Search

Abstract Kawasaki Disease (KD) is a multisystemic vasculitis of medium- and small-sized arteries. Abnormal intimal thickening may develop in the involved arterial area after regression of coronary artery aneurysm (CAA). Intimal dysfunction may induce local stenosis or arteriosclerosis in the future. In this case–control study, we investigated 29 consecutive KD patients [20 male, median current age, 7.9 years; median follow-up duration, 5.7 years] and a group of 29 healthy matched controls (CON) [19 male, median current age, 10.8 years]. They were assesed and compared for CAA, LVFS, GCS, GLS, coronary artery (CA) Z scores, carotid intima-media thickness (IMT) and coronary artery IMT by high-resolution transthoracic echocardiography (hrTTE). Coronary artery IMT (caIMT) was significantly higher in patients with a maximal CA Z score > 2.5 in acute KD than in CON: KD caIMT: 0.62 mm [IQR, 0.57–0.72 mm] vs. 0.53 mm [0.51–0.60 mm], p = 0.043. CAAs were found in 15 (51.7%) patients with acute KD. The maximal median LCA Z score in acute KD was 2.57z [IQR, 1.93—3.2z] and in follow-up −0.39z [IQR, −1.25 to −0.36z]. There was no significant difference in carotid IMT between KD patients and CON. Signs of CA intima-media thickening were detected by hrTTE in patients with a maximal CA Z score > 2.5 in acute KD. These data indicate that these patients may be at risk for cardiovascular sequale even in the absence of permanent CA luminal abnormalities. Therefore long-term follow-up of this group of KD patients may be required.

Abstract Background Whole blood host transcript signatures show great potential for diagnosis of infectious and inflammatory illness, with most published signatures performing binary classification tasks. Barriers to clinical implementation include validation studies, and development of strategies that enable simultaneous, multiclass diagnosis of febrile illness based on gene expression. Methods We validated five distinct diagnostic signatures for paediatric infectious diseases in parallel using a single NanoString nCounter® experiment. We included a novel 3-transcript signature for childhood tuberculosis, and four published signatures which differentiate bacterial infection, viral infection, or Kawasaki disease from other febrile illnesses. Signature performance was assessed using receiver operating characteristic curve statistics. We also explored conceptual frameworks for multiclass diagnostic signatures, including additional transcripts found to be significantly differentially expressed in previous studies. Relaxed, regularised logistic regression models were used to derive two novel multiclass signatures: a mixed One-vs-All model (MOVA), running multiple binomial models in parallel, and a full-multiclass model. In-sample performance of these models was compared using radar-plots and confusion matrix statistics. Results Samples from 91 children were included in the study: 23 bacterial infections (DB), 20 viral infections (DV), 14 Kawasaki disease (KD), 18 tuberculosis disease (TB), and 16 healthy controls. The five signatures tested demonstrated cross-platform performance similar to their primary discovery-validation cohorts. The signatures could differentiate: KD from other diseases with area under ROC curve (AUC) of 0.897 [95% confidence interval: 0.822–0.972]; DB from DV with AUC of 0.825 [0.691–0.959] (signature-1) and 0.867 [0.753–0.982] (signature-2); TB from other diseases with AUC of 0.882 [0.787–0.977] (novel signature); TB from healthy children with AUC of 0.910 [0.808–1.000]. Application of signatures outside of their designed context reduced performance. In-sample error rates for the multiclass models were 13.3% for the MOVA model and 0.0% for the full-multiclass model. The MOVA model misclassified DB cases most frequently (18.7%) and TB cases least (2.7%). Conclusions Our study demonstrates the feasibility of NanoString technology for cross-platform validation of multiple transcriptomic signatures in parallel. This external cohort validated performance of all five signatures, including a novel sparse TB signature. Two exploratory multi-class models showed high potential accuracy across four distinct diagnostic groups.

Kawasaki disease (KD) is an acute systemic vasculitis that mostly affects children less than 5years. Occasionally, it may presents with renal involvement of varying severity. In Nigeria and most of Africa, only a few cases of KD have been reported and these were among children within the typical age group. We report an unusual case of Kawasaki disease with renal manifestation in a 14 year old adolescent. Apart from the principal features of KD comprising of high grade fever, non purulent conjunctivitis, polymorphous rash, right sided cervical lymphadenitis and symmetrical desquamative lesions of the digits of the hands and feet; our patient also had renal involvement. The renal manifestations included mild periorbital edema, oliguria, hypertension (140/90mmHg), hematuria(++), proteinuria(++) and elevated serum urea and creatinine (8.3mmol/L and 1.9mg/dl respectively). He was managed with high dose aspirin at 80mg/kg/day. The dose was reduced (5mg/Kg/day) and subsequently stopped after serial echocardiography showed normal coronary arteries. Intravenous immune globuline (IVIG) could not be started due to non availability. Nevertheless, clinical signs resolved, renal function normalised after 6 weeks and echocardiographic picture did not deteriorate. Patient is currently on follow up at the paediatric cardiology clinic of UDUTH, Sokoto, Nigeria.. Conclusion: Kawasaki disease can occur even in older children and renal manifestation may be self limiting. This report highlights the need for high index of suspicion in all cases.

Objective To evaluate the predictive value for serum albumin-globulin ratio （A/G） for the risk of Kawasaki disease （KD） in febrile children. Methods In this single-center retrospective cohort study， the big-data platform of a certain third-tier A-level hospital was employed. Clinical and laboratory data of 432 febrile children aged 0-16 were collected. The children were divided into 4 groups based on the quartiles and median of their serum A/G ratios. The correlation between the A/G and the risk of KD was analyzed. Results The total incidence rate of KD in 432 children with fever was 14.4% （62/432）.A significant negative correlation was observed between the A/G and the risk of KD. For each increase of 0.1 in the A/G， the risk of KD was decreased by 18% （OR=0.82， 95% CI 0.76-0.88， P < 0.001）. Subgroup analysis showed a consistent negative correlation between the A/G and KD risk across different ages， genders， and biochemical markers. Conclusions A/G may be a useful biomarker for assessing the risk of KD in febrile children. As the A/G increases， the risk of KD is gradually decreased. This finding has potential clinical application value for early diagnosis and risk assessment of KD.

Abstract The study aims to explore the central genes that Kawasaki disease (KD) and Obesity (OB) may jointly contribute to coronary artery disease. Investigating single-cell datasets (GSE168732 and GSE163830) from a comprehensive gene expression database, we identified characteristic immune cell subpopulations in KD and OB. B cells emerged as the common immune cell characteristic subgroup in both conditions. Subsequently, we analyzed RNA sequencing datasets (GSE18606 and GSE87493) to identify genes associated with B-cell subpopulations in KD and OB. Lastly, a genome-wide association study and Mendelian randomization were conducted to substantiate the causal impact of these core genes on myocardial infarction. Quantitative real-time PCR (qRT-PCR) to validate the expression levels of hub genes in KD and OB. The overlapping characteristic genes of B cell clusters in both KD and OB yielded 70 shared characteristic genes. PPI analysis led to the discovery of eleven key genes that significantly contribute to the crosstalk. Employing receiver operating characteristic analysis, we evaluated the specificity and sensitivity of these core genes and scored them using Cytoscape software. The inverse variance weighting analysis suggested an association between TNFRSF17 and myocardial infarction risk, with an odds ratio of 0.9995 (95% CI = 0.9990–1.0000, p = 0.049). By employing a single-cell combined transcriptome data analysis, we successfully pinpointed central genes associated with both KD and OB. The implications of these findings extend to shedding light on the increased risk of coronary artery disease resulting from the co-occurrence of OB and KD.

Introduction. Kawasaki disease (KD) and pediatric inflammatory multisystem syndrome (PIMS) are similar vasculitis conditions affecting medium and small vessels, particularly the coronary arteries. This study aimed to highlight diagnostic and treatment challenges in a case initially diagnosed as PIMS, which evolved into atypical KD. Materials and methods. A 2-year-10-month-old male was admitted with a high fever persisting for 6 days, unresponsive to antipyretics. Results. On admission, the patient exhibited a poor general condition, pale skin, non-pruritic erythematous rash, and enlarged, non-painful lymph nodes. Laboratory tests showed elevated inflammatory markers, leukocytosis with neutrophilia, hypoalbuminemia, liver enzyme abnormalities, altered cardiac markers, and elevated D-dimers, but no initial echocardiographic changes. Positive SARS-CoV-2 antibodies led to an initial PIMS diagnosis, and treatment with intravenous methylprednisolone, antiplatelet, anticoagulant therapy, and hepatoprotective agents was started. The patient’s condition initially improved but then worsened with febrile spikes, rash, neutropenia, thrombocytopenia, anemia, and coronary artery dilation on echocardiography. This led to a diagnosis of KD with atypical onset, and treatment with immunoglobulin, antiplatelet, and anticoagulant therapy was initiated. The patient’s condition improved slowly. Conclusions. KD and PIMS share overlapping characteristics, making them difficult to distinguish. There is no definitive criterion to differentiate KD and PIMS, leading to potential diagnostic errors.

Abstract Background Kawasaki disease (KD) is an immune vasculitis of unknown origin, characterized by transient inflammation. The activation of the cGAS-STING pathway, triggered by mitochondrial DNA (mtDNA) release, has been implicated in the onset of KD. However, its specific role in the progression of inflammation during KD's acute phase remains unclear. Methods We measured mtDNA and 2’3’-cGAMP expression in KD patient serum using RT-qPCR and ELISA. A murine model of KD was induced by injecting Lactobacillus casei cell wall extract (LCWE), after which cGAS-STING pathway activation and inflammatory markers were assessed via immunohistochemistry, western blot, and RT-qPCR. Human umbilical vein endothelial cells (HUVECs) were treated with KD serum and modulators of the cGAS-STING pathway for comparative analysis. Mitochondrial function was evaluated using Mitosox staining, mPTP opening was quantified by fluorescence microscopy, and mitochondrial membrane potential (MMP) was determined with JC-1 staining. Results KD patient serum exhibited increased mtDNA and 2’3’-cGAMP expression, with elevated levels of pathway-related proteins and inflammatory markers observed in both in vivo and in vitro models. TEM confirmed mitochondrial damage, and further studies demonstrated that inhibition of mPTP opening reduced mtDNA release, abrogated cGAS-STING pathway activation, and mitigated inflammation. Conclusion These findings indicate that mtDNA released through the mPTP is a critical activator of the cGAS-STING pathway, contributing significantly to KD-associated inflammation. Targeting mtDNA release or the cGAS-STING pathway may offer novel therapeutic approaches for KD management.

Key Clinical Message Kawasaki disease (KD), a self‐limiting vasculitis, can present with a broader spectrum of vascular involvements, necessitating early recognition and prompt management. This case exemplifies the importance of involving multiple teams on board in managing complex presentations of KD. It also highlights the importance of close monitoring for the progression of the disease spectrum as well as family education to ensure favorable outcomes. The case also emphasizes the importance of long‐term follow‐up and further research to understand the prognosis, early screening tools, and possible complications due to multi‐organ involvement in KD along with their management strategies. Abstract Kawasaki disease (KD) is a multisystem vascular inflammatory syndrome, which predominantly affects the small and medium vessels in children within the age group of less than 5 years. The most threatened complication is the development of coronary artery aneurysms (CAAs). We present an extremely rare case of KD in a 2‐month, 21‐day‐old male infant with extensive vascular involvement, expanding the disease spectrum beyond the involvement of coronary arteries. These included aneurysmal dilatations of both internal carotid arteries, the descending aorta, bilateral multilevel intercostal arteries, coeliac artery, superior mesenteric artery, and both renal arteries. Implementing a multidisciplinary approach with early treatment via intravenous immunoglobulin (IVIG) and dexamethasone proved to be most effective in the patient's management. Despite unique challenges such as severe coronary dilation and pseudomonas sepsis during the special care, the patient was stabilized and discharged after 11 days of hospital stay, highlighting the importance of early prompt management and a centered approach to evaluate in a broader spectrum. This case emphasizes the importance of long‐term follow‐up and further research to understand the prognosis, early screening tools, and possible complications due to multi‐organ involvement in KD along with their management strategies.

Abstract Although intravenous immunoglobulin (IVIG)-resistant Kawasaki disease (KD) presents with persistent inflammatory stimulation of the blood vessels and an increased risk of coronary artery dilatation. However, the pathogenesis of this disease is unclear, with no established biomarkers to predict its occurrence. This study intends to explore the utility of S100A12/TLR2-related signaling molecules and clinical indicators in the predictive modeling of IVIG-resistant KD. The subjects were classified according to IVIG treatment response: 206 patients in an IVIG-sensitive KD group and 49 in an IVIG-resistant KD group. Real-time PCR was used to measure the expression of S100A12, TLR2, MYD88, and NF-κB in peripheral blood mononuclear cells of patients, while collecting demographic characteristics, clinical manifestations, and laboratory test results of KD children. Multi-factor binary logistic regression analysis identified procalcitonin (PCT) level (≥ 0.845 ng/mL), Na level (≤ 136.55 mmol/L), and the relative expression level of S100A12 (≥ 10.224) as independent risk factors for IVIG-resistant KD and developed a new scoring model with good predictive ability to predict the occurrence of IVIG-resistant KD.

The virus called SARS-CoV-2 emerged in 2019 and quickly spread worldwide, causing COVID-19. It has greatly impacted on everyday life, healthcare systems, and the global economy. In order to save as many lives as possible, precautions such as social distancing, quarantine, and testing policies were implemented, and effective vaccines were developed. A growing amount of data collected worldwide allowed the characterization of this new disease, which turned out to be more complex than other common respiratory tract infections. An increasing number of convalescents presented with a variety of nonspecific symptoms emerging after the acute infection. This possible new global health problem was identified and labelled as long COVID. Since then, a great effort has been made by clinicians and the scientific community to understand the underlying mechanisms and to develop preventive measures and effective treatment. The role of autoimmunity induced by SARS-CoV-2 infection in the development of long COVID is discussed in this review. We aim to deliver a description of several conditions with an autoimmune background observed in COVID-19 convalescents, including Guillain-Barré syndrome, antiphospholipid syndrome and related thrombosis, and Kawasaki disease highlighting a relationship between SARS-CoV-2 infection and the development of autoimmunity. However, further studies are required to determine its true clinical significance.

Objective·To analyze the progression of children with severe coronary artery lesions due to Kawasaki disease by coronary artery angiography, and evaluate the diagnostic value of echocardiography in these children.Methods·A retrospective analysis was performed to enroll children with Kawasaki disease whose coronary artery lesions were graded Ⅳ or above from Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, from January 2013 to January 2023. The subjects were required to have received at least 2 times of coronary angiogram, and their clinical and imaging data were collected to analyze the progression of the lesions. Echocardiography results were compared with the results of the coronary angiogram.Results·A total of 21 children were included, including 15 males and 6 females, with a median age at onset of 3 years and 6 months, a median age at initial coronary angiography of 7 years and 11 months, a median interval of 4 years and 5 months between the time of onset and initial angiography, a median age at angiographic review of 9 years and 2 months, and a median interval of 1 year and 3 months between the time of initial angiography and review. Coronary stenosis or occlusion was detected in 13 children in the initial angiography, of whom 6 underwent coronary artery bypass grafting (CABG) and had their angiography reviews 1 year later. The review results showed that the bridging vessels were unobstructed and no obvious stenosis was observed. Fifteen children had progression of the lesions detected by echocardiography in the subsequent follow-up and had their angiogram reviews, of whom 8 had significant progression of the coronary lesions. Intracoronary balloon dilatation was performed in 1 case, and CABG was performed in another case. Sixteen lesions of coronary stenosis or occlusion were detected in the initial angiography in 21 children, while only 1 lesion of coronary stenosis was detected by echocardiography during the same period of time. Twenty-eight medium- to large-sized coronary aneurysms were detected in the initial angiography in the 21 children, and the diameters of the 28 aneurysms measured by echocardiography and coronary angiogram were subjected to the Bland-Altman analysis. The Bland-Altman analysis showed that the difference in maximum diameter between 2 methods was (1.63±2.33) mm, with 95%CI of -2.95‒6.21 mm.Conclusion·Coronary artery lesions due to Kawasaki disease may be progressive; in the children with severe lesions, coronary artery stenosis or occlusion may be missed or misdiagnosed and some errors may exist in the measurement of diameters of aneurysms by echocardiography. Regular review of coronary angiography is needed.

Background: Autoimmune diseases are a group of diseases caused by abnormal immune responses to functional body parts. Single-cell RNA-sequencing (scRNA-seq) technology provides transcriptomic information at the single-cell resolution, thus offering a new way to study autoimmune diseases. Most single-cell RNA-seq studies, however, have often focused on one type of autoimmune disease. Methods: We integrated scRNA-seq data from peripheral blood cells of five different autoimmune diseases (IgA nephropathy [IgAN], Kawasaki disease [KD], multiple sclerosis [MS], Sjogren’s syndrome [SS], and systemic lupus erythematosus [SLE]). We performed dimensionality clustering, cellular communication analysis, re-clustering analysis of monocytes, NK cell populations, differential gene expression analysis, and functional enrichment for all immune cells in these data. Results: We integrated the scRNA-seq results of peripheral blood cells from five different autoimmune diseases (IgAN, KD, MS, SS, and SLE). We showed that all samples contained 18 different immune cell subsets, although the cell cluster populations were different among the 5 diseases. Through intercellular communication network analysis, we determined that the signals of classical and non-classical monocytes were significantly enhanced in patients with IgAN and SLE. The signals of naïve B cells were increased in patients KD. Interestingly, the signals of NK and NK-T cells were enhanced in patients with SS, but reduced in patients with IgAN and SLE. Transcriptomic analysis of classical and non-classical monocyte subsets further revealed that pro-inflammatory cytokines and interferon-related genes, including CCL3, IL1B, ISG15, and IFI6, were specifically increased in patients with IgAN and SLE. Unlike monocytes, the number and NK marker genes were decreased in patients with IgAN and KD, but increased in patients with SS. Meanwhile, two NK-T cell subsets were exclusively found in SS. Conclusions: In summary, based on an integration of the single-cell RNA-seq results, we demonstrated changes in the immune cell landscape of five different autoimmune diseases with respect to immune cell subsets, populations, differentially-expressed genes, and the cell-to-cell communication network. Our data provide new insight to further explore the heterogeneity and similarity among different autoimmune diseases.

Kawasaki disease shock syndrome (KDSS) is a rare disease characterized by cardiovascular collapse that requires aggressive supportive and immunomodulatory therapy. The purpose of this report is to highlight our management strategies in KSSS patients. Patients who were followed up with a diagnosis of Kawasaki disease in intensive care unit and those who met the criteria for Kawasaki disease shock syndrome were included in the study. Data were obtained retrospectively from hospital records. Between 2005 and 2020, 5 patients with Kawasaki disease were followed up in the pediatric intensive care unit. Three children in the adolescent age group were diagnosed with Kawasaki disease shock syndrome. Two patients had severe coronary artery dilatation, one patient required therapeutic plasma exchange due to multiple organ failure. Kawasaki disease shock syndrome is a serious, life-threatening form of Kawasaki disease and should be suspected in children with severe inflammation and significant cardiac involvement. Administration of plasmapheresis in addition to steroid therapy appears to be effective in controlling severe disease and should not be delayed.

Introduction Kawasaki disease (KD) is a systemic vasculitis, seen mostly in children. Epidemiology of KD is dependent on geographical location and seasonality. Although many years have passed since the first report of KD, multiple related factors are still unknown. Material and methods We investigated the clinical, paraclinical, and therapeutic aspects of KD in Kerman, Iran by performing a retrospective, descriptive, cross-sectional study on all children hospitalized due to KD between 2007 and 2020. Results A total of 340 patients with mean ±SD age of 29.83 ±22.55 months participated in the study. Most of our patients were two to five years old. The male : female ratio was ~ 1.4 : 1. A few of our patients had a family history of KD or vasculitis (0.3%, 1.7%). Typical KD was more common by a large margin (316 patients with typical KD). More than half of our patients had a duration of hospitalization of under ten days. All of our patients were febrile. Hand/foot and lip/mouth changes were the second and third most common clinical findings in more than 60% of our patients. Other manifestations were conjunctivitis in 40%, skin rashes in 34.8%, gastrointestinal manifestations in 33.9%, and lymphadenopathy in 25.3%. Echocardiography revealed abnormalities in 78.6% of the participants; coronary artery aneurysm (CAA) was the most frequent (22.5%) and follow-up echocardiography revealed that all of them regressed within 6 months after treatment. The two laboratory tests with the highest ratio of abnormality were erythrocyte sedimentation rate (95%) and hemoglobin (83.3%). C-reactive protein and liver function tests were also abnormal in most patients. All of our patients received intravenous immunoglobulin and acetylsalicylic acid. Conclusions Kawasaki disease must be considered in every febrile child, especially those with risk factors, because timely diagnosis and treatment are essential to prevent complications. Health policies should focus on appropriate diagnosis and treatment to prevent the occurrence of sequelae.

Background The incidence of mucocutaneous lymph node syndrome (MCLS), also known as Kawasaki disease (KD), has been increasing year by year. Coronary artery lesions (CAL) induced by KD has become the main cause of acquired heart disease in children. Many clinical studies show that mycoplasma pneumoniae (MP) is associated with CAL in KD, but the strength of association between them differs across original research on different individuals. Objective To systematically evaluate the correlation between MP infection and CAL in KD, providing evidence for early and timely delivery of effective clinical treatment to improve the quality of life and to prevent adverse outcomes in children with KD. Methods Observational studies on KD〔KD patients with MP infection (MP-IgM positive) (exposed group) compared with those with simple KD (control group), with CAL as the outcome measure〕 were retrieved from databases of CNKI, Wanfang Data, VIP, SinoMed, PubMed, Embase, Cochrane Library and Web of Science from inception to April 3, 2022. Two researchers independently performed literature screening and data exaction. The Newcastle-Ottawa Scale (NOS) was used to evaluate the quality of included studies. Stata 15.0 and RevMan 5.4 were used for data analysis. Results A total of 31 studies involving 6 131 subjects were included. The average quality score of the studies rated using the NOS was 7, indicating a high overall quality. Meta-analysis showed that the risk of CAL in the exposed group was higher than that in the control group〔RR=1.65, 95%CI (1.40, 1.94), P1∶160, or by quantitative PCR, or undescribed method (P

Objective To investigate the clinical and pathological characteristics of KDSS with heart failure. Methods The clinical data of a child with KDSS complicated with heart failure were analyzed, and the related literature was reviewed. Results KDSS is uncommon in Kawasaki disease, and it always attended with serious performance, which can lead to circulatory failure in the acute stage. manifested as systemic circulatory failure with tachycardia, prolonged capillary filling time, chills at the end of limbs, weak pulse, reduced urine volume or disturbance of consciousness. Early identification and timely comprehensive treatment of human immunoglobulin and hormones can significantly improve the condition of shock. Conclusion KDSS is a rare but serious clinical manifestation of Kawasaki disease, and it is easy to be complicated with heart failure. Early identification and comprehensive clinical treatment are effective measures to reduce KDSS complicated with heart failure.

Key Clinical Message There was an 83‐year‐old man having coronary artery disease associated with coronary artery ectasia who occurred ventricular fibrillation suddenly during coronary artery angiography. As Kawasaki disease was suspected to the most likely reason which led to coronary artery lesion.

AbstractBackground Intravenous immunoglobulin (IVIG) resistance is of prime importance in Kawasaki disease (KD). In this study, we examined the value and mechanism of serum amyloid A (SAA) level in predicting IVIG resistance in patients with KD.Methods SAA levels were measured in 497 consecutive patients with KD before IVIG therapy in the training set. The patients were divided into two groups (IVIG-responsive and IVIG-resistant) according to the American Heart Association (AHA) definition of IVIG resistance. Demographic, echocardiographic, and laboratory data were also retrospectively analyzed and tabulated to predict IVIG resistance. The predictive value of SAA was validated on test sets of prospective data. Cytokine microarrays were analyzed from 4 patients with resistant to IVIG, 4 patients with responsive to IVIG and 4 healthy volunteers.Results During the training set, 409 patients with KD were enrolled, of whom 43 (10.5%) were resistant to initial IVIG treatment and 47 (11.49%) had coronary artery lesions (CALs). Serum levels of SAA were higher in the IVIG resistant group compared to the IVIG responsive group, (380.00 [204.40–547.25] vs 230.85 [105.40–490.00] mg/L; p = .008). The values of total bilirubin, C-reactive protein, neutrophils, alanine aminotransferase, aspartate aminotransferase, interleukin-6(IL-6), and procalcitonin were significantly higher in the IVIG-resistant group than in the IVIG-responsive group (p

Introduction Differential diagnosis of the systemic juvenile idiopathic arthritis (sJIA) is often complicated, because of the variability in clinical presentation and the absence of specific signs. Material and Methods The PubMed/Medline and Scopus databases from the years 2013–2022 were analysed for full articles in English and the following key words were used: “juvenile idiopathic arthritis” and “MIS-C”; “juve-nile idiopathic arthritis” and “Kawasaki disease”. As an example of the problem the case description of a 3-year-old patient is presented. Results In the first step 167 publications were identified; however, after exclusion of duplicated articles and those not relevant to the topic, only 13 were included in the analysis. We analysed studies that de-scribe overlapping clinical features of sJIA and Kawasaki disease (KD) or multisystem inflammatory syndrome in children (MIS-C). The main issues we discussed were the search for the specific fea-tures that would distinguish one disease from another. Fever refractory to intravenous immuno-globulin treatment was the most frequent indicator among the features of clinical courses. Among other clinical signs prolonged, recurrent fever, rash, an incomplete KD phenotype, Caucasian race, splenomegaly, and complicated macrophage activation syndrome also supported sJIA diagnosis. Among laboratory tests, high ferritin and serum interleukin-18 levels were found to be the most useful in differentiation. The present case demonstrates that prolonged, unexplained, recurrent fe-ver with a specific pattern should be the reason to suspect sJIA. Conclusions Overlapping features of sJIA and SARS-CoV-2-related MIS-C complicates diagnosis in the era of the COVID-19 pandemic. Our case presentation adds symptoms of prolonged, spiking, unexplained, re-current fever with a specific pattern for supporting systemic juvenile idiopathic arthritis diagnosis.

Objective To summarize the clinical characteristics and explore the risk factors of Kawasaki disease(KD) with deep neck space involvement(DNSI). Methods This study was a case-control study. We reviewed KD complicated with DNSI patients in Department of Rheumatology and Immunology of Shenzhen Children's Hospital from January 2018 to December 2020 as DNSI group. Meanwhile, children with KD withoutDNSI during this period were selected by systematic sampling at a ratio of 1∶7 as control group. The clinical characteristics were analyzed by Chi-square test and the Mann-Whitney test and risk factors of KD complicated with DNSI were analyzed by Logistic regression. Results A total of 38 children in the DNSI group who met the inclusion and exclusion criteria and 288 children in the control group were selected. In the DNSI group, 38 children (100%) had fever and cervical lymph node enlargement, and the cervical lymph node enlargement occurred within 5 days of onset; 30 patients (78.9%) had cervical lymph node pain and 25 patients suffered (65.8%) limited movement of neck. Compared with the control group, the clinical data of children in the DNSI group showed a variety of significant changes. In terms of clinical characteristics, the age of onset in the DNSI group was older, the hospital stay was longer and the proportions of cervical lymphadenopathy, cervical lymph node pain, limited neck movement and upper airway obstruction were all higher (all P < 0.05); in terms of laboratory tests, the neutrophil count and its percentage, C-reactive protein (CRP), ferritin (FER), total bile acid, total bilirubin, direct bilirubin, and globulin levels all increased, while platelet and lymphocyte counts and their percentages all decreased(all P < 0.05); in terms of coronary artery damage and treatment effect, the Kobayashi score, Sano score and the proportion of hormone therapy in the DNSI group all increased (all P < 0.05). Multivariate Logistic regression analysis showed that neck lymph node pain (OR=5.523, 95% CI: 1.443-21.141, P=0.013), limited cervical movement (OR=3.947, 95% CI: 1.044-14.928, P=0.043), higher CRP (OR=1.016, 95% CI: 1.002-1.030, P=0.024) and higher FER(OR=1.004, 95% CI: 1.001-1.006, P=0.002) were independent risk factors for KD combined with DNSI. Conclusions Most children with KD complicated with DNSI have clinical symptoms such as cervical lymph node enlargement, cervical lymph node pain, and limited cervical movement, and hematology shows that there is a high-intensity inflammatory response. For KD children with neck pain and limited cervical movement as the main clinical manifestations, accompanied by elevated serum CRP and FER, we should be alert to the possibility of DNSI.

The SARS-CoV2 pandemic is the most significant global health emergency of the last century. While the pathophysiology of SARS-CoV2 is understood, the early and long-term outcomes of natural infection are increasingly being recognised. Multisystem inflammatory syndrome (MIS) represents a manifestation of the extreme immune dysfunction that SARS-CoV2 infection heralds and has been described in both children (MIS-C) and adults (MIS-A). Here, we discuss current knowledge of MIS-A and the vast questions that remain unanswered.

Kawasaki disease is an acute febrile exanthema pediatric disease with systemic vasculitis as the main lesion, which is the most common cause of acquired heart disease in children. The etiology of the disease is not clear, and the pathogenesis remains to be explored. Some cases of Kawasaki disease are prone to misdiagnosis due to atypical clinical presentations, and patients who have not been effectively treated are at increased risk of coronary artery lesion. Therefore, timely diagnosis of Kawasaki disease and prediction of coronary artery lesion have great significance for early treatment and prognosis of Kawasaki disease. MiRNA plays an important role in various life processes of organisms, and dysregulation of miRNA is involved in the occurrence and development of many diseases. Current studies have shown that miRNA can assist in the diagnosis, prognosis assessment and large-scale population screening of diseases, and has potential clinical application prospects as a novel biomarker. Recent studies have found that miRNA is associated with the pathogenesis of Kawasaki disease, and expression dysregulation will lead to immune imbalance and vascular damage in patients with Kawasaki disease. Other studies have shown that circulating miRNA can be used as a potential biomarker of Kawasaki disease, mainly focusing on early diagnosis and efficacy prediction of Kawasaki disease. The current research is still at the exploratory stage, and more clinical studies are needed to verify the early diagnosis and prediction efficacy of miRNA related to Kawasaki disease. This article reviews the functional mechanism and biomarkers of miRNA in Kawasaki disease in recent years.

Background: Identifying risk factors in patients with intravenous immunoglobulin (IVIG) resistance Kawasaki disease (KD) is vital in managing and preventing coronary artery aneurysms (CAAs). We aimed to study the risk factors for IVIG resistance KD in Malaysian children. Methods: This retrospective observational study of children with KD was conducted at two tertiary hospitals in Malaysia from January 2014 to December 2019. Multivariable binary logistic regression was used to analyze the risk factors associated with IVIG resistance. Results: A total of 174 patients, 118 males (67.8%) with a median age of 1.4-year-old (interquartile range: 0.1–12.1-year-old), were analyzed. Early (10 days) IVIG treatments were observed in 14 (8.1%) and19 (11.0%), respectively. Thirty-two patients (18.4%) had IVIG resistance. The independent factors associated with IVIG resistance were high white cell count, hypoalbuminemia, and extremities changes with an odd ratio of 4.7, 3.0, and 4.0, respectively. In addition CAA was significantly higher in IVIG resistance compared to IVIG responder patients (57.5% [19/33] vs. 23.4% [33/141], P < 0.001). The sensitivity was high in Harada (93.8%) but low in Kobayashi and Egami (46.9%and 34.4%, respectively). The specificity was high with Egami (79.6%) but low in Harada and Kobayashi (22.5% and 64.1%, respectively). Conclusion: Leukocytosis, hypoalbuminemia, and extremities changes were independent risk factors for IVIG resistance. The variation in sensitivity and specificity of the Japanese scoring makes it unsuitable for predicting IVIG resistance in Malaysian children.

Objective We aimed to develop a nomogram to predict risk of resistance to intravenous immunoglobulin (IVIG) in children with Kawasaki disease in eastern China. Methods We retrospectively analysed the data of children with Kawasaki disease who received IVIG during hospitalisation at Soochow University Affiliated Children’s Hospital. IVIG resistance was defined as recrudescent or persistent fever ≥36 h after the end of the IVIG infusion. Baseline variables were analysed using least absolute shrinkage and selection operator (LASSO) to identify the predictors of IVIG resistance, which were then used to construct a predictive nomogram. Calibration curve and area under the receiver operating characteristic curve (AUC) were used to evaluate the performance of the model. The predictive nomogram was validated on test sets of external data and prospective data. Results Between January 2015 and December 2020, 1293 Kawasaki disease patients were hospitalized in Soochow University Affiliated Children’s Hospital. Among them, 72 (5.57%) showed IVIG resistance. LASSO identified haemoglobin, percentage of neutrophils, C-reactive protein level, platelet count, serum albumin, serum sodium, serum alkaline phosphatase, coronary artery damage, and complete Kawasaki disease as risk factors for IVIG resistance. The nomogram constructed using these factors showed satisfactory discriminatory power (AUC, 0.75), and sensitivity (0.74) and specificity (0.64). In the external data and prospective data, the AUC was 0.66 and 0.83, respectively, the sensitivity was 0.86 and 1, respectively, and the specificity was 0.49 and 0.60, respectively. Conclusions The predictive nomogram constructed using nine factors associated with IVIG resistance in children with Kawasaki disease could be a useful tool for identifying patients likely to show IVIG resistance. This nomogram may help reduce the risk of coronary artery lesions.Key Messages None of the IVIG resistance scoring systems has shown consistently good performance in previous studies. Tools to predict the risk of IVIG resistance in eastern China are lacking. In our series, haemoglobin level, percentage of neutrophils, platelet count, coronary artery damage, incomplete Kawasaki disease, and CRP, serum albumin, serum sodium, and serum alkaline phosphatase levels were risk factors of IVIG resistance in hospitalized children in the eastern China cities of Suzhou and Fuzhou. We propose an easy-to-use nomogram to predict the risk factors of IVIG resistance in hospitalized children.

Summary: Background: Children with SARS-CoV-2 related Multisystem Inflammatory Syndrome in Children (MIS-C) often present with clinical features that resemble Kawasaki disease (KD). Disease severity in adult COVID-19 is associated to the presence of anti-cytokine autoantibodies (ACAAs) against type I interferons. Similarly, ACAAs may be implicated in KD and MIS-C. Therefore, we explored the immunological response, presence of ACAAs and disease correlates in both disorders. Methods: Eighteen inflammatory plasma protein levels and seven ACAAs were measured in KD (n = 216) and MIS-C (n = 56) longitudinally by Luminex and/or ELISA. Levels (up to 1 year post-onset) of these proteins were related to clinical data and compared with healthy paediatric controls. Findings: ACAAs were found in both patient groups. The presence of ACAAs lagged behind the inflammatory plasma proteins and peaked in the subacute phase. ACAAs were mostly directed against IFN-γ (>80%) and were partially neutralising at best. KD presented with a higher variety of ACAAs than MIS-C. Increased levels of anti-IL-17A (P = 0·02) and anti-IL-22 (P = 0·01) were inversely associated with ICU admission in MIS-C. Except for CXCL10 in MIS-C (P = 0·002), inflammatory plasma proteins were elevated in both KD and MIS-C. Endothelial angiopoietin-2 levels were associated with coronary artery aneurysms in KD (P = 0·02); and sCD25 (P = 0·009), angiopoietin-2 (P = 0·001), soluble IL-33-receptor (ST2, P = 0·01) and CXCL10 (P = 0·02) with ICU admission in MIS-C. Interpretation: Markers of endothelial activation (E-selectin, angiopoietin-2), and innate and adaptive immune responses (macrophages [CD163, G-CSF], neutrophils [lipocalin-2], and T cells [IFN-γ, CXCL10, IL-6, IL-17]), are upregulated in KD and MIS-C. ACAAs were detected in both diseases and, although only partly neutralising, their transient presence and increased levels in non-ICU patients may suggest a dampening role on inflammation. Funding: The Kawasaki study is funded by the Dutch foundation Fonds Kind & Handicap and an anonymous donor. The sponsors had no role in the study design, analysis, or decision for publication.

Background Kawasaki disease (KD) is a multi-systemic vasculitis that primarily affects children and has an unknown cause. Although an increasing number of studies linking the gut microbiota with KD, the unchallengeable etiology of KD is not available. Methods Here, we obtained fecal and oral samples from KD patients and healthy controls, and then we use high-throughput sequencing to examine the diversity and composition of microbiota. Results Results showed that both in the gut and oral microbiota, the diversity of KD patients was significantly lower than that of the healthy controls. In the gut microbiota, a higher abundance of Enterococcus (40.12% vs less than 0.1%), Bifidobacterium (20.71% vs 3.06%), Escherichia-Shigella (17.56% vs 0.61%), Streptococcus (5.97% vs 0.11%) and Blautia (4.69% vs 0.1%) was observed in the KD patients, and enrichment of Enterococcus in the patients was observed. In terms of oral microbiota, the prevalence of Streptococcus (21.99% vs 0.1%), Rothia (3.02% vs 0.1%), and Escherichia-Shigella (0.68% vs 0.0%) were significantly higher in the KD patients, with the enrichment of Streptococcus and Escherichia-Shigella. Additionally, significant differences in microbial community function between KD patients and healthy controls in the fecal samples were also observed, which will affect the colonization and reproduction of gut microbiota. Conclusions These results suggested that the dysbiosis of gut and oral microbiota are both related to KD pathogenesis, of which, the prevalence of Enterococcus in the gut and higher abundance of Streptococcus and Escherichia-Shigella in the oral cavity will be a potential biomarker of the KD. Overall, this study not only confirms that the disturbance of gut microbiota is a causative trigger of KD but also provides new insight into the oral microbiota involved in KD pathogenesis.

Background and purpose: Kawasaki is an acute childhood vasculitis of unknown cause that if left untreated can cause coronary aneurysms in 25% of cases. The disease is reported as the leading cause of acquired heart disease in children in developed countries. The purpose of this study was to investigate the epidemiological and clinical features of Kawasaki disease in northern Iran. Materials and methods: In this descriptive-analytic review study, all registered cases of Kawasaki disease in Mazandaran province between 2007 and 2019 were investigated. Epidemiological, demographic, clinical, periodical tests, risk factors, disease burden, complications, and follow-up information were collected. Data analysis was done applying descriptive statistics, Chi-square test, and logistic regression. Results: The data of 136 children with Kawasaki disease were assessed. Clinical symptoms were found to be significantly different between children with complete and incomplete Kawasaki disease (P0.05). Multivariate regression analysis showed that fever for more than 5 days is a predictor of coronary artery involvement (P= 0.001). Conclusion: The study showed that fever for more than 5 days is a risk factor for heart and coronary artery involvement as one of the complications leading to death in these children. Therefore, accurate control of fever and heart examinations are important in children with symptoms of

Abstract Background The pathogenesis of the novel described multisystem inflammatory syndrome in children (MIS-C) and Kawasaki disease (KD) is still debated as it is not clear if they are the same or different nosological entities. However, for both the diseases a rapid and unequivocal diagnosis is mandatory to start the therapy before the onset of severe complications. In this study, we aimed to evaluate the white cell populations in MIS-C and KD as potential markers to discriminate between the two diseases. Methods We studied white cell populations by flow cytometry in 46 MIS-C and 28 KD patients in comparison to 70 age-matched healthy children. Results MIS-C patients had a significant lymphopenia that involved both B and T populations while KD patients showed a significant neutrophilia and thrombocythemia. Granulocyte/lymphocyte ratio helped to diagnose both MIS-C and KD with a high diagnostic sensitivity, while a multivariate analysis of granulocyte and T lymphocyte number contributed to discriminate between the two diseases. Conclusions The relevant lymphopenia observed in MIS-C patients suggests that the disease would be a post-infectious sequel of COVID-19 immunologically amplified by a massive cytokine release, while the significant neutrophilia and thrombocythemia observed in KD confirmed that the disorder has the genesis of a systemic vasculitis. The analysis of a panel of circulating cells may help to early diagnose and to discriminate between the two diseases.

Kawasaki disease is a common systemic vasculitis in children that can involve multiple organs and tissues, among which coronary artery damage is the most severe. With the increasing incidence of Kawasaki disease, neurological complications have been attracting widespread attention. Clinical manifestations of Kawasaki disease neurological complications are atypical. If not be timely diagnosed and treated, it may cause disability. In this article, neurological complications of Kawasaki disease were reviewed, aiming to enable clinicians to divert attention to this disease.

Key Clinical Message COVID may manifest multisystem inflammatory syndrome in children (MIS‐C) which mimics Kawasaki disease (KD). Differentiating KD and MIS‐C is difficult. Immunomodulatory treatment should be initiated promptly without accurate diagnosis. Abstract A febrile Ukrainian infant developed giant aneurysms in coronary arteries. Differentiating between Kawasaki disease and multisystem inflammatory syndrome in children was difficult. In both illnesses, coronary aneurysm may develop unless treated promptly. Therefore, guidelines should synthesize these clinical entities so that treatment can be initiated before rigorous diagnosis.

Aim:Kawasaki disease (KD) is difficult to diagnose in infants, since the disease course is subtle. We aimed to identify whether infants with KD demonstrate significant alterations in laboratory parameters that can be used to distinguish them from other febrile infants.Materials and Methods:In this retrospective case-control study, infants diagnosed with KD between January 2010 and December 2019 were evaluated and compared to a cohort of febrile infants admitted with prolonged fever during the same period. Demographic, clinical, and laboratory features were recorded and compared between these two groups.Results:A total of 42 infants (27 male) with KD (32 incomplete KD) and 84 age-matched febrile infants (57 male) were evaluated. Coronary artery involvement was identified in 20 (47.6%) infants of whom 5 (25%) had coronary aneurysms. All infants with KD were treated with IVIG and high dose acetylsalicylic acid, and 38 (90.5%) responded to treatment. The duration of fever and hospitalization were longer in infants with KD compared to the controls (p

Kawasaki disease is a medium-size vasculitis with peak incidence among infants of 9 to 11 month age. This case report represents a female infant of 18-month age whom was admitted to the Taleghani Pediatric Center of Gorgan, Iran; with referral for admission due to persistent fever for roughly 2 weeks despite various outpatient treatments. Upon the admission, Echocardiographic study was performed to assess whether the patient fulfils cardiac criteria of Kawaski disease which was consistent with the diagnosis and also positive for serious coronary complications in the infant. General condition of the patient improved as the treatment with both IVIG and Corticosteroids was initiated and carditis seemed to vanish in echocardiographic studies but as expected aneurysmal growth can last for over 80 days and in this case serial echocardiographic studies confirmed the formation of giant coronary aneurysms. Diagnosis of Kawasaki disease is based on persistent fever and consistency with clinical criteria and the main purpose of this case report was to emphasize the need to consider this disease in cases of persistent fever to avoid the serious following complications.

Background: Kawasaki disease is a pediatric, systemic, vasculitic disorder. Its exact etiology is still unknown. Genetic polymorphisms are being investigated as susceptibility factor for this disorder. These are likely to vary among different populations. Aim: To investigate the association of single nucleotide polymorphism (SNP) rs113420705 of CASP3 in Kawasaki disease (KD) from North India. Settings and Design: Observational, case–control study. Methods: Polymerase chain reaction and bidirectional Sanger sequencing was used for determining genotypes of SNP rs113420705 in 45 cases of KD and 50 healthy age- and sex-matched controls. Allele and genotype frequencies were assessed and compared between the groups. Results: Among 45 cases, 32 had TT (71.1%), 13 had CT (28.9%) and none had CC genotype of SNP rs113420705. No significant differences in allele, genotype, or carrier frequencies of rs113420705 were found between the two groups. A comparison was also made between subgroups of KD with coronary abnormality (7 children; 15.5%) and KD with normal coronaries (38 children; 84.4%). The C allele was significantly overexpressed in KD with coronary abnormality group (P = 0.005). However, no difference was noted in the genotype frequencies. Conclusion: CT genotype of rs113420705 of CASP3 showed a trend to significance with the occurrence of KD in children in North India. However, we could not establish any association between minor allele C and susceptibility to KD. C allele appeared to be over expressed in children with KD with coronary abnormalities. Larger studies will help us to reach conclusive evidence applicable to all ethnicities.

Clinical manifestations of SARS-CoV-2 in children are not typical. These range from relatively mild to no symptoms in 90% of patients to a severe Kawasaki like disease compared to adults. The outcome is usually favorable in children. There are relatively few studies related to coronavirus disease (COVID-19) in children, no major clinical trials exist. The experience of this novel disease in adults is being extrapolated to manage pediatric COVID-19 cases. This review summarizes the current understanding of pediatric COVID-19 with regards to the epidemiology, clinical manifestations and management.

Abstract Background Kawasaki disease is an idiopathic medium-sized vasculitis that occurs primarily in infants and children younger than 5 years of age. Atypical Kawasaki disease applies to patients who do not fulfill the complete criteria of fever of 5 days or more with at least four of five features: bilateral conjunctival injection, changes in the lips and oral cavity, cervical lymphadenopathy, extremity changes, and polymorphous rash. Acute kidney injury is defined as a sudden decline in kidney function within hours, including structural injuries and loss of function. Acute kidney injury is extremely common in hospitalized pediatric patients. However, it is rarely documented in Kawasaki disease. Acute kidney injury is underestimated in Kawasaki disease due to the lack of a clear definition of age-specific normal serum creatinine levels and routine renal functions. This report describes a case who presented with clinical features suggestive of atypical Kawasaki disease and developed acute kidney injury. Case presentation A 2-year-old Saudi girl had a history of high-grade fever for 5 days, moderate dehydration, dry cracked lips, poor appetite, and generalized erythematous rash; therefore, she was diagnosed to have incomplete Kawasaki disease. Laboratory investigations revealed normochromic normocytic anemia, leukocytosis, thrombocytosis, high inflammatory markers, and acute kidney injury stage III. An echocardiogram showed a 4-mm dilatation on the left main coronary artery and a 3-mm dilatation on the right. A renal biopsy was not performed to identify the cause of the injury as it showed improvements after the start of the specific therapy for Kawasaki disease; intravenous immune globulin at a dose of 2 g/kg, aspirin at a high dosage of 80 mg/kg/day, and prednisolone at 2 mg/kg. In addition to the acute kidney injury management, normal saline boluses were followed by furosemide at a 2 mg/kg dose. Her urine output increased, and her renal functions normalized. She was discharged in good condition after 10 days. Conclusions It is valuable to check renal function tests in a confirmed case of Kawasaki disease to reduce the negative consequences of late acute kidney injury discovery. Early detection and intervention make a substantial difference in acute kidney injury management.

This report presents a case of a 9-month-old male subject, with an initial presentation of a viral-like illness progressing rapidly to multiple deep neck abscesses involving the bilateral submandibular, parapharyngeal, retropharyngeal, and anterior mediastinal spaces subsequently confirmed with contrast-enhanced computed tomography of the neck. Additionally, the subject developed mucocutaneous signs of generalized erythematous rash, with red, fissured lips along with edema of the extremities. Initial lab parameters were suggestive of high inflammatory markers, low albumin, and deranged coagulation profile. Treatment with broad-spectrum intravenous antibiotics, albumin, and fresh frozen plasma infusions was commenced. Echocardiography revealed dilatation of the left main coronary artery with mild mitral valvular regurgitation. Based on the clinical criteria, a diagnosis of typical Kawasaki disease (KD) on day 5 of illness was made, and the subject received intravenous immunoglobulin (IVIg) and aspirin. The clinical course was complicated by IVIg resistance, for which a second dose of IVIg and pulse systemic corticosteroid therapy was required. Post defervescence, an isolated elevation of activated thromboplastin time, partially corrected with mixing studies and a positive circulating lupus anticoagulant was noted. The patient did not develop any bleeding or thromboembolic complications during his hospital stay. Surgical drainage of the neck abscesses did not yield any pus or fluid collection. After a stormy course of 31 days, the subject was discharged with an improving trend of inflammatory markers and no progression of coronary artery dilatation. Gradual resolution to baseline health was noted upon follow-up, 18 months postdischarge from the hospital. Despite being one of the most common cause of childhood acquired cardiac disease, KD still poses a significant diagnostic and treatment challenge to physicians. This requires physicians to be vigilant for timely diagnosis and appropriate management to prevent significant cardiac comorbidities and be aware of the possible complications that might arise during the treatment.

Introduction: Severe acute respiratory syndrome coronavirus-2 infection has emerged as a multisystem disorder during the pandemic of Coronavirus disease of 2019 (Covid-19). Multisystem inflammatory syndrome of children (MIS-C) is a rare grave complication of Covid-19 due to immunodysregulation. Case Presentation: We present a 14-year-old boy who had fever, cardiac and gastrointestinal symptoms, mucocutaneous manifestations and cervical adenopathy with a background of recent asymptomatic Covid-19 infection. Inflammatory and cardiac biomarker panels were raised in the absence of other microbial causes of inflammation. Discussion: Although there were overlapping features of Kawasaki disease, the ultimate diagnosis was MIS-C. He was successfully managed with immunomodulatory therapy.

Hyponatremia is one of the common electrolyte disorders that can accompany many diseases. Kawasaki disease is also one of these situations. Kawasaki disease is the most common vasculitis in children under the age of five years and can occur with very different clinical pictures. Since Kawasaki shock syndrome has been described more recently, its unknown features are high. In this case report, we presented a patient who presented to the emergency department with the clinical findings of hyponatremia and was diagnosed with Kawasaki shock syndrome.

Introduction Serum ferritin (SF) is an acute-phase reactant in inflammatory diseases. Our aim was to analyze the clinical implications of SF in Kawasaki disease (KD). Material and methods 244 KD children were divided into 6 subgroups. SF, inflammatory mediators and blood cell counts were detected. Results (1) SF dramatically increased in the acute phase of KD and maintained after IVIG therapy; (2) SF increased in IVIG-nonresponsive KD patients (AUC = 0.705; sensitivity: 57.10%; specificity: 82.90%); SF positively correlated with the internal diameter of the coronary artery (AUC = 0.603; sensitivity: 92.30%; specificity: 37.70%); (3) SF increased in 4 patients with the macrophage activation syndrome (MAS)/MAS tendency (979.03 ±474.19 µg/l). Conclusions SF is implied to be a helpful biomarker for forecasting IVIG-nonresponsive KD, coronary artery abnormalities (CAAs) and MAS tendency.

Coronavirus 2 acute and severe respiratory infection virus (SARS-CoV2) has been identified as a pathogen of COVID-19 disease. Initially it was thought that children were safe from the virus, but several reports showed Multisystem Inflammatory Syndrome in Children (MIS-C) as a dangerous complication of COVID-19. There are similarities and differences between MIS-C and Kawasaki disease, Kawasaki shock syndrome, and toxic shock. It is a multisystem disease that affects major systems, including cardiovascular, respiratory, blood and coagulation, kidney, and nervous systems. Diagnosis of MIS-C is based on evidence of recent SARS-CoV2 infection and multiple system involvement, and laboratory criteria for high inflammation in the absence of other causes. In many of these patients chest imaging may show no evidence of COVID-19 involvement, or abnormal findings such as pleural effusion, ground glass patchy opacities, or local density and atelectasis may be seen. Echocardiography shows involvement of pericardium, myocardium, endocardium, and coronary arteries, which may be accompanied by cardiac arrhythmias. On abdominal imaging, evidence of ascites may be reported in these patients. In whole blood tests, lymphopenia, anemia, and thrombocytopenia are common, and inflammatory markers are very high. In mild cases, patients can be closely monitored, but many of these children develop severe forms and require hospitalization or pediatric intensive care unit. This study narratively reviews the clinical manifestations of multisystemic inflammatory syndrome in children following COVID-19.

Abstract Two cases of Kawasaki disease (KD) presented as persistent lung consolidation associated with Group A Streptococcus and Influenza A co‐infection, which resolved following intravenous immunoglobulin. Thus, pediatricians should consider the diagnosis of KD in the presence of pneumonia that is nonresponsive to antibiotic therapy with prolonged fever and inflammatory reactions.

Abstract A 22‐year‐old male with a history of Kawasaki disease was admitted to our emergency department with an inferior ST elevation myocardial infarction (STEMI). He was infected with COVID‐19 one month prior to admission, and thereafter, stopped the medication 10 days prior to the STEMI.

Multisystem inflammatory syndrome in children (MIS-C) is a rare and critical condition that affects children following exposure to severe acute respiratory syndrome Coronavirus 2 (SARS-CoV-2) infection, leading to multiorgan dysfunction and shock. MIS-C has been reported from different parts of the world but rarely from Arab countries. In this report, we describe a 15-year-old Arab boy who was admitted to the ICU during the surge of Coronavirus transmission in Syria with a clinical picture consistent with MIS-C, including high-grade fever, gastrointestinal symptoms, rash, multiorgan dysfunction, and shock. Laboratory profile showed significant elevation of inflammatory markers, negative SARS-CoV-2 RT-PCR testing but positive serologic testing for SARS-CoV-2. The patient received intravenous immunoglobulins (IVIG) and glucocorticoids with remarkable cardiac improvement and significant alleviation in inflammatory markers. To our knowledge, this is the first reported case of MIS-C from Syria, which adds to the epidemiological data about this new syndrome.

Hypertransaminasemia in patients with Kawasaki disease (KD) is reported to be transient. Here, we describe a child with an atypically protracted course of liver tests abnormalities and review the inherent literature. The patient was hospitalized at age 7-months for isolated hypertransaminasemia detected during a classical KD diagnosed 3 months before, and persistent since then. KD clinical evolution had been favorable, with rapid response to acetylsalicylic acid and intravenous immunoglobulins. Liver enzymes however remained persistently elevated with a fluctuating pattern (ALT > AST levels; peak of AST 186 IU/L and ALT 240 IU/L). During follow-up, the main causes of liver dysfunction had to be excluded through appropriate and extensive laboratory investigations. Transaminases values become steadily normal only 7 months after the acute presentation of KD. Conclusions: Our report shows that an atypically protracted courses of KD-related hypertransaminasemia above the previously reported temporal limits should be taken into account during the stepwise diagnostic approach to the patient’s liver dysfunction. Insidious acetylsalycilic acid-hepatotoxicity warrants consideration in the differential diagnosis.

Kawasaki disease (KD) is a systemic vasculitis and is the most frequent pediatric acquired heart disease in developed countries. The diagnosis of KD is typically made by the ≧ 5 of 6 principal signs. However, approximately 20% of KD patients present with less than 5 of these diagnostic signs but may be suffering from coronary artery aneurysms and have been diagnosed with incomplete KD. In this case report, we describe a 2-month-old infant who showed just fever without any other signs of KD but was suffered from multiple arterial aneurysms, including coronary, pulmonary, and carotid arteries. Because she did not respond to intravenous immunoglobulin infusion, we placed her on plasma exchange that has successfully brought defervescence without any significant complications. This case may represent the end spectrum of incomplete KD in very young infants.

Introduction and purpose: Kawasaki disease is an acute, self-limited vasculitis of medium arteries, and it affects children under 5 years old. It can lead to coronary artery aneurysms. Brief description of the state of knowledge: Pathogenesis of Kawasaki disease is unclear. The diagnosis is based on the clinical findings. The characteristic symptoms are polymorphous skin rashes, conjunctivitis, erythema, dryness, cracking and bleeding of the lips, erythema and swelling of palms and soles and cervical lymphadenopathy. Kawasaki disease is defined as a fever for 5 days accompanied by 4 or more of the diagnostic symptoms described above. The classic diagnosis is based on the exclusion of other diseases too. The diagnosis of incomplete Kawasaki disease is suggested if less than 4 main clinical features are found. It is the most common cause of acquired heart disease in children. Kawasaki disease can lead to coronary artery aneurysm, myocarditis and pericarditis so it is very important to treat this illness correctly. Basic treatment is a single infusion of intravenous immunoglobulin and high-dose aspirin. Recurrent Kawasaki disease (persistent fever after first line treatment with IVIG and aspirin) requires the same treatment as used for the first episode. Corticosteroids and immune-modulating therapies can be used as second and third line options. Quick recognition and early treatment result in a reduction of coronary artery abnormalities. Conclusion: This article reviews basic informations, history, epidemiology, pathophysiology, clinical presentation, diagnostic criteria, complications and treatment of Kawasaki disease.

Kawasaki disease (KD) is a systemic vasculitis of small and medium-sized vessels that mainly influences children under five. In the classic diagnostic criteria of KD, there is no Gastrointestinal involvement. Myocarditis is one of the unusual cardiovascular complications of KD, and there is only a very minority of patients have symptoms suggestive of clinical myocarditis. Case Presentation: We reported a 7-year-old boy admitted with a high fever and bloody diarrhea. The stool exam revealed many white blood cells (WBCs) and red blood cells (RBCs). On the 5th day of admission, we found bilateral cervical lymphadenopathy, bilateral nonexudative conjunctivitis, cracked lips, and maculopapular rashes over the extremities. Echocardiography revealed severe mitral regurgitation (MR) with reduced ejection fraction (EF). We treated the patients with oral high-dose aspirin (100 mg/kg/d) plus intravenous immunoglobulin (IVIG 2 gr/kg). Cardiovascular complications such as acute clinical myocarditis should be highly suspected in any patient KD with persistent bloody diarrhea.

We describe 26 children with multisystem inflammatory syndrome associated with coronavirus disease in the pediatric intensive care unit of Roberto del Río Hospital (Santiago, Chile). In total, 10 (38.5%) children required mechanical ventilation; 13 (50.0%) required inotropic support. In addition, 18 (69.2%) patients had echocardiographic abnormalities. No patients died.

BACKGROUND AND OBJECTIVE: Kawasaki disease is an acute vasculitis of childhood and can be associated with complications such as hyponatremia. The present study was conducted to evaluate the serum sodium levels and its association with cardiac complications in children with Kawasaki disease. METHODS: In this cross-sectional study, all Kawasaki patients (according to AHA criteria) referred to Amirkola Children's Hospital in Babol in 2017-2018 were evaluated. Patients underwent serum sodium assessment and echocardiography and the relationship between serum sodium levels and cardiac complications including ectasia and coronary aneurysm, myocarditis, pericarditis and coronary artery occlusion was investigated. FINDINGS: Of the 60 patients with Kawasaki disease, 27 (45%) were male and 33 (55%) were female. Their mean age was 32±8 months. 28 patients (47%) had serum sodium levels lower than 135 mEq/L while normal serum sodium level (135-150 mEq/L1) was reported in 53% of patients. Cardiac complications including coronary ectasia and myocarditis were reported in 16 and 32 patients, respectively. There was no significant difference between serum sodium levels and cardiac complications. There was no difference in the incidence of cardiac complications and hyponatremia in males and females. CONCLUSION: According to the results of the present study, serum sodium levels cannot be a good criterion for cardiac prognosis.