The Shadows of Future Generations

This Note addresses the twin problems of political short-termism and intergenerational equity. Although scholars have discussed these concerns extensively, few scholars have developed proposals to modify democratic institutions-particularly legislatures-to better consider posterity\u27s interests. This Note critiques one such set of proposals by several environmental ethicists for including posterity-oriented legislators in present-generation legislatures. It then proposes a system that ties the long-term outcomes of legislators\u27 policy preferences to their pension plans by creating a new commodities market that values the decisions legislators make and their effect on posterity

The origin and prevention of pandemics.

Despite the fact that most emerging diseases stem from the transmission of pathogenic agents from animals to humans, the factors that mediate this process are still ill defined. What is known, however, is that the interface between humans and animals is of paramount importance in the process. This review will discuss the importance of the human-animal interface to the disease emergence process. We also provide an overview of factors that are believed to contribute to the origin and global spread of emerging infectious diseases and offer suggestions that may serve as future prevention strategies, such as social mobilization, public health education, behavioral change, and communication strategies. Because there exists no comprehensive global surveillance system to monitor zoonotic disease emergence, the intervention measures discussed herein may prove effective temporary alternatives

Lions and Prions and Deer Demise

Background: Contagious prion diseases – scrapie of sheep and chronic wasting disease of several species in the deer family – give rise to epidemics that seem capable of compromising host population viability. Despite this prospect, the ecological consequences of prion disease epidemics in natural populations have received little consideration. Methodology/Principal Findings: Using a cohort study design, we found that prion infection dramatically lowered survival of free-ranging adult (.2-year-old) mule deer (Odocoileus hemionus): estimated average life expectancy was 5.2 additional years for uninfected deer but only 1.6 additional years for infected deer. Prion infection also increased nearly fourfold the rate of mountain lions (Puma concolor) preying on deer, suggesting that epidemics may alter predator–prey dynamics by facilitating hunting success. Despite selective predation, about one fourth of the adult deer we sampled were infected. High prevalence and low survival of infected deer provided a plausible explanation for the marked decline in this deer population since the 1980s. Conclusion: Remarkably high infection rates sustained in the face of intense predation show that even seemingly complete ecosystems may offer little resistance to the spread and persistence of contagious prion diseases. Moreover, the depression of infected populations may lead to local imbalances in food webs and nutrient cycling in ecosystems in which deer ar

Therapeutic Neonatal Hepatic Gene Therapy in Mucopolysaccharidosis VII Dogs

Dogs with mucopolysaccharidosis VII (MPS VII) were injected intravenously at 2–3 days of age with a retroviral vector (RV) expressing canine β-glucuronidase (cGUSB). Five animals received RV alone, and two dogs received hepatocyte growth factor (HGF) before RV in an attempt to increase transduction efficiency. Transduced hepatocytes expanded clonally during normal liver growth and secreted enzyme with mannose 6-phosphate. Serum GUSB activity was stable for up to 14 months at normal levels for the RV-treated dogs, and for 17 months at 67-fold normal for the HGF/RV-treated dog. GUSB activity in other organs was 1.5–60% of normal at 6 months for two RV-treated dogs, which was likely because of uptake of enzyme from blood by the mannose 6-phosphate receptor. The body weights of untreated MPS VII dogs are 50% of normal at 6 months. MPS VII dogs cannot walk or stand after 6 months, and progressively develop eye and heart disease. RV- and HGF/RV-treated MPS VII dogs achieved 87% and 84% of normal body weight, respectively. Treated animals could run at all times of evaluation for 6–17 months because of improvements in bone and joint abnormalities, and had little or no corneal clouding and no mitral valve thickening. Despite higher GUSB expression, the clinical improvements in the HGF/RV-treated dog were similar to those in the RV-treated animals. This is the first successful application of gene therapy in preventing the clinical manifestations of a lysosomal storage disease in a large animal

Stratus 9/VOCALS ninth setting of the Stratus Ocean Reference Station & VOCALS Regional Experiment

The Ocean Reference Station at 20°S, 85°W under the stratus clouds west of northern Chile is being maintained to provide ongoing climate-quality records of surface meteorology; air-sea fluxes of heat, freshwater, and momentum; and of upper ocean temperature, salinity, and velocity variability. The Stratus Ocean Reference Station (ORS Stratus) is supported by the National Oceanic and Atmospheric Administration’s (NOAA) Climate Observation Program. It is recovered and redeployed annually, with cruises that have come between October and December. During the 2008 cruise on the NOAA ship Ronald H. Brown to the ORS Stratus site, the primary activities were recovery of the Stratus 8 WHOI surface mooring that had been deployed in October 2007, deployment of a new (Stratus 9) WHOI surface mooring at that site; in-situ calibration of the buoy meteorological sensors by comparison with instrumentation put on board by staff of the NOAA Earth System Research Laboratory (ESRL); and observations of the stratus clouds and lower atmosphere by NOAA ESRL. A buoy for the Pacific tsunami warning system was also serviced in collaboration with the Hydrographic and Oceanographic Service of the Chilean Navy (SHOA). The DART (Deep-Ocean Assessment and Reporting of Tsunami) carries IMET sensors and subsurface oceanographic instruments. A DART II buoy was deployed north of the STRATUS buoy, by personnel from the National Data Buoy Center (NDBC) Argo floats and drifters were launched, and CTD casts carried out during the cruise. The ORS Stratus buoys are equipped with two Improved Meteorological (IMET) systems, which provide surface wind speed and direction, air temperature, relative humidity, barometric pressure, incoming shortwave radiation, incoming longwave radiation, precipitation rate, and sea surface temperature. Additionally, the Stratus 8 buoy received a partial CO2 detector from the Pacific Marine Environmental Laboratory (PMEL). IMET data are made available in near real time using satellite telemetry. The mooring line carries instruments to measure ocean salinity, temperature, and currents. The ESRL instrumentation used during the 2008 cruise included cloud radar, radiosonde balloons, and sensors for mean and turbulent surface meteorology. Finally, the cruise hosted a teacher participating in NOAA’s Teacher at Sea Program.Funding was provided by the National Oceanic and Atmospheric Administration under Grant No. NA17RJ1223 for the Cooperative Institute for Climate and Ocean Research (CICOR)

Case report: Cystic fibrosis with kwashiorkor: A rare presentation in the era of universal newborn screening

BackgroundUniversal newborn screening changed the way medical providers think about the presentation of cystic fibrosis (CF). Before implementation of universal screening, it was common for children with CF to present with failure to thrive, nutritional deficiencies, and recurrent infections. Now, nearly all cases of CF are diagnosed by newborn screening shortly after birth before significant symptoms develop. Therefore, providers often do not consider this illness in the setting of a normal newborn screen. Newborn screening significantly decreases the risk of complications in early childhood, yet definitive testing should be pursued if a patient with negative newborn screening presents with symptoms consistent with CF, including severe failure to thrive, metabolic alkalosis due to significant salt losses, or recurrent respiratory infections.Case presentationWe present a case of a 6-month-old infant male with kwashiorkor, severe edema, multiple vitamin deficiencies, hematemesis secondary to coagulopathy, and diffuse erythematous rash, all secondary to severe pancreatic insufficiency. His first newborn screen had an immunoreactive trypsinogen (IRT) value below the state cut-off value, so additional testing was not performed, and his growth trajectory appeared reassuring. He was ultimately diagnosed with CF by genetic testing and confirmatory sweat chloride testing, in the setting of his parents being known CF carriers and his severe presentation being clinically consistent with CF. Acutely, management with supplemental albumin, furosemide, potassium, and vitamin K was initiated to correct the presenting hypoalbuminemia, edema, and coagulopathy. Later, pancreatic enzyme supplementation and additional vitamins and minerals were added to manage ongoing deficiencies from pancreatic insufficiency. With appropriate treatment, his vitamin deficiencies and edema resolved, and his growth improved.ConclusionDue to universal newborn screening, symptomatic presentation of CF is rare and presentation with kwashiorkor is extremely rare in resource-rich communities. The diagnosis of CF was delayed in our patient because of a normal newborn screen and falsely reassuring growth, which after diagnosis was determined to be secondary to severe edematous malnutrition. This case highlights that newborn screening is a useful but imperfect tool. Clinicians should continue to have suspicion for CF in the right clinical context, even in the setting of normal newborn screen results

Non-local heat transport in Alcator C-Mod ohmic L-mode plasmas

Non-local heat transport experiments were performed in Alcator C-Mod ohmic L-mode plasmas by inducing edge cooling with laser blow-off impurity (CaF2) injection. The non-local effect, a cooling of the edge electron temperature with a rapid rise of the central electron temperature, which contradicts the assumption of 'local' transport, was observed in low collisionality linear ohmic confinement (LOC) regime plasmas. Transport analysis shows this phenomenon can be explained either by a fast drop of the core diffusivity, or the sudden appearance of a heat pinch. In high collisionality saturated ohmic confinement (SOC) regime plasmas, the thermal transport becomes 'local': the central electron temperature drops on the energy confinement time scale in response to the edge cooling. Measurements from a high resolution imaging x-ray spectrometer show that the ion temperature has a similar behaviour as the electron temperature in response to edge cooling, and that the transition density of non-locality correlates with the rotation reversal critical density. This connection may indicate the possible connection between thermal and momentum transport, which is also linked to a transition in turbulence dominance between trapped electron modes (TEMs) and ion temperature gradient (ITG) modes. Experiments with repetitive cold pulses in one discharge were also performed to allow Fourier analysis and to provide details of cold front propagation. These modulation experiments showed in LOC plasmas that the electron thermal transport is not purely diffusive, while in SOC the electron thermal transport is more diffusive like. Linear gyrokinetic simulations suggest the turbulence outside r/a = 0.75 changes from TEM dominance in LOC plasmas to ITG mode dominance in SOC plasmas.United States. Dept. of Energy (DoE Contract No DE-FC02-99ER54512)Oak Ridge Institute for Science and Education (DOE Fusion Energy Postdoctoral Research Program