Development of necrotizing enterocolitis in full-term infants with duct dependent congenital heart disease.

BACKGROUND: Although many studies have described an increased risk of necrotizing enterocolitis in duct dependent congenital heart diseases, very few have investigated its occurrence in full-term infants with duct dependent congenital heart diseases. METHODS: To evaluate the characteristics and risk factors of necrotizing enterocolitis, we performed a retrospective review of 355 full-term infants with duct dependent congenital heart diseases who received prostaglandin E1 therapy from April 2000 to May 2020. RESULTS: Necrotizing enterocolitis was observed in 10 patients (3.0%). Their average gestational age and birth weight were 38.2 weeks and 2783.5 g, respectively. The median age at diagnosis was 8.0 days (2-70 days). One patient was diagnosed with necrotizing enterocolitis stage IIA, five with stage IIB, two with stage IIIA, and two with stage IIIB; two (20%) received surgical treatment. The duct dependent pulmonary circulation group had higher frequencies of necrotizing enterocolitis (4.4%) than the duct dependent systemic circulation (2.0%) and parallel circulation (1.3%) groups. The necrotizing enterocolitis and the other groups had significantly different birth weight (2783.5 g vs 3170.9 g, respectively) and gestational age (38.2 weeks vs 39.1 weeks, respectively). Gestational age under 38 weeks (OR 8.87, p = 0.002), birth weight of < 2500 g (OR 5.1, p = 0.042), need for mechanical ventilation (OR 4.6, p = 0.021), parenteral nutrition (OR 107.7, p < 0.001), and functional single ventricle (OR 5.8, p = 0.009) were significant risk factors. The case-fatality rate was higher in the necrotizing enterocolitis (40.0%) than in the other group (8.3%, p = 0.009). CONCLUSIONS: Three percent of full-term infants with duct dependent congenital heart diseases developed necrotizing enterocolitis. Neonates with low birth weight, gestational age less than 38 weeks, functional single ventricle, or receiving assisted mechanical ventilation or parenteral nutrition are at increased risk.

Extremely Early Onset Transthyretin Familial Amyloid Polyneuropathy with a Leu55Pro Mutation: A Pediatric Case Report and Literature Review.

Transthyretin familial amyloid polyneuropathy (TTR-FAP) is a life-threatening autosomal dominant disease caused by the deposition of amyloid fibrils composed of TTR proteins. Symptoms of this disease include progressive sensorimotor neuropathy, cardiomyopathy, and involvement of other organs. We described a pediatric case of extremely early onset TTR-FAP with a TTR Leu55Pro mutation. A 17-year-old boy began to suffer from lower limb weakness, gait disturbance, and decreased sensation from 14 years of age onward. He presented with hypertrophic cardiomyopathy, periorbital and scleral ecchymosis, anhidrosis, orthostatic intolerance, and gastrointestinal autonomic dysfunction including nausea, vomiting, and diarrhea alternating with constipation. The patient's older sister had developed similar gastrointestinal symptoms from 20 years of age onward and was diagnosed as having hypertrophic cardiomyopathy. The boy's biopsy results showed infiltrated amyloid deposition on subcutaneous fat tissue and endocardium. Genetic analysis of the TTR gene demonstrated that both the patient and his sister had a pathogenic mutation, c.224T > C (Leu55Pro). Both patients were prescribed tafamidis, a TTR stabilizing agent. Although a majority of TTR-FAPs occur during adulthood, it should be suspected, even in pediatric populations, when symmetric length dependent neuropathy occurs in conjunction with a family history of neuropathy, autonomic neuropathy, and/or cardiomyopathy.

Acute kidney injury after using contrast during cardiac catheterization in children with heart disease.

Acute kidney injury (AKI) is closely associated with the mortality of hospitalized patients and long-term development of chronic kidney disease, especially in children. The purpose of our study was to assess the evidence of contrast-induced AKI after cardiac catheterization in children with heart disease and evaluate the clinical usefulness of candidate biomarkers in AKI. A total of 26 children undergoing cardiac catheterization due to various heart diseases were selected and urine and blood samples were taken at 0 hr, 6 hr, 24 hr, and 48 hr after cardiac catheterization. Until 48 hr after cardiac catheterization, there was no significant increase in serum creatinine level in all patients. Unlike urine kidney injury molecule-1, IL-18 and neutrophil gelatinase-associated lipocalin, urine liver-type fatty acid-binding protein (L-FABP) level showed biphasic pattern and the significant difference in the levels of urine L-FABP between 24 and 48 hr. We suggest that urine L-FABP can be one of the useful biomarkers to detect subclinical AKI developed by the contrast before cardiac surgery.

Natural History and Influencing Factors of Chronic Urticaria in Children.

PURPOSE: Chronic urticaria (CU) can reduce the quality of life of children and their parents, but there are only a few studies on the course of CU in children. This study aimed to investigate the natural course of CU in children and identify the factors that influence its prognosis. METHODS: We evaluated 77 children diagnosed with CU, who were monitored for at least 48 months. Subjects were classified as either chronic spontaneous urticaria (CSU) or other CU, and the clinical features were compared. Remission was defined as having no symptoms without treatment for more than 1 year. The remission rate was analyzed, and the factors influencing the prognosis were investigated. RESULTS: The average age of the study population was 5.96 ± 4.06 years, and 64 (83.1%) patients had CSU. The remission rates at 6 months, 1 year, 2 years, 3 years, and 4 years after symptom onset were 22.1%, 40.3%, 52.0%, 63.7%, and 70.2%, respectively, for children with CU. For children with CSU, these values were 23.4%, 43.7%, 56.2%, 68.7%, and 75.0%, respectively. The total serum immunoglobulin E (IgE) levels were positively correlated with disease duration (r = 0.262, P = 0.021); no other factors were associated with the duration of the disease. CONCLUSIONS: A high proportion of children with CU were classified as CSU. No indicators, except for total IgE were found to predict the timing of spontaneous remission. The CU remission rate identified in this study is expected to be used as one of the reference data for the progress of CU in patients.

Idiopathic cardiomyopathies in Korean children. - 9-Year Korean Multicenter Study-.

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.

Does Early Drain Removal Affect Postoperative Pericardial Effusion after Congenital Cardiac Surgery?

BACKGROUND: Patients undergoing cardiac surgery require postoperative chest drainage. However, the drain is difficult to keep in place in children with congenital heart disease. Since 2015, at Kyungpook National University Hospital, the chest tube is removed on postoperative day 1 in patients who have undergone simple congenital cardiac surgery (i.e., closure of an atrial or ventricular septal defect). In this study, we evaluated the relationship between the duration of drain placement and the likelihood of pericardial effusion after congenital cardiac surgery. METHODS: The medical records of patients who underwent closure of an atrial or ventricular septal defect at our hospital between January 2014 and December 2016 were reviewed. In total, 162 patients who received follow-up echocardiography and had information available on postoperative pericardial effusion after the repair procedure were enrolled. RESULTS: Echocardiography was performed at a median of 5 days (range, 4 to 6 days) postoperatively before discharge from the hospital. Pericardial effusion occurred in 21 patients (13.0%), of whom only 3 (1.9%) had moderate or greater pericardial effusion, regardless of the drain duration. All patients improved during outpatient follow-up without invasive management. No patient had severe complications because of pericardial effusion. The duration of drain placement did not affect the incidence of postoperative pericardial effusion (p=0.069). Operative survival was 100%. CONCLUSION: Based on our study, we recommend removing the drain as soon as its role is complete, generally on postoperative day 1, because early removal does not increase the incidence of pericardial effusion in patients undergoing simple congenital cardiac surgery.

Variation in clinical usefulness of biomarkers of acute kidney injury in young children undergoing cardiac surgery.

BACKGROUND: Acute kidney injury (AKI) is one of the most significant postoperative complications of pediatric cardiac surgery. Because serum creatinine has limitations as a diagnostic marker of AKI, new biomarkers including neutrophil gelatinase-associated lipocalin (NGAL), kidney injury molecule-1 (KIM-1), and interleukin-18 (IL-18) are being evaluated to overcome these limitations and detect AKI at an early stage after cardiac surgery. PURPOSE: This study aimed to investigate the clinical usefulness of these biomarkers in young children. METHODS: Thirty patients with congenital heart diseases who underwent cardiac surgery using cardiopulmonary bypass (CPB) were selected, and their urine and blood samples were collected at baseline and 6, 24, and 48 hours after surgery. Serum creatinine and blood urea nitrogen levels as well as NGAL, KIM-1, and IL-18 levels in urine samples were measured, and clinical parameters were evaluated. RESULTS: Of the 30 patients, 12 developed AKI within 48 hours after cardiac surgery. In the AKI group, 8 of 12 (66.6%) met AKI criteria after 24 hours, and urine KIM-1/creatinine (Cr) level (with adjustment of urine creatinine) peaked at 24 hours with significant difference from baseline level. Additionally, urine KIM-1/Cr level in the AKI group was significantly higher than in the non-AKI group at 6 hours. However, urine NGAL/Cr and IL-18/Cr levels showed no specific trend with time for 48 hours after cardiac surgery. CONCLUSION: It is suggested that urine KIM-1/Cr concentration could be considered a good biomarker for early AKI prediction after open cardiac surgery using CPB in young children with congenital heart diseases.

Importance of pulmonary valve morphology for pulmonary valve preservation in tetralogy of Fallot surgery: comparison of the echocardiographic parameters.

BACKGROUND: The decision to use transannular patching (TAP) during tetralogy of Fallot (TOF) repair depends on the pulmonary valve annulus size; the z score of the pulmonary annulus is the most commonly used predictor. However, definitive results are not obtained with z scores as different z score data sets are used for different parameters. PURPOSE: This study aimed to identify the echocardiographic and other key factors that warranted a change in the surgical method during TOF surgery. METHODS: Sixty-two patients were enrolled and divided into a pulmonary valve (PV) preservation group and a TAP group. Their medical records were reviewed. RESULTS: The z score for PV annulus (PVA), ratio of the PVA to aortic annulus size, and ratio of PVA to descending aorta (DAO) size were significantly different between the PV preservation and TAP groups (-1.72±1.52 vs. -3.07±1.94, P=0.004; 0.62±0.12 vs. 0.50±0.14, P=0.002; and 1.32±0.32 vs. 1.07±0.36, P= 0.008, respectively). For TAP repair, the PVA z score had a sensitivity of 65.4% and specificity of 73.1%, ratio of PVA to aortic annulus size had a sensitivity of 73.1% and specificity of 65.4%, and ratio of PVA to DAO size had a sensitivity of 69.2% and specificity of 57.7%. The TAP group showed more monocuspid PVs (P=0.011), while the PV preservation group showed more tricuspid PVs (P=0.027). Commissurotomy was more frequently performed in the PV preservation group than in the TAP group (P=0.001). Of patients with commissurotomy, 58% showed a PV z score<-2. CONCLUSION: Although various echocardiographic parameters may serve as predictors for determining surgical methods for TOF patients, the PV morphology and tissue characteristics should also be considered.

Infliximab Treatment for Intravenous Immunoglobulin-resistant Kawasaki Disease: a Multicenter Study in Korea.

BACKGROUND AND OBJECTIVES: We investigated the status of infliximab use in intravenous immunoglobulin (IVIG)-resistant Kawasaki disease (KD) patients and the incidence of coronary artery aneurysms (CAAs) according to treatment regimens. METHODS: Between March 2010 and February 2017, 16 hospitals participated in this study. A total of 102 (32.3±19.9 months, 72 males) who received infliximab at any time after first IVIG treatment failure were enrolled. Data were retrospectively collected using a questionnaire. RESULTS: Subjects were divided into two groups according to the timing of infliximab administration. Early treatment (group 1) had shorter fever duration (10.5±4.4 days) until infliximab infusion than that in late treatment (group 2) (16.4±4.5 days; p<0.001). We investigated the response rate to infliximab and the incidence of significant CAA (z-score >5). Overall response rate to infliximab was 89/102 (87.3%) and the incidence of significant CAA was lower in group 1 than in group 2 (1/42 [2.4%] vs. 17/60 [28.3%], p<0.001). CONCLUSIONS: This study suggests that the early administration of infliximab may reduce the incidence of significant CAA in patients with IVIG-resistant KD. However, further prospective randomized studies with larger sample sizes are required.

Multicenter Adherence Study of Asthma Medication for Children in Korea.

PURPOSE: Adherence is a major component of successful medical treatment. However, non-adherence remains a barrier to effective delivery of healthcare worldwide. METHODS: Twenty healthcare facilities (secondary or tertiary hospitals) belonging to the Korean Academy of Pediatric Allergy and Respiratory Diseases (KAPARD) participated. Questionnaires were given to patients currently receiving treatment in the form of inhalant useor oral intake or transdermal patch for mild to moderate asthma. RESULTS: A total of 1,838 patients responded to the questionnaire. Mean age was 5.98 ± 3.79 years (range: 0-18 years). With help from their caregivers, the percentage of patients that answered "taking as prescribed" was 38.04% for inhalant users, 50.09% for oral medication users and 67.42% for transdermal users. Transdermal patch users had significantly greater adherence compared to the other 2 groups (P < 0.001). The 34.15% of inhalant users, 70.33% of oral medication users and 93.00% of transdermal patch users felt that their medication delivery system was "Easy" or "Very easy" to use (P < 0.001). "Method of administration" was deemed to be the most difficult part of the treatment regimen to follow, and 76.7% of patients preferred once-daily administration (i.e., "Frequency of administration"). CONCLUSIONS: Asthma medication adherence in young children was found to be better in the transdermal patch group. This may be due to requiring fewer doses and easy to follow instructions. From an adherence point of view, the transdermal patch seems more useful for long-term asthma control in children compared to oral or inhaled medicine.

Clinical characteristics and etiologies of bronchiectasis in Korean children: A multicenter retrospective study.

BACKGROUND: Bronchiectasis is a chronic pulmonary disease characterized by progressive and irreversible bronchial dilatation. The aim of the present study was to investigate the etiologies and clinical features of bronchiectasis in Korean children. METHODS: We performed a retrospective review of the medical records for children diagnosed with bronchiectasis between 2000 and 2017 at 28 secondary or tertiary hospitals in South Korea. RESULTS: A total of 387 cases were enrolled. The mean age at diagnosis was 9.2 ±â€¯5.1 years and 53.5% of the patients were boys. The most common underlying cause of bronchiectasis was preexisting respiratory infection (55.3%), post-infectious bronchiolitis obliterans (14.3%), pulmonary tuberculosis (12.3%), and heart diseases (5.6%). Common initial presenting symptoms included chronic cough (68.0%), recurrent pneumonia (36.4%), fever (31.1%), and dyspnea (19.7%). The most predominantly involved lesions were left lower lobe (53.9%), right lower lobe (47.1%) and right middle lobe (40.2%). No significant difference was observed in the distribution of these involved lesions by etiology. The forced expiratory volume in 1 s (FEV1) levels were lowest in cases with interstitial lung disease-associated bronchiectasis, followed by those with recurrent aspiration and primary immunodeficiency. CONCLUSIONS: Bronchiectasis should be strongly considered in children with chronic cough and recurrent pneumonia. Long-term follow-up studies on pediatric bronchiectasis are needed to further clarify the prognosis and reduce the disease burden in these patients.

Flavonoids inhibit histamine release and expression of proinflammatory cytokines in mast cells.

Mast cells participate in allergy and inflammation by secreting inflammatory mediators such as histamine and proinflammatory cytokines. Flavonoids are naturally occurring molecules with antioxidant, cytoprotective, and antiinflammatory actions. However, effect of flavonoids on the release of histamine and proinflammatory mediator, and their comparative mechanism of action in mast cells were not well defined. Here, we compared the effect of six flavonoids (astragalin, fisetin, kaempferol, myricetin, quercetin, and rutin) on the mast cell-mediated allergic inflammation. Fisetin, kaempferol, myricetin, quercetin, and rutin inhibited IgE or phorbol-12-myristate 13-acetate and calcium ionophore A23187 (PMACI)-mediated histamine release in RBL-2H3 cells. These five flavonoids also inhibited elevation of intracellular calcium. Gene expressions and secretion of proinflammatory cytokines such as tumor necrosis factor-alpha (TNF-alpha), interleukin (IL)-1beta, IL-6, and IL-8 were assessed in PMACI-stimulated human mast cells (HMC-1). Fisetin, quercetin, and rutin decreased gene expression and production of all the proinflammatory cytokines after PMACI stimulation. Myricetin attenuated TNF-alpha and IL-6 but not IL-1beta and IL-8. Fisetin, myricetin, and rutin suppressed activation of NF-kappaB indicated by inhibition of nuclear translocation of NF-kappaB, NF-kappaB/DNA binding, and NF-kappaB-dependent gene reporter assay. The pharmacological actions of these flavonoids suggest their potential activity for treatment of allergic inflammatory diseases through the down-regulation of mast cell activation.

Long-term therapeutic efficacy of lamivudine compared with interferon-alpha in children with chronic hepatitis B: the younger the better.

OBJECTIVES: To assess and compare the long-term therapeutic response to lamivudine compared with interferon-alpha (IFN-alpha) in children with chronic hepatitis B. METHODS: A total of 40 children (27 male; age, 1.3-18 y, mean, 7.7 y) with chronic hepatitis B who received lamivudine for at least 12 months were followed for a mean period of 39 (24-76) months. Their treatment efficacy was historically compared with that of 19 children (14 male; age, 2.1-17 y; mean, 10 y) who had been treated with IFN-alpha and were followed for a mean period of 39 (24-104) months. Therapeutic responses were compared at 2 y after the initiation of either of the treatment methods. RESULTS: Two years after the initiation of treatment, the results for children treated with lamivudine versus IFN-alpha were as follows: hepatitis B e antigen (HBeAg) seroconversion occurred in 26 (65%) of the 40 children versus 7 (37%) of the 19 children, P < 0.05. In the lamivudine-treated group, the results for children treated before the age of 7 versus age >7 were as follows: HBeAg seroconversion occurred in 17 (89%) of the 19 children versus 9 (43%) of the 21 children, P < 0.01, and loss of hepatitis B surface antigen (HBsAg) occurred in 8 (42%) versus 0%, P < 0.001. CONCLUSIONS: Long-term treatment of lamivudine led to significant improvement in the seroconversion rate of HBeAg in children with chronic hepatitis B compared with IFN-alpha therapy. Furthermore, in preschool-age children, it led to significant improvement in the seroconversion rate of HBeAg and HBsAg compared with school-age children.

Radiofrequency catheter ablation of atrioventricular nodal reentry tachycardia in children and adolescents: a single center experience.

PURPOSE: Atrioventricular nodal reentry tachycardia (AVNRT) is less common in pediatric patients than in adult patients. Thus, data for pediatric AVNRT patients are insufficient. Hence, we aimed to analyze the patient characteristics, treatment, and any recurrences in pediatric AVNRT patients. METHODS: We reviewed the records of 50 pediatric AVNRT patients who had undergone radiofrequency catheter ablation (RFCA) between January 1998 and December 2016 at a single regional center. The patients were aged ≤18 years. RESULTS: Among 190 pediatric patients who underwent RFCA for tachyarrhythmia, 50 (26.3%; mean age, 13.4±2.6 years) were diagnosed as having AVNRT by electrophysiological study. Twenty-five patients (25 of 50, 50%) were male. Twenty patients (20 of 50, 40%) used beta-blockers before RFCA. All patients had no structural heart disease except 1 patient with valvular aortic stenosis and coarctation of the aorta. RFCA was performed using the anatomic approach under fluoroscopic guidance. The most common successfully ablated region was the midseptal region (25 of 50, 50%). Slow pathway (SP) ablation and SP modulation were performed in 43 and 6 patients, respectively. Complication occurred in 1 patient with complete atrioventricular block. During follow-up, 6 patients had recurrence of supraventricular tachycardia, as confirmed by electrocardiography. Among them, 5 underwent successful ablation at the first procedure. In 1 patient, induction failed during the first procedure. CONCLUSION: RFCA is safe and effective in pediatric AVNRT patients. However, further research is needed for establishing the endpoints of ablation in pediatric AVNRT patients and for identifying risk factors by evaluating data on AVNRT recurrence after RFCA.

Comparison between Kawasaki disease with lymph-node-first presentation and Kawasaki disease without cervical lymphadenopathy.

PURPOSE: We evaluated the characteristics of patients with Kawasaki disease (KD) who presented with only fever and cervical lymphadenopathy on admission, and compared them with the characteristics of those who presented with typical features but no cervical lymphadenopathy. METHODS: We enrolled 98 patients diagnosed with KD. Thirteen patients had only fever and cervical lymphadenopathy on the day of admission (group 1), 31 had typical features with cervical lymphadenopathy (group 2), and 54 had typical features without cervical lymphadenopathy (group 3). RESULTS: The mean age (4.3±2.1 years) and duration of fever (7.5±3.6 days) before the first intravenous immunoglobulin (IVIG) administration were highest in group 1 (P=0.001). Moreover, this group showed higher white blood cell and neutrophil counts, and lower lymphocyte counts after the first IVIG administration as compared to the other groups (P=0.001, P=0.001, and P=0.003, respectively). Group 1 also had a longer duration of hospitalization and higher frequency of second-line treatment as compared to groups 2 and 3 (group 1 vs. group 2, P=0.000 and P=0.024; group 1 vs. group 3, P=0.000 and P=0.007). A coronary artery z score of >2.5 was frequently observed in group 1 than in group 3 (P=0.008). CONCLUSION: KD should be suspected in children who are unresponsive to antibiotics and have prolonged fever and cervical lymphadenopathy, which indicates that KD is associated with the likelihood of requiring second-line treatment and risk of developing coronary artery dilatation.

Sensorineural hearing loss in patients with Kawasaki disease.

PURPOSE: Kawasaki disease involves acute febrile systemic vasculitis that can cause a variety of symptoms by affecting various organs. Here, we aimed to evaluate the prevalence, causes, and prognosis of sensorineural hearing loss (SNHL) occurring in children with Kawasaki disease. METHODS: Patients who were diagnosed with Kawasaki disease and received inpatient treatment in the Pediatrics Department at one of three university hospitals in Daegu city from February 2012 to September 2012 were enrolled in the study. The clinical features, hematological results, echocardiography results, audiometry results, and aspirin and salicylic acid serum levels of the patients were evaluated. RESULTS: Of the 59 children enrolled in the study, three showed mild bilateral SNHL on audiometry tests conducted after 48 hours of defervescence; these patients demonstrated normal patterns of recovery on follow-up tests 8 weeks later. Aspirin serum levels were significantly higher in the SNHL group after 48 hours of afebrile condition with high dose aspirin intake (P=0.034). However, no significant differences were found in other laboratory tests or for fever duration (P>0.05). Upon echocardiography, coronary artery abnormality was observed in 9 cases, but none of these patients showed hearing loss. CONCLUSION: The results indicate that SNHL in children with Kawasaki disease might occur during treatment of the acute phase; this SNHL usually involves mild bilateral hearing loss and recovers naturally. However, this study suggests that determination of the causes and clinical implications of hearing loss in Kawasaki disease requires long-term follow-up studies with more cases.

T-Helper Cytokine Profiles in Patients with Kawasaki Disease.

BACKGROUND AND OBJECTIVES: Kawasaki disease is an acute systemic vasculitis of which pathogenesis suspected is caused by immune dysregulation. The goal of this study is to evaluate the activation pattern of T helper cell type 1 (Th1) and T helper cell type 2 (Th2) in patients with Kawasaki disease. SUBJECTS AND METHODS: Prospective study of 60 patients (male 36, female 24) with diagnosis of Kawasaki disease were enrolled. One hundred and eighty blood samples from these patients were collected according to the different clinical stages {before initial intravenous immunoglobulin (IVIG), 5 days after initial IVIG, 2 months after initial IVIG}. The plasma level of Th1 cytokines; interferon-gamma (IFN-γ) & interleukin (IL)-2 and Th2 cytokines; IL-4 & IL-10 were measured by enzyme-liked immunosorbent assay. RESULTS: In all patients, the plasma level of Th1 cytokines (IFN-γ, IL-2) and Th2 cytokines (IL-4 and IL-10) were markedly elevated during the acute stage of Kawasaki disease. Since then, the plasma level of all these cytokines decreased significantly along with the process of clinical stages. Regardless of the existence of coronary artery lesion or no response to initial IVIG treatment, there were no significant differences between them. CONCLUSION: These data suggest that both Th1 and Th2 cells may be activated simultaneously during the acute stage of Kawasaki disease. Further studies are therefore required to establish the difference of activation pattern of T helper cells between Kawasaki disease and other inflammatory diseases.

Outcome of inflammatory response after normothermia during cardiopulmonary bypass surgery in infants with isolated ventricular septal defect.

PURPOSE: A recent study analyzing several cytokines reported that long cardiopulmonary bypass (CPB) time and long aortic cross clamp (ACC) time were accompanied by enhanced postoperative inflammation, which contrasted with the modest influence of the degree of hypothermia. In this present study, we aimed to examine the effect of CPB temperature on the clinical outcome in infants undergoing repair of isolated ventricular septal defect (VSD). METHODS: Of the 212 infants with isolated VSD who underwent open heart surgery (OHS) between January 2001 and December 2010, 43 infants were enrolled. They were classified into 2 groups: group 1, infants undergoing hypothermic CPB (26℃-28℃; n=19) and group 2, infants undergoing near-normothermic CPB (34℃-36℃; n=24). RESULTS: The age at the time of the OHS, and number of infants aged<3 months showed no significant differences between the groups. The CPB time and ACC time in group 1 were longer than those in group 2 (88 minutes vs. 59 minutes, P=0.002, and 54 minutes vs. 37 minutes, P=0.006 respectively). The duration of postoperative mechanical ventilation was 1.6 days in group 1 and 1.8 days in group 2. None of the infants showed postoperative neurological and developmental abnormalities. Moreover, no postoperative differences in the white blood cell count and C-reactive protein levels were noted between two groups. CONCLUSION: This study revealed that hypothermic and near-normothermic CPB were associated with similar clinical outcomes and inflammatory reactions in neonates and infants treated for simple congenital heart disease.

Predictors of nonresponse to intravenous immunoglobulin therapy in Kawasaki disease.

PURPOSE: It has been reported that 10% to 20% of children with Kawasaki disease (KD) will not respond to intravenous immunoglobulin (IVIG) treatment. In this study, we aimed to identify useful predictors of therapeutic failure in children with KD. METHODS: We examined 309 children diagnosed with KD at the Kyungpook National University Hospital and the Inje University Busan Paik Hospital between January 2005 and June 2011. We retrospectively reviewed their medical records and analyzed multiple parameters in responders and nonresponders to IVIG. RESULTS: Among the 309 children, 30 (9.7%) did not respond to IVIG. They had significantly higher proportion of neutrophils, and higher levels of aspartate aminotransferase, alanine aminotransferase (ALT), total bilirubin, and N-terminal fragment of B-type natriuretic peptide than did responders. IVIG-nonresponders had a significantly longer duration of hospitalization, and more frequently experienced coronary artery lesion, and sterile pyuria. No differences in the duration of fever at initial treatment or, clinical features were noted. CONCLUSION: Two independent predictors (ALT≥84 IU/L, total bilirubin≥0.9 mg/dL) for nonresponse were confirmed through multivariate logistic regression analysis. Thus elevated ALT and total bilirubin levels might be useful in predicting nonresponse to IVIG therapy in children with KD.

Electrocardiography recordings in higher intercostal space for children with right ventricular outlet obstruction reconstruction operation.

BACKGROUND AND OBJECTIVES: We checked traditional and high-level precordial electrocardiogram (ECG) leads in patients who had undergone right ventricular outlet obstruction (RVOT) reconstruction surgery and evaluated the effect of ECG lead position on their QRS duration. SUBJECTS AND METHODS: We enrolled 34 patients who had undergone surgery for congenital heart disease with RVOT obstruction and who had received followed up care that included recorded ECG at a pediatric cardiac out-patient clinic. The control group included 29 patients who did not have hemodynamically significant intracardiac abnormality. We recorded traditional standard 12-leads ECG from the 4th intercostals space, and moved the precordial leads to the 3rd and 2nd intercostals spaces, and recorded ECGs repeatedly. RESULTS: In all groups, there was no significant difference of mean QRS duration and QTc interval between traditional standard 12-leads ECGs and ECGs at higher intercostals spaces. There was no significant difference of ECG parameters between groups. In the control group, the degree of the change between the 4th intercostals space (ICS) QRS and 3rd ICS QRS was significant (p=0.031), and although, it was insignificant, ECGs at the 3rd ICS showed decreased QRS duration in group 1 (V1: 3rd ICS 119.21±21.53 msec vs. 4th ICS 122.80±31.78 msec. V2: 3rd ICS 113.68±19.43 msec vs. 4th ICS 118.24±19.16 msec). CONCLUSION: Although the positional change of ECG leads did not result in a significant effect on measuring QRS duration after surgery, ECG leads at the 3rd ICS rather than at the 4th ICS may cause alteration of ECG readings. Therefore, we suggest that ECGs should be recorded in as accurate a position as possible.