Hereditary complement factor I deficiency

Summary We describe four cases (from three families) of hereditary factor I deficiency, bringing the total number of cases now reported to 23. In one family there are two affected siblings: one has suffered recurrent pyogenic infections; the other is asymptomatic. In the second family, the patient had recurrent pyogenic infections and a self-limiting vasculitic illness; in the third family, the patient suffered recurrent pyogenic and neisserial infections. All four patients had markedly reduced concentrations of C3 in the serum (family 1 propositus: 28%; family 1 asymptomatic sibling: 15%; family 2: 31%; and family 3: 31 % normal human serum) which was in the form of C3b. Low lgG2 levels may occur in primary C3 deficiency, and reduction in lgG2 concentration to 1.14 g/l (normal: 1.30-5.90 g/l) was found in the patient from family 2. Using radioligand binding assays, we demonstrated increased binding of C3b to erythrocytes in a patient with factor I deficiency. This C3b could not be cleaved by autologous serum but could be cleaved by normal serum or purified factor I. We review and compare the published cases of C3, factor H and factor I deficienc

Maritime aerosol network as a component of AERONET - First results and comparison with global aerosol models and satellite retrievals

The Maritime Aerosol Network (MAN) has been collecting data over the oceans since November 2006. Over 80 cruises were completed through early 2010 with deployments continuing. Measurement areas included various parts of the Atlantic Ocean, the Northern and Southern Pacific Ocean, the South Indian Ocean, the Southern Ocean, the Arctic Ocean and inland seas. MAN deploys Microtops hand-held sunphotometers and utilizes a calibration procedure and data processing traceable to AERONET. Data collection included areas that previously had no aerosol optical depth (AOD) coverage at all, particularly vast areas of the Southern Ocean. The MAN data archive provides a valuable resource for aerosol studies in maritime environments. In the current paper we present results of AOD measurements over the oceans, and make a comparison with satellite AOD retrievals and model simulations

A Genome‐Wide Association Study Meta‐Analysis of Alpha Angle Suggests Cam‐Type Morphology May Be a Specific Feature of Hip Osteoarthritis in Older Adults

Objective: To examine the genetic architecture of cam morphology using alpha angle (AA) as a proxy measure and conduct an AA genome‐wide association study (GWAS) followed by Mendelian randomization (MR) to evaluate its causal relationship with hip osteoarthritis (OA). Methods: Observational analyses examined associations between AA measurements derived from hip dual x‐ray absorptiometry (DXA) scans from the UK Biobank study and radiographic hip OA outcomes and subsequent total hip replacement. Following these analyses, an AA GWAS meta‐analysis was performed (N = 44,214) using AA measurements previously derived in the Rotterdam Study. Linkage disequilibrium score regression assessed the genetic correlation between AA and hip OA. Genetic associations considered significant (P < 5 × 10−8) were used as AA genetic instrument for 2‐sample MR analysis. Results: DXA‐derived AA showed expected associations between AA and radiographic hip OA (adjusted odds ratio [OR] 1.63 [95% confidence interval (95% CI) 1.58, 1.67]) and between AA and total hip replacement (adjusted hazard ratio 1.45 [95% CI 1.33, 1.59]) in the UK Biobank study cohort. The heritability of AA was 10%, and AA had a moderate genetic correlation with hip OA (rg = 0.26 [95% CI 0.10, 0.43]). Eight independent genetic signals were associated with AA. Two‐sample MR provided weak evidence of causal effects of AA on hip OA risk (inverse variance weighted OR 1.84 [95% CI 1.14, 2.96], P = 0.01). In contrast, genetic predisposition for hip OA had stronger evidence of a causal effect on increased AA (inverse variance weighted β = 0.09 [95% CI 0.04, 0.13], P = 4.58 × 10−5). Conclusion: Expected observational associations between AA and related clinical outcomes provided face validity for the DXA‐derived AA measurements. Evidence of bidirectional associations between AA and hip OA, particularly for risk of hip OA on AA, suggests that hip shape modeling secondary to a genetic predisposition to hip OA contributes to the well‐established relationship between hip OA and cam morphology in older adults

The Atacama Cosmology Telescope: A Catalog of >4000 Sunyaev–Zel’dovich Galaxy Clusters

We present a catalog of 4195 optically confirmed Sunyaev–Zel'dovich (SZ) selected galaxy clusters detected with signal-to-noise ratio >4 in 13,211 deg2 of sky surveyed by the Atacama Cosmology Telescope (ACT). Cluster candidates were selected by applying a multifrequency matched filter to 98 and 150 GHz maps constructed from ACT observations obtained from 2008 to 2018 and confirmed using deep, wide-area optical surveys. The clusters span the redshift range 0.04 1 clusters, and a total of 868 systems are new discoveries. Assuming an SZ signal versus mass-scaling relation calibrated from X-ray observations, the sample has a 90% completeness mass limit of M500c > 3.8 × 1014 M⊙, evaluated at z = 0.5, for clusters detected at signal-to-noise ratio >5 in maps filtered at an angular scale of 2farcm4. The survey has a large overlap with deep optical weak-lensing surveys that are being used to calibrate the SZ signal mass-scaling relation, such as the Dark Energy Survey (4566 deg2), the Hyper Suprime-Cam Subaru Strategic Program (469 deg2), and the Kilo Degree Survey (825 deg2). We highlight some noteworthy objects in the sample, including potentially projected systems, clusters with strong lensing features, clusters with active central galaxies or star formation, and systems of multiple clusters that may be physically associated. The cluster catalog will be a useful resource for future cosmological analyses and studying the evolution of the intracluster medium and galaxies in massive clusters over the past 10 Gyr

Use of SMS texts for facilitating access to online alcohol interventions: a feasibility study

A41 Use of SMS texts for facilitating access to online alcohol interventions: a feasibility study In: Addiction Science & Clinical Practice 2017, 12(Suppl 1): A4

A joint Fermi-GBM and Swift-BAT analysis of gravitational-wave candidates from the third gravitational-wave observing run

We present Fermi Gamma-ray Burst Monitor (Fermi-GBM) and Swift Burst Alert Telescope (Swift-BAT) searches for gamma-ray/X-ray counterparts to gravitational-wave (GW) candidate events identified during the third observing run of the Advanced LIGO and Advanced Virgo detectors. Using Fermi-GBM onboard triggers and subthreshold gamma-ray burst (GRB) candidates found in the Fermi-GBM ground analyses, the Targeted Search and the Untargeted Search, we investigate whether there are any coincident GRBs associated with the GWs. We also search the Swift-BAT rate data around the GW times to determine whether a GRB counterpart is present. No counterparts are found. Using both the Fermi-GBM Targeted Search and the Swift-BAT search, we calculate flux upper limits and present joint upper limits on the gamma-ray luminosity of each GW. Given these limits, we constrain theoretical models for the emission of gamma rays from binary black hole mergers

Electron Flow in Cytochrome c3 Mutant of Desulfovibrio desulfuricans G20

Small-scale (100 kW-5 MW) on-site distributed generation (DG) economically driven by combined heat and power (CHP) applications and, in some cases, reliability concerns will likely emerge as a common feature of commercial building energy systems over the next two decades. Forecasts of DG adoption published by the Energy Information Administration (EIA) in the Annual Energy Outlook (AEO) are made using the National Energy Modeling System (NEMS), which has a forecasting module that predicts the penetration of several possible commercial building DG technologies over the period 2005-2025. NEMS is also used for estimating the future benefits of Department of Energy research and development used in support of budget requests and management decisionmaking. The NEMS approach to modeling DG has some limitations, including constraints on the amount of DG allowed for retrofits to existing buildings and a small number of possible sizes for each DG technology. An alternative approach called Commercial Sector Model (ComSeM) is developed to improve the way in which DG adoption is modeled. The approach incorporates load shapes for specific end uses in specific building types in specific regions, e.g., cooling in hospitals in Atlanta or space heating in Chicago offices. The Distributed Energy Resources Customer Adoption Model (DER-CAM) uses these load profiles together with input cost and performance DG technology assumptions to model the potential DG adoption for four selected cities and two sizes of five building types in selected forecast years to 2022. The Distributed Energy Resources Market Diffusion Model (DER-MaDiM) is then used to then tailor the DER-CAM results to adoption projections for the entire U.S. commercial sector for all forecast years from 2007-2025. This process is conducted such that the structure of results are consistent with the structure of NEMS, and can be re-injected into NEMS that can then be used to integrate adoption results into a full forecast

Electron Flow in Cytochrome c3 Mutant of Desulfovibrio desulfuricans G20

Small-scale (100 kW-5 MW) on-site distributed generation (DG) economically driven by combined heat and power (CHP) applications and, in some cases, reliability concerns will likely emerge as a common feature of commercial building energy systems over the next two decades. Forecasts of DG adoption published by the Energy Information Administration (EIA) in the Annual Energy Outlook (AEO) are made using the National Energy Modeling System (NEMS), which has a forecasting module that predicts the penetration of several possible commercial building DG technologies over the period 2005-2025. NEMS is also used for estimating the future benefits of Department of Energy research and development used in support of budget requests and management decisionmaking. The NEMS approach to modeling DG has some limitations, including constraints on the amount of DG allowed for retrofits to existing buildings and a small number of possible sizes for each DG technology. An alternative approach called Commercial Sector Model (ComSeM) is developed to improve the way in which DG adoption is modeled. The approach incorporates load shapes for specific end uses in specific building types in specific regions, e.g., cooling in hospitals in Atlanta or space heating in Chicago offices. The Distributed Energy Resources Customer Adoption Model (DER-CAM) uses these load profiles together with input cost and performance DG technology assumptions to model the potential DG adoption for four selected cities and two sizes of five building types in selected forecast years to 2022. The Distributed Energy Resources Market Diffusion Model (DER-MaDiM) is then used to then tailor the DER-CAM results to adoption projections for the entire U.S. commercial sector for all forecast years from 2007-2025. This process is conducted such that the structure of results are consistent with the structure of NEMS, and can be re-injected into NEMS that can then be used to integrate adoption results into a full forecast