Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027819 (neuroblastoma)
 27,800 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

19 children between 3 and 23 years underwent 79 leukapheres for collection of blood stem cells. In children suffering from acute lymphoblastic leukemia (ALL), Non Hodgkin's Lymphoma (NHL) and Ewing's Sarcoma (ES) we collected 6.87 x 10(4) CFU-GM/kg (range 2,65-21.7), if collections were started with the first platelet rise. In children with peripheral primitive neuroectodermal tumors (PNET) and neuroblastoma (NBL) we gained only 1.20 x 10(4) CFU-GM/kg (range 0.09-2.24). 17 children received high dose chemoradiotherapy and peripheral stem cell +/- bone marrow rescue. 9 suffered from solid tumors, 8 from hematopoietic malignancies. 9 were transfused with peripheral stem cells only, 8 received bone marrow in addition. Time to reach 0.5 x 10(9)/l granulocytes was very short-median 31 days (12-65), in 4 children receiving more than 5 x 10(4) CFU-GM/kg 12 to 13 days, only. On January 31st, 1989 6/17 children are alive in complete remission after a median observation time of 14.5 months (3-26) after autologous stem cell transfusion, one child is alive in "no remission", 7 died with relapse, 3 died because of infections (2 x aspergillosis, 1 x pseudomonas septicemia). The collection of blood derived stem cells by leukaphereses was well tolerated even in very small children and easily repeatable. With optimal timing high stem cell numbers were obtainable, resulting in a very short duration of posttransplant granulocytopenia.
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PMID:[Autologous peripheral stem cell transplantation in children]. 257 Aug 82

Bone marrow transplantation improves the chances of survival in a variety of hematological malignancies. However, infectious complications during the post-transplant phase contribute significantly to morbidity and mortality. To reduce the duration of granulocytopenia, which is approximately 20 days after BMT, in this study patients with ALL, relapsed or high-grade NHL, relapsed or refractory HD, or Neuroblastoma stage III/IV, were given rh GM-CSF to assess the effects on hematological and immunological reconstitution after conditioning therapy and BMT. The results of 9 patients are presented. After autologous BMT and subsequent rh GM-CSF therapy, a peripheral blood neutrophil count of 500/microliters was reached within 8-12 days, i.e., between 7 and 10 days earlier than would have been expected without rh GM-CSF. Furthermore, it appeared that rh GM-CSF was useful in case of insufficient bone marrow regeneration post autologous transplant. The influence of rh GM-CSF after allogeneic BMT is not yet clear. Further studies will be necessary to evaluate the potential of this promising new drug after BMT.
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PMID:Recombinant human granulocyte-macrophage colony stimulating factor (rh GM-CSF) after bone marrow transplantation. 307 46

High dose chemo-radiotherapy followed by autologous bone marrow transplantation (ABMT) is known to be an effective treatment in stage IV neuroblastoma (NB). Since October '84, 19 children with NB (12 relapsed or resistant: Group A; 7 in first CR: Group B) received ablative therapy (AT) consisting of VCR (4 mg/mg), L-PAM (140 mg/mg) and fractionated TBI (1000 Rads). Induction strategy at diagnosis or at relapse included high dose Peptichemio, 2-3 cycles of Vincristine-Cyclophosphamide--high dose Platinum and surgery. Bone marrow was harvested after 2 evaluation proved negative by cytomorphology, histology and immunofluorescence. Mononuclear cells (median 6.7 x 10(7)/kg) were cryopreserved and reinfused without purging. At the time of AT in Group A8 children were in CR, 4 had minimal diseases; in Group B 6 were in CR and one in PR. One toxicity-related death occurred on day 7 in a child in first CR; median duration of granulocytopenia 0.5 x 10(9)/l and thrombocytopenia less than 50 x 10(9)/l were 20 days (R: 9-40) and 27 days (R: 11-51) respectively. Persistent immune thrombocytopenia occurred in 4 children. Fever higher tha 38 degrees C developed in all patients: sepsis was documented in 6 patients. Extramedullary toxicity was moderate: GI tract was the most affected. Two out of 5 children who received AT having residual disease achieved CR; relapse or progression of disease occurred in all these patients. Four out of 8 children in second or subsequent CR and 4 out of 5 in first CR are alive and well at 3-12 months (median 7).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Total body irradiation, vincristine in continuous infusion and high-dose melphalan with transplant of autologous bone marrow in the treatment of neuroblastoma]. 330 21

The case histories of 72 subsequently treated patients - 44 with acute leukemia, 10 with chronic myeloid leukemia, 16 with severe aplastic anemia and 2 with neuroblastoma - were analyzed after bone marrow transplantation (BMT) with respect to pulmonary diseases. Thirty-eight patients suffered from a total of 51 pulmonary complications, which led to death in 20. Of 13 patients, 3 died of bacterial pneumonia, all of them during granulocytopenia; 2 of 6 patients died of fungal pneumonia and 2 out of 3 of a mixed bacterial-mycotic infection. Adult respiratory distress syndrome (ARDS) led to death in 2 patients. A granulocyte count under 500/microliter correlated significantly (P less than 0.002) with the fatal outcome of bacterial, fungal and ARDS pneumonia as well as with bronchitis. Viral pneumonia led to death in 8 of 9 patients; in each there was a significant correlation (P less than 0.05) with graft-versus-host disease (GvHD). Patients with repeated episodes of pulmonary illness had significantly more chronic GvHD (P less than 0.05); several of these patients displayed a reduction in helper T cells and an increase in suppressor T cells in the peripheral blood. The natural killer (NK) cells were reduced and the percentage of activated NK cell level lay between 6% and 69%. B-cells were absent or deficient. These findings explain in part the absence of specific antibody reactivity. Five of these patients also contracted GvHD-associated obstructive bronchiolitis, which did not respond to therapy. Pulmonary infiltrates of unknown origin (including idiopathic interstitial pneumonia) occurred in 8 of the patients (11.1%), with a fatal outcome in 3 patients. Significant changes (P less than 0.05) in lung function after BMT appeared in the form of reduced vital capacity (VC) increased residual volume (RV) and an increase in RV expressed as the percentage of total lung capacity. Pulmonary diseases were the most common complication and cause of death in our patients after BMT.
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PMID:Lung diseases after bone marrow transplantation. Results of a clinical, radiological, histological, immunological and lung function study. 352 53

One hundred one patients with advanced pediatric malignant solid tumors, refractory to conventional chemotherapy, were given Novantrone in a Phase II study. A dosage of 18 mg/m2 was administered as a short intravenous infusion every 3 weeks. One complete and 2 partial responses were observed among 26 patients treated for rhabdomyosarcoma; one of 22 patients with neuroblastoma developed a partial response. Nausea and vomiting were uncommon. Leukopenia and/or granulocytopenia developed in 90 of 98 evaluable entries. Two patients developed fatal congestive heart failure, which may have been related to the fact that these patients previously had received doxorubicin; 3 other patients developed evidence of changes in cardiac function, without congestive heart failure. Evidence of activity of this agent in patients who had previously received doxorubicin suggests that Novantrone should be evaluated in pediatric subjects with malignant solid tumors who have had no prior exposure to anthracyclines.
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PMID:Novantrone for childhood malignant solid tumors. A pediatric oncology group phase II study. 370 39

Mitoxantrone was administered as a single iv injection once every 3 weeks to 84 children with advanced acute leukemia and solid tumors in a phase I trial. Dose-limiting granulocytopenia occurred at dosages greater than 18 mg/m2 in children with solid tumors, while hospitalization for febrile episodes occurred in nine of 12 patients with acute leukemia receiving dosages greater than 24 mg/m2. Six children developed evidence of cardiac dysfunction, including three instances of congestive heart failure. No other significant toxicity was noted. Complete responses were seen in one child with neuroblastoma metastatic to bone, one with acute lymphoblastic leukemia, and four with acute nonlymphoblastic leukemia.
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PMID:Phase I trial of mitoxantrone in children. 385 68

The increased understanding of the neuroendocrine tumors at a cellular and molecular level has led to the development of new radiopharmaceuticals for imaging. Two of the imaging agents include 131I metaiodobenzylguanidine (131I-MIBG) and 111In-DTPA-D-Phe1-octreotide (111In-pentetreotide) each having specific localization in certain neuroendocrine tumors. The selective uptake of these radiopharmaceuticals by the tumor cells has generated interest in potential use for targeted radiotherapy for neuroendocrine tumors. 131I-MIBG has been used to treat patients with pheochromocytoma, neuroblastoma, carcinoid tumors, medullary thyroid carcinoma, and paragangliomas. The tumor responses have been variable with the most encouraging results being in patients with pheochromocytoma. The dose-limiting toxicity has been thrombocytopenia or granulocytopenia. 111In-pentetreotide has been used as therapy in only a few patients and has resulted in objective evidence of tumor responses. A therapeutic agent using a somatostatin analogue will most likely require radiolabeling with a beta- or possibly an alpha-emitting radionuclide to achieve significant and durable tumor responses.
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PMID:Therapy of neuroendocrine tumors with radiolabeled MIBG and somatostatin analogues. 757 46

The purpose of the study was to determine the maximum tolerated dose of continuous infusion of high-dose VP-16 in combination with high-dose melphalan (HDM) for conditioning before autologous bone marrow transplantation (ABMT). Thirteen children (median age 27 months) with stage IV neuroblastoma were treated with high-dose VP-16 and HDM followed by ABMT as consolidation treatment. All had previously received conventional chemotherapy with a mean number of six drugs. Surgery of the primary tumor had been performed in 12/13. We performed a dose-escalating study of VP-16 from 1800 mg/m2/72 h with 300 mg/m2/72 h dose increments according to toxicity. VP-16 was administered as a 72-h i.v. infusion. Melphalan (140 mg/m2/day) was administered once as an i.v. push. VP-16 pharmacokinetics were analyzed in 12 patients. Five children received 1800 mg/m2/72 h of VP-16, five received 2100 mg/m2/72 h and three, 2400 mg/m2/72 h. The mean duration of granulocytopenia (< 0.5 x 10(9)/1) was 24 days and thrombocytopenia (< 50 x 10(9)/1) was 36 days. No major infectious complications occurred. Gastrointestinal (GI) toxicity was the dose-limiting toxicity. Five severe manifestations of GI toxicity in three patients led us to consider 2400 mg/m2/72 h as the MTD. The mean VP-16 clearance rate was 17.3 ml/min/m2 with continuous infusion. A mean steady-state plasma concentration of 24.2 micrograms/ml (s.d. = 2) and 28.3 micrograms/ml (s.d. = 1.9) was achieved at the 1800 mg/ml and 2100 mg/m2 dose levels, respectively, GI toxicity is dose limiting when VP-16 at 2400 mg/m2/72 h, is associated with HDM. When given as a continuous i.v. infusion, at 2100 mg/m2/72 h, VP-16 associated with HDM is well tolerated before ABMT in young heavily pre-treated children.
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PMID:Phase I study of high-dose continuous intravenous infusion of VP-16 in combination with high-dose melphalan followed by autologous bone marrow transplantation in children with stage IV neuroblastoma. 872 43

Piperacillin/tazobactam (P/T) was used in the treatment of 14 patients at the age of 5 to 15 years: 5 patients with acute lymphoblastic leukemia, 3 with acute nonlymphoblastic leukemia, 4 with severe aplastic anemia, 1 with lymphoma and 1 with neuroblastoma. P/T was administered as intravenous infusions in a daily dose of 200-300/25-37.5 mg/kg body weight divided into 3 or 4 portions. All the patients were subjected to intestine selective antimicrobial decontamination. In 7 patients the afebrile condition was recorded before elimination of agranulocytosis without correction of the therapy i.e. without combination of P/T with some other antibiotics such as amphotericin B, vancomycin or aminoglycosides. In 5 patients the effect was stated after correction of the regimen by its supplementing with amphotericin B. 2 patients died. No side effects of P/T was observed. The afebrile condition was provided in 1 to 7 days. P/T is recommended for the treatment of fever of obscure etiology in patients with agranulocytosis and for effective control of infection in such patients.
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PMID:[Effectiveness of piperacillin/tazobactam in the treatment of fever of unknown etiology in patients with granulocytopenia]. 912 92

The vanilloid-like TRP-channel VRL-1 (TRPV2) is a nonselective cation channel expressed by primary sensory neurons and non-neuronal tissues [Caterina, M.J., Rosen, T.A., Tominaga, M., Brake, A.J and Julius, D. (1999) Nature 398, 436-441]. It is one of the six members of the vanilloid-like TRP-channel family which is now termed the TRPV family [Montell, G., Birnbaumer, L., Flockerzi, V., Bindels, R.J., Brutford, E.A., Caterina, M.J., Clapham, D.E., Harteneck, C., Heller, S., Julius, D., Kojima, I., Mori, Y., Penner, R., Prawitt, D., Scharenberg, A.M., Schultz, G., Shimizu, N. and Zhu, M.X. (2002) Mol. Cell 2, 229-231]. As it is a temperature-gated channel, VRL-1 appears to be functionally related to VR1. In contrast to VR1, VRL-1 is activated at a higher temperature threshold and it does not respond to capsaicin or protons. Here we describe the expression of VRL-1 in the rat dorsal root ganglion-derived cell line F-11, a hybridoma of mouse neuroblastoma (N18TG2) and rat dorsal root ganglion cells. We found by RT-PCR that F-11 cells express not only the rat VRL-1, but also its mouse orthologue in a single cell. The F-11 parental cell line N18TG2 also expressed murine VRL-1. Due to its neuronal character, the DRG-derived F-11 cell line provides an experimental system for the study of VRL-1 biochemistry. However, one has to be aware that both the mouse and the rat protein are expressed simultaneously. Furthermore we cloned VRL-1 from rat brain and analyzed its glycosylation and localization in comparison to the endogenously expressed protein in F-11 cells. In contrast to the endogenous VRL-1 the overexpressed protein is glycosylated. Similar to VR1 the glycosylation is N-linked as shown by an deglycosylation assay. Immunofluorescence analysis of the endogenous VRL-1 in F-11 cells gives only weak signals in the cytoplasm whereas the overexpressed rat VRL-1 appears mainly at the plasma membrane.
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PMID:Dual expression of mouse and rat VRL-1 in the dorsal root ganglion derived cell line F-11 and biochemical analysis of VRL-1 after heterologous expression. 1462 91


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