14 research outputs found

    Reported Energy and Cost Savings from the DOE ESPC Program: FY 2013

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    Reported Energy and Cost Savings from the DOE ESPC Program: FY 2013

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    Reported Energy and Cost Savings from the DOE ESPC Program

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    The objective of this work was to determine the realization rate of energy and cost savings from the Department of Energy's Savings Performance Contract (ESPC) program based on information reported by the energy services companies (ESCOs) that are carrying out ESPC projects at federal sites. Information was extracted from 134 Measurement and Verification (M&V) reports to determine reported, estimated, and guaranteed cost savings and reported and estimated energy savings for the previous contract year. Because the quality of the reports varied, it was not possible to determine all of these parameters for each project. For 133 of the 134 projects, there was sufficient information to compare estimated, reported, and guaranteed cost savings. For this group, the total estimated cost savings for the reporting periods addressed were 95.7million,totalreportedcostsavingswere95.7 million, total reported cost savings were 96.8 million, and total guaranteed cost savings were $92.1 million. This means that on average: ESPC contractors guaranteed 96% of the estimated cost savings, projects reported achieving 101% of the estimated cost savings, and projects reported achieving 105% of the guaranteed cost savings. For 129 of the projects examined, there was sufficient information to compare estimated and reported energy savings. On the basis of site energy, estimated savings for those projects for the previous year totaled 5.371 million MMBtu, and reported savings were 5.374 million MMBtu, just over 100% of the estimated energy savings. On the basis of source energy, total estimated energy savings for the 129 projects were 10.400 million MMBtu, and reported saving were 10.405 million MMBtu, again, just over 100.0% of the estimated energy savings

    Management of acute promyelocytic leukemia: Recommendations from an expert panel on behalf of the European LeukemiaNet

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    The introduction of all-trans retinoic acid (ATRA) and, more recently, arsenic trioxide (ATO) into the therapy of acute promyelocytic leukemia (APL) has revolutionized the management and outcome of this disease. Several treatment strategies using these agents, usually in combination with chemotherapy, but also without or with minimal use of cytotoxic agents, have provided excellent therapeutic results. Cure of APL patients, however, is also dependent on peculiar aspects related to the management and supportive measures that are crucial to counteract life-threatening complications associated with the disease biology and molecularly targeted treatment. The European LeukemiaNet recently appointed an international panel of experts to develop evidence- and expert opinion-based guidelines on the diagnosis and management of APL. Together with providing current indications on genetic diagnosis, modern risk-adapted front-line therapy and salvage treatment, the review contains specific recommendations for the ide

    DYRK1A haploinsufficiency causes a new recognizable syndrome with microcephaly, intellectual disability, speech impairment, and distinct facies

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    Dual-specificity tyrosine-(Y)-phosphorylation-regulated kinase 1 A (DYRK1A ) is a highly conserved gene located in the Down syndrome critical region. It has an important role in early development and regulation of neuronal proliferation. Microdeletions of chromosome 21q22.12q22.3 that include DYRK1A (21q22.13) are rare and only a few pathogenic single-nucleotide variants (SNVs) in the DYRK1A gene have been described, so as of yet, the landscape of DYRK1A disruptions and their associated phenotype has not been fully explored. We have identified 14 individuals with de novo heterozygous variants of DYRK1A; five with microdeletions, three with small insertions or deletions (INDELs) and six with deleterious SNVs. The analysis of our cohort and comparison with published cases reveals that phenotypes are consistent among individuals with the 21q22.12q22.3 microdeletion and those with translocation, SNVs, or INDELs within DYRK1A. All individuals shared congenital microcephaly at birth, intellectual disability, developmental delay, severe speech impairment, short stature, and distinct facial features. The severity of the microcephaly varied from −2 SD to −5 SD. Seizures, structural brain abnormalities, eye defects, ataxia/broad-based gait, intrauterine growth restriction, minor skeletal abnormalities, and feeding difficulties were present in two-thirds of all affected individuals. Our study demonstrates that haploinsufficiency of DYRK1A results in a new recognizable syndrome, which should be considered in individuals with Angelman syndrome-like features and distinct facial features. Our report represents the largest cohort of individuals with DYRK1A disruptions to date, and is the first attempt to define consistent genotype–phenotype correlations among subjects with 21q22.13 microdeletions and DYRK1A SNVs or small INDELs
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