Misdiagnosis of hereditary amyloidosis as AL (Primary) amyloidosis

Background: Hereditary, autosomal dominant amyloidosis, caused by mutations in the genes encoding transthyretin, fibrinogen A -chain, lysozyme, or apolipoprotein A-I, is thought to be extremely rare and is not routinely included in the differential diagnosis of systemic amyloidosis unless there is a family history. Methods: We studied 350 patients with systemic amyloidosis, in whom a diagnosis of the light-chain (AL) type of the disorder had been suggested by clinical and laboratory findings and by the absence of a family history, to assess whether they had amyloidogenic mutations. Results: Amyloidogenic mutations were present in 34 of the 350 patients (9.7 percent), most often in the genes encoding fibrinogen A -chain (18 patients) and transthyretin (13 patients). In all 34 of these patients, the diagnosis of hereditary amyloidosis was confirmed by additional investigations. A low-grade monoclonal gammopathy was detected in 8 of the 34 patients (24 percent). Conclusions: A genetic cause should be sought in all patients with amyloidosis that is not the reactive systemic amyloid A type and in whom confirmation of the AL type cannot be obtained

A new microscopic nucleon-nucleon interaction derived from relativistic mean field theory

A new microscopic nucleon-nucleon (NN) interaction has been derived for the first time from the popular relativistic mean field theory (RMFT) Lagrangian. The NN interaction so obtained remarkably relate to the inbuilt fundamental parameters of RMFT. Furthermore, by folding it with the RMFT-densities of cluster and daughter nuclei to obtain the optical potential, it's application is also examined to study the exotic cluster radioactive decays, and results obtained found comparable with the successfully used M3Y phenomenological effective NN interactions. The presently derived NN-interaction can also be used to calculate a number of other nuclear observables.Comment: 4 Pages 2 Figure

Temperate macroalgae impacts tropical fish recruitment at forefronts of range expansion

Warming waters and changing ocean currents are increasing the supply of tropical fish larvae to temperature regions where they are exposed to novel habitats, namely temperate macroalgae and barren reefs. Here, we use underwater surveys on the temperate reefs of southeastern (SE) Australia and western Japan (~33.5°N and S, respectively) to investigate how temperate macroalgal and non-macroalgal habitats influence recruitment success of a range of tropical fishes. We show that temperate macroalgae strongly affected recruitment of many tropical fish species in both regions and across three recruitment seasons in SE Australia. Densities and richness of recruiting tropical fishes, primarily planktivores and herbivores, were over seven times greater in non macroalgal than macroalgal reef habitat. Species and trophic diversity (K-dominance) were also greater in non macroalgal habitat. Temperate macroalgal cover was a stronger predictor of tropical fish assemblages than temperate fish assemblages, reef rugosities or wave exposure. Tropical fish richness, diversity and density were greater on barren reef than on reef dominated by turfing algae. One common species, the neon damselfish (Pomacentrus coelestis), chose nonmacroalgal habitat over temperate macroalgae for settlement in an aquarium experiment. This study highlights that temperate macroalgae may partly account for spatial variation in recruitment success of many tropical fishes into higher latitudes. Hence, habitat composition of temperate reefs may need to be considered to accurately predict the geographic responses of many tropical fishes to climate change

Constraining Primordial Non-Gaussianity with High-Redshift Probes

We present an analysis of the constraints on the amplitude of primordial non-Gaussianity of local type described by the dimensionless parameter $f_{\rm NL}$. These constraints are set by the auto-correlation functions (ACFs) of two large scale structure probes, the radio sources from NRAO VLA Sky Survey (NVSS) and the quasar catalogue of Sloan Digital Sky Survey Release Six (SDSS DR6 QSOs), as well as by their cross-correlation functions (CCFs) with the cosmic microwave background (CMB) temperature map (Integrated Sachs-Wolfe effect). Several systematic effects that may affect the observational estimates of the ACFs and of the CCFs are investigated and conservatively accounted for. Our approach exploits the large-scale scale-dependence of the non-Gaussian halo bias. The derived constraints on {$f_{\rm NL}$} coming from the NVSS CCF and from the QSO ACF and CCF are weaker than those previously obtained from the NVSS ACF, but still consistent with them. Finally, we obtain the constraints on $f_{\rm NL}=53\pm25$ ($1\,\sigma$) and $f_{\rm NL}=58\pm24$ ($1\,\sigma$) from NVSS data and SDSS DR6 QSO data, respectively.Comment: 16 pages, 8 figures, 1 table, Accepted for publication on JCA

Strong Decays of Strange Quarkonia

In this paper we evaluate strong decay amplitudes and partial widths of strange mesons (strangeonia and kaonia) in the 3P0 decay model. We give numerical results for all energetically allowed open-flavor two-body decay modes of all nsbar and ssbar strange mesons in the 1S, 2S, 3S, 1P, 2P, 1D and 1F multiplets, comprising strong decays of a total of 43 resonances into 525 two-body modes, with 891 numerically evaluated amplitudes. This set of resonances includes all strange qqbar states with allowed strong decays expected in the quark model up to ca. 2.2 GeV. We use standard nonrelativistic quark model SHO wavefunctions to evaluate these amplitudes, and quote numerical results for all amplitudes present in each decay mode. We also discuss the status of the associated experimental candidates, and note which states and decay modes would be especially interesting for future experimental study at hadronic, e+e- and photoproduction facilities. These results should also be useful in distinguishing conventional quark model mesons from exotica such as glueballs and hybrids through their strong decays.Comment: 69 pages, 5 figures, 39 table

Melting of Charge/Orbital Ordered States in Nd1/2_{1/2}Sr1/2_{1/2}MnO3_3: Temperature and Magnetic Field Dependent Optical Studies

We investigated the temperature ($T=$ 15 $\sim$ 290 K) and the magnetic field ($H=$ 0 $\sim$ 17 T) dependent optical conductivity spectra of a charge/orbital ordered manganite, Nd$_{1/2}$Sr$_{1/2}$MnO$_3$. With variation of $T$ and $H$, large spectral weight changes were observed up to 4.0 eV. These spectral weight changes could be explained using the polaron picture. Interestingly, our results suggested that some local ordered state might remain above the charge ordering temperature, and that the charge/orbital melted state at a high magnetic field (i.e. at $H=$ 17 T and $$ 4.2 K) should be a three dimensional ferromagnetic metal. We also investigated the first order phase transition from the charge/orbital ordered state to ferromagnetic metallic state using the $T$- and $H$% -dependent dielectric constants $\epsilon_1$. In the charge/orbital ordered insulating state, $\epsilon_1$ was positive and $d\epsilon_1/d\omega \approx 0$. With increasing $T$ and $H$, $\epsilon_1$ was increased up to the insulator-metal phase boundaries. And then, $\epsilon_1$ abruptly changed into negative and $d\epsilon_1/d\omega >0$, which was consistent with typical responses of a metal. Through the analysis of $\epsilon_1$ using an effective medium approximation, we found that the melting of charge/orbital ordered states should occur through the percolation of ferromagnetic metal domains.Comment: submitted to Phys. Rev.

The Pandora's Box of Evidence Synthesis and the case for a living Evidence Synthesis Taxonomy.

Have we, as an evidence-based health community, opened the Pandora's box of evidence synthesis? There now exists a plethora of overlapping evidence synthesis approaches and duplicate, redundant and poor-quality reviews.1-4 After years of advocating for the need for systematic reviews of the evidence, there is a risk that this message been disseminated too widely and has been misinterpreted in this process. We have reached a point where in some fields more reviews exist than clinical trials, where same topic reviews are being conducted in parallel, and evidence syntheses possess limited utility for decision-making because of their poor quality or poor reporting.To paraphrase the late Douglas Altman,5 it is possible we are now at a stage where we need less reviews, better reviews and reviews done for the right reason - as opposed to the current state of mass production (approximately 80 reviews per day)6.Zachary Munn, Danielle Pollock, Timothy Hugh Barker, Jennifer Stone, Cindy Stern, Edoardo Aromataris, Holger J Schünemann, Barbara Clyne, Hanan Khalil, Reem A Mustafa, Christina Godfrey, Andrew Booth, Andrea C Tricco, Alan Pearso

Search for composite and exotic fermions at LEP 2

A search for unstable heavy fermions with the DELPHI detector at LEP is reported. Sequential and non-canonical leptons, as well as excited leptons and quarks, are considered. The data analysed correspond to an integrated luminosity of about 48 pb^{-1} at an e^+e^- centre-of-mass energy of 183 GeV and about 20 pb^{-1} equally shared between the centre-of-mass energies of 172 GeV and 161 GeV. The search for pair-produced new leptons establishes 95% confidence level mass limits in the region between 70 GeV/c^2 and 90 GeV/c^2, depending on the channel. The search for singly produced excited leptons and quarks establishes upper limits on the ratio of the coupling of the excited fermio