Abstract 217: Difference of Coronary Artery Lesions or associated Pathogens between the Two Phenotypes of Kawasaki Disease

Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
Kyung Lim Yoon ◽  
Song Ee Youn ◽  
Mi Young Han ◽  
Sung Ho Cha

Introduction: There are several reports that coronary artery lesions (CALs) are increased/or not increased in patients who predominantly showed arthritis in patients with Kawasaki disease (KD). Patients with eczematoid skin lesions which seem like atopic dermatitis have not been reported in association with CALs. We intended to evaluate the risk of development of CALs in patients with two different phenotypes. Materials and methods: We retrospectively reviewed the medical records in 220 patients who diagnosed as KD and received IVIG treatment in Kyung Hee University Hospital at Gangdong from August 2006 to December 2013. In both patients groups (6 patients with arthritis and 52 patients with eczematoid skin lesion), we reviewed the state of coronary artery, clinical characteristics, associated viral or bacterial infections. Results: In patients with eczematoid lesions (52/220, 23.6%), the ages of patients were significantly older, the duration of fever was longer, and the prevalence of CAL was significantly higher than that of controls ( P =.000, P =.041, P =.033, respectively). In patients with arthritis (6/220, 3%), there were higher incidence of methylprednisolone or infliximab therapy ( P =.000, P =.004, respectively), and higher incidence of viral infection like influenza A and B, rhinovirus, parainfluenza 2, metapneumovirus, and coronavirus OC43 ( P =.018). Conclusions: The incidence of CAL was higher in group of eczematoid skin lesion in KD patients than in group of patients with arthritis. Associated viral infections were higher in arthritis group, therefore, it is better to find associated pathogens aggressively that might be a certain trigger of the development of KD in this group of patients.

Rheumatology ◽  
2018 ◽  
Vol 58 (5) ◽  
pp. 770-775 ◽  
Author(s):  
Jong Gyun Ahn ◽  
Yoonsun Bae ◽  
Dongjik Shin ◽  
Jiho Nam ◽  
Kyu Yeun Kim ◽  
...  

Abstract Objectives Kawasaki disease (KD) is an acute systemic vasculitis of unknown aetiology that affects infants and young children. Recent reports of elevated serum high mobility group box 1 (HMGB1) level during the acute phase of KD and its relationship to poor response to IVIG treatment suggest a possible association of HMGB1 polymorphisms with KD. We investigated the association between the polymorphisms of the HMGB1 gene, KD susceptibility, coronary artery lesions, and KD response to IVIG treatment. Methods Whole genome sequencing of the HMGB1 gene was performed to identify causative variants. Two tagging single nucleotide polymorphisms of the HMGB1 gene were selected using linkage disequilibrium analysis. The tagging single nucleotide polymorphisms were genotyped using the TaqMan Allelic Discrimination assay in a total of 468 subjects (265 KD patients and 203 controls). Results The HMGB1 single nucleotide polymorphisms were not associated with KD susceptibility. However, in KD patients, there was a significant association of rs1412125 with coronary artery lesions formation in the recessive model (GG vs AA + GA: odds ratio = 4.98, 95% CI = 1.69–14.66, P = 0.005). In addition, rs1412125 was associated with IVIG resistance in the recessive (GG vs AA + GA: odds ratio = 4.11, 95% CI = 1.38–12.23, P = 0.017) and allelic models (G vs A: odds ratio = 1.80, 95% CI = 1.06–3.06, P = 0.027). Conclusion The rs1412125 in HMGB1 might be a risk factor for the development of coronary artery lesions and IVIG resistance in KD patients.


2020 ◽  
Author(s):  
I Hsin Tai ◽  
Pei-Lin Wu ◽  
Mindy Ming-Huey Guo ◽  
Jessica Lee ◽  
Chi-Hsiang Chu ◽  
...  

Abstract Background: Kawasaki Disease (KD) is considered a major acquired heart disease in children under the age of 5. Coronary artery lesions (CAL) can occur in serious cases despite extreme therapy efforts. Previous studies have reported low serum albumin level was associated with disease outcome, but no further investigation was addressed yet. Method: This retrospective (case-control) study randomly included children with KD who were admitted and underwent laboratory tests before undergoing IVIG treatment in this institution, the largest tertiary medical center in southern Taiwan from 2012-2016. PNI, an albumin-based formula product, was evaluated as a predictor of CAL the first time. The progression of CAL was monitored using serial echocardiography for six months. We performed multivariable logistic regression analysis on the laboratory test and PNI with the disease outcome of the KD patients. Result: Of the 284 children, 158 had CAL, including transient dilatation, while the other 126 did not develop CAL during the 6-month follow-up period. A multivariate logistic regression model revealed that PNI and platelet count are significant predictors of CAL with a 95% confidence interval estimator of 2.532 (1.394-4.599) and1.004 (1.002-1.006), respectively. Using PNI to predict CAL presence gave an area under the receiver-operating-characteristics (ROC) curve of 0.596, and the PNI cut-off point is taken as 55.24, with a sensitivity of 0.509 and specificity of 0.678. Conclusion: This is the first study to demonstrate that PNI, an albumin-based formula product, is a useful index with clearly cut-off value for predicting CAL formation prior to initial IVIG therapy and thus warn clinicians to adopt aggressive therapeutic and coronary arteries imaging surveillance strategies before CAL can develop.


2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Xuting Zhang ◽  
Yuee He ◽  
Yiping Shao ◽  
Biyao Hang ◽  
Zhipeng Xu ◽  
...  

Abstract Background Coronary artery lesions (CALs) are the most severe complication of Kawasaki disease (KD). Approximately 9–20% of the patients with KD develop CAL despite receiving regular treatment (intravenous immunoglobulin [IVIG] and aspirin). Some patients develop coronary aneurysms, leading to coronary artery stenosis or thrombosis, resulting in ischaemic heart disease and significantly affect the patients’ lives. The purpose of this study was to investigate the factors associated with the duration of CAL in patients with KD. Methods The data of 464 patients with KD and CAL admitted to the Children’s Heart Centre, The Second Affiliated Hospital and Yuying Children’s Hospital from 2010 to 2018 were retrospectively analysed. Demographic and clinical information and echocardiographic follow-up data were collected. Kaplan–Meier curves were used to estimate the overall CAL duration, and the log-rank test was used to compare statistical differences. Univariate and multivariate Cox regression models were used to identify variables related to the CAL duration. Results The median CAL duration was 46 days (95% confidence interval: 41–54 days). CALs were observed in 61.5, 41.5, 33.3, 22.3, 10.3, and 7.7% of the patients at 1 month, 2 months, 3 months, 6 months, 1 year, and 2 years after the onset of KD, respectively. Univariate Cox regression model showed that sex (p = 0.016), rash symptoms (p = 0.035), delayed IVIG treatment (p = 0.022), CAL type (p < 0.001), degree of CAL (p < 0.001), white blood cell count before IVIG treatment (p = 0.019), and platelet count after IVIG treatment (p = 0.003) were statistically significant factors associated with the overall CAL duration. Multivariable Cox regression showed that delayed IVIG treatment (p = 0.020), multiple dilatations (p < 0.001), a greater degree of dilatation (p < 0.001), and higher platelet count after IVIG treatment (p = 0.007) were positively related to CAL duration. Conclusions CAL duration was affected by delayed IVIG treatment, type of CAL, degree of CAL, and platelet count after IVIG treatment. These factors should be monitored carefully during the follow-up and management of patients with KD and CAL.


2016 ◽  
Vol 23 (2) ◽  
pp. 102
Author(s):  
Song Ee Youn ◽  
Hee Young Ju ◽  
Kyung Suk Lee ◽  
Sung Ho Cha ◽  
Mi Young Han ◽  
...  

2020 ◽  
Author(s):  
Wan-Tz Lai ◽  
Ying-Hsien Huang ◽  
Mao-Hung Lo ◽  
Ho-Chang Kuo

Abstract BackgroundKawasaki disease (KD) is a form of systemic febrile vasculitis that can be complicated by coronary artery lesions (CAL). A murine model of KD vasculitis showed that the vasculitis depended on intestinal barrier dysfunction, as well as that the tight junctions maintain the intestinal barrier. In this study, we aimed to investigate the role of tight junction Zonula occludens-1 (Zo-1) in intravenous immunoglobulin (IVIG) treatment response and the occurrence of CAL formation in KD patients.MethodsForty KD patients, 12 healthy controls, and 12 febrile controls were enrolled in this study. Tight junction ZO-1 levels were measured in sera by enzyme-linked immunosorbent assay.ResultsThe serum Zo-1 level was higher in the fever control group but did not achieve statistical significance. Patients who received a second dose of IVIG due to a failure to respond to the initial IVIG treatment had a higher serum tight junction Zo-1 level, but also without statistical significance (p value =0.0582). Patients who developed a coronary artery lesion had a lower serum tight junction Zo-1 level with statistical significance (p value =0.0275).ConclusionsTight junction ZO-1 levels decrease in KD patients with coronary artery lesions and are associated with the intestinal barrier dysfunction of Kawasaki disease and the occurrence of CAL in KD patients.


2012 ◽  
Vol 2012 ◽  
pp. 1-5 ◽  
Author(s):  
Chih-Jen Chen ◽  
Fu-Chen Huang ◽  
Mao-Meng Tiao ◽  
Ying-Hsien Huang ◽  
Li-Yan Lin ◽  
...  

Objective. Kawasaki disease (KD) is an acute systematic vasculitis in children which causes coronary arterial lesions and hydrops of gallbladder. Our objective is to correlate the clinical significance and influence on disease outcome of patients with gallbladder abnormalities in Kawasaki dissease.Methods. Children who met KD diagnosis criteria and were admitted for IVIG treatment were retrospectively enrolled for analysis. Patients with abdominal sonography were divided into 2 groups based on the absence (Group A,N=61) or presence (Group B,N=16) of gallbladder abnormalities (GBA), defined as hydrops or acalculous cholecystitis. Between the two groups, clinical features, demographic data (including admission days, coronary artery lesions, IVIG resistance), and laboratory data before/after IVIG treatment were collected for analysis.Results. The presence of sonographic gallbladder abnormalities is correlated with higher levels of serum CRP, GPT, and neutrophils. It also points to an increased number of IVIG resistance rates in group B. There was no significant statistical difference among clinical features, age, gender, admission days, or coronary artery lesions between the two groups.Conclusion. Sonographic gallbladder abnormalities are associated with higher CRP, GPT, neutrophil and IVIG resistance in KD. It can be used as a predictor of IVIG resistance in patients with KD.


2021 ◽  
Vol 12 ◽  
Author(s):  
Toshiyuki Kitoh ◽  
Tsuyoshi Ohara ◽  
Taichiro Muto ◽  
Akihisa Okumura ◽  
Reizo Baba ◽  
...  

Kawasaki disease (KD) is a febrile disease of childhood characterized by systemic vasculitis that can lead to coronary artery lesions (CAL). This was a prospective cohort study to determine the levels of the pentraxin 3 (PTX3), soluble CD24-Subtype (Presepsin) and N-terminal pro-brain natriuretic peptide (NT-pro BNP) in consecutive KD patients. From January 2013 to March 2015, all patients with KD admitted to Aichi Medical University Hospital who provided consent had their plasma saved before IVIG administration. In total, 97 cases were registered. 22 cases of incomplete KD were excluded from the outcome analysis. The total 75 cases were used for statistical analyses. A PTX3 threshold of &gt;7.92 ng/ml provided a specificity of 88.5 %, a sensitivity of 94.4 %, and a likelihood ratio as high as 15.92 for the diagnosis of KD compared with febrile non-KD controls. Although an echocardiographic diagnosis of CAL in the early course of the disease was confirmed in 24 cases, it was not in the remaining 51 cases. Neither NT-proBNP nor Presepsin had statistical significance for the prediction of the echocardiographic CAL diagnosis. Only PTX3 was significantly predictive of the echocardiographic CAL diagnosis (p=0.01). The PTX3 level was significantly higher in the intravenous immunoglobulin (IVIG) non-responders (45.9±7.45) than in the IVIG responders (17.0 ± 1.46 ng/ml) (p&lt; 0.001). The PTX3 level also correlated with the number of IVIG treatment courses needed to resolve fever (R² =0.64). Persistent CAL (pCAL) formation was observed in three cases; one of aneurysm only and two aneurysms with dilatations. The patients with pCAL had significantly higher PTX3 levels (85 ± 8.4 ng/ml) than patients without pCAL (22 ± 2.2 ng/ml) (p&lt; 0.0001). In terms of pCAL prediction, the area under the curve (AUC) of receiver operating characteristic ROC curve of PTX3 was 0.99, and it was significantly greater than that of Presepsin (0.67) or NT-proBNP (0.75). PTX3 is a soluble pattern recognition molecule that acts as a main component of the innate immune system. These data suggest that PTX3 can be utilized as a definitive biomarker for the prediction of IVIG resistance and subsequent CAL formation in patients with KD.


2019 ◽  
Vol 34 (10) ◽  
pp. 1571-1579
Author(s):  
Wataru Tamaki ◽  
Etsuko Tsuda ◽  
Syuji Hashimoto ◽  
Tamami Toyomasa ◽  
Mikiya Fujieda

Children ◽  
2021 ◽  
Vol 8 (5) ◽  
pp. 317
Author(s):  
Ling-Sai Chang ◽  
Ken-Pen Weng ◽  
Jia-Huei Yan ◽  
Wan-Shan Lo ◽  
Mindy Ming-Huey Guo ◽  
...  

(1) Background: Desquamation is a common characteristic of Kawasaki disease (KD). In this study, we analyzed patients’ varying desquamation levels in their hands or feet, in correlation with clinical presentation, to assess the relationship. (2) Methods: We retrospectively reviewed children with KD. We analyzed their age, laboratory data before intravenous immunoglobulin (IVIG) treatment and coronary artery abnormalities (CAA) based on the desquamation level of their hands and feet. We classified the desquamation level from 0 to 3 and defined high-grade desquamation as grade 2 and 3. (3) Results: We enrolled a total 112 patients in the study. We found the hands’ high-grade desquamation was positively associated with age and segmented neutrophil percentage (p = 0.047 and 0.029, respectively) but negatively associated with lymphocyte and monocyte percentage (p = 0.03 and 0.006, respectively). Meanwhile, the feet’s high-grade desquamation was positively associated with total white blood cell counts (p = 0.033). Furthermore, we found that high-grade hand desquamation had less probability of CAA formation compared with that of a low grade (7.1% vs. 40.8%, p = 0.016). (4) Conclusions: This report is the first to demonstrate that the desquamation level of hands or feet in KD is associated with different coronary artery abnormalities and laboratory findings.


2021 ◽  
Vol 13 (1) ◽  
pp. 12
Author(s):  
A. Boutaleb ◽  
D. Hamraoui ◽  
K. Bouayed

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