Background/Aims: Increasing evidence indicates that microRNAs (miRNAs) play important roles in Kawasaki disease (KD). Our previous study demonstrated that hsa-miR-27b-3p (miR-27b) was up-regulated in KD serum. However, the specific role of miR-27b in KD remains unclear. We aimed to investigate that miR-27b could be a biomarker and therapeutic target for KD treatment. As well, the specific mechanism of miR-27b effecting endothelial cell functions was studied. Methods: The expression of miR-27b and Smad7 was measured by qRT-PCR. Gain-of-function strategy was used to observe the effect of miR-27b on human umbilical vein endothelial cells (HUVECs) proliferation and migration. Bioinformatics analyses were applied to predict miR-27b targets and then we verified Smad7 by a luciferase reporter assay. Western blot was performed to detect the protein expression of Smad7, PCNA, MMP9, MMP12 and TGF-β-related genes. Results: We confirmed that miR-27b was shown to be dramatically up-regulated in KD serum and KD serum-treated HUVECs and that elevated expression of miR-27b suppressed the proliferation and migration of HUVECs. Furthermore, our results verified that miR-27b mediated cell functions by affecting the TGF-β via targeting Smad7 in HUVECs. Conclusion: These results suggested that up-regulated miR-27b had a protective role in HUVECs proliferation and migration via targeting Smad7 and affecting TGF-β pathway. Therefore, miR-27b represented a potential biomarker for KD and may serve as a promising therapeutic target for KD treatment.

Delayed IVIG treatment was an independent risk factor for the development of CALs. Children in the delayed IVIG treatment group with higher levels of CRP and ESR (CRP >79 mg/L, ESR >34 mm/h) had the greatest likelihood of developing CALs.

ObjectiveThis study was performed to explore the clinical features of Kawasaki disease shock syndrome and analyse the association between the left ventricular ejection fraction and Kawasaki disease shock syndrome.MethodsWe retrospectively reviewed the medical records of all consecutive inpatients with Kawasaki disease at Wenzhou Medical University Second Affiliated Hospital and Yuying Children’s Hospital in Wenzhou, China from January 2009 to December 2016. We compared the clinical characteristics, laboratory data, and left ventricular ejection fraction between patients with and without Kawasaki disease shock syndrome and analysed the effect of the left ventricular ejection fraction on Kawasaki disease shock syndrome under different clinical conditions of Kawasaki disease.ResultsIn total, 1147 patients were diagnosed with Kawasaki disease. Of these 1147 patients, 17 were diagnosed with Kawasaki disease shock syndrome; 68 patients admitted to the hospital at the same time, ±2 weeks, with Kawasaki disease but without Kawasaki disease shock syndrome served as the control group. Compared with the control group, the Kawasaki disease shock syndrome group had a significantly higher incidence of coronary artery lesions, cardiac troponin I concentration, N-terminal prohormone of brain natriuretic peptide concentration, neutrophil count and ratio, alanine aminotransferase concentration, aspartate aminotransferase concentration, and C-reactive protein concentration and a significantly lower platelet count, serum albumin concentration, and left ventricular ejection fraction. A low left ventricular ejection fraction was associated with Kawasaki disease shock syndrome under different conditions of Kawasaki disease.ConclusionAmong patients with Kawasaki disease, cardiac injury is more likely in those with Kawasaki disease shock syndrome than without, and a low left ventricular ejection fraction may be associated with the development of Kawasaki disease shock syndrome.

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Background: Many children with Kawasaki disease develop coronary artery lesions before intravenous immunoglobulin treatment. However, little data are available on the prognosis of children with Kawasaki disease who developed coronary artery lesions before intravenous immunoglobulin treatment. Aims: To explore the outcomes of coronary artery lesions before intravenous immunoglobulin treatment in children with Kawasaki disease and analyze the factors that influence the duration of coronary artery lesions. Study Design: Retrospective cohort study. Methods: All patients with Kawasaki disease who developed coronary artery lesions before intravenous immunoglobulin treatment in our hospital from January 2009 to December 2014 were reviewed. A Cox proportional hazards model was used to determine the factors influencing the prognosis of coronary artery lesions. Results: Among 182 patients included, 28.6% were male, 83.50% were younger than 36 months, and 181 exhibited resolution of coronary artery lesions 2 years after disease onset. The median duration of coronary artery lesions was 31 days, and the proportion of coronary artery lesions was 52% at 1 month, 35% at 2 months, 33% at 3 months, 25% at 6 months, 14% at 1 year, and 0.5% at 2 years. The univariate analysis showed that overweight status, higher platelet count, lower albumin level, and starting treatment more than 10 days after disease onset were factors that possibly affect the duration of coronary artery lesions in children. The multivariate Cox regression analysis showed that female sex (adjusted hazard ratio, 1.661; 95% confidence interval, 1.117-2.470) was an independent protective factor, and overweight status (adjusted hazard ratio, 0.469; 95% confidence interval, 0.298-0.737), higher platelet count (adjusted hazard ratio, 0.649; 95% confidence interval, 0.443-0.950), and starting treatment more than 10 days after disease onset (adjusted hazard ratio, 0.392; 95% confidence interval, 0.215-0.716) were independent risk factors for a longer duration of coronary artery lesions. Conclusion: The average duration of coronary artery lesions before intravenous immunoglobulin therapy in children with Kawasaki disease is approximately 1 month. Male gender, overweight status, higher platelet count, and initiation of treatment more than 10 days after the onset of the disease are independent risk factors for longer-lasting coronary artery lesions.

BackgroundTo evaluate differences in laboratory parameters, clinical presentation, and incidence of coronary artery lesions (CAL) between children with neutropenic and non-neutropenic Kawasaki disease (KD).MethodsAll consecutive KD patients that presented to the Second Affiliated Hospital and Yuying Children’s Hospital of Wenzhou Medical University in Wenzhou, China between January 2005 and December 2015 were included in this study. Patients were divided into two groups (KD with neutropenia (NKD) and KD without neutropenia (NNKD)) based on whether or not they developed neutropenia during the course of treatment. We compared differences in clinical manifestations, laboratory parameters, and treatment protocols between groups. We also evaluated the relationship between neutropenia with immunoglobulin dosage and incidence of CAL.ResultsAn IVIG treatment regimen of 2 g/kg*1d was associated with a lower incidence of neutropenia compared to the 1 g/kg*2d protocol. The incidence of CAL was higher in KD patients with neutropenia than in those without. Subgroup analysis showed no difference in the incidence of CAL among the different age groups between KD patients with and without neutropenia.ConclusionsFollow up ultrasonic echocardiography should be performed in KD patients with neutropenia in order to allow for early detection of CAL and timely intervention.

SummaryUp to 40% of healthy children have premature ventricular complexes or contractions (PVCs) detected with 24-hour Holter monitoring. We aimed to investigate the morphological characteristics and origins of idiopathic PVCs under a 12-lead electrocardiogram in children with structurally normal hearts. All asymptomatic monomorphic PVC patients with structurally normal hearts under 18 years of age were included in this retrospective study. Characteristics of PVCs in lead V1 under a 12-lead electrocardiogram were classified as left bundle branch block (PVC-LBBB) or right bundle branch block (PVC-RBBB). According to limb leads, PVC-LBBB or PVC-RBBB was divided into: PVCs-LBBB type I; PVCs-LBBB type II; PVCs-RBBB type I; PVCs-RBBB type II; and PVCs-RBBB type III. Out of 178 PVC patients, 94 cases of PVCs-LBBB (PVCs-LBBB type I = 60; PVCs-LBBB type II = 34) and 84 cases of PVCs-RBBB (PVCs-RBBB type I = 3; PVCs-RBBB type II = 55; PVCs-RBBB type III = 26) were identified. The frequency of PVCs-LBBB type I increased with age and the frequency of PVCs-RBBB type II and III decreased with age. Among the children monitor tested, from 1 years old to 18 years old, PVCs originating from the left or right ventricular outflow tract gradually increased with age, while PVCs originating from the branch sources decreased with age.(Int Heart J 2017; 58: 714-719)

The aim of the present study was to identify key genes that may be involved in the pathogenesis of Tetralogy of Fallot (TOF) using bioinformatics methods. The GSE26125 microarray dataset, which includes cardiovascular tissue samples derived from 16 children with TOF and five healthy age-matched control infants, was downloaded from the Gene Expression Omnibus database. Differential expression analysis was performed between TOF and control samples to identify differentially expressed genes (DEGs) using Student's t-test, and the R/limma package, with a log2 fold-change of >2 and a false discovery rate of <0.01 set as thresholds. The biological functions of DEGs were analyzed using the ToppGene database. The ReactomeFIViz application was used to construct functional interaction (FI) networks, and the genes in each module were subjected to pathway enrichment analysis. The iRegulon plugin was used to identify transcription factors predicted to regulate the DEGs in the FI network, and the gene-transcription factor pairs were then visualized using Cytoscape software. A total of 878 DEGs were identified, including 848 upregulated genes and 30 downregulated genes. The gene FI network contained seven function modules, which were all comprised of upregulated genes. Genes enriched in Module 1 were enriched in the following three neurological disorder-associated signaling pathways: Parkinson's disease, Alzheimer's disease and Huntington's disease. Genes in Modules 0, 3 and 5 were dominantly enriched in pathways associated with ribosomes and protein translation. The Xbox binding protein 1 transcription factor was demonstrated to be involved in the regulation of genes encoding the subunits of cytoplasmic and mitochondrial ribosomes, as well as genes involved in neurodegenerative disorders. Therefore, dysfunction of genes involved in signaling pathways associated with neurodegenerative disorders, ribosome function and protein translation may contribute to the pathogenesis of TOF.