• Title/Summary/Keyword: Kawasaki

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Update of genetic susceptibility in patients with Kawasaki disease

  • Yoon, Kyung Lim
    • Clinical and Experimental Pediatrics
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    • v.58 no.3
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    • pp.84-88
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    • 2015
  • Kawasaki disease (KD) is an acute systemic vasculitis that predominantly affects children, and can result in coronary artery lesions (CAL). A patient with KD who is resistant to treatment with intravenous immunoglobulin (IVIG) has a higher risk of developing CAL. Incomplete KD has increased in prevalence in recent years, and is another risk factor for the development of CAL. Although the pathogenesis of KD remains unclear, there has been increasing evidence for the role of genetic susceptibility to the disease since it was discovered in 1967. We retrospectively reviewed previous genetic research for known susceptibility genes in the pathogenesis of KD, IVIG resistance, and the development of CAL. This review revealed numerous potential susceptibility genes including genetic polymorphisms of ITPKC, CASP3, the transforming growth factor-${\beta}$ signaling pathway, B lymphoid tyrosine kinase, FCGR2A, KCNN2, and other genes, an imbalance of Th17/Treg, and a range of suggested future treatment options. The results of genetic research may improve our understanding of the pathogenesis of KD, and aid in the discovery of new treatment modalities for high-risk patients with KD.

Kawasaki disease in infants

  • Yeom, Jung Sook;Woo, Hyang Ok;Park, Ji Sook;Park, Eun Sil;Seo, Ji-Hyun;Youn, Hee-Shang
    • Clinical and Experimental Pediatrics
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    • v.56 no.9
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    • pp.377-382
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    • 2013
  • Kawasaki disease (KD) is an acute febrile illness that is the predominant cause of pediatric acquired heart disease in infants and young children. Because the diagnosis of KD depends on clinical manifestations, incomplete cases are difficult to diagnose, especially in infants younger than 1 year. Incomplete clinical manifestations in infants are related with the development of KD-associated coronary artery abnormalities. Because the diagnosis of infantile KD is difficult and complications are numerous, early suspicion and evaluation are necessary.

A study of serum lipid level and relation between the serum lipid level and coronary aneurysm after kawasaki disease (가와사끼병에서 혈청 지질 대사의 변화 및 관상동맥류에 관한 연구)

  • Chung, Churl Young;Kim, Chang Keun
    • Pediatric Infection and Vaccine
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    • v.2 no.2
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    • pp.186-192
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    • 1995
  • We measured plasma concentrations of high density lipoprotein, total cholesterol and triglycerides in 31 patients with history of kawasaki disease during acute stage and convalescence stage. High density lipoprotein cholesterol and total cholesterol concentrations were significantly lower(P<0.001) in samples taken within 11 days of the onset of illness($28.7{\pm}12.4mg/dl$ and $145.20{\pm}29.60mg/dl$) than in the second samples taken 1-2months after onset of disease($51.5{\pm}15.2mg/dl$ and $175.4{\pm}29.0mg/dl$). Change of triglycerides was not significant. There was no correlation between the serum cholesterol cencentration and coronary aneurysm and continued long term surveillance of much population is necessary to monitor lipid level and their relation to development of premature coronary atherosclerosis.

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Intravenous Immunoglobulin Nonresponsive Symptomatic Myocarditis during the Acute Stage of Incomplete Kawasaki Disease (불완전 가와사키병 급성기에 면역글로불린 치료에 반응하지 않는 유증상 심근염)

  • Sohn, Youngsoo;Kim, Yeo Hyang
    • Pediatric Infection and Vaccine
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    • v.22 no.3
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    • pp.206-209
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    • 2015
  • We report the case of a 7-year-old boy who showed treatment-nonresponsive hypotension (59/29 mmHg) and decreased left ventricular systolic function (fractional shortening 22%) in the acute stage of Kawasaki disease (KD). The present case serves to highlight that methylprednisolone pulse therapy should be considered in patients with intravenous immunoglobulin nonresponsive symptomatic myocarditis during the acute stage of KD.

Reality of Kawasaki disease epidemiology

  • Kim, Gi Beom
    • Clinical and Experimental Pediatrics
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    • v.62 no.8
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    • pp.292-296
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    • 2019
  • Epidemiologic studies of Kawasaki disease (KD) have shown a new pattern or change of its occurrence suggestive of its pathophysiology or risk factors from the first patient with KD reported in 1961. The incidence of KD in Northeast Asian countries including Japan, South Korea, China, and Taiwan is 10-30 times higher than that in the United States and Europe. Knowing the true epidemiology of KD in each country and the availability of publications of KD epidemiology also could benefit general health care providers and general population. This would enable the early detection and treatment of KD, ultimately reducing the incidence of coronary artery complications and mortality. Therefore, efforts to investigate the true epidemiology of KD should be continued in every country using a questionnaire survey, National Health Insurance system data, or combined methods depending on each country's medical environment to ensure high-quality care of patients with KD.

Laboratory Values in Patients with Kawasaki Disease after Intravenous Immunoglobulin : Comparison of Patients with Coronary Artery Lesions to those without Coronary Artery Lesions (가와사끼병에서 정맥용 면역글로불린 투여 후 검사실 지표의 변화 : 관상 동맥 병변이 있는 군과 없는 군과의 비교)

  • Park, Min-Young;Lee, Kyung-Yil;Han, Ji-Whan;Lee, Hyung-Shin;Hong, Ja-Hyun;Whang, Kyung-Tai
    • Clinical and Experimental Pediatrics
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    • v.46 no.2
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    • pp.162-166
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    • 2003
  • Purpose : We evaluated the effects of intravenous immunoglobulin(IVIG) on level of laboratory parameters examined serially according to the existence of coronary artery lesions in children with Kawasaki disease. Methods : Children with Kawasaki disease(n=63), treated with IVIG at a dose of 2.0 g/kg, were classified as a group with coronary artery lesions(CALs+ group, n=9) or a group without coronary artery lesions(CALs- group, n=54). Levels of various laboratory parameters were determined three times during admission; before, 24 hrs after and 7 days after IVIG administration. Results : There were no significant differences in laboratory parameters performing at, before and 7 days after IVIG administration. However WBC and neutrophil counts, and CRP were significantly higher, and the level of albumin was significantly lower at 24 hrs after IVIG administration. Conclusion : Approximately 15% of patients with Kawasaki disease showed CALs in the acute stage. Kawasaki disease patients with CALs were associated with persistent elevated levels of inflammatory parameters including WBC count, neutrophil count and CRP examined 24 hours after IVIG administration.

The Clinical and Laboratory Features of Kawasaki Disease with Nonresponsibility to the Acute Antiinflammatory Treatment (급성기 항염치료에 반응하지 않은 가와사끼병의 임상양상과 검사소견)

  • Kim, Eun-Jung;Hong, Myung-Eun;Lee, Chang-Woo;Oh, Yeon-Geun;Kim, Jong-Duk;Yoon, Hyang-Suk
    • Clinical and Experimental Pediatrics
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    • v.46 no.5
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    • pp.500-504
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    • 2003
  • Purpose : To assess the clinical features and laboratory findings in Kawasaki patients with nonresponsibility to the acute antiinflammatory treatment, and identify the risk factors for the nonresponsibility, we reviewed the medical records of patients with Kawasaki disease. Methods : A retrospective study of 177 patients with Kawasaki disease at Wonkwang University Medical Center from June, 1997 to June, 2002, was performed. High dose intravenous immune globulin(IVIG) and aspirin were all used for the initial acute antiinflammatory treatment. Two groups, group A(n=19) of initial nonresponders and group B(n=158) of initial responders were compared clinically and laboratorically. Results : Nineteen(10.7%) of 177 patients hardly responded to the initial antiinflammatory treatment. Patients with failure to respond to initial treatment(group A) did not differ from the control group in terms of age, sex, WBC count, coronary abnormalities, and evidence of pyuria. Compared with initial responders(group B), the patients who were retreated(group A) had a significantly shorter feveradmission interval(P=0.041), and a higher level of both AST/ALT(P=0.011) and ASO titier(P=0.000). Conclusion : Among Kawasaki disease patients studied, retreatment group with initial nonresponders had significantly shorter fever-admission interval, and higher both AST/ALT level and ASO titer, than the initial response group.

Log-transformed plasma level of brain natriuretic peptide during the acute phase of Kawasaki disease is quantitatively associated with myocardial dysfunction

  • Bang, Sun-Hee;Yu, Jeong-Jin;Han, Myung-Ki;Ko, Hong-Ki;Chun, Sa-Il;Choi, Hyung-Soon;Kim, Young-Hwue;Ko, Jae-Kon;Park, In-Sook
    • Clinical and Experimental Pediatrics
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    • v.54 no.8
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    • pp.340-344
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    • 2011
  • Purpose: Brain natriuretic peptide (BNP) has been considered a biochemical marker for myocarditis in Kawasaki disease. We performed this study to determine its quantitative significance. Methods: We attempted to correlate log-transformed BNP concentrations (log-BNP) and clinical, laboratory, and echocardiographic variables in 81 children with Kawasaki disease. Stepwise multiple linear regression analysis was used to determine the variables independently associated with log-BNP concentration. Results: Serum C-reactive protein level (P<0.0001), serum alanine aminotransferase concentration (P =0.0032), white blood cell count (P=0.0030), and left ventricular mass index (P=0.0024) were positively related with log-BNP, and hemoglobin level (P<0.0001), serum albumin level (P<0.0001), $Na^+$ concentrations (P<0.0001), left ventricular fractional shortening (P=0.0080), and peak early diastolic tissue velocity of the left ventricular basal lateral segment (P=0.0045) were negatively related to the log-BNP concentration. Multiple regression analysis showed that serum albumin concentration ($R_2$=0.31, P=0.0098) and left ventricular mass index ($R_2$=0.09, P=0.0004) were significantly associated with the log-BNP concentration. Conclusion: Elevated BNP levels during the acute phase of Kawasaki disease may be attributable to cardiac dysfunction associated with the increase in left ventricular mass, and log-BNP concentration may be a quantitative biochemical marker of myocarditis in Kawasaki disease.

Corticosteroids Add-on Therapy in the Acute Phase of Kawasaki Disease (고위험군 가와사끼병에서 스테로이드 추가 요법의 효과)

  • Kang, Seon-Mi;Moon, Eun-Kyung;In, Su-Mi;Kil, Hong-Ryang
    • Clinical and Experimental Pediatrics
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    • v.45 no.12
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    • pp.1571-1576
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    • 2002
  • Purpose : Recently, clinical trials of steroid add-on therapy were reported with variable results in Kawasaki disease. We analyzed the clinical outcomes of patients at high risk of with Kawasaki disease(${\geq}4$ points of Harada score) treated by three commonly used different treatment regimens, with or without corticosteroids. Methods : Medical records of 96 children with Kawasaki disease treated with one of the threee regimens were reviewed retrospectively. Regimen 1 was aspirin(100 mg/kg/day) plus intravenous gamma globulin 2 g/kg single dose; regimen 2, aspirin(100 mg/kg/day) plus intravenous gamma globulin 1 g/kg single dose; regimen 3, regimen 2 plus prednisolone(2 mg/kg/day), followed by tapering two weeks and pulse therapy of methyl prednisolone performed in cases of retreatment. Also low dose aspirin was given in all three regimens for eight weeks after the acute phase. The cardiovascular and laboratory evaluations were performed on acute phase, immediate after acute phase, and subacute phase, eight weeks after treatment. Results : The frequency of coronary artery lesions and laboratory findings in the three different regimens were similar. The more rapid control of fever after treatment was noted in regimen 3. Furthermore the frequency of retreatment was decreased in regimen 3 compared to the other two regimens. Conclusion : Steroid add-on therapy showed some beneficial outcome compared to conventional treatment regimens. The role of steroid in the treatment of Kawasaki disease should be reassessed in systemic manner.