To analyse the age of onset, etiologic microorganisms, clinical manifestations, managements and genetic variation of chromic granulomatous disease, the authors reviewed four patients who were diagnosed as CGD at Department of Pediatrics, Seoul National Univesity Children's Hospital. They were siblings in relationship-two of them were brothers, the others were brother-sister. @ES The results were as follows; 1) Initial manifestations developed within 1 year old, and lymphadenopathy associated with BCG vaccination was the most common. 2) In culture study of micro-organism, catalase positive microorganisms such as Staphylococcus aureus, Serratia marcescens, Coagulase (-) Staphylococcus, Enterococcus, Proleus vulgaris, Klebsiella pneumoniae, E. coli and fungus such as Candida albicans were isolated. In 2 cases, culture study revealed no growth. 3) Fever, lymphadenopathy, hepatomegaly, suppurative dermatitis and pneumonia were the most common manifestations. In most of cases, antituberculous medications were administered under the impression of tuberculosis without response. 4) Immunologic screening tests including B-cell system, T-cell system, and complement system were within normal limit except NBT test. 5) In spite of severe infections, NBT scores of all the cases were less than those of controls. Liver biopsies of 3 cases showed granuloma formation with characteristic yellow brown pigment-laden macrophages.
Age of Onset ; B-Lymphocytes ; Biopsy ; Candida albicans ; Catalase ; Coagulase ; Complement System Proteins ; Dermatitis ; Enterococcus ; Fever ; Fungi ; Genetic Variation ; Granuloma ; Hepatomegaly ; Humans ; Klebsiella pneumoniae ; Liver ; Lymphatic Diseases ; Macrophages ; Mass Screening ; Mycobacterium bovis ; Pediatrics ; Pneumonia ; Seoul ; Serratia marcescens ; Siblings ; Staphylococcus ; Staphylococcus aureus ; T-Lymphocytes ; Tuberculosis ; Vaccination

No abstract available.
Nephrotic Syndrome*

Following chemotherapy for acute myelomoncytic leukemia, an 3-year-old boy developed several painful erythematous indurated patches at previous sampling sites and at the site where an arm board and adhesive tape were used in securing an intravenous infusion set. The lesions rapidly progressed to ulcers with central black eschars. Biopsies and cultures demonstrated a fungus, Aspergillus flavs, as the etiologic agent, without evidence of systemic dissemination. Local care, including s irgical debridement, were performed. The patient also received treatment with intravenous; amphotericin B followed by oral itraconazole. Six months later, the skin lesions had healed, leaving some scar and deformities.
Adhesives ; Amphotericin B ; Arm ; Aspergillosis* ; Aspergillus ; Biopsy ; Child* ; Child, Preschool ; Cicatrix ; Congenital Abnormalities ; Debridement ; Drug Therapy ; Fungi ; Humans ; Infusions, Intravenous ; Itraconazole ; Leukemia* ; Male ; Skin ; Ulcer

No abstract available.
Child* ; Humans ; Lupus Erythematosus, Systemic*

No abstract available.
Prescriptions*

No abstract available.
Clone Cells* ; Cloning, Organism* ; DNA* ; Genome* ; Nucleopolyhedrovirus* ; Plasmids*

D variant of encephalomyocarditis (EMC-D) virus causes diabetes in susceptible mice by direct infection and cytolysis of pancreatic beta cells. cDNA covering the major outer capsid protein (VP1) of EMC-D virus was cloned into Mycobacterium bovis bacillus Calmette-Guerin (BCG). None of the SJL/J male mice, immunized with live recombinant BCG-VP1, became diabetic when challenged with highly diabetogenic EMC-D virus. But the control mice inoculated with normal BCG or rBCG transformed with vector alone developed diabetes in the same challenge. VP1-specific antibodies including neutralizing antibodies were markedly increased as time went on and reached to the maximum titer at week 10 after a single immunization. The plateau of the titer lasted longer than following 4 weeks. Guinea pigs immunized with the live rBCG-VP1 showed strong delayed type hypersensitivity (DTH) to the VP1of EMC-D virus. It means that the live rBCG-VP1 elicit efficient humoral and cell-mediated imrnune responses against EMC-D virus, resulting in prevention of virus-induced diabetes in susceptible mice.
Animals ; Antibodies ; Antibodies, Neutralizing ; Bacillus ; Capsid Proteins ; Clone Cells ; DNA, Complementary ; Guinea Pigs ; Humans ; Hypersensitivity ; Immunization* ; Insulin-Secreting Cells ; Male* ; Mice* ; Mycobacterium bovis*

BACKGROUND: Herpes simplex virus encephalitis (HSVE) is a severe disease resulting in high mortality rates and residual sequelae; therefore, early diagnosis and treatment are very important. The purpose of this study was to evaluate the usefulness of polymerase chain reaction (PCR) assay of HSV DNA in cerebrospinal fluid (CSF) from patients with encephalitis for confirmatory diagnosis of herpes simplex virus encephalitis. MATERIALS AND METHODS: PCR analysis was done to detect DNA of HSV in 31 CSF samples obtained from 21 patients with encephalitis, admitted between November 1994 and June 1996, at the Seoul National University Children's Hospital. As controls, 15 samples from patients with proven diseases other than encephalitis were tested. Two different 22 bp- rimers, complementary to sequences within the DNA polymerase regions of HSV gene, were used. After amplification, PCR products were digested with DdeI to confirm the sequence. RESULTS: Three CSF specimens from 2 out of 21 patients with encephalitis were positive for HSV DNA, whereas all the control specimens were negative. PCR was positive in CSF samples obtained at 3 and 7 days after onset of symptoms from one patient and at 5 days from the other. PCR analysis yielded negative results in CSF samples collected at 10 and 14 days after acyclovir treatment. CONCLUSION: In this study, HSVE was diagnosed by PCR amplification of HSV genome from CSF obtained at early stage of the disease from patients with encephalitis. PCR assay of CSF samples could be used for early and confirmatory diagnosis of HSVE.
Acyclovir ; Cerebrospinal Fluid ; Diagnosis* ; DNA ; Early Diagnosis ; Encephalitis ; Genome ; Herpes Simplex* ; Humans ; Mortality ; Polymerase Chain Reaction* ; Seoul ; Simplexvirus*

No abstract available.
Child* ; Humans ; Listeria monocytogenes ; Listeria* ; Listeriosis* ; Meningitis ; Sepsis

BACKGROUND AND OBJECTIVES: Treatment results of laryngeal carcinoma may depend on the microscopic environment of the primary lesion. If there is an abundance of lymphatic channels, the laryngeal lesion is more likely to develop frequent cervical metastases, leading to treatment failure. Prognosis of the laryngeal carcinoma is usually determined by clinical staging. However, it does not account for the oncobiological characteristics and microscopic environments. The importance of biological markers including p53, Rb and MIB1 as a prognostic factor has yet to be determined. To determine the relationship between the prognosis and biological characteristics of p53, Rb, and MIB1 in laryngeal cancers. MATERIALS AND METHODS: Thirty seven laryngeal carcinoma specimens were studied for p53 gene mutation by PCR-SSCP and expression pattern of Rb and MIB1 proteins by immunohistochemistry. RESULTS: p53 gene mutation was found in 32.4% with nine cases in exon 5, two cases in exon 7, and two cases in exon 8, respectively. Higher incidence of p53 gene mutation was found in supraglottic cancer (p>0.05). Negative expression rate for Rb was 29.7% and positive MIB1 expression rate was 32.4%. There was no significant correlation between the treatment success and p53 gene mutation, expression pattern of Rb and MIB1 gene. CONCLUSION: p53 gene mutation and expression pattern of both Rb and MIB1 showed little value as a prognostic factor. However, in cases where p53 and Rb tumor suppressor gene mutation were found in combination, the prognosis was poor. In this regard, such marker might be used as one of the important biological prognostic factors.
Biomarkers ; Carcinoma, Squamous Cell* ; Exons ; Genes, p53 ; Genes, Tumor Suppressor ; Immunohistochemistry ; Incidence ; Laryngeal Neoplasms ; Neoplasm Metastasis ; Population Characteristics ; Prognosis ; Treatment Failure