scholarly journals Long-Term Hypermethylation of FcγR2B in Leukocytes of Patients with Kawasaki Disease

2021 ◽  
Vol 10 (11) ◽  
pp. 2347
Author(s):  
Ling-Sai Chang ◽  
Hong-Ren Yu ◽  
Chiao-Lun Chu ◽  
Kuang-Den Chen ◽  
Ying-Hsien Huang ◽  
...  
Keyword(s):  
Kawasaki Disease ◽  
Dynamic Change ◽  
Disease Onset ◽  
Ivig Treatment ◽  
Gamma Receptor ◽  
Activating Receptors ◽  
Resistant Group ◽  
One Year ◽  
Whole Blood Cells

The Fc gamma receptor family contains several activating receptors and the only inhibitory receptor, FcγR2B. In this study, we investigated the dynamic methylation change of FcγR2B in different stages of Kawasaki disease (KD). We enrolled a total of 116 participants, which included patients with febrile diseases as controls and KD patients. Whole blood cells of KD patients were collected prior to intravenous immunoglobulin (IVIG) treatment (KD1), three to seven days after IVIG (KD2), three weeks after IVIG treatment (KD3), six months after IVIG (KD4), and one year after IVIG treatment (KD5). In total, 76 KD patients provided samples in every stage. Leukocytes of controls were also recruited. We performed DNA extraction and pyrosequencing. FcγR2B methylation levels were higher in KD3 compared to both the controls and KD1. A significantly higher methylation of FcγR2B was found in KD5 when compared with KD1. FcγR2B methylation levels in the IVIG-resistant group were lower than those in the IVIG-responsive group at KD1-3 (p = 0.004, 0.004, 0.005 respectively). This study is the first to report the dynamic change of FcγR2B methylation and to demonstrate long-term hypermethylation one year after disease onset. Hypomethylation of FcγR2B is associated with IVIG resistance.

scholarly journals KAWASAKI DISEASE IN INFANTS LESS THAN ONE YEAR OF AGE: AN ITALIAN COHORT FROM A SINGLE CENTER

2019 ◽  
Author(s):  
Greta Mastrangelo ◽  
Rolando Cimaz ◽  
Giovan Battista Calabri ◽  
Gabriele Simonini ◽  
Donatella Lasagni ◽  
...  
Keyword(s):  
Kawasaki Disease ◽  
Severe Disease ◽  
Disease Onset ◽  
Retrospective Chart Review ◽  
Ivig Treatment ◽  
New Finding ◽  
Mucosal Changes ◽  
One Year ◽  
Few Data ◽  
Italian Cohort

Abstract Background and aims Few data are currently available for Kawasaki disease (KD) below 12 months especially in Caucasians. This study aims to analyze clinical and laboratory features of KD among an Italian cohort of infants. Methods A retrospective chart review of KD children aged less than one year at time of disease onset between January 2008-December 2017 was performed. Clinical data, laboratory parameters, instrumental findings, treatment and outcome were collected in a customized database. Results Among 113 KD patients, 32 (28.3%) were younger than 1 year. Nineteen patients aged below 6 months, and three below 3 months. The median age was 5.7 ±2.7 months. The mean time to diagnosis was 7±3 days and was longer in the incomplete forms (8 ± 4 vs 6 ± 1 days). Conjunctival injection was present in 26 patients (81.2%); rash in 25 (78.1%); extremity changes in 18 (56.2%); mucosal changes in 13 (40.6%,) and lymphadenopathy only in 7 (21.8%). Mucosal changes were the least common features in incomplete forms (18.2%). Twenty-two patients (68.7%) had incomplete KD. Nineteen (59.4%) had cardiac involvement, of whom 13 (59.0%) had incomplete form. ESR, PCR and platelet values were higher in complete KD; especially, ESR resulted significantly higher in complete forms (80 ± 25.7 mm/h vs 50 ± 28.6 mm/h; p = 0.01). Conversely, AST level was statistically significant higher in patients with incomplete forms (95.4 ± 132.7 UI/L vs 29.8 ± 13.2 UI/L; p = 0.03). All patients received IVIG. Response was reported in 26/32 patients; 6 cases needed a second dose of IVIG and one required a dose of anakinra. Conclusion In our cohort, incomplete disease was commonly found, resulting in delayed diagnoses and poor cardiac prognosis. Infants with incomplete KD seem to have a more severe disease and a greater predilection for coronary involvement than those with complete KD. AST was significantly higher in incomplete forms, thus AST levels might be a new finding in incomplete forms’ diagnosis. Eventually, we highlight a higher resistance to IVIG treatment. To our knowledge this is the first study involving an Italian cohort of patients with KD below 12 months.

scholarly journals KAWASAKI DISEASE IN INFANTS LESS THAN ONE YEAR OF AGE: AN ITALIAN COHORT FROM A SINGLE CENTER

2019 ◽  
Author(s):  
Greta Mastrangelo ◽  
Rolando Cimaz ◽  
Giovan Battista Calabri ◽  
Gabriele Simonini ◽  
Donatella Lasagni ◽  
...  
Keyword(s):  
Kawasaki Disease ◽  
Severe Disease ◽  
Disease Onset ◽  
Retrospective Chart Review ◽  
Ivig Treatment ◽  
New Finding ◽  
Mucosal Changes ◽  
One Year ◽  
Few Data ◽  
Italian Cohort

Abstract Background and aims Few data are currently available for Kawasaki disease (KD) below 12 months especially in Caucasians. This study aims to analyze clinical and laboratory features of KD among an Italian cohort of infants. Methods A retrospective chart review of KD children aged less than one year at time of disease onset between January 2008-December 2017 was performed. Clinical data, laboratory parameters, instrumental findings, treatment and outcome were collected in a customized database. Results Among 113 KD patients, 32 (28.3%) were younger than 1 year. Nineteen patients aged below 6 months, and three below 3 months. The median age was 5.7 ±2.7 months. The mean time to diagnosis was 7±3 days and was longer in the incomplete forms (8 ± 4 vs 6 ± 1 days). Conjunctival injection was present in 26 patients (81.2%); rash in 25 (78.1%); extremity changes in 18 (56.2%); mucosal changes in 13 (40.6%,) and lymphadenopathy only in 7 (21.8%). Mucosal changes were the least common features in incomplete forms (18.2%). Twenty-two patients (68.7%) had incomplete KD. Nineteen (59.4%) had cardiac involvement, of whom 13 (59.0%) had incomplete form. ESR, PCR and platelet values were higher in complete KD; especially, ESR resulted significantly higher in complete forms (80 ± 25.7 mm/h vs 50 ± 28.6 mm/h; p = 0.01). Conversely, AST level was statistically significant higher in patients with incomplete forms (95.4 ± 132.7 UI/L vs 29.8 ± 13.2 UI/L; p = 0.03). All patients received IVIG. Response was reported in 26/32 patients; 6 cases needed a second dose of IVIG and one required a dose of anakinra. Conclusion In our cohort, incomplete disease was commonly found, resulting in delayed diagnoses and poor cardiac prognosis. Infants with incomplete KD seem to have a more severe disease and a greater predilection for coronary involvement than those with complete KD. AST was significantly higher in incomplete forms, thus AST levels might be a new finding in incomplete forms’ diagnosis. Eventually, we highlight a higher resistance to IVIG treatment. To our knowledge this is the first study involving an Italian cohort of patients with KD below 12 months.

scholarly journals Kawasaki Disease In Infants Less Than One Year Of Age: An Italian Cohort From A Single Center

2019 ◽  
Author(s):  
Greta Mastrangelo ◽  
Rolando Cimaz ◽  
Giovan Battista Calabri ◽  
Gabriele Simonini ◽  
Donatella Lasagni ◽  
...  
Keyword(s):  
Kawasaki Disease ◽  
Severe Disease ◽  
Disease Onset ◽  
Retrospective Chart Review ◽  
Ivig Treatment ◽  
New Finding ◽  
Mucosal Changes ◽  
One Year ◽  
Few Data ◽  
Italian Cohort

Abstract Background and aims Few data are currently available for Kawasaki disease (KD) below 12 months especially in Caucasians. This study aims to analyze clinical and laboratory features of KD among an Italian cohort of infants. Methods A retrospective chart review of KD children aged less than one year at time of disease onset between January 2008-December 2017 was performed. Clinical data, laboratory parameters, instrumental findings, treatment and outcome were collected in a customized database. Results Among 113 KD patients, 32 (28.3%) were younger than 1 year. Nineteen patients aged below 6 months, and three below 3 months. The median age was 5.7 ±2.7 months. The mean time to diagnosis was 7±3 days and was longer in the incomplete forms (8 ± 4 vs 6 ± 1 days). Conjunctival injection was present in 26 patients (81.2%); rash in 25 (78.1%); extremity changes in 18 (56.2%); mucosal changes in 13 (40.6%,) and lymphadenopathy only in 7 (21.8%). Mucosal changes were the least common features in incomplete forms (18.2%). Twenty-two patients (68.7%) had incomplete KD. Nineteen (59.4%) had cardiac involvement, of whom 13 (59.0%) had incomplete form. ESR, PCR and platelet values were higher in complete KD; especially, ESR resulted significantly higher in complete forms (80 ± 25.7 mm/h vs 50 ± 28.6 mm/h; p = 0.01). Conversely, AST level was statistically significant higher in patients with incomplete forms (95.4 ± 132.7 UI/L vs 29.8 ± 13.2 UI/L; p = 0.03). All patients received IVIG. Response was reported in 26/32 patients; 6 cases needed a second dose of IVIG and one required a dose of anakinra. Conclusion In our cohort, incomplete disease was commonly found, resulting in delayed diagnoses and poor cardiac prognosis. Infants with incomplete KD seem to have a more severe disease and a greater predilection for coronary involvement than those with complete KD. AST was significantly higher in incomplete forms, thus AST levels might be a new finding in incomplete forms’ diagnosis. Eventually, we highlight a higher resistance to IVIG treatment. To our knowledge this is the first study involving an Italian cohort of patients with KD below 12 months.

scholarly journals KAWASAKI DISEASE IN INFANTS LESS THAN ONE YEAR OF AGE: AN ITALIAN COHORT FROM A SINGLE CENTER

2019 ◽  
Author(s):  
Greta Mastrangelo ◽  
Rolando Cimaz ◽  
Giovan Battista Calabri ◽  
Gabriele Simonini ◽  
Donatella Lasagni ◽  
...  
Keyword(s):  
Kawasaki Disease ◽  
Severe Disease ◽  
Disease Onset ◽  
Retrospective Chart Review ◽  
Ivig Treatment ◽  
New Finding ◽  
Mucosal Changes ◽  
One Year ◽  
Few Data ◽  
Italian Cohort

Abstract Background and aims Few data are currently available for Kawasaki disease (KD) below 12 months especially in Caucasians. This study aims to analyze clinical and laboratory features of KD among an Italian cohort of infants. Methods A retrospective chart review of KD children aged less than one year at time of disease onset between January 2008-December 2017 was performed. Clinical data, laboratory parameters, instrumental findings, treatment and outcome were collected in a customized database. Results Among 113 KD patients, 32 (28.3%) were younger than 1 year. Nineteen patients aged below 6 months, and three below 3 months. The median age was 5.7 ±2.7 months. The mean time to diagnosis was 7±3 days and was longer in the incomplete forms (8 ± 4 vs 6 ± 1 days). Conjunctival injection was present in 26 patients (81.2%); rash in 25 (78.1%); extremity changes in 18 (56.2%); mucosal changes in 13 (40.6%,) and lymphadenopathy only in 7 (21.8%). Mucosal changes were the least common features in incomplete forms (18.2%). Twenty-two patients (68.7%) had incomplete KD. Nineteen (59.4%) had cardiac involvement, of whom 13 (59.0%) had incomplete form. ESR, PCR and platelet values were higher in complete KD; especially, ESR resulted significantly higher in complete forms (80 ± 25.7 mm/h vs 50 ± 28.6 mm/h; p = 0.01). Conversely, AST level was statistically significant higher in patients with incomplete forms (95.4 ± 132.7 UI/L vs 29.8 ± 13.2 UI/L; p = 0.03). All patients received IVIG. Response was reported in 26/32 patients; 6 cases needed a second dose of IVIG and one required a dose of anakinra. Conclusion In our cohort, incomplete disease was commonly found, resulting in delayed diagnoses and poor cardiac prognosis. Infants with incomplete KD seem to have a more severe disease and a greater predilection for coronary involvement than those with complete KD. AST was significantly higher in incomplete forms, thus AST levels might be a new finding in incomplete forms’ diagnosis. Eventually, we highlight a higher resistance to IVIG treatment. To our knowledge this is the first study involving an Italian cohort of patients with KD below 12 months.

Abstract O.73: Long-term Results Of Percutaneous Transluminal Coronary Rotational Atherectomy For Localized Stenosis Caused By Kawasaki Disease

Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
Etsuko Tsuda ◽  
Shinsuke Hoshino ◽  
Yasuhide Asaumi ◽  
Yosuke Hayama ◽  
Osamu Yamada
Keyword(s):  
Kawasaki Disease ◽  
Graft Patency ◽  
Rotational Atherectomy ◽  
Long Term Results ◽  
Cardiac Events ◽  
Main Trunk ◽  
One Year ◽  
Percutaneous Transluminal ◽  
Localized Stenosis

We report the results of percutaneous transluminal coronary rotational atherectomy (PTCRA) for localized stenosis caused by Kawasaki disease (KD). Thirteen male and a female, aged 5 to 29 years (median 13 years), underwent PTCRA and the interval from the PTARA to the latest angiogram ranged from 3 months to 16 years (median 6 years). The target vessels were the left anterior descending artery (3 patients), the left circumflex (2), left main trunk (2) and the right coronary artery (7). The immediate results of PTCRA were successful in all patients, and the mean stenosis degree improved from 86 ± 11% to 36 ± 13%. Five cardiac events occurred within one year (acute myocardial infarction 2, transient complete atrioventricular block 1 and re-PTCRA 2). The survival rate and cardiac event free rate at 15 years after PTCRA were 93% and 71%, respectively. For the graft patency, 4 pts who underwent PTCRA within 10 yeas old, had asymptomatic occlusion within 1 year. The patency rate at 15 years after PTCRA was 69%, in 10 pts who underwent it more than 10 years old. Cardiac events and restenosis occurred within a year after PTCRA. The results in patients less than 10 years old was poor. If a graft is patent in one year after procedure, long-term patency may be expected in patients more than 10 years old.

scholarly journals Prediction of Repeated Intravenous Immunoglobulin Resistance in Children With Kawasaki Disease

2021 ◽  
Author(s):  
Yaheng Lu ◽  
Tingting Chen ◽  
Yizhou Wen ◽  
Feifei Si ◽  
Xindan Wu ◽  
...  
Keyword(s):  
Kawasaki Disease ◽  
Intravenous Immunoglobulin ◽  
Multiple Logistic Regression Analysis ◽  
Laboratory Data ◽  
Ivig Treatment ◽  
Roc Curve Analysis ◽  
Ivig Resistance ◽  
Intravenous Immunoglobulin Resistance ◽  
Resistant Group ◽  
Resistance Prediction

Abstract Background: Repeated intravenous immunoglobulin (IVIG) resistance prediction is one of the pivotal topics in Kawasaki disease (KD). Those non-responders of repeated IVIG treatment might be improved by an early-intensified therapy to reduce coronary artery lesion and medical costs. This study investigated predictors of resistance to repeated IVIG treatment in KD.Methods: A total of 94 children with IVIG-resistant KD treated at our hospital between January 2016 and August 2020 were retrospectively analyzed. According to the therapeutic effect of a second dose IVIG treatment, the children were divided into repeated IVIG-responsive group and repeated IVIG-resistant group, and the clinical and laboratory data were compared. Predictors of repeated IVIG resistance and the optimal cut-off value were determined by multiple logistic regression analysis and receiver operating characteristic (ROC) curve analysis.Results: The laboratory data of the percentage of neutrophils (N%) and levels of serum procalcitonin (PCT), N-terminal pro-brain natriuretic peptide (NT-proBNP) on admission were significantly higher in repeated IVIG-resistant group compared with repeated IVIG-responsive group, while levels of serum sodium (Na+) and albumin (ALB) were significantly lower (P<0.05). The clinic data showed no significant differences between the two groups. PCT exhibited the largest AUC (0.751) in predicting repeated IVIG resistance in KD compared with N%, Na+, ALB, and NT-proBNP. PCT>1.81ng/ml was an independent predictor of repeated IVIG resistance in KD (OR 4.161, 95% CI 1.441~12.017, P=0.008). Conclusions: Our study illustrates the serum PCT level before initial IVIG treatment could be used to predict repeated IVIG resistance in KD.

scholarly journals Prediction of repeated intravenous immunoglobulin resistance in children with Kawasaki disease

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Yaheng Lu ◽  
Tingting Chen ◽  
Yizhou Wen ◽  
Feifei Si ◽  
Xindan Wu ◽  
...  
Keyword(s):  
Kawasaki Disease ◽  
Intravenous Immunoglobulin ◽  
Multiple Logistic Regression Analysis ◽  
Laboratory Data ◽  
Ivig Treatment ◽  
Roc Curve Analysis ◽  
Reactive Protein ◽  
Ivig Resistance ◽  
Intravenous Immunoglobulin Resistance ◽  
Resistant Group

Abstract Background Repeated intravenous immunoglobulin (IVIG) resistance prediction is one of the pivotal topics in Kawasaki disease (KD). Those non-responders of repeated IVIG treatment might be improved by an early-intensified therapy to reduce coronary artery lesion and medical costs. This study investigated predictors of resistance to repeated IVIG treatment in KD. Methods A total of 94 children with IVIG-resistant KD treated at our hospital between January 2016 and August 2020 were retrospectively analyzed. According to the therapeutic effect of a second dose IVIG treatment, the children were divided into repeated IVIG-responsive group and repeated IVIG-resistant group, and the clinical and laboratory data were compared. Predictors of repeated IVIG resistance and the optimal cut-off value were determined by multiple logistic regression analysis and receiver operating characteristic (ROC) curve analysis. Results The Pre-IVIG laboratory data showed the percentage of neutrophils (N%) and levels of serum procalcitonin (PCT), N-terminal pro-brain natriuretic peptide (NT-proBNP) were significantly higher in repeated IVIG-resistant group compared with repeated IVIG-responsive group, while levels of serum sodium and albumin (ALB) were significantly lower (P < 0.05). The post-IVIG laboratory values of N% and C-reactive protein (CRP) were significantly higher in the repeated IVIG-resistant group compared with repeated IVIG-responsive group, while hemoglobin and ALB were lower (P < 0.05). Pre-IVIG PCT and post-IVIG CRP exhibited AUC of 0.751 and 0.778 respectively in predicting repeated IVIG resistance in KD. Pre-IVIG PCT > 1.81ng/ml (OR 4.1, 95 % CI 1.4 ~ 12.0, P < 0.05) and post-IVIG CRP > 45 mg/L (OR 4.6, 95 % CI 1.3 ~ 16.2, P < 0.05) were independent predictors of repeated IVIG resistance in KD. Conclusions Our study illustrates the serum PCT level before initial IVIG treatment and CRP after initial IVIG could be used to predict repeated IVIG resistance in KD.

scholarly journals Prediction of Resistant to Intravenous Immunoglobulin (IVIG) Treatment in Patients with Kawasaki Disease in the Tertiary Care Hospital.

2021 ◽  
Author(s):  
Sarinrath Charoonrochana ◽  
Kittipol Klaiklueng ◽  
Rattawanlop Somanundana
Keyword(s):  
Kawasaki Disease ◽  
Serum Albumin ◽  
Predictive Factors ◽  
Serum Sodium ◽  
Scoring System ◽  
Study Patient ◽  
Ivig Treatment ◽  
P Value ◽  
Close Monitoring ◽  
Resistant Group

Abstract Background: Kawasaki disease (KD)is an acute autoimmune systemic vasculitis disease that mainly occurs in infancy and younger children. It has the potential to cause coronary artery lesions (CALs) develop in up to 20-25% of patients with untreated KD caused morbidity and mortality in children worldwide especially in Asia. Objective: To determine the predictive factors of IVIG resistant Kawasaki disease in Thailand Method: A retrospective descriptive study. Patient charts of all patients who had newly diagnosed of KD in Bhumibol Adulyadej Hospital, Thailand between January 2010 and December 2020 were reviewed. The protocol was approved by the ethic review committee. Results: A total of 102 subjects met the inclusion criteria and were enrolled into the study, including 17 cases of IVIG resistant (17/97, 28.5%) and 80 cases of IVIG responder (80/97, 71.4%).Demographic and laboratory characteristics were obtained. Male gender has greater in number in IVIG resistant group. Average age is 27 months and IVIG resistant tends to have age below 6 months old. Two variables were significantly lower in IVIG-resistant group than IVIG-responder group, including serum albumin (3.01±0.51vs 3.48±0.49 g/dL, p-value = 0 . 01) and serum sodium (133.24±3.43 vs 135.79±3.19 mEq/L, p-value = 0.01) meanwhile age at time of diagnosis less than 6 months (29.4% vs 8.75%, p-value = 0.02) and serum ALT (78.29±74.19 versus 71.01±80.19 IU/L, p-value = 0.02) were significantly higher in IVIG-resistant group than IVIG-responder group. Conclusion: The predictive factors of our study by univariate analysis included age below 6 months, serum albumin <3.5g/dL, serum sodium <135mmol / L and high serum ALT≥45 IU/L. The principal findings in our predictive model is the scoring system for predicting IVIG-resistant patients in Thai population, the scoring system includes total peripheral WBC count >18,000 mm 3 . (1 point), serum albumin < 3.5 g/dl (1 point) and serum ALT ≥ 45 IU/L (1.5 points), and a total score equal 2 points and higher yielded a sensitivity and a specificity of 82.35% and 62.50%, respectively for IVIG resistance prediction, who require more close monitoring which may help physicians make more rational decisions regarding an initial treatment of KD and be the candidates for additional therapies. Our study is similar to previous studies in Japan, a risk scoring system prior to administrate IVIG.

Abstract 95: Increased Circulating Endothelial Microparticles In The Acute Phase Of Kawasaki Disease

Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
Hideyuki Nakaoka
Keyword(s):  
Endothelial Dysfunction ◽  
Kawasaki Disease ◽  
Initial Treatment ◽  
Systemic Vasculitis ◽  
Disease Onset ◽  
Ivig Treatment ◽  
Healthy Children ◽  
Ivig Infusion ◽  
Endothelial Microparticles ◽  
Coronary Syndrome

Backgroud: Kawasaki disease (KD) is a typically acute inflammatory syndrome that takes the form of systemic vasculitis. The acute inflammation and subsequent reparative process may lead to lasting changes in arterial structure even in the convalescence of KD including increased endothelial dysfunction. Endothelial microparticles (EMPs) are vesicles formed by the cell membrane after endothelial activation, and their composition can be used to characterize the status of the parent endothelial cell. EMPs were reported in cardiovascular diseases with endothelial dysfunction, such as acute coronary syndrome, pulmonary hypertension, diabetes, and vasculitis. Our aim of this study is to elucidate whether EMPs are involved in vasculitis during acute stage of Kawasaki disease. Method: We enrolled 9 patients (aged 3 months to 14 years, 6 male, 3 female), 7 common febrile children and 5 healthy children. KD patients in the convalescent phase were divided into two subgroups; coronary artery lesion (CAL, n=2) and no coronary lesion (NCAL, n=7). Blood samples were collected at the time of diagnosis before the initiation of IVIG treatment, then immediately after the first IVIG infusion and at 2-4 weeks after disease onset. Samples were measured using flow cytometry. Result: The percentage counts of EMPs were 2.90±1.26% in KD children before initial treatment, which were significantly higher (P<0.005) than those of disease controls (0.12±0.13%) and healthy controls (0.09±0.08%) before initial treatment, and reached normal levels within 4 weeks (0.05±0.05%). The highest percentage count of EMPs (5.47%) was observed in the patient with CAL before initial treatment. Further, prolonged high percentage count of EMPs (3.34%) was recognized in the patient with multiple gaint aneurysms at 2 weeks after onset. Conclusion: The relation between the increased levels of EMPs and the involvement of CAL may suggest that EMPs could serve as a sensitive marker of the severity of endothelial dysfunction and vasculitis in patients with KD. Although the function of EMPs has not been fully elucidated, there is evidence that it plays an important role for distinct inflammatory reactions in endothelium.

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