• Clinical and Experimental Pediatrics
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• v.63 no.3
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• pp.96-97
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• 2020

• Clinical and Experimental Pediatrics
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• v.59 no.sup1
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• pp.60-63
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• 2016

Herein, we report a rare case of hemolytic anemia with reticulocytopenia following intravenous immunoglobulin therapy in a young infant treated for Kawasaki disease. A 2-month-old boy presented with fever lasting 3 days, conjunctival injection, strawberry tongue, erythematous edema of the hands, and macular rash, symptoms and signs suggestive of incomplete Kawasaki disease. His fever resolved 8 days after treatment with aspirin and high dose infusion of intravenous immunoglobulin. The hemoglobin and hematocrit decreased from 9.7 g/dL and 27.1% to 7.4 g/dL and 21.3%, respectively. The patient had normocytic hypochromic anemia with anisocytosis, poikilocytosis, immature neutrophils, and nucleated red blood cells. The direct antiglobulin test result was positive, and the reticulocyte count was 1.39%. The patient had an uneventful recovery. However, reticulocytopenia persisted 1 month after discharge.

• Clinical and Experimental Pediatrics
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• v.64 no.2
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• pp.68-75
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• 2021

The novel coronavirus disease 2019 (COVID-19) caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection has been spreading worldwide since December 2019. Hundreds of cases of children and adolescents with Kawasaki disease (KD)-like hyperinflammatory illness have been reported in Europe and the United States during the peak of the COVID-19 pandemic with or without shock and cardiac dysfunction. These patients tested positive for the polymerase chain reaction or antibody test for SARS-CoV-2 or had a history of recent exposure to COVID-19. Clinicians managing such patients coined new terms for this new illness, such as COVID-19-associated hyperinflammatory response syndrome, pediatric inflammatory multisystem syndrome temporally associated with COVID-19, or COVID-19-associated multisystem inflammatory syndrome in children (MIS-C). The pathogenesis of MIS-C is unclear; however, it appears similar to that of cytokine storm syndrome. MIS-C shows clinical features similar to KD, but differences between them exist with respect to age, sex, and racial distributions and proportions of patients with shock or cardiac dysfunction. Recommended treatments for MIS-C include intravenous immunoglobulin, corticosteroids, and inotropic or vasopressor support. For refractory patients, monoclonal antibody to interleukin-6 receptor (tocilizumab), interleukin-1 receptor antagonist (anakinra), or monoclonal antibody to tumor necrosis factor (infliximab) may be recommended. Patients with coronary aneurysms require aspirin or anticoagulant therapy. The prognosis of MIS-C seemed favorable without sequelae in most patients despite a reported mortality rate of approximately 1.5%.

• Pediatric Infection and Vaccine
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• v.19 no.2
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• pp.79-83
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• 2012

Reye syndrome is a rapidly progressive encephalopathy with hepatic dysfunction, which often begins several days after apparent recovery from a viral illness, especially varicella or influenza A or B. Salicylate use was identified as a major precipitating factor for the development of Reye syndrome. With the recommendation to avoid use of salicylates in children, Reye syndrome has virtually disappeared in recent years. We report a case of Reye syndrome in a 5-month-old infant who had been treated with intravenous immunoglobulin and aspirin under the diagnosis of Kawasaki disease, and showed symptoms of sudden onset of irritability, rigidity, decreased activity, vomiting, poor appetite, lethargy, liver dysfunction without jaundice, coagulopathy, and hyperammonemia.

• Clinical and Experimental Pediatrics
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• v.62 no.9
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• pp.334-339
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• 2019

Kawasaki disease (KD) is a systemic vasculitis in infants and young children. However, its natural history has not been fully elucidated because the first case was reported in the late 1960s and patients who have recovered are just now entering middle age. Nevertheless, much evidence has raised concerns regarding the subclinical vascular changes that occur in post-KD patients. KD research has focused on coronary artery aneurysms because they are directly associated with fatality. However, aneurysms have been reported in other extracardiac muscular arteries and their fate seems to resemble that of coronary artery aneurysms. Arterial strokes in KD cases are rarely reported. Asymptomatic ischemic lesions were observed in a prospective study of brain vascular lesions in KD patients with coronary artery aneurysms. The findings of a study of single-photon emission computed tomography suggested that asymptomatic cerebral vasculitis is more common than we believed. Some authors assumed that the need to consider the possibility of brain vascular lesions in severe cases of KD regardless of presence or absence of neurological symptoms. These findings suggest that KD is related with cerebrovascular lesions in children and young adults. Considering the fatal consequences of cerebral vascular involvement in KD patients, increased attention is required. Here we review our understanding of brain vascular involvement in KD.

• Proceedings of the Korean Society of Plant Pathology Conference
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• 2004.10a
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• pp.52-52
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• 2004

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• Clinical and Experimental Pediatrics
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• v.60 no.7
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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.

• Pediatric Infection and Vaccine
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• v.17 no.2
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• pp.177-181
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• 2010

Few cases of macrophage activation syndrome (MAS) or reactive hemophagocytic lymphohistiocytosis (HLH) during the acute febrile phase of Kawasaki disease (KD) have been reported. We report on a case of a 19 month-old girl with MAS or reactive HLH during the course of KD. Despite immunoglobulin and steroid therapy, she showed persistent fever with hepatosplenomegaly and evidence of hemophagocytosis in the bone marrow. A high index of suspicion for clinical features associated with MAS is necessary for KD patients in order to provide appropriate treatment.

• Korean Journal of Radiology
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• v.23 no.3
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• pp.308-321
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• 2022

Transabdominal ultrasound (TAUS) is useful in all aspects of lesion screening, monitoring activity, or treating/diagnosing any related complications of inflammatory bowel disease. Its ability to screen or diagnose complications is almost the same as that of other methods, such as CT or MRI. Moreover, its noninvasiveness makes it a first-line examination method. A TAUS image depicting ulcerative colitis will show large intestinal wall thickening that is continuous from the rectum, which is mainly due to mucosal layer thickening, while for Crohn's disease, a TAUS image is characterized by a diversity in the areas affected, distribution, and layer structure. Indicators of activity monitoring include wall thickness, wall structure, and vascular tests that use Doppler ultrasound or contrast agents. While all of these have been reported to be useful, at this time, no single parameter has been established as superior to others; therefore, a comprehensive evaluation of these parameters is justified. In addition, evaluating the elasticity of lesions using elastography is particularly useful for distinguishing between fibrous and inflammatory stenoses. However, the lack of objectivity is the biggest drawback of using ultrasound. Standardizing and popularizing the ultrasound process will be necessary, including scanning methods, equipment settings, and image analysis.

• Pediatric Infection and Vaccine
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• v.21 no.1
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• pp.29-36
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• 2014

Purpose: The objective of this study was to find the predictors and generate a prediction scoring model of nonresponse to intravenous immunoglobulin in patients with Kawasaki disease. Methods: We examined 573 children diagnosed with KD at the Severance Children's Hospital between January 2009 and december 2012. We retrospectively reviewed their medical records. These patients were divided into 2 groups; the experimental group (N=433) and the validation group (N=140). Each group were divided into 2 groups the intravenous immunoglobulin nonresponders and the responders. Multivariate logistic regression analysis identified predictive factors of intravenous immunoglobulin nonresponders which make predictive scoring model. We practice internal validation and external validation. Results: Multivariate logistic regression analysis identified male, cervical lymphadenopathy, changes of the extremities, platelet, total bilirubin, alkaline phophatase, lactate dehydrogenase, C-reactive protein as significant predictors for nonresponse to intravenous immunoglobulin. We generated prediction score assigning 1 point for (1) male, (2) cervical lymphadenopathy, (3) changes of the extremities, (4) platelet (${\leq}368,000/mm^3$), (5) total bilirubin (${\geq}0.4mg/dL$), (6) alkaline phophatase (${\geq}227IU/L$), (7) lactate dehydrogenase (${\geq}268IU/L$), (8) C-reactive protein (>77.1 mg/dL). Using a cut-off point of 4 and more with this prediction score, we could identify the intravenous immunoglobulin nonresponder group. Sensitivity and specificity were 52.5% and 82.4% in experimental group and 37.8% and 81.8% in validation group, respectively. Conclusion: Our predictive scoring models had high specificity and low sensitivity in Korean patients. Therefore it is useful in predicting nonresponse to intravenous immunoglobulin with Kawasaki disease.