scholarly journals 2021 Update on the Clinical Management and Diagnosis of Kawasaki Disease

2021 ◽  
Vol 23 (3) ◽  
Author(s):  
Frank Zhu ◽  
Jocelyn Y. Ang
Keyword(s):  
High Risk ◽  
Kawasaki Disease ◽  
Corticosteroid Therapy ◽  
Clinical Management ◽  
Treatment Guidelines ◽  
Day Treatment ◽  
Significant Proportion ◽  
Clinical Manifestations ◽  
Heart Association ◽  
Ivig Resistance

Abstract Purpose of Review Provide an updated review of the clinical management and diagnosis of Kawasaki disease with inclusion of potential diagnostic difficulties with multisystem inflammatory syndrome in children (MIS-C) given the ongoing COVID-19 pandemic. Recent Findings Adjunctive corticosteroid therapy has been shown to reduce the rate of coronary artery dilation in children at high risk for IVIG resistance in multiple Japanese clinical studies (most notably RAISE study group). Additional adjunctive therapies (etanercept, infliximab, cyclosporin) may also provide limited benefit, but data is limited to single studies and subgroups of patients with cardiac abnormalities. The efficacy of other agents (atorvastatin, doxycycline) is currently being investigated. MIS-C is a clinically distinct entity from KD with broad clinical manifestations and multiorgan involvement (cardiac, GI, hematologic, dermatologic, respiratory, renal). MIS-C with Kawasaki manifestations is more commonly seen in children < 5 years of age. Summary The 2017 American Heart Association (AHA) treatment guidelines have included changes in aspirin dosing (including both 80–100 mg/kg/day and 30–50 mg/kg/day treatment options), consideration of the use of adjuvant corticosteroid therapy in patients at high risk of IVIG resistance, and the change in steroid regimen for refractory KD to include both pulse-dose IVMP and longer course of prednisolone with an oral taper. A significant proportion of children diagnosed with MIS-C, a post-infectious syndrome of SARS-CoV-2 infection, meet criteria for Kawasaki disease. Further investigation is warranted to further delineate these conditions and optimize treatment of these conditions given the ongoing COVID-19 pandemic.

Clinical features and diagnosis of acute rheumatic fever

ESC CardioMed ◽  
2018 ◽  
pp. 1138-1140
Author(s):  
Antoinette Cilliers
Keyword(s):  
High Risk ◽  
Rheumatic Fever ◽  
Acute Rheumatic Fever ◽  
Clinical Signs ◽  
Clinical Manifestations ◽  
Heart Association ◽  
Risk Groups ◽  
Group A ◽  
High Risk Populations ◽  
Minor Criteria

The diagnosis of acute rheumatic fever cannot be made using a single test. The diagnosis requires the recognition of a complex of clinical signs divided into major and minor manifestations as well as laboratory investigations aided by application of the Jones criteria, originally devised in 1944. The clinical manifestations are secondary to the effects of antibodies produced against the group A Streptococcus organism which cross-react against cardiac, skin, synovial, and neurological tissue associated with signs of inflammation. Several adjustments to the Jones criteria have been published over the last 70 years. The latest 2015 American Heart Association modification includes echocardiography/Doppler studies to diagnose subclinical carditis and also incorporates risk stratification whereby at-risk populations are divided into low- and moderate-to-high-risk groups. The presence of a single episode of a fever of at least 38°C and a slight elevation of the erythrocyte sedimentation rate to at least 30 mm/hour are classified as minor criteria in moderate- and high-risk populations. A monoarthritis or polyarthralgia are included as major criteria in the same risk group.

scholarly journals 1025. Prediction of Intravenous Immunoglobulin Resistance and Coronary Artery Dilatation in Kawasaki Disease: a Multicenter Study from Oman

2021 ◽  
Vol 8 (Supplement_1) ◽  
pp. S604-S604
Author(s):  
Fatma Al Mwaiti ◽  
Zaid Alhinai ◽  
Safiya AlAbrawi ◽  
Asmhan A L mamari ◽  
Reem Abdwani ◽  
...  
Keyword(s):  
Logistic Regression ◽  
Coronary Artery ◽  
High Risk ◽  
Kawasaki Disease ◽  
Disease Severity ◽  
Primary Outcome ◽  
Male Gender ◽  
Ivig Resistance ◽  
Coronary Artery Dilatation

Abstract Background Prediction of intravenous immunoglobulin (IVIG) resistance and coronary artery dilatation continues to be a challenge in the management of Kawasaki disease. Significant differences exist among different populations. Methods Children &lt; 13 years of age who presented to the two main tertiary care hospitals in Oman (Royal Hospital and Sultan Qaboos University Hospital) between 2008 and 2019 with a diagnosis of Kawasaki disease were included. Diagnosis was confirmed and clinical, laboratory and echocardiography data was systematically collected and checked for accuracy. The primary outcome was the presence of IVIG resistance or coronary artery dilatation at the 6-week follow-up. Bivariate analysis was used to identify significant predictors of the primary outcome, followed by multivariable logistic regression to determine independent predictors. The Muscat Index of Kawasaki disease Severity (MIKS) score was created based on the results. Results 156 children with Kawasaki disease were included. Median age was 2.1 years (IQR 0.9-3.8), and 64% were males. All patients received IVIG, 26 (17%) received steroids, and one received infliximab. Coronary dilatation was identified in 41 (26%) patients on initial echocardiogram, and 26 (18%) at the 6-week follow-up visit. Variables significantly associated with the primary outcome were age ≤15 months (P=0.031), hemoglobin (P=0.009), WBC count (P=0.002), absolute neutrophil count (P=0.006), and CRP ≥150 mg/L (P=0.015). These variables in addition male gender (P=0.058), ALT &gt;80 IU/L (P=0.10) and serum sodium (P=0.10), were entered into multivariable logistic regression. A predictive model based on CRP ≥150 mg/L (LR=2.2, P=0.049), male gender (LR=2.1, P=0.095) and WBC (LR=1.1, P=0.017) resulted, and it was used as basis for the MIKS score (Table 1). The MIKS score performed favorably to the Kobayashi score in its sensitivity to predict the primary outcome and its separate components (Table 2). Combining the MIKS score with other high-risk criteria had a sensitivity of 95% in predicting the primary outcome and a specificity of 56%. Table 1. Calculation of the Muscat Index of Kawasaki disease Severity (MIKS) score Table 2. Sensitivity, specificity and P value for the Kobayashi, MIKS, and combined high risk criteria in predicting IVIG resistance, coronary dilatation at 6 weeks, separately or in combination, among patients with Kawasaki disease. MIKS: Muscat Index of Kawasaki disease Severity. *High risk: presence of coronary artery dilatation on initial echocardiogram or age &lt;1&gt; Conclusion The MIKS score predicts IVIG resistance and coronary artery dilatation in Kawasaki disease in our setting, with favorable performance compared to the Kobayashi score. Disclosures All Authors: No reported disclosures

scholarly journals Association between Alanine Aminotransferase/Aspartate Aminotransferase Ratio (AST/ALT Ratio) and Coronary Artery Injury in Children with Kawasaki Disease

2020 ◽  
Vol 2020 ◽  
pp. 1-7
Author(s):  
Jinxin Wang ◽  
Jiawen Li ◽  
Yue Ren ◽  
Hongying Shi ◽  
Xing Rong ◽  
...  
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
Aspartate Aminotransferase ◽  
Alanine Aminotransferase ◽  
Laboratory Data ◽  
Clinical Manifestations ◽  
Resistance Rate ◽  
Thrombin Time ◽  
Illness Onset ◽  
Ivig Resistance

Objective. To investigate the association between the aspartate aminotransferase (AST)/alanine aminotransferase (ALT) ratio (AST/ALT ratio, AAR) and intravenous immunoglobulin (IVIG) resistance, coronary artery lesions (CAL), and coronary artery aneurysms (CAA) in children with Kawasaki disease (KD). Design. We retrospectively studied 2678 children with KD and divided them into two groups: a low-AAR group and a high-AAR group with a median AAR of 1.13 as the cut-off point. The differences in laboratory data, clinical manifestations, and coronary artery damage rates were compared between the two groups. Results. The incidence of CAL was higher in the low-AAR group than in the high-AAR group at 2 and 3-4 weeks after illness onset (p<0.001, respectively). The IVIG resistance rate was significantly higher in the low-AAR group than in the high-AAR group (29.94% vs 21.71%, p<0.001). The levels of C-reactive protein, erythrocyte sedimentation rate, white blood cell count, bilirubin, fibrinogen, thrombin time, D-dimer, and brain natriuretic peptide were also significantly higher in the low-AAR group compared with the high-AAR group. The levels of albumin and IgG were significantly lower in the low-AAR group compared with those of the high-AAR group. The proportion of typical KD cases in the low-AAR group was significantly higher than that in the high-AAR group. Low-AAR correlated with the risk of coronary artery damage and IVIG resistance. Conclusion. Children with KD who had low-AAR value were more likely to develop coronary artery damage and IVIG resistance. Low AAR is a risk factor for CAL, CAA, and IVIG resistance in KD.

scholarly journals High risk of coronary artery aneurysm in Kawasaki disease

Rheumatology ◽  
2020 ◽  
Author(s):  
Maria Mossberg ◽  
Aladdin J Mohammad ◽  
Fredrik Kahn ◽  
Mårten Segelmark ◽  
Robin Kahn
Keyword(s):  
Coronary Artery ◽  
High Risk ◽  
Kawasaki Disease ◽  
Intravenous Immunoglobulin ◽  
Demographic Data ◽  
Artery Aneurysm ◽  
Heart Association ◽  
Coronary Aneurysms ◽  
Immunoglobulin Treatment ◽  
Timely Treatment

Abstract Objective Kawasaki disease (KD) is a vasculitis of unknown aetiology with a high risk of coronary aneurysms if untreated. Timely treatment with intravenous immunoglobulin decreases the risk for coronary artery aneurysms (CAA). In this study, we set out to elucidate the factors associated with the risk of developing CAA. Methods Records of all KD-diagnosed children in Skåne between 2004 and 2014 were collected and clinical and demographic data were compiled. KD is defined according to the revised American Heart Association diagnostic criteria and classified as either complete KD (cKD) or incomplete KD (iKD). Results KD was diagnosed in 77 children and CAA was found in 31% (n = 24). Children with CAA were younger compared with children without (median; 20 vs 34 months) and intravenous immunoglobulin treatment within 10 days was less likely to be received (75% vs 91%). In children presenting with iKD, 47% developed CAA compared with 21% in cKD patients. Using multivariate analysis, an association between the risk of CAA with low age in children with iKD was observed. Conclusion The risk of CAA development is disturbingly high in young children with iKD. This highlights the importance of rapid intense treatment and vigilance in infants, who are the most difficult to diagnose, in order to reduce the frequency of CAA.

scholarly journals Current Treatment Guidelines of SARS-CoV-2 Related Multisystem Inflammatory Syndrome in Children: A Literature Review and Expert Opinion

2021 ◽  
Vol 11 (01) ◽  
pp. e133-e140
Author(s):  
Alireza Ghodsi ◽  
Mehrdad Sarabi ◽  
Abdolreza Malek ◽  
Ali Khakshour
Keyword(s):  
Kawasaki Disease ◽  
Treatment Guidelines ◽  
Clinical Manifestations ◽  
Current Treatment ◽  
Current Management ◽  
Management Guidelines ◽  
Current Review ◽  
Systemic Disorder ◽  
Clinical Questions ◽  
Inflammatory Syndrome

AbstractMultisystem inflammatory syndrome in children (MIS-C) is a systemic disorder that seems to be associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Since April 2020, there have been multiple reports about children with this new condition worldwide, including Europe, Asia, Latin America, and North America. The symptoms of this syndrome mimic the clinical manifestations of Kawasaki disease; therefore, the treatment of Kawasaki disease, as well as supportive care, was the management of choice in children with MIS-C in the early days of recognizing it. It is important to precisely ascertain the risk of COVID-19 infection and its severity in children and to acknowledge the management of this syndrome, with reliable data from cohorts, trials, and experts' opinions. In the current review, we summarize the current management guidelines for MIS-C and present our own protocol to answer some clinical questions regarding MIS-C management during the COVID-19 pandemic.

scholarly journals Application of C-reactive Protein/Albumin Ratio (CAR) in Kawasaki Disease

2021 ◽  
Author(s):  
Zhenquan Wang ◽  
Yiping Shao ◽  
Xing Rong ◽  
Huixian Qiu ◽  
Jinxing Wang ◽  
...  
Keyword(s):  
Kawasaki Disease ◽  
Roc Curve ◽  
Laboratory Data ◽  
Clinical Manifestations ◽  
Curve Analysis ◽  
C Reactive Protein ◽  
Roc Curve Analysis ◽  
Albumin Ratio ◽  
Reactive Protein ◽  
Ivig Resistance

Abstract Objective: To investigate the association between the C-reactive protein/albumin ratio (CAR) and coronary artery lesions (CAL), intravenous immunoglobulin (IVIG) resistance in children with Kawasaki disease (KD).Methods: We retrospectively studied 753 children with KD, categorizing them into the CAL group(n=238) and the No-CAL group(n=515), the IVIG-resistance group(n=61) and the No-IVIG- resistance(n=653) group. The differences in laboratory data, clinical manifestations, the relationship between CAR and CAL as well as IVIG resistance were compared between the two cohort groups.Results: Compared with No-CAL group, KD with CAL had a higher CAR (2.12 vs 1.69, p <0.001). And CAR was significantly higher in KD children with IVIG resistance (2.42 vs 1.85, p<0.001). Multivariable logistic regression analysis demonstrated that higher CAR was a risk factors of CAL(OR=1.198, p<0.001) and IVIG resistance (OR=1.297, p<0.001), respectively. CAL and IVIG resistance interact with each other. ROC curve analysis performed for the prediction of CAL, the best cut-off point for CAR was 1.80(AUC=0.602, sensitivity 64.7%, specificity 54.8%). When predicting IVIG resistance according to the ROC curve analysis, the optimal cutoff point for CAR was 2.20(AUC=0.621, sensitivity 59.0%, specificity 61.1%).Conclusions: CAR is a valid indicator in KD children. Higher CAR may be helpful in predicting CAL and IVIG resistance in KD.

Predictive tool for intravenous immunoglobulin resistance of Kawasaki disease in Beijing

2018 ◽  
Vol 104 (3) ◽  
pp. 262-267 ◽  
Author(s):  
Shuai Yang ◽  
Ruixia Song ◽  
Junmei Zhang ◽  
Xiaohui Li ◽  
Caifeng Li
Keyword(s):  
High Risk ◽  
Kawasaki Disease ◽  
Intravenous Immunoglobulin ◽  
External Validation ◽  
Predictive Tool ◽  
Validation Data ◽  
Data Set ◽  
Children's Hospital ◽  
Ivig Resistance ◽  
Children’S Hospital

ObjectiveTo construct a predictive tool for the efficacy of intravenous immunoglobulin (IVIG) therapy in children with Kawasaki disease (KD) in Beijing, China.DesignThis was a cohort study. Data set (including clinical profiles and laboratory findings) of children with KD diagnosed between 1 January 2010 and 31 December 2015 was used to analyse the risk factors and construct a scoring system. Data set of children with KD diagnosed between 1 January 2016 and 1 December 2016 was used to validate this model.SettingChildren’s Hospital Capital Institute of Pediatrics and Beijing Children’s Hospital.Patients2102 children diagnosed with KD.InterventionsNo.Main outcome measuresResponsiveness to IVIG.ResultsThe predictive tool included C reactive protein ≥90 mg/L (3 points), neutrophil percentage ≥70% (2.5 points), sodium ion concentration <135 mmol/L (3 points), albumin <35 g/L (2.5 points) and total bilirubin >20 μmol/L (5 points), which generated an area under the the receiver operating characteristic curve of 0.77 (95% CI 0.71 to 0.82) for the internal validation data set, and 0.69 (95% CI 0.58 to 0.81) and 0.63 (95% CI 0.53 to 0.72) for two external validation data sets, respectively. If a total of ≥6 points were considered high-risk for IVIG resistance, sensitivity and specificity were 56% and 79% in the internal verification, and the predictive ability was similar in the external validation.ConclusionsThe predictive tool is helpful in early screening of high-risk IVIG resistance of KD in the Beijing area. Consequently, it will guide the clinician in selecting appropriate individualised regimens for the initial treatment of this disease, which is important for the prevention of coronary complications.

scholarly journals Association of Thrombomodulin Gene C1418T Polymorphism with Susceptibility to Kawasaki Disease in Chinese Children

2018 ◽  
Vol 2018 ◽  
pp. 1-5 ◽  
Author(s):  
Yapeng Lu ◽  
Rui Liu ◽  
Luting Zha ◽  
Shan Yuan ◽  
Lang Tian ◽  
...  
Keyword(s):  
Coronary Artery ◽  
High Risk ◽  
Kawasaki Disease ◽  
Protein C ◽  
Systemic Vasculitis ◽  
Vascular Diseases ◽  
Healthy Children ◽  
Coronary Artery Lesions ◽  
Ivig Resistance ◽  
Anticoagulant System

Kawasaki disease (KD) is an acute systemic vasculitis that predominantly affects children and can result in coronary artery lesions (CALs). Thrombomodulin (TM) is a critical cofactor in the protein C anticoagulant system. The TM C1418T (rs1042579) polymorphism is associated with a high risk of cardiac-cerebral vascular diseases. But the association of the TM C1418T polymorphism with susceptibility to KD, CAL formation, and intravenous immunoglobulin (IVIG) resistance is still unclear. In our study, we examined the TM C1418T polymorphism in 122 children with KD and 126 healthy children and revealed the correlation between the TM C1418T polymorphism and KD, CAL formation, and IVIG resistance.

scholarly journals Defining Kawasaki disease and pediatric inflammatory multisystem syndrome-temporally associated to SARS-CoV-2 infection during SARS-CoV-2 epidemic in Italy: results from a national, multicenter survey

2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Marco Cattalini ◽  
◽  
Sara Della Paolera ◽  
Fiammetta Zunica ◽  
Claudia Bracaglia ◽  
...  
Keyword(s):  
Kawasaki Disease ◽  
Clinical Laboratory ◽  
Troponin T ◽  
Inflammatory Diseases ◽  
Age At Onset ◽  
Laboratory Data ◽  
Clinical Manifestations ◽  
Chi Square ◽  
Pediatric Society ◽  
Multicenter Survey

Abstract Background There is mounting evidence on the existence of a Pediatric Inflammatory Multisystem Syndrome-temporally associated to SARS-CoV-2 infection (PIMS-TS), sharing similarities with Kawasaki Disease (KD). The main outcome of the study were to better characterize the clinical features and the treatment response of PIMS-TS and to explore its relationship with KD determining whether KD and PIMS are two distinct entities. Methods The Rheumatology Study Group of the Italian Pediatric Society launched a survey to enroll patients diagnosed with KD (Kawasaki Disease Group – KDG) or KD-like (Kawacovid Group - KCG) disease between February 1st 2020, and May 31st 2020. Demographic, clinical, laboratory data, treatment information, and patients’ outcome were collected in an online anonymized database (RedCAP®). Relationship between clinical presentation and SARS-CoV-2 infection was also taken into account. Moreover, clinical characteristics of KDG during SARS-CoV-2 epidemic (KDG-CoV2) were compared to Kawasaki Disease patients (KDG-Historical) seen in three different Italian tertiary pediatric hospitals (Institute for Maternal and Child Health, IRCCS “Burlo Garofolo”, Trieste; AOU Meyer, Florence; IRCCS Istituto Giannina Gaslini, Genoa) from January 1st 2000 to December 31st 2019. Chi square test or exact Fisher test and non-parametric Wilcoxon Mann-Whitney test were used to study differences between two groups. Results One-hundred-forty-nine cases were enrolled, (96 KDG and 53 KCG). KCG children were significantly older and presented more frequently from gastrointestinal and respiratory involvement. Cardiac involvement was more common in KCG, with 60,4% of patients with myocarditis. 37,8% of patients among KCG presented hypotension/non-cardiogenic shock. Coronary artery abnormalities (CAA) were more common in the KDG. The risk of ICU admission were higher in KCG. Lymphopenia, higher CRP levels, elevated ferritin and troponin-T characterized KCG. KDG received more frequently immunoglobulins (IVIG) and acetylsalicylic acid (ASA) (81,3% vs 66%; p = 0.04 and 71,9% vs 43,4%; p = 0.001 respectively) as KCG more often received glucocorticoids (56,6% vs 14,6%; p < 0.0001). SARS-CoV-2 assay more often resulted positive in KCG than in KDG (75,5% vs 20%; p < 0.0001). Short-term follow data showed minor complications. Comparing KDG with a KD-Historical Italian cohort (598 patients), no statistical difference was found in terms of clinical manifestations and laboratory data. Conclusion Our study suggests that SARS-CoV-2 infection might determine two distinct inflammatory diseases in children: KD and PIMS-TS. Older age at onset and clinical peculiarities like the occurrence of myocarditis characterize this multi-inflammatory syndrome. Our patients had an optimal response to treatments and a good outcome, with few complications and no deaths.

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