Dysplasia epiphysialis punctata is a rare congenital disorder of infancy affecting in particular cartilage, muscle, jointtcapsules and the eyes. A case of dysplasia epiphysialis punctata with involvement of all epiphyses of extremities, spine and pelvis in 2 days old male is to be reported with review of literature.
Cartilage ; Chondrodysplasia Punctata ; Congenital, Hereditary, and Neonatal Diseases and Abnormalities ; Epiphyses ; Extremities ; Humans ; Male ; Pelvis ; Spine

No abstract available.
Anemia, Aplastic* ; Antilymphocyte Serum* ; Granulocyte-Macrophage Colony-Stimulating Factor* ; Granulocytes* ; Humans*

No abstract available.
Cytarabine* ; Cytosine* ; Humans ; Idarubicin* ; Induction Chemotherapy* ; Leukemia, Myeloid, Acute*

BACKGROUND: Blood transfusion is important for life saving treatment in many patients with tolerable adverse effects. Some data suggest that transfusions might cause an increased risk for post-operative infections and a higher relapse or mortality rate in cancer patients. We investigated whether immune dysfunction might result after transfusions from the cellular components. METHODS: We studied 5-week-old mice BALB/c (H-2d, donor), C3H/He (H-2k, recipient), and C57/BL (H-2b, third party). We obtained irradiated spleen cells (SP) from the BALB/c or C57/BL, and injected them into the C3H/He with intraperitoneal IL-2 administration. After 24 hours, we obtained bone marrow (BM), thymus and SP. We identified mixed lymphocyte proliferation (MLR) by the BrdU method and we used irradiated BALB/c SP, as a stimulator for that trial. For the analysis of immune cells, we analyzed the cell surface markers from each organ. For cytokines, we identified TNF-alpha, IFN-gamma, TGF-beta, and IL-10 by ELISA from the supernatant of the MLR. RESULTS: The cell proliferation decreased according to specific H-2 complexes. There were increased CD4+CD25+ cells in the thymus. For the paracrine effects, the B-C3H SP showed ratio-dependent inhibitory effects, although the C-C3H SP inhibited some cell proliferation. There was no difference in the IFN-gamma, TNF-alpha and TGF-beta between the control and experimental groups. However, IL-10 was higher in the 1:10 ratio mixture in the control and transfused SP compared to the other groups. CONCLUSION: The results of this study suggested that the cellular components in transfusions might contribute to the immune regulatory effects by CD4+CD25+ cells after 24 hours.
Animals ; Blood Transfusion ; Bone Marrow ; Bromodeoxyuridine ; Cell Proliferation ; Cytokines ; Enzyme-Linked Immunosorbent Assay ; Humans ; Immunomodulation* ; Interleukin-10 ; Interleukin-2 ; Lymphocytes ; Mice ; Mortality ; Recurrence ; Spleen ; Thymus Gland ; Transforming Growth Factor beta ; Tumor Necrosis Factor-alpha

PURPOSE: Valproic acid (VPA) has been used as an anticonvulsant for a long time. Recently, there are many reports on VPA activity with regards to intracellular signal transduction, including differentiation, proliferation, and apoptosis. We experienced several hematologic toxicities during the long-term use of VPA. Therefore, we investigated whether VPA has effects on short-term or long-term hematopoiesis with respect to differing concentrations. METHODS: We obtained bone marrow mononuclear cells (BMMNC) from a 5 week old female C3H/He strain mouse. The BMMNC were cultured in semi-solid media mixed with VPA according to the concentrations of colony forming unit for granulocyte-monocytes (CFU-GM). The concentrations of VPA were used as follows: 0.01 mM, 0.1 mM, 1 mM, and 10 mM (therapeutic level: 0.07~1.1 mM). We performed long-term liquid culture under VPA to compare the frequency of long-term culture initiating cells (LTC-IC) according to various VPA levels. RESULTS: The number of CFU-GM was highest with 1 mM of VPA (45.2+/-13.5), with higher therapeutic level than control (25.7+/-11.9), in 0.01 mM of VPA (26.5+/-12.1) and in 0.1 mM of VPA (26.6+/-12.2). In 10 mM of VPA, a toxic level of VPA, was the lowest at 1.6+/-1.1 (P< 0.01). In long-term culture, the frequency of LTC-IC was increased in 0.1 mM of VPA (67.7+/-16.3%), lower therapeutic level than in control (5.5+/-10.6%). In 1 mM of VPA, the high therapeutic level decreased to 81.6+/-9.3%. With toxic levels of VPA, 10 mM, there was no hematopoiesis. CONCLUSION: The VPA might enhance short-term hematopoiesis at high therapeutic levels, while preserving LTC-IC in long-term hematopoiesis under low therapeutic concentrations. Therefore, we suggest that VPA to be used within a low therapeutic level to escape from hematopoietic suppression when using VPA as long-term medication for seizure control.
Animals ; Apoptosis ; Bone Marrow ; Female ; Granulocyte-Macrophage Progenitor Cells ; Hematopoiesis ; Humans ; Mice* ; Seizures ; Signal Transduction ; Stem Cells ; United Nations ; Valproic Acid*

BACKGROUND: The iron chelating agents (ICA) have various biological effects besides iron chelation. We investigated the immunomodulatory effects of Deferasirox (DFS) compared to Deferoxamine (DFO). METHODS: Spleen cells (SP) were obtained from 5 week-old C57/BL6 (H-2(b)). The cytotoxicity of ICAs was examined using the CCK8 method. For the cell proliferation assay, SP were cultured with irradiated in addition to 10, 50, 100micrometer of DFS or DFO and 200ng/mL of cyclosporin A (CSA). Cytokines and nitrite levels were evaluated from supernatants by ELISA. RESULTS: The viability of ICA was reported to be over 100%. Both DFS and DFO inhibited cell proliferation in a manner comparable to CSA. Cell proliferation without iron was reduced at the concentration of 100micrometer of DFO. With iron treatment, the reduction of the stimulation index was dependent on DFO concentrations. DFS decreased the proliferation without reference to the concentrations. After stimulation of phytohemagglutinin, the nitrite concentrations increased with iron. With lipopolysaccharides, the nitrite levels were higher in DFO with iron than control, but similar in DFS regardless of iron treatment. The levels of interleukin-2 were not different. Interleukin-10 was more abundantly produced in 50micrometer of DFO compared to DFS. Transforming growth factor-beta was higher in DFS than DFO at the low concentration, but opposite at the high concentration. CONCLUSION: These data suggested that both iron chelating agents possessed immune suppressive effects comparable to CSA. The immunosuppressive effect of DFS may be distinct from DFO. More experiments are required to determine the exact mechanism of the immunosuppressive effect of DFS.
Benzoates ; Cell Proliferation ; Cyclosporine ; Cytokines ; Deferoxamine ; Interleukin-10 ; Interleukin-2 ; Iron ; Iron Chelating Agents ; Lipopolysaccharides ; Spleen ; Triazoles

BACKGROUND: Rotor off (R/O) fraction obtained by counterflow centrifugal elutriation (CCE) contains small number of T cells and many hematopoietic stem cells. Since megakaryocytes and its progenitors are larger than other cells in bone marrow, it may be easier to be separated by CCE and newly applied to hematopoietic stem cell transplantation (HSCT) for early megakaryocytes reconstitution. METHODS: The marrow cells of BALB/c mice in each group (17, 25, 28mL/min, and R/O fraction) were cultured for quantifying CFU-MK and measured after 10 days. BALB/c mice were lethally irradiated and transplanted with R/O cells. The dosages of transplanted cells were 5x104 in Group A, 5x105 in Group B, and 5x106 in Group C. The platelet counts in peripheral blood were measured up to post-transplant day 14. RESULTS: After CCE, recovery rate of the loaded cells was 82.2% and the R/O fraction was 35.9%. Most CFU-MK were formed in R/O fraction, and Group C showed the fastest recovery. Group A couldn't reach the level of 100x109/L until post-transplant day 14, and Group B showed slower recovery compared to Group C. All 5 mice survived in Group C, but 2 out of 5 mice survived in Group A and B. CONCLUSIONS: R/O fraction contains higher number of megakaryocyte progenitors, and CCE could be an effective method for separating megakaryocyte progenitors essential for reconstituting platelet after HSCT. The cell dose of 5x106 was required for the effective recovery of platelet and the survival of BALB/c mice in syngeneic bone marrow transplantation with R/O cells.
Animals ; Blood Platelets* ; Bone Marrow ; Bone Marrow Transplantation ; Hematopoietic Stem Cell Transplantation ; Hematopoietic Stem Cells ; Megakaryocyte Progenitor Cells ; Megakaryocytes ; Mice* ; Platelet Count ; T-Lymphocytes ; Transplantation, Isogeneic*

Gastric bezoar has been known to occur occasionally in the gastrointestinal tract, as a result of foreign material accumulating in the stomach. Most case have been managed by surgical methods. Currently, the endoscopic mathod is after used for the gastrointestinal disease, and therefore we treated two cases of huge bezoars using the endoscopic polypectomy snare and lithotriptor. Subsequently, we are reporting these cases and have incorporated relevant literature which was reviewed for our report for the subject case.
Bezoars* ; Gastrointestinal Diseases ; Gastrointestinal Tract ; SNARE Proteins* ; Stomach

BACKGROUND: Intravenous injection of mesenchymal and hematopoietic stem cells (MSCs, HSCs) has the disadvantages of low delivery rate to bone marrow and sequestration of cells in the lung and liver. This study was designed to determine whether there is a relationship between the administration route and dosage of stem cells and GVHD and survival. METHODS: MSCs were retrieved from five subcultured C3H/10T1/2, cell lines from C3H/He mice. HSCs were transplanted by injecting 1 x 10(7) of bone marrow mononuclear cells and 5 x 10(6) of spleen cells from six to eight week old female C3H/He mice into six week old irradiated female BALB/c mice. The groups were divided into intravenous injection (IV) and intra-marrow (IM) injection groups. IV and IM+MSC groups consisted of mice transplanted with the same bone marrow mononuclear cells and SP, IV and IM groups, with the additional co-injection of 1 x 10(6) MSCs. RESULTS: Evaluation of all mice, in both groups, showed no difference in GVHD and survival. However, high dose injection with 1 x 10(6) MSCs led to a decreased incidence of GVHD (P<0.05) and improved survival (P<0.01) in both groups. CONCLUSION: The results of this study showed that the positive effects of MSC on GVHD and survival were primarily dependent on the number of injected cells.
Animals ; Bone Marrow ; Cell Line ; Female ; Graft vs Host Disease* ; Hematopoietic Stem Cell Transplantation* ; Hematopoietic Stem Cells* ; Humans ; Incidence ; Injections, Intravenous ; Liver ; Lung ; Mesenchymal Stromal Cells* ; Mice ; Spleen ; Stem Cells ; Transplants*

BACKGROUND: In kidney transplantation, donor specific transfusion may induce tolerance as a result of some immune regulatory cells against the graft. In organ transplantation, the immune state arises from a relationship between the immunocompromised graft and the immunocompetent host. However, a reverse immunological situation exists between the graft and the host in hematopoietic stem cell transplantation (HSCT). In addition, early IL-2 injections after an allogeneic murine HSCT have been shown to prevent lethal graft versus host disease (GVHD) due to CD4+ cells. We investigated the induction of the regulatory CD4+CD25+ cells after a transfusion of irradiated recipient cells with IL-2 into a donor. METHODS: The splenocytes (SP) were obtained from 6 week-old BALB/c mice (H-2(d)) and irradiated as a single cell suspension. The donor mice (C3H/ He, H-2(k)) received 5x10(6) irradiated SP, and 5,000 IU IL-2 injected intraperitoneally on the day prior to HSCT. The CD4+CD25+ cell populations in SP treated C3H/He were analyzed. In order to determine the in vivo effect of CD4+CD25+ cells, the lethally irradiated BALB/c were transplanted with 1x10(7) donor BM and5x10(6) CD4+CD25+ cells. The other recipient mice received either 1x10(7) donor BM with 5x10(6) CD4+ CD25- cells or the untreated SP. The survival and GVHD was assessed daily by a clinical scoring system. RESULTS: In the MLR assay, BALB/c SP was used as a stimulator with C3H/He SP, as a responder, with or without treatment. The inhibition of proliferation was 30.0 13% compared to the control. In addition, the MLR with either the CD4+CD25+ or CD4+CD25- cells, which were isolated by MidiMacs, from the C3H/He SP treated with the recipient SP and IL-2 was evaluated. The donor SP treated with the recipient cells and IL-2 contained more CD4+CD25+ cells (5.4+/-1.5%) than the untreated mice SP (1.4+/-0.3%)(P<0.01). There was a profound inhibition in the CD4+CD25+ cells (61.1+/-6.1%), but a marked proliferation in the CD4+CD25- cells (129.8+/-65.2%). Mice in the CD4+CD25+ group showed low GVHD scores and a slow progression from the post-HSCT day 4 to day 9, but those in the control and CD4+CD25- groups had a high score and rapid progression (P<0.001). The probability of survival was 83.3% in the CD4+CD25+ group until post-HSC day 35 and all mice in the control and CD4+CD25- groups died on post-HSCT day 8 or 9 (P=0.0105). CONCLUSION: Donor graft engineering with irradiated recipient SP and IL-2 (recipient specific transfusion) can induce abundant regulatory CD4+CD25+ cells to prevent GVHD.
Animals ; Graft vs Host Disease* ; Hematopoietic Stem Cell Transplantation* ; Hematopoietic Stem Cells* ; Humans ; Interleukin-2* ; Kidney Transplantation ; Mice ; Organ Transplantation ; T-Lymphocytes* ; Tissue Donors ; Transplants*