BACKGROUND: The survival rate of children with neuroblastoma has been improved over 20 years, excluding the metastatic disease, in which it does not exceed 20% so far. New treatment modalities have been developed to improve the outcome in metastatic disease. Preoperative chemotherapy reduce the size, the vascularity and the adhesiveness, so increase the resectability of the primary tumor. This retrospective clinical study was designed to review the survival rate in neuroblastoma and to analyze the effect of preoperative chemotherapy in the view point of neoadjuvant therapy on long-term survival in advanced disease. METHODS: One hundred and thirty-four cases were reviewed from 135 patients with neuroblastoma registered at the Department of Pediatrics in Seoul National University Children's Hospital from January, 1985 till December, 1995. The survival rate was reviewed according to the stage. The age, sex of the patients, the stage, anatomical site of the tumor, the level of serum ferritin and neuron-specific enolase were analyzed for the risk factors on survival. RESULTS: Ranges of age at diagnosis were from 1 month to 166 months with the median of 39 months. Five year survival rates and five year disease-free survival rates were 100%, 100% in stage 1(n=5), 90.9%, 90.9% in stage 2(n=13), 43.4%, 40.6% in stage 3(n=19), 27.1%, 19.8% in stage 4(n=95) and 100%, 100% in stage 4S(n=2), respectively. In stage 3, five year survival rate was 52.5% in group receiving neoadjuvant chemotherapy, 28.6% in control group(P=0.02). Five year disease-free survival rate was also noted as 48.6%, 28.6% in each group(P=0.02). In stage 4, five year and ten year survival rates were 27.6%, 23.6% in group receiving neoadjuvant chemotherapy, 26.9%, 0% in control group(P=0.02). Five year and ten year disease-free survival rates were 14.3%, 14.3% in group receiving neoadjuvant chemotherapy, 20%, 0% in control group (P=0.11). In univariate analysis, the age, the stage, and the site of primary tumor appeared to affect the long-term survival. CONCLUSION: Neoadjuvant chemotherapy and delayed primary surgery contribute for advance in survival in advanced neuroblastoma via increasing the resectability of the primary tumor.
Adhesiveness ; Child ; Diagnosis ; Disease-Free Survival ; Drug Therapy* ; Ferritins ; Humans ; Neoadjuvant Therapy ; Neuroblastoma ; Pediatrics ; Phosphopyruvate Hydratase ; Retrospective Studies ; Risk Factors ; Seoul ; Survival Rate

No abstract available.
Magnetic Resonance Imaging* ; Osteosarcoma*

A case of primary gastric carcinoma with patterns resembling those of hepatocellular carcinoma(HCC) is reproted. This patient who represented high serum alpha-fetoprotein(AFP) underwent a subtotal gastrectomy for stomach cancer and lobectomy of liver metastasis. The hepatoid component of the tumor was characterized by solid nests of large polygonal cells arranged in cords and trabeculae, with central nuclei, prominent nucleoli, and abundant eosinophilic cytoplasm; single-nuclear giant cells were frequently noted. These tumor cells of stomach stained positive immunohistochemically for AFP, ACT(alpha-1-antitrypsin), AACT(alpha-1-antichy-motrypsin). Thus, this hepatoid gastric adenocarcinoma tumor cells demonstrated both morphologic and immunohistochemical features of partial differentiation to the HCC. Careful histologic examination in conjunction with the immunohistochemical demonstration of AFP can provide a useful contribution to the diagnosis of this rare histological type of gastric carcinoma And the presence of metastatic hepatoid adenocarcinoma from the stomach should be considered in a patient with hepatic tumor mimicking HCC. Because of frequent venous and lymphatic invasion, as well as liver metastasis, prognosis appears to be poor. The authors reviewed 45 cases of hepatoid adenocarcinoma of stomach reported in the literature, also.
Adenocarcinoma ; Carcinoma, Hepatocellular ; Neoplasm Metastasis ; Stomach Neoplasms

No abstract available.

BACKGROUND: Hereditary spherocytosis(HS) is a clinically and biochemically very heterogeneous disorder The purpose of this study is to detect erythrocyte membrane protein abnormalities by SDS-PAGE and to investigate the frequency of erythrocyte membrane protein defects in hereditary spherocytosis and correlation between some of the hereditary spherocytosis biochemical subsets and the selected clinical phenotype. METHODS: We evaluated the clinical and laboratory characteristics of 14 normal healthy persons and 23 hereditary spherocytosis patients and 8 their family members. The patients were divided into three groups based on clinical and hematological severity(mild, typical, severe). In addition to routine hematologic determlnatlons, osmotic fragility and autohemolysis, RBC membrane protein analysis were performed in all patients by densitometric tracing of SDS-PAGE(sodium dodecyl sulphate polyacrylamide gel electrophoresis) stained by Coomassle blue utilizing both the discontinuous buffer system of Laemmli with acrylamide linear gradient from 4% to 12% and the continuous buffer system of Fairbank with exponential gradient of acrylamide from 3.5% to 17%. RESULTS: 1) The patients could be seperated into three classes of different clinical severity as mild(3 cases), moderate(16 cases) and severe(4 cases) on the clinical feature. 2) Eighteen patients(82.6%) among 23 hereditary spherocytosis revealed abnormal erythrocyte membrane protein and we detected six patients(26.1%) with spectrin deficiency combined with ankyrin reduction, 4 patients(17.4%) with ankyrin deficiency, 4 patients(17.4%) with isolated spectrin deficiency and 3 patients(13.0%) with band 3 deficiency. Five HS patients(21.7%) showed normal RBC membrane protein. 3) Eight HS and their family members showed same RBC membrane protein deficiency. 4) The type and degree of RBC membrane protein reduction were variale with spectrin at 66~94%, with ankyrin at 48~82% of normal levels. These showed that each patient had different clinical severities according to different RBC membrane protein levels and type. CONCLUSION: RBC membrane protein abnormalities were observed in 82.6% of HS patients. The combined spectrin and ankyrin deficiency is the most common molecular defect in HS. The clinical severity and biochemical expression is heterogeneous. SDS-PAGE analysis of RBC membrane protein was provided the diagnosis of RBC membrane defects and basic molecular studies. We believed that the early identification of the biochemical defect responsible for HS is important because it is helpful starling point for the identification of the primary molecular defect, and it could help to anticipate the clinical outcome of the disease. For these reasons, we consider the SDS-PAGE of the red cell membrane to be of crucial importance for a complete evaluation of children with HS. Further studies with more cases would be to clarify the correlation between clinical and biochemical phenotypes.
Acrylamide ; Ankyrins ; Cell Membrane ; Child ; Diagnosis ; Electrophoresis, Polyacrylamide Gel* ; Erythrocyte Membrane* ; Erythrocytes* ; Erythrocytes, Abnormal ; Humans ; Membrane Proteins ; Membranes ; Osmotic Fragility ; Phenotype ; Population Characteristics* ; Spectrin ; Starlings

Ehlers-Danlos syndrome type IV(the arterial-ecchymotic type) which has skin fragility, easy bruisability, and j oint hyperextensibility is occasionally combined with large vessel involve-ment and spontaneous catastrophic bleeding. As even a small inj ury can cause profound vascular tearing and damage, surgical management is hazardous and often unrewarding. We report a successful surgical treatment of an acute type A aortic dissection associated with Ehlers-Danlos syndrome.
Ehlers-Danlos Syndrome* ; Hemorrhage ; Skin

Acinetobacter species encounters frequently with clinical specimens and now accounts for a substantial proportion of endemic nosocomial infections in Korea. Recent trends indicate that the antimicrobial resistant strains of Acinetobacter species are increasing. Sixty-one strains were isolated from specimens of patients suspected of nosocomial infections during 1991 to 1996. At present, phenotypic identification of Acinetobacter using biochemical test may not be reliable and resulted in the difficulty to clarify the source of infections and epidemiological study of hospital-acquired infections. Aware of the importance of rational taxonomic proposal for these isolates, correct species identification of these organisms by molecular typing method was carried out. A total of fifty-four strains of A. calcoaceticus-A. baumannii complex species which were identified to genospecies 2 and 13 by biochemical characteristics was subjected to identify by ribotyping using restriction endonuclease EcoRI, ClaI, and SalI. Of fifty-four strains, twenty-five strains were identified as A. baumannii (genospecies 2) and twenty-one strains as genospecies 13, and six strains changed to genospecies 3, and the rest two strains were confirmed as A. haemolyticus (genospecies 4). This result suggests that the ribotyping may be of value for identification of genospecies and epidemiological information of Acinetobacter strains.
Acinetobacter baumannii* ; Acinetobacter calcoaceticus* ; Acinetobacter* ; Cross Infection ; DNA Restriction Enzymes ; Humans ; Korea ; Molecular Typing ; Ribotyping*

We described the clinical manifestation of herpes zoster in thirteen children with underlying malignancies. Among the associated malignancies, hematologic malignancy including acute lymphotytic leukemia was the commonest and CNS tumors were also frequently associated. Pain was mild, and some showed high fever and abnormal liver function test results. Recurrent attacks were observed in 3 cases(23%). Thoracic segment and trigeminal nerve were commonly affected. In most cases, herpes zoster developed within two years after the diagnosis of the malignancy.
Child* ; Diagnosis ; Fever ; Hematologic Neoplasms ; Herpes Zoster* ; Humans ; Leukemia ; Liver Function Tests ; Trigeminal Nerve

No abstract available.
Child* ; Humans ; Leukemia, Myeloid, Acute* ; Typhlitis*

BACKGROUND: Cytokine-mediated ex vivo expansion has been proposed as a means of increasing the number of cord blood (CB) hematopoietic stem cells for transplantation. As well as stem cell number, stromal cells are necessary for functional maturation of hematopoiesis. The purpose of this study was to analyze the development of stromal cells during ex vivo expansion of CB CD34+ cells. METHODS: CD34+ cells were purified from CB by magnetic bead selection. The levels of of interleukin-3, interleukin-1 beta, interleukin-6, granulocyte macrophage-colony stimulating factor and tumor necrosis factor-alpha were measured in culture supernatants on 0, 1, 2, and 3 weeks, using ELISA techniques. CB CD34+ cells were expanded in Iscoves modified Dulbeccos medium in the presence of several cytokines. The expression of E-selectin, vascular cell adhesion molecule- 1, intercellular adhesion molecule- 1, platelet/endothelial cell adhesion molecule-1, von Willebrand factor, vimentin, and CD14 in newly developed stromal cells was examined by immunocytochemical method. Relevant extracellular matrix (ECM) proteins and proper cytokines were also assayed for the most suitable condition for expansion of stromal cells. RESULTS: Several cytokines were found to have been produced by CB CD34+ cells as well as bone marrow-derived CD34+ cells. During ex vivo expansion of CB CD34+ cells, stromal cells appeared in the culture by day 4 and expanded over the following 7- 10 days before being confluent by day 2 1. These cells expressed surface markers characteristic of cells of endothelial lineage. Furthermore, these stroaml cells also expanded effectively when treated with thrombopoietin+flt-3 ligand+stem cell factor+leukemia inhibitory factor or 0. 1% poly-L-lysine-coated wells. CONCLUSION: Stromal cells were developed during ex vivo expansion of CB CD34+ cells and that this development could be enhanced further by treating the stromal cells with cytokines or ECM.
Cell Adhesion ; Cytokines ; E-Selectin ; Enzyme-Linked Immunosorbent Assay ; Extracellular Matrix ; Fetal Blood* ; Granulocytes ; Hematopoiesis ; Hematopoietic Stem Cells ; Interleukin-1beta ; Interleukin-3 ; Interleukin-6 ; Stem Cells ; Stromal Cells* ; Tumor Necrosis Factor-alpha ; Vimentin ; von Willebrand Factor