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BACKGROUND Constitutional DICER1 mutations were recently reported to cause familial pleuropulmonary blastoma (PPB). AIM To investigate the contribution and phenotypic spectrum of constitutional and somatic DICER1 mutations to cancer. METHODS AND RESULTS The authors sequenced DICER1 in constitutional DNA from 823 unrelated patients with a variety of(More)
PURPOSE Dactinomycin (actinomycin D) is an antitumor antibiotic used routinely to treat certain pediatric and adult cancers. Despite concerns over the incidence of toxicity, little is known about the pharmacology of dactinomycin. A study was done to investigate dactinomycin pharmacokinetics in children. EXPERIMENTAL DESIGN Dactinomycin was administered to(More)
OBJECTIVE To assess the variation in the current UK management strategies for the treatment of febrile neutropenia in childhood. DESIGN AND SETTING A postal survey of all 21 United Kingdom Children's Cancer Study Group (UKCCSG) centres assessing and collating local policies, protocols or guidelines relating to the management of febrile neutropenia.(More)
We present a case of a 4-month-old female infant with a maxillary melanotic neuroectodermal tumor of infancy (MNTI) and review the pooled data from previous publications on this entity. The literature to date comprises 378 reported cases from 1918 to the present, from which data on the presence or absence of metastatic disease was available in 311, and on(More)
PURPOSE Patients with febrile neutropaenia (FN) can be stratified according to their risk of significant complications, allowing reduced intensity therapy for low risk (LR) episodes. Serious events are very rare in low risk episodes making randomised trials difficult. Introduction of new evidence-based guidelines followed by re-auditing of the outcome is an(More)
AIMS To assess the annual risk of influenza infection in children with cancer and the immunogenicity of a trivalent split virus influenza vaccine in these children. METHODS Eighty four children with cancer were tested for susceptibility to the circulating strains of influenza virus in autumn 1995 and 1996. Non-immunised children were reassessed the(More)
Febrile neutropenia (FN) is only second to chemotherapy administration as a cause of hospital admission during treatment for cancer. As FN may signify serious or life-threatening infection, management protocols have focussed on trying to prevent adverse outcomes in these patients. However, it is now possible to identify a subset of patients with FN at low(More)
PURPOSE To improve the risk stratification of patients with rhabdomyosarcoma (RMS) through the use of clinical and molecular biologic data. PATIENTS AND METHODS Two independent data sets of gene-expression profiling for 124 and 101 patients with RMS were used to derive prognostic gene signatures by using a meta-analysis. These and a previously published(More)
BACKGROUND In March 2002, the Royal College of Paediatrics and Child Health (RCPCH) introduced guidelines for re-immunisation of children after completion of standard-dose chemotherapy and after haematopoietic stem cell transplant (HSCT). AIMS To ascertain whether these guidelines form standard unit policy by undertaking a survey of UK paediatric(More)