• Pediatric Infection and Vaccine
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• 제17권2호
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• pp.177-181
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• 2010

KD의 급성 열성기에 드물지만 MAS가 발생할 수 있다. 저자들은 19개월 여아의 KD 치료 중에 발생된 MAS를 경험하였다. 환아는 IVIG과 steroid 치료를 받았지만, 발열과 간비종대는 지속되었고 골수생검에서 혈구탐식구증이 관찰되었다. KD에 동반된 MAS를 인지하여 적기에 치료하기 위해서는 임상양상과 치료반응을 토대로, MAS의 가능성을 고려하는 것이 필요하다.

• Pediatric Infection and Vaccine
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• 제22권1호
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• pp.40-44
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• 2015

비정형 가와사키병은 가와사키병의 진단기준을 충족하지 않는 경우를 말하며, 주로 6개월 미만의 영아에게서 발현하는 경우가 많다. 비정형 가와사키병의 임상소견은 뇌수막염과 같은 감염질환과 비슷할 때가 있어서, 이러한 경우 임상적으로 비정형 가와사키병을 감염질환과 감별하기 어려울 때가 많다. 또한 가와사키병과 연관되어 보고된 신경계 이상은 무균수막염, 경막하삼출, 안면신경마비, 뇌경색증, 뇌병증, 뇌자기공명영상의 가역적 뇌량팽대 변화 등이 있다. 본 저자들은 뇌척수액세포증가증과 경막외삼출액이 동반된 비정형 가와사키병으로 진단된 5개월 여아에 대해 보고하는 바이다. 환자의 심장초음파검사에서 관상동맥이 늘어나 있었고, 추적관찰에서 경막외 삼출액과 관상동맥 합병증이 모두 회복되었으며 생후 12개월에 발달이정표는 정상이었다.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제3권1호
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• pp.47-55
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• 2000

Purpose: The aim of this research is to examine the abnormal liver function complicated with Kawasaki disease (KD) and to analyze its clinical characteristics and complications. Methods: Ninty eight cases were diagnosed as having KD, among which thirty four cases had abnormal alanine aminotransferase (ALT) (>30 IU/L). These abnormal ALT cases were evaluated in terms of age and sex distribution, major symptoms, complications, laboratory and histopathologic findings. Results: Male to female ratio was 2.4:1 and most patients (91.2%) were under 5 years of age. Cervical lymphadenopathy was observed in 3 cases (8.8%); and recurred cases were two (5.8%). Average fever duration was 8 days; average length of hospitalization was 9 days; and average recovery period was 13 days. Immediately after admission, positive CRP was observed in 31 cases (91.2%), leukocytosis (> $10,000/mm^3$) in 26 cases (76.0%), thrombocytosis (> $450,000/mm^3$) in 7 cases (20.6%), and anemia (<10 gm/dl) in 7 cases (20.6%), respectively. GB hydrops or cholecystitis were noted in 3 cases (8.8%), abnormal ECG finding in 1 case (2.9%), coronary dilatation or aneurysm in 2 cases (5.9%). Liver biopsy was done in four cases and revealed mild infiltration of lymphocytes on the portal area and mild bile duct proliferations. Conclusion: The abnormal liver function was noted in 34.7% of KD patients, and subsided all within one month. But the liver function test should be checked closely in patients of the abnormal liver function test complicated with KD despite of its good prognosis.

• Clinical and Experimental Pediatrics
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• 제56권9호
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• pp.389-395
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• 2013

Purpose: This single-center study was conducted to assess the changes in epidemiological and clinical characteristics and outcomes of patients with Kawasaki disease (KD) over the past 7 years. Methods: This retrospective study included 135 children with KD, admitted to Chungnam National University Hospital, Daejeon, between 2004 and 2005 (group A, n=53) and between 2011 and 2012 (group B, n=82). Medical records were reviewed to obtain information regarding the presenting signs and symptoms, demographic characteristics, and laboratory and echocardiographic findings associated with KD. Results: The hospital admission date after onset was significantly earlier in group B than in group A (P=0.008). The proportion of patients with incomplete KD was 45.3% and 65.9% in group A and B, respectively (P=0.018). The number of pretreatment coronary artery lesions (CALs) were significantly lesser in group B than in group A. (10/53 vs. 5/82, P=0.021). No significant differences was observed in the incidence of CALs at discharge, febrile phase duration, hospital stay duration, incidence of retreatment, and intravenous immunoglobulin dose between 2 groups. The total febrile phase was shorter in patients with incomplete KD than in those with complete KD in both groups. Conclusion: The proportion of incomplete KD has become higher. Furthermore, early admission and management of patients with KD may be related to increased incomplete KD and decreased CALs. Therefore, we believe that a diagnostic strategy for incomplete KD should be established regardless of the presence of coronary lesions.

• Clinical and Experimental Pediatrics
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• 제60권7호
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• pp.208-215
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• 2017

Purpose: Activation of Toll-like receptor 2 (TLR2) present on circulating monocytes in patients with Kawasaki disease (KD) can lead to the production of proinflammatory cytokines and interleukin-10 (IL-10). We aimed to determine the association of the frequency of circulating TLR2+/ CD14+ monocytes (FTLR2%) with the outcomes of KD, as well as to compare FTLR2% to the usefulness of sIL-10. Methods: The FTLR2% in patients with KD was measured by flow cytometry. Serum levels of IL-10 (sIL-10) were determined in 31 patients with KD before the initial treatment with intravenous immunoglobulin (IVIG) and in 21 febrile controls by using enzyme-linked immunosorbent assay. Patients were classified as having coronary artery lesions (CALs) based on the maximal internal diameters of the proximal right coronary artery and proximal left anterior descending coronary artery one month after the initial diagnosis. Results: We found that FTLR2% greater than 92.62% predicted CALs with 80% sensitivity and 68.4% specificity, whereas FTLR2% more than 94.61% predicted IVIG resistance with 66.7% sensitivity and 71.4% specificity. Moreover, sIL-10 more than 15.52 pg/mL predicted CALs and IVIG resistance with 40% and 66.7% sensitivity, respectively, and 73.7% and 76.2% specificity, respectively. Conclusion: We showed that measuring FTLR2% before the initial treatment could be useful in predicting CAL development with better sensitivity than sIL-10 and with results comparable to sIL-10 results for the prediction of IVIG resistance in patients with KD. However, further studies are necessary to validate FTLR2% as a marker of prognosis and severity of KD.

• Clinical and Experimental Pediatrics
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• 제59권10호
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• pp.408-413
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• 2016

Purpose: This study investigated predictors of unresponsiveness to second-line intravenous immunoglobulin (IVIG) treatment for Kawasaki disease (KD). Methods: This was a single-center analysis of the medical records of 588 patients with KD who had been admitted to Asan Medical Center between 2006 and 2014. Related clinical and laboratory data were analyzed by univariate and multivariate logistic regression analyses. Results: Eighty (13.6%) of the 588 patients with KD were unresponsive to the initial IVIG treatment and received a second dose. For these 80 patients, univariate analysis of the laboratory results obtained before administering the second-line IVIG treatment showed that white blood cell count, neutrophil percent, hemoglobin level, platelet count, serum protein level, albumin level, potassium level, and C-reactive protein level were significant predictors. The addition of methyl prednisolone to the second-line regimen was not associated with treatment response (odds ratio [OR], 0.871; 95% confidence interval [CI], 0.216-3.512; P=0.846). Multivariate analysis revealed serum protein level to be the only predictor of unresponsiveness to the second-line treatment (OR, 0.160; 95% CI, 0.028-0.911; P=0.039). Receiver operating characteristic curve analysis to determine predictors of unresponsiveness to the second dose of IVIG showed a sensitivity of 100% and specificity of 72% at a serum protein cutoff level of <7.15 g/dL. Conclusion: The serum protein level of the patient prior to the second dose of IVIG is a significant predictor of unresponsiveness. The addition of methyl prednisolone to the second-line regimen produces no treatment benefit.

• Clinical and Experimental Pediatrics
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• 제59권4호
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• pp.178-182
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• 2016

Purpose: Medium-dose (1 g/kg) intravenous immunoglobulin (IVIG) is effective in the majority of patients with Kawasaki disease (KD) but some patients who do not respond to medium-dose IVIG are at high risk for the development of coronary artery lesions (CALs). The purpose of this study was to identify the clinical predictors associated with unresponsiveness to medium-dose IVIG and the development of CALs. Methods: A retrospective study was performed in 91 children with KD who were treated with mediumdose IVIG at our institution from January 2004 to December 2013. We classified the patients into responders (group 1; n=68) and nonresponders (group 2; n=23). We compared demographic, laboratory, and echocardiographic data between the 2 groups. Results: Multivariate logistic regression analysis identified 6 variables as predictors for resistance to medium-dose IVIG. We generated a predictive scoring system assigning 1 point each for percentage of neutrophils ${\geq}65%$, C-reactive protein ${\geq}100mg/L$, aspartate aminotransferase ${\geq}100IU/L$, and alanine aminotransferase ${\geq}100IU/L$, as well as 2 points for less than 5 days of illness, and serum sodium level ${\leq}136mmol/L$. Using a cutoff point of ${\geq}4$ with this scoring system, we could predict nonresponsiveness to medium-dose IVIG with 74% sensitivity and 71% specificity. Conclusion: If a patient has a low-risk score in this system, medium-dose IVIG can be recommended as the initial treatment. Through this process, we can minimize the adverse effects of high-dose IVIG and incidence of CALs.

• Clinical and Experimental Pediatrics
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• 제61권5호
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• pp.160-166
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• 2018

Purpose: This study aimed to analyse laboratory values according to fever duration, and evaluate the relationship across these values during the acute phase of Kawasaki disease (KD) to aid in the early diagnosis for early-presenting KD and incomplete KD patients. Methods: Clinical and laboratory data of patients with KD (n=615) were evaluated according to duration of fever at presentation, and were compared between patients with and without coronary artery lesions (CALs). For evaluation of the relationships across laboratory indices, patients with a fever duration of 5 days or 6 days were used (n=204). Results: The mean fever duration was $6.6{\pm}2.3days$, and the proportions of patients with CALs was 19.3% (n=114). C-reactive proteins (CRPs) and neutrophil differential values were highest and hemoglobin, albumin, and lymphocyte differential values were lowest in the 6-day group. Patients with CALs had longer total fever duration, higher CRP and neutrophil differential values and lower hemoglobin and albumin values compared to patients without CALs. CRP, albumin, neutrophil differential, and hemoglobin values at the peak inflammation stage of KD showed positive or negative correlations each other. Conclusion: The severity of systemic inflammation in KD was reflected in the laboratory values including CRP, neutrophil differential, albumin, and hemoglobin. Observing changes in these laboratory parameters by repeated examinations prior to the peak of inflammation in acute KD may aid in diagnosis of early-presenting KD patients.

• Clinical and Experimental Pediatrics
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• 제61권5호
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• pp.167-173
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• 2018

Purpose: Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome with many causes, including Kawasaki disease (KD). The purpose of this study was to identify the laboratory tests needed to easily differentiate KD with HLH from incomplete KD alone. Methods: We performed a retrospective study on patients diagnosed with incomplete KD and incomplete KD with HLH (HLH-KD) between January 2012 and March 2015. We compared 8 secondary HLH patients who were first diagnosed with incomplete KD with all 247 incomplete KD diagnosed patients during the study period. The complete blood count, erythrocyte sedimentation rate, platelet count, and serum total protein, albumin, triglyceride, C-reactive protein, N-terminal pro-brain natriuretic peptide (NT-proBNP), and ferritin levels were compared. Clinical characteristics and echocardiography findings were also compared between the 2 groups. Results: The total duration of fever was longer in the HLH-KD group than in the KD group. White blood cell and platelet counts were higher in the KD group. Alanine aminotransferase, ferritin, and coronary artery diameter were increased in the HLH-KD group compared with those in the KD group. The median of NT-proBNP was significantly higher in the HLH-KD group than in the KD group at 889.0 (interquartile range [IQR], 384.5-1792.0) pg/mL vs. 233.0 (IQR, 107.0-544.0) pg/mL. Conclusion: The NT-proBNP level may be helpful in distinguishing incomplete KD from KD with HLH. The NT-proBNP level should be determined in KD patients with prolonged fever, in addition to the white blood cell count, platelet count, and ferritin level, to evaluate secondary HLH.

• Journal of Yeungnam Medical Science
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• 제31권2호
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• pp.94-98
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• 2014

Background: We analyzed the changes in QT dispersion (QTd) in children with Kawasaki disease (KD), and determined the presence of repolarization abnormality in these children even in the absence of coronary artery abnormalities. Methods: Ninety-one children with KD and 20 healthy controls were enrolled in this retrospective study. Serial echocardiographic and electrocardiographic (ECG) measurements in the beginning of treatment, 2nd month and 6th month after the diagnosis were compared. Fifty-one of 91 children had at least 2 serial ECG data. The number of patients who had 3 consecutive ECG data was 23. Results: Among the 67 KD patients with no coronary artery changes, the consecutive mean QTd values were 41.86 ms, 37.84 ms, and 25.47 ms, respectively (26 ms for controls). In the analysis of changes among KD patients without coronary artery abnormalities, QTd showed a significant decrease with time (p=0.01). Especially, the 1st month and the 6th month QTd values were significantly different (p=0.028). The mean QTd values in KD patients with coronary artery changes were significantly higher than those in KD patients with no coronary artery changes at each time (1st, 2nd, and 6th month exam). Conclusion: QTd is significantly increased in children during the early stage of KD. Repolarization abnormality may exist during the acute stage of KD, regardless of the echocardiographic changes.