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Kawasaki disease (KD) is an idiopathic form of acute systemic vasculitis, which clinically mimics febrile diseases. Although it has been hypothesized that immune system malfunction is associated with KD, its etiology remains unclear. The aim of the present study was to identify a KD‑associated antibody. Immunoproteomic methods were used to identify KD‑associated antigens that could be recognized in the sera of patients with KD. HeLa cells were used as an antigen source and KD sera were used as probe antibodies to determine the binding of the antibodies using an indirect immunofluorescence assay. Western blotting was performed to identify KD‑associated antigens in HeLa whole cell lysates. Eight out of 12 serum samples obtained from patients with KD demonstrated immunoreactive bands at ~70 kDa, which was later determined to be heat shock cognate 71 kDa protein (HSP7C) by mass spectrometry. The diagnostic value of serum anti‑HSP7C antibodies for KD was assessed using ELISA. Using a cut‑off value of 0.267, anti‑HSP7C antibodies were observed to be present in the sera of 60.00% (30/50) of patients with KD, in 21.05% (8/38) of non‑KD febrile controls, and in 5.26% (2/38) of healthy controls. High serum levels of anti‑HSP7C antibodies were detected in the peripheral circulation of patients with KD. To the best of our knowledge, the present study is the first to observe the high expression levels of anti‑HSP7C antibodies in patients with KD. Therefore, anti‑HSP7C antibodies may be used as a diagnostic marker to detect KD.

Syncope belongs to the transient loss of consciousness (TLOC), characterized by a rapid onset, short duration, and spontaneous complete recovery. It is common in children and adolescents, accounting for 1% to 2% of emergency department visits.Recurrent syncope can seriously affect children's physical and mental health, learning ability and quality of life and sometimes cardiac syncope even poses a risk of sudden death. The present guideline for the diagnosis and treatment of syncope in children and adolescents was developed for guiding a better clinical management of pediatric syncope. Based on the globally recent development and the evidence-based data in China, 2018 Chinese Pediatric Cardiology Society (CPCS) guideline for diagnosis and treatment of syncope in children and adolescents was jointly prepared by the Pediatric Cardiology Society, Chinese Pediatric Society, Chinese Medical Association (CMA)/Committee on Pediatric Syncope, Pediatricians Branch, Chinese Medical Doctor Association (CMDA)/Committee on Pediatric Cardiology, Chinese College of Cardiovascular Physicians, Chinese Medical Doctor Association (CMDA)/Pediatric Cardiology Society, Beijing Pediatric Society, Beijing Medical Association (BMA). The present guideline includes the underlying diseases of syncope in children and adolescents, the diagnostic procedures, methodology and clinical significance of standing test and head-up tilt test, the clinical diagnosis vasovagal syncope, postural orthostatic tachycardia syndrome, orthostatic hypotension and orthostatic hypertension, and the treatment of syncope as well as follow-up.

Mutations that affect the STING1 (TMEM173) gene cause a rare autoinflammatory syndrome, which is known as STING-associated vasculopathy with onset in infancy (SAVI) and which was initially described in 2014 (1). Thus far, only four reports have been conducted regarding families affected with SAVI in the literature. In this article, the clinical, laboratory, and genetic characteristics of two generations (three cases) of SAVI are described. Unlike previously reported cases that were caused by STING1 mutation, the initial and major clinical manifestations of the mentioned cases are largely identified in the lungs with interstitial lung disease (ILD), and the evidence of typical extrapulmonary symptoms of early-onset systemic inflammation (e.g., cutaneous vasculopathy) were minimal except for the proband, who was diagnosed with arthritis 8 years after onset. In addition, a younger sibling showed no symptoms. Such reports are rarely related to mutations in STING1. The proband was examined with bronchoscopy and alveolar lavage to determine the cause. This study emphasizes that, in the clinical assessment of interstitial pneumonia in children, the possibility of STING1 mutation should be considered, especially in patients with arthritis in addition.

Previous studies have shown that children with Kawasaki disease (KD) who fail to respond to intravenous immunoglobulin (IVIG) therapy are at higher risk of developing coronary artery abnormalities. We aimed to conduct a meta-analysis to uncover the risk factors that were closely related to IVIG resistance in children with KD. PubMed, Embase and Cochrane Library databases were searched up to 31st October 2019, 23 case-control studies were finally eligible, enrolling 2053 patients of IVIG resistance and 16635 patients of IVIG sensitivity. We comprehensively analyzed potential factors using stata15 software with a standard meta-analysis procedure and consequently found that in addition to patients with polymorphous rash or swelling of extremities symptom had a tendency to be non-responders, IVIG resistance was more likely to occur in patients with severe anemia, hypoalbuminemia, lower baseline platelet count, and higher erythrocyte sedimentation rate (ESR), bilirubin, alanine aminotransferase (ALT) and percentage of neutrophils. Particularly, male sex, hyponatraemia, high aspartate aminotransferase (AST) and C-reactive protein (CRP) were confirmed as the risk factors favor IVIG resistance in Mongoloid patients from Asia countries, but not in Caucasians from non-Asia regions. White blood cell count, age, conjunctivitis, oral changes and cervical lymphadenopathy were identified as unrelated factors. In summary, we report various risk factors and irrelevant factors of IVIG resistance in children with KD and recommend clinicians consider these factors closely, a reduction in coronary artery lesions (CAL) rates is prospectively expected with accurate prediction and timely aggressive initial treatment in high-risk patients.

Background: Kawasaki disease (KD) is an acute vasculitis often complicating coronary arterial lesions, and gradually becomes the leading cause of acquired heart disease instead of rheumatism. Previous studies often regards elevation of serum lactate dehydrogenase (LDH) level as an auxiliary diagnosis marker when judging myocardial diseases. Methods: We analyzed the serum LDH levels in newly confirmed KD patients, compared with febrile non-KD patients and healthy donors as controls. Then a subtype of LDH protein (LDHA) were over-expressed and purified for antigenicity analysis of potential anti-LDHA autoantibodies in IgA, IgM and IgG isotype by homemade ELISA, respectively. Results: An obvious elevation serum levels of LDH was found in 5/27 KD patients (19%, p

AimThis study was designed to investigate the value of history taking in identifying children with cardiac syncope, and to improve diagnostic efficiency and accuracy in children with cardiac syncope.Methods and ResultsWe compared the characteristics of a group of children and adolescents with cardiac syncope at the Pediatric Syncope Unit of five hospitals in China with those with typical vasovagal syncope. We included a cohort of 275 patients in Pediatric Syncope Unit. A cardiac cause of syncope was established in 31 patients, autonomic-mediated reflex syncope in 214, non-syncopal attacks in 15, and in the remaining 15 the cause of syncope remained unexplained. Cardiac syncope was triggered by exercise, whereas vasovagal syncope by prolonged standing, warm-crowded place, and fear or pain emotion. Syncopal spells occurred at various positions in cardiac syncope. Children who had prodromal symptoms with cardiac syncope were significantly fewer than those with vasovagal syncope. Most children with cardiac syncope had history of abnormal electrocardiogram findings when compared with children suffering from vasovagal syncope. On multivariable analysis, history of abnormal electrocardiogram findings and exercise-triggered syncope were independent predictors of cardiac syncope.ConclusionChildren and adolescents with a history of abnormal electrocardiogram findings and exercise-related syncope spells were at high risk for cardiac syncope.

Aim: The appropriate diagnostic protocol for children with syncope has not been well established. A diagnostic protocol was developed and prospectively implemented to improve the diagnostic performance of paediatricians. Methods: The study population included 474 consecutive patients (range 6–17 years) presenting with a syncopal spell in one of the five participating hospitals of China. In step 1, all patients underwent initial evaluation for history, physical examination, standing test and standard electrocardiography (ECG). In step 2, priority was given to cardiographic tests for possibly cardiogenic syncope, or electroencephalographic examination and brain imaging for suspected neurological syncope, or psychiatric tests for suspected psychiatric syncope. Patients with unexplained syncope underwent head-up tilt testing (HUT). Results: The initial evaluation gave a definite diagnosis in 59 (12.4%) and possible diagnosis in 54 of the 474 patients. Further testing gave a definite diagnosis for 326 patients (69.7%). After the entire diagnostic protocol, definite diagnosis was established in 385 patients (81.1%). Autonomic-mediated reflex syncope (AMS) accounted for 73.0% of cases. The average cost of diagnostic results per patient was RMB 1030.24 ± 150.09 ($118.42 ± 17.25). Conclusion: The use of a simplified diagnostic protocol for children and adolescents with syncope improves diagnostic yield.

To assess the exposure status of preconception health risk of Chinese reproductive women and analyze the changes of their exposure status during 2010-2012.The "Core Indicators of Preconception Health Risk Exposure Status in Chinese reproductive couples" were used to evaluate the preconception health risk of women during reproductive period (aged 21-49 years) who participated in National Free Preconception Health Examination Project. From January 1, 2010 to December 31 2012 a total of 2 120 131 women were recruited from 220 pilot counties of 31 provinces. The risks involved the following eight aspects: genetic risk, reproductive risk, chronic disease risk, infectious risk, nutritive risk, behavioral risk, environmental risk and psychosocial risk.The overall rate of preconception health risk in Chinese reproductive women (aged 21-49 years) was 54.63% (1 124 552/2 058 648). Among all age groups, the lowest rate was 53.05% (262 646/495 092) in 21-24 age group while the highest rate 66.94% (7616/11379) in 45-49 age group. The top three risks were nutritive risk [35.74% (726 826/2 033 730)], infectious risk [10.16% (206 983/2 037 022)] and chronic disease risk [6.96% (142 130/2 042 110)]. From 2010 to 2012, the rate of preconception health risk in Chinese reproductive women (aged 21-49) were 55.35% (182 087/328 991) (2010), 56.03% (502 888/897 511) (2011) and 52.82% (439 577/832 146) (2012) respectively. In different years, the rates of nutritive, infectious, behavioral and genetic risks were all highest in 2011. And the rates of eight types of risks were all lowest in 2012. In different age groups, all eight risks were lowest in 21-24 age group and highest in 45-49 age group. And the preconception health risk increased with age.The general status of preconception health of reproductive women is fair. However, there are still some risks. The preconception health risk in Chinese reproductive women showed a declining trend during 2010-2012.

To evaluate the prepregnancy nutritional status and analyze its trend in Chinese women of reproductive age during the period of 2010-2012.Analyses of prepregnancy nutritional indicators were conducted for a total of 2 120 131women of reproductive age (aged 21-49 years) from 220 pilot counties of 31 provinces who participated in National Free Prepregnancy Health Examination Project from January 1, 2010 to December 31, 2012. The nutritional indicators involving body mass index (BMI), hemoglobin (HB) concentration and fasting plasma glucose (FBG) were statistically analyzed with SPSS 17.0 software.There were a sustained increase in prepregnancy HB concentration and declined levels of prepregnancy BMI and FBG during 2010-2012. The clinical data were categorized into 4 subgroups. The data of age composition showed the age group of 25-34 years was the largest proportion of the cohort. The prevalence in prepregnancy low-BMI population subgroup increased from 10.4% in 2010 to 14.14% in 2012. The prepregnancy obesity and overweight subgroups revealed the lowest prevalences of 10.65% and 2.32% respectively in 2012. A decreased prevalence of prepregnancy low FBG subgroup occurred from 5.45% in 2010 to 5.23% in 2012. The prevalences of prepregnancy impaired fasting plasma glucose and diabetes subgroups decreased year-on-year from 3.17% and 1.64% in 2010 to 2.71% and 1.05% in 2012 respectively. The prevalences of prepregnancy mild and severe anemia subgroups decreased from 12.29% and 0.95% to 9.62% and 0.78% respectively during the period 2010-2012. High hemoglobin concentration subgroup also exhibited a decreasing prevalence. Statistical analysis indicated significant differences (P0.001) in the evaluation and trend analysis of prepregnancy nutrition in Chinese women of reproductive age.Chinese women of reproductive age show a fair status of prepregnancy nutrition. And there are decreasing prevalences in obesity, anemia and diabetes.

To assess the exposure status of preconception health risk of Chinese males aged 21-49 years in 31 provinces and analyze the changes of their exposure status during 2010-2012.The "Core Indicators of Preconception Health Risk Exposure Status in Chinese reproductive couples" were used to evaluate the preconception health risk of males aged 21-49 years who participated in National Free Preconception Health Examination Project. From January 1, 2010 to December 31, 2012, a total of 2 030 083 males were recruited from 220 pilot counties of 31 provinces. The risks involved three aspects closely correlated with offspring health: behavioral risk, infectious risk and genetic risk.The overall smoking rate of Chinese rural males aged 21-49 years was 31.19% (622 837/1 997 051). They smoked a daily average of 10 cigarettes. The median number of cigarettes smoked was 10 per day and it increased with age. The overall drinking rate was 31.81% (634 556/1 994 925). Meanwhile, median alcohol consumption increased with age. The rate of males with a history of birth defect, family inbreeding and familial genetic disease accounted for 0.12% (2 344/1 994 324), 0.20% (4 043/1 997 707) and 0.94% (18 736/2 000 005) respectively. The rates of males with a history of hepatitis B and sexually transmitted diseases were 0.67% (13 317/1 988 904) and 0.05% (912/1 988 904) respectively. HBsAg positive rate was 6.32% (124 274/1 965 821). And the positive screening rate of Treponema pallidum was 0.37% (7 169/1 947 021). Among three types of risks, the highest risk was behavioral risk while the lowest risk genetic risk. Behavioral risk, genetic risk and sexually transmitted diseases in infectious risk were all lowest in 2012 while HBV infection in infectious risk was lowest in 2010.The general status of preconception health is fair for rural males aged 21-49 years in 31 provinces. However, there are still some behavior, genetic and infectious risks. The preconception health risk of rural males showed an overall declining trend during 2010-2012.

Coronary artery damage from Kawasaki disease (KD) is closely linked to the dysfunction of the endothelial progenitor cells (EPCs). The aim of the present study was to evaluate the modulatory effect of granulocyte colony-stimulating factor (G-CSF) on EPCs and elastin breakdown of coronary arteries in a KD mouse model.A Lactobacillus casei cell wall extract (LCWE)-induced KD model was established in C57BL/6 mice that were subsequently administrated with recombinant human G-CSF (rhG-CSF). Nω-nitro-L-arginine methyl ester (L-NAME) was administrated for the negative intervention. Evaluations included coronary artery lesions, EPC number and functions, and the plasma concentration of nitric oxide (NO).Elastin breakdown was found in the coronary arteries of model mice 56 days after injection of LCWE. The number of circulating EPCs, plasma concentration of NO, and functions of bone marrow EPCs, including proliferation, adhesion, and migration abilities, were all lower in the KD model group compared with those in the control group. After administration of rhG-CSF, the number of circulating EPCs and plasma concentration of NO were increased significantly compared with those in the KD model group. There were also increases in the functional indexes of EPCs. Furthermore, rhG-CSF administration improved the elastin breakdown effectively. However, these protective effects of rhG-CSF on coronary arteries were attenuated by L-NAME.The present study indicated that the administration of G-CSF prevents elastin breakdown of the coronary arteries by enhancing the number and functions of EPCs via the NO system, and then accelerates the repair of coronary artery lesions in the KD.

To explore the changes of expression and biological activity of nuclear factor-kappa B (NF-κB) and matrix metalloproteinase-9 (MMP-9) after using intravenous immunoglobulin (IVIG) in a murine model of Kawasaki disease (KD) and elucidate the therapeutic mechanism of IVIG for the treatment of KD.A total of 72 mice were categorized randomly into IVIG, KD and control groups.Lactobacillus casei cell wall extract (LCWE) was prepared and injected intraperitoneally into C57BL/6 mice to induce KD (0.5 mg single injection).IVIG group received an intraperitoneal injection of IVIG (2 mg/g) while KD model group had an intraperitoneal injection of normal saline. At Days 14, 28 and 56, the diameter of coronary artery was by echocardiography in 8 mice of each group. At the same time, the stains of hematoxylineosin and elastic fiber were used to observe the pathological damage of coronary artery. Western blot was used to evaluate the expressions of NF-κB and MMP-9, electrophoretic mobility shift assay (EMSA) was used to measure the activity of NF-κB and Gelatin zymography was used to evaluate the activity of MMP-9 in heart samples of murine model of KD.The local inflammatory infiltrate, composed predominantly of mononuclear lymphocytes, of coronary artery trunk and branches was observed at Days 14 and 28 while broken elastin was observed at Day 56. And the inflammatory cell infiltrate was less severe and no apparent broken elastin was observed in IVIG and control groups. On echocardiography, the average value of diameter of left coronary artery in KD model group was higher than that in IVIG and control groups (28 d:(0.48 ± 0.07) vs (0.41 ± 0.03) and (0.35 ± 0.02) mm, all P0.01). Compared with the other two groups, the result of Western blot showed that the expressions of NF-κB and MMP-9 in KD model group were markedly higher than those in IVIG treatment group and that in control group at each time point (28 d: (58 ± 14) vs (25 ± 14)(19 ± 11) µg/L, (100 ± 41) vs (39 ± 19)(35 ± 19) µg/L, all P0.01). The activity of NF-κB by EMSA and the result from KD model group were much higher than those from the control and IVIG groups (28 d: (84 788 ± 2 081) vs (27 220 ± 4 990)(50 192 ± 1 586) µg/L, all P0.01]. And it was in accord with the expression of NF-κB. The outcome of gelatin zymography demonstrated that the activity of MMP-9 had similar change with the expression of MMP-9(18 560 ± 7 963) vs (9 112 ± 3 398)(11 834 ± 4 996) µg/L, all P0.05).NF-κB/MMP-9 is overexpressed and over-activated in the heart of KD mouse models. IVIG may inhibit the inflammatory cell infiltration and alleviate coronary artery. And such a therapeutic effect is possibly achieved by a suppression of the overexpression and over-activation of NF-κB/MMP-9 pathway.

The lightning performance on four 500/220-kV segments of quadruple circuit lines on the same tower of Nanyan line is analyzed by traveling wave method and electric geometry method in this paper. The results indicate that double-circuit 220-kV transmission line protect double-circuit 500-kV transmission line because of different insulation protection, the lightning-understanding level of 220-kV line is corresponding to the usual single lines, and the level of double circuit 500-kV line is higher. Also the back flashover tripout rate of transmission line increases with the increase of the height of tower and the ground resistance. The shielding failure calculation results show that the protect angle of 500-kV transmission line is negative, and the insulation level is high, the shielding failure mainly occur on 220 kV transmission lines. And the shielding failure tripout rate increases with the increase of protect angle of shielding line and the obliquity of ground.

To evaluate the initial application and value of 64-slice multidetector computed tomography as an alternative diagnostic modality in the follow-up of young children with coronary artery aneurysm due to Kawasaki disease. Twelve boys (mean age 5.1 years, range 1.8-7.8 years) for follow-up (time range from 1.1 to 5.1 years) of known Kawasaki disease and coronary artery aneurysm underwent 64-slice MDCT ECG-gated coronary angiography. All data were acquired without breath holding. Two pediatric radiologists independently assessed image quality and the diameter of all coronary segments were measured for each patient. The number, position, shape and size of each coronary artery aneurysm were observed and compared with those of ECHO performed previously. A total of 118/156 segments permitted visualization with diagnostic image quality, the CT measurements showed good inter-observer and intra-observer reliability, coefficients were 0.93 and 0.88, respectively. A total of 30 coronary artery aneurysms were identified with measured mean of 7.5+/-3.8 mm in diameter, and of 12.4+/-9.1 mm in longitudinal lengths.10 tumors were small, 8 tumors were medium and 12 tumors were giant aneurysm. The affected segments included LM7/12(58.3%), 9/12(75%) of LAD1, 4/12(33.3%) of LAD2, 2/12(16.7%) of LCX1; 6/12(50%) of RCA1, 9/12(75%) of RCA2 and 4/12(33.3%) of RCA3, including affected two segments in 9 tumors and three segments in 1 tumor. Calcifications were found in 5 aneurysms and 3/5 with thrombosis; six stenotic segments were found. ECHO failed to detect 8 tumors with 2/8 in LAD, 1/8 in LCX and 5/8 in RCA, and those included 4 small aneurysms. The use of 64-slice MDCT angiography proved valuable for monitoring young children with Kawasaki disease. However, further study is necessary to specify the sensitivity and specificity of MDCT in the follow-up.

According to alternative grounding place of +800kV DC project, this paper selects the theoretically suitable ones to design several different arrangement scenarios of vertical grounding electrodes, and then compares the technical and economic performance when the sub-electrode length remains invariable while other parameters are changed or when the sub-electrode quantity remains invariable while other parameters are changed. The results show that both step voltage and grounding resistance always conform to the system requests in these scenarios, but soil temperature rise near ground rods can not satisfy the request. Simultaneously, a series of conclusions are reached by selecting a best one of these scenarios to compare with level two-cirques grounding electrode. (8 pages)

Objective. The objective of the study is to describe recent epidemiological patterns of Kawasaki disease in China and to make a comparison between China and Japan.Methods. The survey form and diagnostic guideline of Kawasaki disease that had been approved by the Kawasaki Disease Research Committee were sent to all pediatric departments of county hospitals and urban medical centers where pediatric beds existed in Jiangsu (197 hospitals), Shannxi (150 hospitals), Guangdong (156 hospitals) provinces and Beijing City (39 hospitals). A retrospective survey was carried out and all of the hospitalized patients during a 5-years-period had been included into this research.Results. The total number of recorded cases during the 5-years-period was 2127. The incidence rates for per 100,000 children younger than 5 years of age were 1.85 in Jiangsu, 2.34 in Shan'xi, 24.4 in Beijing and 5.93 in Guangdong. The male:female ratio was from 1.70 to 2.23. The peak of age at onset was 1 to 2 years old. The disease occurred more frequently in spring and summer, with the lower incidence in autumn and early winter. Patients who developed cardiac sequalae accounted for 13.6%, 14%, 21.5% and 30% in Jiangsu, Shan'xi, Beijing and Guangdong respectively. Fatality rate was 0.2%