1,721,021 research outputs found

    Ifosfamide in pediatric solid tumors.

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    Phase II studies conducted in Europe and the USA on pediatric solid tumors have shown that ifosfamide, as a single agent, is an active drug against a variety of neoplasms - rhabdomyosarcoma (RMS), some non-RMS soft tissue sarcomas, Wilms' tumor, bone sarcomas and neuroblastoma. Furthermore, an increase in tumor response rate has been observed when ifosfamide has been used in combination with other drugs. The usual dose of ifosfamide varies from 1.8 to 3 g/m(2)/day for 2-5 days according to the different regimens. Some controversies still exist on the modality of drug administration and more precisely on the time of infusion, however in pediatric practice, short infusion (e.g. 3 h) is usually preferred because of the reduced neurotoxicity in comparison to lengthier administration (e.g. 24 h). Ifosfamide is currently included in the standard therapy of pediatric bone and soft tissue sarcomas. It is also used in a selected high-risk group of patients with Wilms' tumor, neuroblastoma and germ cell tumors. Copyright 2003 S. Karger AG, Base

    Dapsone treatment in a girl with severe chronic thrombocytopenic pupura does it work? Don’t touch it!

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    Dapsone has been shown to be effective in treating immune thrombocytopenic purpura (ITP) in adults. In children the experience is limitated. We describe our experience using dapsone in a female with refractory, symptomatic ITP who suffered intracranic haemorrhage and massive gastric bleeding. After treatment platelet counts was more than 100 x 103/μL, and reached 1000x103/μL. Discontinuation resulted in a rapid decrease in platelet counts, with severe intracranial haemorrhage (ICH). The recovery of dapsone led the platelets count to lower values. We suggest that treatment should not be discontinued in responders, at least in children with symptomatic ITP. Additional studies of dapsone in children are warranted

    Prevalence and significance of BRAF alterations in a pediatric population with low-grade gliomas

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    Background Low-grade gliomas (LGG) are the most common brain tumours in children, represented by heterogeneous pathological entities. BRAF gene alterations have been recently identified as responsible of constitutive activation of the mitogen-activated protein kinase pathway (MAPK) and hence involved in the development of LGG in children. There is evidence that the BRAF V600E mutation is more common in supratentorial LGG while the KIAA1549:BRAF fusion in posterior fossa pilocytic astrocytoma (PA). Objective This case series describes the prevalence of BRAF alterations in LGG patients, trying to relate them to outcome. Methods Children aged 0–14 years, with a diagnosis of LGG, referred to a single neuro-oncologic centre, were retrospectively reviewed to analyze clinical and histopathological features related to BRAF alterations. Results A total of 35 patients were included (16 males, median age 85.5 ± 81.3 months). BRAF mutations were searched on 7/35 children (20 %) resulting positive in 5/7 (71 %). Two of them (40 %) showed the KIAA1549:BRAF fusion. They were both pilocytic astrocytomas located in posterior fossa: 1/2 (50 %) was totally resected, showing stable disease (SD) 1 year after surgey; the other was not surgically treatable and showed a progressive disease during chemotherapy. Three out of five (60 %) presented V600E mutation. All of them were supratentorial: 1 pilomyxoid astrocytoma, treated with two partial resections and subse- quent chemotherapy, was in SD 5 years after diagnosis; 1 diffuse pleomorphic xanthoastrocytoma underwent three partial resections and showed SD after 5 years of vemurafenib; 1 glioneuronal tumor was in SD after complete resection and radiotherapy. Conclusion Our data support the evidence that specific mutations of the BRAF pathway are related to site and histological subtype of brain tumors. This sample is too small to help supporting the hypothesis that these alterations have a prognostic impact. Extent of surgery and localization seem to be the most important prognostic factors

    Going Beyond Counting First Authors in Author Co-citation Analysis

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    The present study examines one of the fundamental aspects of author co-citation analysis (ACA) - the way co-citation counts are defined. Co-citation counting provides the data on which all subsequent statistical analyses and mappings are based, and we compare ACA results based on two different types of co-citation counting - the traditional type that only counts the first one among a cited work's authors on the one hand and a non-traditional type that takes into account the first 5 authors of a cited work on the other hand. Results indicate that the picture produced through this non-traditional author co-citation counting contains more coherent author groups and is therefore considerably clearer. However, this picture represents fewer specialties in the research field being studied than that produced through the traditional first-author co-citation counting when the same number of top-ranked authors is selected and analyzed. Reasons for these effects are discussed
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