Kawasaki disease (KD) is an immune-mediated disease which is a leading cause of acquired cardiovascular disease in developed country. Recently, tumor necrosis factor-alpha (TNF-alpha) blocker, infliximab has been considered a promising option for patients with refractory KD. Although chronic use of a TNF-alpha blocker could increase risk of opportunistic infections, a few studies have documented that use of infliximab was safe without serious adverse effects in patients with KD. We observed serious bacterial infection after infliximab treatment in an infant with refractory KD. Our patient was a 5-month-old male infant diagnosed with KD who did not respond to repeated doses of intravenous immunoglobulin. We effectively treated him with a single infusion of infliximab (5 mg/kg), but gram-negative (Acinetobacter lwoffii) septicemia developed after infliximab infusion. Therefore, we report a case of serious septicemia after treatment with infliximab, and suggest considering the risk of severe infection when deciding whether to prescribe infliximab to an infant with refractory KD.
Bacterial Infections ; Cardiovascular Diseases ; Developed Countries ; Humans ; Immunoglobulins ; Infant* ; Male ; Mucocutaneous Lymph Node Syndrome* ; Opportunistic Infections ; Sepsis* ; Tumor Necrosis Factor-alpha ; Infliximab

BACKGROUND: EMLA cream is a eutectic mixture of lidocaine and prilocaine and has excellent anesthetic effect on tympanic membrane but histologic influence on tympanic membrane is uncertain. OBJECTIVE: To investigate its histologic effects on tympanic membrane. MATERIALS AND METHODS: 18 Sprague-Dawly rats were divided into 6 groups. Each group was treated with application of EMLA cream into external ear canal and then were sacrificed at 4 hours, 24 hours, 3 days, 7 days, and 14 days after application of the agents. RESULTS: 1) Transmission electron microscopy revealed partial loss of epithelial cell at 4 hours after application of the agents. 2) The specimen showed damaged cells in the epidermal layer and partial loss of basement membrane at 24 hours after application of the agents. 3) At 1 week after application of the agents epidermal layer and inner epithelium with connective tissue predominated. Thus the fibrous layer represent only 1/3 of total drum thickness. In the basal layers widened intercellular spaces were noted. 4) At 2 weeks after application of the agents newly formed connective tissue was found at fibrous layer and numerous fibroblasts were noted at inner epithelial layer. but overall histologic changes of the drum were not significant and healing processes was noted. CONCLUSION: EMLA cream has less histopathologic effects on tympanic membrane, and early recovery process occurred.
Anesthetics ; Animals ; Basement Membrane ; Connective Tissue ; Ear Canal ; Epithelial Cells ; Epithelium ; Extracellular Space ; Fibroblasts ; Lidocaine ; Microscopy, Electron, Transmission ; Prilocaine ; Rats* ; Tympanic Membrane*

DNase I footprinting assay using liver nuclear extracts revealed six protected regions between nucleotide -600 and +110 and hence named Box I-VI. Upstream promoter element (UPE), a DNA element playing crucial role in transcriptional control of the tissue specific expression of pancreatic beta-cell, has been detected within the proximal region of rat GLUT2 promoter. This region is included in Box VI. The protein-DNA interaction in this region (Box VI) was confirmed by mobility shift assay using liver nuclear extracts. Deletion of the region between -585 bp and -146 bp resulted in dramatic changes in promoter activity when they were expressed in liver and beta-cell derived cell line. When -585/-146 construct was expressed in liver, the activity was decreased to 46%, whereas the activity in beta-cell line, HIT-T15 cell, was increased by 84% when compared to -146/+190 construct. These opposing phenomena can be explained by the fact that beta-cell specifically expresses the UPE binding protein. Assuming that there may be Box VI-binding protein playing negative roles both in hepatocyte and beta-cell, and that the protein acts as a negative regulator of GLUT2 gene, the UPE binding protein in the beta-cell may overcome the inhibition by binding to the protein.
Animal ; Binding Sites ; Cell Line ; Comparative Study ; DNA Footprinting ; Deoxyribonuclease I ; Gene Expression Regulation ; Islets of Langerhans/metabolism* ; Islets of Langerhans/cytology ; Liver/metabolism* ; Liver/cytology ; Monosaccharide Transport Proteins/genetics ; Monosaccharide Transport Proteins/biosynthesis* ; Promoter Regions (Genetics)* ; Protein Binding ; Rats ; Transcription Factor AP-1

PURPOSE: Survivin is a member of the inhibitors of apoptosis family. It has recently comes into the limelight as a promising tumor marker, but many previous reports have shown controversial results regarding the significance and prognostic value of a survivin expression. In this study we determined the correlation between the survivin expression and the conventional prognostic markers and we also investigated the outcomes according to the localization of the survivin expression. METHODS: Tissue microarray (TMA) blocks were made with formalin-fixed paraffin-embedded tissues from 185 breast cancer patients and the immunohistochemical staining was done using an anti-survivin antibody. Among these, 157 patients were available for a survivin expression. The conventional clinicopathologic features and overall survival were correlated with the localization of the survivin expression. RESULTS: Survivin was expressed in 101 breast cancers (64.3%). A higher cytoplasmic survivin expression were noted in the older group (p=0.003), in the node-negative cancers (p=0.012), in the earlier tumor stages (p=0.012) and in the cancers that had not been treated with adjuvant chemotherapy (p=0.014). On the contrary, a higher nuclear survivin expression was inversely correlated with an estrogen expression (p=0.006) and a progesterone receptor (p=0.043) expression. In terms of survival, a cytoplasmic expression was associated with improved overall survival (p=0.01) but a nuclear survivin expression was correlated with unfavorable overall survival (p=0.002). A high cytoplasmic to nuclear ratio of survivin was associated with improved overall survival (p=0.001) conversely, increased nuclear to cytoplasmic survivin ratio was correlated with unfavorable overall survival (p<0.0001). Multivariate analysis revealed that nuclear survivin expression (p=0.001) and high nuclear to cytoplasmic survivin ratio (p=0.012) were independent predictor of overall survival. CONCLUSION: Survivin is frequently expressed in primary breast cancer. A cytoplasmic survivin expression is a good prognostic predictor for patients with axillary node negative early breast cancers and a nuclear survivin expression is a worse independent predictor of overall survival for patients with axillary node positive breast cancers.
Apoptosis ; Breast ; Breast Neoplasms ; Chemotherapy, Adjuvant ; Cytoplasm ; Estrogens ; Humans ; Multivariate Analysis ; Receptors, Progesterone

PURPOSE: The aim of this study was to investigate the pathophysiologic role of serum E-selectin, vascular endothelial growth factor(VEGF)-induced cell adhesion mollecule in Kawasaki disease(KD) and to look for the evidence of direct relationship between the plasma levels of soluble E-selectin and the incidence of coronary artery lesion(CAL). METHODS: Changes in plasma levels of sE-selectin(n=98) over time were measured by enzyme-linked immunosorbent assay(ELISA) in 23 patients with acute KD and 25 age-matched febrile children. RESULTS: Compared with control values, the peak levels of plasma sE-selectin were significantly elevated(mean+/-S.E.:22.89+/-12.53 ng/mL vs 10.65+/-3.42 ng/mL, P=0.01) in KD. 5 patients with CAL, plasma sE-selectin levels before treatment were higher than in 18 patients without CAL(mean+/-S.E.:39.43+/-15.08 ng/mL and 19.00+/-8.32 ng/mL, respectively; P=0.01). Plasma sE-selectin declined rapidly in the majority of KD patients regardless of the presence of CAL. Plasma sE-selectin levels after treatment and convalesent period were similar in KD patients with and without CAL. The plasma levels sE-selectin were correlated with those of white blood cell count(r=0.299, P<0.05), CRP(r=0.430, P<0.05), serum albumin(r=-0.483, P<0.05), serum protein(r=-0.502, P<0.05) and hemoglobin(r=-0.372, P<0.05) not with those of ESR, platelet, or duration of fever. There were significant differences in the initial level of serum sE-selectin between KD with and without CAL(mean+/-S.E.:39.44+/-15.08 ng/mL vs. 19.00+/-17.18 ng/mL) in multivariated linear tests. CONCLUSION: Plasma sE-selectin levels were significantly higher in KD than in other febrile illness. Higher plamsa levels of sE-selectin may have potential as a predictor of CAL in patients with KD.
Blood Platelets ; Cell Adhesion ; Child ; Coronary Vessels ; E-Selectin* ; Fever ; Humans ; Incidence ; Leukocytes ; Mucocutaneous Lymph Node Syndrome* ; Plasma

PURPOSE: High-Sensitivity C-reactive protein(hs-CRP) has been recognized as a very useful and sensitive predictor of the future risk of myocardial infarction. But the clinical significance of hs-CRP in children remains uncertain. To confirm the existence of obesity-induced vascular inflammation and the association between metabolic syndromes and elevation of CRP in children, we investigated the relationship among CRP, obesity, blood pressure(BP), and serum lipids in schoolboys. METHODS: Twenty-eight obese(BMI 29.61+/-3.29 kg/m2) and 93 non-obese(BMI 18.99+/-2.21 kg/m2) boys aged 14 years were examined. Serum CRP levels was measured by the high sensitive latex turbidimetric immunoassay and subjects with CRP levels below 0.3 mg/dL were adopted to avoid the influence of acute infection. RESULTS: Obese children had significantly higher hs-CRP levels than their non-obese group(0.104+/-0.075 vs. 0.054+/-0.005 mg/dL). In the obese group, BMI, systolic blood pressure, diastolic blood pressure, apolipoprotein B, atherogenic index, and triglyceride were significantly higher than in non-obese. The BMI, diastolic blood pressure, apolipoprotein E, atherognic index, and triglyceride showed positive correlation with log CRP by simple regression. Multiple regression analysis indicated that BMI and apolipoprotein E were strongly related to CRP. CONCLUSION: This study revealed that obese children tended to have higher levels of serum hs-CRP, BP elevation and dyslipidemia than the control group and that BMI and apolipoprotein E were strongly related to CRP. These results indicate that obesity related metabolic syndrome can be developed in children.
Apolipoproteins ; Blood Pressure ; C-Reactive Protein* ; Child ; Dyslipidemias ; Humans ; Immunoassay ; Inflammation ; Latex ; Myocardial Infarction ; Obesity ; Triglycerides

BACKGROUND/AIM: There is little data on whether plastic stents with a larger diameter are patent for longer than small stents in patients with bile duct cancer. The aim of this study was to compare the stent survival between 7-French (Fr) and 10-Fr plastic stents and evaluate the factors affecting stent survival. METHODS: Patients with biliary obstruction due to biliary tract cancer were enrolled at Yonsei University Wonju College of Medicine from January 2010 to October 2014. RESULTS: A total of 215 patients (7-Fr:10-Fr = 89:126 patients) were retrospectively enrolled. The primary tumor sites were common bile duct (n = 111), hilar (n = 45), and ampulla of Vater (n = 59). Rates of stent migration and stent obstruction were not different between the two groups. The median duration of stent survival was 3.3 months in the 7-Fr group and 5.9 months in the 10-Fr group (p = 0.543). The diameter of the stent did not have an effect on stent survival (hazard ratio 1.11, 95% confidence interval 0.71-1.73, p = 0.649). CONCLUSIONS: 7-Fr and 10-Fr stents have similar rates of stent migration and stent obstruction. The stent survival of 7-Fr was not inferior to 10-Fr stents in the management of biliary tract cancer.
Ampulla of Vater ; Bile Duct Neoplasms ; Biliary Tract Neoplasms* ; Biliary Tract* ; Common Bile Duct ; Gangwon-do ; Humans ; Plastics* ; Retrospective Studies ; Stents*

Wilson s disease is an autosomal recessive disorder characterized by degenerative changes in the brain, liver, and cornea. Treatment includes D-penicillamine, trientine, and zinc sulfate. D-penicillamine has been used frequently as first line therapy for Wilson s disease. However, nephrotoxicity can occur after D-penicillamine treatment. Among them membranous glomerulopathy is the most common histological abnormality but minimal change lesions have also been reported. Nephrotic syndrome is a late complication of D-penicillamine treatment but very rarely can occur within 2 months after treatment of D-penicillamine. We report the early development of minimal change nephrotic syndrome in a 3-year-old girl with Wilson s disease 3 weeks after initiation of D-penicillamine.
Brain ; Child, Preschool ; Cornea ; Female ; Glomerulonephritis, Membranous ; Humans ; Liver ; Nephrosis, Lipoid* ; Nephrotic Syndrome ; Penicillamine* ; Trientine ; Zinc Sulfate

Neonatal lupus erythematosus (NLE) is a rare disease characterized by typical clinical features and the transplacental passage of maternal autoantibodies, particularly anti-SSA/Ro. The major clinical manifestations are cutaneous lupus lesions, congenital heart block, hematological disorders, and hepatobiliary diseases. We report a case of NLE presenting with multiple round and oval target-like erythematous skin lesions and abnormal liver function, born to a clinically asymptomatic mother whose diagnosis was made retrospectively only after her newborn's diagnosis. Both the infant and the mother were positive for the anti-SSA/Ro and anti-SSB/La antibodies.
Antibodies ; Autoantibodies ; Heart Block ; Humans ; Infant ; Liver ; Lupus Erythematosus, Systemic ; Mothers ; Rare Diseases ; Retrospective Studies ; Skin

PURPOSE: To evaluate the surgical outcome of autologous transplantation of internal limiting membrane (ILM) for the treatment of large macular hole. METHODS: Twenty-five gauge pars plana vitrectomy was performed for the treatment of patients with full thickness macular hole larger than 400 microm. ILM was stained using 0.025% brilliant blue G. ILM around the hole was circumferentially peeled as large as 2.5 disc diameter (DD) in size and then transplanted inside the hole. ILM was peeled out additionally approximately 1.5 DD in size. Fluid-air exchange and gas injection were performed. After surgery, the hole was scanned using spectral domain optical coherence tomography. RESULTS: A total of 5 eyes were included in the present study. The mean age was 65.0 +/- 11.8 years (52-77) and mean best corrected visual acuity (log MAR) was 0.80 +/- 0.27. The mean refractive error was -2.0 +/- 2.2 diopter, mean horizontal size of hole was 701.4 +/- 129.4 microm, mean vertical size was 630.2 +/- 202.8 microm, mean hole base size was 1,043.4 +/- 225.0 microm and hole height was 464.4 +/- 218.9 microm. At the first day after surgery, transplanted ILM was detected inside the hole in all 5 eyes and complete closure of the hole occurred in 4 eyes. One hole was closed between postoperative days 4 and 7. Foveal contour improved gradually but photoreceptor integrity did not during the follow-up period. Two eyes showed visual improvement but 3 did not after surgery. CONCLUSIONS: Macular hole was closed successfully and quickly using the autologous ILM transplantation technique. Based on our results, the autologous ILM should be considered the treatment of choice for large macular holes.
Autografts* ; Follow-Up Studies ; Humans ; Membranes* ; Refractive Errors ; Retinal Perforations* ; Tomography, Optical Coherence ; Transplantation, Autologous* ; Visual Acuity ; Vitrectomy