What metrics best reflect the energy and carbon intensity of cities? Insights from theory and modeling of 20 US cities

Three broad approaches have emerged for energy and greenhouse gas (GHG) accounting for individual cities: (a) purely in-boundary source-based accounting (IB); (b) community-wide infrastructure GHG emissions footprinting (CIF) incorporating life cycle GHGs (in-boundary plus trans-boundary) of key infrastructures providing water, energy, food, shelter, mobility-connectivity, waste management/sanitation and public amenities to support community-wide activities in cities - all resident, visitor, commercial and industrial activities; and (c) consumption-based GHG emissions footprints (CBF) incorporating life cycle GHGs associated with activities of a sub-set of the community - its final consumption sector dominated by resident households. The latter two activity-based accounts are recommended in recent GHG reporting standards, to provide production-dominated and consumption perspectives of cities, respectively. Little is known, however, on how to normalize and report the different GHG numbers that arise for the same city. We propose that CIF and IB, since they incorporate production, are best reported per unit GDP, while CBF is best reported per capita. Analysis of input-output models of 20 US cities shows that GHGCIF/GDP is well suited to represent differences in urban energy intensity features across cities, while GHGCBF/capita best represents variation in expenditures across cities. These results advance our understanding of the methods and metrics used to represent the energy and GHG performance of cities

CONVERSION OF URANIUM DIOXIDE TO URANIUM CARBIDE IN AN INDUCTION PLASMA TORCH REACTOR.

Exploratory studies were conducted to determine the feasibility of using an induction plasma torch reactor for converting uranium dioxide-graphite powder agglomerates to uranium carbide spheres

On the tail decay of M/G/1-type Markov renewal processes

The tail decay of M/G/1-type Markov renewal processes is studied. The Markov renewal process is transformed into a Markov chain so that the problem of tail decay is reformulated in terms of the decay of the coefficients of a suitable power series. The latter problem is reduced to analyze the analyticity domain of the power series

The Role of Networks, Mentors, and the Law in Overcoming Barriers to Organizational Leadership for Women with Children

The 2012 election brought headlines such as Another \u27Year of Women\u27 in Congress. Although the number of women in the highest legislative offices increased, their numbers are still significantly lower than those of men. Fewer than 100 women hold office in both houses of Congress. Corporate America similarly reflects significantly low female leadership numbers. For example, fewer than 20% of finance industry directors and executives are women, and [there are] no women leading the 20 biggest U.S. banks and securities firms. Women make up nearly half the workforce and hold 60% of bachelor degrees, yet they hold only 14% of senior executive positions at Fortune 500 companies and 40% of managerial positions overall. These figures have persisted unchanged since 2005. Subtle yet entrenched forms of gender discrimination have clearly stalled women\u27s career progress, and more must be done to rectify these disparities. The differences in gender outcome may be a function of demand-side (work-related) and supply-side (worker-related) characteristics. These include personal characteristics, human and social capital, and developmental, interpersonal, and situational factors. Gender overlaps with multiple group memberships based on family status, race, religion, national origin, and disability. These group memberships also influence women\u27s status and power dynamics in the family, the workplace, and in other communities. The interplay of these factors makes gender-based discrimination an interesting phenomenon to explore. Having dependents is an important family status variable in the larger scheme of social differentiators that account for sex differences in careerrelated outcomes. In this study, we are interested in whether men or women benefit more from having access to networks when they have dependents. Prior studies have shown that mentoring and networking are major components of professional development that lead to career advancement. We are also interested in whether the outcome differs for those men and women who report having mentors

Stopping Light All-Optically

We show that light pulses can be stopped and stored all-optically, with a process that involves an adiabatic and reversible pulse bandwidth compression occurring entirely in the optical domain. Such a process overcomes the fundamental bandwidth-delay constraint in optics, and can generate arbitrarily small group velocities for light pulses with a given bandwidth, without the use of any coherent or resonant light-matter interactions. We exhibit this process in optical resonator systems, where the pulse bandwidth compression is accomplished only by small refractive index modulations performed at moderate speeds. (Accepted for publication in Phys. Rev. Lett. Submitted on Sept. 10th 2003)Comment: 18 pages including 3 figures. Accepted for publication in Phys. Rev. Let

Effects of Baseline Left Ventricular Hypertrophy and Decreased Renal Function on Cardiovascular and Renal Outcomes in Patients with Fabry Disease Treated with Agalsidase Alfa: A Fabry Outcome Survey Study

PURPOSE: The initiation of enzyme-replacement therapy prior to the occurrence of substantial and irreversible organ damage in patients with Fabry disease is of critical importance. The Fabry Outcome Survey is an international disease registry of patients with a confirmed diagnosis of Fabry disease. In this study, data from the Fabry Outcome Survey were used for the assessment of the risks for cardiovascular and renal events in patients who received agalsidase alfa treatment. METHODS: Eligible patients were males and females aged ≥18 years with Fabry disease treated with agalsidase alfa. Cardiovascular events included myocardial infarction, left ventricular hypertrophy (LVH), heart failure, arrhythmia, conduction abnormality, and cardiac surgery. Renal events included dialysis, transplantation, and renal failure. Kaplan-Meier curves and log-rank tests were used for comparing event-free probabilities and time to first cardiovascular or renal event, from agalsidase alfa initiation to a maximum of 120 months, in patients with LVH versus normal left ventricular mass index (LVMI; ≤50 g/m2.7 in males and ≤48 g/m2.7 in females) at treatment initiation (baseline), and in patients with a low estimated glomerular filtration rate (eGFR; <90 mL/min/1.73 m2) versus normal eGFR at baseline. Multivariate Cox regression analysis was used for examining the association between key study variables and the risks for cardiovascular and renal events. FINDINGS: Among the 560 patients (269 males; 291 females) with available LVMI data, 306 (55%) had LVH and 254 (45%) had normal LVMI at baseline. The risk for a cardiovascular event was higher in the subgroup with LVH versus normal LVMI at baseline (hazard ratio [HR] = 1.57; 95% CI, 1.21-2.05; P < 0.001), but the risk for a renal event was similar between the 2 subgroups (HR = 1.90; 95% CI, 0.94-3.85; P = 0.074). Among the 1093 patients (551 males; 542 females) with available eGFR data, 433 (40%) had a low eGFR and 660 (60%) had a normal eGFR at baseline. The subgroup with a low eGFR at baseline had a significantly higher risk for a cardiovascular event (HR = 1.33; 95% CI, 1.04-1.70; P = 0.021) or a renal event (HR = 5.88; 95% CI, 2.73-12.68; P < 0.001) compared with patients with a normal eGFR at baseline. IMPLICATIONS: In the present study, the presence of LVH and/or reduced renal function at agalsidase alfa initiation was associated with a significantly higher risk for a cardiovascular or renal event, indicating that cardiovascular and renal pathologies in Fabry disease may be inter-related. Early initiation of agalsidase alfa treatment prior to the onset of severe organ damage may improve outcomes. ClinicalTrials.gov identifier: NCT03289065

Characterization of early disease status in treatment-naive male paediatric patients with Fabry disease enrolled in a randomized clinical trial.

Trial designThis analysis characterizes the degree of early organ involvement in a cohort of oligo-symptomatic untreated young patients with Fabry disease enrolled in an ongoing randomized, open-label, parallel-group, phase 3B clinical trial.MethodsMales aged 5-18 years with complete α-galactosidase A deficiency, without symptoms of major organ damage, were enrolled in a phase 3B trial evaluating two doses of agalsidase beta. Baseline disease characteristics of 31 eligible patients (median age 12 years) were studied, including cellular globotriaosylceramide (GL-3) accumulation in skin (n = 31) and kidney biopsy (n = 6; median age 15 years; range 13-17 years), renal function, and glycolipid levels (plasma, urine).ResultsPlasma and urinary GL-3 levels were abnormal in 25 of 30 and 31 of 31 patients, respectively. Plasma lyso-GL-3 was elevated in all patients. GL-3 accumulation was documented in superficial skin capillary endothelial cells (23/31 patients) and deep vessel endothelial cells (23/29 patients). The mean glomerular filtration rate (GFR), measured by plasma disappearance of iohexol, was 118.1 mL/min/1.73 m(2) (range 90.4-161.0 mL/min/1.73 m(2)) and the median urinary albumin/creatinine ratio was 10 mg/g (range 4.0-27.0 mg/g). On electron microscopy, renal biopsy revealed GL-3 accumulation in all glomerular cell types (podocytes and parietal, endothelial, and mesangial cells), as well as in peritubular capillary and non-capillary endothelial, interstitial, vascular smooth muscle, and distal tubules/collecting duct cells. Lesions indicative of early Fabry arteriopathy and segmental effacement of podocyte foot processes were found in all 6 patients.ConclusionsThese data reveal that in this small cohort of children with Fabry disease, histological evidence of GL-3 accumulation, and cellular and vascular injury are present in renal tissues at very early stages of the disease, and are noted before onset of microalbuminuria and development of clinically significant renal events (e.g. reduced GFR). These data give additional support to the consideration of early initiation of enzyme replacement therapy, potentially improving long-term outcome.Trial registrationClinicalTrials.gov NCT00701415

Management of pain in Fabry disease in the UK clinical setting: consensus findings from an expert Delphi panel

Background: Fabry disease is a rare, X-linked inherited lysosomal storage disorder, that manifests as a heterogeneous disease with renal, cardiac and nervous system involvement. The most common pain experienced by people with Fabry disease are episodes of neuropathic pain reported in up to 80% of classical hemizygous male patients and up to 65% of heterozygous female patients. No clear consensus exists within UK clinical practice for the assessment and management of pain in Fabry disease based on agreed clinical practice and clinical experience. Here we describe a modified Delphi initiative to establish expert consensus on management of pain in Fabry disease in the UK clinical setting. Methods: Delphi panel members were identified based on their demonstrated expertise in managing adult or paediatric patients with Fabry disease in the UK and recruited by an independent third-party administrator. Ten expert panellists agreed to participate in two survey rounds, during which they remained anonymous to each other. Circulation of the questionnaires, and collection and processing of the panel’s responses were conducted between September 2021 and December 2021. All questions required an answer. Results: The Delphi panel reached a consensus on 21 out of 41 aspects of pain assessment and management of pain in Fabry disease. These encompassed steps in the care pathway from the goals of therapy through to holistic support, including the use of gabapentin and carbamazepine as first-line analgesic medications for the treatment of neuropathic pain in Fabry disease, as well as the proactive management of symptoms of anxiety and/or depression associated with Fabry pain. Conclusions: The consensus panel outcomes reported here have highlighted strengths in current UK clinical practice, along with unmet needs for further research and agreement. This consensus is intended to prompt the next steps towards developing clinical guidelines

Co-existence of Phenylketonuria and Fabry disease on a 3 year-old boy: case report

Background: The co-existence of two genetically distinct metabolic disorders in the same patient has rarely been reported. Phenylketonuria (PKU) is an inborn error of the metabolism resulting from a phenylalanine hydroxylase defi ciency. Fabry disease (FD) is an X-linked lysosomal storage disorder due to a defi ciency of the enzyme alpha-galactosidase A. Case presentation: We report a case of a 3-year-old boy affected by classic PKU and FD, both confi rmed by molecular data. The FD was suspected at the age of 21 months on the presence of non-specifi c GI symptoms (severe abdominal pain and periodically appearance of not specifi c episodes of gastroenteritis) apparently non related to PKU. Conclusion: This is the fi rst report of co-existence of FD and PKU, two different congenital inborn of metabolism and in consideration of the prevalence of each disease this chance association is a very unusual event. The co-existence of these diseases made very diffi cult the correct interpretation of clinical symptoms as lack of appetite, severe abdominal pain and non-specifi c gastroenteritis episodes. Furthermore, this case report helps to defi ne the early clinical phenotype of FD