No abstract available.
Hematopoietic Stem Cell Transplantation* ; Hematopoietic Stem Cells*

No abstract available.
Child* ; Down Syndrome* ; Humans

No abstract available.
Turner Syndrome*

No abstract available.
Klinefelter Syndrome*

BACKGROUND: The prognosis for children with relapsed acute lymphoblastic leukemia remains dismal. Ifosfamide has previously been shown to be active as a single agent and in combination with doxorubicin, etoposide, and teniposide in pediatric solid tumors, recurrent acute lymphoblastic leukemia and adult acute leukemia. We assessed the efficacy and the toxicity of the drug combination with ifosfamide and etoposide in patients with relapsed refractory acute lymphoblastic leukemia. METHODS: Between April 1995 and May 1996, twenty children aged 1 to 14 years with ALL in Catholic Medical Center, all heavily pretreated and in bone marrow relapse, were enrolled in this study. Drugs were given intravenously each day for 5 days at the following doses ; ifosfamide 1.8 g/m2/day, etoposide 100 mg/m2/day and mesna 1440 mg/ m2/day(as a uroprotectant) ; Cycles were repeated every 28 days for two cycles. RESULTS: 1) Twenty heavily pretreated patients were entered on study. At study entry, seventeen patients were in first relapse, two were in second relapse and one was in third relapse. 2) Six patients(30%) achieved complete remission, and eight patients(40%) achieved partial remission. Overall response rate was 70%. 3) Duration of remission ranged from 30 days to 230 days. 4) The toxicity of the regimen was tolerated. Moderate or severe toxicity evaluated on a per cycle basis included : neutropenia 52.5%, thrombocytopenia 45%, hemorrhagic cystitis 12.5% and mucositis 2.5%. 5) Two patients went on to bone marrow transplantation with histocompatibility matched sibling donors while in remission. CONCLUSION: The combination of ifosfamide and etoposide with mesna uroprotection has significant activity in relapsed refractory childhood lymphoblastic leukemia with tolerable toxicity. We recommended bone marrow transplantation after successful reinduction because of short remission duration of this regimen.
Adult ; Bone Marrow ; Bone Marrow Transplantation ; Child ; Cystitis ; Doxorubicin ; Etoposide* ; Histocompatibility ; Humans ; Ifosfamide* ; Leukemia ; Mesna ; Mucositis ; Neutropenia ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Prognosis ; Recurrence ; Siblings ; Teniposide ; Thrombocytopenia ; Tissue Donors

Eight strains of multidrug-resistant (MDR) Salmonella typhi were isolated from Kyonggi area during January-February,1997. They were resistant to ampiciUin, amoxicillin, carbeniciillin, tetracycline, chloramphenicol, trimethoprim/sulfamethoxazole, trimethoprim. Eight strains had one plasmid respectively which size was approximately M.W 220 kb and showed same restriction pattern by endonuclease HindIII. The plasmid was similar to the plasmid in size that was related to multidrug resistant S. typhi isolated from southeast Asia. It were transferred by conjugation to recipient E, coli K-12 in frequency of 2.43 x10-4 - 1.73 x 10-2 and transconjugant showed same drug-resistant pattem with donor cells. All of 8 strains produced B-lactamase that was assummed to TEM-1 type by isoelectric focusing and PCR.
Amoxicillin ; Asia, Southeastern ; Chloramphenicol ; Deoxyribonuclease HindIII ; Gyeonggi-do ; Humans ; Isoelectric Focusing ; Korea* ; Plasmids* ; Polymerase Chain Reaction ; Salmonella typhi* ; Salmonella* ; Tetracycline ; Tissue Donors ; Trimethoprim

BACKGROUND: This study was carried out to evaluate the efficacy of desferrioxamine as a chelating agent in iron overloaded patients with severe aplastic anemia due to multiple transfusion. METHODS AND MATERIALS: From Oct. 1995 to Aug. 1996, 15 patients with aplastic anemia, diagnosed from May 1995 to Jan. 1996 at St. Mary's Hospital, who had a transfusional hemosiderosis were included in this study. They received 19 courses of high-dose desfer-rioxamine therapy for 6 days(20 to 30 mg/kg daily as a 24-hour intravenous infusion) . Before and after treatment, we measured serum ferritin, iron, TIBC, 24-hour urinary excretion of iron. RESULTS: 1) The range of iron load before treatment was between 4.5 and 20.0 gram. 2) Because of limit of detection(1,800 microgram/L), it was difficult to compare the changes of serum ferritin level after therapy to those of before therapy. 3) There was no significant differences between the levels of serum iron before and after therapy(214.3+/-62.8 vs 220.0+/-53.3). And there was no significant differences between TIBC before and after therapy(235.8+/-64.6 vs 259.4+/-60.1). 4) Iron/TIBC ratios were significantly deceased after desferrioxamine treatment compared to those of before therapy(0.90+/-0.04 vs 0.85+/-0.04, P<0.001) and mean urinary excretions of iron were increased by high-dose desferrioxamine compared to those by test dose(6.5+/-7.6 vs 29.1+/-14.3, P<0.001) CONCLUSION: High-dose desferrioxamine therapy is very effective for chelating and excretion of iron in iron overloaded patients with severe aplastic anemia due to multiple transfusion. A repeat administration of desferrioxamine is necessary for the iron overloaded patient to eliminate the risk of a transfusional hemosidersis.
Anemia, Aplastic* ; Deferoxamine* ; Ferritins ; Hemosiderosis* ; Humans ; Iron ; Iron Overload

urpose:Adipocyte specific hormone, leptin is thought to regulate appetite and body weight, and serum level of leptin correlate with body fat content not only in adulthood but also in childhood. Adults who received cranial irradiation for childhood acute lymphoblastic leukemia are prone to hypothalamic damage in the form of growth hormone deficiency and leptin insensitivity. The purpose of this study is to evaluate the effect of total body irradiation on serum leptin concentration at short- term follow-up. METHODS:Twenty-four acute leukemia patients undergoing bone marrow transplantation(BMT) were enrolled in this study. They were divided into total body irradiation(TBI) group(n=13) and non total body irradiation(NTBI) group(n=11). Body mass index and leptin concentration at 1 week before BMT and 1 day, 1 week and 4 weeks after BMT were checked. Serum leptin concentration was measured by RIA method. RESULTS:Leptin concentration was well correlated with BMI(r=0.689, P<0.05). BMI were 17.96+/-2.48, 17.58+/-2.39, 17.39+/-2.30, 17.74+/-2.31 in TBI group and 16.91+/-1.88, 16.53+/-1.91, 16.50+/-1.96, 16.78+/-1.82 in NTBI group at 1 week before BMT and 1 day, 1 week and 4 weeks after BMT. Lepin concentrations were 4.31+/-3.57ng/ml, 3.22+/-2.87ng/ml, 3.78+/-4.41ng/ml, 3.46+/-2.96ng/ml in TBI group and 3.46+/-1.84ng/ml, 2.60+/-1.11ng/ml, 2.71+/-1.61ng/ml, 3.61+/-3.27ng/ml in NTBI group at 1 week before BMT and 1 day, 1 week and 4 weeks after BMT. BMI was different at each time period(P<0.05) but not different between two groups. Lepin concentration was not different between two groups and at each time period. CONCLUSION: Serum leptin concentration was not changed, but BMI was significantly decreased by total body irradiation at short-term follow-up.
Adipose Tissue ; Adult ; Appetite ; Body Mass Index ; Body Weight ; Bone Marrow Transplantation* ; Bone Marrow* ; Child* ; Cranial Irradiation ; Follow-Up Studies ; Growth Hormone ; Humans ; Leptin* ; Leukemia* ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Whole-Body Irradiation*

No abstract available.
Molecular Biology

Aplastic anemia is a rare disease, which is characterized by pancytopenia and hypocellular bone marrow without infiltration of abnormal cells or fibrosis. The incidence in Asia is higher than in the West and new cases are diagnosed at a rate of 5.1 per million pediatric populations per year in Korea. The pathophysiology is understood roughly by defective hematopoiesis, impaired bone marrow microenvironment and immune mechanism. Treatments are performed on basis of pathogenesis and selected depending on the severity. Immunosuppressive therapy with antilymphocyte or antithymocyte globulin and cyclosporine is effective in the majority of patients but has some problems including relapse or clonal evolution. Recently, there have been clinical trials of immunosuppression with hematopoietic growth factors or other drugs. Allogeneic hematopoietic stem cell transplantation (HSCT) is curative in children with severe aplastic anemia. The overall survival in HSCT from HLA-identical sibling is higher than alternative donor, including HLA matched unrelated donor or cord blood. We have to consider quality of life after HSCT because of high survival rate. However, chronic graft versus host disease and graft failure are important factors that affect the quality of life and overall survival. We need further investigation to make new regimens aimed at overcoming these risk factors and perform clinical trials.
Anemia, Aplastic* ; Antilymphocyte Serum ; Asia ; Bone Marrow ; Child ; Clonal Evolution ; Cyclosporine ; Fetal Blood ; Fibrosis ; Graft vs Host Disease ; Hematopoiesis ; Hematopoietic Stem Cell Transplantation ; Humans ; Immunosuppression ; Incidence ; Intercellular Signaling Peptides and Proteins ; Korea ; Pancytopenia ; Quality of Life ; Rare Diseases ; Recurrence ; Risk Factors ; Siblings ; Survival Rate ; Tissue Donors ; Transplants ; Unrelated Donors