Growth of children with Langerhans cell histiocytosis

Conclusion: GH deficiency is not a common manifestation of LCH in childhood and GH provocation tests are only indicated when there is a poor or decelerating growth rate. In our patients the number of organs involved and/or the treatment modality did not influence the growth in all but one. Diseases in childhood have an impact on growth. The influence of Langerhans cell histiocytosis (LCH) on growth has never been studied well. Recently a patient with LCH was treated with human growth hormone (GH) because of severe GH deficiency due to LCH involvement of both the hypothalamus and pituitary. This led us to review our charts from 1971 onward for evaluation of the growth patterns in patients with LCH. Here the long-term growth of 22 patients with LCH is reported, the median follow up being 7 years and 1 month. The height data were converted into standard deviation scores (SDS). At diagnosis the mean SDS of patients with isolated LCH at diagnosis was 0.04 and -0.37 in patients with disseminated LCH. Of the total group, 12 patients did not show any influence from the LCH or therapy on their growth. The remaining 10 patients reached, after a minimum of 3 years, a percentile clearly higher than that at diagnosis. However all the ten above mentioned patients, either isolated or disseminated LCH, had a lesion in the facial side of the skull

The prognosis of oncologic patients in the pediatric intensive care unit

Objective: To evaluate the predicted mortality rate of oncologic patients in the PICU using the PRISM score and factors that might influence short-term outcomes. Design: Retrospective study. Setting: Pedriatic ICU in a university hospital. Patients and Methods: The medical charts of all oncologic patients admitted to the PICU during the period from January 1983 to December 1992 were reviewed. Main Results: Over a period of 10 years, 51 oncologic patients were admitted on 57 occasions to the PICU. The mortality was 32%. This is significantly higher than the overall mortality in the PICU (8%). Comparison of observed and predicted mortality, derived from the PRISM score, using chi square goodness-of-fit tests showed a significantly higher observed mortality (χ2(5) = 20.1, P < 0.01). Patients admitted for circulatory failure had the highest mortality (47%), followed by those with respiratory failure due to tachypnea/cyanosis (36%), central nervous system deterioration (27%), respiratory failure due to airway obstruction (25%), and metabolic disorders (20%). Of the 31 patients who needed mechanical ventilation, 17 died (55%), and when they needed inotropic support as well, the mortality increased to 69%. The mortality rose to 100% when the patient was admitted with a septic shock, necessitating mechanical ventilation and inotropic support. The median PRISM score was 5 in the survivor group and 18.5 in the non-survivor group; this difference was found to be significant using the Wilcoxon test (P = 0.01). However, some patients with high scores were found in the survivor group, as well as some with low scores in the non-survivor group. Conclusion: The decision to treat oncologic patients in a PICU remains difficult and has to be considered on an individual basis. However, oncologic patients do benefit from admission to the PICU. The PRISM score is not suitable for oncologic patients in the PICU, because it underestimates the observed mortality. Other factors like neutropenia, septic shock, the need for mechanical ventilation, and inotropic support should be taken into consideration

Atmospheric transport and chemistry of trace gases in LMDz5B: evaluation and implications for inverse modelling

Representation of atmospheric transport is a major source of error in the estimation of greenhouse gas sources and sinks by inverse modelling. Here we assess the impact on trace gas mole fractions of the new physical parameterizations recently implemented in the atmospheric global climate model LMDz to improve vertical diffusion, mesoscale mixing by thermal plumes in the planetary boundary layer (PBL), and deep convection in the troposphere. At the same time, the horizontal and vertical resolution of the model used in the inverse system has been increased. The aim of this paper is to evaluate the impact of these developments on the representation of trace gas transport and chemistry, and to anticipate the implications for inversions of greenhouse gas emissions using such an updated model. Comparison of a one-dimensional version of LMDz with large eddy simulations shows that the thermal scheme simulates shallow convective tracer transport in the PBL over land very efficiently, and much better than previous versions of the model. This result is confirmed in three-dimensional simulations, by a much improved reproduction of the radon-222 diurnal cycle. However, the enhanced dynamics of tracer concentrations induces a stronger sensitivity of the new LMDz configuration to external meteorological forcings. At larger scales, the inter-hemispheric exchange is slightly slower when using the new version of the model, bringing them closer to observations. The increase in the vertical resolution (from 19 to 39 layers) significantly improves the representation of stratosphere/troposphere exchange. Furthermore, changes in atmospheric thermodynamic variables, such as temperature, due to changes in the PBL mixing modify chemical reaction rates, which perturb chemical equilibriums of reactive trace gases. One implication of LMDz model developments for future inversions of greenhouse gas emissions is the ability of the updated system to assimilate a larger amount of high-frequency data sampled at high-variability stations. Others implications are discussed at the end of the paper

A view of the European carbon flux landscape through the lens of the ICOS atmospheric observation network

The ICOS (Integrated Carbon Observation System) network of atmospheric measurement stations produces standardized data on greenhouse gas concentrations at 46 stations in 16 different European countries (March 2023). The placement of instruments on tall towers and mountains results in large influence regions (“concentration footprints”). The combined footprints for all the individual stations create a “lens” through which the network sees the European CO2 flux landscape. In this study, we summarize this view using quantitative metrics of the fluxes seen by individual stations and by the current and extended ICOS networks. Results are presented from both country level and pan-European perspectives, using open-source tools that we make available through the ICOS Carbon Portal. We target anthropogenic emissions from various sectors, as well as the land cover types found across Europe and their spatiotemporally varying fluxes. This recognizes different interests of different ICOS stakeholders. We specifically introduce “monitoring potential maps” to identify which regions have a relative underrepresentation of biospheric fluxes. This potential changes with the introduction of new stations, which we investigate for the planned ICOS expansion with 19 stations over the next few years. In our study focused on the summer of 2020, we find that the ICOS atmospheric station network has limited sensitivity to anthropogenic fluxes, as was intended in the current design. Its representation of biospheric fluxes follows the fractional representation of land cover and is generally well balanced considering the pan-European view. Exceptions include representation of grass and shrubland and broadleaf forest which are abundant in south-eastern European countries, particularly Croatia and Serbia. On the country scale, the representation shows larger imbalances, even within relatively densely monitored countries. The flexibility to consider individual ecosystems, countries, or their integrals across Europe demonstrates the usefulness of our analyses and can readily be reproduced for any network configuration within Europe.</p

Inverse modelling of European CH4 emissions during 2006-2012 using different inverse models and reassessed atmospheric observations

We present inverse modelling (top down) estimates of European methane (CH4) emissions for 2006-2012 based on a new quality-controlled and harmonised in situ data set from 18 European atmospheric monitoring stations. We applied an ensemble of seven inverse models and performed four inversion experiments, investigating the impact of different sets of stations and the use of a priori information on emissions.The inverse models infer total CH4 emissions of 26.8 (20.2-29.7) TgCH(4) yr(-1) (mean, 10th and 90th percentiles from all inversions) for the EU-28 for 2006-2012 from the four inversion experiments. For comparison, total anthropogenic CH4 emissions reported to UNFCCC (bottom up, based on statistical data and emissions factors) amount to only 21.3 TgCH(4) yr(-1) (2006) to 18.8 TgCH(4) yr(-1) (2012). A potential explanation for the higher range of top-down estimates compared to bottom-up inventories could be the contribution from natural sources, such as peatlands, wetlands, and wet soils. Based on seven different wetland inventories from the Wetland and Wetland CH4 Inter-comparison of Models Project (WETCHIMP), total wetland emissions of 4.3 (2.3-8.2) TgCH(4) yr(-1) from the EU-28 are estimated. The hypothesis of significant natural emissions is supported by the finding that several inverse models yield significant seasonal cycles of derived CH4 emissions with maxima in summer, while anthropogenic CH4 emissions are assumed to have much lower seasonal variability. Taking into account the wetland emissions from the WETCHIMP ensemble, the top-down estimates are broadly consistent with the sum of anthropogenic and natural bottom-up inventories. However, the contribution of natural sources and their regional distribution remain rather uncertain.Furthermore, we investigate potential biases in the inverse models by comparison with regular aircraft profiles at four European sites and with vertical profiles obtained during the Infrastructure for Measurement of the European Carbon Cycle (IMECC) aircraft campaign. We present a novel approach to estimate the biases in the derived emissions, based on the comparison of simulated and measured enhancements of CH4 compared to the background, integrated over the entire boundary layer and over the lower troposphere. The estimated average regional biases range between -40 and 20% at the aircraft profile sites in France, Hungary and Poland.</p

TOPBP1 missense variant Arg309Cys and breast cancer in a German hospital-based case-control study

The DNA double strand break repair gene TOPBP1 has been suggested as a breast cancer susceptibility gene and a missense variant Arg309Cys was observed at elevated frequency in familial breast cancer cases compared to healthy controls from Finland. We found the Arg309Cys allele at a 13% carrier frequency in a hospital-based series of 1064 German breast cancer patients and at a 14% carrier frequency in 1014 population controls (OR 0.89, 95%CI 0.69-1.15; p = 0.4). Arg309Cys carriers were not enriched among patients with a family history of breast cancer (OR = 0.87, 95%CI 0.53-1.43, p = 0.6) and were slightly underrepresented in patients with bilateral disease (OR = 0.49, 95%CI = 0.24-0.99; p = 0.047). In the latter group, the mean age at diagnosis was 62 years in carriers and 54 years in non-carriers (p = 0.004). We conclude that there is no evidence for the TOPBP1*Arg309Cys variant to confer an increased risk for breast cancer in the German population

First Measurement of the Transverse Spin Asymmetries of the Deuteron in Semi-Inclusive Deep Inelastic Scattering

First measurements of the Collins and Sivers asymmetries of charged hadrons produced in deep-inelastic scattering of muons on a transversely polarized 6-LiD target are presented. The data were taken in 2002 with the COMPASS spectrometer using the muon beam of the CERN SPS at 160 GeV/c. The Collins asymmetry turns out to be compatible with zero, as does the measured Sivers asymmetry within the present statistical errors.Comment: 6 pages, 2 figure

High-E_T dijet photoproduction at HERA

The cross section for high-E_T dijet production in photoproduction has been measured with the ZEUS detector at HERA using an integrated luminosity of 81.8 pb-1. The events were required to have a virtuality of the incoming photon, Q^2, of less than 1 GeV^2 and a photon-proton centre-of-mass energy in the range 142 < W < 293 GeV. Events were selected if at least two jets satisfied the transverse-energy requirements of E_T(jet1) > 20 GeV and E_T(jet2) > 15 GeV and pseudorapidity requirements of -1 < eta(jet1,2) < 3, with at least one of the jets satisfying -1 < eta(jet) < 2.5. The measurements show sensitivity to the parton distributions in the photon and proton and effects beyond next-to-leading order in QCD. Hence these data can be used to constrain further the parton densities in the proton and photon.Comment: 36 pages, 13 figures, 20 tables, including minor revisions from referees. Accepted by Phys. Rev.