Miracle-dz/UniFAR-FTUs huggingface dataset:
A Novel Presentation of Proopiomelanocortin ( POMC ) Deficiency

Proopiomelanocortin (POMC) deficiency is a rare form of monogenic obesity which typically presents in infancy with hypoglycemic seizures and reduced cutaneous pigmentation. Over time, these patients develop morbid obesity along with multiple endocrinopathies including adrenal insufficiency, hypogonadism, hypothyroidism and growth hormone deficiency. Here, we describe an 11 year old male with adrenal insufficiency, seizure disorder, morbid obesity, and intellectual disability of unknown etiology recently confirmed to have POMC deficiency based on whole-exome sequencing (WES). This case highlights the advances in molecular genetic testing and opportunities for diagnosis in patients with complex medical histories or atypical clinical manifestations. Cohen L*, Feuer A and Bergstrom K Department of Pediatrics, Weill Cornell Medical College, USA Cohen L, et al. Annals of Clinical Case Reports Pediatrics Remedy Publications LLC. , | http://anncaserep.com/ 2016 | Volume 1 | Article 1021 2 weaned after recovery from his acute illness. At 7 months of age, physiologic dosing of hydrocortisone was initiated after cosyntropin stimulation testing revealed undetectable baseline cortisol and ACTH levels and a low stimulated cortisol level of 1.5ug/dL. The results of his endocrine testing are listed in Table 1. The patient was noted to have rapid weight gain between 8 to 12 months and his weight was >99th percentile by 21 months of age. He continued to have exponential weight gain despite dietary modification and increased physical activity. He was started on levothyroxine supplementation at 18 months of age for persistently elevated thyroid stimulating hormone (TSH) levels in the context of negative thyroid antibodies. He has profound developmental delay and autism and continues to have seizures twice monthly despite antiepileptic medications. There has been no reported hypoglycemia during any of his seizures to date. His linear growth rate has been appropriate throughout childhood. He was recently referred to our genetics clinic for a new consultation by the neurologist for genetic testing related to his seizures and autism. On exam, his height, weight and body mass index were: 151.9 cm (72%, Z = 0.58), 68.6 kg (99%, Z = 2.25), and 29.23 Kg/m2 (99%, Z = 2.25) respectively. He was a well-appearing, non-dysmorphic male with tan skin and dark brown hair. He was in early puberty with Tanner II pubic hair and 4cc testes bilaterally. He had marked acanthosis nigricans of the posterior neck, axillae and groin. Molecular genetic studies were obtained with informed consent from the patient’s mother. Genomic DNA was extracted from peripheral blood samples.
HIV/AIDS Risk for Young Female Petty Traders at Car Parks

Though the HVI/AIDS prevalence rate in The Gambia is one of the lowest in the sub-region, the disease and its associated effects has always been a concern both to the government, non-governmental organizations, professionals, families, communities, development partners, etc. Over the decade a number of studies have been conducted in the areas of its mode of transmission, prevention, impacts, etc. However, there is none on the disease and the mobile population.
Network Resource Management Optimization for SDN based on Statistical Approach

Software-Defined Networking (SDN) is a new networking scheme in network technologies in which the data plane and network plane are separated. It can be considered as an umbrella including numerous sorts of network topologies, such as linear, minimal, tree, datacenter topologies and several others, proposed principally to solve problems of diverse nature of applications and different demands for resource management and performance. Where traditional networks would utilize a specific machine, for example, a firewall or load balancer, a Software-Defined Networking conveys an application that uses the controller (like OpenDaylight, Floodlight, OVS, etc.) to oversee information plane conduct. SDN can be implemented on Mininet emulator or NS3 simulator, which are both Linux basis but can also be implemented on other operating systems.
Spontaneous Wave Generation at Strongly Strained Density Fronts

AbstractA simple analytical model is presented describing the spontaneous generation of inertia–gravity waves at density fronts subjected to strong horizontal strain rates. The model considers fronts of arbitrary horizontal and vertical structure in a semi-infinite domain, with a single boundary at the ocean surface. Waves are generated because of the acceleration of the steady uniform strain flow around the density front, analogous to the generation of lee waves via flow over a topographic ridge. Significant wave generation only occurs for sufficiently strong strain rates α > 0.2f and sharp fronts H/L > 0.5f/N, where f is the Coriolis parameter, N is the stratification, and H and L are the height and width scales of the front, respectively. The frequencies of the generated waves are entirely determined by the strain rate. The lowest-frequency wave predicted to be generated via this mechanism has a Lagrangian frequency ω = 1.93f as measured in a reference frame moving with the background strain flow. The .
Impact of Instant Controlled Pressure Drop (DIC) Treatment on Drying Kinetics and Caffeine Extraction from Green Coffee Beans

The present work is directed towards the impacts of Detente InstantaneeControleeDIC (French, for instant controlled pressure-drop) in terms of decaffeination and drying of Ethiopian green coffee beans (GCBs). DICconsisted in subjecting the product to a high-pressure saturated steam during some seconds and ended with an abrupt pressure drop towards a vacuum. A conventional aqueous extraction and a hot air-drying took place after DIC treatment. Inthis study, Response Surface Method (RSM) was used withDIC saturated steam pressure P, thermal treatment time t, and initial moisture content W asthe independent variables. Both direct DICextract recovered from the vacuum tank and the aqueous extracts wereanalyzed and quantified using the reversed phase-HPLC. With decaffeination ratiosas dependent variables, P and Wwere the most significant operating parameters; whilet was much weaker.Total decaffeination ratio could reach 99.5% after DIC treatment at specificconditions of W=11.00% db, P=0.1 MPa, and t=35swhile it was only 58% when achieved with untreated raw material. The effective diffusivity Deff and the starting accessibility δWs were calculated from the diffusion/surface interaction kinetic model of hotair drying after DIC treatment. They dramatically increased with P and t while W had a weak impact.Thus, at the optimized DICconditions, and increased from 0.33 to 12.60 10 -10 m²
The Static and Dynamic Transfer-Matrix Methods in the Analysis of Distributed-Feedback Lasers

Numerical simulation plays a decisive role in the design of modern optical communication components. However, time efficient and user-friendly numerical techniques that perform simulations in that area are not easily available. In this chapter, an example concerning the use of a numerical simulation method, designated by transfer-matrix-method (TMM), is presented. Although the TMM is a numerical simulation tool especially adequate for the design of distributed feedback (DFB) laser structures in high bit rate optical communication systems (OCS), it represents a paradigmatic example of a numerical method related to heavy computational times. Nowadays, distributed-feedback lasers are indispensable in high-bit rate OCS (Bornholdt et al., 2008; Sato et al., 2005; Tang et al., 2006; Utake et al., 2009; Wedding & Pöhlmann, 2004; Wedding et al., 2003), where they should present single-longitudinal mode (SLM) operation over the largest range of current injection. In order to assure sufficient intensity modulation bandwidth in high-bit rate systems, the current injected into the laser cavity can assume high values, (Sato et al., 2005; Wedding & Pöhlmann, 2004; Wedding et al., 2003). On the other hand, to fulfill the SLM condition, high mode selectivity and almost uniform intracavity field distributions are demanded. In the context of OCS, DFB lasers with a 90o phase discontinuity near the centre of the cavity1 are commonly cited in the specialized literature, due to their high mode selectivity, small current bias and zero frequency detuning at threshold condition (Ghafouri-Shiraz, 2003). Their main drawback is related to the high non-uniformity of the field distribution along the cavity, which presents a strong peak near the centre. Above-threshold, this non-uniformity induces important variations of the carrier and refractive-index distributions arising from the spatial hole-burning (SHB) effect, with serious consequences in the laser behavior in the high power regime, namely: increased linewidth (Ghafouri-Shiraz, 2003), multi-mode emission (Morthier, 1997) and less flat laser frequency modulation response (Agrawall & Dutta, 1986). Therefore, a careful and suitable design of the laser emitter, with a rigorous assessment of the electric field distribution along the laser cavity, is crucial in order to reduce the impact of SHB in OCS. DFB structure analysis can be performed assuming the classical solution of the wave propagation inside periodic structures. The grating of the cavity is responsible for a
Reaction-diffusion on random spatial networks with scale-free jumping rates via effective medium theory

We study epidemic processes using a metapopulation approach on the line featuring random transport rates between arbitrarily distant sites. An average transport network is found using a recently developed variant of the effective medium approximation (EMA) that is capable of dealing with these long-range connections. Using a Feynman-Kac argument in the effective medium, we derive an estimate on the size of the infected domain, and reproduce the known result of its exponential growth in time. We hereby demonstrate the applicability of long-range EMA to dynamical processes on networks more intricate than simple diffusion.
How long is a vocal tract? Comparison of acoustic impedance spectrometry with magnetic resonance imaging

The acoustic impedance spectrum of the vocal tract, measured during phonation (Hanna et al., 2016. JASA, 139, 2924–2936), shows qualitative and semi-quantitative similarity to that of a simple cylindrical duct of length l. But how does l compare with geometrical length(s) measured from a magnetic resonance imaging (MRI) scan. One male subject (age 34, height 184 cm) (1) performed a neutral /з/ vowel during impedance measurement; (2) was MRI scanned for the same gesture with his lips around a plastic tube with the same dimensions as the impedance head; and (3) was scanned during closed mouth nasal breathing. For the neutral vowel, the effective acoustic length is a weakly increasing function of frequency: the first four acoustic tract resonances give lengths rising from 155 to 195 mm; i.e. the higher resonances occur at slightly lower frequencies than the expected 1:3:5:7 ratios for cylindrical geometry. One factor is that the tract cross-section is on average greater near the lips than near the glottis. I.
TeV cosmic-ray proton and helium spectra in the myriad model II

Recent observations show that the spectra of cosmic ray nuclei start to harden above ∼ 102 GeV, which contradicts the conventional steady-state cosmic ray model. We had suggested that this anomaly is due to the propagation effect of cosmic rays released from local young cosmic ray sources; the total flux of cosmic rays should be computed with the Myriad Model, where a contribution from sources in the local catalog is added to the background. However, although the hardening could be elegantly explained in this model, the model parameters obtained from the fit are skewed toward a region with fast diffusion and a low supernova rate in the Galaxy, in disagreement with other observations. We further explore this model in order to set up a concordant picture. Two possible improvements related to cosmic ray sources are considered. First, instead of the usual axisymmetric disk model, we examine a spiral model for the source distribution. Second, for nearby and young sources which are necessary to explain the hardening, we allow for an energy-dependent escape. We find that a major improvement comes from incorporating an energy-dependent escape time for local sources, and with both modifications not only are the cosmic ray proton and helium anomalies resolved, but also the parameters attain values in a reasonable range compatible with other analyses.
ISOTOPIC RECORDS FROM EARLY WHALES AND SEA COWS: CONTRASTING PATTERNS OF ECOLOGICAL TRANSITION

Abstract Recent fossil discoveries of early cetaceans and sirenians document the functional transitions that occurred as each group adapted to a completely aquatic existence, but the timing and path of their ecological transition remain uncertain. We analyzed the stable-isotope composition of tooth enamel from several early members of each group to reconstruct the dietary, foraging, and habitat preferences of basal taxa. Carbon isotope (δ13C) values provided evidence of foraging within freshwater, terrestrial, and marine environments, including seagrass beds, whereas oxygen isotope (δ18O) variation was used as a measure of commitment to aquatic habitats. Enamel samples were collected from four regions (south Asia, north and west Africa, and southern Europe) spanning the late early Eocene (ca. 53.5 Ma) to the late Eocene (ca. 36 Ma). Sirenian and cetacean taxa included species that were likely capable of some terrestrial locomotion, and more specialized forms that were morphologically fully aquatic. Cetacean δ13C and δ18O values indicate that some early members of this group (some pakicetids) inhabited freshwater environments, but later members (e.g., remingtonocetids, protocetids, and basilosaurids) moved quickly into estuarine and marine environments. Low δ18O variation confirms that all of these early forms were primarily aquatic, but δ13C and δ18O values for early sirenians indicate an early transition to a marine seagrass diet without any evidence of an intermediate connection to freshwater habitats.
The measurement model of supply chain uncertainty and risk in the Australian courier industry

Courier is a booming industry in today's world. Courier company is one of the most significant 3PLs modules. With the development of globalisation, e-business, shorter product and technology life cycles and higher customer expectations, courier service providers face more supply chain uncertainty and risk than ever before. The supply chain uncertainty and risk has significant impacts on logistics performance in the courier service providers. Uncertainties and risks are interchangeable and inseparable. Moreover, in a real world environment, managers have to face and manage both supply chain uncertainty and risk simultaneously. The purpose of this paper is to present the results for development of an empirically validated measurement of supply chain uncertainty and risk in the Australian courier industry. The empirical data was collected in the Australian courier industry. A measurement model of structural equation modelling is used to identify the underlying factors of supply chain uncertainty and risk in the Australian courier industry. They are (1) company-side uncertainty and risk, (2) customer-side uncertainty and risk, and (3) environment uncertainty and risk. The results indicate high levels of reliability and validity of the measurement. This measurement model contributes to the supply chain uncertainty and risk literature. In addition, it provides an insight into assess supply chain uncertainty and risk in an industry.
Communicative Theories of Punishment and the Impact of Apology

The incarceration system in the United States is broken. It does not effectively—much less efficiently—serve the forward-looking goals of punishment, which rely heavily on fear of incarceration. It does not rehabilitate criminals nor prevent recidivism effectively. Evidence suggests the current system fails to deter any more than shorter sentences or better conditions would (e.g., Cullen et al. 2011). And the incarceration system does not serve the backwardslooking retributive goals of punishment effectively either, in part because, as we discuss below, most people are not satisfied with punishment involving merely impersonal incarceration or the attendant suffering of wrongdoers, except in response to the worst crimes. Rather, we want punishment not only to send a message to wrongdoers but also for the message to be received, internalized, and acted upon. The U.S. penal system is not effective at leading wrongdoers to recognize the harm they have done, to want to repair the harm, and to change so that they will not repeat such harms. Criminal behavior may indicate that the offender does not sufficiently value the community’s norms. Punishment might be most effective in achieving multiple aims if it expresses both this (backwards-looking) message about the offender’s undervaluation of the community’s norms and the (forward-looking) message that the offender must increase his valuation of those norms.
Assessing the Environmental Impact of Geothermal Power Utilization Using Isotope Ratios (C, N, S, Pb) in Moss (Rhacomitrium Lanuginosum)

Conventional geothermal power plants are sources of industrial waste including spent fluids and non-condensable gases. Increasingly spent fluids are being re-injected in to the geothermal systems reducing significantly the environmental impact of power production and sustaining pressure within the system. While efforts are afoot to sequester some of the non-condensable gases (e.g. CO2 and H2S) a significant portion of the gases are currently vented to the atmosphere. Little is known of the effects of long term exposures to low levels of hydrogen sulfide. However, it has been suggested that H2S plays a role in the decline of vegetation around the geothermal power plant in Hellisheiði. In this study we examine the stable isotopic composition of woolly fringe moss (Rhacomitrium lanuginosum) from lava-fields around geothermal power plants in south-west Iceland. A sample suite originally collected for trace element analysis was analyzed for the isotopic composition of sulfur (S), nitrogen (N), carbon (C) and lead (Pb). The isotope ratio of sulfur varies by about 16 per mil and is controlled by binary mixing, with the most probable end members being i) hydrothermal/magmatic (“mantle-derived”) sulfur and ii) marine-derived sulfur. The isotopic composition of N ( 15 N) varies from -10.3 to -6.0 (‰ PDB). The more enriched (less negative -values) samples are more variable in terms of N content and are have comparatively high Pb content. The isotopic composition of C ( 13 C) varies from -26.1 to -23.0 (‰ PDB). The more enriched samples have typically lower C content possibly reflecting addition of dust (carbon free) to the samples. The dust may contribute Pb to the moss samples. The change in discrimination for the heavy isotopes ( 15 N and 13 C) may be a result of environmental factors such as reduced precipitation or due to an increased anthropogenic influence.
Credible Commitment, Political Uncertainty or Policy Complexity? Explaining Variations in the Independence of Non-majoritarian Institutions in France

A common feature of contemporary political systems is the increasing amount of delegation from governments to non-majoritarian institutions. Governments may decide to delegate authority to such institutions for reasons relating to credible commitments, political uncertainty and policy complexity. This article focuses on Independent Administrative Authorities ( Autorites administratives independantes ) in France. We demonstrate that these institutions enjoy varying degrees of independence. We find that the degree of independence varies as a function of two factors: the need to make a credible commitment in areas subject to market opening and the complexity of policy in particular areas.
Ship emissions measurement in the Arctic by plume intercepts of the Canadian Coast Guard icebreaker Amundsen from the Polar 6 aircraft platform

Abstract. Decreasing sea ice and increasing marine navigability in northern latitudes have changed Arctic ship traffic patterns in recent years and are predicted to increase annual ship traffic in the Arctic in the future. Development of effective regulations to manage environmental impacts of shipping requires an understanding of ship emissions and atmospheric processing in the Arctic environment. As part of the summer 2014 NETCARE (Network on Climate and Aerosols) campaign, the plume dispersion and gas and particle emission factors of effluents originating from the Canadian Coast Guard icebreaker Amundsen operating near Resolute Bay, NU, Canada, were investigated. The Amundsen burned distillate fuel with 1.5 wt % sulfur. Emissions were studied via plume intercepts using the Polar 6 aircraft measurements, an analytical plume dispersion model, and using the FLEXPART-WRF Lagrangian particle dispersion model. The first plume intercept by the research aircraft was carried out on 19 July 2014 during the operation of the Amundsen in the open water. The second and third plume intercepts were carried out on 20 and 21 July 2014 when the Amundsen had reached the ice edge and operated under ice-breaking conditions. Typical of Arctic marine navigation, the engine load was low compared to cruising conditions for all of the plume intercepts. The measured species included mixing ratios of CO2, NOx, CO, SO2, particle number concentration (CN), refractory black carbon (rBC), and cloud condensation nuclei (CCN). The results were compared to similar experimental studies in mid-latitudes. Plume expansion rates (γ) were calculated using the analytical model and found to be γ = 0.75 ± 0.81, 0.93 ± 0.37, and 1.19 ± 0.39 for plumes 1, 2, and 3, respectively. These rates were smaller than prior studies conducted at mid-latitudes, likely due to polar boundary layer dynamics, including reduced turbulent mixing compared to mid-latitudes. All emission factors were in agreement with prior observations at low engine loads in mid-latitudes. Ice-breaking increased the NOx emission factor from EFNOx = 43.1 ± 15.2 to 71.6 ± 9.68 and 71.4 ± 4.14 g kg-diesel−1 for plumes 1, 2, and 3, likely due to changes in combustion temperatures. The CO emission factor was EFCO = 137 ± 120, 12.5 ± 3.70 and 8.13 ± 1.34 g kg-diesel−1 for plumes 1, 2, and 3. The rBC emission factor was EFrBC = 0.202 ± 0.052 and 0.202 ± 0.125 g kg-diesel−1 for plumes 1 and 2. The CN emission factor was reduced while ice-breaking from EFCN = 2.41 ± 0.47 to 0.45 ± 0.082 and 0.507 ± 0.037 × 1016 kg-diesel−1 for plumes 1, 2, and 3. At 0.6 % supersaturation, the CCN emission factor was comparable to observations in mid-latitudes at low engine loads with EFCCN = 3.03 ± 0.933, 1.39 ± 0.319, and 0.650 ± 0.136 × 1014 kg-diesel−1 for plumes 1, 2, and 3.
SPHERICAL AGGLOMERATION OF KETOPROFEN BY SOLVENT CHANGE METHOD

Ketoprofen, an anti-inflammatory drug, exhibits poor water solubility and flow properties. Spherical agglomerates were prepared by solvent change method. Solvent composition for spherical agglomeration was determined by constructing ternary diagram. Crystallization medium used for spherical agglomerates of ketoprofen consisted of Isopropyl alcohol (good solvent); water (poor solvent); chloroform (bridging liquid) in the ratio of 25:60:15, respectively. Spherical agglomerates were characterized by differential scanning calorimetry, Infrared spectroscopy, X-ray diffractometry and scanning electron microscopy. Micromeritic and dissolution behavior studies were carried out. Process variables such as amount of bridging liquid, stirring time and duration of stirring were optimized. Dissolution profile of the spherical agglomerates was compared with pure sample and recrystallized sample. Spherical agglomerates exhibited decreased crystallinity and improved micromeritic properties. The dissolution of the spherical agglomerates was improved compared with pure sample.