Intussusception and Meckel's diverticulum are very rare disorders in intrauterine or neonatal periods, which are causes of intestinal obstruction. We experienced a case of intussusception due to Meckel's diverticulum which caused intestinal obstruction in the neonate who had bilious vomiting a few hours after birth. We report this case with a brief review of the literature.
Humans ; Infant, Newborn* ; Intestinal Obstruction ; Intussusception* ; Meckel Diverticulum* ; Parturition ; Vomiting

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

PURPOSE: Because of the unavailability of marrow transplantation, umbilical cord blood (CB) is increasingly being used. We evaluated the potential of ex vivo expansion and clonality in CD34+ cells separated from cord blood source and mobilized peripheral blood (PB) in a serum-free media. METHODS: The CD34+ cells, selected from CB and mobilized PB, were expanded with hematopoietic growth factors. They were then cultured for burst-forming units of erythrocytes (BFU-E), colony-forming units of granulocytes and monocytes (CFU-GM) and colony-forming units of megakaryocytes (CFU-Mk) at culture days 0, day 4, day 7, and day 14 with various growth factors. RESULTS: The CB-selected CD34+ cells showed significantly higher total cell expansion than those from the PB at day 7 (2 fold increase than PB). The CB-selected CD34+ cells produced more BFU-E colonies than did the PB on culture at days 7 and at day 14. Also, the CB-selected CD34+ cells produced more CFU-Mk colonies than did the PB on culture at day 4 and at day 7. CONCLUSION: The ex vivo expansion of the CB cells may be promising in producing total cellular expansion, CFU-Mk and BFU-E compared with PB for 7 to 14 days. The growth factors combination including megakaryocyte growth and development, flt3-ligand and interleukin-3 showed more expansion in the view of total cells and clonal maintenance compared with less combination.
Bone Marrow ; Culture Media, Serum-Free ; Erythrocytes ; Erythroid Precursor Cells ; Fetal Blood* ; Granulocytes ; Growth and Development ; Intercellular Signaling Peptides and Proteins ; Interleukin-3 ; Megakaryocytes ; Monocytes ; Stem Cells

PURPOSE: Kawasaki disease is the most common cause of systemic vasculitis in children less than 5 years of age. Recent immunohistochemistry findings suggest that many vascular growth factors play a role in the formation of the coronary artery lesions. Active remodeling of the coronary artery lesions in Kawasaki disease continues in the form of intimal proliferation and neoangiogenesis for several years after the onset of the disease. Intravenous immunoglobulin (IVIG) and corticosteroid have been used in the treatment of Kawasaki disease but the exact mechanism is not clear. We have investigated that IVIG and corticosteroid inhibited vascular endothelial growth factor (VEGF)- induced tube formation of endothelial cells in vitro on Matrigel assay. METHODS: Human umbilical vein endothelial cells (HUVECs) were cultured and seeded on Matrigel coated 24 well plates in medium with or without the following agents: VEGF, VEGF plus IVIG, VEGF plus VEGF antibody, VEGF plus methylprednisolone, VEGF, IVIG plus methylprednisolone for 18 hours. The total length of tube structures in each photograph was quantified. RESULTS: IVIG significantly inhibited the proliferation of HUVECs. The inhibitory effect of IVIG was also reversible. In the meantime, VEGF induced the differentiation of HUVECs into capillary like structures on Matrigel, which was inhibited by VEGF antibody in a dose-dependent manner. Interestingly, IVIG and methylprednisolone inhibited VEGF-induced tube formation of HUVECs. IVIG was more effective in inhibition than methylprednisolone alone. CONCLUSION: We revealed that VEGF induced the differentiation of HUVECs and this effect was inhibited by IVIG and methylprednisolone.
Capillaries ; Child ; Coronary Vessels ; Endothelial Cells* ; Human Umbilical Vein Endothelial Cells ; Humans ; Immunoglobulins* ; Immunoglobulins, Intravenous ; Immunohistochemistry ; Intercellular Signaling Peptides and Proteins ; Methylprednisolone* ; Mucocutaneous Lymph Node Syndrome ; Systemic Vasculitis ; Vascular Endothelial Growth Factor A

PURPOSE: Airway dehydration and subsequent hyperosmolarity of periciliary fluid are considered critical events in exercise-induced bronchoconstriction. The aim of this study was to establish if a hyperosmolar challenge could induce activation of eosinophils. METHODS: Human eosinophilic leukaemic cell lines, EoL-1 cells were incubated with hyperosmolar solutions for 15 minutes. Activation of EoL-1 cells was monitored by degranulation and superoxide anion production. In addition, we examined surface expression of CD69 and ICAM-1. RESULTS: Hyperosmolar stimuli didn't induce superoxide anion production and degranulation. In addition, EoL-1 cells cultured with hyperosmolar medium at 930 mOsm/kg H2O resulted in no significant increment in fluorescent intensity of CD69 and ICAM-1 expression compared with results for cells incubated with isomolar medium. CONCLUSION: We found that hyperosmolar stimuli don't cause activation of EoL-1 cells, but further studies are required to determine the role of eosinophil in the mechanism of exercise-induced asthma.
Asthma, Exercise-Induced ; Bronchoconstriction ; Cell Line ; Dehydration ; Eosinophils* ; Humans* ; Intercellular Adhesion Molecule-1 ; Superoxides

PURPOSE: Glycogen storage disease type Ia (GSD Ia) is an autosomal recessive disorder caused by the deficiency of glucose-6-phosphatase (G6Pase). The aim of the study was to investigate the spectrum of G6Pase gene mutations and relationship between genotype and clinical findings in Korean patients with GSD Ia. METHODS: Genomic DNA was extracted from peripheral leukocytes of 20 patients with GSD Ia. The five exons of G6Pase gene were amplified and PCR products were directly sequenced. The frequency of short stature, hypoglycemia, hypercholesterolemia, hyperuricemia, hypercalciuria, nephrocalcinosis and hepatic adenoma was compared between 727G> T homozygotes and 727G> T compound heterozygotes. RESULTS: A total of 5 different mutations were identified. The most common mutation was the 727G> T with an allele frequency of 80%. All patients were either homozygous (12/20) or heterozygous (8/20) for the 727G> T mutation. G122D was found in 3 patients, P178A in 1, G222R in 2, and S339R in 2. There was no difference in the frequency of short stature, hypoglycemia, hypercholesterolemia, hyperuricemia, nephrocalcinosis, and hepatic adenoma between 727G> T homozygotes and heterozygotes. CONCLUSION: Diagnosis of GSD Ia can be based on clinical and biochemical abnormalities combined with mutation analysis instead of enzymatic diagnosis that requires liver biopsy. Homozygosity for the 727G> T does not seem to alter the disease phenotype as compared with the heterozygous state.
Adenoma ; Biopsy ; Diagnosis ; DNA ; Exons ; Gene Frequency ; Genotype* ; Glucose-6-Phosphatase ; Glycogen Storage Disease* ; Glycogen* ; Heterozygote ; Homozygote ; Humans ; Hypercalciuria ; Hypercholesterolemia ; Hyperuricemia ; Hypoglycemia ; Leukocytes ; Liver ; Nephrocalcinosis ; Phenotype ; Polymerase Chain Reaction

PURPOSE: Recently, it was reported that tumor necrosis factor (TNF) and lymphotoxin-alpha (LT-alpha) gene regions might be a susceptible loci to type 1 diabetes in Japanese. The purpose of this study was to investigate the association of TNF and LT-alpha gene polymorphisms with disease susceptibility in Korean children with type 1 diabetes. METHODS: Forty-nine Korean children with type 1 diabetes (29 girls and 20 boys) and 94 healthy Koreans were investigated in this study. Genotyping for -857T/C polymorphism in the TNF promoter region and LT-alpha gene polymorphism were performed by PCR-RFLP (restriction fragment length polymorphism). TNF promoter -1031C/T polymorphism was detected by allele-specific PCR. RESULTS: The distribution of the -857T/C and -1031C/T genotype in the TNF promoter region was not different between diabetic children and the controls. The frequency of TT genotype in the distribution of TNF -1031C/T polymorphism in diabetic children with diabetic ketoacidosis (DKA) at diagnosis was significantly lower than those without DKA (P< 0.05). No significant difference in the distribution of LT-alpha gene polymorphism was observed between diabetic children and the controls. There was no association between clinical characteristics of type 1 diabetes and LT-alpha gene polymorphisms. CONCLUSION: These results suggest that TNF promoter -857T/C and LT-alpha gene polymorphisms are not associated with susceptibility to type 1 diabetes in Korean children. TNF promoter -1031C/T polymorphism might be related to clinical manifestations (DKA) of type 1 diabetes.
Asian Continental Ancestry Group ; Child* ; Diabetic Ketoacidosis ; Diagnosis ; Disease Susceptibility ; Female ; Genotype ; Humans ; Lymphotoxin-alpha* ; Polymerase Chain Reaction ; Promoter Regions, Genetic ; Tumor Necrosis Factor-alpha*

PURPOSE: The use of growth hormone (GH) to promote growth in normal short children without classical GH deficiency is controversial. Numerous foreign studies have shown the effects of GH therapy in children with idiopathic short stature (ISS) whereas few has been interested in Korea. Therefore, this study is designed to investigate the effects of GH therapy on ISS by observing correlations and changes among various growth parameters such as, insulin-like growth factor-I (IGF-I) and insulin-like growth factor binding protein-3 (IGFBP-3). METHODS: This study was conducted retrospectively with 15 children with ISS in Chungbuk National University Hospital in Korea. Mean age was 11.44+/-2.81 and the children were treated with 0.66 IU/kg/wk dosage of GH for 1 or 2 years. Also, the growth parameters before and after the GH therapy were observed. RESULTS: Height standard deviation score (HT-SDS) was increased from -1.85+/-0.70 to -1.58+/-0.56 at 1 year and to -1.21+/-0.37 at 2 years after GH therapy. Predicted adult height standard deviation score (PAH-SDS) was also increased from -2.10+/-0.52 to -1.67+/-0.59 at 1 year, and to -0.96+/-0.60 at 2 years. Serum IGF-I and IGFBP-3 levels were significantly increased after 1 year and marginally increased after 2 years of GH therapy. CONCLUSION: It is concluded that GH therapy has growth promoting effect. The significant increase in IGF-I and IGFBP-3 levels during the GH therapy suggests that IGF-I and IGFBP-3 are useful predictors of response to the use of GH therapy. It is expected that larger patient samples would provide more reliable information about the effect of GH therapy.
Adult ; Body Height ; Child* ; Chungcheongbuk-do ; Growth Hormone* ; Humans ; Insulin-Like Growth Factor Binding Protein 3 ; Insulin-Like Growth Factor I ; Korea ; Retrospective Studies

PURPOSE: The objectives of this study was to evaluate the correlations between the indices of insulin sensitivity using fasting glucose and insulin level, and the body fat mass measured by bioelectrical impedance analysis (BIA) and dual energy X-ray absorptiometry (DEXA), and to determine the clinical usefulness of insulin sensitivity indices when obese children were followed up. METHODS: In this study, 28 simple obese children and adolescents were included. Anthropometric data including body weight, height, obesity degree (OD), body mass index (BMI), and waist-to-hip ratio were collected and then body fat mass was measured by using BIA and DEXA. For metabolic data, 12 hour fasting serum glucose, insulin and lipid profiles were measured and indices for insulin sensitivity (G/I ratio, loginsulin, HOMA-IR, logHOMA-IR, QUICKI) were calculated. RESULTS: BMI had a higher correlation with insulin sensitivity indices than OD (G/I ratio, -0.463 vs -0.209; loginsulin, 0.417 vs 0.196; HOMA-IR, 0.301 vs 0.238; logHOMA-IR, 0.403 vs 0.198; QUICKI, -0.451 vs -0.224). But OD had a higher correlation with body fat mass measured by BIA and DEXA than BMI (BIA, 0.612 vs 0.316; DEXA, 0.667 vs 0.512). The G/I ratio was correlated with body fat mass in BIA (r=-0.420, P< 0.05) and DEXA (r=-0.512, P< 0.01), percentage of body fat (percentage of fat) in BIA (r=-0.366, P< 0.05) and DEXA (r=-0.449, P< 0.01). HOMA-IR was only correlated with body fat mass in DEXA (r=0.341, P< 0.05). CONCLUSION: This study revealed that G/I ratios had a statistically significant correlation with anthropometric obesity indices (OD and BMI) and also had a correlation with both body fat mass and percentage of fat. These results suggest that G/I ratios could be used as useful index when obese children and adolescence are followed up.
Absorptiometry, Photon* ; Adipose Tissue* ; Adolescent ; Blood Glucose ; Body Mass Index ; Body Weight ; Child ; Electric Impedance* ; Fasting ; Glucose ; Humans ; Insulin Resistance* ; Insulin* ; Obesity ; Waist-Hip Ratio

PURPOSE: To measure the peak myocardial tissue velocities and patterns of longitudinal motion of atrioventricular (AV) annuli and assess body weight and heart rates-related changes in normal children. METHODS: Using pulsed wave Tissue Doppler Imaging (TDI), we measured peak systolic, early and late diastolic myocardial velocities in 72 normal children at six different sites in apical-4 chamber (A4C) view and at four different sites in apical-2 chamber (A2C) view and compared those values with each other, also observing effects with body weights and heart rates. Longitudinal motions of the AV annuli were measured at three different sites in A4C. RESULTS: There were no significant differences of the TDI parameters between gender, ECHO- machines and among the three Doctors performing TDI. Peak myocardial velocities were significantly higher at the base of the heart than in the mid-ventricular region and in the right lateral ventricular wall than in the left lateral ventricular wall or IVS. The TDI parameters showed no significant correlation with fractional shortening (%). Peak systolic and early diastolic myocardial velocities had no correlation with heart rates, but peak late diastolic velocities and A/E ratio correlated positively with heart rates. Correlations between the TDI parameters and body weight were inconsistent. Absolute longitudinal displacement and % displacement were not differ between gender and not correlated with the TDI parameters. CONCLUSION: We measured the peak myocardial velocities with TDI and the longitudinal motion of the AV annuli using M-mode echocardiography in normal children. With more large scale evaluation, we may establish reference values in normal children and broaden clinical applicabilities in congenital and acquired heart diseases.
Body Weight ; Child* ; Echocardiography ; Heart Diseases ; Heart Rate* ; Heart* ; Humans ; Pediatrics ; Reference Values*