Despite an increasing attention on the role of land in meeting countries' climate pledges under the Paris Agreement, the range of estimates of carbon fluxes from land use, land-use change, and ...forestry (LULUCF) in available databases is very large. A good understanding of the LULUCF data reported by countries under the United Nations Framework Convention on Climate Change (UNFCCC) - and of the differences with other datasets based on country-reported data - is crucial to increase confidence in land-based climate change mitigation efforts. Furthermore, we compare our NGHGI database with two other sets of country-based data: those included in the UNFCCC GHG data interface, and those based on forest resources data reported by countries to the Food and Agriculture Organization of the United Nations (FAO) and used as inputs into estimates of GHG emissions in FAOSTAT. The first dataset, once gap filled as in our study, results in a net global LULUCF sink of -5.4 Gt CO.sub.2 yr.sup.-1 . The difference with the NGHGI database is in this case mostly explained by more updated and comprehensive data in our compilation for non-Annex I countries. The FAOSTAT GHG dataset instead estimates a net global LULUCF source of +1.1 Gt CO.sub.2 yr.sup.-1 . In this case, most of the difference to our results is due to a much greater forest sink for non-Annex I countries in the NGHGI database than in FAOSTAT. The difference between these datasets can be mostly explained by a more complete coverage in the NGHGI database, including for non-biomass carbon pools and non-forest land uses, and by different underlying data on forest land. The latter reflects the different scopes of the country reporting to FAO, which focuses on area and biomass, and to UNFCCC, which explicitly focuses on carbon fluxes. Bearing in mind the respective strengths and weaknesses, both our NGHGI database and FAO offer a fundamental, yet incomplete, source of information on carbon-related variables for the scientific and policy communities, including under the Global stocktake.
Aims. We perform an extensive characterization of the broadband emission of Mrk 421, as well as its temporal evolution, during the non-flaring (low) state. The high brightness and nearby location (z ...= 0.031) of Mrk 421 make it an excellent laboratory to study blazar emission. The goal is to learn about the physical processes responsible for the typical emission of Mrk 421, which might also be extended to other blazars that are located farther away and hence are more difficult to study. Methods. We performed a 4.5-month multi-instrument campaign on Mrk 421 between January 2009 and June 2009, which included VLBA, F-GAMMA, GASP-WEBT, Swift, RXTE, Fermi-LAT, MAGIC, and Whipple, among other instruments and collaborations. This extensive radio to very-high-energy (VHE; E> 100 GeV) γ-ray dataset provides excellent temporal and energy coverage, which allows detailed studies of the evolution of the broadband spectral energy distribution. Results. Mrk421 was found in its typical (non-flaring) activity state, with a VHE flux of about half that of the Crab Nebula, yet the light curves show significant variability at all wavelengths, the highest variability being in the X-rays. We determined the power spectral densities (PSD) at most wavelengths and found that all PSDs can be described by power-laws without a break, and with indices consistent with pink/red-noise behavior. We observed a harder-when-brighter behavior in the X-ray spectra and measured a positive correlation between VHE and X-ray fluxes with zero time lag. Such characteristics have been reported many times during flaring activity, but here they are reported for the first time in the non-flaring state. We also observed an overall anti-correlation between optical/UV and X-rays extending over the duration of the campaign. Conclusions. The harder-when-brighter behavior in the X-ray spectra and the measured positive X-ray/VHE correlation during the 2009 multi-wavelength campaign suggests that the physical processes dominating the emission during non-flaring states have similarities with those occurring during flaring activity. In particular, this observation supports leptonic scenarios as being responsible for the emission of Mrk 421 during non-flaring activity. Such a temporally extended X-ray/VHE correlation is not driven by any single flaring event, and hence is difficult to explain within the standard hadronic scenarios. The highest variability is observed in the X-ray band, which, within the one-zone synchrotron self-Compton scenario, indicates that the electron energy distribution is most variable at the highest energies.
The autoinflammatory diseases Federici, Silvia; Caorsi, Roberta; Gattorno, Marco
Swiss medical weekly,
2012, Letnik:
142
Journal Article
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The monogenic autoinflammatory syndromes are conditions caused by mutations of genes coding for proteins that play a pivotal role in the regulation of the inflammatory response. Due to their genetic ...nature, most of these disorders have an early onset. Clinically they are characterised by recurrent flares of systemic inflammation presenting most of the time as sudden fever episodes associated with elevation of acute phase reactants and with a number of clinical manifestations such as rash, serositis, lymphadenopathy and arthritis. Symptom-free intervals are characterised by complete wellbeing, normal growth and complete normalisation of acute phase reactants. Familial Mediterranean fever (FMF), mevalonate-kinase deficiency (MKD) and tumour necrosis factor (TNF) receptor-associated periodic syndrome (TRAPS) are the three monogenic disorders subsumed under the term periodic fevers, while a systemic inflammation dominated by a characteristic urticarial rash associated with a number of other clinical manifestations is typical of familial cold autoinflammatory syndrome (FCAS), Muckle-Wells syndrome (MWS) and chronic infantile neurological cutaneous and articular syndrome (CINCA). These diseases represent the clinical spectrum of different mutations of a gene named cold-induced autoinflammatory syndrome 1 (CIAS-1, or NLRP3) coding for a protein called cryopyrin. Hence these disorders are also known as cryopyrin-associated periodic syndromes (CAPS). Other conditions are characterised by typical granulomatous formations (granulomatous disorders). Blau's syndrome (familial juvenile systemic granulomatosis) presents with non-caseating granulomatous inflammation affecting the joint, skin, and uveal tract (the triad of arthritis, dermatitis and uveitis) and is associated with mutations of the NACHT domain of the gene CARD15 (or NOD2).
Thin-film transistors (TFTs), which use zinc oxide (ZnO) as an active layer, were fabricated and investigated in detail. The transport properties of ZnO deposited by spray pyrolysis (SP) on a TFT ...structure are studied in a wide range of temperatures, electrical conditions (i.e., subthreshold, above-threshold linear, and saturation regions), and at different channel lengths. It is shown that ZnO deposited by SP is a nanocrystalline material; its field-effect mobility is temperature activated and increases with carrier concentration. On the basis of this analysis, we propose the multiple-trapping-and-release (MTR)-transport mechanism to describe the charge transport in ZnO. By means of numerical simulations, we prove that MTR is a suitable approach, and we calculate the density of states. We show that the tail states extend in a wide range of energy and that they strongly influence the transport properties. Finally, an analytical physical-based DC model is proposed and validated with experiments and numerical simulations. The model is able to reproduce the measurements on devices with different channel length in a wide range of bias voltages and temperatures by means of a restricted number of parameters, which are linked directly to the physical properties of the ZnO semiconductor. For the first time, the charge transport in the ZnO is investigated by means of the MTR, and a consistent analysis based on experiments, numerical simulations, and analytical modeling is provided.
Pulmonary blastoma is a rare malignant tumor seen in both adults and children. Approximately only 25% of cases occur in pediatric patients, many of whom affected by a congenital pulmonary cystic ...lesion. The clinical features, radiological findings and management of a 3-year-old boy affected by a pulmonary blastoma which arose in a congenital cystic adenomatoid malformation are reported, and an extensive review of the literature is also made. Because of the well-known tendency of cystic pulmonary diseases to develop malignancies, authors recommend the surgical excision of these kind of lesion or at least their close radiological follow-up.
Ground-based gamma-ray astronomy has had a major breakthrough with the impressive results obtained using systems of imaging atmospheric Cherenkov telescopes. Ground-based gamma-ray astronomy has a ...huge potential in astrophysics, particle physics and cosmology. CTA is an international initiative to build the next generation instrument, with a factor of 5–10 improvement in sensitivity in the 100 GeV–10 TeV range and the extension to energies well below 100 GeV and above 100 TeV. CTA will consist of two arrays (one in the north, one in the south) for full sky coverage and will be operated as open observatory. The design of CTA is based on currently available technology. This document reports on the status and presents the major design concepts of CTA.
Objective
To evaluate the efficacy and safety of treatment with the interleukin‐1 receptor antagonist anakinra in patients with tumor necrosis factor receptor–associated periodic syndrome (TRAPS) ...requiring high cumulative doses of steroids.
Methods
Four children (mean age 9.1 years range 4–13 years) and 1 adult (age 33 years) with TRAPS were enrolled in the study. The 3 children with cysteine mutations (C52Y, C55Y, C43R) had prolonged and frequent attacks of fever. One child with the R92Q mutation and the adult patient with the C43R mutation displayed a more chronic disease course, with fluctuating, nearly continuous symptoms and persistent elevation of acute‐phase reactant levels (including serum amyloid A SAA). All patients were treated with anakinra (1.5 mg/kg/day).
Results
All of the patients had a prompt response to anakinra, with disappearance of symptoms and normalization of acute‐phase reactant levels, including SAA. In all pediatric patients, anakinra was withdrawn after 15 days of treatment. After a few days (mean 5.6 days range 3–8) a disease relapse occurred, which dramatically responded to reintroduction of anakinra. During the following period of observation (mean 11.4 months range 4–20 months), the patients did not experience episodes of fever or other disease‐related clinical manifestations. Levels of acute‐phase reactants remained in the normal range. No major adverse reactions or severe infections were observed.
Conclusion
Continuous treatment with anakinra effectively controlled both the clinical and laboratory manifestations in patients with TRAPS and prevented disease relapses.
The high-frequency-peaked BL Lacertae object 1ES 0229+200 is a relatively distant (z = 0.1396), hard-spectrum (Gamma ~ 2.5), very-high-energy (VHE; E > 100 GeV) emitting gamma -ray blazar. VHE ...measurements of this active galactic nucleus have been used to place constraints on the intensity of the extragalactic background light and the intergalactic magnetic field (IGMF). A multi-wavelength study of this object centered around VHE observations by Very Energetic Radiation Imaging Telescope Array System (VERITAS) is presented. This study obtained, over a period of three years, an 11.7 standard deviation detection and an average integral flux F(E > 300 GeV) = (23.3 + or - 2.8 sub(stat) + or - 5.8 sub(sys)) x 10 super(-9) photons m super(-2) s super(-1), or 1.7% of the Crab Nebula's flux (assuming the Crab Nebula spectrum measured by H.E.S.S). Supporting observations from Swift and RXTE are analyzed. The Swift observations are combined with previously published Fermi observations and the VHE measurements to produce an overall spectral energy distribution which is then modeled assuming one-zone synchrotron-self-Compton emission. The chi super(2) probability of the TeV flux being constant is 1.6%. This, when considered in combination with measured variability in the X-ray band, and the demonstrated variability of many TeV blazars, suggests that the use of blazars such as 1ES 0229+200 for IGMF studies may not be straightforward and challenges models that attribute hard TeV spectra to secondary gamma -ray production along the line of sight.
RAPID TeV GAMMA-RAY FLARING OF BL LACERTAE Arlen, T; Aune, T; BEILICKE, M ...
Astrophysical journal/The Astrophysical journal,
01/2013, Letnik:
762, Številka:
2
Journal Article
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We report on the detection of a very rapid TeV gamma-ray flare from BL Lacertae on 2011 June 28 with the Very Energetic Radiation Imaging Telescope Array System (VERITAS). The flaring activity was ...observed during a 34.6 minute exposure, when the integral flux above 200 GeV reached (3.4 + or - 0.6) x 10 super(-6) photons m super(-2) s super(-1), roughly 125% of the Crab Nebula flux measured by VERITAS. The light curve indicates that the observations missed the rising phase of the flare but covered a significant portion of the decaying phase. The exponential decay time was determined to be 13 + or - 4 minutes, making it one of the most rapid gamma-ray flares seen from a TeV blazar. The gamma-ray spectrum of BL Lacertae during the flare was soft, with a photon index of 3.6 + or - 0.4, which is in agreement with the measurement made previously by MAGIC in a lower flaring state. Contemporaneous radio observations of the source with the Very Long Baseline Array revealed the emergence of a new, superluminal component from the core around the time of the TeV gamma-ray flare, accompanied by changes in the optical polarization angle. Changes in flux also appear to have occurred at optical, UV, and GeV gamma-ray wavelengths at the time of the flare, although they are difficult to quantify precisely due to sparse coverage. A strong flare was seen at radio wavelengths roughly four months later, which might be related to the gamma-ray flaring activities. We discuss the implications of these multiwavelength results.
A common paradigm when the reduction of emissions from deforestations is estimated for the purpose of promoting it as a mitigation option in the context of the United Nations Framework Convention on ...Climate Change (UNFCCC) is that high uncertainties in input data—i.e., area change and C stock change/area—may seriously undermine the credibility of the estimates and therefore of reduced deforestation as a mitigation option. In this paper, we show how a series of concepts and methodological tools—already existing in UNFCCC decisions and IPCC guidance documents—may greatly help to deal with the uncertainties of the estimates of reduced emissions from deforestation.