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

This study was performed to observe the elementary body and initial body in the cultured conjuntival epithelial cell, which was co-cultures with Chlamydia trachomatis serotype-D. Following 3 weeks of cultivation of the rabbit conjuntival epithelial cell, Chlamydia trachomatis seretype-D was inoculated into the epithelial cells and co-cultured for 24, 48, and 96 hours respectively. The infected conjunctival epithelial cells was stained with fluorescence-conjugated chlamydial antibody and iodine staining. Regardless of the duration of the cocultivation time, the cultured conjunctival cells showed the positive reaction to immunofluorescent staining and iodine staining. These results indicate that Chlamydia trachomatis can be cultured in the cultured conjuntival epothelial cell of rabbit and iodine staining is a good alternative to the immunofluorescent method.
Chlamydia trachomatis* ; Chlamydia* ; Coculture Techniques ; Epithelial Cells ; Fluorescent Antibody Technique ; Inclusion Bodies ; Iodine

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

Hypocalcemia is due to Hypoparathyroidism, Vitamin D deficiency, Hypomagnesemia, Inadequate calcium intake. The benefits of breast-feeding are well established. There are no need to supply calcium or Vitamin D in breast-fed infant. We report a case of infantile hypocalcemia caused by Vitamin D deficiency in exclusively breast-fed infant. He had no hypocalcemic symptom and hypocalcemia was found incidentally by routine laboratory tests during pneumonia treatment. He was presented with a low serum calcium level and 1,25(OH)2 Vit D3 level and high PTH. He was improved by Calcium and Vitamin D supplement. After then his mother continued breast feeding exclusively and resisted to feed her baby weaning food. During follow up period, hypocalcemia was recheked after discontinuation of vitamin D supplement. At 11 months of age, the calcium level was normal without vitamin D supplement after he had eaten weaning food. This report describes a case of hypocalcemia induced by vitamin D deficiency in exclusively breast-fed infant, with review of the literature.
Breast Feeding ; Calcium ; Follow-Up Studies ; Humans ; Hypocalcemia* ; Hypoparathyroidism ; Infant* ; Mothers ; Pneumonia ; Vitamin D Deficiency* ; Vitamin D* ; Vitamins* ; Weaning

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

Bickerstaff's brainstem encephalitis is characterized by ophthalmoplegia, ataxia, and disturbance of consciousness. It is similar to Miller Fisher syndrome, a variant of Guillain-Barre syndrome, in that they share features such as ophthalmoplegia and ataxia. The difference is that patients with Bickerstaff's brainstem encephalitis have impaired consciousness, whereas patients with Miller Fisher syndrome have alert consciousness and areflexia. Here, we report the case of a 3-year-old child who was diagnosed with Bickerstaff's brainstem encephalitis presenting typical clinical features and interesting radiological findings. The patient showed ophthalmoplegia, ataxia, and subsequent stuporous mentality. Brain magnetic resonance imaging revealed high signal intensity in the pons and cerebellum around the 4th ventricle on a T2-weighted image. He was successfully treated with intravenous immunoglobulin. Differentiation of Bickerstaff's brainstem encephalitis and Miller Fisher syndrome is often difficult because they possess many overlapping features. Brain magnetic resonance imaging may be helpful in diagnosing Bickerstaff's brainstem encephalitis, especially when lesions are definitely found.
Ataxia ; Brain ; Brain Stem* ; Cerebellum ; Child ; Child, Preschool ; Consciousness ; Encephalitis* ; Guillain-Barre Syndrome ; Humans ; Immunoglobulins ; Magnetic Resonance Imaging ; Miller Fisher Syndrome ; Ophthalmoplegia ; Pons ; Stupor

Since Jacob and associates in 1959 were the first to report a case of triple X syndrome associated with ovarian failure, the incidence of trisomy X in newborn population is estimated to be 1 in 1,000 live born female. Most of them have normal physical appearance and puberty. We report a case of a newborn with triple X syndrome confirmed by chromosomal study whose clinical features included left preauricular pit, broad nose, thin lip, anogenital anomaly. Echocardiography showed atrial septal defect and ventricular septal defect.
Adolescent ; Echocardiography ; Female ; Heart Septal Defects, Atrial ; Heart Septal Defects, Ventricular ; Humans ; Incidence ; Infant, Newborn ; Lip ; Nose ; Puberty ; Trisomy

PURPOSE: The aim of this study was to identify usefulness of simple oral and gastric pH measurement using pH paper on detection of symptomatic gastroesophageal reflux in neonates. METHODS: This prospective study included a total of 66 neonates born at Konyang University Hospital from June 2004 to June 2005. Each neonate's oral and gastric pH levels measured with pH paper at 6 hourly intervals. Suspected gastroesophageal refluex neonates were studied 24-hr lower esophageal pH monitoring or upper GI series and confirmed. We compared oral and gastric pH between symptomatic gastroesophageal reflux (GER) group and asymptomatic (control) group. RESULTS: GER group consist of 12 neonates and control group consist of 54 neonates. Oral and gastric pH were 5.4+/-0.6, 2.9+/-0.5 in GER group, 6.0+/-0.3, 3.9+/-0.9 in control group, the differences between two groups were significant (P<0.05). All neonates of GER group were corfirmed gastroesophageal reflux by 24-hr lower esophageal pH monitoring or upper GI series studies. Our data indicate as a predictor for significantly symptomatic gastroesophageal reflux, at oral pH 5.75, has a sensitivity 92%, specificity 89%, positive predictive value of 65%, and negative predictive value of 98%. The difference between oral and gastric pH (oral pH-gastric pH) was not significant in each group. CONCLUSION: In neonates with symptomatic gastroesophageal reflux oral and gastric pH were significantly lower than asymptomatic neonates. Oral and gastric pH were related with clinically significant symptoms of gastroesophageal reflux. We suggest that pH measurement could be a possible simple screening test of symptomatic gastroesophageal reflux.
Esophageal pH Monitoring ; Gastroesophageal Reflux* ; Humans ; Hydrogen-Ion Concentration ; Infant, Newborn* ; Mass Screening ; Prospective Studies ; Sensitivity and Specificity

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: Philadelphia(Ph) chromosome is found in about 95 percent of chronic myelogenous leukemia(CML) patients. Ph chromosome results from a reciprocal translocation between the long arms of chromosomes 9 and 22, and the fusion gene, BCR-ABL contribute to oncogenesis. Three to five years after first diagnosis, CML progresses to the blast crisis, and is accompanied by secondary cytogenetic changes in about 85% of cases. In this study, we investigated the incidence of ABL deletion of derivative 9 chromosome in CML and evaluated the association between this deletion and progression to the blast crisis by interphase fluorescence in situ hybridization(FISH). METHOD: The subjects included in this study were a consecutive series of 58 patients who were diagnosed as CML at Seoul National University Hospital between January 1997 and April 2000. On 90 archival bone marrow aspirate samples from these 58 CML patients, interphase FISH was performed with a commercially available probe. RESULTS: The ABL deletion of derivative 9 chromosome was detected in 17(29.3%) of 58 patients with CML. Eighteen of 58 patients progressed to blast crisis in this period. ABL deletion was found in 7 of 18 patients with blast crisis, and not in 11 remainders. The mean duration from the diagnosis to blast crisis was 37.1 months in 7 patients with the ABL deletion, while the mean duration was 74.2 months in 11 patients without the ABL deletion. The mean duration from the diagnosis to blast crisis in patients with ABL deletion was significantly shorter than in patients without ABL deletion(P=0.043). CONCLUSIONS: We found that 29.3% of patients with CML had the ABL deletion on derivative 9 chromosome. In these patients, the time taken for evolution to blast crisis was significantly shorter than that of the patients without ABL deletion.
Arm ; Blast Crisis* ; Bone Marrow ; Carcinogenesis ; Cytogenetics ; Diagnosis ; Fluorescence* ; Humans ; Hydrogen-Ion Concentration ; In Situ Hybridization* ; Incidence* ; Interphase* ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive* ; Philadelphia Chromosome ; Seoul