Myeloproliferative Diseases as Possible Risk Factor for Development of Chronic Thromboembolic Pulmonary Hypertension—A Genetic Study

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare disease which is often caused by recurrent emboli. These are also frequently found in patients with myeloproliferative diseases. While myeloproliferative diseases can be caused by gene defects, the genetic predisposition to CTEPH is largely unexplored. Therefore, the objective of this study was to analyse these genes and further genes involved in pulmonary hypertension in CTEPH patients. A systematic screening was conducted for pathogenic variants using a gene panel based on next generation sequencing. CTEPH was diagnosed according to current guidelines. In this study, out of 40 CTEPH patients 4 (10%) carried pathogenic variants. One patient had a nonsense variant (c.2071A>T p.Lys691*) in the BMPR2 gene and three further patients carried the same pathogenic variant (missense variant, c.1849G>T p.Val617Phe) in the Janus kinase 2 (JAK2) gene. The latter led to a myeloproliferative disease in each patient. The prevalence of this JAK2 variant was significantly higher than expected (p < 0.0001). CTEPH patients may have a genetic predisposition more often than previously thought. The predisposition for myeloproliferative diseases could be an additional risk factor for CTEPH development. Thus, clinical screening for myeloproliferative diseases and genetic testing may be considered also for CTEPH patients

Molecular Phylogeny of the Acanthocephala (Class Palaeacanthocephala) with a Paraphyletic Assemblage of the Orders Polymorphida and Echinorhynchida

Acanthocephalans are attractive candidates as model organisms for studying the ecology and co-evolutionary history of parasitic life cycles in the marine ecosystem. Adding to earlier molecular analyses of this taxon, a total of 36 acanthocephalans belonging to the classes Archiacanthocephala (3 species), Eoacanthocephala (3 species), Palaeacanthocephala (29 species), Polyacanthocephala (1 species) and Rotifera as outgroup (3 species) were analyzed by using Bayesian Inference and Maximum Likelihood analyses of nuclear 18S rDNA sequence. This data set included three re-collected and six newly collected taxa, Bolbosoma vasculosum from Lepturacanthus savala, Filisoma rizalinum from Scatophagus argus, Rhadinorhynchus pristis from Gempylus serpens, R. lintoni from Selar crumenophthalmus, Serrasentis sagittifer from Johnius coitor, and Southwellina hispida from Epinephelus coioides, representing 5 new host and 3 new locality records. The resulting trees suggest a paraphyletic arrangement of the Echinorhynchida and Polymorphida inside the Palaeacanthocephala. This questions the placement of the genera Serrasentis and Gorgorhynchoides within the Echinorhynchida and not the Polymorphida, necessitating further insights into the systematic position of these taxa based on morphology

Sum rules and spectral patterns of dichroism in inner-shell photoelectron spectra

A theoretical description of dichroism in the photoelectron spectra of atoms polarized in different directions is presented. It is based on the general expression for the photoelectron angular distribution written in LSJ approximation. We show that, for photon energies far above threshold, the spectral distribution of dichroism in the photoelectron spectrum in most cases does not depend on the photoionization dynamics; for atoms with S symmetry, simple spectral patterns are obtained. Useful sum rules are given for the dichroism integrated over groups of photoelectron multiplet lines. Recent experimental data on magnetic dichroism and linear dichroism in the nonresonant inner-shell spectra of Cr (3p shell), Eu (4f shell), and Fe (3p shell) are analyzed. An outlook is given on the dichroism in the region of autoionization resonances

Magnetic dichroism in the 4f4f photoelectron spectra of free Eu atoms: Experimental proof of the atomic character of thin film Eu–Gd MCD

The 4f photoionization of Eu atoms oriented in the ground state was investigated by means of photoelectron spectroscopy with linearly polarized vacuum ultraviolet radiation. Strong magnetic dichroism could be observed for atoms oriented parallel/antiparallel to the VUV photon beam by optical pumping with circularly polarized laser radiation. The magnetic dichroism of the free atoms, well described within the general theory of photoelectron angular distribution, displays the same characteristic features as the CMD of thin Eu films on a ferromagnetic Gd substrate

Kombinierte Experimente zum XUV-Dichroismus polarisierter Metallatome

The 4f photoionization of polarized Europium atoms in an atomic beam was studied by electron spectroscopy. The atoms were prepared in the oriented or aligned ground state by optical pumping with circularly or linearly polarized laser radiation. Monochromatized synchrotron radiation of the electron storage ring BESSY (Berlin) was used to ionize the atoms well above the 5p thresholds. A strong dichroism in the 4f main line was observed for oriented as well as for aligned Eu atoms. The shape of the dichroism in the photoelectron spectrum could be explained very well within an LS-coupling model. Detailed information on the amplitudes and phases of the dipole matrix elements, and therefore on the dynamics of photoionization, could be extracted by normalization to the photoionization cross section. Strong configuration interaction in the final state, which was confirmed by independent high-resolution photoelectron spectroscopy, established the necessity to take into account the many-body aspect of atomic photoionization. The dichroism in the atomic spectrum closely agrees with the corresponding circular magnetic dichroism in the 4f spectrum of a thin film of Eu on Gd, which emphasizes the atomic character of dichroism in the 4f photoionization of bound Eu and Gd atoms. (orig.)107 refs.Available from TIB Hannover: RR 8919(1998-030) / FIZ - Fachinformationszzentrum Karlsruhe / TIB - Technische InformationsbibliothekSIGLEDEGerman

Coplanar branch-line and rat-race couplers for W-band applications

In this paper, we report on coplanar branch-line and rat-race couplers on GaAs for use in balanced mixers, investigated theoretically and experimentally over the frequency range from DC to 120 GHz. For optimum spurious response suppression at the IF-port of a balanced mixer, a coupler must provide the required phase delay at its ports feeding the non-linear devices. Whereas in microstrip (MS) technology these types of couplers have been extensively investigated in the past, coplanar waveguide (CPW) technology is still lacking similar broadband models, making millimetre-wave circuit design difficult. For the characteristic impedance levels 30,50 and 67 Ohm, a model library was developed for transmission lines and line elements like air bridges and corners, and implemented into HP-MDS. On the basis of these models, we designed a branch-line coupler, a capacitively loaded branch-line coupler and a rat-race coupler for W-band applications. For the analysis of the phase relation between the different ports of these hybrids, 2-port test structures were designed and processed. The 'on-wafer' measured S- parameters of the couplers show excellent agreement with the simulated data in both amplitude and phase over the entire frequency range

Breakdown of the three-parameter model for the dichroism in the 4f4f photoelectron spectrum of laser-aligned Eu atoms

The dichroism in the 4f photoelectron spectra of laser-aligned Eu atoms has been determined with linearly polarized vacuum ultraviolet radiation. The experimental results disagree with the prediction of the three-parameter model based on the absolute magnitude and relative phase of the 4ftovarepsilon d,varepsilon g transition matrix elements. It is suggested that strong configuration interactions, confirmed in high-resolution photoelectron spectra, are the cause of the breakdown of the three-parameter model