Developing Methodology for the Creation of Clinical Practice Guidelines for Rare Diseases : A Report from RARE-Bestpractices

Date of Acceptance: 29/05/2015 The research leading to these results has received funding from the (FP7/2007–2013), under grant agreement n 305690 (RARE-Bestpractices project). The opinions, presented here reflect only the authors’ views. The European Union is not liable for any, use that may be made of the information contained herein.Peer reviewedPublisher PD

How rare are diffusive rare events?

We study the time until first occurrence, the first-passage time, of rare density fluctuations in diffusive systems. We approach the problem using a model consisting of many independent random walkers on a lattice. The existence of spatial correlations makes this problem analytically intractable. However, for a mean-field approximation in which the walkers can jump anywhere in the system, we obtain a simple asymptotic form for the mean first-passage time to have a given number k of particles at a distinguished site. We show numerically, and argue heuristically, that for large enough k, the mean-field results give a good approximation for first-passage times for systems with nearest-neighbour dynamics, especially for two and higher spatial dimensions. Finally, we show how the results change when density fluctuations anywhere in the system, rather than at a specific distinguished site, are considered.Comment: 6 pages, 5 figures. Accepted for publication in Europhysics Letters (http://www.iop.org/EJ/journal/EPL

Rare earth - no case for government intervention

China has officially restricted exports of rare earth for several years and announced this year it will further tighten exports. Rare earth is a group of 17 different metals, usually found clustered together. These metals have hundreds of different industry applications. For example, they are used in certain high capacity magnets, batteries and lasers. As the rare earth elements are used in sectors that are assumed to have an over-proportionate growth potential (eg. green-technology), policy makers are paying particular attention to them. In the policy debate, the different elements are considered jointly, as they are typically clustered together. But each element has its own supply and demand characteristics. Consequently, prices for the individual elements might differ by factor 20 (in mid 2010, Samarium was quoted at $32 per kilogram while Terbium was quoted at$600). As China is currently the predominant producer of rare earth (>95% of total production), the reduction of Chinese rare earth exports will have effects on global supplies. Thus, rare earth elements have been very actively covered in the media and various G20 policy makers (secretary of state Clinton, chancellor Merkel, etc.) have expressed their concern. Business interest groups want to put rare earth on the official G20 agenda â?? regardless, the issue will be unofficially discussed in Seoul. But should policy makers really care? This depends on what China wants to achieve by restricting rare earth exports. Chinese export restrictions for rare earth could be interpreted in five different ways: (1) China wants to save rare earth resources for future generations. As rare earth elements are abundant in both China and the rest of the world, this is a rather unlikely explanation. But even if easily produced sources are rare, a slow exploration for fear of overexploitation should not merit political concern, as the Chinese interest of a stable, long-term supply would be aligned with the global interest. (2) China uses rare earth to exercise political influence. The current case-in-point is a supposed freeze in exports to Japan, allegedly due to political disputes. In Foreign Policy, Tim Worstall argues convincingly that rare earth elements are an unlikely tool for exercising political pressure as they cannot be effectively monopolised by China. The reason is, that rare earth can be produced in many different countries (China only holds about one third of the known resources), admittedly at higher cost. (3) China is trying to reduce the ecological impact of rare earth production. The production of rare earth in China is very environmental unfriendly and hazardous for the corresponding workforce. Consequently, Chinese export reductions might be a move to reduce the price paid by workers and the environment for growth going elsewhere. In this context, export reductions might be an important tool to gain control of production in illegal mines. Even though paying higher prices for rare earth would not be appreciated by the Western industrial consumer, correctly pricing pollution and labour are well-accepted principles in Western countries. (4) China tries to maximize the profits from exporting rare earth. As in the short-run Chinese rare earth could only be replaced by producers with significantly higher costs, China is currently able to increase its profits by restricting exports of rare earth as the decreased export volumes are offset by the increased prices. Numbers quoted in the press, arguing that in 2010 a 30% decrease in exports occurred while prices increased threefold, would be consistent with a profit maximisation strategy. Extracting monopoly rents on natural resources is common around the globe. Most oil producers for example employ duties on oil exports to ensure that the fuel is exported at prices above the production cost. Such a profit maximisation is constrained by the entry of new suppliers. If China raised the price above a certain level, profit seeking mining companies would start producing non-Chinese rare earth resources (eg. in the US or Australia). Consequently, either China keeps the price slightly below the entry threshold or a limited number of producers would secure stable supplies of rare earth at prices supposedly somewhat above the production cost of the most expensive supplier. (5) China is using export restrictions for domestic industrial policy reasons. Restricting exports lead to a de facto double pricing. Domestic prices of rare earth would drop, while foreign prices would rise. This would give domestic high-tech producers a cost advantage over their foreign competitors. This last point has been the most discussed in recent months, as it seems to be in line with Chinaâ??s mercantilist economic policy. This raises the question: Could such a strategy be successful? And would this harm Western economic interest? Subsidizing Chinese high-tech companiesâ?? by double-pricing rare earth could of course be effective in increasing these companiesâ?? world market share or profit in the short run. This does not mean, however, that such an intervention is efficient. First, higher world market prices will make other rare earth sources available. Consequently, the current cost spread between China, which is producing on a large scale, and the rest of the world that currently is not, will not persist. High prices will thereby not only drive the exploitation of new non-Chinese resources, but also encourage the development of new technologies for exploring, producing and processing rare earth (the separation of the different rare earth elements is one of the most costly parts of the production process). Consequently, market forces are likely to drive down Western production costs in the mid-term. Second, due to the export restriction, the demand for Chinese rare earth would be artificially low. This would lead to the intended lower price for rare earth. Chinese investors would make their decisions with respect to the internal price, as Chinese companies would need two commodities for exporting: the rare earth valued at the domestic price and the export rights valued at the price differential between internal and external market. Therefore, it should play no role, whether the export rights are allocated for free to some companies or auctioned off. The low Chinese rare earth price, however, would distort the incentives to invest in new resources and technologies in China. Third, in many applications, rare earth elements might be replaced. If, for example, the cost of certain rare earth magnets became too high, companies might decide to use lesser quality magnets. Thus, wind turbines will continue to be built in Western countries, even though replacing the rare earth magnets might imply some efficiency losses. This is reflected by the rather small share of the rare earth cost in the value of most total products. The total market value of separated rare earth is several billion dollars (USD), while the value of iron ore is a few dozen billions and crude oil several trillion. Thus, the price of rare earth is unlikely to be a key driver for the location of most downstream value chains. Fourth, a subsidisation of rare earth would be intended to allow lower prices at the next stage of the value chain (eg. rare earth magnets). To achieve the industry policy goal, it would be necessary, however, to also ensure that these intermediate products (magnets) are not exported in order to allow cheaper products at the subsequent stage (eg. generators) and so forth. Thus, an entire sector would be created on incorrect relative prices. In other words, it is more profitable to use a cheap machine that wastes some of the rare earth than to install a more expensive machine. The corresponding overuse of rare earth and rare earth products would make the entire value chain uncompetitive as soon as rare earth cost inside and outside China converge. Double pricing will lead to opposing effects in China and the West. In China, the underinvestment in mining and the wasteful use of rare earth will increase demand and decrease supply. In the West, the overinvestment in supply and the substitution of rare earth in some areas will decrease demand and increase supply. Consequently, markets alone will force rare earth prices in the West and China to converge. [Furthermore, double pricing is difficult to sustain as it implies internal redistribution. When double pricing is effective, the right to export becomes very valuable. In this case, the worse-off companies would oppose the scheme and might find ways to circumvent it.] Therefore, restricting the export of rare earth is unlikely to prove an efficient tool for attracting highly-skilled and capital-intensive, high-tech industries. So what do Western companies have to fear? Companies in all sectors know that depending on only one supplier of a crucial input can be very painful. Thus, companies can chose between vertical integration, contracts that reduce the risk of hold-up or diversification. The first two options are not very viable, as Western companies are unlikely to either strike renegotiation-proof deals with Chinese companies or even integrate Chinese suppliers. Thus, diversifying supplies by either contracting some volumes with alternative suppliers, building up stocks, financially hedging against increasing prices or even buying into rare earth production projects should be the answer for industrial users of rare earth. The optimal degree of diversification can only be delivered by the market. The reason is that the willingness to pay for diversification largely depends on private information about the individual companiesâ?? cost of decreasing rare earth consumption. Furthermore, the large number of consumers from different countries should assure the development of a sufficiently liquid market for rare earth. Thus, the market will provide a close-to- optimal level of production capacity and stocks. [As for most natural resource markets, there will be some concentration on the supply side due to the high optimal size of the mines leading to prices above production cost.] Therefore, government support to rare earth consumer, administrative stocks to smooth market prices or government support to increase non-Chinese production would make things worse by punishing companies that invested in risk mitigation. As double pricing will be an inefficient industrial policy and markets will deliver sufficient rare earth supplies outside China, Western governments should restrain from protectionist countermeasures or unjustified counteroffers. That being said, trading the opening of the Chinese rare earth sector against the opening of another G20 or WTO market (eg. agriculture) would be a win-win situation.

SLIDES: Recommended Best Management Practices for Plants of Concern: Practices Developed to Reduce the Impacts of Oil and Gas Development Activities to Plants of Concern

Presenter: Brian Kurzel, Colorado Natural Areas Program (CNAP) 27 slide

Rare diseases: matching wheelchair users with rare metabolic, neuromuscular or neurological disorders to electric powered indoor/outdoor wheelchairs (EPIOCs)

Purpose: To describe the clinical features of electric powered indoor/outdoor wheelchair (EPIOC) users with rare diseases (RD) impacting on EPIOC provision and seating. Method: Retrospective review by a consultant in rehabilitation medicine of electronic and case note records of EPIOC recipients with RDs attending a specialist wheelchair service between June 2007 and September 2008. Data were systematically extracted, entered into a database and analysed under three themes; demographic, diagnostic/clinical (including comorbidity and associated clinical features (ACFs) of the illness/disability) and wheelchair factors. Results: Fifty-four (27 male) EPIOC users, mean age 37.3 (SD 18.6, range 11–70) with RDs were identified and reviewed a mean of 64 (range 0–131) months after receiving their wheelchair. Diagnoses included 27 types of RDs including Friedreich’s ataxia, motor neurone disease, osteogenesis imperfecta, arthrogryposis, cerebellar syndromes and others. Nineteen users had between them 36 comorbidities and 30 users had 44 ACFs likely to influence the prescription. Tilt-in-space was provided to 34 (63%) users and specialised seating to 17 (31%). Four users had between them complex control or interfacing issues. Conclusions: The complex and diverse clinical problems of those with RDs present unique challenges to the multiprofessional wheelchair team to maintain successful independent mobility and community living

Rare earth based nanostructured materials: Synthesis, functionalization, properties and bioimaging and biosensing applications

Rare earth based nanostructures constitute a type of functional materials widely used and studied in the recent literature. The purpose of this review is to provide a general and comprehensive overview of the current state of the art, with special focus on the commonly employed synthesis methods and functionalization strategies of rare earth based nanoparticles and on their different bioimaging and biosensing applications. The luminescent (including downconversion, upconversion and permanent luminescence) and magnetic properties of rare earth based nanoparticles, as well as their ability to absorb X-rays, will also be explained and connected with their luminescent, magnetic resonance and X-ray computed tomography bioimaging applications, respectively. This review is not only restricted to nanoparticles, and recent advances reported for in other nanostructures containing rare earths, such as metal organic frameworks and lanthanide complexes conjugated with biological structures, will also be commented on.European Union 267226Ministerio de Economía y Competitividad MAT2014-54852-

Rare Decays

Studies of rare decays play an important role in the search of physics beyond the standard model. New particles may participate in the loop processes and can be probed by seeing any deviations from the standard model predictions. The very rare decay $B_s\to\mu^+\mu^-$ has been observed with the data collected by CMS and LHCb experiments. The signal seen by the ATLAS experiment is less significant but is compatible with the predictions. The measurement itself provides stringent constraints to new physics models. The first effective lifetime measurement with $B_s\to\mu^+\mu^-$ candidates has been carried out by the LHCb experiment. More data are still required to observe the $B^0\to\mu^+\mu^-$ decays. The $B\to K^*\mu^+\mu^-$ decay also proceeds through a flavour changing neutral current process, and is sensitive to the new physics. Extended measurements are carried out for $B\to K^*\mu^+\mu^-$ decays. Most of the classical physics parameters are found to be consistent with the predictions, but tensions do emerge in some of the observables. More data will help to clarify these potential deviations.Comment: 7 pages, for LHCP 2017 conferenc

A Portfolio of Carnegie Libraries : Being a Separate Issue of the Illustrations from a Book of Carnegie Libraries

This Portfolio contents illustrations and plates of Carnegie libraries.https://scholarsjunction.msstate.edu/caad-rare-books/1000/thumbnail.jp

Risk Factors for Severe Outcomes in Patients With Systemic Vasculitis and COVID-19 : A Binational, Registry-Based Cohort Study

© 2021 The Authors. Arthritis & Rheumatology published by Wiley Periodicals LLC on behalf of American College of Rheumatology.Peer reviewedPublisher PD

Rare Charm Decays

Rare FCNC decay modes of charmed mesons are reviewed. The standard model predictions, including both short and long distance contributions, are summarized. Several new physics options that can give detectable signals are described.Comment: 4 pages. Presented at the 6th International Conference on Hyperons, Charm and Beauty Hadrons, IIT, Chicago, june 27-july 3, 2004. To be published in the Proceeding