No abstract available.
Meningitis* ; Salmonella*

No abstract available.
Fever of Unknown Origin* ; Fever* ; Liver Abscess, Pyogenic*

No abstract available.
Thanatophoric Dysplasia*

PURPOSE: Medium chain acyl-CoA dehydrogenase (MCAD) deficiency is an autosomal recessive disoder of beta oxidation of fatty acids and characterized by episodic hypoglycemia, vomiting, convulsion, encephalopathy, apnea, and sudden death related to fasting or infection resembling Reye syndrome or sudden infant death syndrome. In acute stage, mortality rate is very high and survivors have significant risk of developmental disability and chronic somatic illness. However, the high mortality and morbidity can be totally prevented by appropriate dietary management on the basis of early and accurate diagnosis. Recently, a single point mutation (A985G) in the MCAD gene has been described that accounts for most of MCAD deficiency. The prevalence of MCAD deficiency shows marked racial differences. And population-based DNA screening for this potentially fatal disorder might be justified in countries with high frequency of the mutation. The prevalence of A985G mutation in the MCAD gene was studied in neonates using Guthrie cards for neonatal screening. METHODS: Dried blood spots on Guthrie cards originally used for neonatal screening programs obtained from 500 live newborn babies born in a private obstetric clinic or Seoul Red Cross Hospital in Seoul during the period from Jan. 1, 1995 to Jul. 31, 1995 were collected. DNA was extracted from the dried blood spots, and a segment of the MCAD gene was amplified from the DNA using polymerase chain reaction technique. The PCR products were electrophoresed on a polyacrylamide gel after treatment of a restriction enzyme, NcoI. And the restriction pattern was analyzed with ethidium bromide staining of the gel. RESULTS: The PCR was successful with all DNAs from Guthrie cards. And the A to G transition at nucleotide position 985 in the MCAD gene was not demonstrated in any of the specimen. Conlusions : 1) The frequency of A985G mutation in the MCAD gene is extremely low in Korean population. 2) The methodology used in this study can be applied to population-based molecular genetic studies for other hereditary diseases.
Acyl-CoA Dehydrogenase* ; Apnea ; Death, Sudden ; Developmental Disabilities ; Diagnosis ; DNA ; Ethidium ; Fasting ; Fatty Acids ; Genetic Diseases, Inborn ; Genetics, Population ; Humans ; Hypoglycemia ; Infant, Newborn* ; Mass Screening ; Molecular Biology ; Mortality ; Neonatal Screening ; Point Mutation ; Polymerase Chain Reaction ; Prevalence* ; Red Cross ; Reye Syndrome ; Seizures ; Seoul ; Sudden Infant Death ; Survivors ; Vomiting

PURPOSE: Medium chain acyl-CoA dehydrogenase (MCAD) deficiency is an autosomal recessive disoder of beta oxidation of fatty acids and characterized by episodic hypoglycemia, vomiting, convulsion, encephalopathy, apnea, and sudden death related to fasting or infection resembling Reye syndrome or sudden infant death syndrome. In acute stage, mortality rate is very high and survivors have significant risk of developmental disability and chronic somatic illness. However, the high mortality and morbidity can be totally prevented by appropriate dietary management on the basis of early and accurate diagnosis. Recently, a single point mutation (A985G) in the MCAD gene has been described that accounts for most of MCAD deficiency. The prevalence of MCAD deficiency shows marked racial differences. And population-based DNA screening for this potentially fatal disorder might be justified in countries with high frequency of the mutation. The prevalence of A985G mutation in the MCAD gene was studied in neonates using Guthrie cards for neonatal screening. METHODS: Dried blood spots on Guthrie cards originally used for neonatal screening programs obtained from 500 live newborn babies born in a private obstetric clinic or Seoul Red Cross Hospital in Seoul during the period from Jan. 1, 1995 to Jul. 31, 1995 were collected. DNA was extracted from the dried blood spots, and a segment of the MCAD gene was amplified from the DNA using polymerase chain reaction technique. The PCR products were electrophoresed on a polyacrylamide gel after treatment of a restriction enzyme, NcoI. And the restriction pattern was analyzed with ethidium bromide staining of the gel. RESULTS: The PCR was successful with all DNAs from Guthrie cards. And the A to G transition at nucleotide position 985 in the MCAD gene was not demonstrated in any of the specimen. Conlusions : 1) The frequency of A985G mutation in the MCAD gene is extremely low in Korean population. 2) The methodology used in this study can be applied to population-based molecular genetic studies for other hereditary diseases.
Acyl-CoA Dehydrogenase* ; Apnea ; Death, Sudden ; Developmental Disabilities ; Diagnosis ; DNA ; Ethidium ; Fasting ; Fatty Acids ; Genetic Diseases, Inborn ; Genetics, Population ; Humans ; Hypoglycemia ; Infant, Newborn* ; Mass Screening ; Molecular Biology ; Mortality ; Neonatal Screening ; Point Mutation ; Polymerase Chain Reaction ; Prevalence* ; Red Cross ; Reye Syndrome ; Seizures ; Seoul ; Sudden Infant Death ; Survivors ; Vomiting

BACKGROUND: Heat shock proteins (HSPs), or stress proteins, are immunodominant antigens of many microorganisms. In this study, we have detected the anti-HSP 70 antibody and tried to explain the role of the antibody with respect to the pathogenesis of SLE. Furthermore, we have attempted to find out the possibility to link the presence of the autoantibody with the monitoring and diagnosis of systemic lupus erythematosus (SLE). METHODS: A total of 80 samples from 55 SLE patients were screened for the presence of anti-HSP 70 antibodies. Simultaneously 59 healthy people were tested as a control group. The anti-HSP 70 antibodies were measured by enzyme-linked immunosorbent assay (ELISA) and confirmed by western blot in anti-HSP 70 antibody ELISA positive samples. The activity of disease state was confirmed by the patients' medical record and systemic lupus activity measure (SLAM). RESULTS: The mean optical density (O.D.450) of ELISA in healthy controls and SLE patients were 0.15+/-0.18 (mean+/-S.D.) and 0.13+/-0.14. The correlation of SLAM Score and ELISA O.D. was r2=0.19, P=0.014. And, the mean O.D. value of ELISA was 0.18+/-0.02 and 0.11+/-0.01 before and after treatment (P <0.05). We compared samples with SLAM Score. The O.D. of anti-HSP 70 ELISA in these patients were 0.20+/-0.02 and 0.08+/-0.002 before and after treatment respectively (n=10, mean+/-S.D., P <0.01). CONCLUSIONS: Anti-HSP 70 antibody was not a clinically useful diagnostic marker in SLE patients. However, the titer of anti-HSP 70 antibody can be used for the monitoring of the therapeutic effectiveness in these patients.
Antibodies ; Blotting, Western ; Diagnosis ; Enzyme-Linked Immunosorbent Assay ; Heat-Shock Proteins ; Humans ; Immunodominant Epitopes ; Lupus Erythematosus, Systemic* ; Medical Records

BACKGROUND: To collect high concentration of granulocytes for transfusion to neutropenic cancer patients with infections, we investigated the effect of G-CSF or dexamethasone as granulocyte mobilizers and 10% pentastarch (PS) as the sedimentation agent in granulocyte collection by leukapheresis. Subsequently, the therapeutic effect of the granulocyte transfusions was assessed. METHODS: Forty five leukapheresis were performed with CS-3000Plus (Baxter, Deerfield, IL, USA) using 10% pentastarch. The donors were classified into three groups according to their premedication drugs and the interface detector offset; group 1 used dexamethasone with offset 15 (n=16), group 2 used dexamethasone with offset 33 (n=16), and group 3 used G-CSF with offset 33 (n=10). We compared total collected granulocyte counts and granulocyte collection efficiency (GCE). RESULTS: The mean counts of total granulocytes collected and GCE were as follows; 0.9 0.5 x 1010 and 31.6 14.3% in group 1, 1.3 0.6 x 1010 and 39.0 14.2% in group 2, and 1.6 0.9 x 1010 and 63.9 32.2% in group 3, respectively. The counts of granulocytes collected in group 3 was significantly higher than that in group 1 (P<0.05). The GCE of group 3 was significantly higher than that of group 1 and group 2 (P<0.05). Sixteen granulocyte transfusions were performed to 11 patients. We observed successful therapeutic effects in 10 out of 16 transfusions (63%). CONCLUSIONS: G-CSF indicates greater potency than dexamethasone although its high cost is limitation of routine use as mobilizing agents and PS was an excellent red cell sedimenting agent in granulocyte collection. Large volume granulocyte transfusions allow high therapeutic effects in neutropenic patients with marrows of sufficient regenerating capacity.
Bone Marrow ; Dexamethasone ; Granulocyte Colony-Stimulating Factor ; Granulocytes* ; Humans ; Hydroxyethyl Starch Derivatives* ; Leukapheresis* ; Neutropenia ; Premedication ; Tissue Donors

BACKGROUND: A key to successful peripheral blood stem cell transplantation is to harvest a sufficient amount of hematopoietic stem cells. A method of quickly detecting hematopoietic stem cells in peripheral blood with simple procedures using the SE-9000TM IMI channel (TOA Medical Electronics Co., Ltd., Kobe, Japan) was developed. In this study, usefulness of determining the optimal time for peripheral blood stem cell harvest using IMI channel was investigated. METHODS: Seventy nine peripheral blood stem cell collections were performed from thirteen patients with hematologic malignancy and nineteen patients with solid organ malignancy. In 13 cases, G-CSF was administrered following chemotherapy. In 19 cases only G-CSF was used to mobilize the peripheral blood stem cells. The counts of leukocytes, mononuclear cells, CD34 positive cells, and IMI in peripheral blood and leukapheresis products were determined. RESULTS: The CD34 positive cell count in harvested PBSC showed positive correlation with leukocyte cell, mononuclear cell, CD34 positive cell, and IMI in peripheral blood, with correlation coefficients of 0.48, 0.27, 0.63, 0.66, respectively. Positive correlation was presented between IMI and CD34 positive cell in peripheral blood and harvested PBSC, with a correlation coefficient, 0.83 and 0.74, respectively. CONCLUSIONS: As the SE-9000TM enables determination of the number of PBSC easily and rapidly, within approximately 85 seconds, whereas CD34 assays is expensive and needs skilled operator, the measurement of IMI positive cells is clinically useful for monitoring the peripheral blood stem cell mobilization.
Cell Count ; Drug Therapy ; Electronics, Medical ; Granulocyte Colony-Stimulating Factor ; Hematologic Neoplasms ; Hematology* ; Hematopoietic Stem Cell Mobilization ; Hematopoietic Stem Cells ; Humans ; Leukapheresis ; Leukocytes ; Leukocytes, Mononuclear ; Peripheral Blood Stem Cell Transplantation ; Stem Cells*

BACKGROUND: Although ondansetron is effective at preventing and treating postoperative nausea and vomiting (PONV), the optimal timing of its administration has not been established. In this study we evaluated the effect of the timing of ondansetron administration on its antiemetic efficacy in patients undergoing thyroidectomy. METHODS: One hundred and twelve patients undergoing thyroidectomy were randomized to receive placebo (control group, n = 40) or 70microgram/kg of ondansetron prior to induction (Pre-group, n = 36), or 70microgram/kg of ondansetron at the end of surgery (Post- group, n = 36). The incidence of PONV, adverse events, the need for rescue antiemetics, and nausea severity scores were assessed at 0 to 1 hour and 1 to 24 hours postoperatively. RESULTS: During the first 24 hours after anesthesia, the incidences of PONV in the control, and Pre- and Post-groups were 62.5%, 52.8%, and 52.8%, and there was no significant difference among the groups. During the period 1 hour to 24 hours after anesthesia, the incidences of vomiting (with nausea) and rescue antiemetics were significantly lower in the Pre- and Post-groups than in the control group (P < 0.05). Overall, the incidence of vomiting (with nausea) was significantly lower in the Pre-group than in the control group and the incidence of rescue antiemetics was significantly lower in the Pre- and Post-groups than in the control group (P < 0.05). CONCLUSIONS: In patients with thyroidectomy, the perioperative administration of 70microgram/kg ondansetron was found to reduce the incidence of vomiting and the need for rescue antiemetics. However, the timing of ondansetron administration did not affect antiemetic efficacy.
Anesthesia ; Antiemetics ; Humans ; Incidence ; Nausea ; Ondansetron* ; Postoperative Nausea and Vomiting ; Thyroidectomy* ; Vomiting

PURPOSE: To evaluate MRI and histopathologic findings of toluene inhalation rat brain. MATERIALS AND METHODS: Forty-eight Sprague-Dawley rats, weighing 200-300g, were divided into six groups : the control group and five experimental groups each of eight rats, divided as follows, according to the concentration and duration of inhalation of toluene : 2500 ppm of toluence vapor for 2 hours only, 2 hours daily for 1 week, and 2 hours daily for 3 weeks ; 4000 ppm of toluence vapor for 2 hours only and 2 hours daily for 1 week. For all these five groups, a 0.02 m3; whole body exposure chamber was used. Spin echo and field echo (FE, gradient echo) MR images were obtained at 0.5 T, and then histopathologic examination of the brain was performed. MR signal changes were statistically assessed for contrast to noise ratio (CNR). RESULTS: On T2-weighted MR images, the toluene-inhalation groups revealed diffuse hypointensity in the corpus striatum and thalamus, and diffuse hyperintensity in cerebral white matter, with statistically significant CNR change, compared with the control group. On T1-weighted and FE images, CNR differences in the corpus striatum, thalamus and cerebral white matter between the toluene inhalation groups were not statistically significant. Histopathologic study of these groups showed (1)neuronal degeneration such as shrinkage of neuronal cells and increase of the number of autophagosomes, (2)myelin degeneration and regeneration, and (3)focal axonal degeneration, In groups in which toluene inhalation was at higher concentrations and for longer, these phenomena were more extensive. CONCLUSION: As seen on MRI, toluene inhalation changes the signal intensity of the corpus striatum, the thalamus, and cerebral white matter. Neuronal, myelinic and axonal degeneration probably contribute to these signal changes.
Animals ; Axons ; Brain* ; Corpus Striatum ; Inhalation ; Magnetic Resonance Imaging* ; Myelin Sheath ; Neurons ; Noise ; Rats* ; Rats, Sprague-Dawley ; Regeneration ; Thalamus ; Toluene*