The purpose of this study was to identify the clinical features and laboratory factors that are predictive of intravenous immunoglobulin (IVIG)-resistant Kawasaki disease. Multiple databases were searched for relevant studies on IVIG-resistant Kawasaki disease published from January 2002 to April 2017. Eligible studies were retrieved by manual review of the references. Stata 12 was used for the meta-analysis. Weighted mean differences and odds ratios with 95% confidence intervals were calculated for several indices. Twenty-eight studies involving 26,260 patients comprising 4442 IVIG-resistant Kawasaki disease patients and 21,818 IVIG-sensitive Kawasaki disease patients were included. The meta-analysis showed that the erythrocyte sedimentation rate (ESR) in the IVIG-resistant group was significantly higher than that in the IVIG-sensitive group, and that platelet count and hemoglobin levels were significantly lower in the IVIG-resistant group. The patients with oral mucosa alterations, cervical lymphadenopathy, swelling of the extremities, polymorphous rash, and initial administration of IVIG ≤ 4.0 days after the onset of symptoms were more likely to be IVIG resistant.

Background: Essential hypertension in adults may begin in childhood. The damages to the heart and blood vessels in children with essential hypertension are hidden and difficult to detect. We noninvasively examined changes in cardiovascular structure and function in children with hypertension at early stage using ultrasonography. Methods: All patients with essential hypertension admitted from March 2020 to May 2021 were classified into simple hypertension (group 1, n = 34) and hypertension co-existing with obesity (group 2, n = 11) isolation. Meanwhile 32 healthy children were detected as control heathly group (group 3). We used pulse-wave Doppler to measure carotid–femoral pulse wave velocity (cfPWV), intimal–medial thickness (cIMT) and distensibility of carotid artery (CD). Cardiac structure and function (left atrial diameter [LAD], left ventricular mass [LVM], LVM index [LVMI], relative wall thicknes [RWT], end-diastolic left ventricular internal diameter [LVIDd], diastolic interventricular septum thickness [IVSd], diastolic left ventricular posterior wall thickness [LVPWd], root diameter of aorta [AO], E peak, A peak, E' peak, A' peak, E/E' ratio, and E/A ratio) were measured by echocardiography. Results: The cfPWV of children in group 1 and group 2 were significantly higher than healthy children in group 3. Significant differences were observed in LVM, LVMI, RWT, LVIDd, IVSd, LVPWd, LAD, A peak, E' peak, A' peak, and E/E’ among three groups. Conclusion: Children and adolescents with essential hypertension demonstrate target organ damages in the heart and blood vessels.

Background: Kawasaki disease (KD) often complicates coronary artery lesions (CALs). Despite the established significance of STAT3 signaling during the acute phase of KD and signal transducer and activator of transcription 3 (STAT3) signaling being closely related to CALs, it remains unknown whether and how STAT3 was regulated by ubiquitination during KD pathogenesis. Methods: Bioinformatics and immunoprecipitation assays were conducted, and an E3 ligase, murine double minute 2 (MDM2) was identified as the ubiquitin ligase of STAT3. The blood samples from KD patients before and after intravenous immunoglobulin (IVIG) treatment were utilized to analyze the expression level of MDM2. Human coronary artery endothelial cells (HCAECs) and a mouse model were used to study the mechanisms of MDM2-STAT3 signaling during KD pathogenesis. Results: The MDM2 expression level decreased while the STAT3 level and vascular endothelial growth factor A (VEGFA) level increased in KD patients with CALs and the KD mouse model. Mechanistically, MDM2 colocalized with STAT3 in HCAECs and the coronary vessels of the KD mouse model. Knocking down MDM2 caused an increased level of STAT3 protein in HCAECs, whereas MDM2 overexpression upregulated the ubiquitination level of STAT3 protein, hence leading to significantly decreased turnover of STAT3 and VEGFA. Conclusions: MDM2 functions as a negative regulator of STAT3 signaling by promoting its ubiquitination during KD pathogenesis, thus providing a potential intervention target for KD therapy.

Objective: To characterize the left atrial (LA) strain of pediatric dilated cardiomyopathy (DCM) on two-dimensional speckle tracking echocardiography (2D-STE), and elucidate the progression of LA strain with long-term medical therapy in such patients. Methods: Children with DCM who received standardized medical treatment for > 1 year at Children’s Hospital of Soochow University between January 2018 – January 2021 were retrospectively reviewed. Age- and sex-matched patients with normal echocardiography were included as controls. Routine echocardiography was performed. 2D-STE was applied to obtain LA strain and tissue mitral annular displacement (TMAD) parameters. Echocardiographic images at baseline, 1-year post-treatment (Period 1), and > 2-year post-treatment (Period 2) were analyzed. Results: A total of 33 DCM patients were included, among whom 19 had isolated DCM, while 14 had DCM associated with left ventricular (LV) hypertrabeculation. At baseline, the age range of these children was 1 month to 144 months, and significantly lower left ventricular ejection fraction (LVEF) and fractional shortening (LVFS) ( P < 0.05) were observed among the patients compared to controls. Among the LA strain parameters, significantly lower reservoir strain (LASr) and conduit strain (LAScd) ( P < 0.05) were also demonstrated. And significantly higher LVEF, LVFS, LASr and LAScd and significantly lower IVSd-Z and LVEDd-Z were observed in DCM associated with LV hypertrabeculation patients compared to isolated DCM patients. Between Period 1 and 2, significant increase in LVEF, LVFS, LASr, and LAScd were observed ( P < 0.05). Conclusions: Following long-term standardized medication treatment, children with DCM exhibited significant improvements in left atrial reservoir and conduit functions. LAScd carries the potential as a sensitive imaging modality for LA strain in pediatric DCM.