Spectrum

Mitochondrial DNA depletion syndromes (MDSs) form a group of autosomal recessive disorders characterized by profoundly decreased mitochondrial DNA copy numbers in affected tissues. Three main clinical presentations are known: myopathic, encephalomyopathic and hepatocerebral. The first is associated with mutations in thymidine kinase 2 (TK2) and p53-induced ribonucleotide reductase B subunit (RRM2B); the second with mutations in succinate synthase A (SUCLA2) and B (SUCLG1); the third with mutations in Twinkle (PEO1), pol-γA (POLG1), deoxyguanosine kinase (DGUOK) and MPV17 (MPV17). In this work, we review the MDS-associated phenotypes and present our own experience of 32 MDS patients, with the aim of defining the mutation frequency of the known genes, the clinical spectrum of the diseases, and the genotype–phenotype correlations. Five of our patients carried previously unreported mutations in one of the eight MDS genes.

This is a literature review about large granular lymphocyte leukemia (LGLL), a rare and misdiagnosed oncohematological disease, characterized by a clonal expansion of T-cells (T-LGLL) or NK-cells (NK-LGLL) in the bone marrow and/or peripheral blood. The clinical features of LGLL include cytopenias (anemia, neutropenia and thrombocytopenia), lymphocytosis (usually discrete), lymphadenopathy, hepatomegaly, splenomegaly, immune abnormalities and constitutional symptoms (fever, night sweats and weight loss). The diagnosis is based on the confirmation of the clonality of T-cells or NK-cells (polymerase chain reaction and Southern blot are the two methods most commonly used) and typical findings of the immunophenotypic analysis of peripheral blood lymphocytes (flow cytometry analyses for specific surface antigens). In contrast to the chronic and indolent course of T-LGLL, NK-LGLL has an acute presentation and poor clinical outcome. There are different current treatment options, depending on clinical presentation.

The SystemVerilog 3.1 language initiative includes the requirement to bring together design, testbench, and assertion-based descriptions into a consistent, backward compatible, evolutionary language standard that achieves determinism and common semantics across the spectrum of design and verification tools. This paper describes the algorithm proposed for the new scheduling semantics that meets these demanding requirements. This algorithm achieves predictable and consistent results across design and verification tools. This proposal has been donated to the Accellera SystemVerilog 3.1 standardization technical committees. Standardization of this new algorithm will extend these benefits to users of other tools.

F. Granelli et al. (Eds.): MOBILIGHT 2009, LNICST 13, pp. 230–241, 2009. © ICST Institute for Computer Sciences, Social-Informatics and Telecommunication Engineering 2009 ... Self-management in Future Internet Wireless Networks: ... Dynamic Resource Allocation and Traffic Routing ...

Critical Infrastructures, such as energy, banking, and transport, are an essential pillar to the well-being of the national and international economy, security and quality of life. These infrastructures are dependent on a spectrum of highly interconnected information infrastructures for their smooth, reliable and continuous operation. The field of protecting such Critical Information Infrastructures, or CIIP, faces numerous challenges, such as managing the secure interaction between peers, assuring the resilience and robustness of ...

We investigate symbolic sequences and in particular information carriers as e.g. books and DNA–strings. First the higher order Shannon entropies are calculated, a characteristic root law is detected. Then the algorithmic entropy is estimated by using Lempel–Ziv compression algorithms. In the third section the correlation function for distant letters, the low frequency Fourier spectrum and the characteristic scaling exponents are calculated. We show that all these measures are able to detect long–range correlations. However, as demonstrated by shuffling experiments, different measures operate on different length scales. The longest correlations found in our analysis comprise a few hundreds or thousands of letters and may be understood as long–wave fluctuations of the composition. 1

Data from paleomagnetic and sedimentological studies carried out on four cores from the bottom sediments of Lake Moreno (southern Argentina) were integrated with data obtained by Creer et al., Geophys. J. R. Astron. Soc., 74 (1983), 109. Within-lake correlations are based on the magnetic susceptibility and intensity of remanent magnetization. The individual measurements were stacked and transformed to a time-scale

Cognitive radio has the ability to sense the environment and adapt its behavior to optimize communication features, such as quality of service in the presence of interference and noise. In physical layer to achieve this goal, different phases of sensing, channel estimation, and configuration selection are necessary. The sensing part measures the interference level, recognize the spectrum holes, and send this information to channel estimator. In the next step, channel state information (CSI) is used for data detection and also sent to the transmitter through a limited feedback. CSI feedback consists of achievable rate, SNR value, modulation or coding schemes (MCS). Feedback link improves the system performance in the cost of complexity and delay. In this paper, we present and compare different feedback schemes for cognitive radio and study the channel capacity when an imperfect feedback link is corrupted by noise and delay.

A new paradigm for active galactic jet kinematics has emerged through detailed investigations of BL Lac objects using very long baseline radio interferometry. In this new scheme, most, if not all, jet components appear to remain stationary with respect to the core but show significant non-radial motions. This paper presents results from our kinematic investigation of the jets of a statistically complete sample of radio-loud flat-spectrum active galaxies, focusing on the comparison between the jet kinematic properties of BL Lacs and flat-spectrum radio-quasars. It is shown that there is a statistically significant difference between the kinematics of the two AGN classes, with BL Lacs showing more bent jets, that are wider and show slower movement along the jet axis, compared to flat-spectrum radio-quasars. This is interpreted as evidence for helically structured jets.

Publish-subscribe middleware allows the components of a distributed application to subscribe for event notifications and provides the infrastructure enabling event routing from sources to subscribers. This model decouples publishers from subscribers, and in principle makes it amenable to highly dynamic environments. Nevertheless, publish-subscribe systems exploiting a distributed event dispatcher are typically not able to rearrange dynamically their operations to adapt to changes impacting the topology of the dispatching infrastructure. In this work, we first describe two solutions available in the literature that constitute the extremes of the reconfiguration spectrum in terms of the number of nodes potentially affected by the reconfiguration. They differ essentially in the tradeoffs between simplicity and efficiency. Then, we introduce our contribution as a new algorithm that strikes a balance between the aforementioned solutions by tolerating frequent reconfigurations at the cost of moderate overhead.

The results of investigation of the spectral characteristics of radiation in the presence of the photonic flame effect recently discovered experimentally are presented. Radiation from a ruby laser operating in the Q-factor modulation regime with a generation wavelength of 694.3 nm is focused near the oriented surface of a globular photon crystal (artificial opal with a globule size of 230 nm) placed on a copper cold guide whose temperature is close to the liquid-nitrogen boiling point (77 K). The spectrum of long-term afterglow (with a duration of about several seconds when the duration of a pump pulse is equal to 20 ns) appearing in the photonic flame effect is detected using a fiber optic minispectrometer and consists of several comparatively narrow lines whose intensity strongly varies with the pumping power. A similar glow is observed in opal samples filled with acetone or ethanol.