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.

Cognitive development of children with Kawasaki Disease and the parenting stress of their caregivers

2020 ◽  
Author(s):  
Liang-Jen Wang ◽  
Zi-Yu Tsai ◽  
Ling-Sai Chang ◽  
Ho-Chang Kuo
Keyword(s):  
Cognitive Function ◽  
Kawasaki Disease ◽  
Parenting Stress ◽  
Short Form ◽  
Systemic Vasculitis ◽  
Stress Index ◽  
Healthy Children ◽  
Ivig Infusion ◽  
Cross Sectional ◽  
Developmental Index

Abstract Background Kawasaki disease (KD) is an acute form of febrile vasculitis that occurs in early childhood. The multi-systemic vasculitis common in KD patients may influence blood perfusion in the brain, and thus caregivers of KD children may feel stress with regard to caring for them. Intravenous immunoglobulin (IVIG) infusion is the standard treatment for acute KD, and the most serious complication of KD is coronary artery aneurysms (CAL). This study aimed to investigate the relationships between KD heterogeneity and the risk of patients’ cognitive impairment or caregivers’ parenting stress. Methods This cross-sectional study consisted of 176 patients with KD (mean age: 5.5 years, 60.8% male) and 85 healthy children (mean age: 6.4 years, 54.1% male). Based on the children’s age, each KD patient and control subject was administered an assessment using the Mullen Scales of Early Learning or the Wechsler Intelligence Scale, and parenting function of their caregivers was assessed using the Parenting Stress Index-Short Form (PSI). Results We observed no significant differences in any developmental index, cognitive function, or parenting stress between KD patients and controls. Among the KD children, IVIG administration nor CAL was associated with children’s cognitive scores. However, the caregivers of patients who had CAL suffered from greater PSI total scores than those of patients without CAL (p = 0.019). Furthermore, the caregivers who had education levels of a master’s degree or above showed less parenting stress than those who had an education level of college (p = 0.010) or lower (p = 0.021). Conclusion No significant differences in developmental index, cognitive function or parenting stress between KD patients and controls. In KD patients, neither IVIG response nor CAL appear to have a relationship with development milestones or cognitive function. However, caregivers’ education is associated to parenting stress, and caregivers of KD patients who developed CAL may feel stress about the unpredictable sequela caused by CAL for their children. Such caregivers may require support to fulfill their parenting roles.

scholarly journals Association of PECAM-1 Gene Polymorphisms with Kawasaki Disease in Chinese Children

2017 ◽  
Vol 2017 ◽  
pp. 1-6 ◽  
Author(s):  
Zhuoying Li ◽  
Dong Han ◽  
Jie Jiang ◽  
Jia Chen ◽  
Lang Tian ◽  
...  
Keyword(s):  
Coronary Artery Disease ◽  
Coronary Artery ◽  
Kawasaki Disease ◽  
Gene Polymorphisms ◽  
Cell Adhesion Molecule ◽  
Systemic Vasculitis ◽  
Healthy Children ◽  
Coronary Artery Lesions ◽  
Artery Disease ◽  
The Relationship

Kawasaki disease (KD) is an acute systemic vasculitis complicated by development of coronary artery lesions. PECAM-1 is a kind of cell adhesion molecule, which plays an important role in coronary artery disease. The relationship between PECAM-1 gene polymorphisms and their susceptibility to Kawasaki diseases (KD) is still unclear. In our study, we examined the PECAM-1 gene polymorphisms in 44 KD patients and 59 healthy children and revealed the correlation of PECAM-1 gene polymorphisms in KD children with and without coronary artery lesions (CAL).

scholarly journals Clinical Implications of Procalcitonin in Kawasaki Disease: A Useful Candidate For Differentiating From Sepsis and Evaluating IVIG Responsiveness

2021 ◽  
Author(s):  
Man Man Niu ◽  
Qi Jiang ◽  
Jin Wei Ruan ◽  
Hui Hui Liu ◽  
Wei Xia Chen ◽  
...  
Keyword(s):  
Kawasaki Disease ◽  
Clinical Implications ◽  
Healthy Children ◽  
C Reactive Protein ◽  
Significant Elevation ◽  
Ivig Infusion ◽  
Blood Samples ◽  
Reactive Protein ◽  
Cell Counts ◽  
Childhood Vasculitis

Abstract Objective Kawasaki disease (KD) is a common childhood vasculitis absent of the specific laboratory definitions, besides a significant elevation in several inflammatory mediators, such as procalcitonin (PCT). However, whether PCT can serve as a useful candidate for differentiating KD from sepsis, and even for predicting incomplete KD, intravenous immunoglobulin (IVIG) nonrespondsiveness and coronary artery abnormalities (CAAs) remains unclear.Methods 254 Chinese KD children were enrolled and divided into 6 subgroups, including complete KD, incomplete KD, IVIG-responsive KD, IVIG-nonresponsive KD, KD with CAAs and KD without CAAs. Blood samples were collected from all subjects within 24-h pre- and 48-h post-IVIG infusion, respectively. PCT, C-reactive protein, sedimentation rate and blood cell counts were detected. In addition, both 261 sepsis children and 251 healthy children sex- and age-matched with KD children were enrolled in the same period.Results (1) PCT experienced the highest increase in sepsis patients before antibiotic therapy, followed by acute KD patients and the healthy controls. (2) The proportion of KD patients with a PCT concentration below 0.25 ng/ml was 11 folds higher than that of sepsis patients. (3) PCT had a sensitivity of 91.7% and a specificity of 30.3% at a cut-off value of >0.15 ng/ml to predict IVIG nonresponsiveness, and the proportion of IVIG-nonresponders with a PCT concentration of 0.25-0.50 ng/ml was 2 folds higher than that of IVIG-responders. Conclusions The PCT concentrations below 0.25 ng/ml may be useful for discriminating KD from sepsis, and moreover, the PCT concentrations of 0.25-0.50 ng/ml may be helpful in predicting IVIG nonresponsiveness.

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 AB1002 IS KAWASAKI DISEASE OR SYSTEMIC JUVENILE IDIOPATHIC ARTHRITIS IN CHILDREN WITH FEVER WITHOUT A SOURCE?

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1794.2-1794
Author(s):  
B. Sözeri ◽  
F. Demir ◽  
T. Merter ◽  
M. Karacan
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Kawasaki Disease ◽  
Clinical Features ◽  
Systemic Vasculitis ◽  
Systemic Juvenile Idiopathic Arthritis ◽  
Clinical Feature ◽  
Unknown Etiology ◽  
Ivig Treatment ◽  
Factors Affecting ◽  
The Mean

Background:Fever without a source (FWS) is caused by various diseases, making differential diagnosis difficult. Clinical similarities between Kawasaki disease (KD) and systemic Juvenile Idiopathic Arthritis (sJIA) are well known. Kawasaki disease (KD), a self-limiting systemic vasculitis, remains of unknown etiology and can cause irreversible coronary artery aneurysms (CAAs). SoJIA is sometimes confused with incomplete KD because both diseases have overlapping clinical features and can be accompanied with CAAs and/or SJIA with macrophage activation syndrome (MAS).Objectives:In this study, the frequency of both KD and SJIA among the patients evaluated with FWS and the clinical features of patients diagnosed with Kawasaki disease.Methods:Medical records of patients who first visited our department between January 2016 and December 2019 were reviewedResults:A total of 107 patients were enrolled in this study, including 43 patients (40.2%, 23 males) who fulfilled the criteria of Kawasaki disease and 64 patients (59.8%, 39 males) who did not fulfill them. In patients who fulfilled the criteria of classical FWS, 36(33.6%, 20 males) patients were diagnosed with systemic juvenile idiopathic arthritis. The mean age of the patients with Kawasaki disease was 30.0±20,4 months (median 25 months), the mean age of other patients was 52,6±40 months (median 39,5 months). The mean age of the patients with sJIA patients was 87,6±49,8 (median 80months). Kawasaki patients were younger than others (p=0.01). There was no difference in gender between groups.In Kawasaki patients, the most common clinical feature at diagnosis was fever (100%) followed by conjunctival congestion and mucosal changes (69%). The last two findings are more significant in kawasaki patients than others (p<0,00). Twenty-six (59%) patients had completed KD while 25% had incomplete KD. 7 (16%) patients had atypical KD. The mean fever duration was longer in sJIA patients than KD and others (median 14,8 and 7 days, p<0.00). All patients with KD received IVIG (2 g/kg, infusion in 12 h) and aspirin (60 mg/kg/day). 13.6% of the patients also received oral corticosteroids because of IVIG resistance. Thirty-one patients (72.1%) responded to IVIG treatment, whereas 12 (6 female, 6 male) were IVIG resistant. CAI was detected in echocardiography at diagnosis in 10 (22.7%) (6 female; 4 male) patients. We also detected 4 patients pericarditis with /without CIA.Conclusion:The clinical presentations of KD and sJIA are quite similar with fever, rash, hepatomegaly, and lymphadenopathy. All 2 entities may provide clues to potentially shared immunopathology.References:[1]Arslanoglu Aydin E et al. The factors affecting the disease course in Kawasaki disease. Rheumatol Int. 2019 Aug;39(8):1343-1349[2]Dong S et al. Diagnosis of systemic-onset juvenile idiopathic arthritis after treatment for presumed Kawasaki disease. J Pediatr. 2015 May;166(5):1283-8.Disclosure of Interests:None declared

scholarly journals Long-term evaluation of endothelial function in Kawasaki disease patients

2012 ◽  
Vol 23 (4) ◽  
pp. 517-522 ◽  
Author(s):  
Fátima F. Pinto ◽  
Sérgio Laranjo ◽  
Filipa Paramés ◽  
Isabel Freitas ◽  
Miguel Mota-Carmo
Keyword(s):  
Risk Factors ◽  
Endothelial Dysfunction ◽  
Kawasaki Disease ◽  
Augmentation Index ◽  
Systemic Vasculitis ◽  
Cardiac Complications ◽  
Control Group ◽  
Reactive Hyperaemia ◽  
Coronary Lesions

AbstractBackgroundKawasaki disease is an acute systemic vasculitis. Cardiac complications are frequent and include endothelial dysfunction in patients with coronary anomalies. So far, the presence of endothelial dysfunction in patients with no coronary lesions has not been demonstrated. Peripheral arterial tonometry (Endo-PAT) measures the microvascular function in response to local ischaemia and has been validated in adult population, but its use in children is scarce.AimTo evaluate endothelial dysfunction in children as a long-term complication after Kawasaki disease using Endo-PAT.MethodsWe evaluated two groups of subjects: (1) Kawasaki disease patients over 11 years of age, diagnosed for >5 years, with no coronary lesions, or any other risk factors for cardiovascular disease; (2) control group of individuals without cardiovascular risk factors. Patients and controls were clinically accessed. Endo-PAT was performed to determine reactive hyperaemia index and augmentation index.ResultsA total of 35 individuals (21 males, age 21 ± 6 years) were evaluated (group 1: 19; controls: 16). Kawasaki disease patients presented significant lower reactive hyperaemia index (1.68 ± 0.49 versus 2.31 ± 0.53; p = 0.001). Augmentation index was similar in both groups (−10 ± 7 versus −11 ± 5; p > 0.005). Most patients with Kawasaki disease disclosed endothelial dysfunction (68%) compared with only 12% in controls.ConclusionsEndo-PAT is feasible and reproducible in the child population. Endothelial dysfunction is a frequent long-term complication in patients after Kawasaki disease with normal appearing coronary arteries. However, these results need validation in a larger population.

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 36: FCGR2A Promoter Methylation and Risks for IVIG Unresponsiveness in Kawasaki Disease

Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
Ho-Chang Kuo ◽  
Kai-Sheng Hsieh ◽  
Wei-Chiao Chang ◽  
Keyword(s):  
Kawasaki Disease ◽  
Promoter Methylation ◽  
Systemic Vasculitis ◽  
Methylation Status ◽  
Cpg Islands ◽  
Gene Promoter ◽  
Ivig Therapy ◽  
Ivig Treatment ◽  
Fc Fragment ◽  
Cpg Sites

Kawasaki disease (KD) is characterized by pediatric systemic vasculitis of an unknown cause and the Fc Fragment of IgG, Low Affinity IIa, Receptor ( FCGR2A ) gene was reported to involve in increasing susceptibility of KD. Because DNA methylation is one of the epigenetic mechanisms that control gene expression, we hypothesized that methylation status of CpG islands in FCGR2A promoter predisposes an individual to Kawasaki disease. We recruited 36 KD patients and 24 healthy subjects with informed consents. And eleven potential methylation loci within the targeted promoter region (chr1:161474603-161475102) of Fc Fragment of IgG, Low Affinity IIa, Receptor were selected for investigation. Methylation at the CpG sites G, H and J displayed a strongly associations with KD, whereas CpG sites B,C,E,F,H,J and K were found to be correlated with non-responsive to IVIG treatment. In addition, CpG sites G, J and K were predicted as the significant transcription factor binding site for NF-kB, Myc-Max and SP2 respectively. Our study reports a significant association between the promoter methylation of FCGR2A , susceptibility of Kawasaki disease and therapeutic outcomes of IVIG treatment. The methylation levels of CpG sites of FCGR2A gene promoter may be an important marker for optimizing IVIG therapy.

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.

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