Rezidivierende Urolithiasis mit progredienter Niereninsuffizienz

Zusammenfassung: Bei einem 30-jährigen Patienten bestand bei rezidivierender Urolithiasis und progredienter Niereninsuffizienz die initiale Diagnose einer sekundären Hyperoxalurie. Die vertiefte Anamnese sowie neue klinische Aspekte ließen dann eine primäre Hyperoxalurie (PH) vermuten, die molekulargenetisch als PH1 bestätigt werden konnte. Pathogenese, klinischer Verlauf und therapeutische Optionen der PH werden diskutier

Mandelbrot set in coupled logistic maps and in an electronic experiment

We suggest an approach to constructing physical systems with dynamical characteristics of the complex analytic iterative maps. The idea follows from a simple notion that the complex quadratic map by a variable change may be transformed into a set of two identical real one-dimensional quadratic maps with a particular coupling. Hence, dynamical behavior of similar nature may occur in coupled dissipative nonlinear systems, which relate to the Feigenbaum universality class. To substantiate the feasibility of this concept, we consider an electronic system, which exhibits dynamical phenomena intrinsic to complex analytic maps. Experimental results are presented, providing the Mandelbrot set in the parameter plane of this physical system.Comment: 9 pages, 3 figure

Nonequilibrium statistical operator method in the Renyi statistics

The generalization of the Zubarev nonequilibrium statistical operator method for the case of Renyi statistics is proposed when the relevant statistical operator (or distribution function) is obtained based on the principle of maximum for the Renyi entropy. The nonequilibrium statistical operator and corresponding generalized transport equations for the reduced-description parameters are obtained. A consistent description of kinetic and hydrodynamic processes in the system of interacting particles is considered as an example.Comment: 13 pages, RevTeX4-forma

PENGEMBANGAN BUKU AJAR AKUNTANSI KAS BERBASIS SCIENTIFIC APPROACH PADA MATA PELAJARAN AKUNTANSI KEUANGAN KELAS XI DI SMK NEGERI SE SURABAYA

Penelitian pengembangan ini bertujuan untuk menghasilkan buku ajar akuntansi kas berbasis scientific approach pada mata pelajaran akuntansi keuangan kelas XI di SMK Negeri Se Surabaya agar dapat meningkatkan motivasi belajar, meningkatkan penguasaan konsep materi dan pemahaman siswa dalam proses pembelajaran materi yang bersangkutan. Selain itu, penelitian ini bertujuan untuk mengetahui kelayakan dan respon siswa terhadap buku ajar akuntansi kas berbasis scientific approach yang dikembangkan. Model pengembangan yang digunakan diadaptasi dari model pengembangan 4P yang terdiri dari empat tahapan yaitu tahap pendefinisisan, tahap perancangan, tahap pengembangan, dan tahap penyebaran sehingga menghasilkan buku ajar akuntansi kas berbasis scientific approach. Hasil pengembangan menunjukkan bahwa rata-rata kelayakan buku ajar sebesar 83,5%. Sedangkan respon siswa diperoleh persentase sebesar 88,7%. Dengan demikian, buku ajar akuntansi kas berbasis scientific approach dinyatakan sangat layak digunakan sebagai bahan ajar untuk meningkatkan penguasaan konsep materi dan pemahaman siswa kelas XI di SMK Negeri Se Surabaya pada mata pelajaran akuntansi keuangan materi akuntansi kas. Kata Kunci:  Buku ajar, Scientific Approach, Akuntansi Ka

Binary decay of Ni56 formed in the 32S+24Mg reaction

Fully energy-damped yields from the Mg32 reaction have been measured at center-of-mass energies of Ec.m.=51.6 and 60.5 MeV with the use of an experimental arrangement where both of the resulting heavy fragments could be detected in coincidence. Energy, velocity, and angular distributions of the reaction fragments have been determined. The cross sections prior to secondary light-particle emission have been deduced for the breakup of the compound system into different mass channels. These data are discussed in terms of two possible reaction mechanisms: fusion followed by fission and deep-inelastic orbiting

Asymmetric fission of Ni56

Cross sections for the two-body channels populated in the S32+Mg24 reaction at Ec.m.=60.8 MeV have been measured by use of a coincidence technique which allows correction for secondary light-particle evaporation. The data show reaction yields with full equilibration of energy and mass-asymmetry coordinates. These results suggest an asymmetric fission mechanism and are contrary to what is expected from the previously proposed "orbiting" mechanism in light systems

Consequences of temperature fluctuations in observables measured in high energy collisions

We review the consequences of intrinsic, nonstatistical temperature fluctuations as seen in observables measured in high energy collisions. We do this from the point of view of nonextensive statistics and Tsallis distributions. Particular attention is paid to multiplicity fluctuations as a first consequence of temperature fluctuations, to the equivalence of temperature and volume fluctuations, to the generalized thermodynamic fluctuations relations allowing us to compare fluctuations observed in different parts of phase space, and to the problem of the relation between Tsallis entropy and Tsallis distributions. We also discuss the possible influence of conservation laws on these distributions and provide some examples of how one can get them without considering temperature fluctuations.Comment: Revised version of the invited contribution to The European Physical Journal A (Hadrons and Nuclei) topical issue about 'Relativistic Hydro- and Thermodynamics in Nuclear Physics' guest eds. Tamas S. Biro, Gergely G. Barnafoldi and Peter Va

Germline C1GALT1C1 mutation causes a multisystem chaperonopathy

Mutations in genes encoding molecular chaperones can lead to chaperonopathies, but none have so far been identified causing congenital disorders of glycosylation. Here we identified two maternal half-brothers with a novel chaperonopathy, causing impaired protein O-glycosylation. The patients have a decreased activity of T-synthase (C1GALT1), an enzyme that exclusively synthesizes the T-antigen, a ubiquitous O-glycan core structure and precursor for all extended O-glycans. The T-synthase function is dependent on its specific molecular chaperone Cosmc, which is encoded by X-chromosomal C1GALT1C1. Both patients carry the hemizygous variant c.59C>A (p.Ala20Asp; A20D-Cosmc) in C1GALT1C1. They exhibit developmental delay, immunodeficiency, short stature, thrombocytopenia, and acute kidney injury (AKI) resembling atypical hemolytic uremic syndrome. Their heterozygous mother and maternal grandmother show an attenuated phenotype with skewed X-inactivation in blood. AKI in the male patients proved fully responsive to treatment with the complement inhibitor Eculizumab. This germline variant occurs within the transmembrane domain of Cosmc, resulting in dramatically reduced expression of the Cosmc protein. Although A20D-Cosmc is functional, its decreased expression, though in a cell or tissue-specific manner, causes a large reduction of T-synthase protein and activity, which accordingly leads to expression of varied amounts of pathological Tn-antigen (GalNAcα1-O-Ser/Thr/Tyr) on multiple glycoproteins. Transient transfection of patient lymphoblastoid cells with wild-type C1GALT1C1 partially rescued the T-synthase and glycosylation defect. Interestingly, all four affected individuals have high levels of galactose-deficient IgA1 in sera. These results demonstrate that the A20D-Cosmc mutation defines a novel O-glycan chaperonopathy and causes the altered O-glycosylation status in these patients

Setting a baseline for global urban virome surveillance in sewage

The rapid development of megacities, and their growing connectedness across the world is becoming a distinct driver for emerging disease outbreaks. Early detection of unusual disease emergence and spread should therefore include such cities as part of risk-based surveillance. A catch-all metagenomic sequencing approach of urban sewage could potentially provide an unbiased insight into the dynamics of viral pathogens circulating in a community irrespective of access to care, a potential which already has been proven for the surveillance of poliovirus. Here, we present a detailed characterization of sewage viromes from a snapshot of 81 high density urban areas across the globe, including in-depth assessment of potential biases, as a proof of concept for catch-all viral pathogen surveillance. We show the ability to detect a wide range of viruses and geographical and seasonal differences for specific viral groups. Our findings offer a cross-sectional baseline for further research in viral surveillance from urban sewage samples and place previous studies in a global perspective

Avances en teledetección para la prevención y detección temprana de Xylella fastidiosa en el marco de los proyectos H2020 POnTE y XF-ACTORS.

Informational article of the tasks carried out in WP6 of POnTE and WP3 of XF-ACTORS in a professional Spanish magazin