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??Neurally mediated syncope??NMS??is the most common type of syncope??whose exact pathogenesis is still unknown. The characteristics offamilyclustershow that NMS has a geneticmechanism. In recent years??geneticresearches of NMS are mainly concentrated on the candidate gene polymorphisms of autonomic nervous system??neurotransmitterand vasoactivemolecules??family history and twin study.     

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川崎病(Kawasaki disease,KD)于1967年由Tomosaki Kawasaki首次报道,是一种全身中小血管炎性病变为主的急性发热出疹性疾病,尤其累及冠状动脉,目前已成为儿童最常见的获得性心脏病之一[1]。KD急性期的诊断和治疗方案已经比较成熟。尽管通过正规治疗,冠状动脉病变     

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??WANG Yan*??YU Jie??LI Xiao-jing??ZHOU Min??HUANG Cheng??WANG Xue-mei.* Key Laboratory of Child Development and Disorders Cofounded by Chongqing and Ministry of Education ??Chongqing Key Laboratory of Pediatrics??Chongqing International Science and Technology Cooperation Center for Child Development and Disorders??Children’s Hospital of Chongqing Medical University??Chongqing 400014??China
Objective To explore the inhibition effect of Tanshinone IIA on the activation of nuclear factor-kappa B ??NF-κB?? and the expression of TNF-α??IL-8 in peripheral blood mononuclear cells ??PBMC?? of the acute phase of Kawasaki disease??KD?? and to explore the pathogenesis of KD and to explore the anti-inflammatory effect of Tanshinone IIA. Methods PBMCs were isolated and purified from peripheral blood of 20 KD children and 20 healthy children by density gradient centrifugation. In every sample PBMC were divided into five groups.The first group was cultured naturally?? the second one was stimulated by phorbol 12-myristate-13-acetate ??PMA????while other groups were stimulated by PMA and TanshinoneIIA which was treated with different concentrations.Each group was cultured in carbon dioxide incubator. Activation of NF-κB in PBMCs was determined by immunocytochemistry?? and ELISA was used to measure the concentration of TNF-α??IL-8 in supernatant. Results Under PMA culturing?? the activation of NF-κB was significantly higher than the blank group both in KD and control group??P??0.05???? in supernatant of the two?? the expression of TNF-α??IL-8 was significantly higher than the blank group??P??0.05??P??0.01??.Under PMA+ final concentration of 3mg / l Tan II A culturing?? the activation of NF-κB in KD and control group was significantly lower than the PMA group??P??0.05???? the expression of IL-8 in KD and control group and the expression of TNF-α in KD group were significantly lower than the PMA group??P??0.05????while the control group??concerning of TNF-α?? there was no significant decline??P??0.05??. Under PMA+ final concentration of 10mg / l Tan II A culturing?? the activation of NF-κB in KD and control group was significantly lower than the PMA group??P??0.05???? in supernatant of the KD and control group the expression of TNF-α and IL-8 was significantly lower too?? and the PMA+ final concentration of 10mg / l Tan II A group was lower than the PMA+ final concentration of 3mg / l Tan II A group??P??0.05?? both in the KD and control group. There was obvious positive correlation between the activation of NF-κB in PBMCs and the expression of TNF-α??IL-8 in the cultured supernatant??r = 0.817??r = 0.782??P??0.01????and there was also obvious positive correlation between the expression of TNF-α and IL-8??r = 0.709?? P??0.01??.Conclusion The activation of NF-κB is enhanced and the expression of TNF-α??IL-8 is increased in PBMC of the acute phase of Kawasaki disease??which may be involved in immune inflammatory response and may mediate immune vasacular injury. The anti-inflammatory mechanism of TanshinoneIIA in PBMC of the acute phase of Kawasaki disease may inhibit the activation of NF-κB and the subsequent expression of TNF-α and IL-8??and this anti-inflammatory effect has a dose-dependence     

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目的探讨血管内皮细胞抗体(AECA)检测对川崎病(KD)患儿诊断及预后评价。方法2007年8月至2008年5月在广州市儿童医院住院的5岁以下KD患儿55例,采用以人脐静脉内皮细胞和猴骨骼肌为基质的间接免疫荧光技术检测55例急性期KD患儿和43例对照组(发热对照23例,健康对照20名)血清中AECA,并通过比较AECA阴性和阳性组相应的实验室、临床指标,初步评估AECA对KD早期诊断、预后转归的临床应用价值。结果KD患儿中AECA阳性率为40.0%,明显高于健康对照组(5.0%,P=0.004),但与发热对照组差异无统计学意义(17.4%,P=0.053);AECA对KD诊断的灵敏度为40.0%,特异性为88.4%,阳性预测值与阴性预测值则分别为80.0%、53.5%,准确率为60.4%。抗中性粒细胞浆抗体(ANCA)、蛋白酶3抗体(抗-PR3)阳性KD患儿中AECA阳性率显著高于阴性者(P值分别为0.013、0.034)。AECA阳性组与阴性患儿组临床指标、实验室指标差异无统计学意义。AECA阳性组冠状动脉瘤发生率高于阴性组(P<0.05),发生动脉瘤的相对危险度为6.67(95%CI1.2～36.1)。结论...     

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目的观察不同的治疗方案下川崎病(KD)患儿治疗前后血清白介素-6(IL-6)、白介素-8(IL-8)、白介素-1β(IL-1β)、肿瘤坏死因子-α(TNF-α)、P-选择素(GMP-140)和血小板数量的变化,分析丹参酮IIA(TanIIA)对各指标的影响,探讨其作用机制。方法 2009年10月至2010年9月成都市儿童医院住院治疗的KD患儿61例。将住院KD患儿随机分为TanIIA加常规治疗组和常规治疗组,并与健康体检儿童作对照。采用双抗体酶联免疫吸附法(ELISA)检测治疗前后患儿血清IL-1β、IL-6、IL-8、TNF-α和GMP-140,用全自动血细胞分析仪检测外周血血小板计数。结果 KD患儿治疗前血清IL-6、IL-8、TNF-α、GMP-140和血小板数量均较正常对照组显著升高,治疗后5～7d血小板数量明显升高,血清IL-6、IL-8、IL-1β、TNF-α、GMP-140显著降低,其中TanIIA加常规治疗组较常规治疗组血清GMP-140、IL-6、IL-1β下降更明显,差异有统计学意义(P<0.05)。结论 TanIIA可以一定程度地降低KD患者血清IL-6、IL-1β、GMP-140,从而...     

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目的评价健康教育结合口服补液盐(ORS)治疗儿童神经介导性晕厥(NMS)的疗效。方法对2001年3月至2011年8月在中南大学湘雅二医院儿童晕厥专科门诊就诊或住院的不明原因晕厥或晕厥先兆、头晕、头痛、胸闷等儿童,经直立倾斜试验(HUTT)诊断为NMS185例,男86例,女99例,年龄4.00～17.82岁,平均(11.19±2.66)岁;病程(11.97±19.50)个月。给予健康教育及ORS治疗0.3～51.9(7.1±8.8)周后来院复诊,询问临床症状变化及复查HUTT。结果 NMS儿童接受健康教育及ORS治疗,医嘱执行依从性较好。主诉晕厥或头晕等症状好转率100%。利用HUTT客观判断,NMS患儿好转135例(73.0%),恶化50例(27.0%),好转组年龄低于恶化组[(10.92±0.24)岁v(s11.90±0.29)岁,t=-2.253,P<0.05]。口服ORS未见明显副反应。结论健康教育结合ORS治疗能有效改善NMS儿童直立不耐受症状。     

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??Kawasaki disease is an acute self-limited vasculitis. Timely and accurate diagnosis and treatment can significantly reduce the incidence of coronary artery dilatation or coronary artery aneurysm.However??there are difficulties in the diagnosis and treatment of Kawasaki disease for the time being. We need to make efforts to further improve the accuracy of diagnosis??early predict and identify the high risk population of refractory Kawasaki disease and coronary aneurysm??and optimize the initial treatment of stratified patients according to the coronary risk they are facing. We should constantly promote the experience of long-term follow-up management of Kawasaki disease so as to improve the level of diagnosis and treatment.     

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??Abstract?? The incidence of Kawasaki disease??KD?? is increasing in Asian countries??even worldwide. Refractory cases still have a strong challenge. Incomplete KD and non-responders to initial IVIG treatment may increase the rate of the coronary artery lesions??CAL??. Because of the rate of resistance to initial IVIG treatment??timely additional treatment can significantly reduce the risk of echocardiologically defined CAL in the majority of patients. It’s important to diminish inflammation?? endothelial dysfunction and CAL formation??including application of tumor necrosis factor-alpha antagonist?? methylprednisolone pulse therapy??ulinastatin and cytotoxic agents??which helps to decrease the life-long sequelae of the cardiovascular lesions.     

高分辨力超声评价川崎病后肱动脉内皮功能及静脉输注维生素C疗效观察

为探讨川崎病后肱动脉内皮依赖性血管舒张,明确川崎病急性期丙种球蛋白的应用是否对远期内皮功能异常有作用,以及维生素C的快速输注是否能改善外周血管内皮功能。应用高分辨力超声评价39例有川崎病病史患者(KD组)及17例健康对照者的肱动脉内皮功能;对KD组行静脉内输注维生素C(VC组)或安慰剂(NS组),在输注后重新评价肱动脉的内皮功能。结果显示,反应性充血所致的肱动脉内径百分变化率(RH％)在KD组明显低于对照组(P<0.0001);在接受和未行丙种球蛋白治疗者RH％无显著性差异(P=0.33);VC组输注后RH％明显增加(P<0.0001),NS组输注后RH％无明显增加(P=0.20),RH％在KD组较健康儿童减低。提示川崎病后存在周围血管内皮功能的异常;川崎病急性期大剂量丙种球蛋白的早期治疗并未能干预远期周围血管内皮功能,而快速静脉内输注维生素C可使川崎病后受损的血管内皮功能得以恢复。     

血浆内皮微粒水平变化与川崎病冠状动脉损伤关系的研究

目的 通过观察川崎病患儿血浆内皮微粒(endothelial microparticle,EMP)水平的变化,确定EMP水平变化与川崎病冠状动脉损伤的关系,探讨EMP测定在早期诊断冠状动脉损伤中的价值.方法 30例川崎病患儿均符合日本川崎病研究委员会第4次修订的诊断标准,其中24例为完全型川崎病,6例为不完全型川崎病,并按病程分为急性期、亚急性期、恢复期.根据超声心动图将川崎病组又分为冠状动脉损伤组(6例)和冠状动脉无损伤组(24例).以10例发热伴有皮疹的患儿及10例健康儿童为发热对照组和正常对照组.采用流式细胞术检测血浆中CD31+/CD42b- EMP水平.结果 川崎病患儿急性期血浆EMP水平[(8.18±2.29)％]明显高于恢复期[(2.77±0.85)％]和正常对照组[(1.34±0.38)％](P＜0.01);亚急性期血浆EMP水平[(5.93±1.05)％]明显高于恢复期和正常对照组(P＜0.01);发热对照组血浆EMP水平[(3.66±1.16)％]高于正常对照组(P＜0.05);急性期川崎病患儿中冠状动脉损伤组血浆EMP水平高于冠状动脉无损伤组,差异有统计学意义(P＜0.01).结论 血浆EMP的检测分析有助于川崎病的早期诊断及冠状动脉损伤的早期发现.     

Febrile convulsion during the acute phase of Kawasaki disease

BACKGROUND: Although seizures occur in association with meningitis or encephalitis in Kawasaki disease, febrile convulsions in Kawasaki disease are considered to be extremely rare. The aim of the present study is to elucidate the incidence of febrile convulsion in the acute phase of Kawasaki disease, in Niigata City General Hospital, Niigata, Japan. METHODS: The study included 177 patients with Kawasaki disease. Patients ranged in age from 2 months to 10 years (mean age 26.89 +/- 22.44 months). The study included 105 males and 72 females. The clinical records of Kawasaki disease patients were examined retrospectively. RESULTS: Febrile convulsions were not recognized in these 177 patients throughout the course of the disease, despite the presence of a high grade fever and their young age. However, eight of the 177 patients had experienced simple febrile convulsions during other febrile illness except for those with Kawasaki disease. In the acute phase of Kawasaki disease, only two patients showed generalized convulsion associated with prolonged consciousness disturbance and pleocytosis in the cerebrospinal fluid. CONCLUSION: The incidence of febrile convulsions in the acute phase of Kawasaki disease might be extremely low, confirming the results of previous reports. Kawasaki disease is characterized by systemic vasculitis and is sometimes complicated by intracranial vasculitis. The incidence of electroencephalographic abnormalities and pleocytosis in the cerebrospinal fluid is higher in patients with Kawasaki disease. However, the reason why febrile convulsions did not occur in the acute phase of Kawasaki disease remains unknown, despite the presence of central nervous system involvement.     

维生素D与川崎病的关系及作用机制研究进展

维生素D是重要的类固醇激素,具有广泛的生物学效应,是心血管疾病及其他多种疾病的保护因素。目前川崎病（KD）的病因及发病机制尚不清楚,但近年来的研究发现,维生素D血清水平不足或缺乏与KD存在相关性。维生素D不足或缺乏可能通过影响炎性反应、脂肪细胞因子、内皮功能、血小板功能及DNA甲基化等途径影响KD,增加其冠脉损伤发生的风险。该综述就维生素D与KD的关系及其可能存在的多种作用机制的研究进展进行阐述。     

Increased nitric oxide production by neutrophils in early stage of Kawasaki disease

Recent observations suggested that nitric oxide (NO) has a role in triggering the early endothelial dysfunction in Kawasaki disease (KD). We investigated the amount of NO in conjunction with reactive oxygen species (ROS) produced by neutrophils in children with acute KD by a newly developed flow cytometric analysis. Forty children with acute KD (n = 14), non-KD febrile disease (n = 14), and afebrile control (n = 12) were enrolled (age, 3 to 88 months). Neutrophils in KD produced significantly higher amount of NO compared to others (p < 0.05). With regard to ROS, significant increase was not only found in KD but also in non-KD febrile children (p < 0.05 and p < 0.01, respectively). In KD patients, the amount of NO produced by neutrophils decreased after immunoglobulin (IVIG) treatment, while there was no significant change in ROS production. The amount of NO in KD patients also correlated well with the days from the onset. One patient who developed coronary arterial lesion showed the highest value of NO. In conclusion, neutrophils in acute KD generate both NO and ROS considerably, while NO production is exclusive in the early stage of KD before IVIG treatment. Abnormal immune system in KD might be characterized by an overproduction of NO, whereas the role of NO in endothelial damage remains to be elucidated.     

川崎病冠状动脉血栓形成发病机制及治疗进展

川崎病并发冠状动脉血栓形成(coronary artery thrombosis,CAT)机制尚不明确,目前认为内皮细胞损伤、血液高凝状态以及冠状动脉扩张所致血流动力学改变是其主要原因。川崎病 CAT 急性期常无特异性临床表现,需积极行心脏超声监测,在血栓形成12 h 内或合并急性冠脉阻塞时需进行溶栓治疗,介入或静脉应用溶栓药物均有不同程度效果。血栓形成后慢性期应用阿司匹林联合华法林等口服药物可减少心肌梗死发生,提高远期存活率。在推荐剂量内使用溶栓及抗凝药物时较少发生不良反应,但仍需严密监测出血倾向。     

Age-Related Acceleration of Endothelial Dysfunction and Subclinical Atherosclerosis in Subjects with Coronary Artery Lesions After Kawasaki Disease

The objective of this study was to test the hypothesis that accelerated endothelial dysfunction and the development of premature atherosclerosis are associated with age in subjects with coronary artery lesions after Kawasaki disease (KD). A case-control study was performed at a university hospital that included 35 post-KD subjects across a wide age range (range, 8–42 years) without traditional cardiovascular risk factors and 35 age- and sex-matched healthy control subjects (Cont). Flow-mediated dilatation (FMD) of the brachial artery-induced by reactive hyperemia, intima media thickness (IMT), and elastic modulus (Ep) of the common carotid artery were compared between KD and Cont subjects assessed against age. KD subjects had slightly higher levels of body mass index, lipid profile, and HbA1c than Cont subjects, but the differences were not significant. The mean IMT (p < 0.001), age-adjusted percentage normal IMT (%N IMT; p < 0.0001), and Ep (p < 0.001) were significantly higher in KD than Cont subjects, and the peak FMD% (p < 0.01) was significantly lower in KD than Cont subjects. There were significant correlations between FMD% and age (r = −0.51 p < 0.0001), IMT and age (r = 0.68, p < 0.001), and Ep and age (r = 0.58, p < 0.01) in KD but not Cont subjects. When the difference in FMD% between KD and matched Cont subjects (ΔFMD%) was plotted against age, no significant relationship was found, although significant correlations between ΔIMT and age (r = 0.52, p < 0.01) as well as between ΔEp and age (r = 0.46, p < 0.05) were observed. When we defined values that were +2.0 SD over the mean control values (i.e., %N IMT ≥ 120% and/or Ep ≥ 50 kPa) as markers of subclinical atherosclerosis, 15 subjects met the criteria. Subjects over the age of 22 years were more likely to have (OR = 16.54, p = 0.0001) subclinical atherosclerosis in this cohort. Our results suggest that endothelial dysfunction and the development of premature atherosclerosis were accelerated in adult post-KD compared to Cont subjects.     

Serial changes of plasma nitrate in the acute phase of Kawasaki disease

BACKGROUND: Endogenous nitric oxide (NO) production increases with clinical conditions associated with immune stimulation. In Kawasaki disease (KD), various cytokines play a role in inflammatory reactions in the cardiovascular system. The authors hypothesized that elevated concentrations of nitrate was related to the severity of vasculitis. The aim of the present study was to evaluate serial changes of plasma nitrate concentrations in the acute phase of KD and to consider how NO is related to the inflammatory process of KD and to the coronary artery lesion (CAL). METHODS: Thirty patients with KD and 20 age-matched healthy controls were enrolled in the present study. Blood samples were obtained weekly for the first and second months. The patients were divided into two groups: one with CAL (n = 11) and another without CAL (n = 19). Plasma nitrate was measured by high-performance liquid chromatography. RESULTS: In both groups, plasma nitrate increased remarkably from the first week to the third week. Peak concentrations of nitrate (mean +/- SD, micro mol/L) in each group were as follows: 56.9 +/- 23.8 in the CAL(+) group and 68.2 +/- 33.8 in the CAL(-) group. Plasma nitrate decreased from the third week to the second month but was still elevated in both groups in comparison with the age-matched healthy controls. There was no correlation between plasma nitrates and white blood cell count or C-reactive protein, respectively (r = 0.013, 0.075). CONCLUSIONS: The results suggest that NO production may not be related to the severity of vascular inflammation and that elevated nitrate during the first month of illness may not be associated with a higher risk of CAL.     

Prevalence of coronary artery abnormality in incomplete Kawasaki disease

BACKGROUND: The aim of the present study was to determine the prevalence of coronary artery abnormality (CAA) and other clinical features in patients with incomplete Kawasaki disease (iKD) using the data from the 17th Japanese nationwide survey of KD. METHODS: iKD was defined as the presence of four or fewer of the principal symptoms of the Japanese diagnostic guidelines, regardless of whether the patient had CAA. A total of 15,857 cases were analyzed. RESULTS: Among 15,857 cases, 83.9% of patients had five to six principal symptoms (complete KD: cKD), and 16.1% had iKD. The prevalence of CAA in cKD was 14.2%, and 18.4% in iKD. The prevalence of CAA in patients with four principal symptoms was 18.1%, which was higher than in cKD cases (14.2%). Although the reliability of the data has some limitations, the prevalence of CAA among patients with one to three symptoms was 19.3%. Among all CAA patients, 14% had four symptoms, and 6% had only one to three symptoms. CONCLUSION: Incomplete KD should not be equated with mild KD. Patients with four principal symptoms were comparable to cKD with respect to CAA occurrence. In patients with one to three symptoms also, especially in those under 1 year and older than 4 years of age, other significant symptoms and laboratory findings of the guidelines are very important in making a correct and early diagnosis of iKD so as to prevent CAA.     

Changes in apolipoproteins during the acute phase of Kawasaki disease

To determine disturbances of lipid metabolism in patients with Kawasaki disease, we investigated changes in the serum levels of apolipoproteins and serum lipids. Results were as follows. Total serum cholesterol (TC) and high-density lipoprotein cholesterol (HDL-C) decreased during the early stage of Kawasaki disease. The apo A-I and A-II levels were low until the 2nd week of illness. Apo B decreased during the 1st week of illness, but rose slightly during the 2nd and 3rd weeks. The apo B/apo A-I ratio and the apo B/TC ratio were high in the early stages of illness. The group of patients with coronary artery lesions showed low levels of TC and HDL-C, and low levels of apo A-I, A-II and B during the early stages, compared with the group without coronary artery lesions. The apo B/TC ratio was significantly higher in the patients with coronary artery lesions during the 2nd week of illness. Our findings suggest an association between changes in serum lipids and apolipoproteins and coronary artery involvement in Kawasaki disease. These abnormalities may indicate the presence of early coronary arteriosclerosis.