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Background In recent days, the prevalence of childhood metabolic syndrome (MS) has increased substantially due to the increasing rate of childhood obesity on a global scale. The aims of this study were to detect the important parameters and provide the screening system to prevent cardiovascular disease in adolescents with MS. Methods Ninety one male adolescents were divided into two groups based on the presence or absence of MS. Anthropometric measurement and laboratory study were studied. Intimal medial thickness and pulse wave velocity were estimated. Left ventricular mass index (LVMI), ejection fraction, myocardial velocity, strain and strain rate were measured by tissue Doppler imaging and strain rate imaging. Results The prevalence of MS was 7.7%. Weight, body mass index (BMI), waist circumference (WC), glucose, insulin, homeostasis model assessment of insulin resistance, triglyceride and LVMI were significantly increased in the MS group. High density lipoprotein-cholesterol (HDL-C), peak early diastolic myocardial velocity (e'), systolic myocardial velocity (s') and global longitudinal strain were significantly lower in the MS group. In univariant analysis, LVMI was significantly correlated with BMI, WC, fat %, fat mass, systolic blood pressure, alanine aminotransferase, total cholesterol (TC) and low density lipoprotein-cholesterol. e' was significantly correlated with BMI, fat %, fat mass, and HDL-C. Global circumferential strain had significant correlation with glucose and TC. Basal anterolateral strain rate was significantly correlated with weight, BMI, WC, fat %, and fat mass. Conclusion LVMI, strain and strain rate are practical and accurate parameters for assessment of left ventricular function in adolescents with MS.

Background: Idiopathic cardiomyopathies (CMPs) are an important heterogeneous group of diseases. With the advance of therapeutic strategies, epidemiologic data on CMP have become very important, but only a few have been reported in Asian children. We conducted a retrospective epidemiologic study of primary CMP in Korean children. Methods and Results: Using a multicenter survey, we studied primary CMP among Korean children from January 1998 to December 2006 based on classification (2006) of CMP by the American Heart Association. A total of 277 primary CMP patients were reported from 17 cardiovascular centers. The average annual occurrence of new cases of primary CMP was 0.28 per 100,000 Korean children younger than 15 years of age (95% confidence interval (CI) 0.24-0.31). Dilated CMP (DCMP) was 66.43%, hypertrophic CMP (HCMP) 23.47%, restrictive CMP (RCMP) 6.50% and others 3.61%. The point prevalence of primary CMP at the end of the study was estimated as 2.11/100,000 (95%CI 1.83-2.43), DCMP 1.39/100,000, HCMP 0.51/100,000, RCMP 0.16/100,000 and others 0.04/100,000. Survival rates over 9 years were 69.8% in DCMP, 90.3% in HCMP, and 47.2% in RCMP. Conclusions: Recent point prevalence of childhood primary CMP in Korea was estimated as 2.11/100,000. Further epidemiologic study with a nationwide survey is necessary. (Circ J 2011; 75: 2228-2234)

Purpose : Adiponectin is an endogenous modulator of vascular remodeling that suppresses vascular inflammation. However, the role of adiponectin in Kawasaki disease (KD) has not been elucidated. The purpose of this study is to investigate the correlation between serum adiponectin level and several parameters, such as interleukin (IL)-6, tumor necrosis factor (TNF)-α, lipid profile, and C reactive protein (CRP), and to clarify the association between adiponectin and cardiac function. Methods : Twenty-two KD patients (22 patients in acute phase and 20 patients in subacute phase) were enrolled in the study group. The control group consisted of 31 subjects (13 febrile patients and 18 healthy children). Both groups underwent blood sampling and tissue Doppler imaging (TDI). Results : CRP was significantly increased in the KD group compared with the control group. There were no significant differences in serum TNF-α, IL-6, and adiponectin levels between groups. However, a negative correlation was found between adiponectin level and CRP level or platelet count. Systolic myocardial velocity and A myocardial velocity measured by TDI were decreased significantly in the acute KD group compared with the subacute KD group and control group. Positive correlations were found between adiponectin level and systolic myocardial velocity or A myocardial velocity. Conclusion : In acute KD patients, low adiponectin level was related to severe inflammatory reactions and decreased left ventricular functions. (Korean J Pediatr 2010;53:41-47)

Purpose Tumor necrosis factor (TNF)-α is thought to contribute to pulmonary hypertension. We aimed to investigate the effect of infliximab (TNF-α antagonist) treatment on pathologic findings and gene expression in a monocrotaline-induced pulmonary hypertension rat model. Methods Six-week-old male Sprague-Dawley rats were allocated to 3 groups: control (C), single subcutaneous injection of normal saline (0.1 mL/kg); monocrotaline (M), single subcutaneous injection of monocrotaline (60 mg/kg); and monocrotaline + infliximab (M+I), single subcutaneous injection of monocrotaline plus single subcutaneous injection of infliximab (5 mg/kg). The rats were sacrificed after 1, 5, 7, 14, or 28 days. We examined changes in pathology and gene expression levels of TNF-α, endothelin-1 (ET-1), endothelin receptor A (ERA), endothelial nitric oxide synthase (eNOS), matrix metalloproteinase (MMP)2, and tissue inhibitor of matrix metalloproteinase (TIMP). Results The increase in medial wall thickness of the pulmonary arteriole in the M+I group was significantly lower than that in the M group on day 7 after infliximab treatment (P

Purpose The incidence of Kawasaki disease (KD) is rare in young infants (less than 3 months of age), who present with only a few symptoms that fulfill the clinical diagnostic criteria. The diagnosis for KD can therefore be delayed, leading to a high risk of cardiac complications. We examined the clinical characteristics and measured the serum levels of N-terminal pro-brain natriuretic peptide (NT-proBNP) levels of these patients for assessing its value in the early detection of KD. Methods We retrospectively reviewed the data of young infants diagnosed with KD from 2004 to 2012. The control group included 20 hospitalized febrile patients. Laboratory data, including NT-proBNP were obtained for each patient in both groups. Results Incomplete KD was observed in 21/24 patients (87.5%). The mean fever duration on admission was 1.36±1.0 days in the KD group. Common symptoms included erythema at the site of Bacille Calmette-Guerin inoculation (70.8%), skin rash (50.0%), changes of oropharyngeal mucosa (29.1%), and cervical lymphadenopathy (20.8%). The mean number of major diagnostic criteria fulfilled was 2.8±1.4. Five KD patients (20.8%) had only one symptom matching these criteria. The incidence of coronary artery complications was 12.5%. The mean serum NT-proBNP level in the acute phase, in the KD and control groups, were 4,159±3,714 pg/mL and 957±902 pg/mL, respectively, which decreased significantly in the convalescent phase. Conclusion Incomplete KD was observed in 87.5% patients. Serum NT-proBNP might be a valuable biomarker for the early detection of KD in febrile infants aged

Background and Objectives We sought to determine whether high-dose aspirin is necessary for the acute therapy of Kawasaki disease (KD) in the intravenous immunoglobulin (IVIG) era. Subjects and Methods Two groups of KD patients treated during the different periods were included. Study group (n=51, treated with IVIG without concomitant use of aspirin in the acute phase) was compared with control group (n=129, treated with IVIG plus high-dose aspirin) with regard to the response to IVIG, duration of fever after IVIG completion, time to C-reactive protein (CRP)

Purpose Kawasaki disease (KD) is the main cause of acquired heart disease in children. In addition to cardiovascular involvement, many complications have been recognized in KD. However, respiratory complications have been rarely reported. We investigated the differences in clinical characteristics, laboratory findings, radiography findings, and echocardiography findings of Mycoplasma pneumoniae infection and other types of pneumonia in KD patients. Methods Among 358 patients with KD, 54 developed concurrent pneumonia. Among the 54 patients, 12 (22.2%) with high titers of anti-M. pneumoniae antibody (AMA) (>1:640) were grouped in the M. pneumoniae group and 42 were included in the control group. Serum AMA was measured in each patient. Clinical laboratory findings and total duration of fever were analyzed. Results The duration of fever, serum hemoglobin, white blood cell count, platelet count, erythrocyte sedimentation rate, C-reactive protein level, albumin level, and the incidence of coronary arterial lesions showed no statistical difference in the 2 groups. Neutrophil count was significantly higher in the M. pneumoniae group than in the control group. Among various radiography findings observed in pneumonia, consolidation and pleural effusion were more frequent in the M. pneumoniae group than in the control group. On the other hand, parahilar peribronchial opacification, diffuse interstitial lesion, and normal findings prevailed in the control group. Conclusion KD patients can have concurrent infections, especially pulmonary symptoms. The cause of KD is likely to be associated with M. pneumoniae infection. Thus, immediate treatment of M. pneumoniae infection in KD patients is very important.

Background and Objectives This was a multicenter study to evaluate the usefulness of the tumor necrosis factor-alpha (TNF-α) blocker infliximab for treatment of Korean pediatric patients with refractory Kawasaki disease (KD). Subjects and Methods Data from 16 patients throughout Korea who were diagnosed with refractory KD and received infliximab were collected retrospectively. Results Complete response to therapy with cessation of fever occurred in 13 of 16 patients. C-reactive protein (CRP) concentrations decreased following infliximab infusion in all 14 patients in whom it was measured before and after treatment. There were no infusion reactions or complications associated with infliximab except in 1 case with acute hepatitis occurring during treatment followed by calculous cholecystitis 4 months later. Fifteen patients had coronary artery (CA) abnormalities before infliximab therapy. Three had transient mild dilatation and 9 had CA aneurysms, with subsequent normalization in 4 patients, persistent mild dilatation in 3, persistent aneurysm in 2, and there were 3 cases (2 with CA aneurysm, 1 with mild CA dilatation) without follow-up echocardiography. Conclusion The results of this study suggest that infliximab may be useful in the treatment of refractory KD, and it appears that there is no significant further progression of CA lesions developing after infliximab treatment. Multicenter trials with larger numbers of patients and long-term follow-up are necessary to assess the clinical efficacy and safety of infliximab in refractory KD.

Purpose : We investigated the relationship between thyroid hormone and serum tumor necrosis factor (TNF), 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, 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 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 T3 syndrome occurred in 63.5% of KD patients. T3 in the acute phase of KD was lower than that in the control. In KD patients, T3 was lowered in the acute phase and elevated in the subacute phase, whereas TNF, 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 T3 than in those with normal T3. In addition, T3 inversely correlated with IL-6 and NT-proBNP. Of the 4 patients with CAL, 3 had very low T3. Compared with intravenous immunoglobulin (IVIG)-responsive patients, IVIG-resistant patients had lower T3 and higher IL-6 and NT-proBNP. Conclusion : T3 decreases in the acute phase of KD and normalizes in the subacute phase without thyroid hormone replacement. Low T3 may be partially induced by IL-6 rather than TNF, and is strongly associated with high NT-proBNP. T3 in KD may be used for the differential diagnosis, monitoring the activity of the disease, and predicting the severity of inflammation.

Purpose : Indomethacin is widely used for the prophylaxis and treatment of patent ductus arteriosus (PDA); however, it is associated with side effects such as renal failure, intraventricular hemorrhage, and gastrointestinal bleeding. Intravenous ibuprofen has been shown to be as effective as indomethacin in prompting PDA closure. If treatment with oral ibuprofen is as effective as indomethacin, it would have the advantages of greater availability, simpler administration, and lower cost. We conducted this study to compare the efficacy and side effects of indomethacin with those of oral ibuprofen, vis- a-vis on the pharmacological closure of PDA. Methods : As a randomized double-blind study, 34 preterm infants with respiratory distress syndrome and hemodynamically significant PDA were treated with either intravenous indomethacin or oral ibuprofen. Echocardiography was performed by one cardiologist who was blind to the treatment that any given infant received. The rate of ductal closure, the need for additional drug treatment or surgical ligation, clinical outcome, and the side effects of drug treatment were compared. Results : Ductal closure occurred in 16 of 18 patients (88.9%) from the indomethacin group and in 14 of 16 patients (87.5%) from the ibuprofen group (P>0.05). Three patients in the indomethacin group and four in the ibuprofen group required a second drug treatment (P>0.05). Three patients (i.e., one patient in the indomethacin group and two in the ibuprofen group) underwent surgical ligation (P>0.05). Between the two groups, there was no significant difference vis- a-vis in side effects or clinical outcome. Conclusion : Compared to indomethacin, oral ibuprofen has the advantages of simpler administration and lower cost, while being as effective; in addition, there are no differences between the two drug treatments with regards to side effects or clinical outcomes. Therefore, the widespread use of oral ibuprofen should be considered in treating PDA in preterm infants. (Korean J Pediatr 2008;51:956-963)

Purpose : Despite the seriousness of bacterial meningitis in children, there is little information on the incidence, causative organisms, mortality rate and age distribution. We studied the frequency by age group and causal pathogens, and clinical characteristics in children with bacterial meningitis in the private sector in Korea. Methods : The medical records containing the data on bacterial meningitis patients under 18 years of age confirmed by cerebrospinal fluid (CSF) findings were retrospectively analyzed from September, 1993 to August, 2006 at Ewha Womans University Mokdong Hospital. Results : Eighty-one cases of bacterial meningitis were observed. Overall the most common organism was Streptococcus agalactiae (group B streptococcus, GBS) (30 cases, 37.0%) followed by Haemophilus influenzae (22 cases, 27.2%), Streptococcus pneumoniae (12 cases, 14.8%), Escherichia coli (3 cases, 3.7%), Neisseria meningitidis (1 case, 1.2%) and others (13 cases, 16.0%). In neonates and young infants under 2 months, the most common organism was GBS. In children between 3 months, and 5 years, the most common organism was H. influenzae. S. pneumoniae was the most common organism in children over 5 years of age. Thirty-one patients (38.3%) had complications. Of all ages, the mortality rate of bacterial meningitis markedly decreased compared with the previously reported rate. Conclusion : In neonates, GBS meningitis was most common. The frequency of H. influenzae meningitis decreased after the introduction of H. influenzae type b vaccination. A strategy for the prevention of GBS meningitis in neonates should be established. The influence of the pneumococcal conjugate vaccine on S. pneumoniae meningitis should be studied.

= Abstract = A group of patients with Kawasaki disease (KD) initially present with cervical lymphadenitis or deep neck infection. These unusual KD presentations lead to unnecessary antibiotic therapy or surgical intervention, thereby delaying intravenous immu noglobulin treatment and increasing the risk of coronary artery damage. We present four KD patients whose initial presenta tions mimicked a retropharyngeal abscess. Nonsuppurative cervical lymphadenitis or suspected neck abscess unresponsive to intravenous antibiotics could signal the possibility of KD. (Korean J Pediatr 2008;51:1023-1027)

Foreign body aspiration (FBA) is a common accident in young children. Undiagnosed and retained foreign bodies may result in severe early and late complications such as asphyxia, pneumonia, atelectasis and bronchiectasis. Moreover, because it can mimic bronchiolitis, croup or asthma, an accurate history and a high index of suspicion are of paramount importance for early diagnosis. With our experience on bronchial FBA initially misdiagnosed as acute bronchiolitis, we emphasize that a minute radiological finding should not be neglected and a repeat chest radiograph may be helpful when the initial study shows normal findings. Multidetector computed tomography is a very useful noninvasive diagnostic modality for FBA.

목 적 : 학동기 아동에서의 가와사끼병은 드물게 나타나며 대부분 불완전한 양상을 보이므로 진단이 늦어지고 심혈관계 합병증의 발생이 증가한다. 본 연구에서는 이들의 임상 특징을 조사하여 이 연령군에서 가와사끼병의 조기 진단에 도움이 되고자 하였다. 방 법 : 1995년 6월부터 2006년 5월까지 가와사끼병으로 입원하...

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-α 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

Purpose:The purpose of this study was to determine whether N-terminal fragment of B-type natriuretic peptide(NT-proBNP) may be used to differentiate acute Kawasaki disease(KD) from other clinically similar diseases. Methods:Using electrochemiluminescence immunoassay, NT-proBNP concentrations were measured in the acute phase within 10 days after the onset of KD(n=58) and in the convalescent phase, 60 to 81 days after the onset(n=51), and also in patients with acute febrile disease as a control(n=34). Echocardiography was performed to detect pericardial effusion(PE) and coronary artery lesions(CAL), and to measure the left ventricular dimension at diastole(LVIDd) and ejection fraction(LVEF). The cutoff value of NT-proBNP for separating KD from other diseases was determined. Results:NT-proBNP concentration in the acute phases of KD was significantly higher than that in the control group(1,501.6±2,132.6 vs. 139.0±88.8 pg/mL, P260 pg/mL may be highly suggestive of acute KD. NT- proBNP may be used as a diagnostic tool for KD.