41.34(49.32) ng/L vs. 11.90(18.84) ng/L], IL-4[0.98(1.42) ng/L vs. 0(0.88) ng/L], IL-2[0.40(0.62) ng/L vs. 0(0.14) ng/L]. The levels of cytokines decreased significantly after treatment in between 2 groups (all P<0.05). (3) Logistic regression analysis showed that IFN-γ, IL-6, fever duration and albumin were independent risk factors for no-response to immunoglobulin. Conclusions:Children with abnormally high serum IFN-γ and IL-6 levels before treatment are more likely to resist immunoglobulin.Therefore, the change of the condition in such children should be closely monitored.If there is no response to immunoglobulin, immunoglobulin should be used again as soon as possible or second-line drugs should be applied to improve the prognosis.

伊伐布雷定是一种选择性窦房结If电流抑制药,具有特异性降低心率的作用.美国食品药品监督管理局于2019年批准其用于治疗6个月及以上儿童患者因扩张型心肌病导致的稳定型心力衰竭,为儿童心力衰竭患者提供了一种新的治疗选择.本文对伊伐布雷定治疗儿童心力衰竭的作用机制、药代动力学及临床应用的现状与研究进展进行综述.

目的 探讨静脉注射免疫球蛋白(intravenous immunoglobulin,IVIG)无反应川崎病(Kawasaki disease,KD)的危险因素.方法 回顾性分析2018年6月至2019年8月首都医科大学附属北京儿童医院心脏内科确诊的391例KD患儿的临床资料,根据IVIG治疗效果分为IVIG敏感组与IVIG无反应组,比较两组发热时间、WBC、中性粒细胞绝对值、CRP、PLT、ESR、血清钠、血清钾、ALB、细胞因子;通过多因素logistic回归分析,评价IVIG无反应的独立危险因素.结果 IVIG无反应患儿45例,占11.5％.KD急性期,IVIG敏感组中性粒细胞绝对值[8.36(5.88,11.08)× 109/L比9.27(7.49,14.99)×109/L]、IFN-γ [1.17(0.40, 2.12) pg/ml比1.86 (0.67,4.34) pg/ml]、IL-10 [5.20 (3.24,8.97) pg/ml比8.02 (5.19,14.03)pg/ml]、IL-6 [19.38(11.12,41.60)pg/ml比87.41 (52.99,172.33) pg/ml]低于IVIG无反应组,ALB高于IVIG无反应组[36.00(33.10,38.33)mg/L比33.90(30.35,36.35)mg/L],差异均有统计学意义(P＜0.05).多因素logistic回归分析显示,中性粒细胞绝对值的OR值(95％CI)为1.169(1.001～ 1.365),IFN-γ的OR值(95％CI)为1.248(1.065～1.464),IL-6的OR值(95％CI)为1.105(1.002～ 1.208),均是IVIG无反应的独立危险因素(P＜0.05),数值越高,IVIG无反应风险越高;ALB的OR值(95％CI)为0.895(0.808～0.991),是IVIG无反应的保护性因素(P＜0.05),ALB越高,IVIG无反应风险越低.结论 中性粒细胞绝对值、IFN-γ、IL-6是IVIG无反应的独立危险因素(P＜0.05),ALB是IVIG无反应的保护性因素(P＜0.05).对首次IVIG治疗无效的高危患儿,应尽早再次IVIG或应用二线治疗药物,以改善预后.

目的:分析急性风湿性心脏瓣膜炎的临床特点及实验室检查,以改善预后指导临床.方法:纳入2016年1月至2019年12月,在首都医科大学附属北京儿童医院,符合研究标准且诊断为风湿热的患儿103例,其中有心脏瓣膜损害并诊断为急性风湿性心脏瓣膜炎的患儿78例,没有心脏瓣膜损害的风湿热患儿25例作为对照组.回顾性分析两组临床特点、实验室检查、心脏彩超检查情况及心电图检查情况.结果:与对照组比较,急性风湿性心脏瓣膜炎组平均年龄较大[(10.0±2.1)vs.(9.0±2.6)岁,t=2.01,P＜0.05],心脏炎发生率(100％vs.48％,x2=46.42,P＜0.05)及舞蹈病发生率(14.1％vs.零,x2=3.95,P＜0.05)较高,LDH水平[237.5(205.0,281.0)vs.205.0(180.5,226.0)U/L,Z=-2.473,P＜0.05]较高,LVEF[(66.87±6.27)％vs.(69.64±3.65)％,t=-0.21,P＜0.05]较低,LVEDD[(46.3±5.8)vs.(40.4±5.1)mm,t=4.5,P＜0.05]较大,PR间期延长比例(32.1％vs.12.0％,x2=5.328,P＜0.05)较多.最常见的瓣膜病变为二尖瓣反流,共66例(84.6％),其次为主动脉瓣反流49例(62.8％),再次为三尖瓣反流46例(59.0％),肺动脉瓣反流4例(5.9％),二尖瓣脱垂3例(3.8％),主动脉瓣脱垂2例(2.6％).未发现瓣膜狭窄患儿.结论:急性风湿性心脏瓣膜炎患儿年龄较大,心脏炎及舞蹈病发生率较高,LDH水平较高,LVEF较低,LVEDD,PR间期延长患儿较多,二尖瓣反流为最常见的瓣膜病变.

川崎病( Kawasaki disease,KD)是一种以全身非特异性血管炎为主要病理改变的儿童急性发热发疹性疾病,多侵犯冠状动脉,引起冠状动脉扩张、冠状动脉瘤,甚至血栓栓塞、冠状动脉狭窄形成,已成为儿童获得性心脏病的主要病因[1] .部分患儿巨大冠状动脉瘤合并血栓形成可导致无症状性心肌缺血、缺血性心脏病等病理状态,严重者可发生心肌梗死或猝死[2] .现将首都医科大学附属北京儿童医院心脏内科收治的1 例难治性KD伴巨大冠状动脉瘤及血栓导致猝死病例报告如下.

目的 探讨与常规标测相比,EnSite-NavX三维标测系统引导下的儿童快速心律失常射频导管消融手术的优势.方法 回顾性分析2003年3月至2015年4月于首都医科大学附属北京儿童医院行射频消融术的773例患儿的临床资料,282例应用EnSite-NavX三维标测系统(研究组),491例应用常规标测系统(对照组).比较2组间的射频消融成功率、并发症发生率、复发率及X线辐射剂量.结果 2组患者手术的成功率、并发症发生率及复发率差异均无统计学意义,研究组的辐射剂量(35.36± 11.95) mGy明显低于对照组(235.34±78.02)mGy(P＜ 0.001).结论 与常规标测相比,采用EnSite-NavX三维标测系统指导儿童快速心律失常的射频消融术具有相同的成功率及安全性,并能显著减少X线辐射剂量.

Objective To investigate the relationship between β1 adrenergic receptor(β1-AR)gene polymorphism and outflow tract ventricular premature(OTVP)in Han Chinese children.Methods A case-control study was performed.A total of 151 children with OTVP were recruited,who were diagnosed in Beijing Children's Hospital from January 2014 to December 2015,and 150 healthy children served as controls.Peripheral blood sanples were collected from all subjects and the genotype of β1-AR were determined by PCR,followed by direct sequencing.The frequency distribution of genotypes was compared between the OTVP and control groups.The association between polymorphism of β1-AR and OTVP was analyzed.Results The frequencies of β1-AR genotypes Arg/Arg,Arg/Gly and Gly/Gly were 50.5％,38.5％ and 11％ in all children.The allelic frequencies of Arg389 and Gly389 were 69.8％ and 30.2％.The allelic frequencies of Arg389 in female chidren were higher than in male (P =0.005).There were significant differences in the genotype and allele frequencies of β1-AR gene Arg389Gly between the OTVP and controls (P=0.012,P =0.002),and Arg389 allele was a risk factor (OR =1.733,95％CI:1.218 ～2.466).No differences of genotype were observed between different genders in children with OTVP (P =0.088),but there were significant differences in the genotype between loads of ventricular premature and nonsustained ventricular tachycardia (P =0.029,P =0.019).Conclusion The gene polymorphism of Arg389Gly in β1-AR might be associated with the OTVP in Han Chinese children and Arg389 allele gene is a risk factor.The genotype of Arg/Arg might be a genetic susceptibility factor of high-level loads of ventricular premature and nonsustained ventricular tachycardia in children with OTVP.

Objective To observe the clinical efficacy and safety of Peramivir in the treatment of influenza among children with cardiac insulfficiency.Methods Based on the prospective study design,the children patients with cardiac insulfficiency and influenza at the same time were divided into the treatment group and the control group according to whether they accepted voluntarily peramivir injection.These patients were admitted to the cardiology department of Beijing Children's Hospital from January 2016 to January 2017 and all cases were treated with conventional symptomatic treatment.Both groups were evaluated for fever remission time,symptom relief time,adverse reactions,complications,antibiotic use and so on.Results There were 30 cases in treatment group,and 30 cases in control group.The average fever remission time was (19.9±10.6) h and (57.6 ± 33.7) h in the treatment group and control group,respectively.The mean symptom relief times were (39.9 ± 39.8) h and (60.7 ± 33.3) h in the two groups.The relief time of main symptoms such as fever,cough,sore throat,nasal obstruction / nasal catarrh in the treatment group were significantly less than that of the control group (P＜0.05).The incidence rates of complications were 10.0％ (3/30) and 40.0％ (12/30) in the two groups,respectively,and the rates of antibiotics usagewere 30％ (9/30) and 66.7％ (20/30).The complications,the use of antibiotics and the time of medication in the treatment group were significantly less than those in the control group after treatment (P＜0.05).The average costs was 658 yuan and 792 yuan in the two groups respectively with no significant difference between the two groups (P＞0.05).Conclusions The use of peramivir in children with cardiac insulfficiency can significantly shorten the remission time of fever,quickly relieve symptoms,reduce complications and the use of antibiotics,without increasing of the total cost.

21世纪临床输血已经进入成分输血时代,机采血小板的应用得到了临床医学的广泛认同.但在机采血小板过程中,如何安全有效节省献血后按压止血的时间,避免因更换手指按压带给献血员麻烦及按压失败导致出血,造成献血员心理负面影响等.我科于2013年1月应用采血护士手指按压献血员手指的同时按压创可贴的方法,取得较好效果.

目的 探讨骨科手术患者自体血回输的安全性和有效性. 方法 回顾性分析我院2013年5月—2015年5月收治的235例骨科手术患者的临床资料,其中118例采用自体血回输的患者作为观察组,异体输血的117例为对照组. 观察两组患者手术时间、输血量及输血前及输血后12 h监测电解质及血常规,观察术后12 h血液流变学情况,对输血反应发生情况进行记录. 结果 两组输血量及手术时间比较差异无统计学意义( P>0. 05 ). 两组组内不同时间段电解质与凝血各指标比较差异无统计学意义(P>0. 05);观察组输血后12 h血红蛋白与红细胞压积高于对照组(P<0. 05). 两组血液流变学及住院天数比较差异均有统计学意义(P<0. 05). 术后两组均未出现明显输血反应、出凝血功能异常及全身感染等症状. 结论 回收式的自体输血是骨科手术重要的血液保护措施,对安全、节约用血有积极作用.

目的：探讨首诊误诊为淋巴结炎的不完全川崎病（IKD）的临床特征，以降低此类川崎病（KD）的误诊率，减少冠状动脉并发症的发生。方法回顾性分析2002年1月至2010年12月北京儿童医院收治的 KD患儿临床资料，以发热和颈部淋巴结大为首发症状而误诊为淋巴结炎的 IKD 患儿作为 IKD 组，典型 KD 患儿作为 KD 组，比较2组患儿临床特征的差异。结果共1004例患儿纳入研究，IKD 组31例（3.1%），KD 组973例（96.9%），2组患儿均以1~2岁起病多见，分别占38.7%（12例）、26.4%（200例），男女构成比分别为2.44：1.00、1.76：1.00，IKD 组平均发热时间[（10.3±5.7）d]显著长于 KD 组患儿[（7.4±3.6）d]（t ＝0.162，P ﹤0.05）。IKD 组以发热、颈部淋巴结大为主要临床表现，单侧与双侧淋巴结大患儿各8例（各占25.8%），余患儿单双侧不详；其中4例（12.9%）伴皮疹，6例（19.3%）伴口唇改变，7例（22.6%）伴指端脱皮，10例（32.3%）伴结膜充血。实验室检查中 IKD 组的 WBC、C 反应蛋白（CRP）水平低于 KD 组（P ﹤0.05），清蛋白、乳酸脱氢酶水平高于 KD 组。IKD 组患儿对静脉用丙种球蛋白无反应的发生率低于 KD 组（3.6%比16.2%，χ2＝3.817，P ﹤0.05）。IKD 组患儿冠状动脉扩张、冠状动脉瘤和巨大冠状动脉瘤的发生率分别为71.0%（22/31例）、16.1%（5/31例）和9.7%（3/31例），高于 KD 组[28.1%（273/973例）、4.9%（48/973例）、1.1%（11/973例）]（χ2＝3.540，P ﹤0.05）。结论首诊误诊为淋巴结炎的 IKD 较典型 KD 患儿发热时间长、冠状动脉并发症的发生率高，实验室检查中 WBC、CRP 偏低，在临床工作中应对其提高警惕，对于发热合并淋巴结改变的患儿注意除外 KD 诊断，减少因误诊或漏诊导致的冠状动脉病变的发生。

目的:探讨婴幼儿无休止特发性室性心动过速的临床特点及诊治方法.方法:对2005年7月至2013年8月,在北京儿童医院住院确诊为婴幼儿无休止特发性室性心动过速的18例患儿的临床表现、实验室检查及治疗方法,进行回顾性分析.结果:婴幼儿无休止特发性室性心动过速男女比例2.6∶1,平均年龄13个月,就诊形式以烦躁哭闹为表现的6例,呕吐尿少的3例,发现心跳快的8例,伴有阿-斯发作(adams-stokes综合征)即心源性脑缺血综合征的4例,肺炎时发现心脏大的1例.心电图表现为持续的室性心动过速,速率160～ 300次/min,QRS时限为0.08～0.12s,V1以右束支阻滞形态的15例,来源于左心室,V1以左束支阻滞形态的3例,来源于右心室.心脏彩超4例正常,7例心脏左心室扩大,7例左心室中至重度扩大,心脏收缩功能减低,提示为心动过速心肌病,左心室型的盐酸维拉帕米缓释片应用12例,7例有效,5例无效,盐酸普罗帕酮片应用9例,3例有效,胺碘酮应用5例,1例有效.右心室型的应用酒石酸美托洛尔片3例,2例有效,胺碘酮应用1例,无效.洋地黄、三磷酸腺苷、利多卡因及艾斯洛尔无效,电击复律6例(20次),2例(4次)有效.4例行射频消融,室性心动过速消失,均无复发.结论:婴幼儿无休止特发性室性心动过速临床症状轻重不一,由于速率快,持续时间长易出现心动过速心肌病,并发心力衰竭、心源性休克及心脏猝死.药物治疗难度大,准确的分型有利于准确用药.射频消融为较好的根治方法.

Objective To study the clinical characteristics and curing methods of non-compaction of ventricular myocardium(NVM) in children with a view to improve its diagnosis and treatment.Methods From Jan.2009 to Dec.2012,the clinical data,treatment and follow-up of 22 patients with NVM in Beijing Children's Hospital Affiliated to Capital Medical University were analyzed.Results The 22 patients included 13 boys and 9 girls aged from 4 months to 13 years and 3 months with an average age of 3 years and 7 months.Five of them had a family history of cardiac diease.Twenty patients suffered from ventricular dysfunction,with 2 cases at level Ⅰ,5 cases at level Ⅱ,7 cases at level Ⅲ and 6 cases at level Ⅳ.Asymptomatic heart murmur was heard in 6 patients and creatine kinase car-diac isoenzymes raise was found in 6 patients.X-ray indicated that heart shadow enlargement and electrocardiographic abnormalities in all 22 patients.Echocardiography features of all cases indicated left ventricular enlargement,and 20 cases of the children had ventricular systolic dysfunction.Left ventricle was detected in 21 patients,while double ventricle dysfunction detected in 1 patient.Thrombus was found in none of the patients.Magnetic resonance imaging was performed in 8 cases of the patients and CT was performed in 14 cases,all of them showed abnormalities.Anti-heart failure medications were administered to 20 patients and aspirin to all of the children.The patients were followed up for 0.5 to 3.0years.Two patients died from heart failure during the follow-up.In 2 cases whose cardiac ejection fraction was more than 60％ had little change of the internal diameter of left ventricle and cardiac function.In 3 cases,the left ventricular diameter narrowed by 3 to 5 millimeter and ejection fraction increased by 5％ to 10％ over the previous.In 5 cases,the left ventricular diameter expanded by 5 to 7 millimeter and ejection fraction decreased by 3％ to 5％.In 10 cases,the left ventricular diameter and heart function had no significant change.Conclusions The main clinical manifestions of NVM in children are cardiac dysfunction,and the prognosis is usually poor.Echocardiography is the principal means of diagnosis.Magnetic resonance imaging can contribute to the diagnosis.

Objective To investigate clinical characteristics of children with fulminant myocarditis . Methods The 50 cases of fulminant myocarditis were retrospectively analyzed .The children′s gender, age, clini-cal manifestations , treatment and prognosis were summarized .Results Among fifty cases of fulminant myocarditis in children,16 cases(32.0%) had digestive tract symptoms, 14 cases (28.0%) had cardiovascular symptom, 8 cases (16.0%) had nervous system symptoms .Asperger syndrome was the primary symptom in 26.0%of chil-dren;46 cases (92.0%) had abnormal electrocardiogram;positive rate of myocardial enzyme index and heart col-or doppler flow imaging were 48.0%(24 cases) and 50.0%(25 cases);32 cases were given immunoglobulin in-fusion;44 cases had prednisolone; 28 cases with Ⅲ degree atrioventricular block had temporary pacemaker in-stalled based on the comprehensive treatment .4 cases were given final permanent pacemaker installation .6 cases had lidocaine;7 cases had amiodarone;8 cases were given synchronous cardioerter .17 tachycardia was termina-ted;one case had temporary pacemaker installed after converting sinus rhythm;2 cases with ventricular fibrillation died.Conclusions Most of children with fulminant myocarditis have diverse symptoms and most are not typical ;misdiagnosis rate is high .Comprehensive analysis of the results of electrocardiogram ,echocardiography and myocar-dial enzymology markers may improve the diagnosis of fulminant myocarditis and may also indicate the severity and prognosis.