ISSN:0192-8562    

出版商:OVID    

年代:1993

数据来源: OVID

摘要:

Purpose:Due to its important role in immunoregulation, we have investigated serum levels of tumor necrosis factor-α (TNFα), in children with newly, diagnosed, untreated, malignant disease.Patients and Methods:These levels have been related to the presence of infection and to the serum content of three other cytokines, namely interleukin-1β, interleukin-2, and interferon-γ. All cytokine analyses were performed using highly sensitive radioimmunoassays.Results:Children with leukemia had higher mean levels of TNFα (63.6 ± 12.3 pg/ml) than did children with solid tumors (21.5 ± 4.2 pg/ml) and control patients (10.5 ± 2.6 pg/ml). TNFα levels in patients did not correlate with the levels of the other cytokines or with the presence of infection.Conclusions:Children with malignant disease often have elevated TNFα levels. This elevation is dependent on the malignant disease process itself, and could either reflect the host immunological response or tumor cell production of TNFα.

ISSN:0192-8562    

出版商:OVID    

年代:1993

数据来源: OVID

摘要:

Purpose:We report here our experience in using intravenous methohexital (MHX), an ultrashort-acting barbiturate, for brief unconscious sedation of pediatric oncology outpatients undergoing painful, invasive procedures.Methods:Following published monitoring guidelines for deep pediatric sedation, 1.0 mg/kg MHX was administered immediately before the procedure, 1% xylocaine was given locally, and MHX was additionally titrated to maintain minimal response to pain during the procedure. Clinical data reported here were gathered retrospectively from permanent medical records.Results:Data reported here represent 132 evaluable consecutive procedures in 33 patients ranging in age from 1.6 to 20.5 years. Patients underwent an average of 4 ± 3 procedures and received a mean total MHX dose per procedure of 5.8 ± 2.1 mg/kg. The mean length of time from start of sedation to full arousability was 30 ± 12 min. Twenty-three (17.4%) procedures were associated with clinically insignificant decreases in diastolic blood pressure or heart rate below resting normal ranges for age. Eight (6.1%) procedures in six patients were associated with minor complications requiring no intervention, such as transient behavioral changes, transient myoclonus, and minimal stridor. Five procedures (3.8%) in five patients required simple suctioning to manage secretions. Only two procedures (1.5%) in two patients required brief bag-mask ventilation plus suctioning for suspected laryngospasm. None required intubation. No differences in clinical features or MHX doses were noted for patients with, as compared to those without, complications. All procedures were completed with a satisfactory level of sedation.Conclusions:Our experience indicates that MHX, with appropriate monitoring as described here, is a safe and effective agent for use in pediatric oncology outpatient sedation programs.

ISSN:0192-8562    

出版商:OVID    

年代:1993

数据来源: OVID

摘要:

Purpose:Five patients with Fanconi anemia have been treated by bone marrow transplantation.Patients and Methods:They were conditioned with cyclophosphamide (CY) (20–150 mg/kg), antilymphocyte globulin, and thoracoabdominal irradiation (4–6 Gy). The dose of CY for preconditioning was adjusted individually, based on the in vitro effect of CY metabolites on the chromosomes of patients with Fanconi anemia. Four patients received marrow from human leukocyte antigen (HLA)-identical siblings, and one received marrow from his HLA phenotypically identical father.Results:All patients achieved engraftment, and acute graft-versus-host disease (GVHD) grade II or more was not observed. Three developed chronic GVHD. All patients are surviving 2–5 years after grafting, with hematological improvement.Conclusions:These results indicate that the individual dose adjustment of CY used for preconditioning may prevent graft failure and severe acute GVHD.

ISSN:0192-8562    

出版商:OVID    

年代:1993

数据来源: OVID

摘要:

Giant hemangiomas occurring in the neonatal period often present a therapeutic challenge, especially when confounded by consumptive coagulopathy (Kasabach-Merritt syndrome). We treated three infants with tranexamic acid after therapy with corticosteroids was ineffective. One patient had a partial response. The remaining two developed progressive disease.

ISSN:0192-8562    

出版商:OVID    

年代:1993

数据来源: OVID

摘要:

Purpose:T cell-mediated red cell aplasia in a 4 1/2-year-old child with transient erythroblastopenia of childhood (TEC) is described.Patients and Methods:Erythropoiesis was studied by assessing the colony growth of marrow erythroid progenitors at the time of diagnosis and during recovery.Results:The colony-forming unit-erythroid (CFU-E) growth of whole marrow at diagnosis was only 28% that of the control. T-cell depletion of the patient's marrow was followed by a more than fivefold increase in CFU-E growth, as compared with 20% inhibition of CFU-E and 40% inhibition of burst-forming unit-erythroid (BFU-E) growth in control marrow. The number of colony-forming unit-granulocyte-macrophage (CFU-GM) in both control and patient's marrow was not significantly altered by all of these manipulations. During early and late recovery, CFU-E and BFU-E growth improved substantially, and the effect of T-cell depletion diminished. Increased numbers of peripheral T-suppressor lymphocytes, as well as activation of natural killer (NK) cells and high levels of interferon, all consistent with viral infection, were found at presentation. Clinical recovery was associated with normalization of T-suppressor lymphocyte number.Conclusions:The results suggest that in this child with TEC, a preceding viral infection may have caused activation of suppressor T-cells and interferon secretion leading to cell-mediated suppression of erythropoiesis.

ISSN:0192-8562    

出版商:OVID    

年代:1993

数据来源: OVID

摘要:

Purpose:A unique myelodysplastic syndrome referred to as transient abnormal myelopoiesis (TAM) has been reported to occur primarily in infants with Down's syndrome (DS) or other abnormalities of chromosome 21. This disorder raises basic questions regarding the pathogenesis of leukemia, yet its natural history is poorly documented and derives from small series and isolated case reports.Patients and Methods:To better characterize TAM, we accumulated data on 35 cases identified through a questionnaire mailed to pediatric oncologists in the United States. These cases, pooled with two that we recently encountered, and 58 comparable cases reported in the literature comprise a series of 95 cases of TAM in DS.Results:The patients in this series were notable for the high morbidity and mortality of this reportedly benign condition. Eleven percent of the patients died during the initial event, and the overall mortality for the entire series was 27%. Twenty-eight of the 85 patients (33%) who survived the initial event developed a subsequent hematologic disorder, most often acute nonlymphocytic leukemia, at a median age of 16 months.Conclusions:No initial clinical or hematologic features predicted the development of a subsequent hematologic disorder. However, those patients initially mosaic for the presence of trisomy 21 did not develop subsequent abnormalities. This series reviews questions regarding leukemogenesis in DS and underscores the importance of conducting future prospective studies of this unique hematologic disorder.

ISSN:0192-8562    

出版商:OVID    

年代:1993

数据来源: OVID

摘要:

Purpose:A prospective study of coagulation in 15 children who received L-asparaginase, vincristine, or prednisone plus or minus an anthracycline as part of a treatment program for leukemia or leukemia-lymphoma syndrome was conducted.Patients and Methods:One patient developed a central nervous system thrombosis.Results:The inhibitors of coagulation, including antithrombin-III, protein C, protein S, and plasminogen, were decreased in many individuals, but were not significantly different in the patient who had experienced the thrombotic event. Platelet aggregations to low molar ADP were performed in four patients, and in three patients showed a hyperaggregable pattern.Conclusions:The patient with thrombosis developed a transient acquired type II pattern on multimeric analysis of the von Willebrand factor, which was not seen in the other individuals studied.

ISSN:0192-8562    

出版商:OVID    

年代:1993

数据来源: OVID

摘要:

Purpose:Six children who had mature or immature sacrococcygeal teratomas diagnosed in the newborn period have since been registered on a Pediatric Oncology Group/Children's Cancer Group germ cell study with recurrent malignant neoplasia [pure yolk sac tumor (YST) or teratoma with yolk sac elements].Results:Four of the children have responded to therapy, one has died, and one has been lost to follow-up. Review of the slides from five of the original tumors identified microscopic foci of YST in four.Conclusions:Detection of such foci in neonatal tumors is important because serum α-fetoprotein concentrations may not be helpful since they may normally be high in the newborn period due to fetal production.

ISSN:0192-8562    

出版商:OVID    

年代:1993

数据来源: OVID

摘要:

Purpose:13-cis-Retinoic acid (cis-RA) has efficacy in the treatment and prevention of certain malignancies. In vitro effects against neuroblastoma include induction of differentiation, inhibition of proliferation, and decreased N-mycexpression. We hypothesized that cis-RA may be effective against minimal residual disease in neuroblastoma patients. A phase I trial to determine the maximal tolerated dosage and toxicity of cis-RA in pediatric patients with neuroblastoma after bone marrow transplantation was initiated.Patients and Methods:Forty-nine pediatric patients (status post-bone marrow transplant for neuroblastoma) were treated for 14 days with oral cis-RA in escalating doses from 100 to 200 mg/m2/day followed by a 14-day rest period for up to 12 months.Results:In three of 39 patients (7.7%) evaluable for calcium levels, hypercalcemia (12.6–18.7 mg/dl) was the dose-limiting toxicity. Grade 1–3 hypercalcemia occurred in nine of 39 patients (23%). The overall incidence of hypercalcemia was 31% (12 of 39). Only one patient was symptomatic due to the hypercalcemia, with arthralgias and myalgias. The hypercalcemia resolved with temporary discontinuation of the drug and a 25% dose reduction for subsequent courses.Conclusions:Hypercalcemia is a novel dose-limiting toxicity for cis-RA. Patients receiving high doses of cis-RA should have monitoring of serum calcium levels.

ISSN:0192-8562    

出版商:OVID    

年代:1993

数据来源: OVID