No abstract available.
Cell Line* ; Hantaan virus* ; Humans*

Vascular leak syndrome(VLS) is characterized by endothelial damage, which causes extravasation of plasma proteins and fluid from capillaries into the extravascular space. It has been suggested that the increased vascular permeability is the result of an IL-2-induced suppression of endothelin-1 secretion by endothelial cells, an IL-2-induced activation of the complement cascade or TNF-alpha release from IL-2-activated T-cells. A 13-year-old male patient visited our hospital with abdominal pain. He was diagnosed with acute lymphoblastic leukemia(T cell type) by bone marrow study on his 2 nd day in hospital. On the 3 rd day of induction chemotherapy(prednisone, L-asparaginase, vincristine, intrathecal methotrexate), pulmonary edema and pleural effusion, ascites and generalized edema developed and lasted for 53 days without responding to supportive care. The laboratoy finding was that TNF-alpha was increased without evidence of infection and hypoalbuminemia was noted. It was suggested that the patient's clinical feature was induced by VLS.
Abdominal Pain ; Adolescent ; Ascites ; Blood Proteins ; Bone Marrow ; Capillaries ; Capillary Permeability ; Complement System Proteins ; Edema ; Endothelial Cells ; Endothelin-1 ; Humans ; Hypoalbuminemia ; Induction Chemotherapy* ; Male ; Pleural Effusion ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Pulmonary Edema ; T-Lymphocytes ; Tumor Necrosis Factor-alpha ; Vincristine

No abstract available.
Herpesvirus 3, Human* ; Korea* ; Polymorphism, Restriction Fragment Length*

No abstract available.
Animals ; Cell Line* ; Guinea Pigs* ; Guinea* ; Hantaan virus* ; Humans*

We immunized sixty two healthy subjects with the five different viral titers (300, 500, 1000, 1500 and 2000 plaque forming unit; pfu) of the MAV/06 strain of live attenuated Varicella-zoster virus (VZV) in order to gain sufficient information on safety and immuogenicity as a vaccine strain. Humoral immunity of all vaccine recipients was tested by the fluorescent antibody to membrane antigen (FAMA) assay and Enzyme-linked immunosorbent assay (ELISA) for the quantitative detection of IgG antibody. We tested neutralized antibody in 62 subjects by plaque reduction neutralization test (PRNT50). All of thirty two subjects with initial seronegative response had antibody by FAMA method at four weeks after immunization with four different preparations of dosage. The geometric mean titers (GMTs) of VZV antibody to membrane antigen was 160.9 in 6 subjects with 1500 pfu group; 83.3 in 14 subjects with 1000 pfu group: 116.2 in 7 subjects with 500 pfu groups and 72.0 in 6 subjects with 300 pfu group. Thirty subjects who had VZV antibody at the time before immunization demonstrated elevated antibody titer by FAMA assay and PRNT50 test. Side reactions of the vaccination was not demonstrated in all cases.
Chickenpox* ; Enzyme-Linked Immunosorbent Assay ; Herpesvirus 3, Human ; Immunity, Humoral ; Immunization ; Immunoglobulin G ; Membranes ; Neutralization Tests ; Vaccination

No abstract available.
Herpesvirus 3, Human* ; Korea*

No abstract available.
Herpesvirus 3, Human* ; Korea*

Sclerosing panniculitis refers to indurated erythema with hyperpigmented scleroderma-like hardening on the medial lower third of the legs. It has been called indurated cellulitis, chronic cellulitis, hypodermitis sclerodermiformis or lipodermatosclerosis. It has been suggested that sclerosing panniculitis is associated with venous insufficiency. The treatment of sclerosing panniculitis is very difficult. Stanozolol is valuable in treating intractable sclerosing panniculitis, giving relief of pain, reducing induration, inflammation, tenderness and pigmentation by its fibrinolytic activity. We report a case of sclerosing panniculitis, clinically improved by stanozolol.

PURPOSE:The lesion of Congenital Lipoid Adrenal Hyperplasia has been suggested to be in the 1st step of steroidogenesis of conversion of cholesterol to pregnenolone by P450scc. In 1995, however, the molecular defect of this disease has been located in the transport of cholesterol into mitochondria due to defective regulatory protein called Steroidogenic Acute Regulatory Protein(StAR), while the enzyme P450scc itself is normal. This genetic study using automatic sequence analyzer aimed at elucidating the molecular defect in the StAR gene of the two patients. METHODS:This study was performed on the two patients of Congenital Lipoid Adrenal Hyperplasia. Both children were phenotypically females. However, one turned out to have a karyotype of 46, XY, the other 46, XX. Genomic DNAs were extracted from their peripheral blood. We amplified the last exon, hot spot, of the StAR gene using 1 set of primer, S4, 5'-CCT GGC AGC CTG TTT GTG ATA G-3' AS4, 5'-CCT CAT GTC ATA GCT AAT CAG TG-3'. Subsequently, one PCR product have been directly sequenced by dideoxy termination method, and also the other products(patient's and her father's) have been sequenced by automatic sequence analyzer. RESULTS:The mutation was identified in the last exon of the StAR gene, substituting T for A at codon 258, replacing glutamine by stop codon in the two unrelated Korean patients with congenital lipoid adrenal hyperplasia. One patient were found to be homozygote, but the other to be heterozygote for the mutation. CONCLUSIONS:These results indicate that Korean children with congenital lipoid adrenal hyperplasia may be genetically identical as in Japanese. But, we discovered that the hot spot, codon 258, are not always homozygote. We want to emphasize the different point, and to say that we did experiece the automatic sequence analyzer successfully.
Animals ; Asian Continental Ancestry Group ; Cats ; Child ; Cholesterol ; Codon ; Codon, Terminator ; DNA ; Exons ; Female ; Glutamine ; Heterozygote ; Homozygote ; Humans ; Hyperplasia ; Karyotype ; Mitochondria ; Molecular Biology* ; Polymerase Chain Reaction ; Pregnenolone

Diffuse plane xanthoma is a group of plane xanthomas that appear as yellow-brown flat patches or slightly elevated plaques with a wide-spread distribution. Cutaneous xanthomas may occur either hyperlipoproteinemic or normolipoproteinemic states. Diffuse plane xanthoma with normolipoproteinemia is often associated with multiple myeloma, other reticulo-endothelial malignancies and monoclonal gammopathy of unknown significance(MGUS). We report a case of diffuse plane xanthoma associated with monoclonal gammopathy of unknown significance(MGUS) and normolipoproteinemia.
Multiple Myeloma ; Paraproteinemias* ; Xanthomatosis*