Familial hemophagocytic lymphohistiocytosis (FHL) is a rare disease characterized by fever, hepatosplenomegaly, cytopenia and non-malignant lymphohistiocytic infiltration with hemophagocytosis in reticulendothelial organs. We experienced three cases of FHL in identical male twins and their younger brother who presented with fever and severe hepatosplenomegaly. Cytopenia, elevated serum transaminase and low serum albumin levels, hypertriglyceridemia were common laboratory findings of them. One of them showed markedly decreased phytohemagglutinin induced lymphocyte proliferation and reversed CD4/CD8 ratio (0.52) in flowcytometric lymphocyte subset analysis. Aspirate of bone marrow revealed typical features consistent with FHL in two of them. In spite of recent therapeutic approaches, none of them survived.
Bone Marrow ; Fever ; Humans ; Hypertriglyceridemia ; Lymphocyte Subsets ; Lymphocytes ; Lymphohistiocytosis, Hemophagocytic* ; Male ; Rare Diseases ; Serum Albumin ; Siblings

PURPOSE: Recently, the list of Epsterin-Barr Virus (EBV)-associated diseases has been growing. It is now known that there are two types (type 1 & 2) of EBV, but it has yet to be fully investigated as to whether type B EBV differs in any way from type A virus in terms of geographical distribution or disease association. We performed this study to find out the prevalence of the two types of EBV in EBV seropositive healthy children and adults by using one step Polymerase chain reaction(PCR). METHODS: Fifty-four viral capsid antigen (VCA) IgG positive healthy children (5 to 15 yrs of age) and 60 healthy adults (20 to 50 yrs of age) were involved in this study. DNA extracted from saliva samples of healthy study children and adults were subjected to amplification using the modified one step general PCR, which was developed by M. Kunimoto et al for detection of EBV types. And B95-82 cells (type 1) and Jijoye cells (type 2) were used as positive controls, and K-562 cells was used as a negative control. RESULTS: EBV was detected in mouth washing saliva of 8 (14.8%) of 54 healthy children. Five EBV detected cases contained type 1 and the remaining three cases contained type 2. EBV was detected in mouth washing saliva of 21 (35%) of 60 healthy adults. Twenty EBV detected cases contained type 1 and the remaining one was type 2. In total, EBV was detected in the saliva samples of 29 cases (25.4%), and 25 (86.2%) contained type 1. CONCLUSION: The results of our study indicate that type 1 is highly dominant in Korea, and EBV excretion is more frequent in EBV positive healthy adults compared to EBV positive healthy children in Korea.
Adult* ; Capsid ; Child* ; DNA ; Herpesvirus 4, Human* ; Humans ; Immunoglobulin G ; Korea ; Mouth ; Polymerase Chain Reaction ; Prevalence ; Saliva

OBJECTIVE: To investigate the association between endometriosis and polymorphisms of N-acetyl transferase 2 (NAT2), glutathione S-transferase M1 (GSTM1), and cytochrome P450 (CYP) 1A1 genes in Korean infertile patients. MATERIALS AND METHODS: A total of 303 infertile patients who had undertaken diagnostic laparoscopy during January, 2001 through December, 2003 at Samsung Cheil Hospital enrolled in this study. The patients were grouped according to laparoscopic findings: minimal to mild endometriosis (group I: n=147), moderate to severe endometriosis (group II: n=57), normal pelvic cavity (n=99). Peripheral blood was obtained and genomic DNA was extracted. The genotypes of each genes were analyzed using polymerase chain reaction (PCR) and restriction fragment length polymorphism (RFLP). For NAT2, RFLP was used to detect the wild type (wt) and mutant (mt) alleles, enabling classification into slow (mt/mt) or fast (wt/wt or wt/mt) acetylation genotypes. For GSTM1, PCR was used to distinguish active (+/- or +/+) from null (-/-) genotypes. For CYP1A1, MspI digestion was used to detect the wild type (A1A1), heterozygote (A1A2) or mutant (A2A2) genotypes. RESULTS: The genotype frequencies of NAT2 slow acetylator was 12.8%, 10.9%, 12.8% in group I, group II and control, respectively. The genotype frequencies of GSTM1 null mutation was 55.3%, 41.8%, 53.2% in group I, group II and control, respectively. The genotype frequencies of CYP1A1 MspI polymorphism was 16.3%, 9.1%, 18.1% in group I, group II and control, respectively. No significant difference was observed between endometriosis and normal controls in the genotype frequencies of the NAT2, GSTM1, CYP1A1 MspI polymorphism. CONCLUSION: The NAT2, GSTM1, CYP1A1 gene polymorphism may not be associated with the susceptibility of endometriosis in Korean women.
Acetylation ; Alleles ; Classification ; Cytochrome P-450 CYP1A1 ; Cytochrome P-450 Enzyme System* ; Cytochromes* ; Digestion ; DNA ; Endometriosis* ; Female ; Genotype ; Glutathione Transferase* ; Glutathione* ; Heterozygote ; Humans ; Laparoscopy ; Polymerase Chain Reaction ; Polymorphism, Restriction Fragment Length ; Transferases*

The immunogenicity of a single dose of Salmonella typhi(S.typhi) Vi capsular polysaccharide(CPS) vaccine was evaluated before, and at 1, 3, 12, and 36 months after vaccination. Eighty-five adults(20-28 years of age) and sixty-four children(8-16 years of age) received a single dose of 25 micrograms Vi CPS vaccine intramuscularly, and antibody titers to Vi CPS were measured by passive hemagglutination. Of 149 vaccinees, 138(92.6%) showed seroconversion at 1 month after vaccination, and then 138 out of 141(97.9%) did at 3 months. Of 137 vaccinees, 116(84.7%) maintained a persistent rise in Vi antibody titer 12 months after vaccination, and 55 out of 100(55.0%) had a 4-fold or greater rise at 36 months. No significant adverse reactions were observed. Booster injection may be needed 3-5 years after vaccination.
Adolescent ; Adult ; Antibodies, Bacterial/blood ; Antigens, Bacterial/immunology ; Child ; Evaluation Studies ; Human ; Polysaccharides, Bacterial/*immunology ; Salmonella typhi/*immunology ; Typhoid Fever/prevention & control ; Typhoid-Paratyphoid Vaccines/adverse effects/immunology/*therapeutic use

No abstract available.
Rectum*

BACKGROUND: This study was performed to evaluate the incidence and clinical characteristics of symptomatic pneumothorax in the full-term neonate. MATERIALS AND METHODS: We retrospectively reviewed the medical records of 32 symptomatic pneumothorax patients in the full term neonates who admitted to the neonatal intensive care unit in Ulsan Dong Kang General Hospital from January, 2000 to December, 2004. The subjects were divided into two groups according to underlying causes; spontaneous pneumothorax group and secondary pneumothorax group, then each clinical characteristics were assessed. RESULTS: Spontaneous pneumothorax patients were 10 (31%) and secondary pneumothorax patients were 22 (69%). Overall incidence of spontaneous pneumothorax was 0.4%. Most common cause of secondary pneumothorax was pneumonia. Twelve cases (54.5%) among secondary pneumothorax patients were associated with mechanical ventilator care. Clinical characteristics, courses and managements were similar between two groups, but more shorter duration of admission and chest-tube insertion in spontaneous pneumothorax group CONCLUSION: The patient with symptomatic pneumothorax needs careful observation and proper management with or without underlying respiratory diseases
Hospitals, General ; Humans ; Incidence ; Infant, Newborn* ; Intensive Care, Neonatal ; Medical Records ; Pneumonia ; Pneumothorax* ; Retrospective Studies ; Ulsan ; Ventilators, Mechanical

OBJECTIVE: To test the hypothesis that a safety margin may affect local tumor recurrence (LTR) in subsegmental chemoembolization. MATERIALS AND METHODS: In 101 patients with 128 hepatocellular carcinoma (HCC) nodules (1-3 cm in size and < or = 3 in number), cone-beam CT-assisted subsegmental lipiodol chemoembolization was performed. Immediately thereafter, a non-contrast thin-section CT image was obtained to evaluate the presence or absence of intra-tumoral lipiodol uptake defect and safety margin. The effect of lipiodol uptake defect and safety margin on LTR was evaluated. Univariate and multivariate analyses were performed to indentify determinant factors of LTR. RESULTS: Of the 128 HCC nodules in 101 patients, 49 (38.3%) nodules in 40 patients showed LTR during follow-up period (median, 34.1 months). Cumulative 1- and 2-year LTR rates of nodules with lipiodol uptake defect (n = 27) and those without defect (n = 101) were 58.1% vs. 10.1% and 72.1% vs. 19.5%, respectively (p < 0.001). Among the 101 nodules without a defect, the 1- and 2-year cumulative LTR rates for nodules with complete safety margin (n = 52) and those with incomplete safety margin (n = 49) were 9.8% vs. 12.8% and 18.9% vs. 19.0% (p = 0.912). In multivariate analyses, ascites (p = 0.035), indistinct tumor margin on cone-beam CT (p = 0.039), heterogeneous lipiodol uptake (p = 0.023), and intra-tumoral lipiodol uptake defect (p < 0.001) were determinant factors of higher LTR. CONCLUSION: In lipiodol chemoembolization, the safety margin in completely lipiodolized nodule without defect will not affect LTR in small nodular HCCs.
Adult ; Aged ; Carcinoma, Hepatocellular/radiography/*therapy ; Chemoembolization, Therapeutic ; Cone-Beam Computed Tomography ; Ethiodized Oil/*administration & dosage ; Female ; Follow-Up Studies ; Humans ; Liver Neoplasms/radiography/*therapy ; Male ; Middle Aged ; Multivariate Analysis ; Neoplasm Recurrence, Local/radiography

PURPOSE: Klebsiella pneumoniae is a major nosocomial pathogen in neonatal intensive care unit (NICU). Sequential outbreaks of K. pneumoniae infection in NICU could occur due to antibiotic resistant strains or persistent environmental sources in NICU. When these outbreaks are happen, epidemiological analysis must be performed to discover the nosocomial sources and clarify the nature of the outbreaks strains. We conducted a molecular epidemiological study to recognize sources and natures of the repeated K. pneumoniae infections in our NICU from July to November 1997. METHODS: Fourteen clinical specimens isolated from K. pneumoniae infected newborn infants and 2 environmental K. pneumoniae strains isolated from surveillance cultures were studied. To establish the epidemiological analysis, we used field inversion gel electrophoresis (FIGE) for genomic DNA typing, and plasmid DNA typing. Also, an antibiogram was obtained from susceptibility tests of isolated K. pneumoniae. RESULTS: The results of genomic DNA typing using FIGE and antibiogram showed 4 different patterns, and plasmid DNA typing analysis showed 9 different patterns. Twelve strains out of 14 clinical isolates were almost identical with the two environmental strains, when comparing the genotypic patterns. And these 12 identical strains of FIGE profile showed similar plasmid DNA patterns. The strains could be classified into 5 different types. CONCLUSION: Plasmid DNA typing method and FIGE have the power to discriminate in epidemiological analysis of K. pneumoniae outbreaks. Our study results suggest that repeated K. pneumoniae infections in NICU can be caused by clusters of K. pneumoniae from environmental sources.
Disease Outbreaks ; DNA ; DNA Fingerprinting ; Electrophoresis, Gel, Pulsed-Field ; Epidemiologic Studies ; Humans ; Infant, Newborn ; Intensive Care, Neonatal* ; Klebsiella pneumoniae ; Klebsiella* ; Microbial Sensitivity Tests ; Molecular Epidemiology ; Plasmids ; Pneumonia

PURPOSE: The purpose of this study was to evaluate the clinical and abdominal ultrasonogra- phic (US) features of spontaneously reduced transient small bowel intussusception in chlidren. METHODS: We retrospectively reviewed the clinical and US findings of 98 children with intussusception who were admitted to the Pediatric Department of Ulsan Dong-Kang General Hospital from Mar. 1999 to Feb. 2000. RESULTS: 1) Among 98 cases, there were 12 cases (12.3%) of transient small bowel intussusception (TSBI) and 86 cases (87.7%) of classic intussusception (CI). 2) The peak incidence of age in TSBI was over 3 years, which was older than that in CI. With regard to sex distribution, male predominated in both type. 3) Clinical symptoms and signs including cyclic irritability, vomiting, bloody stool, and abdominal mass in TSBI group were less common than those in CI group (41.7%, 33.4%, 0.0%, 0.0% vs 91.9%, 59.3%, 41.9%, 26.7%, respectively) but persistent abdominal pain was more common in TSBI group than in CI group (58.5% vs 11.2% ). 4) The size of total target sign and surrounding peripheral hypoechoic rim of TSBI group on US were smaller than those of CI group (11.9±2.61 mm, 2.08±1.15 mm vs 26.91±5.98 mm, 7.86±2.77 mm, respectively). 5) Concomittant illness was found more frequently in TSBI group than in CI group (66.7% vs 26.7%). 6) All case of TSBI group were reduced spontaneouly, which were confirmed by US, but none of CI group. CONCLUSION: Transient small bowel intussusception is probably more common than generally thought and its clinical and US findings is quite different from classical obstructing intussus-ception. Because all of our cases resulted in spontaneous reduction, we recommend careful observation and repeat examination rather than an immediate operation in transient small bowel intussusception.
Abdominal Pain ; Child ; Hospitals, General ; Humans ; Incidence ; Intussusception ; Male ; Retrospective Studies ; Sex Distribution ; Ulsan ; Ultrasonography ; Vomiting

BACKGROUND: Human herpesvirus 6 (HHV6), which was first discovered by Salahuddin in 1986, can be classified as HHV6 A and HHV6 B subgroups. It has been known that HHV6 B, which causes exanthem subitum, is related to the febrile illness of children while HHV6 A can be found in immunodeficiency patients. Over 90% of those who went through primary infection before two years of age show seropositive in adulthood. The virus can be detected in over 85% of the adult saliva. This suggests that the asymptomatic latent virus in normal adult saliva may be a source of primary infection by excretion. The objective of this study is to provide the basic seroepidemiology by finding the age of HHV6 primary infection and the extent of latent infection with classifying subgroups for healthy Korean children. METHODS: Healthy Korean children under the age of fifteen were classified into fifteen groups in total. Those groups under one year old age were divided into twelve groups with the interval of one month while those over one year old age were divided into three groups with the interval of five years. Thirty children were involved in each group, and sex ratio was almostly equal. The specific IgG antibody of HHV6 was evaluated by using an ELISA method. HHV6 DNA extracted from the saliva of the subject of fifty healthy children ranging from ages five to fifteen were detected and classified two subgroups by using a nested PCR. RESULTS: Statistically, the specific IgG antibody, which was highly maintained since neonatal age, became significantly decreased between 4 months and 6 months, and thereafter increased rapidly until it reached a peak for infants between 11 months and 12 months, and maintained a stationary high antibody level and high antibody positive rates (above 85%) until 15 years of age. The HHV6 DNA, detected in 18 (36%) out of 50 normal children between 5 and 15 years old, was totally confirmed as HHV6 B. CONCLUSION: Our data suggest that primary infection of HHV6 for Korean children may be occurred after 6 months age, and primary infection of HHV6 may be almostly happened before 1year age old. And HHV6 is excreted intermittently from the saliva of healthy children as in the case of adults. HHV6 B may be the major cause of HHV6 infection in healthy Korean children.
Adolescent ; Adult ; Child* ; DNA ; Enzyme-Linked Immunosorbent Assay ; Exanthema ; Herpesvirus 6, Human* ; Humans* ; Immunoglobulin G ; Infant ; Polymerase Chain Reaction ; Saliva ; Sex Ratio