• Pediatric Infection and Vaccine
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• v.24 no.2
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• pp.102-107
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• 2017

Purpose: Coronary arterial lesions (CALs) were reported to have developed in children with systemic inflammatory diseases, as well as those with Kawasaki disease (KD). The purpose of this study was to confirm that the CAL development in children with KD occurs in a mouse model of sepsis presenting typical systemic inflammatory response syndrome (SIRS). Methods: To induce the sepsis mouse model with SIRS, 6-week-old C57BL/6 mice were intraperitoneally injected with endotoxin. We compared histological findings of the major organs between the control and the sepsis groups and examined CAL in the heart of the septic mice. Results: Infiltrating inflammatory cells were relatively increased in the heart, liver, and kidneys of the sepsis group, compared with those of the control group. We confirmed lymphocytic infiltration in the myocardium (myocarditis) and the pericardial soft tissue of the heart. Furthermore, coronary artery of the septic mouse was identified, but CAL was not observed. Conclusions: In this study, we failed to confirm the existence of CAL in a mouse model of sepsis. However, it is well-known that CALs are seen in many kinds of diseases that cause SIRS. Our findings suggest further investigation into the clinical significance of CAL in various systemic inflammatory diseases, including KD.

• Clinical and Experimental Pediatrics
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• v.52 no.4
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• pp.476-480
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• 2009

Purpose : The purpose of this study was to investigate the polymorphisms of the TNF-alpha promotor gene, its susceptibility to Kawasaki disease (KD) and to assess whether the TNF-alpha promotor gene polymorphism was related the risk of coronary artery lesions (CALs). Methods : From January 2003 to January 2007, 51 children (30 boys and 21 girls) with KD and 48 children forming an age-matched control group were studied. DNA from the peripheral blood of all the children was sampled, and the DNA polymorphisms of the 5' flanking regions of the TNF-alpha promoter gene at position -308 [guanine (G) to adenine (A)] were determined by polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP). Then, the relationship between KD and the TNF-alpha promotor gene polymorphisms was evaluated. Results : The A allele frequency of the -308 site of the TNF-alpha promotor gene was 17.6% (9/51) for children with KD and 6.8% (3/48) for the control group children, but this result was not statistically significant. Twenty-four patients experienced CALs within 60 days after the onset of symptoms. KD children with TNF-alpha -308 A allele had lower frequencies of CALs (12.5% versus 22.2%, P>0.05). Conclusion : The DNA polymorphism of the -308 site TNF-alpha gene was not associated with susceptibility to KD and a risk of CALs. Multicenter, large-scale randomized controlled trials are needed for further study.

• Clinical and Experimental Pediatrics
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• v.48 no.3
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• pp.321-326
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• 2005

Kawasaki disease is an acute systemic vasculitis of unknown origin. Giant coronary aneurysm is one of the most serious complications, although peripheral artery vasculitis can produce life-threatening events. Myocardial ischemia and infarction can be caused by coronary artery stenosis, aneurysm, and stagnation of blood flow in coronary arteries which triggers thromboembolism. Atypical presentation in young infants often interferes with prompt diagnosis and timely treatment, resulting in poor outcomes. We describe a 3-month-old infant with multiple giant coronary aneurysms with flow stagnation, stenosis and large mural thrombus due to Kawasaki disease. He presented with a prolonged course of severe coronary involvement in spite of all measures to reduce coronary complications. Finally, surgical intervention was tried because of the worsening coronary artery abnormalities. The patient died of acute cardiorespiratory failure shortly after weaning from cardiopulmonary bypass.

• Clinical and Experimental Pediatrics
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• v.48 no.8
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• pp.901-906
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• 2005

Kawasaki disease (KD) is a leading cause of acquired heart disease in children. Yet the etiology of KD is still unknown and diagnosis depends on the exclusion of other diseases and the clinical manifestations meeting the defined criteria. Young infants frequently show atypical clinical courses and are frequently complicated with coronary aneurysms. Some cases show thrombocytopenia, which is known as one of the risk factors for complications with coronary aneurysms. So, a high index of suspicion is the most important factor for the diagnosis of KD in very young infants or adolescents whose clinical courses are equivocal. We report herein on a case of KD in an 80-day-old female infant with fever and seizure with bloody stool; laboratory findings were those of sepsis with disseminated intravascular coagulopathy. In spite of aggressive treatments, fever and thrombocytopenia persisted for two weeks and huge coronary aneurysms developed at the third week in all three major coronary arteries; the diameter of the right one was as large as the aortic annulus. Three months later, huge pulsatile masses developed in both axillas; these were found to be huge axillary aneurysms defined very clearly on multi-detector CT scan. She has been under follow up with antiplatelets and anticoagulation therapy with poor regression of the aneurysms.

• Clinical and Experimental Pediatrics
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• v.52 no.9
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• pp.1029-1034
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• 2009

Kawasaki disease (KD) is an acute systemic vasculitis of unknown etiology that affects children. There are few reports that describe the Epstein-Barr virus (EBV) as the possible infectious agent of KD. Here, we describe a case of KD in a 15-year-old boy complicated with giant coronary artery aneurysms, pericardial effusion, and splenic infarction. The clinical course of KD was refractory to intravenous gamma globulin and aspirin. Our patient also showed typical findings of concomitant EBV-associated infectious mononucleosis, such as hepatosplenomegaly and generalized lymphadenopathy, with EBV-positive atypical lymphoid hyperplasia. He improved dramatically after receiving intravenous methylprednisolone followed by oral prednisolone. Ultimately, the coronary artery aneurysms remained as the only sequelae. We report a rare case of adolescent KD with EBV-associated infectious mononucleosis and splenic infarction.

• Clinical and Experimental Pediatrics
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• v.49 no.11
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• pp.1202-1210
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• 2006

Purpose : A quantitative and easily measured Doppler index of combined systolic and diastolic ventricular myocardial performance (Tei index) was recently proposed as a potentially useful predictor of global myocardial performance. However, presence of heart rate fluctuation makes it unreliable. Therefore, the modified Tei index was introduced by using tissue Doppler imaging (TDI) which enables measuring contraction and relaxation velocities from myocardium simultaneously. The purpose of this study was to investigate the effectiveness of the modified Tei index in the evaluation of global cardiac function. Methods : Forty eight patients in the acute phase of Kawasaki disease (KD) were studied. These patients were divided into two groups according to the modified Tei index. TDI and conventional echocardiography were performed. Systolic velocity, systolic displacement, E' velocity, E' displacement, A' velocity and A' displacement were measured at the base, mid-septum and the apex of the interventricular septum. Ejection fraction (EF), Tei index and modified Tei index were estimated. Results : Modified Tei index correlated negatively with systolic displacement, E' displacement and A' displacement at the base. Modified Tei index correlated positively with ESR and CRP. EF and Tei index were normal in KD patients. Conclusion : Modified Tei index is a safe, feasible and sensitive index for evaluating global ventricular functions in spite of normal EF and Tei index in KD.

• Pediatric Infection and Vaccine
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• v.24 no.3
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• pp.141-145
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• 2017

Purpose: Clinical and laboratory features of two Kawasaki disease (KD) groups were evaluated; the patient with pyuria and those without pyuria. Methods: From January 2015 to December 2016, the medical records of 140 (86 males and 54 females) inpatients with KD were retro-spectively analyzed. Results: Forty-eight KD patients (34.3%) presented with pyuria. KD patients with pyuria showed a higher level of C-reactive protein (CRP) and a higher proportion of elevated liver enzymes than those without pyuria. There were no differences in the proportions of unresponsiveness to intravenous immunoglobulin and coronary artery lesions between the two groups. Six KD patients (12.5%) with pyuria underwent a renal imaging study to rule out the possibility of a urinary tract infections. Thirty-two KD patients (66.7%) with pyuria received treatment with antibiotics in addition to the standard treatment for KD. Conclusions: KD patients with pyuria showed a higher level of CRP and elevated levels of liver enzymes than those without pyuria. These findings suggest that KD patients with pyuria have more severe systemic inflammation than those without pyuria.

• Pediatric Infection and Vaccine
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• v.30 no.2
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• pp.97-103
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• 2023

Kawasaki disease (KD) is an acute, systemic inflammatory disorder that often targets coronary arteries. Being the common cause of acquired heart disease in children, timely diagnosis and intravenous immunoglobulin treatment are crucial. However, it is challenging for physicians to diagnose KD if it presents with atypical manifestations. We report the case of a 5-year-old boy who initially presented with appendicitis; after an appendectomy, he had a prolonged fever. He was finally diagnosed with atypical KD and successfully recovered after intravenous immunoglobulin treatment. Through a literature review, we found 21 cases of appendicitis associated with KD. In most cases, the patients were male with a mean age of 5.3 years. Most had higher proportions of incomplete KD and coronary artery complications than expected for typical KD. In conclusion, appendicitis could be a rare complication of KD; therefore, multidisciplinary cooperation and early recognition of atypical KD are essential for timely diagnosis.

• Clinical and Experimental Pediatrics
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• v.52 no.2
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• pp.234-241
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• 2009

Purpose : We investigated the relationship between thyroid hormone and serum tumor necrosis factor (TNF-${\alpha}$), interleukin (IL-6) and N-terminal fragment of pro-brain natriuretic peptide (NT-proBNP) in patients with Kawasaki disease (KD). Methods : Serum levels of thyroid hormone, TNF-${\alpha}$, IL-6, and NT-proBNP were measured in 52 KD patients in the acute and subacute phase and 10 patients with acute febrile illness (control group). TNF-${\alpha}$ and IL-6 were determined by sandwich enzyme-linked immunosorbent assay (ELISA). Echocardiography was performed to detect coronary artery lesions (CAL) in KD patients. Results : Low $T_3$ syndrome occurred in 63.5% of KD patients. $T_3$ in the acute phase of KD was lower than that in the control. In KD patients, $T_3$ was lowered in the acute phase and elevated in the subacute phase, whereas TNF-${\alpha}$, IL-6 and NT-proBNP were elevated in the acute phase and decreased in the subacute phase. NT-proBNP, and IL-6 were higher in patients with low $T_3$ than in those with normal $T_3$. In addition, $T_3$ inversely correlated with IL-6 and NT-proBNP. Of the 4 patients with CAL, 3 had very low $T_3$. Compared with intravenous immunoglobulin (IVIG)-responsive patients, IVIG-resistant patients had lower $T_3$ and higher IL-6 and NT-proBNP. Conclusion : $T_3$ decreases in the acute phase of KD and normalizes in the subacute phase without thyroid hormone replacement. Low $T_3$ may be partially induced by IL-6 rather than TNF-${\alpha}$, and is strongly associated with high NT-proBNP. $T_3$ in KD may be used for the differential diagnosis, monitoring the activity of the disease, and predicting the severity of inflammation.

• Clinical and Experimental Pediatrics
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• v.52 no.8
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• pp.930-937
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• 2009

Purpose : We evaluated the effectiveness of treatment and cardiac complications of replacing a high dose of aspirin with a high dose of ibuprofen for children in acute phase of Kawasaki disease. We also analyzed the possibility of replacing a high dose of aspirin with a high dose of ibuprofen to prevent complications such as Reye󰡑s syndrome caused by aspirin. Methods : One hundred eight children with Kawasaki disease were admitted in the pediatrics department from January 1, 2004 to December 31, 2008. Echocardiography and laboratory tests were performed during diagnosis, and the children were followed-up at 6-8 weeks after the diagnosis. We retrospectively analyzed their characteristics and clinical results. Results : The children were assigned to receive either a high dose of aspirin with intravenous immunoglobulin (IVIG) (aspirin group) or a high dose of ibuprofen with IVIG (ibuprofen group). A total of 55 and 53 children were included in the aspirin and ibuprofen groups, respectively. The mean defervescence period was 6.5${\pm}$2.1 days in the aspirin group, and $6.9{\pm}1.9$ days in the ibuprofen group (P=0.309). The number of failed treatments, during and after treatment, was 8 in the aspirin group and 10 in the ibuprofen group (P=0.547). There were 11 initial cardiac complications in the aspirin group, and 14 in the ibuprofen group, but children who showed improvement at follow-up was 7 and 13 in the aspirin and ibuprofen groups, respectively (P=0.133). Laboratory findings were also improved in both groups. Conclusion : We can be considered whether we will replace high dose of aspirin by high dose of ibuprofen in acute phase of Kawasaki disease. Therefore, we can prevent the severe complications of aspirin use, such as Reye's syndrome.