Guest Artist Recital: Edward J. Kaizer, Piano; February 11, 1974

Centennial East Recital HallMonday EveningFebruary 11, 19748:15 p.m

Intrathecal enzyme replacement for Hurler syndrome: biomarker association with neurocognitive outcomes.

PurposeAbnormalities in cerebrospinal fluid (CSF) have been reported in Hurler syndrome, a fatal neurodegenerative lysosomal disorder. While no biomarker has predicted neurocognitive response to treatment, one of these abnormalities, glycosaminoglycan nonreducing ends (NREs), holds promise to monitor therapeutic efficacy. A trial of intrathecal enzyme replacement therapy (ERT) added to standard treatment enabled tracking of CSF abnormalities, including NREs. We evaluated safety, biomarker response, and neurocognitive correlates of change.MethodsIn addition to intravenous ERT and hematopoietic cell transplantation, patients (N = 24) received intrathecal ERT at four peritransplant time points; CSF was evaluated at each point. Neurocognitive functioning was quantified at baseline, 1 year, and 2 years posttransplant. Changes in CSF biomarkers and neurocognitive function were evaluated for an association.ResultsOver treatment, there were significant decreases in CSF opening pressure, biomarkers of disease activity, and markers of inflammation. Percent decrease in NRE from pretreatment to final intrathecal dose posttransplant was positively associated with percent change in neurocognitive score from pretreatment to 2 years posttransplant.ConclusionIntrathecal ERT was safe and, in combination with standard treatment, was associated with reductions in CSF abnormalities. Critically, we report evidence of a link between a biomarker treatment response and neurocognitive outcome in Hurler syndrome

Snapshot of the Atlantic Forest canopy: surveying arboreal mammals in a biodiversity hotspot

The Atlantic Forest of South America supports a rich terrestrial biodiversity but has been reduced to only a small extent of its original forest cover. It hosts a large number of endemic mammalian species but our knowledge of arboreal mammal ecology and conservation has been limited because of the challenges of observing arboreal species from ground level. Camera trapping has proven to be an effective tool in terrestrial mammal monitoring but the technique has rarely been used for arboreal species. For the first time in the Atlantic Forest, we obtained data on the arboreal mammal community using arboreal camera trapping, focusing on Caparaó National Park, Brazil. We placed 24 infrared camera traps in the forest canopy in seven areas within the Park, operating them continuously during January 2017–June 2019. During this period the camera traps accumulated 4,736 camera-days of footage and generated a total of 2,256 photographs and 30-s videos of vertebrates. The arboreal camera traps were able to detect arboreal mammals of a range of body sizes. The mammal assemblage comprised 15 identifiable species, including the Critically Endangered northern muriqui Brachyteles hypoxanthus and buffy-headed marmoset Callithrix flaviceps as well as other rare, nocturnal and inconspicuous species. We confirmed for the first time the occurrence of the thin-spined porcupine Chaetomys subspinosus in the Park. Species richness varied across survey areas and forest types. Our findings demonstrate the potential of arboreal camera trapping to inform conservation strategies

Cesium, iodine and tritium in NW Pacific waters - a comparison of the Fukushima impact with global fallout

Radionuclide impact of the Fukushima Dai-ichi nuclear power plant accident on the distribution of radionuclides in seawater of the NW Pacific Ocean is compared with global fallout from atmospheric tests of nuclear weapons. Surface and water column samples collected during the <i>Ka'imikai-o-Kanaloa</i> (<i>KOK</i>) international expedition carried out in June 2011 were analyzed for ¹³⁴Cs, ¹³⁷Cs, ¹²⁹I and ³H. The ¹³⁷Cs, ¹²⁹I and ³H levels in surface seawater offshore Fukushima varied between 0.002–3.5 Bq L⁻¹, 0.01–0.8 μBq L⁻¹, and 0.05–0.15 Bq L⁻¹, respectively. At the sampling site about 40 km from the coast, where all three radionuclides were analyzed, the Fukushima impact on the levels of these three radionuclides represents an increase above the global fallout background by factors of about 1000, 50 and 3, respectively. The water column data indicate that the transport of Fukushima-derived radionuclides downward to the depth of 300 m has already occurred. The observed ¹³⁷Cs levels in surface waters and in the water column are compared with predictions obtained from the ocean general circulation model, which indicates that the Kuroshio Current acts as a southern boundary for the transport of the radionuclides, which have been transported from the Fukushima coast eastward in the NW Pacific Ocean. The ¹³⁷Cs inventory in the water column is estimated to be about 2.2 PBq, what can be regarded as a lower limit of the direct liquid discharges into the sea as the seawater sampling was carried out only in the area from 34 to 37° N, and from 142 to 147° E. About 4.6 GBq of ¹²⁹I was deposited in the NW Pacific Ocean, and 2.4–7 GBq of ¹²⁹I was directly discharged as liquid wastes into the sea offshore Fukushima. The total amount of ³H released and deposited over the NW Pacific Ocean was estimated to be 0.1–0.5 PBq. These estimations depend, however, on the evaluation of the total ¹³⁷Cs activities released as liquid wastes directly into the sea, which should improve when more data are available. Due to a suitable residence time in the ocean, Fukushima-derived radionuclides will provide useful tracers for isotope oceanography studies on the transport of water masses during the next decades in the NW Pacific Ocean

O Impacto da Regulamentação Contábil sobre o Conservadorismo nas Empresas do Setor Elétrico do Brasil

This article examines whether the application of conservatism is different between companies in the Brazilian electric power sector (which are subject to specific accounting regulations) and other Brazilian companies not subject to any specific rules. Accounting has been studied for its ability to provide information through accounting variables, which is useful for the users' decisions. One of the most important of these variables is accounting income. However, the information content of this income may not accurately reflect a firm's real situation because it can be influenced by factors and characteristics of Brazilian accounting. Among these factors is the existence of specific regulations. The sample consisted of data on companies in the Brazilian electric power sector and other Brazilian companies not subject to any specific accounting rules, covering the period from December 1998 to December 2006, taken from the Economática database. To investigate the differences in accounting income, we used the model proposed by Basu (1997). We found that companies in other sectors act more conservatively. It can be inferred, then, that incidence of specific accounting regulations can influence the application of conservatism in the companies studied.El artículo tiene como objetivo investigar si la aplicación del Conservadorismo es diferente entre empresas del sector eléctrico brasileño (que sufren incidencia de reglamentación contable específica) y empresas brasileñas que no sufren incidencia de reglamentación contable específica. La contabilidad ha sido estudiada a partir de su capacidad de suministrar informaciones útiles al proceso de decisión de los usuarios y eso ocurre por medio de las variables contables. Una de esas principales variables es el beneficio contable. Sin embargo, el contenido informativo del beneficio puede no reflejar la realidad de las empresas, pues puede ser influido por factores y características de la contabilidad brasileña. Entre esos factores, se puede citar la incidencia de reglamentación contable específica. La muestra fue formada con datos colectados de empresas del sector eléctrico brasileño y de empresas brasileñas que no sufren incidencia de reglamentación contable específica, buscados en el banco de datos Economática. En esta pesquisa, fue utilizado el modelo propuesto por Basu (1997). El período analizado comprende diciembre de 1998 a diciembre de 2006. Se verificó que existe una aplicación menor del Conservadorismo en las empresas de los otros sectores frente a las empresas de energía eléctrica, evidenciando, por tanto, diferencia en la aplicación del Conservadorismo entre los dos grupos de empresas. Se puede inferir que la incidencia de reglamentación contable específica puede influenciar la elaboración del beneficio contable y, consecuentemente, influir en la aplicación del Conservadorismo en las empresas estudiadas.O artigo objetiva investigar se a aplicação do Conservadorismo é diferente entre empresas do setor elétrico brasileiro (que sofrem incidência de regulamentação contábil específica) e empresas brasileiras que não sofrem incidência de regulamentação contábil específica. A contabilidade tem sido estudada a partir de sua capacidade de fornecer informações úteis ao processo de decisão dos usuários e isso ocorre por meio das variáveis contábeis. Uma dessas principais variáveis é o lucro contábil. Porém, o conteúdo informacional do lucro pode não refletir a realidade das empresas, pois pode ser influenciado por fatores e características da contabilidade brasileira. Entre esses fatores, pode-se citar a incidência de regulamentação contábil específica. A amostra foi formada com dados coletados de empresas do setor elétrico brasileiro e de empresas brasileiras que não sofrem incidência de regulamentação contábil específica, buscados no banco de dados Economática. Nesta pesquisa, foi utilizado o modelo proposto por Basu (1997). O período analisado compreende dezembro de 1998 a dezembro de 2006. Verificou-se que existe uma menor aplicação do Conservadorismo nas empresas dos outros setores frente às empresas de energia elétrica, evidenciando, portanto, diferença na aplicação do Conservadorismo entre os dois grupos de empresas. Pode-se inferir que a incidência de regulamentação contábil específica pode influenciar a elaboração do lucro contábil e, consequentemente, influenciar a aplicação do Conservadorismo nas empresas estudadas

Faculty Recital

Program listing performers and works performe

The Large Enriched Germanium Experiment for Neutrinoless Double Beta Decay (LEGEND)

The observation of neutrinoless double-beta decay (0${\nu}{\beta}{\beta}$) would show that lepton number is violated, reveal that neutrinos are Majorana particles, and provide information on neutrino mass. A discovery-capable experiment covering the inverted ordering region, with effective Majorana neutrino masses of 15 - 50 meV, will require a tonne-scale experiment with excellent energy resolution and extremely low backgrounds, at the level of $\sim$0.1 count /(FWHM$\cdot$t$\cdot$yr) in the region of the signal. The current generation $^{76}$Ge experiments GERDA and the MAJORANA DEMONSTRATOR utilizing high purity Germanium detectors with an intrinsic energy resolution of 0.12%, have achieved the lowest backgrounds by over an order of magnitude in the 0${\nu}{\beta}{\beta}$ signal region of all 0${\nu}{\beta}{\beta}$ experiments. Building on this success, the LEGEND collaboration has been formed to pursue a tonne-scale $^{76}$Ge experiment. The collaboration aims to develop a phased 0${\nu}{\beta}{\beta}$ experimental program with discovery potential at a half-life approaching or at $10^{28}$ years, using existing resources as appropriate to expedite physics results.Comment: Proceedings of the MEDEX'17 meeting (Prague, May 29 - June 2, 2017

Randomized comparison of gemcitabine, dexamethasone, and cisplatin versus dexamethasone, cytarabine, and cisplatin chemotherapy before autologous stem-cell transplantation for relapsed and refractory aggressive lymphomas: NCIC-CTG LY.12

PURPOSE: For patients with relapsed or refractory aggressive lymphoma, we hypothesized that gemcitabine-based therapy before autologous stem-cell transplantation (ASCT) is as effective as and less toxic than standard treatment. PATIENTS AND METHODS: We randomly assigned 619 patients with relapsed/refractory aggressive lymphoma to treatment with gemcitabine, dexamethasone, and cisplatin (GDP) or to dexamethasone, cytarabine, and cisplatin (DHAP). Patients with B-cell lymphoma also received rituximab. Responding patients proceeded to stem-cell collection and ASCT. Coprimary end points were response rate after two treatment cycles and transplantation rate. The noninferiority margin for the response rate to GDP relative to DHAP was set at 10%. Secondary end points included event-free and overall survival, treatment toxicity, and quality of life. RESULTS: For the intention-to-treat population, the response rate with GDP was 45.2%; with DHAP the response rate was 44.0% (95% CI for difference, -9.0% to 6.7%), meeting protocol-defined criteria for noninferiority of GDP (P = .005). Similar results were obtained in a per-protocol analysis. The transplantation rates were 52.1% with GDP and 49.3% with DHAP (P = .44). At a median follow-up of 53 months, no differences were detected in event-free survival (HR, 0.99; stratified log-rank P = .95) or overall survival (HR, 1.03; P = .78) between GDP and DHAP. Treatment with GDP was associated with less toxicity (P < .001) and need for hospitalization (P < .001), and preserved quality of life (P = .04). CONCLUSION: For patients with relapsed or refractory aggressive lymphoma, in comparison with DHAP, treatment with GDP is associated with a noninferior response rate, similar transplantation rate, event-free survival, and overall survival, less toxicity and hospitalization, and superior quality of life