Kawasaki Disease Research Articles

Comparison of six Z-score formulas based on echocardiography for coronary artery lesions in Kawasaki disease

GoalKawasaki disease (KD) patients are at risk of developing the serious complication of coronary artery dilation (CAD). To diagnose CAD caused by KD, various Z-Score formulas are used worldwide. This paper aims to evaluate the differences and inclusiveness among the six most commonly used Z-Score formulas in diagnosing CAD in Suzhou, China. Additionally, the study seeks to compare the differences in CAD diagnosis among different high-risk factor groups. By doing so, this research provides a valuable reference for accurately diagnosing CAD in KD patients. MethodThis paper presents a retrospective analysis of 1509 patients diagnosed with KD at the Children's Hospital of Soochow University between January 2018 and December 2020. We collected the patients' clinical and echocardiographic data and used six Z-Score formulas (Kobayashi et al., de Zorzi et al., Kurotobi et al., McCrindle et al., Olivieri et al., and Dallaire et al.) to diagnose the degree of CAD in different segments. We then compared the diagnostic differences and inclusiveness of these formulas, especially the diagnostic differences in medium to giant CAA. To achieve this, we divided the patients into groups based on their age (≤12 months, 13–30 months, and > 30 months) and fever duration (≤5 days, 6–7 days, 8–9 days, and ≥ 10 days). Using the McNemar test and the Kappa test, we compared the differences and the consistencies of CDA diagnosis among the six Z-Score formulas. Moreover, we used the Friedman test and Chi-square segmentation formula to compare the differences in age and number of fever duration between groups and to compare each Z-Score formula pair within the group. ResultsExcept for the LMCA segment, where there were no statistically significant differences between de Zorzi formula and McCrindle formula, the Z-score formulas showed statistically significant differences in the degree of CAD diagnosis across all other segments. Inclusiveness assessment revealed that Kobayashi formula and Dallaire formula showed significantly higher rates of dilatation (6.58% and 5.32%), or of small aneurysms (6.52% and 4.52%) compared to other formulas (1.0%–1.73%). Medium aneurysms were also more likely to be identified with Kobayashi and Dallaire formulas (0.8% and 0.8%) compared to the remaining formulas (0.13–0.40%). There are significant differences in the diagnoses of medium to giant CAA made by these six formulas in LAD and RCA. The longer the duration of fever and the younger the age, the higher the diagnosis rates of CAD and CAA. There were no statistically significant differences between de Zorzi formula and McCrindle formula, de Zorzi formula and Oliveri formula, and Kurotobi formula and Dallaire formula within the four groups based on the duration of fever. Similarly, there were no statistically significant differences between Kobayashi formula and Dallaire formula, and between de Zorzi formula and Oliveri formula in the age groups of ≤12 months and 13–30 months. ConclusionThere are diagnostic differences among these six Z-score formulas, considering the aforementioned statistics. Kobayashi formula and Dallaire formula are more inclusive, and less likely to under-diagnose significant CAD. They perform evenly for dilatation only, for small aneurysms and the median size aneurysms, and that is for segments of LMCA, LAD and RCA. In addition, McCrindle formula joins the “inclusive” pack for LAD and RCA in the matter of CAD. The younger the age of the patients and the longer the duration of fever, the higher the diagnosis rates of CAD and CAA. Furthermore, the younger the age of the patients and the shorter the duration of fever, the greater the differences between the various formulas.

Semaphorin 7A promotes endothelial permeability and inflammation via plexin C1 and integrin β1 in Kawasaki disease

BackgroundKawasaki disease (KD) is a pediatric systemic vasculitis characterized by endothelial cell dysfunction. Semaphorin 7A (Sema7A) has been reported to regulate endothelial phenotypes associated with cardiovascular diseases, while its role in KD remains unknown. This study aims to investigate the effect of Sema7A on endothelial permeability and inflammatory response in KD conditions.MethodsBlood samples were collected from 68 KD patients and 25 healthy children (HC). The levels of Sema7A and A Disintegrin and Metalloprotease 17 (ADAM17) in serum were measured by enzyme-linked immunosorbent assay (ELISA), and Sema7A expression in blood cells was analyzed by flow cytometry. Ex vivo monocytes were used for Sema7A shedding assays. In vitro human coronary artery endothelial cells (HCAECs) were cultured in KD sera and stimulated with Sema7A, and TNF-α, IL-1β, IL-6, and IL-18 of HCAECs were measured by ELISA and qRT-PCR. HCAECs monolayer permeability was measured by FITC-dextran.ResultsThe serum level of Sema7A was significantly higher in KD patients than in HC and correlated with disease severity. Monocytes were identified as one of the source of elevated serum Sema7A, which implicates a process of ADAM17-dependent shedding. Sera from KD patients induced upregulation of plexin C1 and integrin β1 in HCAECs compared to sera from HC. Sema7A mediated the proinflammatory cytokine production of HCAECs in an integrin β1-dependent manner, while both plexin C1 and integrin β1 contributed to Sema7A-induced HCAEC hyperpermeability.ConclusionsSema7A is involved in the progression of KD vasculitis by promoting endothelial permeability and inflammation through a plexin C1 and integrin β1-dependent pathway. Sema7A may serve as a potential biomarker and therapeutic target in the prognosis and treatment of KD.

Heterogeneity of macrophage activation syndrome and treatment progression.

Macrophage activation syndrome (MAS) is a rare complication of autoimmune inflammatory rheumatic diseases (AIIRD) characterized by a progressive and life-threatening condition with features including cytokine storm and hemophagocytosis. Predisposing factors are typically associated with microbial infections, genetic factors (distinct from typical genetically related hemophagocytic lymphohistiocytosis (HLH)), and inappropriate immune system overactivation. Clinical features include unremitting fever, generalized rash, hepatosplenomegaly, lymphadenopathy, anemia, worsening liver function, and neurological involvement. MAS can occur in various AIIRDs, including but not limited to systemic juvenile idiopathic arthritis (sJIA), adult-onset Still's disease (AOSD), systemic lupus erythematosus (SLE), Kawasaki disease (KD), juvenile dermatomyositis (JDM), rheumatoid arthritis (RA), and Sjögren's syndrome (SS), etc. Although progress has been made in understanding the pathogenesis and treatment of MAS, it is important to recognize the differences between different diseases and the various treatment options available. This article summarizes the cell types and cytokines involved in MAS-related diseases, the heterogeneity, and treatment options, while also comparing it to genetically related HLH.

Human Adipose Tissue-Derived Stem Cells Inhibit Coronary Artery Vasculitis in a Mouse Model of Kawasaki Disease.

Adipose tissue-derived mesenchymal stem cells (ADSCs) are used for the treatment of various diseases because of their rapid proliferation and high anti-inflammatory and tissue repair properties. Kawasaki disease is a systemic vasculitis with coronary arteritis and aneurysms occurring in pediatric patients. In this study, we examined serologically and pathologically whether the administration of human ADSCs (hADSCs) to a mouse model of Kawasaki disease could suppress vasculitis. Candida albicans water-soluble fractions were intraperitoneally injected into DBA/2 mice for 5 consecutive days to generate a mouse model of Kawasaki disease. The model mice were intravenously administered hADSCs or phosphate-buffered saline (PBS). Serum samples collected on days 15 and 29 were used to compare cytokine levels. Mouse hearts dissected on day 29 were subjected to hematoxylin and eosin and immunohistological staining using Galectin-1 (Gal-1), a protein involved in cardiovascular homeostasis, and CD44, a cell-surface marker of hADSCs. Comparison of inflammation-related cytokines showed a significant decrease in IL-1α expression at day 15 (P<0.05) and IL-6 expression at day 29 (P<0.01) in the hADSCs-treated group compared to the PBS group. Evaluation by hematoxylin and eosin staining showed decreased inflammatory cell infiltration and a tendency towards increased Gal-1 expression in the hADSCs group. CD44 expression was not observed in both the groups. The survival curve showed that the hADSCs group had a significantly longer survival time (P<0.05). The present experimental results indicate that hADSCs have an early anti-inflammatory effect, and that Gal-1 may be involved in preventing inflammation and reducing tissue damage.

Features of diagnosis of incomplete Kawasaki disease in young children. A clinical case of Kawasaki disease with the formation of giant coronary artery aneurysms

Kawasaki disease (KD) is a mucocutaneous lymphonodular syndrome, a vasculitis of unknown etiology that mainly affects children under the age of 5 years. Today, KD is the most common cause of acquired heart disease in children in developed countries. Given the absence of pathognomonic diagnosis of the disease, the diagnosis is based solely on the identification of clinical signs and the exclusion of other clinically similar diseases. Timely therapy with intravenous immunoglobulin has significantly reduced the incidence of complications, such as coronary artery aneurysms, from 25 to ≈ 4 %. The long-term prognosis of the disease depends on the initial and current level of coronary artery damage. Patients with coronary artery aneurysms remain at high risk of developing myocardial ischaemia due to thrombosis and stenosis of the coronary arteries, which can occur in case of untimely treatment for KD. Therefore, the life of these patients depends on lifelong thromboprophylaxis and timely diagnosis of stenosis. The article presents a clinical case of incomplete Kawasaki disease, which was complica­ted by the development of giant coronary artery aneurysms.

Characteristics of the course of multisystem inflammatory syndrome associated with COVID-19 in children according to the observations of the Kyiv City Children’s Clinical Hospital 2

Background. In the context of coronavirus diseases 2019 (COVID-19) pandemic associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), a significant number of cases resembling Kawasaki disease in children have been reported worldwide and eventually termed “multisystem inflammatory syndrome in children (MIS-C) associated with SARS-CoV-2”. Objective: to compare the clinical presentation, laboratory fin­dings, and instrumental examination data in children with Kawasaki-like MIS-C phenotype and MIS-C with a shock phenotype with cases, which met the US Centers for Disease Control and Prevention criteria. Materials and methods. The article presents the results of a retrospective analysis of the disease histories in 20 children aged 2.5 to 16 years with a Kawasaki-like MIS-C phenotype and with MIS-C with a shock phenotype associated with SARS-CoV-2 infection who were hospitalized in the MNPE “Kyiv City Children’s Clinical Hospital 2” in 2002–2021. Results. Patients were divided into 2 groups according to symptoms and pathological conditions. Group I included children with clinical signs similar to the Kawasaki disease (n = 8). They were classified as those with Kawasaki-like MIS-C phenotype. Complications such as coronary artery dilatations and aneurysms occurred only in this group. Group II consisted of patients (n = 12) who were classified as those with MIS-C with a shock phenotype. Children in this group had a higher number of involved organ systems, were more likely to have shock, pleuritis, peritonitis, and a higher prevalence of abdominal pain. Furthermore, they exhibited elevated levels of leukocytes and neutrophils (p = 0.043; p = 0.047), along with a higher neutrophil-to-lymphocyte ratio (p = 0.05), compared to the patients with Kawasaki-like phenotype. Conclusions. Our results suggest that multisystem inflammatory syndrome associated with SARS-CoV-2 in children is characterized by a wide range of clinical, laboratory and instrumental signs. Moreover, our findings highlight that children with features that correspond to MIS-C with a shock phenotype tend to have a more severe course of the disease and a higher rate of complications.

Coronary artery lesions in children with Kawasaki disease: status quo and nursing care.

Coronary artery lesion (CAL) is a common yet serious complication in children with Kawasaki disease. The aim of the present study was to evaluate the influencing factors of CAL in children with Kawasaki disease, to provide reference for the clinical treatment and care of children with Kawasaki disease. A retrospective cohort study. Children with Kawasaki disease treated in a tertiary hospital in China between 1 January 2021 and 31 December 2022 were selected. The characteristics and clinical data of children with Kawasaki disease were analyzed. Spearman's correlation analysis was conducted to evaluate the relationship between CAL and the characteristics of children with Kawasaki disease. A logistic regression analysis was used to analyze the influencing factors of CAL in children with Kawasaki disease. In total, 185 children with Kawasaki disease were included; the incidence of CAL in children with Kawasaki disease was 18.38%. Pearson's correlation analysis showed that gender (r = 0.504), age (r = 0.611), duration of fever ≥10 days (r = 0.579), hemoglobin (Hb) (r = 0.623), and C-reactive protein (CRP) (r = 0.558) were all correlated with the CAL in children with Kawasaki disease (all p < 0.05). Logistic regression analyses showed that male [odds ratio (OR) = 2.543, 95% confidence interval (CI): 1.801-3.077, p = 0.040], age ≤2 years (OR = 3.002, 95% CI: 2.744-3.641, p = 0.012), duration of fever ≥10 days (OR = 2.089, 95% CI: 1.624-2.515, p = 0.028), Hb ≤105 g/L (OR = 1.914, 95% CI: 1.431-2.406, p = 0.013), and CRP ≥100 mg/L (OR = 2.168, 95% CI: 1.893-2.531, p = 0.035) were the risk factors of CAL in children with Kawasaki disease (all p < 0.05). The incidence of CAL in children with Kawasaki disease is high and there are many related risk factors. Clinical medical workers should take early warning and carry out interventions and nursing care according to these risk factors to improve the prognosis of children with Kawasaki disease.

Incidence and seasonality of Kawasaki disease in children in the Philippines, and its association with ambient air temperature.

Despite an unknown cause, Kawasaki disease (KD) is currently the primary leading cause of acquired heart disease in developed countries in children and has been increasing in recent years. Research efforts have explored environmental factors related to KD, but they are still unclear especially in the tropics. We aimed to describe the incidence of KD in children, assess its seasonality, and determine its association with ambient air temperature in the National Capital Region (NCR), Philippines from January 2009 to December 2019. Monthly number of KD cases from the Philippine Pediatric Society (PPS) disease registry was collected to determine the incidence of KD. A generalized linear model (GLM) with quasi-Poisson regression was utilized to assess the seasonality of KD and determine its association with ambient air temperature after adjusting for the relevant confounders. The majority of KD cases (68.52%) occurred in children less than five years old, with incidence rates ranging from 14.98 to 23.20 cases per 100,000 population, and a male-to-female ratio of 1.43:1. Seasonal variation followed a unimodal shape with a rate ratio of 1.13 from the average, peaking in March and reaching the lowest in September. After adjusting for seasonality and long-term trend, every one-degree Celsius increase in the monthly mean temperature significantly increased the risk of developing KD by 8.28% (95% CI: 2.12%, 14.80%). Season-specific analysis revealed a positive association during the dry season (RR: 1.06, 95% CI: 1.01, 1.11), whereas no evidence of association was found during the wet season (RR: 1.10, 95% CI: 0.95, 1.27). We have presented the incidence of KD in the Philippines which is relatively varied from its neighboring countries. The unimodal seasonality of KD and its linear association with temperature, independent of season and secular trend, especially during dry season, may provide insights into its etiology and may support enhanced KD detection efforts in the country.

Kawasaki disease versus Multisystem Inflammatory Syndrome COVID-19 with Kawasaki disease phenotype. Single-center experience

to compare the clinical presentation, laboratory testing, and evolution of KD with PIMS Kawasaki phenotype (PIMS-KD) in patients hospitalized before the pandemic, compared with the pandemic period. Cross-sectional study in two groups of patients at the Hospital Exequiel González Cortés: typical KD (group 1) and PIMS-KD (group 2). Data on demographic, clinical, and biochemical details were collected, as well as echocardiogram, treatment, and evolution records. IgG and IgM serology for SARS-CoV-2 was performed in both groups. In the KD group and the PIMS-KD group, 20 and 33 patients were analyzed, respectively. There were differences in age, days of fever, count of leukocytes, lymphocytes, and platelets, erythrocyte sedimentation rate (ESR), and hospital stay. In 25% of the KD group, there were alterations in the echocardiogram and, in the PIMS-K group, all patients received corticosteroids and 25 patients received intravenous immunoglobulin (IVIG). In both groups, a favorable clinical evolution was observed, characterized by the absence of complications and mortality. Based on the data obtained in our study, the importance of the epidemiological link is emphasized as an essential factor in differentiating between both pathologies, highlighting the need to consider factors such as age, duration of fever, count of leukocytes, lymphocytes, and platelets, and degree of cardiac involvement, for a differential evaluation between patients with PIMS-KD versus KD.

Multi-Modality Imaging in Vasculitis.

Systemic vasculitides are a rare and complex group of diseases that can affect multiple organ systems. Clinically, presentation may be vague and non-specific and as such, diagnosis and subsequent management are challenging. These entities are typically classified by the size of vessel involved, including large-vessel vasculitis (giant cell arteritis, Takayasu's arteritis, and clinically isolated aortitis), medium-vessel vasculitis (including polyarteritis nodosa and Kawasaki disease), and small-vessel vasculitis (granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis). There are also other systemic vasculitides that do not fit in to these categories, such as Behcet's disease, Cogan syndrome, and IgG4-related disease. Advances in medical imaging modalities have revolutionized the approach to diagnosis of these diseases. Specifically, color Doppler ultrasound, computed tomography and angiography, magnetic resonance imaging, positron emission tomography, or invasive catheterization as indicated have become fundamental in the work up of any patient with suspected systemic or localized vasculitis. This review presents the key diagnostic imaging modalities and their clinical utility in the evaluation of systemic vasculitis.

Assessment of Gastrointestinal Manifestations and Predictive Factors Associated with Their Occurrence in Kawasaki Disease Patients: A Cross-Sectional Study

Background: Kawasaki disease is an idiopathic febrile systemic vasculitis, recognized as the leading cause of acquired heart disease in pediatric patients. Gastrointestinal manifestations can lead to delayed treatment, underscoring the importance of identifying and predicting factors associated with these manifestations to enable timely intervention. Objectives: The primary objective of this study was to identify the aforementioned factors. Methods: This retrospective study collected demographic information, medical history, physical examination findings, and laboratory results of 359 children suspected of having Kawasaki disease and referred to Mofid Children’s Hospital between 2013 and 2022. The study assessed the frequency of gastrointestinal manifestations and their association with physical examination findings and laboratory data. Results: A total of 359 patients were included in the study, comprising 153 males and 206 females. The mean age of participants was 2.8 ± 2.6 years. Abdominal pain was the most common clinical manifestation, reported in 39% of patients. Elevated levels of aspartate aminotransferase (AST) were observed in 38.1% of patients, while 30.2% showed elevated alanine aminotransferase (ALT) levels, and 10.5% exhibited direct hyperbilirubinemia. Patients with elevated ALT and AST levels had a higher incidence of abdominal pain, nausea/vomiting, and anorexia. Moreover, patients with positive findings of red blood cell (RBC) and white blood cell (WBC) in their stool examination had a higher incidence of fever and abdominal pain. Conclusions: Based on our statistical analysis, elevated AST and ALT levels, hyperbilirubinemia, and positive stool findings of RBC and WBC appear to be predictive factors for gastrointestinal manifestations in Kawasaki disease.

Environmental Exposures and Pediatric Cardiology: A Scientific Statement From the American Heart Association.

Environmental toxicants and pollutants are causes of adverse health consequences, including well-established associations between environmental exposures and cardiovascular diseases. Environmental degradation is widely prevalent and has a long latency period between exposure and health outcome, potentially placing a large number of individuals at risk of these health consequences. Emerging evidence suggests that environmental exposures in early life may be key risk factors for cardiovascular conditions across the life span. Children are a particularly sensitive population for the detrimental effects of environmental toxicants and pollutants given the long-term cumulative effects of early-life exposures on health outcomes, including congenital heart disease, acquired cardiac diseases, and accumulation of cardiovascular disease risk factors. This scientific statement highlights representative examples for each of these cardiovascular disease subtypes and their determinants, focusing specifically on the associations between climate change and congenital heart disease, airborne particulate matter and Kawasaki disease, blood lead levels and blood pressure, and endocrine-disrupting chemicals with cardiometabolic risk factors. Because children are particularly dependent on their caregivers to address their health concerns, this scientific statement highlights the need for clinicians, research scientists, and policymakers to focus more on the linkages of environmental exposures with cardiovascular conditions in children and adolescents.

Kawasaki disease in Kenya and review of the African literature

BackgroundKawasaki disease has been described across the globe, although publications from Africa are limited. To our knowledge, there are no publications on Kawasaki disease from Kenya, which triggered this report.MethodsA retrospective cross-sectional study was undertaken to identify in-patients with a discharge diagnosis of Kawasaki disease, over 2 different 5-year periods, at two pediatric hospitals in Nairobi, Kenya. We reviewed the medical records of all patients and report their clinical findings, diagnostic workup and treatment. In addition, we undertook a detailed review of the literature.ResultsTwenty-three patients with Kawasaki disease were identified, of those 12 (52.2%) had incomplete disease. The mean age was 2.3 years (SD+/-2.2) (range 0.3–10.3) with a male to female ratio of 1:1. The mean duration of fever at diagnosis was 8.3 days (SD+/-4.7) (range 2–20). Oral changes were the most common clinical feature and conjunctivitis the least common. Thrombocytosis at diagnosis was seen in 52% (12/23). Twenty-one patients (91.3%) were treated with intravenous immunoglobulin and all except 1 received aspirin. Baseline echocardiograms were performed in 95.7% (22/23) and found to be abnormal in 3 (13.6%). Follow-up data was limited. Our literature review identified 79 publications with documented cases of Kawasaki disease in children from 22 countries across the African continent with a total of 1115 patients including those from this report. Only 153 reported cases, or 13.7%, are from sub-Saharan Africa.ConclusionsThis is the first publication on Kawasaki disease from Kenya and one of the largest reports from sub-Saharan Africa. It is the first to have a complete review of the number of published cases from the African continent. Challenges in the diagnosis and management of Kawasaki disease in many African countries include disease awareness, infectious confounders, access and cost of intravenous immunoglobulin, access to pediatric echocardiography and follow-up. Increasing awareness and health care resources are important for improving outcomes of Kawasaki disease in Africa.

Immune control in Kawasaki disease knowledge mapping: a bibliometric analysis.

Kawasaki disease is a systemic vascular disease with an unclear pathophysiology that primarily affects children under the age of five. Research on immune control in Kawasaki disease has been gaining attention. This study aims to apply a bibliometric analysis to examine the present and future directions of immune control in Kawasaki disease. By utilizing the themes "Kawasaki disease," "Kawasaki syndrome," and "immune control," the Web of Science Core Collection database was searched for publications on immune control in Kawasaki disease. This bibliometric analysis was carried out using VOSviewers, CiteSpace, and the R package "bibliometrix." In total, 294 studies on immune control in Kawasaki disease were published in Web of Science Core Collection. The three most significant institutions were Chang Gung University, the University of California San Diego, and Kaohsiung Chang Gung Memorial Hospital. China, the United States, and Japan were the three most important countries. In this research field, Clinical and Experimental Immunology was the top-referred journal, while the New England Journal of Medicine was the most co-cited journal. The Web of Science Core Collection document by McCrindle BW et al. published in 2017 was the most cited reference. Additionally, the author keywords concentrated on "COVID-19," "SARS-CoV-2," and "multisystem inflammatory syndrome in children" in recent years. The research trends and advancements in immune control in Kawasaki disease are thoroughly summarised in this bibliometric analysis, which is the first to do so. The data indicate recent research frontiers and hot directions, making it easier for researchers to study the immune control of Kawasaki disease.

Homotherapy for heteropathy: Interleukin-41 and its biological functions.

Interleukin-41 (IL-41) is a newly discovered cytokine, named Cometin, Subfatin, meteorin-like transcription (Metrnl), and so forth. It is widely expressed in animals and can exert its biological roles through autocrine and paracrine forms. It has functions such as anti-inflammatory, improving body metabolism, regulating immunity, regulating fat metabolism and participates in the process of autoimmune disease or inflammatory injury. It plays an important role in psoriasis, diabetes, Crohn's disease (CD), osteoarthritis, Kawasaki disease (KD), Graves' disease, autoimmune hepatitis, infertility, obesity, sepsis, cardiovascular diseases and respiratory diseases. This paper reviews the biological functions of IL-41, the relationship between IL-41 and diseases, the effects of IL-41 in the cytokine network and the possible signalling pathways. In order to explore the same target or the same drug for the treatment of different diseases from the perspective of homotherapy for heteropathy, cytokine strategies based on IL-41 have been put forward for the precise treatment of immune diseases and inflammatory diseases. It is worth noting that IL-41 related preparations for lung protection and smoking cessation are interesting research fields.

Decreased incidence of Kawasaki disease in South Korea during the SARS-CoV-2 pandemic.

Analyzing Kawasaki disease epidemiology during the SARS-CoV-2 pandemic in South Korea using 2012-2020 National Health Insurance Service data. The incidence of Kawasaki disease for 2012-2020 was investigated to identify changes in incidence after the start of the pandemic. National Health Insurance Service data from the Republic of Korea were used. Kawasaki disease was defined based on the International Statistical Classification of Diseases and Related Health Problems, the Tenth Revision diagnostic code (M30.3), and the intravenous immunoglobulin prescription code. Prescription history was collected for the following medications: intravenous immunoglobulin, aspirin, corticosteroids, tumor necrosis factor-α antagonist, clopidogrel, and anticoagulation drugs. The Kawasaki disease incidence per 100,000 individuals younger than 5 years was 238.9, 230.0, and 141.2 in 2018, 2019, and 2020, respectively. Regarding the incidence from 2012 to 2020, it was the highest in 2018 and decreased to 141.2 (p < 0.001) in 2020, after the start of the pandemic. In 2020, 28.3% of all patients with KD were infants, a percentage significantly higher than that of the previous year (p < 0.001). There was biphasic seasonality in the monthly Kawasaki disease incidence. The Kawasaki disease incidence was the highest in winter followed by that in early summer. After the start of the pandemic, the Kawasaki disease incidence decreased, and the percentage of patients with Kawasaki disease aged <1 year increased. These findings provide support for the hypothesis suggesting an infectious trigger in Kawasaki disease.

Nursing of Children with Incomplete Kawasaki Disease

Objective: To explore how to care for incomplete Kawasaki disease (IKD).&#x0D; Methods: To study the nursing of IKD caused by coronary artery disease by referring to relevant literature. Through the analysis of the existing domestic and foreign literature, it is found that correct and timely nursing plays a vital role in the treatment of IKD.&#x0D; Results: Clinical practice showed that timely and effective nursing measures are the important means to treat IKD. Eye care, skin care, and fever care can reduce the pain caused by this disease.&#x0D; Conclusion: Correct, effective and timely nursing measures play an important role in the treatment of IKD.

Inhibition of IL-1 Ameliorates Cardiac Dysfunction and Arrhythmias in a Murine Model of Kawasaki Disease.

Kawasaki disease (KD) is an acute febrile illness and systemic vasculitis often associated with cardiac sequelae, including arrhythmias. Abundant evidence indicates a central role for IL (interleukin)-1 and TNFα (tumor necrosis factor-alpha) signaling in the formation of arterial lesions in KD. We aimed to investigate the mechanisms underlying the development of electrophysiological abnormalities in a murine model of KD vasculitis. Lactobacillus casei cell wall extract-induced KD vasculitis model was used to investigate the therapeutic efficacy of clinically relevant IL-1Ra (IL-1 receptor antagonist) and TNFα neutralization. Echocardiography, in vivo electrophysiology, whole-heart optical mapping, and imaging were performed. KD vasculitis was associated with impaired ejection fraction, increased ventricular tachycardia, prolonged repolarization, and slowed conduction velocity. Since our transcriptomic analysis of human patients showed elevated levels of both IL-1β and TNFα, we asked whether either cytokine was linked to the development of myocardial dysfunction. Remarkably, only inhibition of IL-1 signaling by IL-1Ra but not TNFα neutralization was able to prevent changes in ejection fraction and arrhythmias, whereas both IL-1Ra and TNFα neutralization significantly improved vasculitis and heart vessel inflammation. The treatment of L casei cell wall extract-injected mice with IL-1Ra also restored conduction velocity and improved the organization of Cx43 (connexin 43) at the intercalated disk. In contrast, in mice with gain of function of the IL-1 signaling pathway, L casei cell wall extract induced spontaneous ventricular tachycardia and premature deaths. Our results characterize the electrophysiological abnormalities associated with L casei cell wall extract-induced KD and show that IL-1Ra is more effective in preventing KD-induced myocardial dysfunction and arrhythmias than anti-TNFα therapy. These findings support the advancement of clinical trials using IL-1Ra in patients with KD.

Investigating the comparative effect of vitamin D level with the type of complications in Henoch Schönlein purpura and Kawasaki disease

Introduction and objectivesHenoch Schönlein purpura (HSP) and Kawasaki disease (KD) are two main inflammatory diseases among childhood vasculitis. Considering the anti-inflammatory effects of 25-hydroxyvitamin D3, we decided to investigate the association of serum 25-hydroxy vitamin D3 level with the type and severity of these conditions. Materials and methodsThe present study was performed as a historical cohort of 254 affected children with KD and HSP vasculitis. The required data were extracted, using a researcher-made questionnaire from patients’ electronic file, and then they were analyzed after collecting information of the patients. ResultsIn HSP group, 54% of participants were boys. Similarly, in KD group, boys were more affected than girls. The comparative 25-hydroxyvitamin vitamin D3 level in HSP patients with and without renal involvement (P=0.02), hematuria (P=0.14), and in two groups with and without heart disease, and also with and without coronary artery dilatation in KD patients (P<0.001) were significant. Discussion and conclusionsThe findings showed that insufficient level of vitamin D3 were significantly associated with the exacerbation of complications of both diseases, and therefore it seems that vitamin D deficiency can be an effective predictive factor of severity in HSP and KD patients.

The landscape of hot topics and research frontiers in Kawasaki disease: Scientometric analysis

PurposeKawasaki disease(KD) is a vascular inflammatory disease that was first identified in 1967. Numerous studies have been conducted on KD and have yielded valuable recent insights. This current bibliometric analysis aimed to determine the intellectual landscape of research interest in KD. MethodsPublications were collected from the Web of Science Core Collection. Bibliometric tools such as CiteSpace and VOSviewer were utilized to analyze the research focus, emerging trends, frontiers, and hot topics in this specific field. ResultsA total of 6122 articles on KD were retrieved. Pediatric Cardiology, Pediatrics International, and Pediatric Infections Disease Journal were the three most productive journals reporting KD development. The University of California San Diego was the most productive institution, with 230 publications. The USA was the most productive country, with 1661 articles in KD. SARS-CoV-2, diagnostic serum biomarkers, and risk factor prediction models for coronary arterial lesions and subtypes of KD are popular topics in KD research. Factors that induce smooth muscle cell transition to myofibroblastic cell, potentially halting the subacute/chronic vasculitis process and endothelial dysfunction in macrophage activation syndrome associated with KD were the frontiers in the study of KD. ConclusionKD has attracted widespread attention worldwide that has continued to increase since 1974. The most productive institution and country are the University of California San Diego and the USA, respectively. SARS-CoV-2, serum biomarkers, and prediction models are hot topics in this field.