The impact of category, cytopathology and cytogenetics on development and progression of clonal and malignant myeloid transformation in inherited bone marrow failure syndromes

Inherited bone marrow failure syndromes are a group of rare, heterogeneous genetic disorders with a risk of clonal and malignant myeloid transformation including clonal marrow cytogenetic abnormalities, myelodysplastic syndrome and acute myeloid leukemia. The clinical characteristics, risk classification, prognostic factors and outcome of clonal and malignant myeloid transformation associated with inherited bone marrow failure syndromes are largely unknown. The aims of this study were to determine the impact of category, cytopathology and cytogenetics, the three components of the “Category Cytology Cytogenetics” classification of pediatric myelodysplastic syndrome, on the outcome of clonal and malignant myeloid transformation associated with inherited bone marrow failure. We used data from the Canadian Inherited Marrow Failure Registry. Among 327 patients with inherited bone marrow failure syndrome enrolled in the registry, the estimated risk of clonal and malignant myeloid transformation by the age of 18 years was 37%. The risk of clonal and malignant myeloid transformation varied according to the type of inherited bone marrow failure syndrome but was highest in Fanconi anemia. The development of clonal and malignant myeloid transformation significantly affected overall survival. Mortality varied based on cytopathological group. The largest group of patients had refractory cytopenia. Clonal marrow cytogenetic abnormalities were identified in 87% of patients with clonal and malignant myeloid transformation, and different cytogenetic groups had different impacts on disease progression. We conclude that category, cytopathology and cytogenetics in cases of clonal and malignant myeloid transformation associated with inherited bone marrow failure syndromes have an important impact on outcome and that the classification of such cases should incorporate these factors

Estimating Fuel Cycle Externalities: Analytical Methods and Issues, Report 2

The activities that produce electric power typically range from extracting and transporting a fuel, to its conversion into electric power, and finally to the disposition of residual by-products. This chain of activities is called a fuel cycle. A fuel cycle has emissions and other effects that result in unintended consequences. When these consequences affect third parties (i.e., those other than the producers and consumers of the fuel-cycle activity) in a way that is not reflected in the price of electricity, they are termed ''hidden'' social costs or externalities. They are the economic value of environmental, health and any other impacts, that the price of electricity does not reflect. How do you estimate the externalities of fuel cycles? Our previous report describes a methodological framework for doing so--called the damage function approach. This approach consists of five steps: (1) characterize the most important fuel cycle activities and their discharges, where importance is based on the expected magnitude of their externalities, (2) estimate the changes in pollutant concentrations or other effects of those activities, by modeling the dispersion and transformation of each pollutant, (3) calculate the impacts on ecosystems, human health, and any other resources of value (such as man-made structures), (4) translate the estimates of impacts into economic terms to estimate damages and benefits, and (5) assess the extent to which these damages and benefits are externalities, not reflected in the price of electricity. Each step requires a different set of equations, models and analysis. Analysts generally believe this to be the best approach for estimating externalities, but it has hardly been used! The reason is that it requires considerable analysis and calculation, and to this point in time, the necessary equations and models have not been assembled. Equally important, the process of identifying and estimating externalities leads to a number of complex issues that also have not been fully addressed. This document contains two types of papers that seek to fill part of this void. Some of the papers describe analytical methods that can be applied to one of the five steps of the damage function approach. The other papers discuss some of the complex issues that arise in trying to estimate externalities. This report, the second in a series of eight reports, is part of a joint study by the U.S. Department of Energy (DOE) and the Commission of the European Communities (EC)* on the externalities of fuel cycles. Most of the papers in this report were originally written as working papers during the initial phases of this study. The papers provide descriptions of the (non-radiological) atmospheric dispersion modeling that the study uses; reviews much of the relevant literature on ecological and health effects, and on the economic valuation of those impacts; contains several papers on some of the more complex and contentious issues in estimating externalities; and describes a method for depicting the quality of scientific information that a study uses. The analytical methods and issues that this report discusses generally pertain to more than one of the fuel cycles, though not necessarily to all of them. The report is divided into six parts, each one focusing on a different subject area

Natural disturbance impacts on trade-offs and co-benefits of forest biodiversity and carbon

With accelerating environmental change, understanding forest disturbance impacts on trade-offs between biodiversity and carbon dynamics is of high socio-economic importance. Most studies, however, have assessed immediate or short-term effects of disturbance, while long-term impacts remain poorly understood. Using a tree-ring-based approach, we analysed the effect of 250 years of disturbances on present-day biodiversity indicators and carbon dynamics in primary forests. Disturbance legacies spanning centuries shaped contemporary forest co-benefits and trade-offs, with contrasting, local-scale effects. Disturbances enhanced carbon sequestration, reaching maximum rates within a comparatively narrow post-disturbance window (up to 50 years). Concurrently, disturbance diminished aboveground carbon storage, which gradually returned to peak levels over centuries. Temporal patterns in biodiversity potential were bimodal; the first maximum coincided with the short-term post-disturbance carbon sequestration peak, and the second occurred during periods of maximum carbon storage in complex old-growth forest. Despite fluctuating local-scale trade-offs, forest biodiversity and carbon storage remained stable across the broader study region, and our data support a positive relationship between carbon stocks and biodiversity potential. These findings underscore the interdependencies of forest processes, and highlight the necessity of large-scale conservation programmes to effectively promote both biodiversity and long-term carbon storage, particularly given the accelerating global biodiversity and climate crises

GEOSPATIAL DATA FOR COMPUTERISATION OF PUBLIC ADMINISTRATION IN THE CZECH REPUBLIC

The main aim of the eGovernment programme in the Czech Republic is to enhance the efficiency of public administration. The Digital Map of Public Administration (DMVS) should be composed of digital orthophotographs of the Czech Republic, digital and digitised cadastral maps, digital purpose cadastral map (ÚKM) and a technical map of municipality, if available. The DMVS project is a part of computerisation of public administration in the Czech Republic. The project enhances the productivity of government administration and also simplifies the processes between citizens and public administration. The DMVS project, that should be compliant with the INSPIRE (Infrastructure for Spatial Information in the European Community) initiative, generates definite demand for geodata on the level of detail of land data model. The user needs that are clearly specified and required are not met due to inconsistencies in terminology, data management and level of detail

Ion formation in an argon and argon-oxygen gas mixture of a magnetron sputtering discharge

Formation of singly and doubly charged Arq+ and Tiq+ (q = 1,2) and of molecular Ar 2 +, ArTi+, and Ti 2 + ions in a direct current magnetron sputtering discharge with a Ti cathode and argon as working gas was investigated with the help of energy-resolved mass spectrometry. Measured ion energy distributions consist of low-energy and high-energy components resembling different formation processes. Intensities of Ar 2 + and ArTi+ dimer ions strongly increase with increasing gas pressure. Addition of oxygen gas leads to the formation of positively charged O+, O2 +, and TiO+ and of negatively charged O− and O2 - ions