• Clinical and Experimental Pediatrics
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• v.48 no.4
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• pp.416-424
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• 2005

Purpose : To find the risk factors associated with coronory artery lesions, non-responsiveness to intravenous immunoglobulin(IVIG) treatment, and recurrences in Kawasaki disease patients. Methods : We retrospectively analyzed 1,000 Kawasaki disease patients who were admitted to Yonsei University Medical Center from September 1990 to December 2003. We compared between responder and non-responder groups to IVIG treatment as well as between relapsed and non-relapsed groups, and as to the relapsed group, we also compared variables between patients in their first and second attack states. Finally, factors associated with longer-fever duration from disease onset were evaluated. Results : Longer fever durations before and after IVIG treatment, male sex, lower Hgb and Hct level, higher WBC count and segmented WBC proportion, and higher CRP and Harada's score were related with coronary artery lesions. Non-responsiveness was related to higher WBC count, segmented WBC proportion, CRP, SGPT, Harada's score, and pyuria. Moderate-to-severe coronary artery dilatations and recurrences were more commonly seen among the non-responder group. No significant predictive factors for recurrence were found. In the relapsed group, lower WBC count, CRP, and shorter fever duration from disease onset were observed in their second attack state. Fever duration from disease onset showed positive correlation with WBC count, CRP, and Harada's score and negative correlation with Hgb levels. Conclusion : Higher WBC count, CRP, and higher Harada's score were related to both higher incidences of coronary artery lesions and non-responsiveness to IVIG treatment, and these factors were also related with longer fever duration. Non-responders to IVIG treatment showed higher recurrence rate and more moderate-to-severe coronary artery dilatations than responders.

• Clinical and Experimental Pediatrics
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• v.49 no.12
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• pp.1348-1353
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• 2006

Purpose : The reticuloendothelial system is composed of sinusoidal capillaries, through which even large protein molecules are freely movable between plasma and interstitial space, including the lymphatic system. Therefore, high-dose intravenous immunoglobulin (IVIG) would cause a redistribution of proteins between two compartments. To investigate this hypothesis, we measured plasma protein and lipid levels in patients with Kawasaki disease before and after high-dose IVIG treatment. Methods : Thirty four children with Kawasaki disease who had complete responses to high-dose IVIG treatment (1 g/kg/day for two consecutive days), were analyzed. Before and after the administration of IVIG, serum analyses were performed for such parameters as total protein, albumin, ${\gamma}$-globulins (IgG, IgM, IgA), ${\alpha}1-$, ${\alpha}2-$, and ${\beta}-$ globulin fractions, and lipid profiles (total cholesterol, HDL-cholesterol, LDL-cholesterol and triglyceride). Results : The levels of ${\gamma}$-globulins including IgG, IgM, IgA were significantly increased, and IgG was increased by $1,779{\pm}304mg/dL$ after two-dose of IVIG infusion. The levels of albumin, ${\alpha}1-$, ${\alpha}2-$, and ${\beta}$ globulin fractions were significantly decreased by 18 percent, 24 percent, 19 percent and 12 percent, respectively. HDL-cholesterol level was significantly decreased by 20 percent, while LDL-cholesterol and triglyceride levels were significantly increased by 21 percent and 50 percent, respectively. The total cholesterol level was not changed. Conclusion : High-dose IVIG treatment decreased the levels of a variety of proteins except immunoglobulins, and the increase of IgG after IVIG treatment was lower than expected. Our results suggest that a part of infused IVIG and plasma proteins, including etiologic proteins for Kawasaki disease, may be distributed to the extravascular compartments. The rapid improvement of symptoms induced by IVIG in Kawasaki disease might be explained by this mode of action of IVIG.

• Clinical and Experimental Pediatrics
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• v.48 no.10
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• pp.1139-1142
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• 2005

Purpose : The aim of this study was to investigate the epidemiologic and clinical profiles of Kawasaki disease(KD) in children 8 years of age and older. Methods : For the epidemiologic study of KD in Korea, data of total 15,692 KD patients were collected from 1994 to 2002 on a 3 year basis, by the retrospective survey. Among them, data of 211 KD patients 8 years of age and older were analyzed and compared with those of the KD patients less than 8 years of age. Results : 211 patients 8 years of age and older(1.3% of total KD patients) with the mean age of 9.8 years, included 142 cases aged 8-10 years, 42 cases aged 10-12 years, and 27 cases aged 12 years and older. The male-to-female ratio was 1.9 : 1, the recurrence rate was 3.8%, and the prevalence of cases in sibling was zero. Coronary arterial(CA) abnormalities were detected by echocardiography in 25.4% of the cases, including 7.6% of CA aneurysm cases. There was 1 case complicated by myocardial infarction. Conclusion : Comparing with the data of the KD patients less than 8 years of age, data of 211 patients 8 years of age and older showed significantly higher incidences of CA abnormalities(25.4% : 19.8%) and CA aneurysms(7.6% : 4.0%).

• Clinical and Experimental Pediatrics
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• v.52 no.1
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• pp.87-92
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• 2009

Purpose : Many gene polymorphisms are associated with coronary artery abnormalities in Kawasaki disease. Catechol-O- methyltransferase (COMT) plays an important role in the metabolism of catecholamines, catechol estrogen, and catechol drugs. Polymorphisms of the COMT gene are reported to be associated with myocardial infarction and coronary artery abnormalities. The aim of this study was to evaluate the relationship between COMT gene polymorphisms and coronary artery abnormalities in Kawasaki disease patients. Methods : One hundred and one Korean children with Kawasaki disease and 306 healthy Korean control subjects were enrolled in this study. The polymorphisms of the COMT gene were analyzed by direct sequencing. Results : There were no differences in the genotype and allelic frequency of the rs4680 and rs769224 polymorphic sites between Kawasaki disease and control subjects. Further, no significant difference was found in the rs4680 polymorphism between patients with coronary artery abnormalities and patients without coronary artery abnormalities (codominant P=0.32, dominant P=0.74, recessive P=0.13). However, the distribution of the rs769224 polymorphism was significantly different between patients with coronary artery abnormalities and patients without coronary artery abnormalities (codominant P= 0.0077, dominant P=0.0021, recessive P=0.16). Conclusion : Our results indicate that the polymorphisms of the rs769224 gene might be related to the development of coronary artery abnormalities in Kawasaki disease.

• Clinical and Experimental Pediatrics
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• v.51 no.9
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• pp.1012-1017
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• 2008

Purpose : This study aimed to assess the mechanical properties of the abdominal aorta in school-aged patients treated for Kawasaki disease and in normal, healthy children. Methods : This study examined 28 children with Kawasaki disease who had been followed up on and 30 healthy subjects of the same age and gender. We recorded systolic (Ps) and diastolic (Pd) blood pressure values and the aortic diameter at both minimum diastolic (Dd) and maximum systolic (Ds) expansion using two-dimensional echocardiography. These measurements were used to determine 1) aortic strain: S=(Ds-Dd)/Dd; 2) pressure strain elastic modulus: Ep=(Ps-Pd)/S; and 3) normalized Ep: $Ep^*=Ep/Pd$. Results : Ep (P=0.008) and $Ep^*$ (P=0.043) of the Kawasaki disease group were relatively high compared to those of the control group. Ep (P=0.002) and $Ep^*$ (P=0.015) of patients with coronary aneurysm were also relatively high compared with those of patients without coronary aneurysm, but lipid profiles did not differ, except for homocysteine (P=0.008). Therefore, in patients with coronary aneurysm, aortic stiffness was higher, compared to not only the control group but also patients without coronary aneurysm. However, in patients without coronary aneurysm, aortic stiffness was not significant, different compared to the control group. Conclusion : Measuring aortic distensibility may be helpful in assessing the risk of early atheroscletic change in the long-term management of Kawasaki disease.

• Pediatric Infection and Vaccine
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• v.17 no.2
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• pp.169-176
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• 2010

Purpose : We aimed to evaluate predictive parameters for non-response to intravenous immunoglobulin (IVIG) in patients with Kawasaki disease (KD) before IVIG use using two controls. Methods : We evaluated 229 consecutive KD patients who were treated with 2 g/kg of IVIG at a single center. Those who had persistent fever >24 hours after IVIG infusion made up the 23 IVIG non-responders; the first control included a total 206 defervesced cases and the second control included 46 cases that were matched for age and pre-treatment fever duration to non-responders. Results : Demographic and clinical characteristics were similar in IVIG non-responders and responders at presentation. As for laboratory findings, the neutrophil differential, CRP, AST, ALT, and LDH were higher, and lymphocyte differential, total protein, albumin, platelet count, and total cholesterol were significantly lower in IVIG non-responders compared to responders by univariate analysis in both study designs. However in multivariate analysis, non-responders showed a significantly higher neutrophil differential (cutoff value, >77%, sensitivity 68.4% and specificity 79.5%) and lower cholesterol (<124 mg/dL, sensitivity 79% and specificity 70.5%). Whereas plasma albumin (<3.6 g/dL, sensitivity 73.7% and specificity 60%) was the sole laboratory parameter of non-responders in the second study design. Conclusion : Severity of inflammation in KD was reflected by higher or lower laboratory values at presentation. Because the multivariate analysis for these indices may be influenced by some confounding factors, including the numbers of patients of different ages and fever duration, other assessment modalities are needed for KD patients with the greatest risk of coronary artery lesions.

• Clinical and Experimental Pediatrics
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• v.51 no.12
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• pp.1320-1323
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• 2008

Purpose : The aim of this study was to investigate the epidemiologic status of Kawasaki disease (KD) in infants ${\leq}6months$ of age. Methods : For the epidemiologic study of KD in Korea, data from 22,674 KD patients were collected from 1997 to 2005 on a 3-year basis by a retrospective survey. From this survey, data of 1,739 KD patients ${\leq}6months$ of age were analyzed and compared with those of KD patients >6 months of age. Results : A total of 1,739 patients ${\leq}6months$ of age, including 1,088 males and 651 females, represented 7.7% of total KD patients. These subjects included 22 patients aged <1 month, 171 patients aged 1-2 months, 304 patients aged 2-3 months, 407 patients aged 3-4 months, 372 patients aged 4-5 months, and 463 patients aged 5-6 months. Their mean age of onset was 4.3 months, and the male-to-female ratio was 1.67:1. Incidences of coronary arterial (CA) abnormalities (21.0% versus 18.7%) and CA aneurysms (4.7% versus 3.1%) detected by echocardiography showed differences between patients with KD younger and older than 6 months, respectively. Conclusion : Comparison of data from KD patients >6 months old with data from 1,739 KD patients ${\leq}6months$ old showed significantly higher incidences of CA abnormalities and CA aneurysms in the younger patients.