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Author's summary Hypertrophic cardiomyopathy (HCM) in infancy is rare and many fulminant cases are fatal. Infantile HCM shows a rapid progressive clinical course and different characteristics compared with late-onset HCM presenting during the prepubertal age. There are also spontaneously resolving phenotypes of HCM that are diagnosed in neonates being treated for bronchopulmonary dysplasia with corticosteroids or in those with other problems related to maternal endocrine diseases. The pathophysiology of infantile HCM has not been well described. Therefore, this review updates the pathophysiology of infantile HCM and includes molecular studies on maturation of cardiomyocytes from a clinician's point of view., Hypertrophic cardiomyopathy (HCM) is characterized by ventricular wall hypertrophy with diastolic dysfunction. Pediatric HCM is distinguished from the adult in many aspects. Most children with HCM do not present clinically until the adolescent period, even when they are born with genetic mutations. Some infants with early-onset HCM present with massive progressive myocardial hypertrophy in the first few months of life, which is often fatal. The mortality of pediatric HCM peaks during the infantile and adolescent periods. These periods roughly correlate with children's growth spurt. Non-sarcomeric causes of HCM are more frequent in pediatric HCM, while sarcomeric causes are more common in adults. From the perspective of cardiac development, the fetal heart has immature cardiomyocytes, which are characterized by proliferation and exit their cell cycles with a decreased regenerative property after birth. In the perinatal period, there is a dynamic change in maturation of cardiomyocytes from immature to mature cells. Infants who are treated with steroids or born to mothers with diabetes or hyperthyroidism often show phenotypes of HCM, which gradually resolve. With remarkable advancement of molecular biology, understanding on maturation of cardiomyocytes has increased. Neonates undergo abrupt environmental changes during the transitional circulation, which is affected by oxygen, metabolic and hormonal fluctuations. Derangement in physiological transition to the normal postnatal environment may influence maturation of proliferative immature cardiomyocytes during early infancy. This article reviews updates of infantile HCM and recent molecular studies related to maturation of cardiomyocytes from the clinical point of view of identifying distinct characteristics of infantile HCM.

Background and objectives Hypertension is becoming one of the most common health conditions in children and adolescents due to increasing childhood obesity. We aimed to provide the auscultatory blood pressure (BP) normative reference values for Korean non-overweight children and adolescents. Methods BP measurements in children and adolescents aged 10 to 18 years were performed in the Korean National Health and Nutrition Examination Survey (KNHANES) from 1998 to 2016. BP was measured using a mercury sphygmomanometer. Sex-, age- and height-specific systolic BP (SBP) and diastolic BP (DBP) percentiles were calculated in the non-overweight children (n=10,442). We used the General Additive Model for Location Scale and Shape method to calculate BP percentiles. Results The 50th, 90th, 95th, and 99th percentiles of SBP and DBP tables and graphs of non-overweight children and adolescents aged 10 to 18 years were presented by age and height percentiles. We found that the SBP and DBP at the 95th percentile were well correlated with height. The BP tables presented by height contained BP values from 124 cm to 190 cm for boys and from 120 cm to 178 cm for girls. Boys had higher SBP and DBP. Conclusions We provided the sex-, age- and height-specific auscultatory BP values using the KNHANES big data. These may be useful in diagnosis and treatment of hypertension in Korean children and adolescents.

Graves disease (GD) can lead to complications such as cardiac arrhythmia and heart failure. Although dilated cardiomyopathy (DCMP) has been occasionally reported in adults with GD, it is rare in children. We present the case of a 32-month-old boy with DCMP due to GD. He presented with irritability, vomiting, and diarrhea. He also had a history of weight loss over the past few months. On physical examination, he had tachycardia without fever, a mild diffuse goiter, and hepatomegaly. The chest radiograph showed cardiomegaly with pulmonary edema, while the echocardiography revealed a dilated left ventricle with an ejection fraction (EF) of 28%. The thyroid function test (TFT) showed elevated serum T3 and decreased thyroid stimulating hormone (TSH) levels. The TSH receptor autoantibody titer was elevated. He was diagnosed with DCMP with GD; treatment with methylprednisolone, diuretics, inotropics, and methimazole was initiated. The EF improved after the TFT normalized. At follow-up several months later, although the TFT results again showed evidence of hyperthyroidism, his EF had not deteriorated. His cardiac function continues to remain normal 1.5 months after treatment was started, although he still has elevated T3 and high TSH receptor antibody titer levels due to poor compliance with drug therapy. To summarize, we report a young child with GD-induced DCMP who recovered completely with medical therapy and, even though the hyperthyroidism recurred several months later, there was no relapse of the DCMP.

Purpose: There are no specific tests for diagnosing Kawasaki disease (KD). Additional diagnostic criteria are needed to prevent the delayed diagnosis of incomplete Kawasaki disease (IKD). This study compared the frequency of coronary artery lesions (CALs) in IKD patients with and without anterior uveitis (AU) and elucidated whether the finding of AU supported the diagnosis of IKD. Methods: This study enrolled patients diagnosed with IKD at The Catholic University of Korea, Uijeongbu St. Mary’s Hospital from January 2010 to December 2014. The patients were divided into 2 groups: group 1 included patients with IKD having AU; and group 2 included patients with IKD without AU. We analyzed the demographic and clinical data (age, gender, duration of fever, and the number of diagnostic criteria), laboratory results, and echocardiographic findings. Results: Of 111 patients with IKD, 41 had uveitis (36.98%, group 1) and 70 did not (63.02%, group 2). Patients in group 1 had received a diagnosis and treatment earlier, and had fewer CALs (3 of 41, 1.7%) than those in group 2 (20 of 70, 28.5%) (P=0.008). All 3 patients with CALs in group 1 had coronary dilatation, while patients with CALs in group 2 had CALs ranging from coronary dilatation to giant aneurysm. Conclusion: The diagnosis of IKD is challenging but can be supported by the presence of features such as AU. Group 1 had a lower risk of coronary artery disease than group 2. Therefore, the presence of AU is helpful in the early diagnosis and treatment of IKD and can be used as an additional diagnostic tool.

Kawasaki disease (KD) may be an acute systemic immune-mediated disease which occurs after infection of unknown KD pathogen(s). The aim of this study is to evaluate the changes in platelet count and immunoglobulin (Ig) levels (IgG, IgM, IgA, and IgE) during hospitalization. Forty-three patients with complete KD who received intravenous Ig at 2 g/kg were enrolled in South Korea. The platelet count and Ig levels of the patients were examined twice at presentation and around discharge (mean 6.2 ± 2.4 days apart) and the relationships between platelet level and Ig levels were evaluated. The mean patient age was 31 ± 18 months; 28 patients were male and 15 were female. The values of all parameters measured, with the exception of IgE, were significantly increased at the second examination compared with their values at presentation. These values gradually increased over time after fever onset, over periods ranging from 2 to 16 days. The extent by which platelet levels increased over these 2 time points was correlated with the extents by which IgG (P

Dilated cardiomyopathy (DCMP) remains a life threatening disease in young patients and is often difficult to differentiate from myocarditis. Early recognition and treatment of DCMP are crucial for good prognoses in this patient population. The clinical course of patients with DCMP that result in cardiogenic shock varies according to the etiology as well as patient age. The volumetric expansion of the enlarged heart can compress adjacent structures causing a number of related symptoms, especially in infants with soft cartilaginous bronchi. Therapeutic strategies for treating these issues vary according to the type of complication encountered. We report a case of severe DCMP with sudden onset of massive cardiomegaly with heart failure complicated by bronchial obstruction in an infant.

BACKGROUND The goal of this study is to evaluate the changes in the epidemiologic and clinical features of Kawasaki disease (KD) in a period of 20 years. METHODS We compared the epidemiologic features of 3 KD patient groups: those admitted from 1987 to 1990 (122 cases), from 1997 to 2000 (137 cases) and from 2007 to 2010 (102 cases). For clinical features, we reviewed the medical records of patients admitted from 1996 to 2000 (141 cases) and from 2006 to 2010 (121 cases). RESULTS There were a total of 772 KD patients during the study period (mean age: 27.2 months; male-to-female ratio: 1.6:1). There were some variations in the mean ages (23.8, 27.8 and 30 months), in the male-to-female ratios (1.9:1, 1.3:1 and 1.8:1) and in the age distributions, including the patients of ages 5 years, but the differences were not statistically significant across the groups. The seasonal distributions were relatively consistent with some variations. In clinical features, the preadmission and total fever durations were shorter (P < 0.001, respectively), and the proportions of incomplete KD tended to increase, and the rates of coronary artery aneurysm tended to decrease in recently admitted patients. CONCLUSIONS There were no significant changes in the demographic and seasonal features of KD, although the evaluation of the clinical features showed shorter fever duration suggestive of improved identification and treatment of KD over time.

Respiratory difficulty resulting from congenital chylothorax is usually relieved by postnatal thoracentesis, closed chest drainage, and oxygen therapy. However, early occurrence of congenital chylothorax or accumulation of a large amount of chylous fluid sometimes leads to pulmonary hypoplasia or persistent pulmonary hypertension of the newborn, which requires further customized mechanical ventilatory support. In these cases, conventional mechanical ventilation is primarily used during initial treatment and is later replaced by high-frequency ventilation, but the advantages of inhaled nitric oxide treatment have rarely been described. This case suggests the benefits of inhaled nitric oxide in patients with congenital chylothorax, even when mechanical ventilation cannot improve respiratory distress because of severe pulmonary hypertension of the newborn leading to right cardiac dysfunction and possibly cholestasis.

REFER TO THE PAGE 123-127 Recent advancement of technology of medical imaging systems has enabled in-depth real time spatiotemporal diagnosis of human heart disease. It is regardless to mention that prognosis of cardiac disease is based critically on prompt 3 dimensional (3D) diagnosis of a structural anomaly, functional analysis and followed by a proper treatment. Although we are in current era of 3D skills in the spotlight, yet real time 3D echocardiography (RT3DE) is not so appealing to very busy pediatric and adult cardiologists. Mental reconstruction of harvested 2 dimensional (2D) echocardiographic images is a still much faster and simpler way to understand complex cardiac structural anomaly than doing a few more steps for subsequent data analysis with a software program of RT3DE. On the other hand, considering superiority of function of volume assessment, RT3DE can be very useful in clinical application as it can provide numeric data more precisely than morphologic visual assessment of each cardiac chambers that enables assessment longitudinally during the clinical course. In congenital heart disease (CHD), volume or pressure overloaded condition frequently results in distortion of septal planes followed by a geometric change in either ventricles that hampers precise ventricular volume assessment using conventional 2D echocardiography.1),2) There are many reports on the reproducibility of RT3DE in clinical application to adults population without a complex heart disease.3) It is known that current RT3DE has relatively a high intraobserver and interobserver variability.4) In pediatric cases with a complex CHD, additional time consuming effort is required for the assessment of detailed anatomical defect during navigation of intriguing small cardiac structures to acquire optimal planes for secondary 3D reconstruction. Novel imaging tools including RT3DE always require its validation steps. Many studies on the left ventricular volume and ejection fraction compared to cardiac magnetic resonance imaging (MRI) data, currently a gold standard method of cardiac volume assessment have been published during the last ten years.5),6) It is known that the results acquired by RT3DE of left ventricular volume assessment has strong correlation with cardiac MRI although ventricular volume by RT3DE has tendency of underestimation.6),7),8) Studying with cardiac MRI in pediatric patients in critical condition with CHD has several limitations as to its long scanning time and practical issues on patient's sedation and infeasibility in patients with pacemaker. If we mention about volumetric assessment of right ventricle considering its 3 dimensionally distorted morphology compared to left ventricle, there should be no doubt on the superiority of RT3DE to 2D echocardiography and related issues are published.9),10) As RT3DE is a noninvasive method, if it can overcome current several issues of its limitation, it might be the most useful tool to examine children with CHD. Many experts in the field of echocardiography believe that its clinical application by skillful clinicians will provide infinite derivative outcomes in longitudinal monitoring of functional abnormality especially in cases with surgically corrected complex CHD.11),12) However in reality, likewise cardiac MRI, RT3DE is still a secondary option to conventional 2D echocardiography with its limitations and infeasibility to pediatric populations with CHD. Therefore, further studies on this issue are strongly encouraged for further analysis. An inspiring outcome shown here titled 'Assessment of left ventricular volume and function using real-time 3D echocardiography versus angiocardiography in children with Tetralogy of Fallot' is that RT3DE can be practically and convincingly used in volumetric assessment in CHD patients with real time and non-invasive scanning.13) Authors tried to validate RT3DE comparing with angiographic data. Considering hard working in assessment of complicated geometric deformation of left ventricle in tetralogy of Fallot and technical difficulty in handling pediatric patients, this study is deserved the spotlight. They showed that there was a strong correlation between the mean value of the left ventricular end diastolic volume and its index measured by RT3DE and angiography with good intra- and inter-observer reliability. With training time and experience, unquestionably a simple 'crop box' will give us an infinitely useful information with simplified off-line analysis of harvested images with a cutting-edge software program. With an optimistic view towards the RT3DE in CHD, current doubt on values of RT3DE hopefully should be faded with an accelerated speed with the help of advancement of technology in the near future.

This study examines online social media diffusion across different countries that will help to provide a picture of current global online social network services (SNSs). Analyzing country-level data drawn from 78 nations, we find that non-technological factors such as culture and language as well as technological factors including Internet penetration rate and smartphone adoption rate have significant effects on online social media diffusion. These findings, derived from a broad range of different countries, not only provide theoretical insights into understanding critical factors that enable successful global expansion of online social media services but also help practitioners plan their marketing strategies more effectively in a global context.

Myocardial infarction (MI) is one of the leading causes of morbidity and mortality world-wide. Whether endogenous repair and regenerative ability could be augmented by drug administration is an important issue for generation of novel therapeutic approach. Recently it was reported that in mice pretreated with thymosin beta 4 (TB4) and subsequently subjected to experimental MI, a subset of epicardial cells differentiated into cardiomyocytes. In clinical settings, epicardial priming with TB4 prior to MI is impractical. Here we tested if TB4 treatment after MI could reprogram epicardium into cardiomyocytes and augment the epicardium's injury response. Using epicardium genetic lineage trace line Wt1CreERT2/+ and double reporter line Rosa26mTmG/+, we found post-MI TB4 treatment significantly increased the thickness of epicardium and coronary capillary density. However, epicardium-derived cells did not adopt cardiomyocyte fate, nor did they migrate into myocardium to become coronary endothelial cells. Our result thus indicates that TB4 treatment after MI does not alter epicardial cell fate to include the cardiomyocyte lineage, providing both cautions and insights for the full exploration of the potential benefits of TB4 in the clinical settings. This article is part of a Special Issue entitled ‘Possible Editorial’.

The epicardium makes essential cellular and paracrine contributions to the growth of the fetal myocardium and the formation of the coronary vasculature. However, whether the epicardium has similar roles postnatally in the normal and injured heart remains enigmatic. Here, we have investigated this question using genetic fate-mapping approaches in mice. In uninjured postnatal heart, epicardial cells were quiescent. Myocardial infarction increased epicardial cell proliferation and stimulated formation of epicardium-derived cells (EPDCs), which remained in a thickened layer on the surface of the heart. EPDCs did not adopt cardiomyocyte or coronary EC fates, but rather differentiated into mesenchymal cells expressing fibroblast and smooth muscle cell markers. In vitro and in vivo assays demonstrated that EPDCs secreted paracrine factors that strongly promoted angiogenesis. In a myocardial infarction model, EPDC-conditioned medium reduced infarct size and improved heart function. Our findings indicate that epicardium modulates the cardiac injury response by conditioning the subepicardial environment, potentially offering a new therapeutic strategy for cardiac protection.

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 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 medium-dose 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 ≥65%, C-reactive protein≥100 mg/L, aspartate aminotransferase≥100 IU/L, and alanine aminotransferase≥100 IU/L, as well as 2 points for less than 5 days of illness, and serum sodium level≤136 mmol/L. Using a cutoff point of ≥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.

The lung is enveloped by a layer of specialized epithelium, the pulmonary mesothelium. In other organs, mesothelial cells undergo epithelial–mesenchymal transition and contribute to organ stromal cells. The contribution of pulmonary mesothelial cells (PMCs) to the developing lung has been evaluated with differing conclusions. PMCs have also been indirectly implicated in lung fibrosis in the progressive, fatal lung disease idiopathic pulmonary fibrosis. We used fetal or postnatal genetic pulse labeling of PMCs to assess their fate in murine development, normal lung homeostasis, and models of pulmonary fibrosis. We found that most fetal PMC-derived mesenchymal cells (PMCDCs) expressed markers of pericytes and fibroblasts, only a small minority expressed smooth muscle markers, and none expressed endothelial cell markers. Postnatal PMCs did not contribute to lung mesenchyme during normal lung homeostasis or in models of lung fibrosis. However, fetal PMCDCs were abundant and actively proliferating within fibrotic regions in lung fibrosis models, suggesting that they actively participate in the fibrotic process. These data clarify the role of fetal and postnatal PMCDCs in lung development and disease.

Purpose: This study aimed to evaluate the autonomic imbalance in syncope by comparing the baseline heart rate variability (HRV) between healthy children and those with vasovagal syncope. Methods: To characterize the autonomic profile in children experiencing vasovagal syncope, we evaluated the HRV of 23 patients aged 7–18 years and 20 healthy children. These children were divided into preadolescent (

The authors compared pre-treatment and post-treatment characteristics of 206 prospectively enrolled patients with Kawasaki disease (KD) responsive to intravenous immunoglobulin (IVIG) with those of 23 (10% of total) IVIG non-responders. Demographic characteristics were similar in both groups. Compared to IVIG responders, non-responders had a longer total duration of fever and a higher incidence of coronary artery lesions. Prior to IVIG, non-responders had higher neutrophil differential and C-reactive protein, and lower cholesterol. 24 hours after the IVIG infusion, a total leucocyte count13.1×10(9)/l, neutrophil differential51% and total protein72 g/l showed reasonable sensitivity (91%, 91% and 64%, respectively) and specificity (89%, 76% and 78%, respectively) as independent characteristics of non-response to IVIG. Laboratory parameters before and shortly after IVIG may reflect the severity of inflammation in KD patients and assist in informing further management.

Purpose With feasibility in the diagnoses of congenital heart disease (CHD) in the antenatal period, we suspect changes have occurred in its incidence. No data have been reported about the current incidence of simple forms of CHD in Korea. We have attempted to assess the recent incidence and characteristics of CHD in the neonatal care unit of a secondary referral medical center. Methods Medical records of 497 neonatal care unit patients who underwent echocardiography in the past 5 years were reviewed. Pre-term infants with patent ductus arteriosus and other transient, minimal lesions were excluded from this study. Results Although the number of inpatients remained stable, the incidence of simple forms of CHD showed a gradual decrease over the 5-year study period; a markedly low incidence of complex forms was seen as well. CHD was observed in 3.7% full-term and 6.8% pre-term infants. CHD was observed in 152 infants weighing >2,500 g (3.5% of corresponding birth weight infants); 65 weighing 1,000 to 2,500 g (9.3%); and 6 weighing

Background Kawasaki disease is a leading cause of acquired heart disease in children. The prevalence rate varies in different ethnic groups. Recently, with the clinical application of molecular genetic technology, human leukocyte antigen (HLA) polymorphisms associated with several diseases have been identified by DNA analysis. This study aimed to assess the association of HLA alleles with susceptibility and complications of Kawasaki disease in Korean children. Methods In this study, DNA was extracted from 74 children with a diagnosis of Kawasaki disease. The polymorphisms of the HLA-A, -B, -C, and -DRB1 alleles of patients with Kawasaki disease were determined by polymerase chain reaction (PCR)–amplification refractory mutation system (ARMS) and PCR–sequence-specific primer (SSP) analysis. The polymorphisms identified were compared with those of 159 normal healthy control subjects. Results There was a significant increase in the frequencies of the HLA-B35, -B75, and -Cw09 alleles in patients with Kawasaki disease compared with the healthy control group. There was no increase in the frequency of HLA-DRB1 alleles among the Kawasaki disease patients compared with a healthy control group. When the patients with Kawasaki disease were divided into two subgroups, with or without coronary complications, the Kawasaki disease patients with coronary complications showed a significantly increased frequency of the HLA-DRB1*11 allele compared with the healthy control group and increased frequency of HLA-DRB1*09 in a comparison of the subgroups. Conclusions This study suggests that polymorphisms in some alleles of B and C in HLA class I genes are associated with Kawasaki disease in Korean children.

Background and objectives This study was aimed at assessing left ventricular torsion (LVtor) mechanics using speckle tracking echocardiography (STE), establishing normal reference values of principal LVtor parameters, and analyzing the age-related changes in normal children. Subjects and methods Eighty children (aged 3 months to 15 years) with normal cardiac function and rhythm were recruited. LVtor parameters including rotations, twist and untwist, torsion, and their rate indices were measured using STE. Age and heart rate related changes of the parameters were analyzed. Results Speckle tracking echocardiography analyses for LVtor parameters had excellent reliability in 64 of 80 subjects (80%) (intraclass correlation coefficients; 0.93-0.97). Early systolic twist (EST) motions (-8.4--0.1°) were observed in all subjects during an early 20±7% of systolic time intervals. The peak systolic twist and torsion were 17.0±6.5° and 2.9±1.3°/cm, respectively. The peak twist velocity was recorded at 51±13% of systolic time and the peak untwist velocity at 13.8±11.5% of diastolic time intervals. Multivariate analysis showed that heart rate change was an independent predictor of changes in torsion parameters; significantly decreasing LV length-normalized apical and basal rotation, torsion, and twist and untwist rate with increasing age. Isovolumetric recoil rate was independent of change in age and heart rate. Conclusion Left ventricle showed unique torsion mechanics in children with EST, torsion, and untwists. Heart rate was an independent predictor of the change in torsion parameters with aging.

Acute otitis media (AOM) is one of the most common childhood infectious diseases. Despite antibiotic treatment for AOM, AOM and its complication still continue to develop. Acute mastoiditis is a serious complication of AOM and epidural abscess constitutes the commonest of all intracranial complication of AOM. Neurological complication of acute mastoiditis are rare but can be life threatening. Their presentation may be masked by the use of antibiotics. We report the rare case of acute otitis media progressing to acute mastoiditis, epidural abscess formation and lateral sinus thrombophlebitis caused by Streptococcus pneumoniae in a child. She was admitted with acute otitis media with fever. Despite proper antibiotics, acutemastodititis and epidural abscess were developed, and after surgical drainage and antibiotics therapy she was recovered without sequalae.KeyWords:Epidural abscess, Mastoiditis, Otitis media, Streptococcus pneumoniae

Pathologically, Kawasaki disease (KD) is a systemic vasculitis which can show some complications as arthritis during the natural course. It is observed usually in the acute or subacute stage of 15%–45% of KD patients [3, 4, 5]. The 5 children, out of 217 patients with KD, who presented with arthritis after defervescence on intravenous immunoglobulin (IVIG) treatment were retrospectively reviewed. All patients met the diagnostic criteria of KD at the initial time of presentation and patients 1, 2, 4 and 5 were readmitted because of arthritis. Initially, all patients were treated with IVIG at a dose of 2 g/kg, and with acetylsalicylic acid (30– 50 mg/kg). Two patients (numbers 3 and 4) failed to respond the first dose of IVIG and became afebrile after the second dose of IVIG (1 g/kg). The arthritis was observed at mean of 10.4±1.3 days (range 8 to 13 days) after the onset of fever, and at a mean of 5.8±1.8 days (range 3 to 10 days) after defervescence after the final dose of IVIG. For the types of arthritis, the polyarticular cases showed involvement of all phalangeal joints of both hands with additional involvement of at least one other joint including the left knee joint in patient 1, and bilateral knee, wrist and ankle joints in patient 3. In the pauciarticular types, there was bilateral involvement of hip joints in patient 2, right hip joints and the right third interphalangeal joint in patient 4, and the left knee joint in patient 5. All patients with arthritis responded effectively to high doses of acetylsalicylic acid (80 mg/kg/day for 10–14 days, patients 1 and 2) or ibuprofen (30 mg/ kg/day for 2–4 weeks, patients 4 and 5) or corticosteroids (patient 3). One child (patient 3), after responding to the second dose of IVIG therapy, showed an acute onset of polyarthritis with relapsed fever and elevated laboratory values. She was unresponsive to ibuprofen and received the methylprednisolone pulse therapy (15 mg/kg for 3 days) with ibuprofen for 2 months. There has been no relapse for 6 months after treatment. HLA B27 was negative for all three cases which were examined (Table 1). Children with KD in the acute stage commonly present with oedematous hands and feet, especially in young infants. Considering the pathogenesis of KD as a systemic vasculitis, the swelling and tenderness of extremities may be combined with arthritis. However, in this study, we selected those patients with arthritis which occurred after defervescence with IVIG treatment. Among 217 KD patients observed over a 6-year period (1998–2003), only 5 (2%) showed this type of arthritis. IVIG therapy in KD is more effective than high dose acetylsalicylic acid therapy in inducing defervescence, normalising the laboratory findings and preventing cardiac complication [2, 6]. Because the anti-inflammatory effect of IVIG on arthritis also is more effective than that of acetylsalicylic acid, the incidence of arthritis in the subacute stage of KD has decreased in the IVIG era. Arthritis after infection with Streptococcus, Shigella or Chlamydia is known as reactive arthritis or postinfectious arthritis. Reactive arthritis develops within 1–4 weeks after the primary infection and some patients are positive for HLA B27 [1]. The fact that arthritis occurs regardless of high dose acetylsalicylic acid therapy in the subacute stage [3] and after high dose IVIG therapy suggests that this type of arthritis in KD is a reactive arthritis due to an unknown pathogen.

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.

Congenital central hypoventilation syndrome (CCHS) or Ondine's curse is a very rare sleep disorder that is the result of a congenital failure of the autonomic control of ventilation caused by insensitivity of the chemoreceptor to hypercapnea during sleep. Gastrointestinal motility disorders, particularly a congenital megacolon (Hirschsprung disease) is often combined with CCHS. This combination can be explained by a defect in the migration of neuronal cells from the neural crest (neurocristopathy) during the intrauterine period. A diagnosis of CCHS is made by confirming the failure of adequate ventilation in response to hypercapnea and hypoxia during sleep and the exclusion of other diseases. Young infants frequently show atypical clinical courses, and their conditions are frequently complicated with the long-term sequela of hypoxemic episodes. Therefore, a high index of suspicion and active treatment with mechanical ventilation are important for reducing recurrent hypoxemic episodes in the neonatal period. This paper reports the follow up of a case of CCHS in a neonate who showed frequent intractable apnea and cyanosis and was given artificial mechanical ventilation during sleep.