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2019年浙江省医学会儿科学分会学术大会

作者： Youyang Shi Jing Huang Tingting wu Songyue Zhang Hao Li Qiaoyu Wang Ping Li rongzhou wu Children’s Heart Center The Second Affiliated Hospital and Yuying Children’s Hospital Institute of Cardiovascular Development and Translational Medicine Wenzhou Medical University

Objectives: To evaluate the feasibility, safety, and efficacy of the SHSMA patent ductus arteriosus(PDA) occluder for closure of ***: The new type SHSMA patent ductus arteriosus(PDA) occluder is novel, self-shaping Nitinol wire device with nano fabric patches integrated into the shank of the device to assure a better obturation of the ductus. The SHSMA PDA occluder has undergone animal ***: A prospective, randomized pilot study was started in August 2014.13 patients were included until June 2017. Patients weighing less than 6 kg or those with associated cardiac anomalies that required surgery were excluded. All patients were followed up by transthoracic echocardiography, electrocardiogram, and chest radiographs at 24 hours, 30, 90, 180, 360 and 720 days after implantation. All occluders were delivered via 7-9 F long sheaths and PDA closures were performed following standard ***: Thirteen patients(6 nano-film single-rivet, 7 double-rivet), were included. All the 13 patients were closed successfully using SHSMA ductal occluder, 5 patients(83.3%) in nano fabric patches singlerivet group, and 7 patients(100%) in double-rivet group had immediate and complete closure on angiography. Within 24 hours, color Doppler revealed complete closure in all 13 patients, 100% at 30 days, and in 100% of patients at 2 years. There was no device embolization, hemolysis, or obstruction to the pulmonary artery or descending ***: The new type SHSMA PDA occluder is feasible, safe and effective.

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Comprehensive Metabolomic Characterization in Coronary Artery Lesions of Kawasaki Disease

Comprehensive Metabolomic Characterization in Coronary Arter...

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2018年浙江省医学会儿科学分会学术年会

作者： Xuliang Wang Ping Li Yaoyao Yan Youyang Shi yi Zhan rongzhou wu jingzhou Chen Children's Heart Center The Second Affiliated Hospital and Yuying Children's HospitalInstitute of Cardiovascular Development and Translational MedicineWenzhou Medical University State Key Laboratory of Cardiovascular Disease FuWai HospitalNational Center for Cardiovascular DiseasesPeking Union Medical College & Chinese Academy of Medical Sciences

Background:Coronary artery lesion(CAL) was a serious complication of Kawasaki Disease(KD) which will result in stenosis and *** uncleared pathogenesis and lacking of the effective diagnostic biomarks according to CAL of KD was necessary for clinical *** and diagnostic biomarks could be discovered by metabolomic profiling of ***:40 of CAL and 39 of non-coronary artery lesion(NCAL) patients of KD after drug treatment enrolled and the plasma were collected for metabolomic *** metabolites were detected by ultra high performance liquid tandem chromatography quadrupole time of flight mass spectrometry and analyzed by multivariate statistics between ***:The metabolic profiles could clearly distinguish CAL and NCAL patients of KD at acture *** bile acid biosynthesis,phenylalanine metabolism and fatty acid biosynthesis were disorded in coronary artery *** 14 significantly different expressed metabolites were found,and palmitic acid had the biggest fold chang at 9.879 among them.A panel of 4 metabolites,1-stearoyl-sn-glycerol,palmitic acid,heptadecanoic acid and phenylethylamine had a well power to diagnosis CAL in KD patients accroding to ROC curve,the areas under the curve,sensitivity,and specificity at 0.978,95%,100%.Concluions:Plasma metabolomics had a powerful ability to discover biomarks and potential pathogenesis in KD.A panel of 4 metabolites could be biomarks to identify CAL in KD *** palmitic acid could be a new insight in furture research of KD with CAL.

关键词： Kawasaki disease Mucocutaneous Lymph Node Syndrome Coronary disease Metabolomics

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Body mass index and risk of coronary artery lesion among Kawasaki disease patients in China

Body mass index and risk of coronary artery lesion among Kaw...

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2018年浙江省医学会儿科学分会学术年会

作者： Hongying Shi Fengfeng Weng Zhenquan Wang Chen Li Zengyou Jin Zhangke Tang rongzhou wu Maoping Chu Department of Preventive Medicine School of Environmental Science and Public HealthWenzhou Medical University Children's Heart Center the Second Affiliated Hospital and Yuying Children's HospitalInstitute of cardiovascular development and translational medicineWenzhou Medical University Children's Hospital Zhejiang University School of Medicine Department of Pediatrics the First Affiliated Hospital of Wenzhou Medical University

Background and Objective:Whether body mass index (BMI) had effect on prognosis of Kawasaki Disease (KD)patients was *** aim of this study was to explore the association between BMI and coronary artery lesions(CAL) in KD ***:All consecutive KD patients from January 2009 to December 2014 in one city in China were *** on the demographics and clinical characteristics’comparison among four different BMI groups,the study finally evaluated the independent effect of BMI on CAL after adjustment for confounding factors.

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Survivin protects neonatal mouse cardiomyocytes from CVB3-induced apoptosis in a caspase-dependent manner

Survivin protects neonatal mouse cardiomyocytes from CVB3-in...

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2019年浙江省医学会儿科学分会学术大会

作者： Tingting wu Ping Li Hao Li Yi Zhan Songyue Zhang Youyang Shi Yaoyao Yan Tianhe Xia Zhenquan Wang rongzhou wu The Second Affiliated Hospital of Wenzhou Medical University

Background Viral myocarditis(VMC) is a kind of inflammatory cardiomyopathy mainly affecting children and young adults and resulting in heart failure due to dilated cardiomyopathy or cardiac arrest. Cardiomyocyte apoptosis has been shown to play a critical role in the pathogenesis of coxsackievirus B3(CVB3) induced VMC and blocking of this process may contribute to the therapeutic effect towards VMC. Therefore, the aim of this study was to examine whether survivin, one of the strongest antiapoptotic proteins, participates in the apoptotic activity after CVB3 infection and further reveal its related *** and results Herethe cultured neonatal mouse cardiomyocytes(NMCs) were exposed to CVB3 to establish the cell model of VMC and we found a time-dependent manner of survivin expression after CVB3 infection by Western Blot. Lentivirus was next used to mediate survivin expression thereby examine the function of survivin in CVB3-infected NMCs. TUNEL assay demonstrated that survivin overexpression interrupted CVB3-induced apoptosis and significantly decreased the apoptotic rate at 36 h and 60 h after CVB3 infection. It was next examined whether caspase-3 and-9 were involved in the antiapoptotic pathway initiated by survivin via Western Blot. The results showed a reverse relationship between survivin expression and cleaved caspase-3 and-9 expression, suggesting that survivin may inhibit apoptosis by restraining the activity of caspase-3 and-9. Moreover, the supernatant from cultured NMCs was extracted to detect the quantitation of released LDH and a sharp decrease was shown in survivin overexpressed group compared to CVB3 infected group, indicating a protective role of survivin in CVB3-induced cell *** In the present study, we demonstrated that survivin was triggered by CVB3 infection in NMCs in a time-dependent manner and executed its antiapoptotic effects via caspase-3-and caspase-9-dependent signaling pathway.

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primary carnitine deficiency case report

primary carnitine deficiency case report

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2019年浙江省医学会儿科学分会学术大会

作者： Yaoyao Yan rongzhou wu Zhenquan Wang Ping Li Yi Zhan Youyang Shi Tingting wu Hao Li Qiaofang Ye Qiaoyu Wang The Second Affiliated Hospital and Yuying Children’s Hospital of Wenzhou Medical Universuty

Objective:To explore primary carnitine deficiency(PCD)-induced cardiomyopathy patients’ clinical features,gene mutation and prognosis. To analyze the therapeutic dose of L-carnitine and myocardial recovery. To improve the understanding of cardiomyopathy caused by heterozygous mutation of PCD so as to promote the timely diagnosis and the accurate *** Analyzing the clinical data of PCD hospitalized in The Second Affiliated Hospital of Wenzhou Medical University in 2016, summarized and analyzed the results of gene test and patient’s clinical feature,electrocardiogram, echocardiography, plasma free carnitine level before and after *** involvement is one of the most common clinical features of PCD, which occurs on average in children aged 2-4 years. In this case, the female, who was 5 years old at the time of diagnosis, was admitted to hospital due to *** was hepatomegaly and blood ammonia increased to 110 umol/L(normally 9-30μmol/L) on admission;echocardiography showed left ventricular hypertrophy: left ventricular posterior wall during diastole(LVPWd) was 8.0 mm(normally 5-7 mm);left ventricular enlargement: Left ventricular internal diameter at end-diastolic(LVIDd) is 38 mm(normal value is less than 34 mm) and left ventricular ejection fraction(LVEF) is 58%(normal value is greater than 55%);chest radiographs suggest an increase in cardiothoracic ratio with 57.8%;electrocardiogram showed high T waves;first screen plasma free carnitine concentration was decreased to 1.19μmol/L(normal value 10-60μmol/L) and with other kinds of acylcarnitine decreased. Finally,the heterozygous mutation in exon 4 of the SLC22 A5 gene was found by the second generation DNA sequencing technique and further diagnosed as PCD. At the early stage of diagnosis, 100 mg/(kg·day) L-carnitine was given intravenously to supplement carnitine, after two weeks,the serum carnitine level returned to normal, and then changed to L-carnitine oral solution(D

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丹参酮通过调节JAK2/STAT1通路减轻病毒性心肌炎小鼠的心肌损伤

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中华心血管病杂志 2015年第2期43卷 167-172页

作者：夏天和吴婷婷邬涛任跃王珍全吴蓉洲温州医科大学附属第二医院育英儿童医院儿童心脏中心 325027

目的观察丹参酮对病毒性心肌炎小鼠心肌是否具有保护作用,并探讨其作用机制.方法清洁级近交系Balb/c小鼠,雄性,4～6周龄,共110只.采用随机数字表法将小鼠分为5组,分别为心肌炎对照组、JAK激酶2（janus kinase 2,JAK2）抑制剂组（JAK2... 详细信息

目的观察丹参酮对病毒性心肌炎小鼠心肌是否具有保护作用,并探讨其作用机制.方法清洁级近交系Balb/c小鼠,雄性,4～6周龄,共110只.采用随机数字表法将小鼠分为5组,分别为心肌炎对照组、JAK激酶2（janus kinase 2,JAK2）抑制剂组（JAK2抑制剂组）、丹参酮ⅡA组（丹参酮组）、JAK2抑制剂加用丹参酮组（JAK2抑制剂＋丹参酮组）各25只,以及正常对照组10只.苏木精伊红（HE）染色后光镜下观察小鼠心肌组织病理学改变.免疫组织化学法、蛋白质印迹分析法检测心肌信号转导子与转录激活子1（STAT1）磷酸化表达水平.化学发光法测定小鼠血清心肌肌钙蛋白-Ⅰ（cTnI）的表达水平.将心肌病理积分与磷酸化STAT1（p-STAT1）的免疫组织化学吸光度值、血清cTnI含量作相关性分析.结果（1）心肌病理炎症积分检测结果：JAK2抑制剂组和JAK2抑制剂＋丹参酮组小鼠心肌病理炎症积分均明显高于心肌炎对照组（3.35±0.57和3.34±0.54比2.12±0.39,P均＜0.01）,丹参酮组小鼠心肌病理炎症积分明显低于心肌炎对照组（1.40 ±0.34比2.12±0.39,P＜0.01）.（2）心肌细胞p-STAT1表达水平的检测结果：JAK2抑制剂组和JAK2抑制剂＋丹参酮组小鼠心肌p-STAT1表达水平的平吸光度值均明显低于心肌炎对照组（0.017±0.010和0.020±0.010比0.246±0.010,P均＜0.01）,与正常对照组比较差异则无统计学意义（P＞0.05）,而丹参酮组心肌p-STAT1表达水平则明显高于心肌炎对照组（0.444±0.060比0.246±0.010,P＜0.01）.（3）血清cTnI水平检测结果：心肌炎对照组、JAK2抑制剂组、丹参酮组及JAK2抑制剂＋丹参酮组小鼠血清cTnI水平均明显高于正常对照组[（0.42±0.06）、（1.17±0.25）、（0.23-±0.05）和（1.04±0.19） μg/L比（0.02±0.01） μg/L,P均＜0.01].JAK2抑制剂组和JAK2抑制剂＋丹参酮组小鼠血清cTnI水平均明显高于心肌炎对照组[（1.17±0.25）和（1.04±0.19） μg/L比（0.42 ±0.06） μg/L,P均＜0.01],丹参酮组小鼠血清cTnI水平则明显低于心肌炎对照组[（0.23±0.05） μg/L比（0.42±0.06） μg/L,P＜0.01].（4）相关性分析结果：p-STAT1的免疫组织化学吸光度值与心肌病理炎症积分呈负相关（R2 =0.738,P＜0.01）,血清cTnI含量与心肌病理炎症积分呈正相关（R2=0.766,P＜0.01）.结论丹参酮可通过活化JAK2-STAT1通路,上调p-STAT1表达,从而减轻病毒性心肌炎小鼠的心肌损伤.

关键词：心肌炎丹参酮

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A long-term follow-up study on clinical features and coronary artery outcomes of different age patients with Kawasaki disease

A long-term follow-up study on clinical features and coronar...

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第十三届江浙沪儿科学术会议暨2016年浙江省医学会儿科学学术年会

作者： Zengyou Jin Hongying Shi Huixian Qiu Niannian Huang Lianhong Jia Jiacheng Lu Zhimin Geng Lulu Pan rongzhou wu Maoping Chu Department of Pediatrics the First Affiliated Hospital of Wenzhou Medical University Department of preventive medicine School of Environmental Science and Public HealthWenzhou Medical University Children's Heart Center the Second Affiliated Hospital & Yuying Children's HospitalInstitute of cardiovascular development and translational medicineWenzhou Medical University

Background:Kawasaki disease(KD) is the leading cause of the acquired heart disease in children,which predominantly affects coronary *** short-term,small sample studies suggest thatyoung infants might be susceptible to developing coronary artery lesion(CAL) than older ones,although the mechanism remains *** study was to identify the clinical features and coronary artery outcomes in a 5-years follow-up period among1135 patients of KD with different ***:The clinical features and echocardiography of coronary artery lesions of KD patients in the follow-up period were *** Cox regression analysis was used to screen out the independent risk factors for the development of coronary artery ***:During the follow-up,the rate of new developed CAL in the 60 months age *** CAL incidence in the 60 months age groups after intravenous immunoglobulin(IVIG) treatment during follow-up *** CAL recover among three age groups,who had CAL before treatment,was ***,incomplete KD,IVIG method,IVIG-nonresponse and hypoalbuminemia were also identified as independent prognostic factors for CAL development in addition to ***:This large sample,long-term follow-up study demonstrates that the KD patient younger than 12 months is a high risk population of CAL,even after *** Results remind us to pay more attention to these high risk KD patients who may need a more active therapy.

关键词： Kawasaki disease coronary artery age clinical features prognostic factor

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Interventional treatment of patent ductus arteriosus combined with congenital bronchial artery pulmonary arteriovenous fistula: clinical outcomes and follow-up

Interventional treatment of patent ductus arteriosus combine...

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2019年浙江省医学会儿科学分会学术大会

作者： Ping Li Songyue Zhang Yaoyao Yan Bo Cheng Yue Ren Xing Rong Zhenquan Wang Huixian Qiu Youyang Shi rongzhou wu The Second Affiliated Hospital of Wenzhou Medical University

Background Congenital bronchial artery to pulmonary artery or vein fistula(BA-PAVF) is a rare abnormality which may induce recurrent pulmonary infection, fatal hemoptysis, and respiratory failure and it is even scarcely reported that BA-PAVF coexisted with patent ductus arteriosus(PDA).Therefore, BA-PAVF can be easily neglected or misdiagnosed in clinic without being treated *** and results In our study, 22 children were diagnosed with congenital BA-PAVF and PDA by cardiac catheterization and selective bronchial *** BAPAVF which was greater than the horizontal adjacent bronchial artery, vascular plug, MicroNester embolization coils, and interlock fibered IDC occlusion system were respectively used to close BA-PAVF after the transcatheter closure of PDA. And the size, shape, number, tortuosity degree of the fistula, the location of the lesions, the specific complications and family economic status were seriously considered before the procedure. 2 of the patients respectively existed small atrial septal defect(ASD) and small ventricular septal defect(VSD). Finally, 9 of them accepted transcatheter embolization(TCE) according to the size of the fistula. After intermediate to long-term followup, all patients have no abnormal ***-stage interventional closure of BA-PAVF and PDA can be used safely and effectively in children coexisted with BA-PAV and PDAand selective bronchial arteriography is an optimal examination to precisely localize BA-PAVF, but the establishment of specific therapy criteria needs more clinical data and summary.

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硫化氢对柯萨奇病毒B3心肌炎小鼠血红素加氧酶-1/一氧化碳通路的影响

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中华心血管病杂志 2017年第9期45卷 765-769页

作者：张松跃吴婷婷任跃夏天和吴蓉洲温州医科大学附属第二医院育英儿童医院儿童心脏中心温州医科大学心脏发育与转化医学研究所 325027

目的探讨硫化氢（H2S）对柯萨奇病毒B3（CVB3）心肌炎小鼠血红素加氧酶-1（HO-1）/一氧化碳通路的影响.方法清洁级近交系雄性Balb/c小鼠70只,分为正常组（n=10）、心肌炎组（n=20）、炔丙基甘氨酸组（PAG组,n=20）和硫氢化钠组（NaHS... 详细信息

目的探讨硫化氢（H2S）对柯萨奇病毒B3（CVB3）心肌炎小鼠血红素加氧酶-1（HO-1）/一氧化碳通路的影响.方法清洁级近交系雄性Balb/c小鼠70只,分为正常组（n=10）、心肌炎组（n=20）、炔丙基甘氨酸组（PAG组,n=20）和硫氢化钠组（NaHS组,n=20）.心肌炎组、PAG组和NaHS组分别在第0天每只小鼠腹腔注射0.1 ml10-5 69TCID50m·ml-1的CVB3建立心肌炎模型,从第0天起PAG组予以炔丙基甘氨酸（PAG）40 mg·kg-1·d-1腹腔注射,NaHS组予以硫氢化钠（NaHS） 50 μmol·kg-1·d-1腹腔注射,心肌炎组予以等量磷酸盐缓冲液（PBS）腹腔注射.对照组从第0天起每只小鼠每日腹腔注射不含病毒的等量灭菌PBS液.观察至接种后第10天各组取10只小鼠,记录小鼠体重,采血后处死留取心脏标本并记录其质量.采用心脏质量/体重比值衡量心肌水肿程度,以反应心肌炎症程度.苏木素伊红染色观察小鼠心肌病理改变并计算炎症积分.检测各组小鼠血清碳氧血红蛋白（COHb）表达水平.逆转录聚合酶链反应检测心肌细胞中HO-1 mRNA的表达水平.结果（1）各组小鼠心肌病理学特征：正常组小鼠心肌细胞结构正常,心肌纤维排列整齐,胞浆丰富均匀,横纹清晰,间质无水肿.心肌炎组、PAG组和NaHS组小鼠心肌内均可见心肌细胞坏死崩解,周围伴有大量炎症细胞浸润,以单核细胞和淋巴细胞为主,PAG组心肌细胞坏死及炎症浸润更为明显,坏死灶内可见蓝色钙化颗粒,胞核和细胞轮廓消失,NaHS组炎症较轻,心肌炎症细胞浸润范围较心肌炎组和PAG组小.（2）各组小鼠心脏质量/体重比值和心肌炎症积分：心肌炎组、PAG组和NaHS组小鼠心脏质量/体重比值均明显高于正常组（P均＜0.05）,其中PAG组又明显高于心肌炎组（P＜0.05）,而NaHS组则明显低于心肌炎组（P=0.001）.心肌炎组、PAG组和NaHS组小鼠心肌炎症积分均明显高于正常组（P均＜0.05）,但NaHS组明显低于心肌炎组（P =0.006）,PAG组则明显高于心肌炎组（P =0.038）.（3）各组小鼠血清COHb的表达水平：心肌炎组、PAG组和NaHS组小鼠血清COHb表达水平均明显高于正常组（P均＜0.05）,其中PAG组又明显低于心肌炎组（P=0.000）,NaHS组则明显高于心肌炎组（P =0.000）.（4）各组小鼠心肌HO-1 mRNA的表达水平：心肌炎组、PAG组和NaHS组小鼠心肌HO-1 mRNA的表达水平均明显高于正常组（P均＜0.05）,但PAG组明显低于心肌炎组（P＜0.05）,NaHS组则明显高于心肌炎组（P＜0.05）.（5）小鼠全血COHb表达水平、心肌细胞HO-1 mRNA表达水平及心肌炎症积分三者间的相关性：小鼠全血COHb表达水平与心肌细胞HO-1 mRNA表达水平呈正相关（r=0.927,P=0.000）,心肌炎症积分与全血COHb表达水平呈负相关（r=-0.753,P=0.000）,心肌炎症积分与心肌细胞HO-1 mRNA表达水平呈负相关（r=-0.754,P=0.000）.结论 H2S可诱导CVB3心肌炎小鼠心肌组织HO-1的表达从而上调HO-1/一氧化碳通路,进而发挥心肌保护作用.

关键词：心肌炎硫化氢碳氧血红蛋白血红素加氧酶-1 一氧化碳

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Prophylactic Transcatheter Bronchial Arterial Embolization in Treatment of Congenital Bronchial Artery to Pulmonary Artery or Vein Fistula

Prophylactic Transcatheter Bronchial Arterial Embolization i...

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2018年浙江省医学会儿科学分会学术年会

作者： Songyue Zhang Ping Li Youyang Shi Bo Cheng Yue Ren Xing Rong Zhenquan Wang Huixian Qiu rongzhou wu Maoping Chu Children’s Heart Center the Second Affiliated Hospital and Yuying Children’s HospitalInstitute of Cardiovascular Development and Translational MedicineWenzhou Medical University

Objective:This study sought to demonstrate that prophylactic transcatheter embolization(TCE) can be used safely and effectively for the treatment of congenital bronchial artery to pulmonary artery or vein(BA-PAV) fistula and selective bronchial arteriography is an optimal examination to precisely localize these abnormal ***:BA-PAV fistula is a rare abnormality and may induce recurrent pulmonary infection,fatal hemoptysis and respiratory ***,there is no recommended treatment experience or protocol in the pediatric population.

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