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)

The binding of [3H]cyclosporine A (CsA) to BALB/c mouse spleen cells was examined with a novel centrifugation assay which rapidly removes free [3H]CsA from cell surfaces with a minimal loss of low affinity specifically bound [3H]CsA. A single class of specific and saturable CsA binding sites was found under equilibrium binding conditions. Scatchard analysis of the data resulted in a straight line with KD and Bmax values of 95 nM and 2.4 million binding sites/cell, respectively. Kinetic studies conducted with a wider range of [3H]CsA concentrations revealed two distinct binding sites, with KD's of 290 nM and 9.6 microM, respectively. [3H]CsA bound only nonspecifically to phosphatidylcholine:cholesterol liposomes. Specific [3H]CsA binding sites were found in murine WEHI-5 B-lymphoma cells, murine N1E-115 neuroblastoma cells and human A204 rhabdosarcoma cells. We conclude from these results that there are at least two nonlipid CsA binding sites in BALB/c mouse spleen cells and that CsA binding sites are present in both lymphoid and nonlymphoid tissue.
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PMID:A novel cyclosporine binding assay. 348 40

All cases diagnosed in Finland as non-Hodgkin's lymphoma (NHL), Hodgkin's disease or histiocytosis X in children younger than 15 years in 1953 to 1973, according to the Finnish Cancer Registry, were reexamined histologically. Only 55% of the cases originally diagnosed as NHL were regarded as such at reexamination. The others were mainly malignant nonlymphatic tumors such as neuroblastoma and different kinds of sarcomas. Seventy-two NHLs were diagnosed in 50 boys and 22 girls. The corrected age-specific incidence rate was 0.32/10(5). The most common histologic types were Burkitt's lymphoma (BL) (30 cases), lymphoblastic lymphoma (LBL) (26), large cell lymphomas (LCL) (six), and non-Burkitt's lymphoma (n-BL) (three). There were marked differences between BL and LBL in the course of the disease: BL was extranodal in 83%, LBL only in 4% (mediastinum was regarded as nodal); BL showed initial abdominal or pelvic involvement in 60% whereas LBL showed none; BL had initial mediastinal involvement in 7%, and LBL had it in 62%; all patients with LBL died whereas 23% of those with BL survived. Other types of NHL resembled BL in their course of disease. Patients with initial tonsillary involvement appeared to have the best prognosis and patients with mediastinal involvement the poorest. The importance of accurate histologic classification is emphasized. It appears to be most important to differentiate LBL from other types of NHL.
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PMID:Non-Hodgkin's lymphomas in childhood. A clinicopathologic and epidemiologic study in Finland. 349 36

The great vessels' interspace, in the pediatric retroperitoneum, deserves special attention during abdominal sonographic examination. By rotating the child into the right anterior oblique position, the full length of this interspace is demonstrated. Normally, it has a uniform appearance. When invaded by disease, the sonographic pathology can be identified and differentiated from the surrounding structures. The studies were gathered from experience with 1658 retroperitoneal sonographic examinations. Fourteen children were found with disease involving the great vessels' interspace: 4 patients with lymphoma, 3 patients with sympathetic ganglioneuroblastoma, 2 patients with retroperitoneal rhabdomyosarcoma, and one case each, respectively, of adrenal neuroblastoma, Wilms' tumour, clear cell carcinoma of the kidney, retroperitoneal teratoma and Toxocara canis.
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PMID:The sonographic evaluation of the great vessels' interspace in the pediatric retroperitoneum. 352 17

A panel of monoclonal antibodies including antibodies against neuroectodermal antigens, (UJ13A, UJ127.11, UJ181.4), leukocytes (2D1), intermediate filament antigens cytokeratin (LE61), Vimentin, Desmin (Labsystems, Helsinki, Finland), myoglobin, and neurofilament (155) antigens were assessed for their use as an adjunct to light microscopy in pediatric pathology, with particular emphasis on the "small round cell" tumors. One hundred thirty-four tumors were studied using immunofluorescence and immunoperoxidase techniques. The differentiation of neuroblastoma from lymphoma proved to have a clear-cut immunologic profile, as did the rhabdomyosarcomas, which showed consistent positivity with neuroectodermal antibody UJ13A and in positive binding with antidesmin. Ewing's sarcoma did not give a clear immunohistologic pattern with these antibodies. This panel was shown to have been a valuable aid to diagnosis in 12% of cases studied. The future use of such a panel for routine diagnostic use is discussed, but it is emphasized that the binding pattern of these tumors is often heterogeneous, and examination in conjunction with conventional histology is essential if the correct conclusions are to be made.
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PMID:The use of a panel of monoclonal antibodies in pediatric oncology. 354 86

Teniposide (VM-26) is an effective anticancer drug usually administered as a short infusion in doses of 150 to 165 mg/m2. The objectives of the trial reported here were to evaluate clinical responses and assess pharmacokinetic parameters as a determinant of outcome when VM-26 was administered as a 72-hour continuous infusion (CI) with doses escalated from 300 to 750 mg/m2 per course. Twenty-eight patients with recurrent leukemia, lymphoma, or neuroblastoma received 53 courses of CI VM-26 and 16 had measurable responses. There were two partial remissions and one stable disease among seven neuroblastoma patients and 13 of 21 leukemia/lymphoma patients had oncolytic responses (greater than or equal to 75% decrease in circulating blasts). Toxicity included moderate to severe mucositis and myelosuppression. Pharmacokinetic parameters determined during 35 courses administered to 23 patients were highly variable. Clearance (CI) ranged between 3.7 and 43.8 ml/min/m2, resulting in VM-26 plasma concentrations from 2.8 to 30.6 mg/L across all dose levels. The interpatient pharmacokinetic variability reflected in CI and VM-26 steady state concentrations (Css), obscured any dose-response relationship. However, when pharmacokinetic parameters for responding and nonresponding patients were compared, statistically significant relationships were observed. For responders, the mean Css was 15.2 mg/L and mean CI was 12.1 mL/min/m2; for nonresponders, mean Css was 6.2 mg/L (P less than .01) and mean CI was 21.3 mL/min/m2 (P less than .05). Thus, patients with higher CI and lower Css were less likely to respond. Clinical responses occurred in ten of ten patients with Css greater than 12 mg/L, and only five of 13 patients with Css less than 12 mg/L (P less than .01). In this study, interpatient pharmacokinetic variability yielded a four- to sixfold difference in intensity of systemic exposure (Css) within the same dose level, which was an important determinant of clinical response. These data indicate that achieving a VM-26 target concentration for individual patients could ensure an increased intensity of systemic exposure in patients with a high CI and improve the likelihood of effective therapy.
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PMID:Clinical pharmacodynamics of continuous infusion teniposide: systemic exposure as a determinant of response in a phase I trial. 359 7

Statistical procedures were used to estimate lectin receptor distribution on the surface of ascite lymphoma cells, neuroblastoma C-1300 cells and of transformed human T- and B-derived lymphoid cell lines. Relationships between the arithmetic means and mean square variances for sample populations from each cell and ferritin- or colloidal gold-lectin combination were used to define four types of topographical distributions: uniform-ordered, uniform-random, random and clustered.
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PMID:[Evaluation of the distribution of lectin receptors on the surface of tumor and transformed cells using methods of variation statistics]. 360 7

Cellular and humoral markers of malignancy play several roles at many levels in the evaluation and staging of children with cancer. Cytogenetic analysis of constitutional cells can be used to determine the genetic risk of developing certain cancers, such as retinoblastoma and Wilms' tumor in high-risk families. Urinary metabolites of neuroblastoma have been studied not only for accurate diagnostic ability in children with "small round cell" tumors, but as a screen for the presence of the tumor in large normal populations. Markers are valuable as prognostic factors at the time of cancer diagnosis; for example, the use of cell surface antigens and cytogenetics in leukemia phenotyping, leading to alterations in initial therapy. Once found at diagnosis, both specific and nonspecific markers can then be utilized to follow the regression and recurrence of a malignancy, such as serum ferritin in neuroblastoma or lactate dehydrogenase in non-Hodgkin's lymphoma. Presence of cell surface antigens to which monoclonal antibodies can be directed are becoming increasingly helpful in both tumor localization, such as in radioisotope scanning, and in therapeutic intervention, such as in purging autologous bone marrow of malignant cells prior to use as a rescue after massive cytoreduction. Finally, cellular markers have lead to a better understanding of the basic biology of particular neoplasms; for example, gene rearrangements in lymphoma, which will ultimately lead to better diagnostic and therapeutic ability.
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PMID:The use and significance of biologic markers in the evaluation and staging of a child with cancer. 371 38

A 2-year-old girl being prepared for a bone marrow transplant for treatment of stage IV neuroblastoma suffered a fatal graft v host (GVH) reaction following a transfusion on nonirradiated packed RBC. GVH disease is a recognized complication of transfusion in patients with leukemia and lymphoma and is a frequent complication following bone marrow transplantation. GVH disease has also been reported in pediatric patients with solid tumors who have received sufficient chemotherapy to render them immunocompromised. GVH disease can be prevented by irradiating all blood products with at least 1,500 rad for patients having total lymphocyte counts less than 1,000/microL or for those who have received greater than 30 mg/kg/d of Cytoxan.
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PMID:Fatal graft v host disease in a child with neuroblastoma following a blood transfusion. 379 74

Current therapy for children with cancer includes a variety of invasive procedures many of which require repeated venous access over a considerable period of time. Such procedures are poorly tolerated by children and by their veins. Recently it has become possible to undertake the majority of such procedures by means of permanent indwelling silastic catheters improving the quality of life of the children and their parents and increasing the scope of therapeutic intervention. In the period July '83 - August '84 we have used 46 of these catheters in 45 children with malignant disease, 12 with acute myeloid leukaemia, 12 with neuroblastoma, 7 with B cell leukaemia-lymphoma, 6 with rhabdomyosarcomas, 2 with Ewing's Sarcoma, 2 with Wilms' tumor and 1 case each of Hodgkin's disease, teratocarcinoma, osteosarcoma and juvenile chronic myeloid leukaemia. The children's ages ranged from 2 months to 14 years; 22 were male and 23 female. The catheters were inserted under general anaesthesia (duration 20-40 minutes) usually without difficulty, except for a single patient in whom no suitable vein could be found. No complications connected with the placement of the catheter were observed. Subsequent management of the catheter was initially complicated and time-consuming, but was subsequently simplified so that acceptance by parents, children and nursing staff was eventually excellent. The duration of use of 46 catheters ranges from 7 to 350+ days; 24 catheters are presently in use at 30-350+ days from insertion. Eight children died as a result of disease progression and two of sepsis with the catheter in place.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Advantages of a permanent venous access in children treated for cancer. Preliminary results]. 383 38

We reviewed the Tumor Registry for 1981 at the Children's Hospital of Philadelphia to identify all the children with newly diagnosed cancer who were seen initially in the emergency department (ED). Of the 220 new patients listed, 16 (7.3%) sought initial care in the ED (1 per 4,500 ED visits). Seven had leukemia, five had non-CNS solid tumors (2 lymphoreticular, 1 Wilms', 1 neuroblastoma, and 1 ovarian), and four had CNS tumors. Among the children with leukemia, pallor (6) and decreased activity (4) were the most common complaints. Duration of symptoms ranged from 4 days to 3 weeks. Physical examination showed pallor (5), splenomegaly (4), fever (3), hepatomegaly (3), lymphadenopathy (3), and ecchymoses or petechiae (2). The complete blood count and peripheral smears were all abnormal. The five patients with non-CNS solid tumors had symptoms related to the location of their neoplasms. The patients with Wilms' tumor, neuroblastoma, and ovarian dysgerminoma had abdominal masses; the patient with lymphoma had a large, painful inguinal node; and the patient with histiocytosis X had an infiltrative rash, gingivitis, and pneumonitis. Of the four children with CNS tumors, three had headache, and one had an incidentally detected scotoma following head trauma. All four eventually had abnormal neurologic exams and computer tomographic scans, but two were discharged initially with psychiatric diagnoses. We conclude that cancer, although rare in children, occurs with greater relative frequency in the referral hospital ED than that predicted by published cancer rates from the referring hospital's ED.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Detection of cancer in the pediatric emergency department. 384 22


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