58 research outputs found

    A digital simulation for optimum nonlinear detection of binary signals in impulsive noise

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    The problem of reducing the effects of impulsive noise on binary communication systems is considered. A digital simulation of such a system is developed in order to find a nearly optimum (minimum probability of error criterion) nonlinear device to precede detection. The level of the Gaussian noise power is found to be the most important parameter for determining the nearly optimum nonlinear device --Abstract, page ii

    A Population-Based Study of Childhood Cancer Survivors’ Body Mass Index

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    Background. Population-based studies are needed to estimate the prevalence of underweight or overweight/obese childhood cancer survivors. Procedure. Adult survivors (diagnosed ≤20 years) were identified from the linked Utah Cancer Registry and Utah Population Database. We included survivors currently aged ≥20 years and ≥5 years from diagnosis (N=1060), and a comparison cohort selected on birth year and sex (N=5410). BMI was calculated from driver license data available from 2000 to 2010. Multivariable generalized linear regression models were used to calculate prevalence relative risks (RR) and 95% confidence intervals (95% CI) of BMI outcomes for survivors and the comparison cohort. Results. Average time since diagnosis was 18.5 years (SD=7.8), and mean age at BMI for both groups was 30.5 (survivors SD=7.7, comparison SD=8.0). Considering all diagnoses, survivors were not at higher risk for being underweight or overweight/obese than the comparison. Male central nervous system tumor survivors were overweight (RR=1.12, 95% CI 1.01–1.23) more often than the comparison. Female survivors, who were diagnosed at age 10 and under, had a 10% higher risk of being obese than survivors diagnosed at ages 16–20 (P<0.05). Conclusion. While certain groups of childhood cancer survivors are at risk for being overweight/obese, in general they do not differ from population estimates

    Molecular and phenotypic diversity of <I>CBL</I>-mutated juvenile myelomonocytic leukemia

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    Mutations in the CBL gene were first identified in adults with various myeloid malignancies. Some patients with juvenile myelomonocytic leukemia (JMML) were also noted to harbor mutations in CBL, but were found to have generally less aggressive disease courses compared to patients with other forms of Ras pathway-mutant JMML. Importantly, and in contrast to most reports in adults, the majority of CBL mutations in JMML patients are germline with acquired uniparental disomy occurring in affected marrow cells. Here, we systematically studied a large cohort of 33 JMML patients with CBL mutations and found that this disease is highly diverse in presentation and overall outcome. Moreover, we discovered somatically acquired CBL mutations in 15% of pediatric patients who presented with more aggressive disease. Neither clinical features nor methylation profiling were able to distinguish patients with somatic CBL mutations from those with germline CBL mutations, highlighting the need for germline testing. Overall, we demonstrate that disease courses are quite heterogeneous even among patients with germline CBL mutations. Prospective clinical trials are warranted to find ideal treatment strategies for this diverse cohort of patients

    The genomic landscape of juvenile myelomonocytic leukemia

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    Juvenile myelomonocytic leukemia (JMML) is a myeloproliferative neoplasm (MPN) of childhood with a poor prognosis. Mutations in NF1, NRAS, KRAS, PTPN11 and CBL occur in 85% of patients, yet there are currently no risk stratification algorithms capable of predicting which patients will be refractory to conventional treatment and therefore be candidates for experimental therapies. In addition, there have been few other molecular pathways identified aside from the Ras/MAPK pathway to serve as the basis for such novel therapeutic strategies. We therefore sought to genomically characterize serial samples from patients at diagnosis through relapse and transformation to acute myeloid leukemia in order to expand our knowledge of the mutational spectrum in JMML. We identified recurrent mutations in genes involved in signal transduction, gene splicing, the polycomb repressive complex 2 (PRC2) and transcription. Importantly, the number of somatic alterations present at diagnosis appears to be the major determinant of outcome

    Small center, big heart

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    Extrapleural pneumonectomy for advanced pleuropulmonary blastoma

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    Purpose: Use of extrapleural pneumonectomy for malignancy in children is rarely performed with few descriptions in the literature. In this article, we describe a 19-month-old girl who underwent an extrapleural pneumonectomy with enbloc resection of the diaphragm and pericardium for advanced pleuropulmonary blastoma. We achieved an R0 resection and survival without local disease recurrence at 23 months. Conclusion: Extrapleural pneumonectomy is feasible in some circumstances to achieve oncologic control in cases of advanced PPB. This should be kept as part of the surgeons' armamentarium. Keywords: Pleuropulmonary blastoma, Tension physiology, Pneumonectomy, Extrapleural pneumonectom
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