Phenylketonuria in Portugal: Genotype-Phenotype Correlations Using Molecular, Biochemical, and Haplotypic Analyses

The impairment of the hepatic enzyme phenylalanine hydroxylase (PAH) causes elevation of phenylalanine levels in blood and other body fluids resulting in the most common inborn error of amino acid metabolism (phenylketonuria). Persistently high levels of phenylalanine lead to irreversible damage to the nervous system. Therefore, early diagnosis of the affected individuals is important, as it can prevent clinical manifestations of the disease.info:eu-repo/semantics/publishedVersio

Reliability Assessment of Solder Joints in Power Electronic Modules by Crack Damage Model for Wind Turbine Applications

Wind turbine reliability is an important issue for wind energy cost minimization, especially by reduction of operation and maintenance costs for critical components and by increasing wind turbine availability. To develop an optimal operation and maintenance plan for critical components, it is necessary to understand the physics of their failure and be able to develop reliability prediction models. Such a model is proposed in this paper for an IGBT power electronic module. IGBTs are critical components in wind turbine converter systems. These are multilayered devices where layers are soldered to each other and they operate at a thermal-power cycling environment. Temperature loadings affect the reliability of soldered joints by developing cracks and fatigue processes that eventually result in failure. Based on Miner’s rule a linear damage model that incorporates a crack development and propagation processes is discussed. A statistical analysis is performed for appropriate model parameter selection. Based on the proposed model, a layout for component life prediction with crack movement is described in details

First Byurakan Spectral Survey. Late-Type Stars. Dwarfs

International audienceA total of 18 lists of the First Byurakan Survey of Late-Type Stars (FBS LTS) were published between 1990 and 2016. The stars were found on FBS low-dispersion spectral plates. A systematic search and selection were carried out on a surface of ∼16 000 deg^2 on almost the whole area of the FBS. Since 2007, all FBS low-resolution spectral plates have been digitized. The second version of the "Revised and Updated Catalogue of the First Byurakan Survey of Late-Type Stars", containing data for 1471 M and C (carbon) stars (130 C-type giants, 1105 M - giants, and 236 M dwarfs) was generated. Among the 236 M dwarfs selected, 176 are new discoveries. The Gaia EDR3 G broad-band magnitudes are in the range 11.3 < G < 17.1. New distance information by Bailer-Jones et al. (2021), which is based on the EDR3 parallaxes are used to estimate the G-band absolute magnitudes for M dwarfs. 9 FBS M dwarfs (out of 176 new discovered) lie within 25 pc of the Sun. The object FBS 0909-082 is more distant (r = 780 ̇pc) M dwarf among the sample considered, for which G-band absolute magnitude M(G) = 9.18, M = 0.59 MSun, L = 0.13597 L_sun, and T_eff = 3844 K. This object can be classified as M1-M2 sub-type dwarf. The nearest object is FBS 0250+167, a M7 subtype dwarf with very high proper motion (5.13 arcsec/yr) and is located at 3.83 pc from the Sun. The TESS estimated masses lie in the range 095 (±0.02) M_sun ≤ M ≤ 0.7 (± 0.1) M_sun and T_eff in the range 4000K < T_eff < 2790K for FBS M dwarfs. Color-absolute magnitude (CaMD) diagrams are constructed for the FBS M dwarfs based on Gaia EDR3 and TESS data

First Byurakan Spectral Survey. Late-Type Stars. Dwarfs

International audienceA total of 18 lists of the First Byurakan Survey of Late-Type Stars (FBS LTS) were published between 1990 and 2016. The stars were found on FBS low-dispersion spectral plates. A systematic search and selection were carried out on a surface of ∼16 000 deg^2 on almost the whole area of the FBS. Since 2007, all FBS low-resolution spectral plates have been digitized. The second version of the "Revised and Updated Catalogue of the First Byurakan Survey of Late-Type Stars", containing data for 1471 M and C (carbon) stars (130 C-type giants, 1105 M - giants, and 236 M dwarfs) was generated. Among the 236 M dwarfs selected, 176 are new discoveries. The Gaia EDR3 G broad-band magnitudes are in the range 11.3 < G < 17.1. New distance information by Bailer-Jones et al. (2021), which is based on the EDR3 parallaxes are used to estimate the G-band absolute magnitudes for M dwarfs. 9 FBS M dwarfs (out of 176 new discovered) lie within 25 pc of the Sun. The object FBS 0909-082 is more distant (r = 780 ̇pc) M dwarf among the sample considered, for which G-band absolute magnitude M(G) = 9.18, M = 0.59 MSun, L = 0.13597 L_sun, and T_eff = 3844 K. This object can be classified as M1-M2 sub-type dwarf. The nearest object is FBS 0250+167, a M7 subtype dwarf with very high proper motion (5.13 arcsec/yr) and is located at 3.83 pc from the Sun. The TESS estimated masses lie in the range 095 (±0.02) M_sun ≤ M ≤ 0.7 (± 0.1) M_sun and T_eff in the range 4000K < T_eff < 2790K for FBS M dwarfs. Color-absolute magnitude (CaMD) diagrams are constructed for the FBS M dwarfs based on Gaia EDR3 and TESS data

Multi-State Physics Model for the Reliability Assessment of a Component under Degradation Processes and Random Shocks

International audienceWe extend a multi-state physics model (MSPM) framework for component reliability assessment by including semi-Markov and random shock processes. Dependences between the two processes are considered. A Monte Carlo simulation algorithm is developed to compute component reliability. An example is illustrated with respect to a literature case study