An equation of state from cool-dense fluids to hot gases for mixed elements

An equation of state for the domain extending from hot gases to cool-dense fluids is formulated for a hydrogen-helium mixture. The physical processes take account of temperature ionization and dissociation, electron degeneracy, Coulomb coupling and pressure ionization. Pressure ionization and Coulomb coupling are studied with simple and comprehensive modeling. A single and complete algorithm is achieved with explicit expressions available for the whole domain from hot gases to cool dense fluids ($T>10^2$). Pressure ionization and Coulomb coupling have been examined for their contributions to the pressure and internal energy. The result reveals that their contributions smooth the variation of the pressure and internal energy in the region of pressure ionization even at very low temperatures.Comment: 10 pages, 8 figures, ApJ, accepted, E-mail: [email protected]

Screening for Fabry disease and Hereditary ATTR amyloidosis in idiopathic small-fiber and mixed neuropathy

Abstract Introduction: In this study we assessed the value of genetic screening for Fabry disease (FD) and hereditary ATTR amyloidosis in patients with idiopathic small‐fiber neuropathy (SFN) or mixed neuropathy in a clinical setting. Methods: This was a Nordic multicenter study with 9 participating centers. Patients with idiopathic SFN or mixed neuropathy were included. Genetic sequencing of the TTR and GLA genes was performed. Results: There were 172 patients enrolled in the study. Genetic screening was performed in 155 patients. No pathogenic mutations in the TTR gene were found. A single patient had a possible pathogenic variant, R118C, in the GLA gene, but clinical investigation showed no firm signs of FD. Discussion: Screening for hereditary ATTR amyloidosis and FD in patients with idiopathic SFN or mixed neuropathy without any additional disease‐specific symptoms or clinical characteristics in a Nordic population appears to be of little value in a clinical setting