Background
Multisystem inflammatory syndrome in children (MIS‐C) associated with coronavirus disease 2019 has been increasingly recognized. However, the clinical features of MIS‐C and the differences ...from Kawasaki disease remain unknown. The study aims to investigate the epidemiology and clinical course of MIS‐C.
Methods
PubMed and EMBASE were searched through August 30, 2020. Observational studies describing MIS‐C were included. Data regarding demographic features, clinical symptoms, laboratory, echocardiography and radiology findings, treatments, and outcomes were extracted. Study‐specific estimates were combined using one‐group meta‐analysis in a random‐effects model.
Results
A total of 27 studies were identified including 917 MIS‐C patients. The mean age was 9.3 (95% confidence interval CI, 8.4–10.1). The pooled proportions of Hispanic and Black cases were 34.6% (95% CI, 28.3–40.9) and 31.5% (95% CI, 24.8–38.1), respectively. The common manifestations were gastrointestinal symptoms (87.3%; 95% CI, 82.9–91.6) and cardiovascular involvement such as myocardial dysfunction (55.3%; 95% CI, 42.4–68.2), coronary artery aneurysms (21.7%; 95% CI, 12.8–30.1) and shock (65.8%; 95% CI, 51.1–80.4), with marked elevated inflammatory and cardiac markers. The majority of patients received intravenous immunoglobulin (81.0%; 95% CI, 75.0–86.9), aspirin (67.3%; 95% CI, 48.8–85.7), and corticosteroids (63.6%; 95% CI, 53.4–73.8) with a variety of anti‐inflammatory agents. Although myocardial dysfunction improved in 55.1% (95% CI, 33.4–76.8) at discharge, the rate of extracorporeal membrane oxygenation use was 6.3% (95% CI, 2.8–9.8) and the mortality was 1.9% (95% CI, 1.0–2.8).
Conclusion
Our findings suggest that MIS‐C leads to multiple organ failure, including gastrointestinal manifestations, myocardial dysfunction and coronary abnormalities, and has distinct features from Kawasaki disease.
Abstract Catecholaminergic polymorphic ventricular tachycardia (CPVT) is induced by emotions or exercise in patients without organic heart disease and may be polymorphic or bidirectional in nature. ...The prognosis of CPVT is not good, and therefore prevention of sudden death is of utmost importance. Genetic variants of CPVT include RyR2 , CASQ2 , CALM2 , TRD , and possibly KCNJ2 and ANK2 gene mutations. Hypotheses that suggest the causes of CPVT include weakened binding of FKBP12.6 and RyR2 , a store overload-induced Ca2+ release (SOICR), unzipping of intramolecular domain interactions in RyR2 , and molecular and functional abnormalities caused by mutations in the CASQ2 gene. The incidence of an RyR2 anomaly in CPVTs is about 35–79%, whereas anomalies in the CASQ2 gene account for 3–5% CPVTs. The ping-pong theory, suggesting that reciprocating delayed after depolarization induces bigeminy of the right and left bundle branches, may explain the pathogenesis of bidirectional ventricular tachycardia. Flecainide, carvedilol, left sympathetic nerve denervation, and catheter ablation of the PVC may serve as new therapeutic strategies for CPVT while gene-therapy may be applied to some types of CPVT in the future. Although, not all sudden cardiac deaths in CPVT patients are currently preventable, new medical and interventional therapies may improve CPVT prognosis.
Abstract
The characteristic shape of the T-wave in congenital long-QT syndrome type 3 (LQTS3) is considered a late-onset T-wave. We analyzed the difference in the shapes of the T-waves of V5 in the ...electrocardiograms (ECGs) of LQTS3 cases and normal subjects using generalized Procrustes analysis. The J and Q points of LQTS3 cases are shifted to the upper left compared to those of normal subjects. SdFmax is the point on the ECG where the second derivative is first maximized. SdFmax is the point where the change in the slope of the ascending limb of the T-wave is maximized. SdFmax in LQTS3 cases is shifted to the lower right compared to normal subjects. The interval from J to SdFmax in LQTS3 cases is expanded compared with that of normal subjects. From principal component analysis of the Procrustes mean shape of the T-wave landmarks, the second principal component shows a shift of SdFmax to the lower right. These results can quantitatively explain why the T-wave of LQTS3 cases looks like a late-onset T-wave. After being fitted to a multivariate logistic regression model, LQTS3 cases and normal subjects can be distinguished by the second independent component.
To evaluate the safety and effectiveness of tocilizumab (TCZ) in patients with systemic juvenile idiopathic arthritis (sJIA) in real-world clinical settings in Japan.
Paediatric patients with sJIA ...initiating TCZ between April 2008 and February 2012 and those previously enrolled in clinical trials who initiated TCZ before April 2008 were enrolled in a Japanese registry surveillance programme. Safety and effectiveness parameters were collected for 52 weeks.
Of 417 patients enrolled, mean age was 11.2 years and 48.0% were female. TCZ exposure was 407.0 patient-years (PYs). Baseline corticosteroid use was higher than in clinical trials. Rates of total adverse events (AEs) and serious AEs (SAEs) were 224.3/100 PYs and 54.5/100 PYs, respectively, with SAEs higher than previously reported. The most frequent AEs and SAEs were infections and infestations (69.8/100 PYs and 18.2/100 PYs, respectively). 74 serious infections occurred in 55 patients (18.2/100 PYs); higher than previously reported. 26 macrophage activation syndrome events were reported in 24 patients (6.4/100 PYs). Fever and rash symptoms improved from baseline to week 52 (54.6% to 5.6% and 43.0% to 5.6%, respectively). At 4 weeks, 8 weeks and 52 weeks, 90.5%, 96.2% and 99.0% of patients achieved normal C reactive protein levels (<0.3 mg/dL), respectively.
These first real-world data demonstrated that TCZ was well tolerated, with acceptable safety and effectiveness in patients with sJIA. Higher incidences of SAEs and serious infections may be due to differences, such as corticosteroid use and concomitant diseases, between patient populations enrolled in previously reported clinical trials and this study.
Background:Reference values and the characteristics of the electrocardiographic (ECG) findings using a large number of subjects are lacking for children and adolescents.Methods and Results:A total of ...56,753 digitally stored ECGs of participants in a school-based ECG screening system were obtained between 2006 and 2009 in Kagoshima, Japan. Each ECG was manually reviewed by 2 pediatric cardiologists and only ECGs with sinus rhythm were included. A final total of 48,401 ECGs from 16,773 1st (6 years old, 50% girls), 18,126 7th (12 years old, 51% girls), and 13,502 10th graders (15 years old, 52% girls) were selected. ECG variables showed differences in age and sex. However, the effects of age and sex on ECG variables such as the PQ interval, QRS voltage, and STJ segment were also different. The 98th percentile values of well-known surrogate parameters for ventricular hypertrophy in the present study were much higher than the conventional criteria.Conclusions:The present study of a large number of pediatric subjects showed that the effects of age and sex on ECG parameters are different, and that criteria for ventricular hypertrophy should be newly determined by age and sex. We have developed reference data for STJ segment elevation for children and adolescents. These findings are useful for creating guidelines and recommendations for interpretation of pediatric ECG.
There have been no reports regarding the influence of a change in the pulmonary artery index before or after a Fontan operation. Moreover, the factors that might affect the change in the pulmonary ...artery index are unclear. This was a retrospective study, which included 94 patients who underwent a Fontan operation. We divided the patients into two groups according to the change in the pulmonary artery index before and after the Fontan operation: the increased pulmonary artery index group (
n
= 51) and decreased pulmonary artery index group (
n
= 43). The pulmonary artery index after the Fontan operation was significantly higher in the increased pulmonary artery index group than decreased pulmonary artery index group (
p
< 0.0001). The central venous pressure (
p
< 0.0001) and pulmonary vascular resistance (
p
= 0.007) were significantly lower in the increased pulmonary artery index group. The mixed venous oxygen saturation was significantly higher in the increased pulmonary artery index group (
p
< 0.0001). The pulmonary artery index was more increased in the patients who were administered a phosphodiesterase type 5 inhibitor than in those who were not (
p
= 0.03). The change in the pulmonary artery index reflected the hemodynamics after a Fontan operation during the short term. In that respect, phosphodiesterase type 5 inhibitors might be involved in the process.
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