• Annals of Clinical Neurophysiology
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• v.22 no.2
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• pp.117-120
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• 2020

Miller Fisher syndrome (MFS) is characterized by the acute ophthalmoparesis, ataxia and areflexia. We describe the case of 70-year-old man with cardinal symptom of MFS and active pulmonary tuberculosis (Tb). A thorough evaluation led to the diagnosis of MFS and treatment with intravenous immunoglobulin (IVIg) was started. The complete resolution of ophthalmoparesis and ataxia was observed from the fourth day of IVIg treatment. This is the first report to describe a case of MFS that developed in patient pulmonary tuberculosis.

• Clinical and Experimental Pediatrics
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• v.49 no.9
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• pp.987-990
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• 2006

Intravenous immunoglobulin (IVIG) infusion is an effective therapy for acute Kawasaki disease (KD). Nonetheless, approximately 10 percent to 20 percent of patients have persistent or recrudescent fever despite IVIG treatment, leading to a higher risk for coronary artery aneurysms (CAA). This unresponsiveness may pose a challenge to the clinicians. Tumor necrosis $factor-{\alpha}$ levels are elevated in the acute phase of the disease, especially in patients who develop CAA. We report a 10-month-old male with KD who failed to respond to multiple doses of IVIG and methylprednisolone and who then was treated with infliximab (5 mg/kg single dose). After infliximab treatment, he became afebrile with normalization of inflammatory markers and no further progression of CAA.

• Clinical and Experimental Pediatrics
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• v.58 no.10
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• pp.369-373
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• 2015

Purpose: In 2004, the American Heart Association (AHA) had published an algorithm for the diagnosis of incomplete Kawasaki disease (KD). The aim of the present study was to investigate characteristics of supplemental laboratory criteria in this algorithm. Methods: We retrospectively examined the medical records of 355 patients with KD who were treated with intravenous immunoglobulin (IVIG) during the acute phase of the disease. Laboratory data were obtained before the initial IVIG administration and up to 10 days after fever onset. In 106 patients, laboratory testing was performed more than twice. Results: The AHA supplemental laboratory criteria were fulfilled in 90 patients (25.4%), and the frequency of laboratory examination (odds ratio [OR], 1.981; 95% confidence interval [CI], 1.391-2.821; P<0.001) was a significant predictor of it. The fulfillment of AHA supplemental laboratory criteria was significantly associated with refractoriness to the initial IVIG administration (OR, 2.388; 95% CI, 1.182-4.826; P=0.013) and dilatation of coronary arteries (OR, 2.776; 95% CI, 1.519-5.074; P=0.001). Conclusion: Repeated laboratory testing increased the rate of fulfillment of the AHA supplemental laboratory criteria in children with KD.

• Pediatric Infection and Vaccine
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• v.31 no.1
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• pp.140-146
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• 2024

Multisystem inflammatory syndrome in children (MIS-C) is a rare complication of coronavirus disease 2019 (COVID-19), causing multi-organ damage affecting the heart, lungs, kidneys, digestive tract, and nervous system. As the cases of MIS-C have been increasing following the COVID-19 pandemic, the importance of appropriate management for MIS-C is becoming increasingly apparent. Immunomodulating agents such as anakinra, infliximab, and steroids are regarded as supplementary therapy to the first-line treatment with intravenous immunoglobulin. However, these immunomodulating therapies can potentially precipitate opportunistic infections, including those caused by cytomegalovirus (CMV), Epstein-Barr virus, and tuberculosis, or increase the risk of co-infections. Herein, we report a case of a 3-year-old boy who was treated with immunoglobulin, infliximab, and high-dose methylprednisolone for MIS-C, and subsequently developed a persistent fever lasting 32 days caused by a CMV infection.

• Clinical and Experimental Pediatrics
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• v.48 no.7
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• pp.772-778
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• 2005

Purpose : Kawasaki disease(KD) is a systemic panvasculitis that causes coronary artery lesions. KD is accompanied by immunoregulatory abnormalities. Nitric oxide(NO) can induce relaxation of blood vessels by activating guanylate cyclase in smooth muscle cells and high levels of NO may result in coronary artery lesions. We investigated tumor necrosis factor$(TNF)-{\alpha}$ and NO production before and after intravenous immunoglobulin(IVIG) therapy to study the roles of NO and $TNF-{\alpha}$ in KD with coronary artery lesions. Methods : Serum levels of NO and $TNF-{\alpha}$ were measured in 24 patients with KD(group I, eight patients with normal coronary artery; group II, 16 patients with coronary artery lesions) and 23 controls(group III, 13 afebrile controls; group IV, 10 febrile controls). Blood samples from each subject were drawn before and after IVIG therapy and in the convalescent stage. Serum concentrations of NO and $TNF-{\alpha}$ were measured by enzyme linked immuno sorbent assay. Results : The NO levels before IVIG therapy were significantly higher in group II than in group I, group III and group IV. After IVIG therapy the levels of NO were significantly higher in group I and group II than in group III. The $TNF-{\alpha}$ levels before IVIG therapy were significantly higher in group I and group II than in group III. The serum $TNF-{\alpha}$ and NO levels were higher before IVIG therapy and decreased through the convalescent stage in KD patients. In the acute stage of KD patients with coronary artery lesions, serum NO levels significantly correlated with white blood cells (r=0.43, P<0.05). Conclusion : The serum concentration levels of $TNF-{\alpha}$ and NO were abnormally high in KD patients and NO concentrations were statistically higher in the KD patients with coronary artery abnormalities than those without coronary abnormality during the early stage of the KD. These results suggest NO may be involved in the development of coronary artery lesions.

• Clinical and Experimental Pediatrics
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• v.46 no.6
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• pp.591-596
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• 2003

Purpose : Kawasaki disease(KD) is rare in infants <3 months of age. In this younger group, the diagnosis may be delayed due to lack of most of the clinical criteria, resulting in a high risk of cardiac complications. We examined clinical characteristics in these patients for early recognition and treatment. Methods : We conducted a retrospective study on the infants with KD aged three months or younger treated at our hospital from January 1998 to July 2002. Results : Of a total of 291 patients treated during the study period, 11(3.8%) were three months old or younger. Of the 11 patients, 10 had atypical presentations. Infants had fewer of the accepted criteria, and the most common findings were fever(100%) and oral mucosal changes(72.7%). Erythema at the site of BCG inoculation was observed in six of the 11 patients(unknown in the remaining five). This feature proved a definite diagnostic clue in two patients in whom cardiac complications developed in the subacute phase. Cardiac complications were found in six patients(54.5%) : three had coronary dilatation, two had coronary wall irregularity, and one had mitral valve prolapse with regurgitation. Defervescence occurred within $1.1{\pm}0.3day$ in 10 of the 11 patients treated with intravenous immunoglobulin(IVIG) and one was given a second course of IVIG. Echocardiographic abnormality persisted in only one patient with mitral regurgitation at the 6-month follow-up. Conclusion : Most patients with KD younger than three months of age have atypical presentations and a high complication rate. For early diagnosis, erythema at the BCG inoculation site, if present, could be used as a valid diagnostic clue to atypical KD in this age group.

• Clinical and Experimental Reproductive Medicine
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• v.32 no.3
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• pp.217-222
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• 2005

Objectives: We aimed to investigate the clinical effect of low-dose intravenous immunoglobulin treatment in unexplained recurrent spontaneous aborters (RSA) with elevated peripheral CD56+ natural killer (NK) cell levels and to determine the pre-conceptional NK cell percentage predictive of subsequent successful pregnancy outcome. Materials and Methods: Sixty four cases of unexplained recurrent miscarriage with elevated peripheral NK cells (>15%) were received low dose IVIg infusion at the dosage of 400 mg/Kg/month after confirmation of gestational sac and continued until 20 weeks. The patients were divided into two groups according to the pregnancy outcome: Group I was success of treatment defined as live birth at or after 25 gestational weeks and Group II was failure of treatment. The preconceptional levels of the peripheral blood NK cells were compared between two groups. Results: Fifty-three pregnancies resulted in live births after 25 weeks and 11 resulted in abortion (Overall success rate of IVIG treatment was 82.8%). Preconceptional CD56+ NK cell percentage in group II ($27.4{\pm}1.9%$) was higher than those in group I ($22.3{\pm}0.8%$). By using ROC curve, optimal discrimination between success and failure of treatment was achieved with ${\leq}27%$ of preconceptional NK cell percentage. Conclusion: In RSA patients with elevated NK cells, we suggest that preconceptional peripheral blood CD56+ NK cell level could be a useful marker for predicting successful treatment outcome of low-dose IVIg infusion.

• Pediatric Infection and Vaccine
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• v.28 no.3
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• pp.173-180
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• 2021

After initial reports of multisystem inflammatory syndrome in children (MIS-C) in April 2020 in Europe, this disease has been known to occur in children with recent history of coronavirus disease 2019 (COVID-19) and most cases have been reported in Europe and the Unites States of America. We report a case of a 14-year-old girl who was diagnosed with MIS-C with acute myocarditis and successfully treated with intravenous immunoglobulin (IVIG), methylprednisolone, and anakinra. At initial presentation, she had persistent high fever, generalized rash, generalized swelling, abdominal pain, and low blood pressure. She showed a remarkably elevated level of inflammation and cardiac enzyme markers and had a previous history of COVID-19 5 weeks before the initial presentation. After extensive work up, other infectious and non-infectious causes were excluded. She was diagnosed with MIS-C and initially treated with IVIG and high-dose methylprednisolone; however, despite treatment, her heart function deteriorated and coronary artery dilatation progressed. Therefore, anakinra, an interleukin-1 receptor antagonist, was administered on hospital day 6, after which her cardiac function exhibited improvement. She was discharged on hospital day 19 without any symptoms, and follow-up echocardiography after 1 month revealed fully recovered heart function with normal coronary arteries.

• 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.

• 대한생식의학회:학술대회논문집
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• 2004.11a
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• pp.98-98
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• 2004