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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.

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.

In the present study, the catalytic partial oxidation of methane (CPOM) over various active metals supported on CeO2 (M/CeO2, M = Ir, Ni, Pd, Pt, Rh and Ru) has been investigated. The catalysts were characterized by X-ray diffraction (XRD), BET surface area, H2-temperature programmed reduction (H2-TPR), CO chemisorption and transmission electron microscope (TEM) analysis. Ir/CeO2 catalysts showed higher BET surface area, higher metal dispersion, small active metal nano-particles (approximately 3 nm) than compared to other M/CeO2 catalysts. The catalytic tests were carried out in a fixed R(mix) ratio of 2 (CH4/O2) in a fixed-bed reactor, operating isothermally at atmospheric pressure. From time-on-stream analysis at 700 degrees C for 12 h, a high and stable catalytic activity has been observed for Ir/CeO2 catalysts. TEM analysis of the spent catalysts showed that the decrease in the catalytic activity of Ni/CeO2 and Pd/CeO2 catalysts is due to carbon formation whereas no carbon formation has been observed for Ir/CeO2 catalysts.

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.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.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.

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.