By extending our previously established model, here we present a new model called "PHITS-based Analytical Radiation Model in the Atmosphere (PARMA) version 3.0," which can instantaneously estimate terrestrial cosmic ray fluxes of neutrons, protons, ions with charge up to 28 (Ni), muons, electrons, positrons, and photons nearly anytime and anywhere in the Earth's atmosphere. The model comprises numerous analytical functions with parameters whose numerical values were fitted to reproduce the results of the extensive air shower (EAS) simulation performed by Particle and Heavy Ion Transport code System (PHITS). The accuracy of the EAS simulation was well verified using various experimental data, while that of PARMA3.0 was confirmed by the high R2 values of the fit. The models to be used for estimating radiation doses due to cosmic ray exposure, cosmic ray induced ionization rates, and count rates of neutron monitors were validated by investigating their capability to reproduce those quantities measured under various conditions. PARMA3.0 is available freely and is easy to use, as implemented in an open-access software program EXcel-based Program for Calculating Atmospheric Cosmic ray Spectrum (EXPACS). Because of these features, the new version of PARMA/EXPACS can be an important tool in various research fields such as geosciences, cosmic ray physics, and radiation research.

/pdf/analytical-model-for-estimating-terrestrial-cosmic-ray-xklicd979k.pdf

Analytical Model for Estimating Terrestrial Cosmic Ray Fluxes Nearly Anytime and Anywhere in the World: Extension of PARMA/EXPACS.

Congenital disorders of glycosylation are a genetically and clinically heterogeneous group of >130 diseases caused by defects in various steps along glycan modification pathways. The vast majority of these monogenic diseases are autosomal recessive and have multi-systemic manifestations, mainly growth failure, developmental delay, facial dysmorphisms, and variable coagulation and endocrine abnormalities. Carbohydrate deficient transferrin (CDT) and protein-linked glycan analysis with mass spectrometry can diagnose some subtypes of congenital disorders of glycosylation (CDG), while many currently rely on massively parallel genomic sequencing for diagnosis. Early detection is important, as a few of these disorders are treatable. Molecular and biochemical techniques continue

/pdf/congenital-disorders-of-glycosylation-4rs4zsitvj.pdf

Congenital disorders of glycosylation.

Therapeutic approaches in Congenital Disorders of Glycosylation (CDG) involving N-linked glycosylation: an update

Congenital disorders of glycosylation (CDG) are inborn errors of metabolism due to protein and lipid hypoglycosylation. This rapidly growing family of genetic diseases comprises 103 CDG types, with a broad phenotypic diversity ranging from mild to severe poly-organ -system dysfunction. This literature review summarizes cardiac involvement, reported in 20% of CDG. CDG with cardiac involvement were divided according to the associated type of glycosylation: N-glycosylation, O-glycosylation, dolichol synthesis, glycosylphosphatidylinositol (GPI)-anchor biosynthesis, COG complex, V-ATPase complex, and other glycosylation pathways. The aim of this review was to document and interpret the incidence of heart disease in CDG patients. Heart disorders were grouped into cardiomyopathies, structural defects, and arrhythmogenic disorders. This work may contribute to improved early management of cardiac complications in CDG.

Cardiac complications of congenital disorders of glycosylation (CDG): a systematic review of the literature

Europe Direct is a service to help you find answers to your questions about the European Union (*) Certain mobile telephone operators do not allow access to 00 800 numbers or these calls may be billed. Under the terms of the Treaty establishing the European Atomic Energy Community, the Community, amongst other things, establishes uniform safety standards to protect the health of workers and of the general public against the dangers arising from ionizing radiation. The standards are approved by the Council, on a proposal from the Commission, established taking into account the opinion of the Group of Experts referred to in Article 31 of the Treaty. The most recent version of such standards is contained in Council Directive 96/29/Euratom of 13 May 1996 laying down basic safety standards for the protection of the health of workers and the general public against the dangers arising from ionizing radiation. Directive 96/29/Euratom requires that special protection measures have to be implemented aimed at providing appropriate radiological protection for air crew. Article 42 stipulates, inter alia, that the exposure of air crew needs to be assessed. In practice, the radiation exposure of aircrew is assessed with calculation methods approved by the competent authority. Various calculation codes are currently being used for aircrew dosimetry. The European Radiation Dosimetry Group e.V. (EURADOS) is very active in promoting research, development and cooperation in the field of dosimetry of ionising radiation. The EURADOS Working Group on High Energy Radiation Fields organised an exercise comparing eleven calculation codes currently being used in European countries for legal dose assessment of aircrew or in general for scientific purposes. This report summarises the results of this exercise. The report provides a very good overview of the different codes used, analyses their performance and concludes that the agreement between the codes is considered to be fully satisfactory. The European Commission Directorate General for Energy welcomed the initiative and supported the preparation of this report by hosting two meetings of the Working Group. In order to bring the report to the attention of a broader audience, it was decided to publish it in Abstract 1 ABSTRACT The aim of this report is to compare the doses and dose rates calculated by various codes assessing radiation exposure of aircraft crew due to cosmic radiation. Some of the codes are used routinely for radiation protection purposes while others are purely for scientific use. The calculations …

Comparison of codes assessing radiation exposure of aircraft crew due to galactic cosmic radiation

The problem of finding a simple, generally applicable description of worldwide measured ambient dose equivalent rates at aviation altitudes between 8 and 12 km is difficult to solve due to the large variety of functional forms and parametrisations that are possible. We present an approach that uses Bayesian statistics and Monte Carlo methods to fit mathematical models to a large set of data and to compare the different models. About 2500 data points measured in the periods 1997-1999 and 2003-2006 were used. Since the data cover wide ranges of barometric altitude, vertical cut-off rigidity and phases in the solar cycle 23, we developed functions which depend on these three variables. Whereas the dependence on the vertical cut-off rigidity is described by an exponential, the dependences on barometric altitude and solar activity may be approximated by linear functions in the ranges under consideration. Therefore, a simple Taylor expansion was used to define different models and to investigate the relevance of the different expansion coefficients. With the method presented here, it is possible to obtain probability distributions for each expansion coefficient and thus to extract reliable uncertainties even for the dose rate evaluated. The resulting function agrees well with new measurements made at fixed geographic positions and during long haul flights covering a wide range of latitudes.

http://iopscience.iop.org/article/10.1088/0952-4746/30/3/006/pdf

The ambient dose equivalent at flight altitudes: a fit to a large set of data using a Bayesian approach

Intercomparisons of dosemeters used in radiation protection are performed routinely in reference radiation fields under well-defined conditions. In the case of the radiation protection of aircrew members, such reference fields either do not exist or they can be partially simulated by accelerator-based fields. Another method is to perform simultaneous measurements on board an aircraft under constant flight conditions, i.e. at defined latitude, longitude and altitude. The intercomparison presented in this work is the second one of its kind. As reference instruments, two types of tissue-equivalent proportional counters (TEPC) were used in comparison with different silicon detector systems. The excellent agreement of the FDOScalc code, which is based on measurements made by PTB during different phases of solar cycle 23 with the TEPC measurements and the results of the preceding project "Coordinated Access to Aircraft for Transnational Environmental Research" (CAATER) even allows the in-field calibration of the Si dosemeters.

Field calibration of dosemeters used for routine measurements at flight altitudes

Heart transplantation in a child with congenital disorder of glycosylation

The Earth is permanently exposed to energetic particle radiation from outer space called galactic cosmic rays. The radiation is mainly composed of protons, alpha-particles, some electrons and few heavy nuclei. A part of the galactic cosmic rays can penetrate the Earth’s magnetic field, whereby a particle cascade is initialized by nuclear interaction with the molecules of the atmosphere. The primary cosmic ray particles as well as the produced secondary particles form a natural radiation field composed of charged and neutral particles. The composition of the radiation field depends on the geomagnetic latitude and altitude. Thus, the radiation field changes for different altitudes such as at the orbit of the International Space Station (ISS), at common cruising altitudes, and at the ground. The radiation field at ground level is dominated by the terrestrial radiation and it is not considered in this work. Due to the fact that the exposure of air crew and astronauts is permanently calculated and measured, the radiation field at cruising altitudes and onboard the ISS is well known. For altitudes above 20 km and below the ISS orbit (400 km), however, the radiation exposure is mostly unknown. In future the radiation field in these altitudes will become more important because of the emergences of space tourism and new generation supersonic aircraft. Therefore, this work focuses on the radiation exposure up to an altitude of 25 km. For this purpose a particle telescope consisting of four segmented silicon semiconductor detectors was developed. Due to the arrangement of the detectors, it is possible to separate neutral and charged particles and the belonging calculated dose rates. In this work the particle telescope has been calibrated with several heavy ions, radioactive sources and with neutrons of different energies. Furthermore, measurements onboard aircraft were conducted in order to verify the performance of the telescope in a complex natural radiation field. In order to acquire measurements up to an altitude of 25 km an unmanned balloon flight near the polar circle was conducted. Based on this measurement presented in this work, it is now possible to estimate the radiation exposure up to an altitude of 25 km.

Measurement of the Radiation Exposure for High Altitude Flights in the Polar Region

The NAVIgation DOsimetry System (NAVIDOS) comprises a complete readout system for a radiation detec- tor, an air pressure sensor and a GPS receiver. The detector system DOSTEL uses silicon detectors which make NAVI- DOS light weighted and compact in size. Therefore, NAVI- DOS is well suited to be installed on board aircraft. The cal- ibration of NAVIDOS in terms of ambient dose equivalent is done by an in-field comparison with the PTB reference in- strument DOS. We also show that the dependence of these results on the vertical cut-off rigidity can be explained by the low response of the silicon detectors for neutrons. Therefore, in-flight measurements have been performed together with the PTB reference instrument DOS. The dose rates, cal- culated using the PTB code FDOScalc, were verified with these DOS measurements. The calibration of NAVIDOS was done with FDOScalc and results in calibration factors between 3.4 in the polar and 2.4 in the equator region.

/pdf/in-field-calibration-of-the-navigation-dosimetry-system-2odkqyn6cw.pdf

In-field calibration of the Navigation Dosimetry System (NAVIDOS) during solar minimum conditions

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