Refractory Kawasaki disease: modified methylprednisolone regimen decreases coronary artery dilatation

Author(s):  
Mingming Zhang ◽  
Yang Zheng ◽  
Xiaohui Li ◽  
Shuai Yang ◽  
Lin Shi ◽  
...  
2018 ◽  
Vol 11 (4) ◽  
pp. NP144-NP147
Author(s):  
Aleisha M. Nabower ◽  
Lois J. Starr ◽  
Jonathan Cramer

Kawasaki disease can be difficult to diagnose in infants, putting them at higher risk for developing coronary artery dilatation. It can be even more difficult to diagnose in the setting of preexisting cardiac anomalies such as those found in Williams syndrome. We present a case of a three-month-old male with Williams syndrome with rapidly developing giant coronary aneurysms due to Kawasaki disease. This case demonstrates the importance of repeat echocardiography in diagnosing incomplete Kawasaki disease in infants. We speculate that elastin changes, as present in Williams syndrome, may put affected children at higher risk for development of giant coronary arteries should they acquire Kawasaki disease.


Eye ◽  
2020 ◽  
Vol 34 (10) ◽  
pp. 1883-1887 ◽  
Author(s):  
Reza Shiari ◽  
Mohsen Jari ◽  
Saeed Karimi ◽  
Omid Salehpour ◽  
Khosro Rahmani ◽  
...  

2016 ◽  
Vol 11 (02) ◽  
pp. 051-052
Author(s):  
Jolanta Bernatoniene ◽  
Gareth Morgan ◽  
Paul Heaton

2013 ◽  
Vol 55 (2) ◽  
pp. 237-240 ◽  
Author(s):  
Ibtissama Boukas ◽  
Nagib Dahdah ◽  
Yves Robitaille ◽  
Anne Fournier

2021 ◽  
Vol 8 (Supplement_1) ◽  
pp. S604-S604
Author(s):  
Fatma Al Mwaiti ◽  
Zaid Alhinai ◽  
Safiya AlAbrawi ◽  
Asmhan A L mamari ◽  
Reem Abdwani ◽  
...  

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 < 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 >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 <1> 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


Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
Cedric Manlhiot ◽  
Andrew M Crean ◽  
Nigel Fernandopulle ◽  
Brendan Lew ◽  
Tanveer H Collins ◽  
...  

Background: Long-term outcomes and life expectancy for children with a previous history of Kawasaki disease (KD), particularly those with coronary artery aneurysms (CAA), remain to be determined. Methods: An inception cohort of all patients with KD assessed at The Hospital for Sick Children in Toronto between 1978 and 2013 was assembled. Patient outcomes were obtained throughout their pediatric and adult clinical follow-up as long as available. Prevalence of outcomes over time was modelled with Kaplan-Meier survival curves. Life tables from Statistics Canada were used to obtain age/gender specific cumulative mortality for the general population. Results: The cohort included 2,623 KD patients, of whom 410 (16%) had coronary artery involvement (215 dilatation, 57 non-giant CAA and 138 giant CAA). Average follow-up for patients with coronary artery involvement was 6.7 years (13.3 years for giant CAA); 57 and 34 patients had at least 15 and 25 years of follow-up, respectively. No patients with coronary artery dilatation or non-giant CAA had revascularization or a myocardial infarct. Freedom from revascularization (14 events) for patients with giant CAA was 90±6%, 87±7% and 80±13% at 10, 20 and 40 years of follow-up. Freedom from myocardial infarct (11 events) was 94±4%, 92±5% and 89±7% at 5, 20 and 40 years. For patients without coronary artery involvement, 3 (0.1%) deaths were recorded, one secondary to complications of macrophage activation syndrome during the acute phase of KD and 2 from cancer. No deaths were noted for patients with coronary artery dilatation or non-giant CAA, although clinical follow-up was more limited. For patients with giant CAA, 3 deaths (2.2%) were noted, 2 related to CAA complications and 1 from non-medical cause. Cumulative mortality for patients with giant CAA was 1.5% at 10 years of age (expected mortality 0.7%, HR: 2.2 (0.3-11.5), p=0.08) and 3.1% at 40 years of age (expected 2.3%, HR: 1.3 (0.4-4.0), p=0.37). Conclusions: Despite risks of myocardial infarction and revascularization, patients with giant CAA had life-expectancy similar to that of the general population up to the fourth decade of life. Additional follow-up will be necessary to determine if these trends continue into later decades.


2016 ◽  
Vol 37 (6) ◽  
pp. 1148-1152 ◽  
Author(s):  
Soha Rached-D’Astous ◽  
Ibtissama Boukas ◽  
Anne Fournier ◽  
Marie-Josée Raboisson ◽  
Nagib Dahdah

2010 ◽  
Vol 31 (8) ◽  
pp. 1232-1235 ◽  
Author(s):  
Deane Yim ◽  
James Ramsay ◽  
Darshan Kothari ◽  
David Burgner

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