On the SCA-description of the energy- and impact parameter dependence of K-shell ionization cross sections

International Nuclear Information System (INIS)

Trautmann, D.; Kauer, T.

1989-01-01

We present the application of the SCA-model to impact-parameter and energy-dependent K-shell ionization cross sections for several projectile-target combinations. Then we discuss the successes and failures of the SCA-description and investigate the additional approximations still existing in this approach. It is shown that after the introduction of a fully time-dependent perturbed electronic boundstate wave function many of the former discrepancies between experiment and theory can be resolved and in general very good agreement is obtained. (orig.)

SCaMF–RM: A Fused High-Resolution Land Cover Product of the Rocky Mountains

KAUST Repository

Rodríguez-Jeangros, Nicolás

2017-10-02

Land cover (LC) products, derived primarily from satellite spectral imagery, are essential inputs for environmental studies because LC is a critical driver of processes involved in hydrology, ecology, and climatology, among others. However, existing LC products each have different temporal and spatial resolutions and different LC classes that rarely provide the detail required by these studies. Using multiple existing LC products, we implement our Spatiotemporal Categorical Map Fusion (SCaMF) methodology over a large region of the Rocky Mountains (RM), encompassing sections of six states, to create a new LC product, SCaMF–RM. To do this, we must adapt SCaMF to address the prediction of LC in large space–time regions that present nonstationarities, and we add more flexibility in the LC classifications of the predicted product. SCaMF–RM is produced at two high spatial resolutions, 30 and 50 m, and a yearly frequency for the 30-year period 1983–2012. When multiple products are available in time, we illustrate how SCaMF–RM captures relevant information from the different LC products and improves upon flaws observed in other products. Future work needed includes an exhaustive validation not only of SCaMF–RM but also of all input LC products.

White matter damage is related to ataxia severity in SCA3.

Science.gov (United States)

Kang, J-S; Klein, J C; Baudrexel, S; Deichmann, R; Nolte, D; Hilker, R

2014-02-01

Spinocerebellar ataxia type 3 (SCA3) is the most frequent inherited cerebellar ataxia in Europe, the US and Japan, leading to disability and death through motor complications. Although the affected protein ataxin-3 is found ubiquitously in the brain, grey matter atrophy is predominant in the cerebellum and the brainstem. White matter pathology is generally less severe and thought to occur in the brainstem, spinal cord, and cerebellar white matter. Here, we investigated both grey and white matter pathology in a group of 12 SCA3 patients and matched controls. We used voxel-based morphometry for analysis of tissue loss, and tract-based spatial statistics (TBSS) on diffusion magnetic resonance imaging to investigate microstructural pathology. We analysed correlations between microstructural properties of the brain and ataxia severity, as measured by the Scale for the Assessment and Rating of Ataxia (SARA) score. SCA3 patients exhibited significant loss of both grey and white matter in the cerebellar hemispheres, brainstem including pons and in lateral thalamus. On between-group analysis, TBSS detected widespread microstructural white matter pathology in the cerebellum, brainstem, and bilaterally in thalamus and the cerebral hemispheres. Furthermore, fractional anisotropy in a white matter network comprising frontal, thalamic, brainstem and left cerebellar white matter strongly and negatively correlated with SARA ataxia scores. Tractography identified the thalamic white matter thus implicated as belonging to ventrolateral thalamus. Disruption of white matter integrity in patients suffering from SCA3 is more widespread than previously thought. Moreover, our data provide evidence that microstructural white matter changes in SCA3 are strongly related to the clinical severity of ataxia symptoms.

Spinocerebellar ataxia 36 (SCA36): «Costa da Morte ataxia».

Science.gov (United States)

Arias, M; García-Murias, M; Sobrido, M J

To describe the history of the discovery of SCA36 and review knowledge of this entity, which is currently the most prevalent hereditary ataxia in Galicia (Spain) owing to a founder effect. SCA36 is an autosomal dominant hereditary ataxia with late onset and slow progression. It presents with cerebellar ataxia, sensorineural hearing loss, and discrete motor neuron impairment (tongue atrophy with denervation, discrete pyramidal signs). SCA36 was first described in Japan (Asida River ataxia) and in Galicia(Costa da Morte ataxia). The condition is caused by a genetic mutation (intronic hexanucleotide repeat expansion) in the NOP56 gene on the short arm of chromosome 20 (20p13). Magnetic resonance image study initially shows cerebellar vermian atrophy that subsequently extends to the rest of the cerebellum and finally to the pontomedullary region of the brainstem without producing white matter lesions. Peripheral nerve conduction velocities are normal, and sensorimotor evoked potential studies show delayed conduction of stimuli to lower limbs. In patients with hearing loss, audiometric studies show a drop of >40dB in frequencies exceeding 2,500Hz. Auditory evoked potential studies may also show lack of waves I and II. Costa da Morte ataxia or SCA36 is the most prevalent SCA in the Spanish region of Galicia. Given the region's history of high rates of emigration, new cases may be diagnosed in numerous countries, especially in Latin America. Genetic studies are now available to patients and asymptomatic carriers. Since many people are at risk for this disease, we will continue our investigations aimed at elucidating the underlying pathogenic molecular mechanisms and discovering effective treatment. Copyright © 2014 Sociedad Española de Neurología. Publicado por Elsevier España, S.L.U. All rights reserved.

Wavelength and power stability measurements of the Stanford SCA/FEL

International Nuclear Information System (INIS)

van der Geer, B.; de Loos, M.J.; Conde, M.E.; Leemans, W.P.

1994-08-01

Wavelength and power stability of the Stanford infrared SCA/FEL operating with the TRW wiggler have been measured using a high-resolution spectrometer and an image dissector system. The image dissector is capable of reading the spectrum of every single micropulse at 12 MHz throughout a macropulse of up to 2 ms duration. The intrinsic wavelength and power stability of the SCA/FEL are found to be δλ/λ=0.035% and δP/P=18%. The use of a feedback control system to stabilize the wavelength, and an acousto-optic modulator for output power smoothing, improves the performance to δλ/λ=0.012% and δP/P=7%

SCaN Network Ground Station Receiver Performance for Future Service Support

Science.gov (United States)

Estabrook, Polly; Lee, Dennis; Cheng, Michael; Lau, Chi-Wung

2012-01-01

Objectives: Examine the impact of providing the newly standardized CCSDS Low Density Parity Check (LDPC) codes to the SCaN return data service on the SCaN SN and DSN ground stations receivers: SN Current Receiver: Integrated Receiver (IR). DSN Current Receiver: Downlink Telemetry and Tracking (DTT) Receiver. Early Commercial-Off-The-Shelf (COTS) prototype of the SN User Service Subsystem Component Replacement (USS CR) Narrow Band Receiver. Motivate discussion of general issues of ground station hardware design to enable simple and cheap modifications for support of future services.

Sacrificio cortoplacista adaptativo 2opt (SCA_2opt: Una heurística inspirada en el pensamiento sistémico Sacrifice short-term adaptive 2-opt (SCA_2opt: A heuristic inspired by systems thinking

Directory of Open Access Journals (Sweden)

Jorge Pérez Rave

2013-01-01

Full Text Available Se detalla el origen de dos noveles heurísticas para el TSP simétrico, inspiradas en el pensamiento sistémico: Sacrificio Cortoplacista Adaptativo 2-opt (SCA_2opt y SCA_2_opt_r. Estas surgen del análisis sistémico de la regla de decisión Vecino más cercano, identificándosele el arquetipo "Soluciones contraproducentes". El SCA se basa en que el agente viajero renuncie en un momento dado a una ciudad inmediatamente cercana, y se traslade hacia la segunda ciudad más cercana disponible. A partir de ello, se continúa con la regla del vecino más cercano. Cada que se realiza el SCA (búsqueda global se efectúa una búsqueda local 2_opt. Considerando el binomio eficacia y eficiencia, las dos heurísticas se muestran prometedoras en comparación multicriterio contra 19 metaheurísticas. Se evidencia que el pensamiento sistémico es un campo de inspiración viable para el desarrollo de métodos de optimización combinatoria; se plasman preguntas emergentes para desarrollos futuros, que permitan continuar integrando elementos de la optimización clásica con el pensamiento sistémico; áreas tradicionalmente vistas como antagónicas, pero cuyo diálogo se muestra favorable en este artículo.Outlining two novice heuristics for the symmetric TSP, inspired systemic thinking: Sacrifice short-term adaptive 2-opt (SCA2opt and SCA_2opt_r. These arise from the systemic analysis of the rule of decision, nearest neighbour, identifying the archetype "Counterproductive solutions". SCA relies on that agent traveler, renounces at any given time immediately to a nearby city, and moves towards the second nearest available city. From there, it continues with the nearest neighbour rule. Each is the SCA (global search operates a local search 2opt. Where as the binomial effectiveness and efficiency, the two new heuristics are shown promising in comparison multicriteria against 19 metaheuristics. It is evident that systemic thinking is a field of inspiration viable

Indole and synthetic derivative activate chaperone expression to reduce polyQ aggregation in SCA17 neuronal cell and slice culture models

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Kung PJ

2014-10-01

Full Text Available Pin-Jui Kung,1,* Yu-Chen Tao,1,* Ho-Chiang Hsu,1 Wan-Ling Chen,1 Te-Hsien Lin,1 Donala Janreddy,2 Ching-Fa Yao,2 Kuo-Hsuan Chang,3 Jung-Yaw Lin,1 Ming-Tsan Su,1 Chung-Hsin Wu,1 Guey-Jen Lee-Chen,1 Hsiu-Mei Hsieh-Li1 1Department of Life Science, 2Department of Chemistry, National Taiwan Normal University, Taipei, Taiwan; 3Department of Neurology, Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taipei, Taiwan *These authors contributed equally to this work Abstract: In spinocerebellar ataxia type 17 (SCA17, the expansion of a translated CAG repeat in the TATA box binding protein (TBP gene results in a long polyglutamine (polyQ tract in the TBP protein, leading to intracellular accumulation of aggregated TBP and cell death. The molecular chaperones act in preventing protein aggregation to ameliorate downstream harmful events. In this study, we used Tet-On SH-SY5Y cells with inducible SCA17 TBP/Q79-green fluorescent protein (GFP expression to test indole and synthetic derivative NC001-8 for neuroprotection. We found that indole and NC001-8 up-regulated chaperone expression to reduce polyQ aggregation in neuronal differentiated TBP/Q79 cells. The effects on promoting neurite outgrowth and on reduction of aggregation on Purkinje cells were also confirmed with cerebellar primary and slice cultures of SCA17 transgenic mice. Our results demonstrate how indole and derivative NC001-8 reduce polyQ aggregation to support their therapeutic potentials in SCA17 treatment. Keywords: spinocerebellar ataxia type 17, TATA box binding protein, polyQ aggregation, indole and derivative, therapeutics

Cross platform SCA component using C++ builder and KYLIX

International Nuclear Information System (INIS)

Nishimura, Hiroshi; Timossi, Chiris; McDonald, James L.

2003-01-01

A cross-platform component for EPICS Simple Channel Access (SCA) has been developed. EPICS client programs with GUI become portable at their C++ source-code level both on Windows and Linux by using Borland C++ Builder 6 and Kylix 3 on these platforms respectively

Haplotype Study in SCA10 Families Provides Further Evidence for a Common Ancestral Origin of the Mutation.

Science.gov (United States)

Bampi, Giovana B; Bisso-Machado, Rafael; Hünemeier, Tábita; Gheno, Tailise C; Furtado, Gabriel V; Veliz-Otani, Diego; Cornejo-Olivas, Mario; Mazzeti, Pillar; Bortolini, Maria Cátira; Jardim, Laura B; Saraiva-Pereira, Maria Luiza

2017-12-01

Spinocerebellar ataxia type 10 (SCA10) is an autosomal dominant neurodegenerative disorder characterized by progressive cerebellar ataxia and epilepsy. The disease is caused by a pentanucleotide ATTCT expansion in intron 9 of the ATXN10 gene on chromosome 22q13.3. SCA10 has shown a geographical distribution throughout America with a likely degree of Amerindian ancestry from different countries so far. Currently available data suggest that SCA10 mutation might have spread out early during the peopling of the Americas. However, the ancestral origin of SCA10 mutation remains under speculation. Samples of SCA10 patients from two Latin American countries were analysed, being 16 families from Brazil (29 patients) and 21 families from Peru (27 patients) as well as 49 healthy individuals from Indigenous Quechua population and 51 healthy Brazilian individuals. Four polymorphic markers spanning a region of 5.2 cM harbouring the ATTCT expansion were used to define the haplotypes, which were genotyped by different approaches. Our data have shown that 19-CGGC-14 shared haplotype was found in 47% of Brazilian and in 63% of Peruvian families. Frequencies from both groups are not statistically different from Quechua controls (57%), but they are statistically different from Brazilian controls (12%) (p Quechuas, 19-15-CGGC-14-10, is found in 50% of Brazilian and in 65% of Peruvian patients with SCA10. These findings bring valuable evidence that ATTCT expansion may have arisen in a Native American chromosome.

Interferon β induces clearance of mutant ataxin 7 and improves locomotion in SCA7 knock-in mice.

Science.gov (United States)

Chort, Alice; Alves, Sandro; Marinello, Martina; Dufresnois, Béatrice; Dornbierer, Jean-Gabriel; Tesson, Christelle; Latouche, Morwena; Baker, Darren P; Barkats, Martine; El Hachimi, Khalid H; Ruberg, Merle; Janer, Alexandre; Stevanin, Giovanni; Brice, Alexis; Sittler, Annie

2013-06-01

We showed previously, in a cell model of spinocerebellar ataxia 7, that interferon beta induces the expression of PML protein and the formation of PML protein nuclear bodies that degrade mutant ataxin 7, suggesting that the cytokine, used to treat multiple sclerosis, might have therapeutic value in spinocerebellar ataxia 7. We now show that interferon beta also induces PML-dependent clearance of ataxin 7 in a preclinical model, SCA7(266Q/5Q) knock-in mice, and improves motor function. Interestingly, the presence of mutant ataxin 7 in the mice induces itself the expression of endogenous interferon beta and its receptor. Immunohistological studies in brains from two patients with spinocerebellar ataxia 7 confirmed that these modifications are also caused by the disease in humans. Interferon beta, administered intraperitoneally three times a week in the knock-in mice, was internalized with its receptor in Purkinje and other cells and translocated to the nucleus. The treatment induced PML protein expression and the formation of PML protein nuclear bodies and decreased mutant ataxin 7 in neuronal intranuclear inclusions, the hallmark of the disease. No reactive gliosis or other signs of toxicity were observed in the brain or internal organs. The performance of the SCA7(266Q/5Q) knock-in mice was significantly improved on two behavioural tests sensitive to cerebellar function: the Locotronic® Test of locomotor function and the Beam Walking Test of balance, motor coordination and fine movements, which are affected in patients with spinocerebellar ataxia 7. In addition to motor dysfunction, SCA7(266Q/5Q) mice present abnormalities in the retina as in patients: ataxin 7-positive neuronal intranuclear inclusions that were reduced by interferon beta treatment. Finally, since neuronal death does not occur in the cerebellum of SCA7(266Q/5Q) mice, we showed in primary cell cultures expressing mutant ataxin 7 that interferon beta treatment improves Purkinje cell survival.

Passive Thermal Design Approach for the Space Communications and Navigation (SCaN) Testbed Experiment on the International Space Station (ISS)

Science.gov (United States)

Siamidis, John; Yuko, Jim

2014-01-01

The Space Communications and Navigation (SCaN) Program Office at NASA Headquarters oversees all of NASAs space communications activities. SCaN manages and directs the ground-based facilities and services provided by the Deep Space Network (DSN), Near Earth Network (NEN), and the Space Network (SN). Through the SCaN Program Office, NASA GRC developed a Software Defined Radio (SDR) testbed experiment (SCaN testbed experiment) for use on the International Space Station (ISS). It is comprised of three different SDR radios, the Jet Propulsion Laboratory (JPL) radio, Harris Corporation radio, and the General Dynamics Corporation radio. The SCaN testbed experiment provides an on-orbit, adaptable, SDR Space Telecommunications Radio System (STRS) - based facility to conduct a suite of experiments to advance the Software Defined Radio, Space Telecommunications Radio Systems (STRS) standards, reduce risk (Technology Readiness Level (TRL) advancement) for candidate Constellation future space flight hardware software, and demonstrate space communication links critical to future NASA exploration missions. The SCaN testbed project provides NASA, industry, other Government agencies, and academic partners the opportunity to develop and field communications, navigation, and networking technologies in the laboratory and space environment based on reconfigurable, software defined radio platforms and the STRS Architecture.The SCaN testbed is resident on the P3 Express Logistics Carrier (ELC) on the exterior truss of the International Space Station (ISS). The SCaN testbed payload launched on the Japanese Aerospace Exploration Agency (JAXA) H-II Transfer Vehicle (HTV) and was installed on the ISS P3 ELC located on the inboard RAM P3 site. The daily operations and testing are managed out of NASA GRC in the Telescience Support Center (TSC).

Generation of spinocerebellar ataxia type 3 patient-derived induced pluripotent stem cell line SCA3.B11

DEFF Research Database (Denmark)

Hansen, Susanne Kofoed; Borland, Helena; Hasholt, Lis Frydenreich

2016-01-01

Spinocerebellar ataxia type 3 (SCA3) is a dominantly inherited neurodegenerative disease caused by an expansion of the CAG-repeat in ATXN3. In this study, induced pluripotent stem cells (iPSCs) were generated from SCA3 patient dermal fibroblasts by electroporation with episomal plasmids encoding L...

Generation of spinocerebellar ataxia type 3 patient-derived induced pluripotent stem cell line SCA3.A11

DEFF Research Database (Denmark)

Hansen, Susanne Kofoed; Borland, Helena; Hasholt, Lis Frydenreich

2016-01-01

Spinocerebellar ataxia type 3 (SCA3) is a dominantly inherited neurodegenerative disease caused by a CAG-repeat expanding mutation in ATXN3. We generated induced pluripotent stem cells (iPSCs) from a SCA3 patient by electroporation of dermal fibroblasts with episomal plasmids encoding L-MYC, LIN28...

Adventitial SCA-1+ Progenitor Cell Gene Sequencing Reveals the Mechanisms of Cell Migration in Response to Hyperlipidemia

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Ioannis Kokkinopoulos

2017-08-01

Full Text Available Adventitial progenitor cells, including SCA-1+ and mesenchymal stem cells, are believed to be important in vascular remodeling. It has been shown that SCA-1+ progenitor cells are involved in neointimal hyperplasia of vein grafts, but little is known concerning their involvement in hyperlipidemia-induced atherosclerosis. We employed single-cell sequencing technology on primary adventitial mouse SCA-1+ cells from wild-type and atherosclerotic-prone (ApoE-deficient mice and found that a group of genes controlling cell migration and matrix protein degradation was highly altered. Adventitial progenitors from ApoE-deficient mice displayed an augmented migratory potential both in vitro and in vivo. This increased migratory ability was mimicked by lipid loading to SCA-1+ cells. Furthermore, we show that lipid loading increased miRNA-29b expression and induced sirtuin-1 and matrix metalloproteinase-9 levels to promote cell migration. These results provide direct evidence that blood cholesterol levels influence vascular progenitor cell function, which could be a potential target cell for treatment of vascular disease.

SCA-1 Labels a Subset of Estrogen-Responsive Bipotential Repopulating Cells within the CD24+ CD49fhi Mammary Stem Cell-Enriched Compartment

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Genevieve V. Dall

2017-02-01

Full Text Available Estrogen stimulates breast development during puberty and mammary tumors in adulthood through estrogen receptor-α (ERα. These effects are proposed to occur via ERα+ luminal cells and not the mammary stem cells (MaSCs that are ERαneg. Since ERα+ luminal cells express stem cell antigen-1 (SCA-1, we sought to determine if SCA-1 could define an ERα+ subset of EpCAM+/CD24+/CD49fhi MaSCs. We show that the MaSC population has a distinct SCA-1+ population that is abundant in pre-pubertal mammary glands. The SCA-1+ MaSCs have less stem cell markers and less in vivo repopulating activity than their SCA-1neg counterparts. However, they express ERα and specifically enter the cell cycle at puberty. Using estrogen-deficient aromatase knockouts (ArKO, we showed that the SCA-1+ MaSC could be directly modulated by estrogen supplementation. Thus, SCA-1 enriches for an ERα+, estrogen-sensitive subpopulation within the CD24+/CD49fhi MaSC population that may be responsible for the hormonal sensitivity of the developing mammary gland.

Sca-1+ cardiosphere-derived cells are enriched for Isl1-expressing cardiac precursors and improve cardiac function after myocardial injury.

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Jianqin Ye

Full Text Available BACKGROUND: Endogenous cardiac progenitor cells are a promising option for cell-therapy for myocardial infarction (MI. However, obtaining adequate numbers of cardiac progenitors after MI remains a challenge. Cardiospheres (CSs have been proposed to have cardiac regenerative properties; however, their cellular composition and how they may be influenced by the tissue milieu remains unclear. METHODOLOGY/PRINCIPAL FINDING: Using "middle aged" mice as CSs donors, we found that acute MI induced a dramatic increase in the number of CSs in a mouse model of MI, and this increase was attenuated back to baseline over time. We also observed that CSs from post-MI hearts engrafted in ischemic myocardium induced angiogenesis and restored cardiac function. To determine the role of Sca-1(+CD45(- cells within CSs, we cloned these from single cell isolates. Expression of Islet-1 (Isl1 in Sca-1(+CD45(- cells from CSs was 3-fold higher than in whole CSs. Cloned Sca-1(+CD45(- cells had the ability to differentiate into cardiomyocytes, endothelial cells and smooth muscle cells in vitro. We also observed that cloned cells engrafted in ischemic myocardium induced angiogenesis, differentiated into endothelial and smooth muscle cells and improved cardiac function in post-MI hearts. CONCLUSIONS/SIGNIFICANCE: These studies demonstrate that cloned Sca-1(+CD45(- cells derived from CSs from infarcted "middle aged" hearts are enriched for second heart field (i.e., Isl-1(+ precursors that give rise to both myocardial and vascular tissues, and may be an appropriate source of progenitor cells for autologous cell-therapy post-MI.

The SCA1 (Spinocerebellar ataxia type 1 and MJD (Machado-Joseph disease CAG repeats in normal individuals: segregation analysis and allele frequencies

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Cláudia Emília Vieira Wiezel

2003-01-01

Full Text Available Spinocerebellar ataxia type 1 (SCA1 and Machado-Joseph disease (MJD/SCA3 are autosomal dominant neurodegenerative diseases caused by expansions of a CAG trinucleotide repeat in the SCA1 and MJD genes. These expanded sequences are unstable upon transmission, leading to an intergeneration increase in the number of repeats (dynamic mutation. The transmission of the CAG repeat was studied in normal mother-father-child trios, referred for paternity testing (SCA1, n = 367; MJD, n = 879. No segregation distortion was detected. The CAG allele frequencies were determined in 330 unrelated individuals (fathers from couples tested for paternity. The allele frequency distributions did not differ from those previously reported for European populations. The estimated values for the statistic parameters indicating diversity at the SCA1 locus did not differ much from those reported previously for other STRs in the Brazilian population, while those for the MJD locus were close to or higher than the maximum values of previous reports. This shows that SCA1 and MJD are highly informative loci for applications in genetic and population studies and for forensic analysis.

Evaluation of the SCA instrument for measuring patient satisfaction with cancer care administered via paper or via the Internet.

Science.gov (United States)

Kamo, N; Dandapani, S V; Miksad, R A; Houlihan, M J; Kaplan, I; Regan, M; Greenfield, T K; Sanda, M G

2011-03-01

Patients' perspectives provide valuable information on quality of care. This study evaluates the feasibility and validity of Internet administration of Service Satisfaction Scale for Cancer Care (SCA) to assess patient satisfaction with outcome, practitioner manner/skill, information, and waiting/access. Primary data collected from November 2007 to April 2008. Patients receiving cancer care within 1 year were recruited from oncology, surgery, and radiation clinics at a tertiary care hospital. An Internet-based version of the 16-item SCA was developed. Participants were randomised to Internet SCA followed by paper SCA 2 weeks later or vice versa. Seven-point Likert scale responses were converted to a 0-100 scale (minimum-maximum satisfaction). Response distribution, Cronbach's alpha, and test-retest correlations were calculated. Among 122 consenting participants, 78 responded to initial SCA. Mean satisfaction scores for paper/Internet were 91/90 (outcome), 95/94 (practitioner manner/skill), 89/90 (information), and 86/86 (waiting/access). Response rate and item missingness were similar for Internet and paper. Except for practitioner manner/skill, test-retest correlations were robust r = 0.77 (outcome), 0.74 (information), and 0.75 (waiting/access) (all P measurement of cancer care satisfaction for a wide range of cancer diagnoses, treatment modalities, and clinic settings.

The role of interruptions in polyQ in the pathology of SCA1.

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Rajesh P Menon

Full Text Available At least nine dominant neurodegenerative diseases are caused by expansion of CAG repeats in coding regions of specific genes that result in abnormal elongation of polyglutamine (polyQ tracts in the corresponding gene products. When above a threshold that is specific for each disease the expanded polyQ repeats promote protein aggregation, misfolding and neuronal cell death. The length of the polyQ tract inversely correlates with the age at disease onset. It has been observed that interruption of the CAG tract by silent (CAA or missense (CAT mutations may strongly modulate the effect of the expansion and delay the onset age. We have carried out an extensive study in which we have complemented DNA sequence determination with cellular and biophysical models. By sequencing cloned normal and expanded SCA1 alleles taken from our cohort of ataxia patients we have determined sequence variations not detected by allele sizing and observed for the first time that repeat instability can occur even in the presence of CAG interruptions. We show that histidine interrupted pathogenic alleles occur with relatively high frequency (11% and that the age at onset inversely correlates linearly with the longer uninterrupted CAG stretch. This could be reproduced in a cellular model to support the hypothesis of a linear behaviour of polyQ. We clarified by in vitro studies the mechanism by which polyQ interruption slows down aggregation. Our study contributes to the understanding of the role of polyQ interruption in the SCA1 phenotype with regards to age at disease onset, prognosis and transmission.

Link Adaptation for Mitigating Earth-To-Space Propagation Effects on the NASA SCaN Testbed

Science.gov (United States)

Kilcoyne, Deirdre K.; Headley, William C.; Leffke, Zach J.; Rowe, Sonya A.; Mortensen, Dale J.; Reinhart, Richard C.; McGwier, Robert W.

2016-01-01

In Earth-to-Space communications, well-known propagation effects such as path loss and atmospheric loss can lead to fluctuations in the strength of the communications link between a satellite and its ground station. Additionally, the typically unconsidered effect of shadowing due to the geometry of the satellite and its solar panels can also lead to link degradation. As a result of these anticipated channel impairments, NASA's communication links have been traditionally designed to handle the worst-case impact of these effects through high link margins and static, lower rate, modulation formats. The work presented in this paper aims to relax these constraints by providing an improved trade-off between data rate and link margin through utilizing link adaptation. More specifically, this work provides a simulation study on the propagation effects impacting NASA's SCaN Testbed flight software-defined radio (SDR) as well as proposes a link adaptation algorithm that varies the modulation format of a communications link as its signal-to-noise ratio fluctuates. Ultimately, the models developed in this work will be utilized to conduct real-time flight experiments on-board the NASA SCaN Testbed.

A Practical Approach to Rapid Prototyping of SCA Waveforms

OpenAIRE

DePriest, Jacob Andrew

2006-01-01

With the growing interest in software defined radios (SDRs), cognitive radios, the Joint Tactical Radio System (JTRS), and the Software Communication Architecture (SCA) comes the need for a rapid prototyping approach to radio design. In the past, radios have traditionally been designed to have a static implementation with the express goal of implementing a specific type of communication, such as 802.11b, CDMA voice communication, or just a simple FM tuner. However, when designing an ...

Pulse shape analyzer/timing-SCA application to beta measurement

International Nuclear Information System (INIS)

Selvi, S.; Celiktas, C.

2001-01-01

Electrical noise contribution to pulse height distributions from beta sources due to BC-400 plastic scintillator(PS), preamplifier and spectroscopy amplifier was rejected by setting the electronic set-up processing of the modified beta spectrometer consisted of pulse shape analyzer/timing single channel analyzer (PSA/SCA) and related complementary equipments. Improved noise rejection performance was evaluated in terms of elimination practically only all of the noise band of C-14 and Tl-204 spectra obtained using the two alternate beta spectrometer

Isolation, screening and partial purification of antimicrobial antibiotics from soil Streptomyces sp. SCA 7

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P. Saravana Kumar

2014-09-01

Full Text Available Thirty-seven actinomycetes strains were isolated from soil samples collected from an agriculture field in Vengodu, Thiruvannamalai District, Tamil Nadu, India (latitude: 12° 54′ 0033″, North; longitude: 79° 78′ 5216″, East; elevation: 228.6/70.0 ft/m. The isolates were assessed for antagonistic activity against five Gram-positive bacteria, seven Gram-negative bacteria, and two pathogenic fungi. During the initial screening, 43% of the strains showed weak activity, 16% showed moderate activity, 5% showed good activity, and 35% showed no antagonistic activity. Among the strains tested, SCA 7 showed strong antimicrobial activity. Maximum biological activity was obtained on modified nutrient glucose agar (MNGA medium. The mycelia of SCA 7 were extracted with methanol and tested against microbial pathogens using the disc diffusion method. The crude extract was purified partially using column chromatography and assessed for antimicrobial activity. Fraction 10 showed good activity against Staphylococcus epidermidis (31.25 μg/mL and Malassezia pachydermatis (500 μg/mL and the active principle (fraction 10 was identified as 2,4-bis (1,1-dimethylethyl phenol. Based on morphological, physiological, biochemical, cultural, and molecular characteristics (16S rDNA sequencing, this strain was identified as Streptomyces sp. SCA 7. It could be used in the development of new substances for pharmaceutical or agricultural purposes.

SCaMF–RM: A Fused High-Resolution Land Cover Product of the Rocky Mountains

KAUST Repository

Rodrí guez-Jeangros, Nicolá s; Hering, Amanda S.; Kaiser, Timothy; McCray, John E.

2017-01-01

LC products each have different temporal and spatial resolutions and different LC classes that rarely provide the detail required by these studies. Using multiple existing LC products, we implement our Spatiotemporal Categorical Map Fusion (SCa

Neural substrates of motor and cognitive dysfunctions in SCA2 patients: A network based statistics analysis

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G. Olivito

2017-01-01

In the present study, the network-based statistics (NBS approach was used to assess differences in functional connectivity between specific cerebellar and cerebral “nodes” in SCA2 patients. Altered inter-nodal connectivity was found between more posterior regions in the cerebellum and regions in the cerebral cortex clearly related to cognition and emotion. Furthermore, more anterior cerebellar lobules showed altered inter-nodal connectivity with motor and somatosensory cerebral regions. The present data suggest that in SCA2 a cerebellar dysfunction affects long-distance cerebral regions and that the clinical symptoms may be specifically related with connectivity changes between motor and non-motor cerebello-cortical nodes.

SOA for the Business Developer Concepts, BPEL, and SCA

CERN Document Server

Margolis, Ben

2007-01-01

Service-Oriented Architecture (SOA) is a way of organizing software. If your company's development projects adhere to the principles of SOA, the outcome will be an inventory of modular units called "services," which allow for a quick response to change. This book tells the SOA story in a simple, straightforward manner that will help you understand not only the buzzwords and benefits, but also the technologies that underlie SOA: XML, WSDL, SOAP, XPath, BPEL, SCA, and SDO. And through it all, the authors provide business examples and illustrations, giving a practical meaning to abstract ideas

Sustainable Competitive Advantage (SCA Analysis of Furniture Manufacturers in Malaysia: Normalized Scaled Critical Factor Index (NSCFI Approach

Directory of Open Access Journals (Sweden)

Tasmin Rosmaini

2016-06-01

Full Text Available The purpose of this paper is to investigate Malaysian furniture industry via Sustainable competitive advantages (SCA approach. In this case study, sense and respond method and Normalized Scaled Critical Factor Index (NSCFI are used to specify the distribution of companies’ resources for different criteria and detect the attributes which are critical based on expectation and experience of companies’ employs. Moreover, this study evaluates Malaysian furniture business strategy according to manufacturing strategy in terms of analyzer, prospector and defender. Finally, SCA risk levels are presented to show how much company’s resource allocations support their business strategy.

Parkinsonian phenotype in Machado-Joseph disease (MJD/SCA3): a two-case report

NARCIS (Netherlands)

Bettencourt, C.; Santos, C.; Coutinho, P.; Rizzu, P.; Vasconcelos, J.; Kay, T.; Cymbron, T.; Raposo, M.; Heutink, P.; Lima, M.

2011-01-01

Background: Machado-Joseph disease (MJD), or spinocerebellar ataxia type 3 (SCA3), is an autosomal dominant neurodegenerative disorder of late onset, which is caused by a CAG repeat expansion in the coding region of the ATXN3 gene. This disease presents clinical heterogeneity, which cannot be

Sacrificio cortoplacista adaptativo 2opt (SCA_2opt: Una heurística inspirada en el pensamiento sistémico

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Jorge Pérez Rave

2014-03-01

Full Text Available Se detalla el origen de dos noveles heurísticas para el TSP simétrico, inspiradas en el pensamiento sistémico: Sacrificio Cortoplacista Adaptativo 2-opt (SCA_2opt y SCA_2_opt_r. Estas surgen del análisis sistémico de la regla de decisión Vecino más cercano, identificándosele el arquetipo "Soluciones contraproducentes". El SCA se basa en que el agente viajero renuncie en un momento dado a una ciudad inmediatamente cercana, y se traslade hacia la segunda ciudad más cercana disponible. A partir de ello, se continúa con la regla del vecino más cercano. Cada que se realiza el SCA (búsqueda global se efectúa una búsqueda local 2_opt. Considerando el binomio eficacia y eficiencia, las dos heurísticas se muestran prometedoras en comparación multicriterio contra 19 metaheurísticas. Se evidencia que el pensamiento sistémico es un campo de inspiración viable para el desarrollo de métodos de optimización combinatoria; se plasman preguntas emergentes para desarrollos futuros, que permitan continuar integrando elementos de la optimización clásica con el pensamiento sistémico; áreas tradicionalmente vistas como antagónicas, pero cuyo diálogo se muestra favorable en este artículo.

Software Communications Architecture (SCA) Compliant Software Defined Radio Design for IEEE 802.16 Wirelessman-OFDMTM Transceiver

National Research Council Canada - National Science Library

Kian, Low W

2006-01-01

.... Several SDR software architectures have been developed during the last few years. One implementation of the Software Communications Architecture is the Open Source SCA Implementation::Embedded (OSSIE...

Large Polyglutamine Repeats Cause Muscle Degeneration in SCA17 Mice

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Shanshan Huang

2015-10-01

Full Text Available In polyglutamine (polyQ diseases, large polyQ repeats cause juvenile cases with different symptoms than those of adult-onset patients, who carry smaller expanded polyQ repeats. The mechanisms behind the differential pathology mediated by different polyQ repeat lengths remain unknown. By studying knockin mouse models of spinal cerebellar ataxia-17 (SCA17, we found that a large polyQ (105 glutamines in the TATA-box-binding protein (TBP preferentially causes muscle degeneration and reduces the expression of muscle-specific genes. Direct expression of TBP with different polyQ repeats in mouse muscle revealed that muscle degeneration is mediated only by the large polyQ repeats. Different polyQ repeats differentially alter TBP’s interaction with neuronal and muscle-specific transcription factors. As a result, the large polyQ repeat decreases the association of MyoD with TBP and DNA promoters. Our findings suggest that specific alterations in protein interactions by large polyQ repeats may account for the unique pathology in juvenile polyQ diseases.

Large Polyglutamine Repeats Cause Muscle Degeneration in SCA17 Mice

Science.gov (United States)

Huang, Shanshan; Yang, Su; Guo, Jifeng; Yan, Sen; Gaertig, Marta A.; Li, Shihua; Li, Xiao-Jiang

2015-01-01

SUMMARY In polyglutamine (polyQ) diseases, large polyQ repeats cause juvenile cases with different symptoms than adult-onset patients, who carry smaller expanded polyQ repeats. The mechanisms behind the differential pathology mediated by different polyQ repeat lengths remain unknown. By studying knock-in mouse models of spinal cerebellar ataxia-17 (SCA17), we found that a large polyQ (105 glutamines) in the TATA box-binding protein (TBP) preferentially causes muscle degeneration and reduces the expression of muscle-specific genes. Direct expression of TBP with different polyQ repeats in mouse muscle revealed that muscle degeneration is mediated only by the large polyQ repeats. Different polyQ repeats differentially alter TBP’s interaction with neuronal and muscle-specific transcription factors. As a result, the large polyQ repeat decreases the association of MyoD with TBP and DNA promoters. Our findings suggest that specific alterations in protein interactions by large polyQ repeats may account for the unique pathology in juvenile polyQ diseases. PMID:26387956

TXRF analysis of multielements in serum of patients with sickle cell anemia (SCA) by synchrotron radiation

International Nuclear Information System (INIS)

Canellas, Catarine G.L.; Jesus, Edgar F.O. de; Anjos, Marcelino J.; Lopes, Ricardo T.

2009-01-01

The determination of trace elements levels in physiological fluids is of considerable interest in clinical chemistry. Since it has been established these levels in human serum can be utilized as indicators for several pathological conditions, diagnosis and treatment of various diseases. In this work, trace elements were analyzed in serum of patients with sickle cell anemia (SCA) by total reflection X-ray fluorescence using synchrotron radiation (SRTXRF). Sickle cell Anemia is a blood disorder that affects hemoglobin, the protein found in red blood cells that help carry oxygen throughout the body. SCA occurs when a person inherits two abnormal genes (one from each parent) that cause their red blood cells to change shape. These irregular-shaped blood cells die prematurely, resulting in a chronic shortage of red blood cells. We studied forty-three patients (15 males and 28 females) aged 18 to 50 years, suffering SCA and Sixty healthy volunteers (41 males and 19 females) aged 18 to 60 years. All the serum samples had been collected of people who live in the urban area of Rio de Janeiro City/Brazil. The measurements were performed at the X-ray fluorescence beam line at Brazilian National Synchrotron Light Laboratory (LNLS), in Campinas, Sao Paulo using a polychromatic beam. It was possible to determine the concentrations of the following elements: P, S, Cl, K, Ca, Cu, Zn, Br and Rb. (author)

TXRF analysis of multielements in serum of patients with sickle cell anemia (SCA) by synchrotron radiation

Energy Technology Data Exchange (ETDEWEB)

Canellas, Catarine G.L.; Jesus, Edgar F.O. de; Anjos, Marcelino J.; Lopes, Ricardo T., E-mail: catarine@lin.ufrj.b, E-mail: edgar@lin.ufrj.b, E-mail: marcelin@lin.ufrj.b, E-mail: ricardo@lin.ufrj.b [Federal University of Rio de Janeiro (UFRJ), RJ (Brazil). COPPE Technology Center. Nuclear Instrumentation Lab.; Carvalho, Silvia M.F., E-mail: silvia@hemorio.rj.gov.b [State Institute of Hematology Arthur de Siqueira Cavalcanti (HEMORIO), Rio de Janeiro, RJ (Brazil)

2009-07-01

The determination of trace elements levels in physiological fluids is of considerable interest in clinical chemistry. Since it has been established these levels in human serum can be utilized as indicators for several pathological conditions, diagnosis and treatment of various diseases. In this work, trace elements were analyzed in serum of patients with sickle cell anemia (SCA) by total reflection X-ray fluorescence using synchrotron radiation (SRTXRF). Sickle cell Anemia is a blood disorder that affects hemoglobin, the protein found in red blood cells that help carry oxygen throughout the body. SCA occurs when a person inherits two abnormal genes (one from each parent) that cause their red blood cells to change shape. These irregular-shaped blood cells die prematurely, resulting in a chronic shortage of red blood cells. We studied forty-three patients (15 males and 28 females) aged 18 to 50 years, suffering SCA and Sixty healthy volunteers (41 males and 19 females) aged 18 to 60 years. All the serum samples had been collected of people who live in the urban area of Rio de Janeiro City/Brazil. The measurements were performed at the X-ray fluorescence beam line at Brazilian National Synchrotron Light Laboratory (LNLS), in Campinas, Sao Paulo using a polychromatic beam. It was possible to determine the concentrations of the following elements: P, S, Cl, K, Ca, Cu, Zn, Br and Rb. (author)

The SCA description of inner-shell Coulomb ionization and associated phenomena

International Nuclear Information System (INIS)

Hansteen, J.M.

1989-01-01

The semiclassical trajectory method for describing atomic processes induced by charged projectiles is outlined. The framework for the perturbative SCA formalism is sketched with emphasis on the first-order time-dependent approach. Selected results from computations on inner-shell Coulomb ionization and δ-electron emission are presented. The kinematic scaling law for ionization and pair-production phenomena is treated in detail. The importance of this scaling law for high-energy atomic collision physics is stressed. (orig.)

Pulse shape analyzer/timing-SCA application to beta measurement by plastic scintillator

International Nuclear Information System (INIS)

Celiktas, C.; Selvi, S.

2000-01-01

Noise contribution to pulses from BC-400 plastic scintillators, preamplifier and spectroscopy amplifier is rejected by using electronics processing of the modified beta spectrometer containing pulse shape analyzer/timing SCA and related complementary equipment. The noise rejection capability of the spectrometer which have been developed to measure pure and scattered beta spectra, which are reliable in view of evaluations of the detector and target electron scattering characteristics correctly. (author)

Plasma Science Contribution to the SCaLeS Report

International Nuclear Information System (INIS)

Jardin, S.C.

2003-01-01

In June of 2003, about 250 computational scientists and mathematicians being funded by the DOE Office of Science met in Arlington, VA, to attend a 2-day workshop on the Science Case for Large-scale Simulation (SCaLeS). This document was the output of the Plasma Science Section of that workshop. The conclusion is that exciting and important progress can be made in the field of Plasma Science if computer power continues to grow and algorithmic development continues to occur at the rate that it has in the past. Full simulations of burning plasma experiments could be possible in the 5-10 year time frame if an aggressive growth program is launched in this area

Pengaruh Sales Call Anxiety (SCA pada Kinerja Salespersons Asuransi Jiwa dengan Trait Negative Affectivity (Trait-Na

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Rita Rita

2012-11-01

Full Text Available This study empirically examines the effect of sales call anxiety (SCA on salesperson performance, good performance in the form of sales volume and sales interactions. This study tested the hypothesis to identify early factors (antecedents that may affect the sales call anxiety (SCA. Initial factors suspected as the cause of anxiety in sales interactions (SCA are divided into two conditions, namely canvassing and closing condition. Canvassing consisted of meeting new people, customer with high social status, and being assertive, while the closing is a condition consisting of asking for commitment, and discussing performance with sales manager. The population in this study is all life insurance company salesperson in Palangkaraya. In this study, questionnaires were distributed to 200 people a life insurance salesperson, and then used as a sample is a total of 147 respondents who have a tendency to trait-NA. The sampling method used was purposive sampling. The analysis showed that when the salesperson must meet new customers, customers with a high social status, when they should be able to introduce himself and mission well (canvassing, when they should ask for customer commitments after several meetings and interviews with candidates customers, and when the salesperson must discuss the results of their performance with the sales manager (closing, causing anxiety for the salesperson, because when interacting with potential customers, they have a negative perception of the ability of self, negative perceptions of the mind customers in assessing their work when interacting, the emergence of physiological symptoms, and they take action "escape" of the situation, or the so-called protective action.

Spinocerebellar ataxia type 7 (SCA7) : widespread brain damage in an adult-onset patient with progressive visual impairments in comparison with an adult-onset patient without visual impairments

NARCIS (Netherlands)

Rueb, U.; Brunt, E. R.; Seidel, K.; Gierga, K.; Mooy, C. M.; Kettner, M.; Van Broeckhoven, C.; Bechmann, I.; La Spada, A. R.; Schoels, L.; den Dunnen, W.; de Vos, R. A. I.; Deller, T.

Spinocerebellar ataxia type 7 (SCA7) represents a rare and severe autosomal dominantly inherited ataxic disorder and is among the known CAG-repeat, or polyglutamine, diseases. In contrast to other currently known autosomal dominantly inherited ataxic disorders, SCA7 may manifest itself with

MAPK/ERK and Wnt/β-Catenin pathways are synergistically involved in proliferation of Sca-1 positive hepatic progenitor cells

International Nuclear Information System (INIS)

Jin, Caixia; Samuelson, Lisa; Cui, Cai-Bin; Sun, Yangzhong; Gerber, David A.

2011-01-01

Highlights: → Activation of MAPK/ERK pathway with epidermal growth factor (EGF) significantly increased Sca-1 + HPC proliferation and colony formation. → Activation of either IL-6/STAT3 or Wnt/β-Catenin pathway did not independently support cell proliferation and colony formation of HPCs. → Wnt/β-Catenin pathway can cooperate with EGF to significantly promote HPC colony formation and maintain long-term HPCs in vitro. -- Abstract: Hepatic progenitor cells (HPCs) persist in adulthood and have the potential to play a major role in regenerating diseased liver. However, the signaling pathways that both directly and indirectly regulate HPCs' self-renewal and differentiation remain elusive. Previously, we identified a bipotent, stem cell antigen-1 (Sca-1) positive HPC population from naive adult liver tissue. In the present study, we aimed to investigate the involvement of various signaling pathways in Sca-1 + HPC proliferation. Epidermal growth factor (EGF) supplementation shows a significant increase in Sca-1 + HPC proliferation and colony formation while stimulating phosphorylation of ERK1/2 and activating the induction of Cyclin D1. There were no demonstrable effects of EGF on Akt. The MEK inhibitor, PD0325901, inhibits proliferation and ERK1/2 phosphorylation while also suppressing the expression of Cyclin D1. In addition, activation of either IL-6/STAT3 or Wnt/β-Catenin pathway did not independently support cell proliferation and colony formation of HPCs. The Wnt/β-Catenin pathway can cooperate with EGF to significantly promote HPC colony formation ratio and maintain long-term HPC in vitro. The data indicates that the MAPK/ERK pathway is both essential and critical for HPC proliferation, and the Wnt signaling pathway is not sufficient, while it works synergistically with the MAPK/ERK signaling pathway to promote HPC proliferation.

MAPK/ERK and Wnt/{beta}-Catenin pathways are synergistically involved in proliferation of Sca-1 positive hepatic progenitor cells

Energy Technology Data Exchange (ETDEWEB)

Jin, Caixia [Department of Surgery, University of North Carolina at Chapel Hill (United States); Department of Medical Genetics and Cell Biology, Ningxia Medical University, Yinchuan 750004 (China); Samuelson, Lisa; Cui, Cai-Bin; Sun, Yangzhong [Department of Surgery, University of North Carolina at Chapel Hill (United States); Gerber, David A., E-mail: david_gerber@med.unc.edu [Department of Surgery, University of North Carolina at Chapel Hill (United States); Lineberger Cancer Center, University of North Carolina at Chapel Hill (United States)

2011-06-17

Highlights: {yields} Activation of MAPK/ERK pathway with epidermal growth factor (EGF) significantly increased Sca-1{sup +} HPC proliferation and colony formation. {yields} Activation of either IL-6/STAT3 or Wnt/{beta}-Catenin pathway did not independently support cell proliferation and colony formation of HPCs. {yields} Wnt/{beta}-Catenin pathway can cooperate with EGF to significantly promote HPC colony formation and maintain long-term HPCs in vitro. -- Abstract: Hepatic progenitor cells (HPCs) persist in adulthood and have the potential to play a major role in regenerating diseased liver. However, the signaling pathways that both directly and indirectly regulate HPCs' self-renewal and differentiation remain elusive. Previously, we identified a bipotent, stem cell antigen-1 (Sca-1) positive HPC population from naive adult liver tissue. In the present study, we aimed to investigate the involvement of various signaling pathways in Sca-1{sup +} HPC proliferation. Epidermal growth factor (EGF) supplementation shows a significant increase in Sca-1{sup +} HPC proliferation and colony formation while stimulating phosphorylation of ERK1/2 and activating the induction of Cyclin D1. There were no demonstrable effects of EGF on Akt. The MEK inhibitor, PD0325901, inhibits proliferation and ERK1/2 phosphorylation while also suppressing the expression of Cyclin D1. In addition, activation of either IL-6/STAT3 or Wnt/{beta}-Catenin pathway did not independently support cell proliferation and colony formation of HPCs. The Wnt/{beta}-Catenin pathway can cooperate with EGF to significantly promote HPC colony formation ratio and maintain long-term HPC in vitro. The data indicates that the MAPK/ERK pathway is both essential and critical for HPC proliferation, and the Wnt signaling pathway is not sufficient, while it works synergistically with the MAPK/ERK signaling pathway to promote HPC proliferation.

Evidence of oxidative stress and mitochondrial dysfunction in spinocerebellar ataxia type 2 (SCA2) patient fibroblasts

DEFF Research Database (Denmark)

Cornelius, Nanna; Wardman, Jonathan H; Hargreaves, Iain P

2017-01-01

Spinocerebellar ataxia type 2 (SCA2) is a rare neurodegenerative disorder caused by a CAG repeat expansion in the ataxin-2 gene. We show increased oxidative stress, abnormalities in the antioxidant system, changes in complexes involved in oxidative phosphorylation and changes in mitochondrial mor...

The ScaLIng Macroweather Model (SLIMM): using scaling to forecast global-scale macroweather from months to decades

Science.gov (United States)

Lovejoy, S.; del Rio Amador, L.; Hébert, R.

2015-09-01

On scales of ≈ 10 days (the lifetime of planetary-scale structures), there is a drastic transition from high-frequency weather to low-frequency macroweather. This scale is close to the predictability limits of deterministic atmospheric models; thus, in GCM (general circulation model) macroweather forecasts, the weather is a high-frequency noise. However, neither the GCM noise nor the GCM climate is fully realistic. In this paper we show how simple stochastic models can be developed that use empirical data to force the statistics and climate to be realistic so that even a two-parameter model can perform as well as GCMs for annual global temperature forecasts. The key is to exploit the scaling of the dynamics and the large stochastic memories that we quantify. Since macroweather temporal (but not spatial) intermittency is low, we propose using the simplest model based on fractional Gaussian noise (fGn): the ScaLIng Macroweather Model (SLIMM). SLIMM is based on a stochastic ordinary differential equation, differing from usual linear stochastic models (such as the linear inverse modelling - LIM) in that it is of fractional rather than integer order. Whereas LIM implicitly assumes that there is no low-frequency memory, SLIMM has a huge memory that can be exploited. Although the basic mathematical forecast problem for fGn has been solved, we approach the problem in an original manner, notably using the method of innovations to obtain simpler results on forecast skill and on the size of the effective system memory. A key to successful stochastic forecasts of natural macroweather variability is to first remove the low-frequency anthropogenic component. A previous attempt to use fGn for forecasts had disappointing results because this was not done. We validate our theory using hindcasts of global and Northern Hemisphere temperatures at monthly and annual resolutions. Several nondimensional measures of forecast skill - with no adjustable parameters - show excellent

Lodhi 5 Properties Investments CC v FirstRand Bank Limited [2015] 3 All SA 32 (SCA and the Enforcement of Islamic Banking Law in South Africa

Directory of Open Access Journals (Sweden)

Maphuti David Tuba

2017-03-01

Full Text Available On 22 May 2015, the Supreme Court of Appeal (SCA handed down a judgment in the matter of Lodhi 5 Properties Investments CC v FirstRand Bank Limited [2015] 3 All SA 32 (SCA. This judgement considered whether the prohibition against the charging of interest on a loan in terms of Islamic law (Sharia law may be a defence for a claim for mora interest in terms of a loan agreement. This note critically discusses the judgment in the light of the approach adopted by the SCA with regard to addressing a dispute arising from a contract that has Islamic law as a governing law. As this is the first case to come before the SCA in South Africa, this note critically analyses how the court discussed the principles of Islamic law as applicable to the dispute between the parties. In particular, it questions the court's assertion that a claim for mora interest has nothing to do with and is not affected by the Sharia law's prohibition against payment of interest on a loan debt. It also looks at the SCA's approach (as a common law court with regard to the enforcement of the principles of Islamic banking law. The judgment raises important issues regarding the enforceability of Islamic finance law and therefore merits discussion in the context of the continuing growth and expansion of Islamic banking and finance law in South Africa.

Modeling the influence of snow cover on low Arctic net ecosystem exchange

International Nuclear Information System (INIS)

Luus, K A; Kelly, R E J; Lin, J C; Humphreys, E R; Lafleur, P M; Oechel, W C

2013-01-01

The Arctic net ecosystem exchange (NEE) of CO 2 between the land surface and the atmosphere is influenced by the timing of snow onset and melt. The objective of this study was to examine whether uncertainty in model estimates of NEE could be reduced by representing the influence of snow on NEE using remote sensing observations of snow cover area (SCA). Observations of NEE and time-lapse images of SCA were collected over four locations at a low Arctic site (Daring Lake, NWT) in May–June 2010. Analysis of these observations indicated that SCA influences NEE, and that good agreement exists between SCA derived from time-lapse images, Landsat and MODIS. MODIS SCA was therefore incorporated into the vegetation photosynthesis respiration model (VPRM). VPRM was calibrated using observations collected in 2005 at Daring Lake. Estimates of NEE were then generated over Daring Lake and Ivotuk, Alaska (2004–2007) using VPRM formulations with and without explicit representations of the influence of SCA on respiration and/or photosynthesis. Model performance was assessed by comparing VPRM output against unfilled eddy covariance observations from Daring Lake and Ivotuk (2004–2007). The uncertainty in VPRM estimates of NEE was reduced when respiration was estimated as a function of air temperature when SCA ≤ 50% and as a function of soil temperature when SCA > 50%. (letter)

The GBT-SCA, a radiation tolerant ASIC for detector control and monitoring applications in HEP experiments

International Nuclear Information System (INIS)

Caratelli, A.; Bonacini, S.; Kloukinas, K.; Marchioro, A.; Moreira, P.; Oliveira, R. De; Paillard, C.

2015-01-01

The future upgrades of the LHC experiments will increase the beam luminosity leading to a corresponding growth of the amounts of data to be treated by the data acquisition systems. To address these needs, the GBT (Giga-Bit Transceiver optical link [1,2]) architecture was developed to provide the simultaneous transfer of readout data, timing and trigger signals as well as slow control and monitoring data. The GBT-SCA ASIC, part of the GBT chip-set, has the purpose to distribute control and monitoring signals to the on-detector front-end electronics and perform monitoring operations of detector environmental parameters. In order to meet the requirements of different front-end ASICs used in the experiments, it provides various user-configurable interfaces capable to perform simultaneous operations. It is designed employing radiation tolerant design techniques to ensure robustness against SEUs and TID radiation effects and is implemented in a commercial 130 nm CMOS technology. This work presents the GBT-SCA architecture, the ASIC interfaces, the data transfer protocol, and its integration with the GBT optical link

ReSCA: decision support tool for remediation planning after the Chernobyl accident.

Science.gov (United States)

Ulanovsky, A; Jacob, P; Fesenko, S; Bogdevitch, I; Kashparov, V; Sanzharova, N

2011-03-01

Radioactive contamination of the environment following the Chernobyl accident still provide a substantial impact on the population of affected territories in Belarus, Russia, and Ukraine. Reduction of population exposure can be achieved by performing remediation activities in these areas. Resulting from the IAEA Technical Co-operation Projects with these countries, the program ReSCA (Remediation Strategies after the Chernobyl Accident) has been developed to provide assistance to decision makers and to facilitate a selection of an optimized remediation strategy in rural settlements. The paper provides in-depth description of the program, its algorithm, and structure. © Springer-Verlag 2010

50 CFR Figure 20 to Part 679 - Steller sea lion conservation area (SCA) of the Bering Sea

Science.gov (United States)

2010-10-01

... 50 Wildlife and Fisheries 9 2010-10-01 2010-10-01 false Steller sea lion conservation area (SCA) of the Bering Sea 20 Figure 20 to Part 679 Wildlife and Fisheries FISHERY CONSERVATION AND MANAGEMENT... EXCLUSIVE ECONOMIC ZONE OFF ALASKA Pt. 679, Fig. 20 Figure 20 to Part 679—Steller sea lion conservation area...

Influence of aging on the activity of mice Sca-1+CD31- cardiac stem cells.

Science.gov (United States)

Wu, Qiong; Zhan, Jinxi; Pu, Shiming; Qin, Liu; Li, Yun; Zhou, Zuping

2017-01-03

Therapeutic application of cardiac resident stem/progenitor cells (CSC/CPCs) is limited due to decline of their regenerative potential with donor age. A variety of studies have shown that the cardiac aging was the problem of the stem cells, but little is known about the impact of age on the subgroups CSC/CPCs, the relationship between subgroups CSC/CPCs ageing and age-related dysfunction. Here, we studied Sca-1+CD31- subgroups of CSCs from younger(2~3months) and older(22~24months) age mice, biological differentiation was realized using specific mediums for 14 days to induce cardiomyocyte, smooth muscle cells or endothelial cells and immunostain analysis of differentiated cell resulting were done. Proliferation and cell cycle were measured by flow cytometry assay, then used microarray to dissect variability from younger and older mice. Although the number of CSCs was higher in older mice, the advanced age significantly reduced the differentiation ability into cardiac cell lineages and the proliferation ability. Transcriptional changes in Sca-1+CD31- subgroups of CSCs during aging are related to Vitamin B6 metabolism, circadian rhythm, Tyrosine metabolism, Complement and coagulation cascades. Taking together these results indicate that Cardiac resident stem/progenitor cells have significant differences in their proliferative, pluripotency and gene profiles and those differences are age depending.

Intra-myocardial injection of both growth factors and heart derived Sca-1+/CD31- cells attenuates post-MI LV remodeling more than does cell transplantation alone: neither intervention enhances functionally significant cardiomyocyte regeneration.

Science.gov (United States)

Wang, Xiaohong; Li, Qinglu; Hu, Qingsong; Suntharalingam, Piradeep; From, Arthur H L; Zhang, Jianyi

2014-01-01

Insulin-like growth factor 1 (IGF-1) and hepatocyte growth factor (HGF) are two potent cell survival and regenerative factors in response to myocardial injury (MI). We hypothesized that simultaneous delivery of IGF+HGF combined with Sca-1+/CD31- cells would improve the outcome of transplantation therapy in response to the altered hostile microenvironment post MI. One million adenovirus nuclear LacZ-labeled Sca-1+/CD31- cells were injected into the peri-infarction area after left anterior descending coronary artery (LAD) ligation in mice. Recombinant mouse IGF-1+HGF was added to the cell suspension prior to the injection. The left ventricular (LV) function was assessed by echocardiography 4 weeks after the transplantation. The cell engraftment, differentiation and cardiomyocyte regeneration were evaluated by histological analysis. Sca-1+/CD31- cells formed viable grafts and improved LV ejection fraction (EF) (Control, 54.5+/-2.4; MI, 17.6+/-3.1; Cell, 28.2+/-4.2, n = 9, Pcell transplantation as evidenced by increased EF (38.8+/-2.2; n = 9, Pcell engraftment rate, promoted the transplanted cell survival, enhanced angiogenesis, and minimally stimulated endogenous cardiomyocyte regeneration in vivo. The in vitro experiments showed that IGF+HGF treatment stimulated Sca-1+/CD31- cell proliferation and inhibited serum free medium induced apoptosis. Supperarray profiling of Sca-1+/CD31- cells revealed that Sca-1+/CD31- cells highly expressed various trophic factor mRNAs and IGF+HGF treatment altered the mRNAs expression patterns of these cells. These data indicate that IGF-1+HGF could serve as an adjuvant to cell transplantation for myocardial repair by stimulating donor cell and endogenous cardiac stem cell survival, regeneration and promoting angiogenesis.

Intra-myocardial injection of both growth factors and heart derived Sca-1+/CD31- cells attenuates post-MI LV remodeling more than does cell transplantation alone: neither intervention enhances functionally significant cardiomyocyte regeneration.

Directory of Open Access Journals (Sweden)

Xiaohong Wang

Full Text Available Insulin-like growth factor 1 (IGF-1 and hepatocyte growth factor (HGF are two potent cell survival and regenerative factors in response to myocardial injury (MI. We hypothesized that simultaneous delivery of IGF+HGF combined with Sca-1+/CD31- cells would improve the outcome of transplantation therapy in response to the altered hostile microenvironment post MI. One million adenovirus nuclear LacZ-labeled Sca-1+/CD31- cells were injected into the peri-infarction area after left anterior descending coronary artery (LAD ligation in mice. Recombinant mouse IGF-1+HGF was added to the cell suspension prior to the injection. The left ventricular (LV function was assessed by echocardiography 4 weeks after the transplantation. The cell engraftment, differentiation and cardiomyocyte regeneration were evaluated by histological analysis. Sca-1+/CD31- cells formed viable grafts and improved LV ejection fraction (EF (Control, 54.5+/-2.4; MI, 17.6+/-3.1; Cell, 28.2+/-4.2, n = 9, P<0.01. IGF+HGF significantly enhanced the benefits of cell transplantation as evidenced by increased EF (38.8+/-2.2; n = 9, P<0.01 and attenuated adverse structural remodeling. Furthermore, IGF+HGF supplementation increased the cell engraftment rate, promoted the transplanted cell survival, enhanced angiogenesis, and minimally stimulated endogenous cardiomyocyte regeneration in vivo. The in vitro experiments showed that IGF+HGF treatment stimulated Sca-1+/CD31- cell proliferation and inhibited serum free medium induced apoptosis. Supperarray profiling of Sca-1+/CD31- cells revealed that Sca-1+/CD31- cells highly expressed various trophic factor mRNAs and IGF+HGF treatment altered the mRNAs expression patterns of these cells. These data indicate that IGF-1+HGF could serve as an adjuvant to cell transplantation for myocardial repair by stimulating donor cell and endogenous cardiac stem cell survival, regeneration and promoting angiogenesis.

A cellular automata model of Ebola virus dynamics

Science.gov (United States)

Burkhead, Emily; Hawkins, Jane

2015-11-01

We construct a stochastic cellular automaton (SCA) model for the spread of the Ebola virus (EBOV). We make substantial modifications to an existing SCA model used for HIV, introduced by others and studied by the authors. We give a rigorous analysis of the similarities between models due to the spread of virus and the typical immune response to it, and the differences which reflect the drastically different timing of the course of EBOV. We demonstrate output from the model and compare it with clinical data.

Gene expression profile and functionality of ESC-derived Lin-ckit+Sca-1+ cells are distinct from Lin-ckit+Sca-1+ cells isolated from fetal liver or bone marrow.

Directory of Open Access Journals (Sweden)

Irina Fernandez

Full Text Available In vitro bioreactor-based cultures are being extensively investigated for large-scale production of differentiated cells from embryonic stem cells (ESCs. However, it is unclear whether in vitro ESC-derived progenitors have similar gene expression profiles and functionalities as their in vivo counterparts. This is crucial in establishing the validity of ESC-derived cells as replacements for adult-isolated cells for clinical therapies. In this study, we compared the gene expression profiles of Lin-ckit+Sca-1+ (LKS cells generated in vitro from mouse ESCs using either static or bioreactor-based cultures, with that of native LKS cells isolated from mouse fetal liver (FL or bone marrow (BM. We found that in vitro-generated LKS cells were more similar to FL- than to BM LKS cells in gene expression. Further, when compared to cells derived from bioreactor cultures, static culture-derived LKS cells showed fewer differentially expressed genes relative to both in vivo LKS populations. Overall, the expression of hematopoietic genes was lower in ESC-derived LKS cells compared to cells from BM and FL, while the levels of non-hematopoietic genes were up-regulated. In order to determine if these molecular profiles correlated with functionality, we evaluated ESC-derived LKS cells for in vitro hematopoietic-differentiation and colony formation (CFU assay. Although static culture-generated cells failed to form any colonies, they did differentiate into CD11c+ and B220+ cells indicating some hematopoietic potential. In contrast, bioreactor-derived LKS cells, when differentiated under the same conditions failed to produce any B220+ or CD11c+ cells and did not form colonies, indicating that these cells are not hematopoietic progenitors. We conclude that in vitro culture conditions significantly affect the transcriptome and functionality of ESC-derived LKS cells and although in vitro differentiated LKS cells were lineage negative and expressed both ckit and Sca-1

Promoter Variation and Expression Levels of Inflammatory Genes IL1A, IL1B, IL6 and TNF in Blood of Spinocerebellar Ataxia Type 3 (SCA3) Patients.

Science.gov (United States)

Raposo, Mafalda; Bettencourt, Conceição; Ramos, Amanda; Kazachkova, Nadiya; Vasconcelos, João; Kay, Teresa; Bruges-Armas, Jácome; Lima, Manuela

2017-03-01

Age at onset in spinocerebellar ataxia type 3 (SCA3/MJD) is incompletely explained by the size of the CAG tract at the ATXN3 gene, implying the existence of genetic modifiers. A role of inflammation in SCA3 has been postulated, involving altered cytokines levels; promoter variants leading to alterations in cytokines expression could influence onset. Using blood from 86 SCA3 patients and 106 controls, this work aimed to analyse promoter variation of four cytokines (IL1A, IL1B, IL6 and TNF) and to investigate the association between variants detected and their transcript levels, evaluated by quantitative PCR. Moreover, the effect of APOE isoforms, known to modulate cytokines, was investigated. Correlations between cytokine variants and onset were tested; the cumulative modifier effects of cytokines and APOE were analysed. Patients carrying the IL6*C allele had a significant earlier onset (4 years in average) than patients carrying the G allele, in agreement with lower mRNA levels produced by IL6*C carriers. The presence of APOE*ɛ2 allele seems to anticipate onset in average 10 years in patients carrying the IL6*C allele; a larger number of patients will be needed to confirm this result. These results highlight the pertinence of conducting further research on the role of cytokines as SCA3 modulators, pointing to the presence of shared mechanisms involving IL6 and APOE.

Decadal trends in the seasonal-cycle amplitude of terrestrial CO2 exchange resulting from the ensemble of terrestrial biosphere models

Directory of Open Access Journals (Sweden)

Akihiko Ito

2016-05-01

Full Text Available The seasonal-cycle amplitude (SCA of the atmosphere–ecosystem carbon dioxide (CO2 exchange rate is a useful metric of the responsiveness of the terrestrial biosphere to environmental variations. It is unclear, however, what underlying mechanisms are responsible for the observed increasing trend of SCA in atmospheric CO2 concentration. Using output data from the Multi-scale Terrestrial Model Intercomparison Project (MsTMIP, we investigated how well the SCA of atmosphere–ecosystem CO2 exchange was simulated with 15 contemporary terrestrial ecosystem models during the period 1901–2010. Also, we made attempt to evaluate the contributions of potential mechanisms such as atmospheric CO2, climate, land-use, and nitrogen deposition, through factorial experiments using different combinations of forcing data. Under contemporary conditions, the simulated global-scale SCA of the cumulative net ecosystem carbon flux of most models was comparable in magnitude with the SCA of atmospheric CO2 concentrations. Results from factorial simulation experiments showed that elevated atmospheric CO2 exerted a strong influence on the seasonality amplification. When the model considered not only climate change but also land-use and atmospheric CO2 changes, the majority of the models showed amplification trends of the SCAs of photosynthesis, respiration, and net ecosystem production (+0.19 % to +0.50 % yr−1. In the case of land-use change, it was difficult to separate the contribution of agricultural management to SCA because of inadequacies in both the data and models. The simulated amplification of SCA was approximately consistent with the observational evidence of the SCA in atmospheric CO2 concentrations. Large inter-model differences remained, however, in the simulated global tendencies and spatial patterns of CO2 exchanges. Further studies are required to identify a consistent explanation for the simulated and observed amplification trends, including their

Forward-looking Assimilation of MODIS-derived Snow Covered Area into a Land Surface Model

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Zaitchik, Benjamin F.; Rodell, Matthew

2008-01-01

Snow cover over land has a significant impact on the surface radiation budget, turbulent energy fluxes to the atmosphere, and local hydrological fluxes. For this reason, inaccuracies in the representation of snow covered area (SCA) within a land surface model (LSM) can lead to substantial errors in both offline and coupled simulations. Data assimilation algorithms have the potential to address this problem. However, the assimilation of SCA observations is complicated by an information deficit in the observation SCA indicates only the presence or absence of snow, and not snow volume and by the fact that assimilated SCA observations can introduce inconsistencies with atmospheric forcing data, leading to non-physical artifacts in the local water balance. In this paper we present a novel assimilation algorithm that introduces MODIS SCA observations to the Noah LSM in global, uncoupled simulations. The algorithm utilizes observations from up to 72 hours ahead of the model simulation in order to correct against emerging errors in the simulation of snow cover while preserving the local hydrologic balance. This is accomplished by using future snow observations to adjust air temperature and, when necessary, precipitation within the LSM. In global, offline integrations, this new assimilation algorithm provided improved simulation of SCA and snow water equivalent relative to open loop integrations and integrations that used an earlier SCA assimilation algorithm. These improvements, in turn, influenced the simulation of surface water and energy fluxes both during the snow season and, in some regions, on into the following spring.

Impacts of the EA and SCA patterns on the 20th century NAO-winter precipitation relationship in Europe

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Comas-Bru, Laia; McDermott, Frank

2013-04-01

Much of the 20th century multi-decadal variability in the NAO-winter precipitation relationship over the N. Atlantic / European sector can be ascribed to the combined effects of the North Atlantic Oscillation (NAO) and either the East Atlantic pattern (EA) or the Scandinavian pattern (SCA). The NAO, EA and SCA indices employed here are defined as the three leading vectors of the cross-correlation matrix calculated from monthly sea-level pressure anomalies for 138 complete winters from the 20CRv2 dataset (Compo et al., 2011). Winter precipitation data over Europe for the entire 20th century is derived from the high resolution CRU-TS3.1 climate dataset (Mitchell and Jones, 2005). Here we document for the first time, that different NAO/EA and NAO/SCA combinations systematically influence winter precipitation conditions in Europe as a consequence of NAO dipole migrations. We find that the zero-correlated line of the NAO-winter precipitation relationship migrates southwards when the EA is in the opposite phase to the NAO. This can be related to a south-westwards migration of the NAO dipole under these conditions, as shown by teleconnectivity maps. Similarly, a clockwise movement of the NAO-winter climate correlated areas occurs when the phase of the SCA is opposite to that of the NAO, reflecting a clockwise movement of the NAO dipole under these conditions. An important implication of these migrations is that they influence the spatial and temporal stationarity of climate-NAO relationships. As a result, the link between winter precipitation patterns and the NAO is not straightforward in some regions such as the southern UK, Ireland and France. For instance, much of the inter-annual variability in the N-S winter precipitation gradient in the UK, originally attributed to inter-annual and inter-decadal variability of the NAO, reflects the migration of the NAO dipole, linked to linear combinations of the NAO and the EA. Our results indicate that when the N-S winter

A ScaI RFLP demonstrated for the GRO gene on chromosome 4

Energy Technology Data Exchange (ETDEWEB)

Beck, J.S.; Murray, J.C. (Univ. of Iowa Hospitals, Iowa City (USA)); Sager, R. (Dana Farber Cancer Institute, Boston, MA (USA))

1989-11-11

TC870 is a 0.85 kb fragment running from the EcoRI site 200 pb from the 5{prime} end of the human cDNA subcloned into the EcoRI site of pGEM3. ScaI detects a polymorphism with two variable bands of 19 kb and 16 kb. One strong constant band (14 kb) and two fainter constant bands (5 kb and 3 kb) are also present. The polymorphism type is unknown. GRO has been localized to 4q13-4q21 by somatic cell hybrid analysis and in situ hybridization. Codominant segregation and Hardy-Weinberg equilibrium demonstrated in 20 CEPH families. The probe also was assigned to chromosome 4 with significant linkage to ALB, GC, INP10, MT2P1. GRO may be the same gene as MGSA.

Yanqing solar field: Dynamic optical model and operational safety analysis

International Nuclear Information System (INIS)

Zhao, Dongming; Wang, Zhifeng; Xu, Ershu; Zhu, Lingzhi; Lei, Dongqiang; Xu, Li; Yuan, Guofeng

2017-01-01

Highlights: • A dynamic optical model of the Yanqing solar field was built. • Tracking angle characteristics were studied with different SCA layouts and time. • The average energy flux was simulated across four clear days. • Influences of defocus angles for energy flux were analyzed. - Abstract: A dynamic optical model was established for the Yanqing solar field at the parabolic trough solar thermal power plant and a simulation was conducted on four separate days of clear weather (March 3rd, June 2nd, September 25th, December 17th). The solar collector assembly (SCA) was comprised of a North-South and East-West layout. The model consisted of the following modules: DNI, SCA operational, and SCA optical. The tracking angle characteristics were analyzed and the results showed that the East-West layout of the tracking system was the most viable. The average energy flux was simulated for a given time period and different SCA layouts, yielding an average flux of 6 kW/m 2 , which was then used as the design and operational standards of the Yanqing parabolic trough plant. The mass flow of North-South layout was relatively stable. The influences of the defocus angles on both the average energy flux and the circumferential flux distribution were also studied. The results provided a theoretical basis for the following components: solar field design, mass flow control of the heat transfer fluid, design and operation of the tracking system, operational safety of SCAs, and power production prediction in the Yanqing 1 MW parabolic trough plant.

Posterior cerebellar Purkinje cells in an SCA5/SPARCA1 mouse model are especially vulnerable to the synergistic effect of loss of β-III spectrin and GLAST.

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Perkins, Emma M; Suminaite, Daumante; Clarkson, Yvonne L; Lee, Sin Kwan; Lyndon, Alastair R; Rothstein, Jeffrey D; Wyllie, David J A; Tanaka, Kohichi; Jackson, Mandy

2016-10-15

Clinical phenotypes of spinocerebellar ataxia type-5 (SCA5) and spectrin-associated autosomal recessive cerebellar ataxia type-1 (SPARCA1) are mirrored in mice lacking β-III spectrin (β-III-/-). One function of β-III spectrin is the stabilization of the Purkinje cell-specific glutamate transporter EAAT4 at the plasma membrane. In β-III-/- mice EAAT4 levels are reduced from an early age. In contrast levels of the predominant cerebellar glutamate transporter GLAST, expressed in Bergmann glia, only fall progressively from 3 months onwards. Here we elucidated the roles of these two glutamate transporters in cerebellar pathogenesis mediated through loss of β-III spectrin function by studying EAAT4 and GLAST knockout mice as well as crosses of both with β-III-/- mice. Our data demonstrate that EAAT4 loss, but not abnormal AMPA receptor composition, in young β-III-/- mice underlies early Purkinje cell hyper-excitability and that subsequent loss of GLAST, superimposed on the earlier deficiency of EAAT4, is responsible for Purkinje cell loss and progression of motor deficits. Yet the loss of GLAST appears to be independent of EAAT4 loss, highlighting that other aspects of Purkinje cell dysfunction underpin the pathogenic loss of GLAST. Finally, our results demonstrate that Purkinje cells in the posterior cerebellum of β-III-/- mice are most susceptible to the combined loss of EAAT4 and GLAST, with degeneration of proximal dendrites, the site of climbing fibre innervation, most pronounced. This highlights the necessity for efficient glutamate clearance from these regions and identifies dysregulation of glutamatergic neurotransmission particularly within the posterior cerebellum as a key mechanism in SCA5 and SPARCA1 pathogenesis.

GPS and Galileo Developments on Board the International Space Station With the Space Communications and Navigation (SCaN) Testbed

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Pozzobon, Oscar; Fantinato, Samuele; Dalla Chiara, Andrea; Gamba, Giovanni; Crisci, Massimo; Giordana, Pietro; Enderle, Werner; Chelmins, David; Sands, Obed S.; Clapper, Carolyn J.;

A KCNC3 mutation causes a neurodevelopmental, non-progressive SCA13 subtype associated with dominant negative effects and aberrant EGFR trafficking.

Science.gov (United States)

Khare, Swati; Nick, Jerelyn A; Zhang, Yalan; Galeano, Kira; Butler, Brittany; Khoshbouei, Habibeh; Rayaprolu, Sruti; Hathorn, Tyisha; Ranum, Laura P W; Smithson, Lisa; Golde, Todd E; Paucar, Martin; Morse, Richard; Raff, Michael; Simon, Julie; Nordenskjöld, Magnus; Wirdefeldt, Karin; Rincon-Limas, Diego E; Lewis, Jada; Kaczmarek, Leonard K; Fernandez-Funez, Pedro; Nick, Harry S; Waters, Michael F

2017-01-01

The autosomal dominant spinocerebellar ataxias (SCAs) are a diverse group of neurological disorders anchored by the phenotypes of motor incoordination and cerebellar atrophy. Disease heterogeneity is appreciated through varying comorbidities: dysarthria, dysphagia, oculomotor and/or retinal abnormalities, motor neuron pathology, epilepsy, cognitive impairment, autonomic dysfunction, and psychiatric manifestations. Our study focuses on SCA13, which is caused by several allelic variants in the voltage-gated potassium channel KCNC3 (Kv3.3). We detail the clinical phenotype of four SCA13 kindreds that confirm causation of the KCNC3R423H allele. The heralding features demonstrate congenital onset with non-progressive, neurodevelopmental cerebellar hypoplasia and lifetime improvement in motor and cognitive function that implicate compensatory neural mechanisms. Targeted expression of human KCNC3R423H in Drosophila triggers aberrant wing veins, maldeveloped eyes, and fused ommatidia consistent with the neurodevelopmental presentation of patients. Furthermore, human KCNC3R423H expression in mammalian cells results in altered glycosylation and aberrant retention of the channel in anterograde and/or endosomal vesicles. Confirmation of the absence of plasma membrane targeting was based on the loss of current conductance in cells expressing the mutant channel. Mechanistically, genetic studies in Drosophila, along with cellular and biophysical studies in mammalian systems, demonstrate the dominant negative effect exerted by the mutant on the wild-type (WT) protein, which explains dominant inheritance. We demonstrate that ocular co-expression of KCNC3R423H with Drosophila epidermal growth factor receptor (dEgfr) results in striking rescue of the eye phenotype, whereas KCNC3R423H expression in mammalian cells results in aberrant intracellular retention of human epidermal growth factor receptor (EGFR). Together, these results indicate that the neurodevelopmental consequences of

Huntington's disease-like and ataxia syndromes: identification of a family with a de novo SCA17/TBP mutation

DEFF Research Database (Denmark)

Bech, Sara; Petersen, Thor; Nørremølle, Anne

2010-01-01

The autosomal dominant spinocerebellar ataxias, commonly referred to as SCAs, are clinically and genetically heterogeneous neurodegenerative disorders. Twenty-eight genetic subtypes have been identified, of which 7 are caused by expansion of a CAG trinucleotide repeat that encodes a polyglutamine....... The patient's mother and father both carried normal range repeats, 38/38 and 33/39 respectively. Analysis of the repeat structures revealed that the expansion had occurred upon expansion of the longer paternal allele. We conclude that, however rare, SCA17 must be considered as a cause of Huntington's disease...

Hydrological Modelling and data assimilation of Satellite Snow Cover Area using a Land Surface Model, VIC

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S. Naha

2016-06-01

Full Text Available The snow cover plays an important role in Himalayan region as it contributes a useful amount to the river discharge. So, besides estimating rainfall runoff, proper assessment of snowmelt runoff for efficient management and water resources planning is also required. A Land Surface Model, VIC (Variable Infiltration Capacity is used at a high resolution grid size of 1 km. Beas river basin up to Thalot in North West Himalayas (NWH have been selected as the study area. At first model setup is done and VIC has been run in its energy balance mode. The fluxes obtained from VIC has been routed to simulate the discharge for the time period of (2003-2006. Data Assimilation is done for the year 2006 and the techniques of Data Assimilation considered in this study are Direct Insertion (D.I and Ensemble Kalman Filter (EnKF that uses observations of snow covered area (SCA to update hydrologic model states. The meteorological forcings were taken from 0.5 deg. resolution VIC global forcing data from 1979-2006 with daily maximum temperature, minimum temperature from Climate Research unit (CRU, rainfall from daily variability of NCEP and wind speed from NCEP-NCAR analysis as main inputs and Indian Meteorological Department (IMD data of 0.25 °. NBSSLUP soil map and land use land cover map of ISRO-GBP project for year 2014 were used for generating the soil parameters and vegetation parameters respectively. The threshold temperature i.e. the minimum rain temperature is -0.5°C and maximum snow temperature is about +0.5°C at which VIC can generate snow fluxes. Hydrological simulations were done using both NCEP and IMD based meteorological Forcing datasets, but very few snow fluxes were obtained using IMD data met forcing, whereas NCEP based met forcing has given significantly better snow fluxes throughout the simulation years as the temperature resolution as given by IMD data is 0.5°C and rainfall resolution of 0.25°C. The simulated discharge has been validated

Time Distribution Using SpaceWire in the SCaN Testbed on ISS

Science.gov (United States)

Lux, James P.

2012-01-01

A paper describes an approach for timekeeping and time transfer among the devices on the CoNNeCT project s SCaN Testbed. It also describes how the clocks may be synchronized with an external time reference; e.g., time tags from the International Space Station (ISS) or RF signals received by a radio (TDRSS time service or GPS). All the units have some sort of counter that is fed by an oscillator at some convenient frequency. The basic problem in timekeeping is relating the counter value to some external time standard such as UTC. With SpaceWire, there are two approaches possible: one is to just use SpaceWire to send a message, and use an external wire for the sync signal. This is much the same as with the RS- 232 messages and l pps line from a GPS receiver. However, SpaceWire has an additional capability that was added to make it easier - it can insert and receive a special "timecode" word in the data stream.

An open-source software package for multivariate modeling and clustering: applications to air quality management.

Science.gov (United States)

Wang, Xiuquan; Huang, Guohe; Zhao, Shan; Guo, Junhong

2015-09-01

This paper presents an open-source software package, rSCA, which is developed based upon a stepwise cluster analysis method and serves as a statistical tool for modeling the relationships between multiple dependent and independent variables. The rSCA package is efficient in dealing with both continuous and discrete variables, as well as nonlinear relationships between the variables. It divides the sample sets of dependent variables into different subsets (or subclusters) through a series of cutting and merging operations based upon the theory of multivariate analysis of variance (MANOVA). The modeling results are given by a cluster tree, which includes both intermediate and leaf subclusters as well as the flow paths from the root of the tree to each leaf subcluster specified by a series of cutting and merging actions. The rSCA package is a handy and easy-to-use tool and is freely available at http://cran.r-project.org/package=rSCA . By applying the developed package to air quality management in an urban environment, we demonstrate its effectiveness in dealing with the complicated relationships among multiple variables in real-world problems.

Isospin diffusion in binary collisions of 32S+Ca,4840 and 32S+48Ti at 17.7 MeV/nucleon

Science.gov (United States)

Piantelli, S.; Valdré, S.; Barlini, S.; Casini, G.; Colonna, M.; Baiocco, G.; Bini, M.; Bruno, M.; Camaiani, A.; Carboni, S.; Cicerchia, M.; Cinausero, M.; D'Agostino, M.; Degerlier, M.; Fabris, D.; Gelli, N.; Gramegna, F.; Gruyer, D.; Kravchuk, V. L.; Mabiala, J.; Marchi, T.; Morelli, L.; Olmi, A.; Ottanelli, P.; Pasquali, G.; Pastore, G.

2017-09-01

The systems 32S+Ca,4840 and 32S+48Ti at 17.7 MeV/nucleon were investigated with the setup general array for fragment identification and for emitted light particles in dissipative collisions (GARFIELD) plus ring counter (RCo) at Laboratori Nazionali di Legnaro (LNL) of Istituto Nazionale di Fisica Nucleare (INFN). Fusion evaporation (FE), fusion fission (FF), and deep inelastic (DIC) events were identified, also through the comparison with the prediction of a transport model (stochastic mean field, SMF), coupled to GEMINI++ as an afterburner. This work mainly deals with the study of isospin transport phenomena in DIC events. In particular, the isospin diffusion is highlighted by comparing the average isotopic content of the quasiprojectile (QP) remnants observed when the target is the N =Z nucleus 40Ca and when it is the neutron-rich 48Ca. Also, the d /p and t /p ratios for particles forward emitted with respect to the QP were found to increase with increasing N /Z of the target.

A KCNC3 mutation causes a neurodevelopmental, non-progressive SCA13 subtype associated with dominant negative effects and aberrant EGFR trafficking.

Directory of Open Access Journals (Sweden)

Swati Khare

Full Text Available The autosomal dominant spinocerebellar ataxias (SCAs are a diverse group of neurological disorders anchored by the phenotypes of motor incoordination and cerebellar atrophy. Disease heterogeneity is appreciated through varying comorbidities: dysarthria, dysphagia, oculomotor and/or retinal abnormalities, motor neuron pathology, epilepsy, cognitive impairment, autonomic dysfunction, and psychiatric manifestations. Our study focuses on SCA13, which is caused by several allelic variants in the voltage-gated potassium channel KCNC3 (Kv3.3. We detail the clinical phenotype of four SCA13 kindreds that confirm causation of the KCNC3R423H allele. The heralding features demonstrate congenital onset with non-progressive, neurodevelopmental cerebellar hypoplasia and lifetime improvement in motor and cognitive function that implicate compensatory neural mechanisms. Targeted expression of human KCNC3R423H in Drosophila triggers aberrant wing veins, maldeveloped eyes, and fused ommatidia consistent with the neurodevelopmental presentation of patients. Furthermore, human KCNC3R423H expression in mammalian cells results in altered glycosylation and aberrant retention of the channel in anterograde and/or endosomal vesicles. Confirmation of the absence of plasma membrane targeting was based on the loss of current conductance in cells expressing the mutant channel. Mechanistically, genetic studies in Drosophila, along with cellular and biophysical studies in mammalian systems, demonstrate the dominant negative effect exerted by the mutant on the wild-type (WT protein, which explains dominant inheritance. We demonstrate that ocular co-expression of KCNC3R423H with Drosophila epidermal growth factor receptor (dEgfr results in striking rescue of the eye phenotype, whereas KCNC3R423H expression in mammalian cells results in aberrant intracellular retention of human epidermal growth factor receptor (EGFR. Together, these results indicate that the neurodevelopmental

Statutory Interpretation through the Prism of the Bill of Rights: S v Litako 2014 SACR 431 (SCA

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Salona Lutchman

2015-07-01

Full Text Available On 16 April 2014, the Supreme Court of Appeal handed down judgment in the matter of S v Litako 2014 2 SACR 431 (SCA ("Litako". The judgment reconsiders the landmark decision of the same court, S v Ndhlovu 2002 2 SACR 325 (SCA ("Ndhlovu" in which the court held that an informal admission made by one accused could be admitted against a co-accused even if the accused in court denies making the statement and the statement itself is therefore considered to be hearsay. The court in Ndhlovu applied section 3 of the Law of Evidence Amendment Act 45 of 1988 and found that the hearsay extra curial admission could be admitted in the interests of justice. In Litako the court found that section 3 did not overrule an existing common law rule, which is that the extra curial statement of an accused (whether an informal admission or a confession cannot be tendered against a co-accused. This is because section 3 does not expressly overrule this common law rule. Rather, the provision itself requests that its application be subject to the common law. The judgment is important for various reasons. Firstly, it is generally in keeping with the existing rule on the cautionary treatment of accomplice evidence. Secondly, the judgment highlights the current confusion in the relationship between statute and common law with regards to informal admissions and confessions. Thirdly, the court employs methods of statutory interpretation to re-examine the principle from Ndhlovu and finds that the court in that case did not apply its mind correctly in disregarding the common law rule. The court undertook a teleological approach to interpretation by infusing the meaning of the words with the spirit, purport and objects of the Bill of Rights and found that the statute had not overruled the common law rule.

Assessment of Socioeconomic Vulnerability to Floods in the Bâsca Chiojdului Catchment Area

Directory of Open Access Journals (Sweden)

REMUS PRĂVĂLIE

2014-12-01

Full Text Available Hydrological risk phenomena such as floods are among the most costly natural disasters worldwide, effects consisting of socioeconomic damages and deaths. The Bâsca Chiojdului catchment area, by its morphometric and hydrographic peculiarities, is prone to generate these hydrological risk phenomena, so there is a high vulnerability in the socioeconomic elements. This paper is focused on the identification of the main socioeconomic elements vulnerable to hydrological risk phenomena such as floods, based on the assessment of their manifestation potential. Thus, following the delimitation of areas with the highest flood occurrence potential (susceptibility to floods, major socioeconomic factors existing in the basin, considering human settlements (constructions, transport infrastructure, and agricultural areas (the most important category, were superimposed. Results showed a high vulnerability for all three exposed socioeconomic elements especially in valley sectors, of which household structures were the most vulnerable, given both their importance and the high number of areas highly exposed to floods (approximately 2,500 houses and outbuildings, out of a total of about 10,250, intersect the most susceptible area to floods in the study area.

Increased protein kinase C gamma activity induces Purkinje cell pathology in a mouse model of spinocerebellar ataxia 14.

Science.gov (United States)

Ji, Jingmin; Hassler, Melanie L; Shimobayashi, Etsuko; Paka, Nagendher; Streit, Raphael; Kapfhammer, Josef P

2014-10-01

Spinocerebellar ataxias (SCAs) are hereditary diseases leading to Purkinje cell degeneration and cerebellar dysfunction. Most forms of SCA are caused by expansion of CAG repeats similar to other polyglutamine disorders such as Huntington's disease. In contrast, in the autosomal dominant SCA-14 the disease is caused by mutations in the protein kinase C gamma (PKCγ) gene which is a well characterized signaling molecule in cerebellar Purkinje cells. The study of SCA-14, therefore, offers the unique opportunity to reveal the molecular and pathological mechanism eventually leading to Purkinje cell dysfunction and degeneration. We have created a mouse model of SCA-14 in which PKCγ protein with a mutation found in SCA-14 is specifically expressed in cerebellar Purkinje cells. We find that in mice expressing the mutated PKCγ protein the morphology of Purkinje cells in cerebellar slice cultures is drastically altered and mimics closely the morphology seen after pharmacological PKC activation. Similar morphological abnormalities were seen in localized areas of the cerebellum of juvenile transgenic mice in vivo. In adult transgenic mice there is evidence for some localized loss of Purkinje cells but there is no overall cerebellar atrophy. Transgenic mice show a mild cerebellar ataxia revealed by testing on the rotarod and on the walking beam. Our findings provide evidence for both an increased PKCγ activity in Purkinje cells in vivo and for pathological changes typical for cerebellar disease thus linking the increased and dysregulated activity of PKCγ tightly to the development of cerebellar disease in SCA-14 and possibly also in other forms of SCA. Copyright © 2014 Elsevier Inc. All rights reserved.

Coordination of Heat Pumps, Electric Vehicles and AGC for Efficient LFC in a Smart Hybrid Power System via SCA-Based Optimized FOPID Controllers

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Rahmat Khezri

2018-02-01

Full Text Available Due to the high price of fossil fuels, the increased carbon footprint in conventional generation units and the intermittent functionality of renewable units, alternative sources must contribute to the load frequency control (LFC of the power system. To tackle the challenge, dealing with controllable loads, the ongoing study aims at efficient LFC in smart hybrid power systems. To achieve this goal, heat pumps (HPs and electric vehicles (EVs are selected as the most effective controllable loads to contribute to the LFC issue. In this regard, the EVs can be controlled in a bidirectional manner as known charging and discharging states under a smart structure. In addition, regarding the HPs, the power consumption is controllable. As the main task, this paper proposes a fractional order proportional integral differential (FOPID controller for coordinated control of power consumption in HPs, the discharging state in EVs and automatic generation control (AGC. The parameters of the FOPID controllers are optimized simultaneously by the sine cosine algorithm (SCA, which is a new method for optimization problems. In the sequel, four scenarios, including step and random load changes, aggregated intermittent generated power from wind turbines, a random load change scenario and a sensitivity analysis scenario, are selected to demonstrate the efficiency of the proposed SCA-based FOPID controllers in a hybrid two-area power system.

Evaluating MODIS snow products for modelling snowmelt runoff: Case study of the Rio Grande headwaters

Science.gov (United States)

Steele, Caitriana; Dialesandro, John; James, Darren; Elias, Emile; Rango, Albert; Bleiweiss, Max

2017-12-01

Snow-covered area (SCA) is a key variable in the Snowmelt-Runoff Model (SRM) and in other models for simulating discharge from snowmelt. Landsat Thematic Mapper (TM), Enhanced Thematic Mapper (ETM +) or Operational Land Imager (OLI) provide remotely sensed data at an appropriate spatial resolution for mapping SCA in small headwater basins, but the temporal resolution of the data is low and may not always provide sufficient cloud-free dates. The coarser spatial resolution Moderate Resolution Imaging Spectroradiometer (MODIS) offers better temporal resolution and in cloudy years, MODIS data offer the best alternative for mapping snow cover when finer spatial resolution data are unavailable. However, MODIS' coarse spatial resolution (500 m) can obscure fine spatial patterning in snow cover and some MODIS products are not sensitive to end-of-season snow cover. In this study, we aimed to test MODIS snow products for use in simulating snowmelt runoff from smaller headwater basins by a) comparing maps of TM and MODIS-based SCA and b) determining how SRM streamflow simulations are changed by the different estimates of seasonal snow depletion. We compared gridded MODIS snow products (Collection 5 MOD10A1 fractional and binary SCA; SCA derived from Collection 6 MOD10A1 Normalised Difference Snow Index (NDSI) Snow Cover), and the MODIS Snow Covered-Area and Grain size retrieval (MODSCAG) canopy-corrected fractional SCA (SCAMG), with reference SCA maps (SCAREF) generated from binary classification of TM imagery. SCAMG showed strong agreement with SCAREF; excluding true negatives (where both methods agreed no snow was present) the median percent difference between SCAREF and SCAMG ranged between -2.4% and 4.7%. We simulated runoff for each of the four study years using SRM populated with and calibrated for snow depletion curves derived from SCAREF. We then substituted in each of the MODIS-derived depletion curves. With efficiency coefficients ranging between 0.73 and 0.93, SRM

Bone Marrow PDGFR+Sca-1+ Enriched Mesenchymal Stem Cells Support Survival of and Antibody Production by Plasma Cells in vitro through IL-6.

Science.gov (United States)

Kayaba, Atsuko; Itoh-Nakadai, Ari; Niibe, Kunimichi; Shirota, Matsuyuki; Funayama, Ryo; Sugahara-Tobinai, Akiko; Wong, Yi Li; Inui, Masanori; Nakayama, Keiko; Takai, Toshiyuki

2018-02-24

Plasma cells (PCs) acquiring with long lives in bone marrow (BM) play a pivotal role in the humoral arm of immunological memory. The PCs reside in a special BM niche and produce antibodies against past-encountered pathogens or vaccine components for a long time. In BM, cysteine-X-cysteine (CXC) chemokine receptor type 4-expressing PCs and myeloid cells such as dendritic cells are attracted to and held by CXC chemokine ligand 12-secreting stromal cells, where survival of the PCs is supported by soluble factors such as IL-6 and a proliferation-inducing ligand or APRIL produced by neighboring myeloid cells. Although these stromal cells are also supposed to be involved in the support of the survival and antibody production, the full molecular mechanism has not been clarified yet. Here we show that BM PDGFR+Sca-1+ enriched mesenchymal stem cells (MSCs), which can contribute as stromal cells for hematopoietic stem cells, also support in vitro survival of and antibody production by BM PCs. IL-6 produced by MSCs was found to be involved in the support. Immunohistochemistry of BM sections suggested a co-localization of a minor population of PCs with PDGFR+Sca-1+ MSCs in the BM. We also found that the sort-purified MSC preparation was composed of multiple cell groups with different gene expression profiles, as found on single-cell RNA sequencing, to which multiple roles in the in vitro PC support could be attributed.

A Bayesian spatial assimilation scheme for snow coverage observations in a gridded snow model

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S. Kolberg

2006-01-01

Full Text Available A method for assimilating remotely sensed snow covered area (SCA into the snow subroutine of a grid distributed precipitation-runoff model (PRM is presented. The PRM is assumed to simulate the snow state in each grid cell by a snow depletion curve (SDC, which relates that cell's SCA to its snow cover mass balance. The assimilation is based on Bayes' theorem, which requires a joint prior distribution of the SDC variables in all the grid cells. In this paper we propose a spatial model for this prior distribution, and include similarities and dependencies among the grid cells. Used to represent the PRM simulated snow cover state, our joint prior model regards two elevation gradients and a degree-day factor as global variables, rather than describing their effect separately for each cell. This transformation results in smooth normalised surfaces for the two related mass balance variables, supporting a strong inter-cell dependency in their joint prior model. The global features and spatial interdependency in the prior model cause each SCA observation to provide information for many grid cells. The spatial approach similarly facilitates the utilisation of observed discharge. Assimilation of SCA data using the proposed spatial model is evaluated in a 2400 km2 mountainous region in central Norway (61° N, 9° E, based on two Landsat 7 ETM+ images generalized to 1 km2 resolution. An image acquired on 11 May, a week before the peak flood, removes 78% of the variance in the remaining snow storage. Even an image from 4 May, less than a week after the melt onset, reduces this variance by 53%. These results are largely improved compared to a cell-by-cell independent assimilation routine previously reported. Including observed discharge in the updating information improves the 4 May results, but has weak effect on 11 May. Estimated elevation gradients are shown to be sensitive to informational deficits occurring at high altitude, where snowmelt has not started

A stepwise-cluster microbial biomass inference model in food waste composting

International Nuclear Information System (INIS)

Sun Wei; Huang, Guo H.; Zeng Guangming; Qin Xiaosheng; Sun Xueling

2009-01-01

A stepwise-cluster microbial biomass inference (SMI) model was developed through introducing stepwise-cluster analysis (SCA) into composting process modeling to tackle the nonlinear relationships among state variables and microbial activities. The essence of SCA is to form a classification tree based on a series of cutting or mergence processes according to given statistical criteria. Eight runs of designed experiments in bench-scale reactors in a laboratory were constructed to demonstrate the feasibility of the proposed method. The results indicated that SMI could help establish a statistical relationship between state variables and composting microbial characteristics, where discrete and nonlinear complexities exist. Significance levels of cutting/merging were provided such that the accuracies of the developed forecasting trees were controllable. Through an attempted definition of input effects on the output in SMI, the effects of the state variables on thermophilic bacteria were ranged in a descending order as: Time (day) > moisture content (%) > ash content (%, dry) > Lower Temperature (deg. C) > pH > NH 4 + -N (mg/Kg, dry) > Total N (%, dry) > Total C (%, dry); the effects on mesophilic bacteria were ordered as: Time > Upper Temperature (deg. C) > Total N > moisture content > NH 4 + -N > Total C > pH. This study made the first attempt in applying SCA to mapping the nonlinear and discrete relationships in composting processes.

Evaluation, Calibration and Comparison of the Precipitation-Runoff Modeling System (PRMS) National Hydrologic Model (NHM) Using Moderate Resolution Imaging Spectroradiometer (MODIS) and Snow Data Assimilation System (SNODAS) Gridded Datasets

Science.gov (United States)

Norton, P. A., II; Haj, A. E., Jr.

2014-12-01

The United States Geological Survey is currently developing a National Hydrologic Model (NHM) to support and facilitate coordinated and consistent hydrologic modeling efforts at the scale of the continental United States. As part of this effort, the Geospatial Fabric (GF) for the NHM was created. The GF is a database that contains parameters derived from datasets that characterize the physical features of watersheds. The GF was used to aggregate catchments and flowlines defined in the National Hydrography Dataset Plus dataset for more than 100,000 hydrologic response units (HRUs), and to establish initial parameter values for input to the Precipitation-Runoff Modeling System (PRMS). Many parameter values are adjusted in PRMS using an automated calibration process. Using these adjusted parameter values, the PRMS model estimated variables such as evapotranspiration (ET), potential evapotranspiration (PET), snow-covered area (SCA), and snow water equivalent (SWE). In order to evaluate the effectiveness of parameter calibration, and model performance in general, several satellite-based Moderate Resolution Imaging Spectroradiometer (MODIS) and Snow Data Assimilation System (SNODAS) gridded datasets including ET, PET, SCA, and SWE were compared to PRMS-simulated values. The MODIS and SNODAS data were spatially averaged for each HRU, and compared to PRMS-simulated ET, PET, SCA, and SWE values for each HRU in the Upper Missouri River watershed. Default initial GF parameter values and PRMS calibration ranges were evaluated. Evaluation results, and the use of MODIS and SNODAS datasets to update GF parameter values and PRMS calibration ranges, are presented and discussed.

Accuracy assessment of a net radiation and temperature index snowmelt model using ground observations of snow water equivalent in an alpine basin

Science.gov (United States)

Molotch, N. P.; Painter, T. H.; Bales, R. C.; Dozier, J.

2003-04-01

In this study, an accumulated net radiation / accumulated degree-day index snowmelt model was coupled with remotely sensed snow covered area (SCA) data to simulate snow cover depletion and reconstruct maximum snow water equivalent (SWE) in the 19.1-km2 Tokopah Basin of the Sierra Nevada, California. Simple net radiation snowmelt models are attractive for operational snowmelt runoff forecasts as they are computationally inexpensive and have low input requirements relative to physically based energy balance models. The objective of this research was to assess the accuracy of a simple net radiation snowmelt model in a topographically heterogeneous alpine environment. Previous applications of net radiation / temperature index snowmelt models have not been evaluated in alpine terrain with intensive field observations of SWE. Solar radiation data from two meteorological stations were distributed using the topographic radiation model TOPORAD. Relative humidity and temperature data were distributed based on the lapse rate calculated between three meteorological stations within the basin. Fractional SCA data from the Landsat Enhanced Thematic Mapper (5 acquisitions) and the Airborne Visible and Infrared Imaging Spectrometer (AVIRIS) (2 acquisitions) were used to derive daily SCA using a linear regression between acquisition dates. Grain size data from AVIRIS (4 acquisitions) were used to infer snow surface albedo and interpolated linearly with time to derive daily albedo values. Modeled daily snowmelt rates for each 30-m pixel were scaled by the SCA and integrated over the snowmelt season to obtain estimates of maximum SWE accumulation. Snow surveys consisting of an average of 335 depth measurements and 53 density measurements during April, May and June, 1997 were interpolated using a regression tree / co-krig model, with independent variables of average incoming solar radiation, elevation, slope and maximum upwind slope. The basin was clustered into 7 elevation / average

Global burden of sickle cell anaemia in children under five, 2010-2050: modelling based on demographics, excess mortality, and interventions.

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Frédéric B Piel

Full Text Available The global burden of sickle cell anaemia (SCA is set to rise as a consequence of improved survival in high-prevalence low- and middle-income countries and population migration to higher-income countries. The host of quantitative evidence documenting these changes has not been assembled at the global level. The purpose of this study is to estimate trends in the future number of newborns with SCA and the number of lives that could be saved in under-five children with SCA by the implementation of different levels of health interventions.First, we calculated projected numbers of newborns with SCA for each 5-y interval between 2010 and 2050 by combining estimates of national SCA frequencies with projected demographic data. We then accounted for under-five mortality (U5m projections and tested different levels of excess mortality for children with SCA, reflecting the benefits of implementing specific health interventions for under-five patients in 2015, to assess the number of lives that could be saved with appropriate health care services. The estimated number of newborns with SCA globally will increase from 305,800 (confidence interval [CI]: 238,400-398,800 in 2010 to 404,200 (CI: 242,500-657,600 in 2050. It is likely that Nigeria (2010: 91,000 newborns with SCA [CI: 77,900-106,100]; 2050: 140,800 [CI: 95,500-200,600] and the Democratic Republic of the Congo (2010: 39,700 [CI: 32,600-48,800]; 2050: 44,700 [CI: 27,100-70,500] will remain the countries most in need of policies for the prevention and management of SCA. We predict a decrease in the annual number of newborns with SCA in India (2010: 44,400 [CI: 33,700-59,100]; 2050: 33,900 [CI: 15,900-64,700]. The implementation of basic health interventions (e.g., prenatal diagnosis, penicillin prophylaxis, and vaccination for SCA in 2015, leading to significant reductions in excess mortality among under-five children with SCA, could, by 2050, prolong the lives of 5,302,900 [CI: 3

Global burden of sickle cell anaemia in children under five, 2010-2050: modelling based on demographics, excess mortality, and interventions.

Science.gov (United States)

Piel, Frédéric B; Hay, Simon I; Gupta, Sunetra; Weatherall, David J; Williams, Thomas N

2013-01-01

The global burden of sickle cell anaemia (SCA) is set to rise as a consequence of improved survival in high-prevalence low- and middle-income countries and population migration to higher-income countries. The host of quantitative evidence documenting these changes has not been assembled at the global level. The purpose of this study is to estimate trends in the future number of newborns with SCA and the number of lives that could be saved in under-five children with SCA by the implementation of different levels of health interventions. First, we calculated projected numbers of newborns with SCA for each 5-y interval between 2010 and 2050 by combining estimates of national SCA frequencies with projected demographic data. We then accounted for under-five mortality (U5m) projections and tested different levels of excess mortality for children with SCA, reflecting the benefits of implementing specific health interventions for under-five patients in 2015, to assess the number of lives that could be saved with appropriate health care services. The estimated number of newborns with SCA globally will increase from 305,800 (confidence interval [CI]: 238,400-398,800) in 2010 to 404,200 (CI: 242,500-657,600) in 2050. It is likely that Nigeria (2010: 91,000 newborns with SCA [CI: 77,900-106,100]; 2050: 140,800 [CI: 95,500-200,600]) and the Democratic Republic of the Congo (2010: 39,700 [CI: 32,600-48,800]; 2050: 44,700 [CI: 27,100-70,500]) will remain the countries most in need of policies for the prevention and management of SCA. We predict a decrease in the annual number of newborns with SCA in India (2010: 44,400 [CI: 33,700-59,100]; 2050: 33,900 [CI: 15,900-64,700]). The implementation of basic health interventions (e.g., prenatal diagnosis, penicillin prophylaxis, and vaccination) for SCA in 2015, leading to significant reductions in excess mortality among under-five children with SCA, could, by 2050, prolong the lives of 5,302,900 [CI: 3,174,800-6,699,100] newborns with

High spatial resolution radiation budget for Europe: derived from satellite data, validation of a regional model; Raeumlich hochaufgeloeste Strahlungsbilanz ueber Europa: Ableitung aus Satellitendaten, Validation eines regionalen Modells

Energy Technology Data Exchange (ETDEWEB)

Hollmann, R. [GKSS-Forschungszentrum Geesthacht GmbH (Germany). Inst. fuer Atmosphaerenphysik

2000-07-01

Since forty years instruments onboard satellites have been demonstrated their usefulness for many applications in the field of meteorology and oceanography. Several experiments, like ERBE, are dedicated to establish a climatology of the global Earth radiation budget at the top of the atmosphere. Now the focus has been changed to the regional scale, e.g. GEWEX with its regional sub-experiments like BALTEX. To obtain a regional radiation budget for Europe in the first part of the work the well calibrated measurements from ScaRaB (scanner for radiation budget) are used to derive a narrow-to-broadband conversion, which is applicable to the AVHRR (advanced very high resolution radiometer). It is shown, that the accuracy of the method is in the order of that from SCaRaB itself. In the second part of the work, results of REMO have been compared with measurements of ScaRaB and AVHRR for March 1994. The model reproduces the measurements overall well, but it is overestimating the cold areas and underestimating the warm areas in the longwave spectral domain. Similarly it is overestimating the dark areas and underestimating the bright areas in the solar spectral domain. (orig.)

Research progress of spinocerebellar ataxia type 1

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Lin-wei ZHANG

2014-05-01

Full Text Available Spinocerebellar ataxia type 1 (SCA1 is a kind of autosomal dominant genetic neurodegenerative disorder. To date, the pathogenesis of SCA1 remains unclear. Studies in numerous SCA1 experimental models, including transgenic mice, transgenic drosophila and induced pluripotent stem cells, have shown that phosphorylation of S776 in mutant ataxin-1, molecular chaperones, ubiquitin-proteasome system and down-regulation of several components of RAS-MAPK-MSK1 pathway may involve in the pathogenesis of SCA1. In this review, the clinical and pathological features of SCA1, and the latest advances of pathogenesis, model systems and therapeutic exploration will be briefly summarized. doi: 10.3969/j.issn.1672-6731.2014.05.017

Plant model of KIPT neutron source facility simulator

International Nuclear Information System (INIS)

Cao, Yan; Wei, Thomas Y.; Grelle, Austin L.; Gohar, Yousry

2016-01-01

Argonne National Laboratory (ANL) of the United States and Kharkov Institute of Physics and Technology (KIPT) of Ukraine are collaborating on constructing a neutron source facility at KIPT, Kharkov, Ukraine. The facility has 100-kW electron beam driving a subcritical assembly (SCA). The electron beam interacts with a natural uranium target or a tungsten target to generate neutrons, and deposits its power in the target zone. The total fission power generated in SCA is about 300 kW. Two primary cooling loops are designed to remove 100-kW and 300-kW from the target zone and the SCA, respectively. A secondary cooling system is coupled with the primary cooling system to dispose of the generated heat outside the facility buildings to the atmosphere. In addition, the electron accelerator has a low efficiency for generating the electron beam, which uses another secondary cooling loop to remove the generated heat from the accelerator primary cooling loop. One of the main functions the KIPT neutron source facility is to train young nuclear specialists; therefore, ANL has developed the KIPT Neutron Source Facility Simulator for this function. In this simulator, a Plant Control System and a Plant Protection System were developed to perform proper control and to provide automatic protection against unsafe and improper operation of the facility during the steady-state and the transient states using a facility plant model. This report focuses on describing the physics of the plant model and provides several test cases to demonstrate its capabilities. The plant facility model uses the PYTHON script language. It is consistent with the computer language of the plant control system. It is easy to integrate with the simulator without an additional interface, and it is able to simulate the transients of the cooling systems with system control variables changing on real-time.

Plant model of KIPT neutron source facility simulator

Energy Technology Data Exchange (ETDEWEB)

Cao, Yan [Argonne National Lab. (ANL), Argonne, IL (United States); Wei, Thomas Y. [Argonne National Lab. (ANL), Argonne, IL (United States); Grelle, Austin L. [Argonne National Lab. (ANL), Argonne, IL (United States); Gohar, Yousry [Argonne National Lab. (ANL), Argonne, IL (United States)

2016-02-01

Argonne National Laboratory (ANL) of the United States and Kharkov Institute of Physics and Technology (KIPT) of Ukraine are collaborating on constructing a neutron source facility at KIPT, Kharkov, Ukraine. The facility has 100-kW electron beam driving a subcritical assembly (SCA). The electron beam interacts with a natural uranium target or a tungsten target to generate neutrons, and deposits its power in the target zone. The total fission power generated in SCA is about 300 kW. Two primary cooling loops are designed to remove 100-kW and 300-kW from the target zone and the SCA, respectively. A secondary cooling system is coupled with the primary cooling system to dispose of the generated heat outside the facility buildings to the atmosphere. In addition, the electron accelerator has a low efficiency for generating the electron beam, which uses another secondary cooling loop to remove the generated heat from the accelerator primary cooling loop. One of the main functions the KIPT neutron source facility is to train young nuclear specialists; therefore, ANL has developed the KIPT Neutron Source Facility Simulator for this function. In this simulator, a Plant Control System and a Plant Protection System were developed to perform proper control and to provide automatic protection against unsafe and improper operation of the facility during the steady-state and the transient states using a facility plant model. This report focuses on describing the physics of the plant model and provides several test cases to demonstrate its capabilities. The plant facility model uses the PYTHON script language. It is consistent with the computer language of the plant control system. It is easy to integrate with the simulator without an additional interface, and it is able to simulate the transients of the cooling systems with system control variables changing on real-time.

Comparative Analysis of Pain Behaviours in Humanized Mouse Models of Sickle Cell Anemia.

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Jianxun Lei

Full Text Available Pain is a hallmark feature of sickle cell anemia (SCA but management of chronic as well as acute pain remains a major challenge. Mouse models of SCA are essential to examine the mechanisms of pain and develop novel therapeutics. To facilitate this effort, we compared humanized homozygous BERK and Townes sickle mice for the effect of gender and age on pain behaviors. Similar to previously characterized BERK sickle mice, Townes sickle mice show more mechanical, thermal, and deep tissue hyperalgesia with increasing age. Female Townes sickle mice demonstrate more hyperalgesia compared to males similar to that reported for BERK mice and patients with SCA. Mechanical, thermal and deep tissue hyperalgesia increased further after hypoxia/reoxygenation (H/R treatment in Townes sickle mice. Together, these data show BERK sickle mice exhibit a significantly greater degree of hyperalgesia for all behavioral measures as compared to gender- and age-matched Townes sickle mice. However, the genetically distinct "knock-in" strategy of human α and β transgene insertion in Townes mice as compared to BERK mice, may provide relative advantage for further genetic manipulations to examine specific mechanisms of pain.

Improvement of distributed snowmelt energy balance modeling with MODIS-based NDSI-derived fractional snow-covered area data

Science.gov (United States)

Joel W. Homan; Charles H. Luce; James P. McNamara; Nancy F. Glenn

2011-01-01

Describing the spatial variability of heterogeneous snowpacks at a watershed or mountain-front scale is important for improvements in large-scale snowmelt modelling. Snowmelt depletion curves, which relate fractional decreases in snowcovered area (SCA) against normalized decreases in snow water equivalent (SWE), are a common approach to scale-up snowmelt models....

Automated home cage assessment shows behavioral changes in a transgenic mouse model of spinocerebellar ataxia type 17.

Science.gov (United States)

Portal, Esteban; Riess, Olaf; Nguyen, Huu Phuc

2013-08-01

Spinocerebellar Ataxia type 17 (SCA17) is an autosomal dominantly inherited, neurodegenerative disease characterized by ataxia, involuntary movements, and dementia. A novel SCA17 mouse model having a 71 polyglutamine repeat expansion in the TATA-binding protein (TBP) has shown age related motor deficit using a classic motor test, yet concomitant weight increase might be a confounding factor for this measurement. In this study we used an automated home cage system to test several motor readouts for this same model to confirm pathological behavior results and evaluate benefits of automated home cage in behavior phenotyping. Our results confirm motor deficits in the Tbp/Q71 mice and present previously unrecognized behavioral characteristics obtained from the automated home cage, indicating its use for high-throughput screening and testing, e.g. of therapeutic compounds. Copyright © 2013 Elsevier B.V. All rights reserved.

The doctrine of quasi-mutual assent - has it become the general rule for the formation of contracts? The case of Pillay v Shaik 2009 4 SA 74 (SCA)

OpenAIRE

Thejane, P

2012-01-01

The doctrine of quasi-mutual assent is undoubtedly part of our South African law and has been affirmed and applied in a number of leading decisions. The purpose of this note is to offer a critical analysis of the application of the doctrine in the case of Pillay v Shaik 2009 4 SA 74 (SCA). It is argued that the primary basis of contractual liability in South Africa has always been and still remains consensus ad idem as determined in terms of the rules relating to offer and acceptance It is al...

Integrating stomach content and stable isotope analyses to quantify the diets of pygoscelid penguins.

Directory of Open Access Journals (Sweden)

Michael J Polito

Full Text Available Stomach content analysis (SCA and more recently stable isotope analysis (SIA integrated with isotopic mixing models have become common methods for dietary studies and provide insight into the foraging ecology of seabirds. However, both methods have drawbacks and biases that may result in difficulties in quantifying inter-annual and species-specific differences in diets. We used these two methods to simultaneously quantify the chick-rearing diet of Chinstrap (Pygoscelis antarctica and Gentoo (P. papua penguins and highlight methods of integrating SCA data to increase accuracy of diet composition estimates using SIA. SCA biomass estimates were highly variable and underestimated the importance of soft-bodied prey such as fish. Two-source, isotopic mixing model predictions were less variable and identified inter-annual and species-specific differences in the relative amounts of fish and krill in penguin diets not readily apparent using SCA. In contrast, multi-source isotopic mixing models had difficulty estimating the dietary contribution of fish species occupying similar trophic levels without refinement using SCA-derived otolith data. Overall, our ability to track inter-annual and species-specific differences in penguin diets using SIA was enhanced by integrating SCA data to isotopic mixing modes in three ways: 1 selecting appropriate prey sources, 2 weighting combinations of isotopically similar prey in two-source mixing models and 3 refining predicted contributions of isotopically similar prey in multi-source models.

Parkinsonian phenotype in Machado-Joseph disease (MJD/SCA3: a two-case report

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Vasconcelos João

2011-10-01

Full Text Available Abstract Background Machado-Joseph disease (MJD, or spinocerebellar ataxia type 3 (SCA3, is an autosomal dominant neurodegenerative disorder of late onset, which is caused by a CAG repeat expansion in the coding region of the ATXN3 gene. This disease presents clinical heterogeneity, which cannot be completely explained by the size of the repeat tract. MJD presents extrapyramidal motor signs, namely Parkinsonism, more frequently than the other subtypes of autosomal dominant cerebellar ataxias. Although Parkinsonism seems to segregate within MJD families, only a few MJD patients develop parkinsonian features and, therefore, the clinical and genetic aspects of these rare presentations remain poorly investigated. The main goal of this work was to describe two MJD patients displaying the parkinsonian triad (tremor, bradykinesia and rigidity, namely on what concerns genetic variation in Parkinson's disease (PD associated loci (PARK2, LRRK2, PINK1, DJ-1, SNCA, MAPT, APOE, and mtDNA tRNAGln T4336C. Case presentation Patient 1 is a 40 year-old female (onset at 30 years of age, initially with a pure parkinsonian phenotype (similar to the phenotype previously reported for her mother. Patient 2 is a 38 year-old male (onset at 33 years of age, presenting an ataxic phenotype with parkinsonian features (not seen either in other affected siblings or in his father. Both patients presented an expanded ATXN3 allele with 72 CAG repeats. No PD mutations were found in the analyzed loci. However, allelic variants previously associated with PD were observed in DJ-1 and APOE genes, for both patients. Conclusions The present report adds clinical and genetic information on this particular and rare MJD presentation, and raises the hypothesis that DJ-1 and APOE polymorphisms may confer susceptibility to the parkinsonian phenotype in MJD.

Protective Effect of Ginsenoside Rg1 on Hematopoietic Stem/Progenitor Cells through Attenuating Oxidative Stress and the Wnt/β-Catenin Signaling Pathway in a Mouse Model of d-Galactose-induced Aging.

Science.gov (United States)

Li, Jing; Cai, Dachuan; Yao, Xin; Zhang, Yanyan; Chen, Linbo; Jing, Pengwei; Wang, Lu; Wang, Yaping

2016-06-09

Stem cell senescence is an important and current hypothesis accounting for organismal aging, especially the hematopoietic stem cell (HSC). Ginsenoside Rg1 is the main active pharmaceutical ingredient of ginseng, which is a traditional Chinese medicine. This study explored the protective effect of ginsenoside Rg1 on Sca-1⁺ hematopoietic stem/progenitor cells (HSC/HPCs) in a mouse model of d-galactose-induced aging. The mimetic aging mouse model was induced by continuous injection of d-gal for 42 days, and the C57BL/6 mice were respectively treated with ginsenoside Rg1, Vitamin E or normal saline after 7 days of d-gal injection. Compared with those in the d-gal administration alone group, ginsenoside Rg1 protected Sca-1⁺ HSC/HPCs by decreasing SA-β-Gal and enhancing the colony forming unit-mixture (CFU-Mix), and adjusting oxidative stress indices like reactive oxygen species (ROS), total anti-oxidant (T-AOC), superoxide dismutase (SOD), glutathione peroxidase (GSH-px) and malondialdehyde (MDA). In addition, ginsenoside Rg1 decreased β-catenin and c-Myc mRNA expression and enhanced the phosphorylation of GSK-3β. Moreover, ginsenoside Rg1 down-regulated advanced glycation end products (AGEs), 4-hydroxynonenal (4-HNE), phospho-histone H2A.X (r-H2A.X), 8-OHdG, p16(Ink4a), Rb, p21(Cip1/Waf1) and p53 in senescent Sca-1⁺ HSC/HPCs. Our findings indicated that ginsenoside Rg1 can improve the resistance of Sca-1⁺ HSC/HPCs in a mouse model of d-galactose-induced aging through the suppression of oxidative stress and excessive activation of the Wnt/β-catenin signaling pathway, and reduction of DNA damage response, p16(Ink4a)-Rb and p53-p21(Cip1/Waf1) signaling.

Relationship between the clinical global impression of severity for schizoaffective disorder scale and established mood scales for mania and depression.

Science.gov (United States)

Turkoz, Ibrahim; Fu, Dong-Jing; Bossie, Cynthia A; Sheehan, John J; Alphs, Larry

2013-08-15

This analysis explored the relationship between ratings on HAM-D-17 or YMRS and those on the depressive or manic subscale of CGI-S for schizoaffective disorder (CGI-S-SCA). This post hoc analysis used the database (N=614) from two 6-week, randomized, placebo-controlled studies of paliperidone ER versus placebo in symptomatic subjects with schizoaffective disorder assessed using HAM-D-17, YMRS, and CGI-S-SCA scales. Parametric and nonparametric regression models explored the relationships between ratings on YMRS and HAM-D-17 and on depressive and manic domains of the CGI-S-SCA from baseline to the 6-week end point. A clinically meaningful improvement was defined as a change of 1 point in the CGI-S-SCA score. No adjustment was made for multiplicity. Multiple linear regression models suggested that a 1-point change in the depressive domain of CGI-S-SCA corresponded to an average 3.6-point (SE=0.2) change in HAM-D-17 score. Similarly, a 1-point change in the manic domain of CGI-S-SCA corresponded to an average 5.8-point (SE=0.2) change in YMRS score. Results were confirmed using local and cumulative logistic regression models in addition to equipercentile linking. Lack of subjects scoring over the complete range of possible scores may limit broad application of the analyses. Clinically meaningful score changes in depressive and manic domains of CGI-S-SCA corresponded to approximately 4- and 6-point score changes on HAM-D-17 and YMRS, respectively, in symptomatic subjects with schizoaffective disorder. Copyright © 2013 Elsevier B.V. All rights reserved.

L and M shell coulomb ionization by heavy charged projectiles

International Nuclear Information System (INIS)

Karmaker, R.

1980-01-01

Universal cross sections for L and M shell ionization have been extracted from the semiclassical approximation (SCA) model in the straight line path approximation of the projectile. It has been shown that it is possible to organise the calculation of the SCA in a suitable way so that it is not necessary to calculate the cross section for different targets. The agreement between the theoretical curve in the SCA model and the available experimental data for different target elements, is reasonably good. Cross sections for L and M shell ionization in the straight line path of the projectile in the SCA model for Pb, Au and U targets by the impact of protons have been calculated. The results have been compared with those calculated in the Binary Encounter Approximation (BEA) and the Plane Wave Born Approximation (PWBA) as well as with the available experimental results. The present calculations are in good agreement with the existing theoretical and the experimental results. (author)

Compound risk: History of traumatic stress predicts posttraumatic stress disorder symptoms and severity in sudden cardiac arrest survivors.

Science.gov (United States)

Rosman, Lindsey; Ford, Jessica; Whited, Amanda; Cahill, John; Lampert, Rachel; Mosesso, Vincent N; Lawless, Christine; Sears, Samuel F

2016-08-01

Sudden cardiac arrest (SCA) survivors can develop posttraumatic stress disorder (PTSD) which is associated with worse clinical outcomes. The purpose of this study was to evaluate the prevalence and predictors of PTSD in a large sample of SCA survivors. Prior history of psychological trauma and the effects of repeated trauma exposure on subsequent PTSD and symptom severity after SCA were also explored. A retrospective, cross-sectional study of 188 SCA survivors from the Sudden Cardiac Arrest Association patient registry completed an online questionnaire that included measures of PTSD, trauma history, sociodemographics, general health, and cardiac history. Sixty-three (36.2%) SCA survivors in this sample scored above the clinical cutoff for PTSD. Female gender, worse general health, and younger age predicted PTSD symptoms after SCA. Additionally, 50.2% of SCA survivors (n = 95) reported a history of trauma exposure and 25.4% (n = 48) of the total sample endorsed a traumatic stress response to a historic trauma. Results indicated that a traumatic stress response to a historic trauma was a stronger predictor of PTSD after SCA (odds ratio = 4.77) than all other variables in the model. PTSD symptoms are present in over one-third of SCA survivors. While demographic or health history variables predicted PTSD after SCA, a history of traumatic stress response to a previous trauma emerged as the strongest predictor of these symptoms. Routine assessment and interdisciplinary management are discussed as potential ways to expedite survivors' recovery and return to daily living. © The European Society of Cardiology 2015.

Semiotic-conceptual analysis: a proposal

Science.gov (United States)

Priss, Uta

2017-07-01

This paper provides the basic definitions of Semiotic-conceptual analysis (SCA), which is a mathematical modelling of signs as elements of a triadic relation. FCA concept lattices are constructed for each of the three sign components. It is demonstrated how core linguistic and semiotic notions (such as synonymy and icon) can be represented with SCA. While the usefulness of SCA has already been demonstrated in a number of applications and several propositions are proven in this paper, there are still many open questions as to what to do next with SCA. Therefore, this paper is meant as a proposal and encouragement for further development.

Investigation of a calcium-responsive contrast agent in cellular model systems: feasibility for use as a smart molecular probe in functional MRI.

Science.gov (United States)

Angelovski, Goran; Gottschalk, Sven; Milošević, Milena; Engelmann, Jörn; Hagberg, Gisela E; Kadjane, Pascal; Andjus, Pavle; Logothetis, Nikos K

2014-05-21

Responsive or smart contrast agents (SCAs) represent a promising direction for development of novel functional MRI (fMRI) methods for the eventual noninvasive assessment of brain function. In particular, SCAs that respond to Ca(2+) may allow tracking neuronal activity independent of brain vasculature, thus avoiding the characteristic limitations of current fMRI techniques. Here we report an in vitro proof-of-principle study with a Ca(2+)-sensitive, Gd(3+)-based SCA in an attempt to validate its potential use as a functional in vivo marker. First, we quantified its relaxometric response in a complex 3D cell culture model. Subsequently, we examined potential changes in the functionality of primary glial cells following administration of this SCA. Monitoring intracellular Ca(2+) showed that, despite a reduction in the Ca(2+) level, transport of Ca(2+) through the plasma membrane remained unaffected, while stimulation with ATP induced Ca(2+)-transients suggested normal cellular signaling in the presence of low millimolar SCA concentrations. SCAs merely lowered the intracellular Ca(2+) level. Finally, we estimated the longitudinal relaxation times (T1) for an idealized in vivo fMRI experiment with SCA, for extracellular Ca(2+) concentration level changes expected during intense neuronal activity which takes place upon repetitive stimulation. The values we obtained indicate changes in T1 of around 1-6%, sufficient to be robustly detectable using modern MRI methods in high field scanners. Our results encourage further attempts to develop even more potent SCAs and appropriate fMRI protocols. This would result in novel methods that allow monitoring of essential physiological processes at the cellular and molecular level.

Simplified scaling model for the THETA-pinch

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Ewing, K. J.; Thomson, D. B.

1982-02-01

A simple ID scaing model for the fast Theta pinch was developed and written as a code that would be flexible, inexpensive in computer time, and readily available for use with the Los Alamos explosive-driven high magnetic field program. The simplified model uses three successive separate stages: (1) a snowplow-like radial implosion, (2) an idealized resistive annihilation of reverse bias field, and (3) an adiabatic compression stage of a Beta = 1 plasma for which ideal pressure balance is assumed to hold. The code uses one adjustable fitting constant whose value was first determined by comparison with results from the Los Alamos Scylla III, Scyllacita, and Scylla IA Theta pinches.

Aqueous Extract of Paeonia lactiflora and Paeoniflorin as Aggregation Reducers Targeting Chaperones in Cell Models of Spinocerebellar Ataxia 3

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Kuo-Hsuan Chang

2013-01-01

Full Text Available Spinocerebellar ataxia (SCA types 1, 2, 3, 6, 7, and 17 as well as Huntington’s disease are a group of neurodegenerative disorders caused by expanded CAG repeats encoding a long polyglutamine (polyQ tract in the respective proteins. Evidence has shown that the accumulation of intranuclear and cytoplasmic misfolded polyQ proteins leads to apoptosis and cell death. Thus suppression of aggregate formation is expected to inhibit a wide range of downstream pathogenic events in polyQ diseases. In this study, we established a high-throughput aggregation screening system using 293 ATXN3/Q75-GFP cells and applied this system to test the aqueous extract of Paeonia lactiflora (P. lactiflora and its constituents. We found that the aggregation can be significantly prohibited by P. lactiflora and its active compound paeoniflorin. Meanwhile, P. lactiflora and paeoniflorin upregulated HSF1 and HSP70 chaperones in the same cell models. Both of them further reduced the aggregation in neuronal differentiated SH-SY5Y ATXN3/Q75-GFP cells. Our results demonstrate how P. lactiflora and paeoniflorin are likely to work on polyQ-aggregation reduction and provide insight into the possible working mechanism of P. lactiflora in SCA3. We anticipate our paper to be a starting point for screening more potential herbs for the treatment of SCA3 and other polyQ diseases.

Long-term variability in Northern Hemisphere snow cover and associations with warmer winters

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McCabe, Gregory J.; Wolock, David M.

2010-01-01

A monthly snow accumulation and melt model is used with gridded monthly temperature and precipitation data for the Northern Hemisphere to generate time series of March snow-covered area (SCA) for the period 1905 through 2002. The time series of estimated SCA for March is verified by comparison with previously published time series of SCA for the Northern Hemisphere. The time series of estimated Northern Hemisphere March SCA shows a substantial decrease since about 1970, and this decrease corresponds to an increase in mean winter Northern Hemisphere temperature. The increase in winter temperature has caused a decrease in the fraction of precipitation that occurs as snow and an increase in snowmelt for some parts of the Northern Hemisphere, particularly the mid-latitudes, thus reducing snow packs and March SCA. In addition, the increase in winter temperature and the decreases in SCA appear to be associated with a contraction of the circumpolar vortex and a poleward movement of storm tracks, resulting in decreased precipitation (and snow) in the low- to mid-latitudes and an increase in precipitation (and snow) in high latitudes. If Northern Hemisphere winter temperatures continue to warm as they have since the 1970s, then March SCA will likely continue to decrease.

Quantifying the performance of two conceptual models for snow dominated catchments in Austria and Turkey

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Sensoy, Aynur; Parajka, Juraj; Coskun, Cihan; Sorman, Arda; Ertas, Cansaran

2014-05-01

In many mountainous regions, snowmelt makes significant contribution to streamflow, particularly during spring and summer months. Understanding the magnitude and timing of this contribution and hydrological forecasts are essential for a range of purposes concerning the implications with water resources management. Conceptual hydrological models have been widely applied for mountain catchments both for operational and scientific applications. Hydrologiska Byran Vattenbalansavdelning (HBV) and Snowmelt Runoff Model (SRM) are selected in this study as the commonly used conceptual models in hydrological modeling forecasting for a number of basins in several countries. Moreover, this selection is also supported by the experiences on the improvement and application in remote sensing techniques in snow dominated regions. The greatest similarity between the two models is that each uses a temperature index method to predict melt rate whereas the greatest difference lies in the way snow cover is handled. In mountainous regions, data limitations prevent detailed understanding of the variability of snow cover and melt. In situ snowpack measurements are sparsely distributed relative to snowpack heterogeneity therefore, to supplement ground measurements; remotely sensed images of snow covered area (SCA) provide useful information for runoff prediction during the snowmelt season. SCA has been used as a direct input to SRM and as a means of checking the internal validity for HBV model. Moderate Resolution Imaging Spectroradiometer (MODIS) daily snow cover products with 500 m spatial resolution are used to derive SCA data in this study. A number of studies have been reported in the literature indicated that the model performance can vary depending on several factors, including the scale and characteristics of the catchment, availability of the data required and runoff producing mechanism. Therefore, five different catchments including data scare and rich basins, areas and reliefs

Evaluation of sexual maturity among adolescent male sickle cell ...

African Journals Online (AJOL)

Tanner staging and testicular volume assessment were both used as models for evaluating stages of sexual maturation among SCA patients and their normal counterparts matched for age and socioeconomic status. Results. SCA patients showed delayed onset and completion of sexual maturation. TV of subjects was ...

Association of leisure time physical activity and sports competition activities with high blood pressure levels: study carried out in a sample of Portuguese children and adolescents.

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Gaya, A R; Silva, P; Martins, C; Gaya, A; Ribeiro, J C; Mota, J

2011-05-01

A better understanding of the different domains and characteristics of children's and adolescent's physical activity (PA) could be a strategy to clarify the association of this behaviour with the early development of cardiovascular risk factors. To examine the relationship between leisure time physical activity (LTPA) and sports competition activities (SCA) with high blood pressure (HBP) levels in a sample of children and adolescents from Porto, Portugal. This is a cross-sectional study, which comprised 503 boys and 572 girls, aged 8-17 years old. LTPA and SCA were assed by self-reported. Students were assigned as active and non-active in SCA or LTPA according to their self-reported participation. Participants were classified as HBP when they are above of the last quartile for systolic and/or diastolic BP adjusted for gender and age. Do not be engaged in LTPA (OR: 1.47; 95% CI: 1.12; 1.93) and SCA (OR: 1.36; 95% CI: 1.02; 1.81) was significantly associated with the likelihood of being HBP. However, when cardiorespiratory fitness (CRF) (OR: 1.23; 95% CI: 0.91; 1.67) and body mass index (BMI) (OR: 1.31; 95% CI: 0.98; 1.75) were included on SCA model, the association between SCA with HBP did not showed significant results. Children and adolescents who are not engaged in SCA or LTPA are more likely to be classified as having HBP; however, the relationship between SCA and HBP was dependent of CRF and BMI. Further, PA strategies should take these differences on the relationship between HBP, SCA and LTPA. © 2011 Blackwell Publishing Ltd.

Self-consistent clustering analysis: an efficient multiscale scheme for inelastic heterogeneous materials

Energy Technology Data Exchange (ETDEWEB)

Liu, Z.; Bessa, M. A.; Liu, W.K.

2017-10-25

A predictive computational theory is shown for modeling complex, hierarchical materials ranging from metal alloys to polymer nanocomposites. The theory can capture complex mechanisms such as plasticity and failure that span across multiple length scales. This general multiscale material modeling theory relies on sound principles of mathematics and mechanics, and a cutting-edge reduced order modeling method named self-consistent clustering analysis (SCA) [Zeliang Liu, M.A. Bessa, Wing Kam Liu, “Self-consistent clustering analysis: An efficient multi-scale scheme for inelastic heterogeneous materials,” Comput. Methods Appl. Mech. Engrg. 306 (2016) 319–341]. SCA reduces by several orders of magnitude the computational cost of micromechanical and concurrent multiscale simulations, while retaining the microstructure information. This remarkable increase in efficiency is achieved with a data-driven clustering method. Computationally expensive operations are performed in the so-called offline stage, where degrees of freedom (DOFs) are agglomerated into clusters. The interaction tensor of these clusters is computed. In the online or predictive stage, the Lippmann-Schwinger integral equation is solved cluster-wise using a self-consistent scheme to ensure solution accuracy and avoid path dependence. To construct a concurrent multiscale model, this scheme is applied at each material point in a macroscale structure, replacing a conventional constitutive model with the average response computed from the microscale model using just the SCA online stage. A regularized damage theory is incorporated in the microscale that avoids the mesh and RVE size dependence that commonly plagues microscale damage calculations. The SCA method is illustrated with two cases: a carbon fiber reinforced polymer (CFRP) structure with the concurrent multiscale model and an application to fatigue prediction for additively manufactured metals. For the CFRP problem, a speed up estimated to be about

Estimation de la superficie du couvert nival a partir d'une combinaison des donnees de teledetection MODIS et AMSR-E dans un contexte de prevision des crues printanieres au Quebec

Science.gov (United States)

Bergeron, Jean

Snow cover estimation is a principal source of error for spring streamflow simulations in Québec, Canada. Optical and near infrared remote sensing can improve snow cover area (SCA) estimation due to high spatial resolution but is limited by cloud cover and incoming solar radiation. Passive microwave remote sensing is complementary by its near-transparence to cloud cover and independence to incoming solar radiation, but is limited by its coarse spatial resolution. The study aims to create an improved SCA product from blended passive microwave (AMSR-E daily L3 Brightness Temperature) and optical (MODIS Terra and Aqua daily snow cover L3) remote sensing data in order to improve estimation of river streamflow caused by snowmelt with Québec's operational MOHYSE hydrological model through direct-insertion of the blended SCA product in a coupled snowmelt module (SPH-AV). SCA estimated from AMSR-E data is first compared with SCA estimated with MODIS, as well as with in situ snow depth measurements. Results show good agreement (+95%) between AMSR-E-derived and MODIS-derived SCA products in spring but comparisons with Environment Canada ground stations and SCA derived from Advanced Very High Resolution Radiometer (AVHRR) data show lesser agreements (83 % and 74% respectively). Results also show that AMSR-E generally underestimates SCA. Assimilating the blended snow product in SPH-AV coupled with MOHYSE yields significant improvement of simulated streamflow for the aux Écorces et au Saumon rivers overall when compared with simulations with no update during thaw events, These improvements are similar to results driven by biweekly ground data. Assimilation of remotely-sensed passive microwave data was also found to have little positive impact on springflood forecast due to the difficulty in differentiating melting snow from snow-free surfaces. Considering the direct-insertion and Newtonian nudging assimilation methods, the study also shows the latter method to be superior to

A comparative analysis of 9 multi-model averaging approaches in hydrological continuous streamflow simulation

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Arsenault, Richard; Gatien, Philippe; Renaud, Benoit; Brissette, François; Martel, Jean-Luc

2015-10-01

This study aims to test whether a weighted combination of several hydrological models can simulate flows more accurately than the models taken individually. In addition, the project attempts to identify the most efficient model averaging method and the optimal number of models to include in the weighting scheme. In order to address the first objective, streamflow was simulated using four lumped hydrological models (HSAMI, HMETS, MOHYSE and GR4J-6), each of which were calibrated with three different objective functions on 429 watersheds. The resulting 12 hydrographs (4 models × 3 metrics) were weighted and combined with the help of 9 averaging methods which are the simple arithmetic mean (SAM), Akaike information criterion (AICA), Bates-Granger (BGA), Bayes information criterion (BICA), Bayesian model averaging (BMA), Granger-Ramanathan average variant A, B and C (GRA, GRB and GRC) and the average by SCE-UA optimization (SCA). The same weights were then applied to the hydrographs in validation mode, and the Nash-Sutcliffe Efficiency metric was measured between the averaged and observed hydrographs. Statistical analyses were performed to compare the accuracy of weighted methods to that of individual models. A Kruskal-Wallis test and a multi-objective optimization algorithm were then used to identify the most efficient weighted method and the optimal number of models to integrate. Results suggest that the GRA, GRB, GRC and SCA weighted methods perform better than the individual members. Model averaging from these four methods were superior to the best of the individual members in 76% of the cases. Optimal combinations on all watersheds included at least one of each of the four hydrological models. None of the optimal combinations included all members of the ensemble of 12 hydrographs. The Granger-Ramanathan average variant C (GRC) is recommended as the best compromise between accuracy, speed of execution, and simplicity.

Autosomal dominant cerebellar ataxia type I: A review of the phenotypic and genotypic characteristics

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Fujioka Shinsuke

2011-05-01

Full Text Available Abstract Type I autosomal dominant cerebellar ataxia (ADCA is a type of spinocerebellar ataxia (SCA characterized by ataxia with other neurological signs, including oculomotor disturbances, cognitive deficits, pyramidal and extrapyramidal dysfunction, bulbar, spinal and peripheral nervous system involvement. The global prevalence of this disease is not known. The most common type I ADCA is SCA3 followed by SCA2, SCA1, and SCA8, in descending order. Founder effects no doubt contribute to the variable prevalence between populations. Onset is usually in adulthood but cases of presentation in childhood have been reported. Clinical features vary depending on the SCA subtype but by definition include ataxia associated with other neurological manifestations. The clinical spectrum ranges from pure cerebellar signs to constellations including spinal cord and peripheral nerve disease, cognitive impairment, cerebellar or supranuclear ophthalmologic signs, psychiatric problems, and seizures. Cerebellar ataxia can affect virtually any body part causing movement abnormalities. Gait, truncal, and limb ataxia are often the most obvious cerebellar findings though nystagmus, saccadic abnormalities, and dysarthria are usually associated. To date, 21 subtypes have been identified: SCA1-SCA4, SCA8, SCA10, SCA12-SCA14, SCA15/16, SCA17-SCA23, SCA25, SCA27, SCA28 and dentatorubral pallidoluysian atrophy (DRPLA. Type I ADCA can be further divided based on the proposed pathogenetic mechanism into 3 subclasses: subclass 1 includes type I ADCA caused by CAG repeat expansions such as SCA1-SCA3, SCA17, and DRPLA, subclass 2 includes trinucleotide repeat expansions that fall outside of the protein-coding regions of the disease gene including SCA8, SCA10 and SCA12. Subclass 3 contains disorders caused by specific gene deletions, missense mutation, and nonsense mutation and includes SCA13, SCA14, SCA15/16, SCA27 and SCA28. Diagnosis is based on clinical history, physical

Spinocerebellar ataxias in Venezuela: genetic epidemiology and their most likely ethnic descent.

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Paradisi, Irene; Ikonomu, Vassiliki; Arias, Sergio

2016-03-01

Dominantly inherited ataxias (spinocerebellar ataxias, SCAs) are a genetically heterogeneous group of neurologic diseases characterized by progressive cerebellar and spinal tract degeneration with ataxia and other signs, common to all known subtypes. Several types are relatively frequent worldwide, but in several countries, one specific SCA may show a higher prevalence owing to founder phenomena. In Venezuela, genetic epidemiological features of SCAs have been assessed during the last 30 years; mutations in ATXN1 (SCA1), ATXN2 (SCA2), ATXN3 (SCA3), CACNA1A (SCA6), ATXN7 (SCA7), ATXN8 (SCA8), ATXN10 (SCA10), TBP (SCA17) and ATN1 (dentatorubral pallidoluysian atrophy, DRPLA) loci were searched among 115 independent families. SCA7 was the most frequent subtype (26.6%), followed by SCA3 (25.0%), SCA2 (21.9%), SCA1 (17.2%), SCA10 (4.7%) and DRPLA (3.1%); in 43% of the families, the subtype remained unidentified. SCA7 mutations displayed strong geographic aggregation in two independent founder foci, and SCA1 showed a very remote founder effect for a subset of families. SCA10 families were scattered across the country, but all had an identical in-phase haplotype carried also by Mexican, Brazilian and Sioux patients, supporting a very old common Amerindian origin. Prevalence for dominant SCAs in Venezuela was estimated as 1:25 000 nuclear families, provenances of which are either Caucasoid, African or Amerindian.

Improvement of the GRACE star camera data based on the revision of the combination method

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Bandikova, Tamara; Flury, Jakob

2014-11-01

The new release of the sensor and instrument data (Level-1B release 02) of the Gravity Recovery and Climate Experiment (GRACE) had a substantial impact on the improvement of the overall accuracy of the gravity field models. This has implied that improvements on the sensor data level can still significantly contribute to arriving closer to the GRACE baseline accuracy. The recent analysis of the GRACE star camera data (SCA1B RL02) revealed their unexpectedly higher noise. As the star camera (SCA) data are essential for the processing of the K-band ranging data and the accelerometer data, thorough investigation of the data set was needed. We fully reexamined the SCA data processing from Level-1A to Level-1B with focus on the combination method of the data delivered by the two SCA heads. In the first step, we produced and compared our own combined attitude solution by applying two different combination methods on the SCA Level-1A data. The first method introduces the information about the anisotropic accuracy of the star camera measurement in terms of a weighing matrix. This method was applied in the official processing as well. The alternative method merges only the well determined SCA boresight directions. This method was implemented on the GRACE SCA data for the first time. Both methods were expected to provide optimal solution characteristic by the full accuracy about all three axes, which was confirmed. In the second step, we analyzed the differences between the official SCA1B RL02 data generated by the Jet Propulsion Laboratory (JPL) and our solution. SCA1B RL02 contains systematically higher noise of about a factor 3-4. The data analysis revealed that the reason is the incorrect implementation of algorithms in the JPL processing routines. After correct implementation of the combination method, significant improvement within the whole spectrum was achieved. Based on these results, the official reprocessing of the SCA data is suggested, as the SCA attitude data

GEP analysis validates high risk MDS and acute myeloid leukemia post MDS mice models and highlights novel dysregulated pathways.

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Guerenne, Laura; Beurlet, Stéphanie; Said, Mohamed; Gorombei, Petra; Le Pogam, Carole; Guidez, Fabien; de la Grange, Pierre; Omidvar, Nader; Vanneaux, Valérie; Mills, Ken; Mufti, Ghulam J; Sarda-Mantel, Laure; Noguera, Maria Elena; Pla, Marika; Fenaux, Pierre; Padua, Rose Ann; Chomienne, Christine; Krief, Patricia

2016-01-27

In spite of the recent discovery of genetic mutations in most myelodysplasic (MDS) patients, the pathophysiology of these disorders still remains poorly understood, and only few in vivo models are available to help unravel the disease. We performed global specific gene expression profiling and functional pathway analysis in purified Sca1+ cells of two MDS transgenic mouse models that mimic human high-risk MDS (HR-MDS) and acute myeloid leukemia (AML) post MDS, with NRASD12 and BCL2 transgenes under the control of different promoters MRP8NRASD12/tethBCL-2 or MRP8[NRASD12/hBCL-2], respectively. Analysis of dysregulated genes that were unique to the diseased HR-MDS and AML post MDS mice and not their founder mice pointed first to pathways that had previously been reported in MDS patients, including DNA replication/damage/repair, cell cycle, apoptosis, immune responses, and canonical Wnt pathways, further validating these models at the gene expression level. Interestingly, pathways not previously reported in MDS were discovered. These included dysregulated genes of noncanonical Wnt pathways and energy and lipid metabolisms. These dysregulated genes were not only confirmed in a different independent set of BM and spleen Sca1+ cells from the MDS mice but also in MDS CD34+ BM patient samples. These two MDS models may thus provide useful preclinical models to target pathways previously identified in MDS patients and to unravel novel pathways highlighted by this study.

Influência da adulteração de combustíveis no desempenho e na vida útil de motores de combustão interna de ignição por faísca de pequeno porte utilizados em motocicletas.

OpenAIRE

Luiz Vicente Figueira de Mello Filho

2009-01-01

Este trabalho tem como principal objetivo mostrar os efeitos do uso de gasolina adulterada em motores de pequena cilindrada em seus desempenhos e durabilidades. Para tanto, foram realizados ensaios comparativos em três motores carburados de aplicação em motocicletas, de mesmas séries. Estes motores consumiram gasolina C, padrão brasileiro de combustível para motores de ignição por faísca, e misturas desta gasolina com etanol hidratado carburante e solvente C-9. Os resultados mostram alteraçõe...

Peripheral Neuropathy in Spinocerebellar Ataxia Type 1, 2, 3, and 6.

Science.gov (United States)

Linnemann, Christoph; Tezenas du Montcel, Sophie; Rakowicz, Maryla; Schmitz-Hübsch, Tanja; Szymanski, Sandra; Berciano, Jose; van de Warrenburg, Bart P; Pedersen, Karine; Depondt, Chantal; Rola, Rafal; Klockgether, Thomas; García, Antonio; Mutlu, Gurkan; Schöls, Ludger

2016-04-01

Spinocerebellar ataxias (SCAs) are characterized by autosomal dominantly inherited progressive ataxia but are clinically heterogeneous due to variable involvement of non-cerebellar parts of the nervous system. Non-cerebellar symptoms contribute significantly to the burden of SCAs, may guide the clinician to the underlying genetic subtype, and might be useful markers to monitor disease. Peripheral neuropathy is frequently observed in SCA, but subtype-specific features and subclinical manifestations have rarely been evaluated. We performed a multicenter nerve conduction study with 162 patients with genetically confirmed SCA1, SCA2, SCA3, and SCA6. The study proved peripheral nerves to be involved in the neurodegenerative process in 82 % of SCA1, 63 % of SCA2, 55 % of SCA3, and 22 % of SCA6 patients. Most patients of all subtypes revealed affection of both sensory and motor fibers. Neuropathy was most frequently of mixed type with axonal and demyelinating characteristics in all SCA subtypes. However, nerve conduction velocities of SCA1 patients were slower compared to other genotypes. SCA6 patients revealed less axonal damage than patients with other subtypes. No influence of CAG repeat length or biometric determinants on peripheral neuropathy could be identified in SCA1, SCA3, and SCA6. In SCA2, earlier onset and more severe ataxia were associated with peripheral neuropathy. We proved peripheral neuropathy to be a frequent site of the neurodegenerative process in all common SCA subtypes. Since damage to peripheral nerves is readily assessable by electrophysiological means, nerve conduction studies should be performed in a longitudinal approach to assess these parameters as potential progression markers.

Clinical features, neurogenetics and neuropathology of the polyglutamine spinocerebellar ataxias type 1, 2, 3, 6 and 7.

Science.gov (United States)

Rüb, Udo; Schöls, Ludger; Paulson, Henry; Auburger, Georg; Kermer, Pawel; Jen, Joanna C; Seidel, Kay; Korf, Horst-Werner; Deller, Thomas

2013-05-01

The spinocerebellar ataxias type 1 (SCA1), 2 (SCA2), 3 (SCA3), 6 (SCA6) and 7 (SCA7) are genetically defined autosomal dominantly inherited progressive cerebellar ataxias (ADCAs). They belong to the group of CAG-repeat or polyglutamine diseases and share pathologically expanded and meiotically unstable glutamine-encoding CAG-repeats at distinct gene loci encoding elongated polyglutamine stretches in the disease proteins. In recent years, progress has been made in the understanding of the pathogenesis of these currently incurable diseases: Identification of underlying genetic mechanisms made it possible to classify the different ADCAs and to define their clinical and pathological features. Furthermore, advances in molecular biology yielded new insights into the physiological and pathophysiological role of the gene products of SCA1, SCA2, SCA3, SCA6 and SCA7 (i.e. ataxin-1, ataxin-2, ataxin-3, α-1A subunit of the P/Q type voltage-dependent calcium channel, ataxin-7). In the present review we summarize our current knowledge about the polyglutamine ataxias SCA1, SCA2, SCA3, SCA6 and SCA7 and compare their clinical and electrophysiological features, genetic and molecular biological background, as well as their brain pathologies. Furthermore, we provide an overview of the structure, interactions and functions of the different disease proteins. On the basis of these comprehensive data, similarities, differences and possible disease mechanisms are discussed. Copyright © 2013 Elsevier Ltd. All rights reserved.

Intensive precipitation observation greatly improves hydrological modelling of the poorly gauged high mountain Mabengnong catchment in the Tibetan Plateau

Science.gov (United States)

Wang, Li; Zhang, Fan; Zhang, Hongbo; Scott, Christopher A.; Zeng, Chen; Shi, Xiaonan

2018-01-01

Precipitation is one of the most critical inputs for models used to improve understanding of hydrological processes. In high mountain areas, it is challenging to generate a reliable precipitation data set capturing the spatial and temporal heterogeneity due to the harsh climate, extreme terrain and the lack of observations. This study conducts intensive observation of precipitation in the Mabengnong catchment in the southeast of the Tibetan Plateau during July to August 2013. Because precipitation is greatly influenced by altitude, the observed data are used to characterize the precipitation gradient (PG) and hourly distribution (HD), showing that the average PG is 0.10, 0.28 and 0.26 mm/d/100 m and the average duration is around 0.1, 0.8 and 5.2 h for trace, light and moderate rain, respectively. A distributed biosphere hydrological model based on water and energy budgets with improved physical process for snow (WEB-DHM-S) is applied to simulate the hydrological processes with gridded precipitation data derived from a lower altitude meteorological station and the PG and HD characterized for the study area. The observed runoff, MODIS/Terra snow cover area (SCA) data, and MODIS/Terra land surface temperature (LST) data are used for model calibration and validation. Runoff, SCA and LST simulations all show reasonable results. Sensitivity analyses illustrate that runoff is largely underestimated without considering PG, indicating that short-term intensive precipitation observation has the potential to greatly improve hydrological modelling of poorly gauged high mountain catchments.

Dynamic capability in an under-researched cultural environment

Directory of Open Access Journals (Sweden)

Fatemeh Rezaee

2016-02-01

Full Text Available During the past few years, dynamic capability (DC has been considered as an important issue in banking industry. This paper presents a survey on dynamic capability and its role on reaching sustainable competitive advantage (SCA within Mellat bank of Iran (MBI. A valid research instrument is utilized to conduct a survey among 150 managers from MBI. The study utilizes structural equation modelling to examine different hypotheses based on an integrated model of DC and SCA. According to literature studies, expert opinions and exploratory factor analysis, DC is classified into sensing, learning, reconfiguration, and coordination. Furthermore, SCA of the banking industry is classified into three dimensions: market, customer, and financial performance. The results indicate that DC had the greatest effect on the market centered, while it had the least influence on the customer centered.

Efficient and anonymous two-factor user authentication in wireless sensor networks: achieving user anonymity with lightweight sensor computation.

Science.gov (United States)

Nam, Junghyun; Choo, Kim-Kwang Raymond; Han, Sangchul; Kim, Moonseong; Paik, Juryon; Won, Dongho

2015-01-01

A smart-card-based user authentication scheme for wireless sensor networks (hereafter referred to as a SCA-WSN scheme) is designed to ensure that only users who possess both a smart card and the corresponding password are allowed to gain access to sensor data and their transmissions. Despite many research efforts in recent years, it remains a challenging task to design an efficient SCA-WSN scheme that achieves user anonymity. The majority of published SCA-WSN schemes use only lightweight cryptographic techniques (rather than public-key cryptographic techniques) for the sake of efficiency, and have been demonstrated to suffer from the inability to provide user anonymity. Some schemes employ elliptic curve cryptography for better security but require sensors with strict resource constraints to perform computationally expensive scalar-point multiplications; despite the increased computational requirements, these schemes do not provide user anonymity. In this paper, we present a new SCA-WSN scheme that not only achieves user anonymity but also is efficient in terms of the computation loads for sensors. Our scheme employs elliptic curve cryptography but restricts its use only to anonymous user-to-gateway authentication, thereby allowing sensors to perform only lightweight cryptographic operations. Our scheme also enjoys provable security in a formal model extended from the widely accepted Bellare-Pointcheval-Rogaway (2000) model to capture the user anonymity property and various SCA-WSN specific attacks (e.g., stolen smart card attacks, node capture attacks, privileged insider attacks, and stolen verifier attacks).

Efficient and anonymous two-factor user authentication in wireless sensor networks: achieving user anonymity with lightweight sensor computation.

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Junghyun Nam

Full Text Available A smart-card-based user authentication scheme for wireless sensor networks (hereafter referred to as a SCA-WSN scheme is designed to ensure that only users who possess both a smart card and the corresponding password are allowed to gain access to sensor data and their transmissions. Despite many research efforts in recent years, it remains a challenging task to design an efficient SCA-WSN scheme that achieves user anonymity. The majority of published SCA-WSN schemes use only lightweight cryptographic techniques (rather than public-key cryptographic techniques for the sake of efficiency, and have been demonstrated to suffer from the inability to provide user anonymity. Some schemes employ elliptic curve cryptography for better security but require sensors with strict resource constraints to perform computationally expensive scalar-point multiplications; despite the increased computational requirements, these schemes do not provide user anonymity. In this paper, we present a new SCA-WSN scheme that not only achieves user anonymity but also is efficient in terms of the computation loads for sensors. Our scheme employs elliptic curve cryptography but restricts its use only to anonymous user-to-gateway authentication, thereby allowing sensors to perform only lightweight cryptographic operations. Our scheme also enjoys provable security in a formal model extended from the widely accepted Bellare-Pointcheval-Rogaway (2000 model to capture the user anonymity property and various SCA-WSN specific attacks (e.g., stolen smart card attacks, node capture attacks, privileged insider attacks, and stolen verifier attacks.

Lithium carbonate and coenzyme Q10 reduce cell death in a cell model of Machado-Joseph disease

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C.M. Lopes-Ramos

Full Text Available Machado-Joseph disease (MJD or spinocerebellar ataxia type 3 (SCA3 is an autosomal dominant neurodegenerative disorder caused by expansion of the polyglutamine domain of the ataxin-3 (ATX3 protein. MJD/SCA3 is the most frequent autosomal dominant ataxia in many countries. The mechanism underlying MJD/SCA3 is thought to be mainly related to protein misfolding and aggregation leading to neuronal dysfunction followed by cell death. Currently, there are no effective treatments for patients with MJD/SCA3. Here, we report on the potential use of lithium carbonate and coenzyme Q10 to reduce cell death caused by the expanded ATX3 in cell culture. Cell viability and apoptosis were evaluated by MTT assay and by flow cytometry after staining with annexin V-FITC/propidium iodide. Treatment with lithium carbonate and coenzyme Q10 led to a significant increase in viability of cells expressing expanded ATX3 (Q84. In addition, we found that the increase in cell viability resulted from a significant reduction in the proportion of apoptotic cells. Furthermore, there was a significant change in the expanded ATX3 monomer/aggregate ratio after lithium carbonate and coenzyme Q10 treatment, with an increase in the monomer fraction and decrease in aggregates. The safety and tolerance of both drugs are well established; thus, our results indicate that lithium carbonate and coenzyme Q10 are good candidates for further in vivo therapeutic trials.

GEP analysis validates high risk MDS and acute myeloid leukemia post MDS mice models and highlights novel dysregulated pathways

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Laura Guerenne

2016-01-01

Full Text Available Abstract Background In spite of the recent discovery of genetic mutations in most myelodysplasic (MDS patients, the pathophysiology of these disorders still remains poorly understood, and only few in vivo models are available to help unravel the disease. Methods We performed global specific gene expression profiling and functional pathway analysis in purified Sca1+ cells of two MDS transgenic mouse models that mimic human high-risk MDS (HR-MDS and acute myeloid leukemia (AML post MDS, with NRASD12 and BCL2 transgenes under the control of different promoters MRP8NRASD12/tethBCL-2 or MRP8[NRASD12/hBCL-2], respectively. Results Analysis of dysregulated genes that were unique to the diseased HR-MDS and AML post MDS mice and not their founder mice pointed first to pathways that had previously been reported in MDS patients, including DNA replication/damage/repair, cell cycle, apoptosis, immune responses, and canonical Wnt pathways, further validating these models at the gene expression level. Interestingly, pathways not previously reported in MDS were discovered. These included dysregulated genes of noncanonical Wnt pathways and energy and lipid metabolisms. These dysregulated genes were not only confirmed in a different independent set of BM and spleen Sca1+ cells from the MDS mice but also in MDS CD34+ BM patient samples. Conclusions These two MDS models may thus provide useful preclinical models to target pathways previously identified in MDS patients and to unravel novel pathways highlighted by this study.

Stoichiometric Correlation Analysis: Principles of Metabolic Functionality from Metabolomics Data

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Kevin Schwahn

2017-12-01

Full Text Available Recent advances in metabolomics technologies have resulted in high-quality (time-resolved metabolic profiles with an increasing coverage of metabolic pathways. These data profiles represent read-outs from often non-linear dynamics of metabolic networks. Yet, metabolic profiles have largely been explored with regression-based approaches that only capture linear relationships, rendering it difficult to determine the extent to which the data reflect the underlying reaction rates and their couplings. Here we propose an approach termed Stoichiometric Correlation Analysis (SCA based on correlation between positive linear combinations of log-transformed metabolic profiles. The log-transformation is due to the evidence that metabolic networks can be modeled by mass action law and kinetics derived from it. Unlike the existing approaches which establish a relation between pairs of metabolites, SCA facilitates the discovery of higher-order dependence between more than two metabolites. By using a paradigmatic model of the tricarboxylic acid cycle we show that the higher-order dependence reflects the coupling of concentration of reactant complexes, capturing the subtle difference between the employed enzyme kinetics. Using time-resolved metabolic profiles from Arabidopsis thaliana and Escherichia coli, we show that SCA can be used to quantify the difference in coupling of reactant complexes, and hence, reaction rates, underlying the stringent response in these model organisms. By using SCA with data from natural variation of wild and domesticated wheat and tomato accession, we demonstrate that the domestication is accompanied by loss of such couplings, in these species. Therefore, application of SCA to metabolomics data from natural variation in wild and domesticated populations provides a mechanistic way to understanding domestication and its relation to metabolic networks.

Constitutive transgene expression of Stem Cell Antigen-1 in the hair follicle alters the sensitivity to tumor formation and progression

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Rikke Christensen

2017-08-01

Full Text Available The cell surface protein Stem Cell Antigen-1 (Sca-1 marks stem or progenitor cells in several murine tissues and is normally upregulated during cancer development. Although the specific function of Sca-1 remains unknown, Sca-1 seems to play a role in proliferation, differentiation and cell migration in a number of tissues. In the skin epithelium, Sca-1 is highly expressed in the interfollicular epidermis but is absent in most compartments of the hair follicle; however, the function of Sca-1 in the skin has not been investigated. To explore the role of Sca-1 in normal and malignant skin development we generated transgenic mice that express Sca-1 in the hair follicle stem cells that are normally Sca-1 negative. Development of hair follicles and interfollicular epidermis appeared normal in Sca-1 mutant mice; however, follicular induction of Sca-1 expression in bulge region and isthmus stem cells reduced the overall yield of papillomas in a chemical carcinogenesis protocol. Despite that fewer papillomas developed in transgenic mice a higher proportion of the papillomas underwent malignant conversion. These findings suggest that overexpression of Sca-1 in the hair follicle stem cells contributes at different stages of tumour development. In early stages, overexpression of Sca-1 decreases tumour formation while at later stages overexpression of Sca-1 seems to drive tumours towards malignant progression.

Understanding the Pathophysiology of Spinocerebellar Ataxias through genetics, neurophysiology, structural and functional neuroimaging

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Pramod Kumar Pal

2015-12-01

Full Text Available Over the past 10 years a large cohort of 656 index patients with clinically suspected degenerative ataxias were clinically evaluated under various research projects. Of these, 625 index patients underwent genetic tests for the clinically suspected most probable diagnosis. A diagnosis could be achieved in 218 patients (34.9%. Among these 218 index patients, 82 each were SCA1 and SCA2, 32 were SCA3, 4 were SCA12, and 18 were Friedreich's Ataxia. Thus among the Autosomal Dominant Ataxias (SCAs there was equal prevalence of SCA1 and SCA2 (41% each followed by SCA3 (16% and SCA12 (2%. This high prevalence of SCA1 is in contrast to the available National and International literature. The rate of clinical disease progression, especially in SCA2, was dependent on the CAG repeat size, and may commence linearly from birth.Apart from cerebellar involvement, a comprehensive evaluation of the neuroaxis in various subsets of this genetically proved cohort showedsubclinicalinvolvement of the cerebral cortex, central motor and sensory pathways, peripheral nervous system and autonomic nervous system. Important findings include: (aAmixedsensorimotor and pure sensory neuropathy was seen in all the three subtypes of SCAs, while pure motor neuropathy was uncommon; (b There was reduced cortical excitability and prolonged central motor conduction time, most evident in SCA1 and least in SCA2; (c Cardiac autonomic dysfunction, predominantly parasympathetic, was seen in SCA, and the severity correlated with the duration of illness in SCA1; (d In SCA1 there was a global impairment of balance, with greater instability in anterior–posterior than medio–lateral directions; (e In all the three SCAs there was a significant loss of gray matter in both cerebellar hemispheres and vermis. Vermian atrophy was more pronounced in SCA3, while SCA1 and SCA2 had significant white matter atrophy. Pontine white matter atrophy was more pronounced in SCA2; (f Cerebellar activity was

Non Communicable Diseases | Adeniyi | African Health Sciences

African Journals Online (AJOL)

Methods: Forty-nine SCA teenagers were randomized into either the SCA spirometry or the SCA control groups, which had 24 and 25 patients respectively. They were initially compared with 25 matched non-SCA teenagers. The SCA spirometry group went through a six-week, thrice-daily local incentive spirometry while the ...

Multigroup Moderation Test in Generalized Structured Component Analysis

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Angga Dwi Mulyanto

2016-05-01

Full Text Available Generalized Structured Component Analysis (GSCA is an alternative method in structural modeling using alternating least squares. GSCA can be used for the complex analysis including multigroup. GSCA can be run with a free software called GeSCA, but in GeSCA there is no multigroup moderation test to compare the effect between groups. In this research we propose to use the T test in PLS for testing moderation Multigroup on GSCA. T test only requires sample size, estimate path coefficient, and standard error of each group that are already available on the output of GeSCA and the formula is simple so the user does not need a long time for analysis.

Ensemble-based assimilation of fractional snow-covered area satellite retrievals to estimate the snow distribution at Arctic sites

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K. Aalstad

2018-01-01

Full Text Available With its high albedo, low thermal conductivity and large water storing capacity, snow strongly modulates the surface energy and water balance, which makes it a critical factor in mid- to high-latitude and mountain environments. However, estimating the snow water equivalent (SWE is challenging in remote-sensing applications already at medium spatial resolutions of 1 km. We present an ensemble-based data assimilation framework that estimates the peak subgrid SWE distribution (SSD at the 1 km scale by assimilating fractional snow-covered area (fSCA satellite retrievals in a simple snow model forced by downscaled reanalysis data. The basic idea is to relate the timing of the snow cover depletion (accessible from satellite products to the peak SSD. Peak subgrid SWE is assumed to be lognormally distributed, which can be translated to a modeled time series of fSCA through the snow model. Assimilation of satellite-derived fSCA facilitates the estimation of the peak SSD, while taking into account uncertainties in both the model and the assimilated data sets. As an extension to previous studies, our method makes use of the novel (to snow data assimilation ensemble smoother with multiple data assimilation (ES-MDA scheme combined with analytical Gaussian anamorphosis to assimilate time series of Moderate Resolution Imaging Spectroradiometer (MODIS and Sentinel-2 fSCA retrievals. The scheme is applied to Arctic sites near Ny-Ålesund (79° N, Svalbard, Norway where field measurements of fSCA and SWE distributions are available. The method is able to successfully recover accurate estimates of peak SSD on most of the occasions considered. Through the ES-MDA assimilation, the root-mean-square error (RMSE for the fSCA, peak mean SWE and peak subgrid coefficient of variation is improved by around 75, 60 and 20 %, respectively, when compared to the prior, yielding RMSEs of 0.01, 0.09 m water equivalent (w.e. and 0.13, respectively. The ES-MDA either

Ensemble-based assimilation of fractional snow-covered area satellite retrievals to estimate the snow distribution at Arctic sites

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Aalstad, Kristoffer; Westermann, Sebastian; Vikhamar Schuler, Thomas; Boike, Julia; Bertino, Laurent

2018-01-01

With its high albedo, low thermal conductivity and large water storing capacity, snow strongly modulates the surface energy and water balance, which makes it a critical factor in mid- to high-latitude and mountain environments. However, estimating the snow water equivalent (SWE) is challenging in remote-sensing applications already at medium spatial resolutions of 1 km. We present an ensemble-based data assimilation framework that estimates the peak subgrid SWE distribution (SSD) at the 1 km scale by assimilating fractional snow-covered area (fSCA) satellite retrievals in a simple snow model forced by downscaled reanalysis data. The basic idea is to relate the timing of the snow cover depletion (accessible from satellite products) to the peak SSD. Peak subgrid SWE is assumed to be lognormally distributed, which can be translated to a modeled time series of fSCA through the snow model. Assimilation of satellite-derived fSCA facilitates the estimation of the peak SSD, while taking into account uncertainties in both the model and the assimilated data sets. As an extension to previous studies, our method makes use of the novel (to snow data assimilation) ensemble smoother with multiple data assimilation (ES-MDA) scheme combined with analytical Gaussian anamorphosis to assimilate time series of Moderate Resolution Imaging Spectroradiometer (MODIS) and Sentinel-2 fSCA retrievals. The scheme is applied to Arctic sites near Ny-Ålesund (79° N, Svalbard, Norway) where field measurements of fSCA and SWE distributions are available. The method is able to successfully recover accurate estimates of peak SSD on most of the occasions considered. Through the ES-MDA assimilation, the root-mean-square error (RMSE) for the fSCA, peak mean SWE and peak subgrid coefficient of variation is improved by around 75, 60 and 20 %, respectively, when compared to the prior, yielding RMSEs of 0.01, 0.09 m water equivalent (w.e.) and 0.13, respectively. The ES-MDA either outperforms or at least

Hydrological modelling of the Mabengnong catchment in the southeast Tibet with support of short term intensive precipitation observation

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Wang, L.; Zhang, F.; Zhang, H.; Scott, C. A.; Zeng, C.; SHI, X.

2017-12-01

Precipitation is one of the crucial inputs for models used to better understand hydrological processes. In high mountain areas, it is a difficult task to obtain a reliable precipitation data set describing the spatial and temporal characteristic due to the limited meteorological observations and high variability of precipitation. This study carries out intensive observation of precipitation in a high mountain catchment in the southeast of the Tibet during July to August 2013. According to the rain gauges set up at different altitudes, it is found that precipitation is greatly influenced by altitude. The observed precipitation is used to depict the precipitation gradient (PG) and hourly distribution (HD), showing that the average duration is around 0.1, 0.8 and 6.0 hours and the average PG is 0.10, 0.28 and 0.26 mm/d/100m for trace, light and moderate rain, respectively. Based on the gridded precipitation derived from the PG and HD and the nearby Linzhi meteorological station at lower altitude, a distributed biosphere hydrological model based on water and energy budgets (WEB-DHM) is applied to simulate the hydrological processes. Beside the observed runoff, MODIS/Terra snow cover area (SCA) data, and MODIS/Terra land surface temperature (LST) data are also used for model calibration and validation. The resulting runoff, SCA and LST simulations are all reasonable. Sensitivity analyses indicate that runoff is greatly underestimated without considering PG, illustrating that short-term intensive precipitation observation contributes to improving hydrological modelling of poorly gauged high mountain catchments.

ASME XI stroke time testing of solenoid valves at Connecticut Yankee Station

Energy Technology Data Exchange (ETDEWEB)

Martin, C.W.

1996-12-01

Connecticut Yankee Atomic Power Company has developed the capability of measuring the stroke times of AC and DC solenoid valves. This allows the station to measure the stroke time of any solenoid valve in the plant, even those valves which do not have valve stem position indicators. Connecticut Yankee has adapted the ITI MOVATS Checkmate 3 system, using a signal input from a Bruel and Kjaer (B&K) Model 4382 acoustic accelerometer and the Schaumberg Campbell Associates (SCA) Model SCA-1148 dual sensor, which is a combined accelerometer and gaussmeter.

ASME XI stroke time testing of solenoid valves at Connecticut Yankee Station

International Nuclear Information System (INIS)

Martin, C.W.

1996-01-01

Connecticut Yankee Atomic Power Company has developed the capability of measuring the stroke times of AC and DC solenoid valves. This allows the station to measure the stroke time of any solenoid valve in the plant, even those valves which do not have valve stem position indicators. Connecticut Yankee has adapted the ITI MOVATS Checkmate 3 system, using a signal input from a Bruel and Kjaer (B ampersand K) Model 4382 acoustic accelerometer and the Schaumberg Campbell Associates (SCA) Model SCA-1148 dual sensor, which is a combined accelerometer and gaussmeter

Phenotype variability and early onset ataxia symptoms in spinocerebellar ataxia type 7: comparison and correlation with other spinocerebellar ataxias

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Marcus Vinicius Cristino de Albuquerque

2015-01-01

Full Text Available The spinocerebellar ataxias (SCA are a group of neurodegenerative disorders characterized by heterogeneous clinical presentation. Spinocerebellar ataxia type 7 (SCA7 is caused by an abnormal CAG repeat expansion and includes cerebellar signs associated with visual loss and ophthalmoplegia. Marked anticipation and dynamic mutation is observed in SCA7. Moreover, phenotype variability and very early onset of symptoms may occur. In this article, a large series of Brazilian patients with different SCA subtypes was evaluated, and we compared the age of onset of SCA7 with other SCA. From the 26 patients with SCA7, 4 manifested their symptoms before 10-year-old. Also, occasionally the parents may have the onset of symptoms after their children. In conclusion, our study highlights the genetic anticipation phenomenon that occurs in SCA7 families. Patients with very early onset ataxia in the context of a remarkable family history, must be considered and tested for SCA7.

Dantrolene is neuroprotective in Huntington's disease transgenic mouse model

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Chen Xi

2011-11-01

Full Text Available Abstract Background Huntington's disease (HD is a progressive neurodegenerative disorder caused by a polyglutamine expansion in the Huntingtin protein which results in the selective degeneration of striatal medium spiny neurons (MSNs. Our group has previously demonstrated that calcium (Ca2+ signaling is abnormal in MSNs from the yeast artificial chromosome transgenic mouse model of HD (YAC128. Moreover, we demonstrated that deranged intracellular Ca2+ signaling sensitizes YAC128 MSNs to glutamate-induced excitotoxicity when compared to wild type (WT MSNs. In previous studies we also observed abnormal neuronal Ca2+ signaling in neurons from spinocerebellar ataxia 2 (SCA2 and spinocerebellar ataxia 3 (SCA3 mouse models and demonstrated that treatment with dantrolene, a ryanodine receptor antagonist and clinically relevant Ca2+ signaling stabilizer, was neuroprotective in experiments with these mouse models. The aim of the current study was to evaluate potential beneficial effects of dantrolene in experiments with YAC128 HD mouse model. Results The application of caffeine and glutamate resulted in increased Ca2+ release from intracellular stores in YAC128 MSN cultures when compared to WT MSN cultures. Pre-treatment with dantrolene protected YAC128 MSNs from glutamate excitotoxicty, with an effective concentration of 100 nM and above. Feeding dantrolene (5 mg/kg twice a week to YAC128 mice between 2 months and 11.5 months of age resulted in significantly improved performance in the beam-walking and gait-walking assays. Neuropathological analysis revealed that long-term dantrolene feeding to YAC128 mice significantly reduced the loss of NeuN-positive striatal neurons and reduced formation of Httexp nuclear aggregates. Conclusions Our results support the hypothesis that deranged Ca2+ signaling plays an important role in HD pathology. Our data also implicate the RyanRs as a potential therapeutic target for the treatment of HD and demonstrate that Ryan

Dantrolene is neuroprotective in Huntington's disease transgenic mouse model.

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Chen, Xi; Wu, Jun; Lvovskaya, Svetlana; Herndon, Emily; Supnet, Charlene; Bezprozvanny, Ilya

2011-11-25

Huntington's disease (HD) is a progressive neurodegenerative disorder caused by a polyglutamine expansion in the Huntingtin protein which results in the selective degeneration of striatal medium spiny neurons (MSNs). Our group has previously demonstrated that calcium (Ca2+) signaling is abnormal in MSNs from the yeast artificial chromosome transgenic mouse model of HD (YAC128). Moreover, we demonstrated that deranged intracellular Ca2+ signaling sensitizes YAC128 MSNs to glutamate-induced excitotoxicity when compared to wild type (WT) MSNs. In previous studies we also observed abnormal neuronal Ca2+ signaling in neurons from spinocerebellar ataxia 2 (SCA2) and spinocerebellar ataxia 3 (SCA3) mouse models and demonstrated that treatment with dantrolene, a ryanodine receptor antagonist and clinically relevant Ca2+ signaling stabilizer, was neuroprotective in experiments with these mouse models. The aim of the current study was to evaluate potential beneficial effects of dantrolene in experiments with YAC128 HD mouse model. The application of caffeine and glutamate resulted in increased Ca2+ release from intracellular stores in YAC128 MSN cultures when compared to WT MSN cultures. Pre-treatment with dantrolene protected YAC128 MSNs from glutamate excitotoxicty, with an effective concentration of 100 nM and above. Feeding dantrolene (5 mg/kg) twice a week to YAC128 mice between 2 months and 11.5 months of age resulted in significantly improved performance in the beam-walking and gait-walking assays. Neuropathological analysis revealed that long-term dantrolene feeding to YAC128 mice significantly reduced the loss of NeuN-positive striatal neurons and reduced formation of Httexp nuclear aggregates. Our results support the hypothesis that deranged Ca2+ signaling plays an important role in HD pathology. Our data also implicate the RyanRs as a potential therapeutic target for the treatment of HD and demonstrate that RyanR inhibitors and Ca2+ signaling stabilizers such as

Detection of Impaired Cerebral Autoregulation Using Selected Correlation Analysis: A Validation Study.

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Proescholdt, Martin A; Faltermeier, Rupert; Bele, Sylvia; Brawanski, Alexander

2017-01-01

Multimodal brain monitoring has been utilized to optimize treatment of patients with critical neurological diseases. However, the amount of data requires an integrative tool set to unmask pathological events in a timely fashion. Recently we have introduced a mathematical model allowing the simulation of pathophysiological conditions such as reduced intracranial compliance and impaired autoregulation. Utilizing a mathematical tool set called selected correlation analysis (sca), correlation patterns, which indicate impaired autoregulation, can be detected in patient data sets (scp). In this study we compared the results of the sca with the pressure reactivity index (PRx), an established marker for impaired autoregulation. Mean PRx values were significantly higher in time segments identified as scp compared to segments showing no selected correlations (nsc). The sca based approach predicted cerebral autoregulation failure with a sensitivity of 78.8% and a specificity of 62.6%. Autoregulation failure, as detected by the results of both analysis methods, was significantly correlated with poor outcome. Sca of brain monitoring data detects impaired autoregulation with high sensitivity and sufficient specificity. Since the sca approach allows the simultaneous detection of both major pathological conditions, disturbed autoregulation and reduced compliance, it may become a useful analysis tool for brain multimodal monitoring data.

Detection of Impaired Cerebral Autoregulation Using Selected Correlation Analysis: A Validation Study

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Martin A. Proescholdt

2017-01-01

Full Text Available Multimodal brain monitoring has been utilized to optimize treatment of patients with critical neurological diseases. However, the amount of data requires an integrative tool set to unmask pathological events in a timely fashion. Recently we have introduced a mathematical model allowing the simulation of pathophysiological conditions such as reduced intracranial compliance and impaired autoregulation. Utilizing a mathematical tool set called selected correlation analysis (sca, correlation patterns, which indicate impaired autoregulation, can be detected in patient data sets (scp. In this study we compared the results of the sca with the pressure reactivity index (PRx, an established marker for impaired autoregulation. Mean PRx values were significantly higher in time segments identified as scp compared to segments showing no selected correlations (nsc. The sca based approach predicted cerebral autoregulation failure with a sensitivity of 78.8% and a specificity of 62.6%. Autoregulation failure, as detected by the results of both analysis methods, was significantly correlated with poor outcome. Sca of brain monitoring data detects impaired autoregulation with high sensitivity and sufficient specificity. Since the sca approach allows the simultaneous detection of both major pathological conditions, disturbed autoregulation and reduced compliance, it may become a useful analysis tool for brain multimodal monitoring data.

Application of snowmelt runoff model (SRM in mountainous watersheds: A review

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Shalamu Abudu

2012-06-01

Full Text Available The snowmelt runoff model (SRM has been widely used in simulation and forecast of streamflow in snow-dominated mountainous basins around the world. This paper presents an overall review of worldwide applications of SRM in mountainous watersheds, particularly in data-sparse watersheds of northwestern China. Issues related to proper selection of input climate variables and parameters, and determination of the snow cover area (SCA using remote sensing data in snowmelt runoff modeling are discussed through extensive review of literature. Preliminary applications of SRM in northwestern China have shown that the model accuracies are relatively acceptable although most of the watersheds lack measured hydro-meteorological data. Future research could explore the feasibility of modeling snowmelt runoff in data-sparse mountainous watersheds in northwestern China by utilizing snow and glacier cover remote sensing data, geographic information system (GIS tools, field measurements, and innovative ways of model parameterization.

Clinical and molecular characteristics of a Brazilian family with spinocerebellar ataxia type 1 Características clínicas e moleculares de uma família Brasileira com ataxia espinocerebelar tipo 1

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Iscia Lopes-Cendes

1996-09-01

Full Text Available The spinocerebellar ataxias (SCAs are a clinically and genetically heterogeneous group of late onset neurodegenerative disorders. To date, seven different genes causing autosomal dominant SCA have been mapped: SCA1, SCA2, Machado-Joseph disease (MJD/SCA3, SCA4, SCA5, SCA7 and dentatorubropallidoluysian atrophy (DRPLA. Expansions of an unstable trinucleotide CAG repeat cause three of these disorders: SCA1, MJD/SCA3 and DRPLA. We studied one Brazilian family segregating an autosomal dominant type of SCA. A total of ten individuals were examined and tested for the presence of the SCA1, MJD and DRPLA mutations. Three individuals, one male and two females, were considered affected based on neurological examination; ages at onset were: 32, 36 and 41 years. The first complaint in all three patients was gait ataxia which progressed slowly over the years. Six individuals showed one allele containing an expanded CAG repeat in the SCA1 gene. The mean size of the expanded allele was 48.2 CAG units. Instability of the expanded CAG tract was seen in the two transmissions that were observed in this family. In both occasions there was a contraction of the CAG tract. Our study demonstrates that SCA1 occurs in the Brazilian population. In addition, our results stress the importance of molecular studies in the confirmation of diagnosis and for pre-symptomatic testing in SCAs.As ataxias espinocerebelares (AECs fazem parte de um grupo de doenças neurodegenerativas que apresentam grande heterogeneidade clínica e genética. Existem até o momento sete genes mapeados responsáveis pelas AECs de transmissão autossômica dominante: SCA1, SCA2, doença de Machado-Joseph (DA/7 ou SCA3, SCA4, SCA5, SCA7 e atrofia dentatorubropalidoluisiana (ADRPL. Uma expansão de um trínucletídeo CAG foi identificada como a mutação responsável na SCA], DMJ e ADRPL. Estudamos uma família brasileira com uma forma autossômica dominante de AEC. Dez indivíduos foram examinados e

THE DOCTRINE OF QUASI-MUTUAL ASSENT - HAS IT BECOME THE GENERAL RULE FOR THE FORMATION OF CONTRACTS? THE CASE OF PILLAY v SHAIK 2009 4 SA 74 (SCA

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Puseletso Thejane (Rankoane

2012-12-01

Full Text Available The doctrine of quasi-mutual assent is undoubtedly part of our South African law and has been affirmed and applied in a number of leading decisions. The purpose of this note is to offer a critical analysis of the application of the doctrine in the case of Pillay v Shaik 2009 4 SA 74 (SCA. It is argued that the primary basis of contractual liability in South Africa has always been and still remains consensus ad idem as determined in terms of the rules relating to offer and acceptance It is also argued that the doctrine is not an answer to failure by the parties to comply with self-imposed formalities and/or the prescribed manner of acceptance of an offer for the valid formation of contracts. Based on the aim of the incorporation of the doctrine in our law, coupled with its application in previous court decisions, it is concluded that its application in the case of Pillay v Shaik was wrong and sets a bad precedent.

Seroprevalence of parvovirus B19 antibodies and evidence of viremia among Nigerian patients with sickle cell anemia

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Iwalokun, Bamidele Abiodun; Iwalokun, Senapon Olusola; Hodonu, Semande Olufunmilayo

2013-01-01

Clinical, biochemical and molecular evidence for the sickle cell anemia (SCA) crisis in Nigerian patients arising from parvovirus b19 infection remains inadequate. This study determined the prevalence and correlates of anti-parvovirus b19 antibodies in a population of SCA patients and non-SCA healthy controls in Lagos, Nigeria. In this prospective cross-sectional study, we enrolled 73 confirmed SCA patients from 5 district hospitals in Lagos and 81 sex and age-matched non-SCA healthy controls. Serum sample from each study participant was screened for anti-parvovirus b19 by ELISA and PCR techniques. Standard biomedical assays were also done. Anti-parvovirus b19 IgM and IgG antibodies were detected in 22 (14.3%) and 97 (62.9%) of the 154 sera screened, 13 (17.8%) and 45 (61.6%) in SCA patients; 9 (11.1%) and 52 (64.2%) in non-SCA controls. The overall seronegativity rate was 19.5%. Parvovirus B19 DNA was found in 2 (11.1%) of the 18 IgM seropositive SCA serum samples screened. On the whole, parvovirus b19 infection was more commonly asymptomatic in non-SCA controls but caused significant elevation in liver enzymes in infected SCA patients (P parvovirus b19 infection increased 65 times during unsteady state among the SCA patients. Although no deaths of infected patients were recorded during the study, age below 12 years, hospitalization and overcrowded environment were risk factors for infection. We conclude that parvovirus b19 is common in SCA patients, incurring greater susceptibility to infections. PMID:23885266

Seroprevalence of parvovirus B19 antibodies and evidence of viremia among Nigerian patients with sickle cell anemia.

Science.gov (United States)

Iwalokun, Bamidele Abiodun; Iwalokun, Senapon Olusola; Hodonu, Semande Olufunmilayo

2013-07-01

Clinical, biochemical and molecular evidence for the sickle cell anemia (SCA) crisis in Nigerian patients arising from parvovirus b19 infection remains inadequate. This study determined the prevalence and correlates of anti-parvovirus b19 antibodies in a population of SCA patients and non-SCA healthy controls in Lagos, Nigeria. In this prospective cross-sectional study, we enrolled 73 confirmed SCA patients from 5 district hospitals in Lagos and 81 sex and age-matched non-SCA healthy controls. Serum sample from each study participant was screened for anti-parvovirus b19 by ELISA and PCR techniques. Standard biomedical assays were also done. Anti-parvovirus b19 IgM and IgG antibodies were detected in 22 (14.3%) and 97 (62.9%) of the 154 sera screened, 13 (17.8%) and 45 (61.6%) in SCA patients; 9 (11.1%) and 52 (64.2%) in non-SCA controls. The overall seronegativity rate was 19.5%. Parvovirus B19 DNA was found in 2 (11.1%) of the 18 IgM seropositive SCA serum samples screened. On the whole, parvovirus b19 infection was more commonly asymptomatic in non-SCA controls but caused significant elevation in liver enzymes in infected SCA patients (P parvovirus b19 infection increased 65 times during unsteady state among the SCA patients. Although no deaths of infected patients were recorded during the study, age below 12 years, hospitalization and overcrowded environment were risk factors for infection. We conclude that parvovirus b19 is common in SCA patients, incurring greater susceptibility to infections.

Abnormal autonomic cardiac response to transient hypoxia in sickle cell anemia

International Nuclear Information System (INIS)

Sangkatumvong, S; Khoo, M C K; Coates, T D

2008-01-01

The objective of this study was to non-invasively assess cardiac autonomic control in subjects with sickle cell anemia (SCA) by tracking the changes in heart rate variability (HRV) that occur following brief exposure to a hypoxic stimulus. Five African–American SCA patients and seven healthy control subjects were recruited to participate in this study. Each subject was exposed to a controlled hypoxic stimulus consisting of five breaths of nitrogen. Time-varying spectral analysis of HRV was applied to estimate the cardiac autonomic response to the transient episode of hypoxia. The confounding effects of changes in respiration on the HRV spectral indices were reduced by using a computational model. A significant decrease in the parameters related to parasympathetic control was detected in the post-hypoxic responses of the SCA subjects relative to normal controls. The spectral index related to sympathetic activity, on the other hand, showed a tendency to increase the following hypoxic stimulation, but the change was not significant. This study suggests that there is some degree of cardiovascular autonomic dysfunction in SCA that is revealed by the response to transient hypoxia

Mitral valve prolapse and sudden cardiac arrest in the community.

Science.gov (United States)

Narayanan, Kumar; Uy-Evanado, Audrey; Teodorescu, Carmen; Reinier, Kyndaron; Nichols, Gregory A; Gunson, Karen; Jui, Jonathan; Chugh, Sumeet S

2016-02-01

Mitral valve prolapse (MVP) is relatively common in the general population with recently reported prevalence of 1% and familial clustering (Framingham Heart Study). However, its association with ventricular arrhythmias and sudden cardiac arrest (SCA) remains controversial. The purpose of this study was to characterize the frequency and clinical profile of patients with MVP who suffer SCA in the community. Patients with SCA cases were prospectively identified in the population-based Oregon Sudden Unexpected Death Study (population ~1 million). The presence of MVP was identified from echocardiograms recorded prior but unrelated to the SCA event. The detailed clinical profile of patients with SCA and MVP was compared with that of SCA patients without MVP to identify potential differences. A total of 729 SCA patients were evaluated over a 12-year period (mean age 69.5 ± 14.8 years; 64.6% men). MVP was observed in 17 (2.3%) prior to the SCA event (95% confidence interval 1.2%-3.4%). Mitral regurgitation was present in 14 SCA patients with MVP (82.3%) and was moderate or severe in 10 (58.8%). Compared with SCA patients without MVP, SCA patients with MVP were younger (mean age 60.9 ± 16.4 years vs 69.7 ± 14.7 years; P = .02), with fewer risk factors (diabetes 5.9% vs 46.4%; P = .001; hypertension 41.2% vs 78.9%; P = .001) or known coronary disease (29.4% vs 65.6%; P MVP was observed in a small proportion (2.3%) of SCA patients in the general population, suggesting a low risk overall. Since SCA patients with MVP were characterized by younger age and relatively low cardiovascular comorbidity, a focus on imaging for valve structure/insufficiency as well as genetics could aid future risk stratification approaches. Copyright © 2016 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.

The natural history of spinocerebellar ataxia type 1, 2, 3, and 6: a 2-year follow-up study

NARCIS (Netherlands)

Jacobi, H.; Bauer, P.; Giunti, P.; Labrum, R.; Sweeney, M.G.; Charles, P.; Durr, A.; Marelli, C.; Globas, C.; Linnemann, C.; Schols, L.; Rakowicz, M.; Rola, R.; Zdzienicka, E.; Schmitz-Hubsch, T.; Fancellu, R.; Mariotti, C.; Tomasello, C.; Baliko, L.; Melegh, B.; Filla, A.; Rinaldi, C.; Warrenburg, B.P.C. van de; Verstappen, C.C.P.; Szymanski, S.; Berciano, J.; Infante, J.; Timmann, D.; Boesch, S.; Hering, S.; Depondt, C.; Pandolfo, M.; Kang, J.S.; Ratzka, S.; Schulz, J.; Tezenas du Montcel, S.; Klockgether, T.

2011-01-01

OBJECTIVE: To obtain quantitative data on the progression of the most common spinocerebellar ataxias (SCAs) and identify factors that influence their progression, we initiated the EUROSCA natural history study, a multicentric longitudinal cohort study of 526 patients with SCA1, SCA2, SCA3, or SCA6.

Leakage from medically-certified to self-certified workplace absence among norwegian employees

OpenAIRE

Karlsen, Geir Runar; Ytterstad, Elinor

2017-01-01

Source at https://dx.doi.org/10.1080/20021518.2017.1411119 There are two different kinds of sickness absence in Norway: self-certified absence (SCA), and medically-certified absence (MCA). In this study of 6437 Norwegian employees, we applied logistic regression models for a dichotomous SCA variable on initially 38 independent vari- ables including age and gender. Our findings showed that employees reporting long-term health issues but no record of MCA in the past 12 months (...

Lentiviral gene transfer regenerates hematopoietic stem cells in a mouse model for Mpl-deficient aplastic anemia.

Science.gov (United States)

Heckl, Dirk; Wicke, Daniel C; Brugman, Martijn H; Meyer, Johann; Schambach, Axel; Büsche, Guntram; Ballmaier, Matthias; Baum, Christopher; Modlich, Ute

2011-04-07

Thpo/Mpl signaling plays an important role in the maintenance of hematopoietic stem cells (HSCs) in addition to its role in megakaryopoiesis. Patients with inactivating mutations in Mpl develop thrombocytopenia and aplastic anemia because of progressive loss of HSCs. Yet, it is unknown whether this loss of HSCs is an irreversible process. In this study, we used the Mpl knockout (Mpl(-/-)) mouse model and expressed Mpl from newly developed lentiviral vectors specifically in the physiologic Mpl target populations, namely, HSCs and megakaryocytes. After validating lineage-specific expression in vivo using lentiviral eGFP reporter vectors, we performed bone marrow transplantation of transduced Mpl(-/-) bone marrow cells into Mpl(-/-) mice. We show that restoration of Mpl expression from transcriptionally targeted vectors prevents lethal adverse reactions of ectopic Mpl expression, replenishes the HSC pool, restores stem cell properties, and corrects platelet production. In some mice, megakaryocyte counts were atypically high, accompanied by bone neo-formation and marrow fibrosis. Gene-corrected Mpl(-/-) cells had increased long-term repopulating potential, with a marked increase in lineage(-)Sca1(+)cKit(+) cells and early progenitor populations in reconstituted mice. Transcriptome analysis of lineage(-)Sca1(+)cKit(+) cells in Mpl-corrected mice showed functional adjustment of genes involved in HSC self-renewal.

Inner shell ionization by incident nuclei

International Nuclear Information System (INIS)

Hansteen, J.M.

1974-10-01

The atomic Coulomb excitation process induced by impinging heavy charged particles such as protons, deuterons, α-particles and complex heavy ions is reviewed. Recent experimental and theoretical efforts have led toimproved understanding of the atomic Coulomb excitation as well as to discovery of new types of ionization mechanisms. The following models are mentioned: the Plane Wave Born Approximation (PWBA); theeeeeeeeeeeee modified PWBA model; the Binary Encounter Approximation (BEA); the Semi-Classical Approximation (SCA); the Perturbed-Stationary-State model (PSS). The structure of the SCA model is more thoroughly treated. Experimental results on single Coulomb ionizations of the K-, L-, and M-shells, and of the connected sub-shells by protons are compared with predictions. Most calculations are based on straight line projectile paths and non-relativistic hydrogen-like target electron wave functions. The BEA model and the SCA model seem to work reasonably well for multiple Coulomb ionizations by stripped light ions. Background effects in ion-atom collisions are commented upon. Future aspects of atomic Coulomb excitation by incident nuclei and ions are discussed. The interplay between Coulomb induced processes and united atom phenomena is especially mentioned. The simple ionization models have yielded valuable insights but it is suggested that this branch of collision physics has reached a turning point where new and more advanced and unifying models are needed. (JIW)

Optimizing hydroxyurea therapy for sickle cell anemia.

Science.gov (United States)

Ware, Russell E

2015-01-01

Hydroxyurea has proven efficacy in numerous clinical trials as a disease-modifying treatment for patients with sickle cell anemia (SCA) but is currently under-used in clinical practice. To improve the effectiveness of hydroxyurea therapy, efforts should be directed toward broadening the clinical treatment indications, optimizing the daily dosage, and emphasizing the benefits of early and extended treatment. Here, various issues related to hydroxyurea treatment are discussed, focusing on both published evidence and clinical experience. Specific guidance is provided regarding important but potentially unfamiliar aspects of hydroxyurea treatment for SCA, such as escalating to maximum tolerated dose, treating in the setting of cerebrovascular disease, switching from chronic transfusions to hydroxyurea, and using serial phlebotomy to alleviate iron overload. Future research directions to optimize hydroxyurea therapy are also discussed, including personalized dosing based on pharmacokinetic modeling, prediction of fetal hemoglobin responses based on pharmacogenomics, and the risks and benefits of hydroxyurea for non-SCA genotypes and during pregnancy/lactation. Another critical initiative is the introduction of hydroxyurea safely and effectively into global regions that have a high disease burden of SCA but limited resources, such as sub-Saharan Africa, the Caribbean, and India. Final considerations emphasize the long-term goal of optimizing hydroxyurea therapy, which is to help treatment become accepted as standard of care for all patients with SCA. © 2015 by The American Society of Hematology. All rights reserved.

Inner-shell ionization of heavy atoms by slow ions. A study of electronic relativistic effects and projectile Coulomb deflection in the Semiclassical Approximation

International Nuclear Information System (INIS)

Amundsen, P.A.

1978-08-01

Several investigations have been made on K and L shell ionization of the heavy collision partner in slow asymmetric collisions based on the SCA. The use of the SCA can only be defended for slow collisions if the projectile has a charge much less than the target. Thus this approximation should first be tested for proton impact on very heavy target elements. For these elements the inner shell electrons move sufficiently fast for a relativistic description to be mandatory. These relativistic effects are in themselves of some interest, as they can be quite large. After discussion of the formulation of the SCA used throughout this work, a further introduction is given on relativistic effects in Coulomb ionisation. Two papers on electronic relativistic effects in K and L shell ionization follow. The next two papers discuss calculations with an exact Coulomb projectile path. The latter of these also touches upon the inclusion of corrections to the SCA from terms beyond first order perturbation theory. In the last paper of this thesis it is shown how the theoretical apparatus developed for the SCA- calculations can immediately be used also for making calculations of more symmetric systems with the Briggs model. Thus, at least for direct ionization in very slow collisions a unification of the SA and MO approaches has apparently been reached. (JIW)

The effect of marketing knowledge management on sustainable competitive advantage: Evidence from banking industry

Directory of Open Access Journals (Sweden)

Fatemeh Rezaee

2017-11-01

Full Text Available Nowadays, the importance of achieving sustainable organization with competitive advantage in complex environments has many researchers’ interest. So, in this study, we evaluate different views of MKM and its role to reach SCA within banking industry. This research is relatively the scarce empirical study and adds to its originality. The findings offer valuable insights on the generalizability of MKM in a research setting. In fact, the purpose of this study is to empirically test the effect of the marketing knowledge management (MKM on sustainable competitive advantage (SCA within banking industry of Iran. A valid research instrument was utilized to conduct a survey of 150 top- and middle-level managers from Mellat bank of Iran (MBI. With a response rate of 81.3 percent, 122 questionnaires were returned; the number of valid and usable questionnaires was 101. Using exploratory and confirmatory factor analyses, MKM were classified into organizational strategies, culture and performance of senior manager, information technology (IT, research and development (R & D, internal customer (personnel, and external customer (client. Moreover, MBI’ SCA was classified into three dimensions: market, customer, finance. Structural equation modelling was utilized to test the stated hypotheses and model. Statistical support was found for the hypothesized relationships. Moreover it has been shown that MKM maintained the greatest effect on the market centered SCA, while it had the least influence on the customer centered.

Prevalence of Pure Versus Mixed Snow Cover Pixels across Spatial Resolutions in Alpine Environments

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David J. Selkowitz

2014-12-01

and averaged 37 days. Our results suggest that mixed snow-covered snow-free pixels are common at the spatial resolutions imaged by both the Landsat and MODIS sensors. This highlights the additional information available from fractional SCA products and suggests fractional SCA can provide a major advantage for hydrological and climatological monitoring and modeling, particularly when accurate representation of the spatial distribution of snow cover is critical.

Prevalence of pure versus mixed snow cover pixels across spatial resolutions in alpine environments: implications for binary and fractional remote sensing approaches

Science.gov (United States)

Selkowitz, David J.; Forster, Richard; Caldwell, Megan K.

2014-01-01

days. Our results suggest that mixed snow-covered snow-free pixels are common at the spatial resolutions imaged by both the Landsat and MODIS sensors. This highlights the additional information available from fractional SCA products and suggests fractional SCA can provide a major advantage for hydrological and climatological monitoring and modeling, particularly when accurate representation of the spatial distribution of snow cover is critical.

Assessment of biochemical liver function tests in relation to age among steady state sickle cell anemia patients.

Science.gov (United States)

Akuyam, S A; Abubakar, A; Lawal, N; Yusuf, R; Aminu, S M; Hassan, A; Musa, A; Bello, A K; Yahaya, I A; Okafor, P A

2017-11-01

Multiorgan failure including liver dysfunction is a common finding in sickle cell anemia (SCA) patients, the cause of which is multifactorial with advancing age said to be a major determinant. There is a paucity of data on liver function among SCA patients in relation to age in northern Nigerian hospitals, including Ahmadu Bello University Teaching Hospital (ABUTH), Zaria. This study was to assess the biochemical liver function tests (LFTs) as they relate to age among SCA patients in steady state, with a view to improving the overall monitoring of these patients. This study was carried out in ABUTH, Zaria, Northern Nigeria. LFTs were carried out in 100 SCA and 100 apparently healthy participants (controls). The SCA group was made up of fifty adults and fifty children diagnosed of SCA, whereas the control group was made up of fifty adults and fifty children who were apparently healthy and had hemoglobin AA. Paired two-tailed Student's t-test for matched samples and Pearson's linear correlation statistical methods were employed for the data analysis using Microsoft Office Excel 2007. A P ≤ 0.05 was considered as statistically significant. The serum concentrations of total bilirubin (TB), alanine transaminase (ALT), aspartate transaminase (AST), alkaline phosphatase (ALP), and AST/ALT ratio were significantly higher in SCA patients compared to the controls (P = 0.001, P = 0.001, P = 0.05, P = 0.05 and P = 0.001, respectively). Serum total protein (TP) and ALB were significantly lower (P = 0.01 and P < 0.05, respectively) in SCA patients compared with the controls. The levels of TB, ALT, AST, ALP, and AST/ALT were significantly lower in SCA adults compared to SCA children, whereas TP and ALB were higher in SCA adults compared to the SCA children. There were significant negative correlations between age and each of TB, ALT, AST, ALP, and AST/ALT, and significant positive correlations between age and each of TP and ALB in SCA patients. There are mild LFTs derangements

The effect of resource based view on sustainable capability advantage

Directory of Open Access Journals (Sweden)

Mostafa Jafari

2014-12-01

Full Text Available Nowadays, it is important to achieve and to sustain an organization’s competitive advantage in complex environments. This paper evaluates different concepts that have led to such benefits. The study sheds light on resource based view (RBV and its role to reach sustainable competitive advantage (SCA within banking industry of Iran. A valid research instrument was utilized to conduct a survey of 150 top- and middle-level managers from Mellat bank of Iran. With a response rate of 81.3 percent, 122 questionnaires were returned while a number of valid and usable questionnaires were 101. In order to determine validity of questionnaire, the content validity and Cronbach's alpha were used to determine the reliability of the questionnaire (RBV questionnaire 0.934, SCA questionnaire 0.843. The study utilized structural equation modelling, and a series of exploratory and confirmatory factor analyses and they tested the integrated model of MKM and SCA. Statistical support was found for the hypothesized relationships. Moreover it has been shown that RBV had the greatest effect on the market centered SCA, while it had the least influence on the financial centered. The findings offer valuable insights on the generalizability of MKM in a research setting. Structural equation modeling has been implemented and the study also used freedman test to rank the factors and the results show that communication was the most important factor (4.41, followed by process (4.03, knowledge implementation (2.79, decision making (2.54 and human resources (1.22 was the last important factor.

Friedreich's ataxia and other hereditary ataxias in Greece: an 18-year perspective.

Science.gov (United States)

Koutsis, Georgios; Kladi, Athina; Karadima, Georgia; Houlden, Henry; Wood, Nicholas W; Christodoulou, Kyproula; Panas, Marios

2014-01-15

Limited data exist on the spectrum of heredoataxias in Greece, including the prevalence and phenotype of Friedreich's ataxia (FRDA) and the prevalence and subtypes of dominant spinocerebellar ataxias (SCAs). We analyzed clinically and investigated genetically for FRDA and triplet-repeat expansion SCAs a consecutive series of 186 patients with suspected heredoataxia referred to Athens over 18 years. For prevalence estimates we included patients with molecular diagnosis from Cyprus that were absent from the Athens cohort. The minimum prevalence of FRDA was ~0.9/100,000, with clusters of high prevalence in Aegean islands. FRDA was diagnosed in 73 probands. The genotypic and phenotypic spectrum of FRDA was similar to other populations, with one patient compound heterozygote for a known point mutation in FXN (Asn146Lys). Undiagnosed recessive ataxias included FRDA-like and spastic ataxias. The minimum prevalence of dominant SCAs was ~0.7/100,000. SCA1 (4), SCA7 (4), SCA2, SCA6, and SCA17 (1 each) probands were identified. A molecular diagnosis was reached in 31% of dominant cases. Undiagnosed dominant patients included a majority of type III autosomal dominant cerebellar ataxias. FRDA is the commonest heredoataxia in the Greek population with prevalence towards the lower end of other European populations. Dominant SCAs are almost as prevalent. SCA1, SCA2, SCA6, SCA7 and SCA17 patients complete the spectrum of cases with a specific molecular diagnosis. © 2013.

Comparison of SMOS and SMAP Soil Moisture Retrieval Approaches Using Tower-based Radiometer Data over a Vineyard Field

Science.gov (United States)

Miernecki, Maciej; Wigneron, Jean-Pierre; Lopez-Baeza, Ernesto; Kerr, Yann; DeJeu, Richard; DeLannoy, Gabielle J. M.; Jackson, Tom J.; O'Neill, Peggy E.; Shwank, Mike; Moran, Roberto Fernandez;

On the molecular mechanism of surface charge amplification and related phenomena at aqueous polyelectrolyte-graphene interfaces

Directory of Open Access Journals (Sweden)

J.M. Simonson

2011-09-01

Full Text Available In this communication we illustrate the occurrence of a recently reported new phenomenon of surface-charge amplification, SCA, (originally dubbed overcharging, OC, [Jimenez-Angeles F. and Lozada-Cassou M., J. Phys. Chem. B, 2004, 108, 7286] by means of molecular dynamics simulation of aqueous electrolytes solutions involving multivalent cations in contact with charged graphene walls and the presence of short-chain lithium polystyrene sulfonates where the solvent water is described explicitly with a realistic molecular model. We show that the occurrence of SCA in these systems, in contrast to that observed in primitive models, involves neither contact co-adsorption of the negatively charged macroions nor divalent cations with a large size and charge asymmetry as required in the case of implicit solvents. In fact the SCA phenomenon hinges around the preferential adsorption of water (over the hydrated ions with an average dipolar orientation such that the charges of the water's hydrogen and oxygen sites induce magnification rather than screening of the positive-charged graphene surface, within a limited range of surface-charge density.

Subclinical pulmonary involvement in collagen vascular diseases

International Nuclear Information System (INIS)

Dansin, E.; Wallaert, B.; Jardin, M.R.; Remy, J.; Hatron, P.Y.; Tonnel, A.B.

1990-01-01

A recruitment of immune and inflammatory cells into alveolar spaces has been reported in patients with collagen vascular diseases (CVD) and a normal chest radiograph. These findings defined the concept of subclinical alveolitis (SCA). To determine whether SCA may be associated with CT signs of interstitial lung disease (ILD), the authors of this paper compared bronchoalveolar lavage (BAL) findings and high-resolution (HRCT) scans in 36 patients with CVD and normal chest radiographs (systemic sclerosis [SS, n = 21], rheumatoid arthritis [RA, n = 9], primary Sjogren's syndrome [PS, n = 6]). HRCT scans were obtained in supine and prone positions. Results of BAL revealed SCA in 17/36 patients (47%); lymphocyte SCA in 4/36 (24%); neutrophil SCA in 7/36 (41%); and mixed SCA in 6/36 (35%)

Hydroxyurea therapy for sickle cell anemia.

Science.gov (United States)

McGann, Patrick T; Ware, Russell E

2015-01-01

Sickle cell anemia (SCA) is a severe, inherited hemoglobin disorder affecting 100,000 persons in the US and millions worldwide. Hydroxyurea, a once daily oral medication, has emerged as the primary disease-modifying therapy for SCA. The accumulated body of evidence over 30 years demonstrates that hydroxyurea is a safe and effective therapy for SCA, but hydroxyurea remains underutilized for a variety of reasons. In this review, we summarize the available evidence regarding the pharmacology, clinical, and laboratory benefits, and safety of hydroxyurea therapy for the treatment of SCA. The purpose of this review is to provide the reader a comprehensive understanding of hydroxyurea and to reinforce the fact that hydroxyurea is a safe and effective medication for the treatment of SCA. In our opinion, hydroxyurea therapy should be considered standard-of-care for SCA, representing an essential component of patient management. Early initiation and broader use of hydroxyurea will alter the natural history of SCA, so affected children can live longer and healthier lives. In addition, hydroxyurea use should be extended to low-resource settings such as sub-Saharan Africa, where the burden of SCA and the need for hydroxyurea is arguably the greatest.

Stem cell antigen-1 in skeletal muscle function.

Science.gov (United States)

Bernstein, Harold S; Samad, Tahmina; Cholsiripunlert, Sompob; Khalifian, Saami; Gong, Wenhui; Ritner, Carissa; Aurigui, Julian; Ling, Vivian; Wilschut, Karlijn J; Bennett, Stephen; Hoffman, Julien; Oishi, Peter

2013-08-15

Stem cell antigen-1 (Sca-1) is a member of the Ly-6 multigene family encoding highly homologous, glycosyl-phosphatidylinositol-anchored membrane proteins. Sca-1 is expressed on muscle-derived stem cells and myogenic precursors recruited to sites of muscle injury. We previously reported that inhibition of Sca-1 expression stimulated myoblast proliferation in vitro and regulated the tempo of muscle repair in vivo. Despite its function in myoblast expansion during muscle repair, a role for Sca-1 in normal, post-natal muscle has not been thoroughly investigated. We systematically compared Sca-1-/- (KO) and Sca-1+/+ (WT) mice and hindlimb muscles to elucidate the tissue, contractile, and functional effects of Sca-1 in young and aging animals. Comparison of muscle volume, fibrosis, myofiber cross-sectional area, and Pax7+ myoblast number showed little differences between ages or genotypes. Exercise protocols, however, demonstrated decreased stamina in KO versus WT mice, with young KO mice achieving results similar to aging WT animals. In addition, KO mice did not improve with practice, while WT animals demonstrated conditioning over time. Surprisingly, myomechanical analysis of isolated muscles showed that KO young muscle generated more force and experienced less fatigue. However, KO muscle also demonstrated incomplete relaxation with fatigue. These findings suggest that Sca-1 is necessary for muscle conditioning with exercise, and that deficient conditioning in Sca-1 KO animals becomes more pronounced with age.

Dynamical cluster approximation plus semiclassical approximation study for a Mott insulator and d-wave pairing

Science.gov (United States)

Kim, SungKun; Lee, Hunpyo

2017-06-01

Via a dynamical cluster approximation with N c = 4 in combination with a semiclassical approximation (DCA+SCA), we study the doped two-dimensional Hubbard model. We obtain a plaquette antiferromagnetic (AF) Mott insulator, a plaquette AF ordered metal, a pseudogap (or d-wave superconductor) and a paramagnetic metal by tuning the doping concentration. These features are similar to the behaviors observed in copper-oxide superconductors and are in qualitative agreement with the results calculated by the cluster dynamical mean field theory with the continuous-time quantum Monte Carlo (CDMFT+CTQMC) approach. The results of our DCA+SCA differ from those of the CDMFT+CTQMC approach in that the d-wave superconducting order parameters are shown even in the high doped region, unlike the results of the CDMFT+CTQMC approach. We think that the strong plaquette AF orderings in the dynamical cluster approximation (DCA) with N c = 4 suppress superconducting states with increasing doping up to strongly doped region, because frozen dynamical fluctuations in a semiclassical approximation (SCA) approach are unable to destroy those orderings. Our calculation with short-range spatial fluctuations is initial research, because the SCA can manage long-range spatial fluctuations in feasible computational times beyond the CDMFT+CTQMC tool. We believe that our future DCA+SCA calculations should supply information on the fully momentum-resolved physical properties, which could be compared with the results measured by angle-resolved photoemission spectroscopy experiments.

Quantitative Segmentation of Fluorescence Microscopy Images of Heterogeneous Tissue: Application to the Detection of Residual Disease in Tumor Margins.

Science.gov (United States)

Mueller, Jenna L; Harmany, Zachary T; Mito, Jeffrey K; Kennedy, Stephanie A; Kim, Yongbaek; Dodd, Leslie; Geradts, Joseph; Kirsch, David G; Willett, Rebecca M; Brown, J Quincy; Ramanujam, Nimmi

2013-01-01

To develop a robust tool for quantitative in situ pathology that allows visualization of heterogeneous tissue morphology and segmentation and quantification of image features. TISSUE EXCISED FROM A GENETICALLY ENGINEERED MOUSE MODEL OF SARCOMA WAS IMAGED USING A SUBCELLULAR RESOLUTION MICROENDOSCOPE AFTER TOPICAL APPLICATION OF A FLUORESCENT ANATOMICAL CONTRAST AGENT: acriflavine. An algorithm based on sparse component analysis (SCA) and the circle transform (CT) was developed for image segmentation and quantification of distinct tissue types. The accuracy of our approach was quantified through simulations of tumor and muscle images. Specifically, tumor, muscle, and tumor+muscle tissue images were simulated because these tissue types were most commonly observed in sarcoma margins. Simulations were based on tissue characteristics observed in pathology slides. The potential clinical utility of our approach was evaluated by imaging excised margins and the tumor bed in a cohort of mice after surgical resection of sarcoma. Simulation experiments revealed that SCA+CT achieved the lowest errors for larger nuclear sizes and for higher contrast ratios (nuclei intensity/background intensity). For imaging of tumor margins, SCA+CT effectively isolated nuclei from tumor, muscle, adipose, and tumor+muscle tissue types. Differences in density were correctly identified with SCA+CT in a cohort of ex vivo and in vivo images, thus illustrating the diagnostic potential of our approach. The combination of a subcellular-resolution microendoscope, acriflavine staining, and SCA+CT can be used to accurately isolate nuclei and quantify their density in anatomical images of heterogeneous tissue.

From virtual clustering analysis to self-consistent clustering analysis: a mathematical study

Science.gov (United States)

Tang, Shaoqiang; Zhang, Lei; Liu, Wing Kam

2018-03-01

In this paper, we propose a new homogenization algorithm, virtual clustering analysis (VCA), as well as provide a mathematical framework for the recently proposed self-consistent clustering analysis (SCA) (Liu et al. in Comput Methods Appl Mech Eng 306:319-341, 2016). In the mathematical theory, we clarify the key assumptions and ideas of VCA and SCA, and derive the continuous and discrete Lippmann-Schwinger equations. Based on a key postulation of "once response similarly, always response similarly", clustering is performed in an offline stage by machine learning techniques (k-means and SOM), and facilitates substantial reduction of computational complexity in an online predictive stage. The clear mathematical setup allows for the first time a convergence study of clustering refinement in one space dimension. Convergence is proved rigorously, and found to be of second order from numerical investigations. Furthermore, we propose to suitably enlarge the domain in VCA, such that the boundary terms may be neglected in the Lippmann-Schwinger equation, by virtue of the Saint-Venant's principle. In contrast, they were not obtained in the original SCA paper, and we discover these terms may well be responsible for the numerical dependency on the choice of reference material property. Since VCA enhances the accuracy by overcoming the modeling error, and reduce the numerical cost by avoiding an outer loop iteration for attaining the material property consistency in SCA, its efficiency is expected even higher than the recently proposed SCA algorithm.

Spinocerebellar ataxia: a critical review of cognitive and socio-cognitive deficits.

Science.gov (United States)

Giocondo, Flora; Curcio, Giuseppe

2018-02-01

The primary aim of this contribution is to provide a critical discussion on cognitive and sociocognitive implications of spinocerebellar ataxias (SCAs) subtypes. The term SCA refers to a group of neurodegenerative disorders that have been increasingly investigated in the last years, sharing the characteristic of progressive ataxia resulting from degeneration of cerebellum and its connections. In past decades only involvement of cerebellum in behaviour and timing has been investigated, bringing to the belief about its central role in timing of movement and sensation, particularly for short intervals of time. Only very recently the cerebellum has been considered as a potentially important centre for cognitive processing and related spheres of social cognition, so that several studies with SCA patients have been carried out on these topics: as a consequence a section of this review will be dedicated to this important aspect. After a brief discussion on most commonly used methods to assess cognitive and socio-cognitive abilities in SCAs, cognitive and socio-cognitive profiles of principal SCA subtypes have been thoroughly reviewed and critically discussed. Due to the very poor literature in this field the most common SCA variants have been fully included (i.e. SCA1, SCA2, SCA3, SCA6 and SCA7). A comparative summary of the main characteristics of cognitive and social cognition deficit in SCA subtypes has been proposed together with a research agenda for future investigation in this field principally aimed at using measures of cognition and/or social cognition as potential predictors of the extent and progression of disease.

Exploring the architectural trade space of NASAs Space Communication and Navigation Program

Science.gov (United States)

Sanchez, M.; Selva, D.; Cameron, B.; Crawley, E.; Seas, A.; Seery, B.

NASAs Space Communication and Navigation (SCaN) Program is responsible for providing communication and navigation services to space missions and other users in and beyond low Earth orbit. The current SCaN architecture consists of three independent networks: the Space Network (SN), which contains the TDRS relay satellites in GEO; the Near Earth Network (NEN), which consists of several NASA owned and commercially operated ground stations; and the Deep Space Network (DSN), with three ground stations in Goldstone, Madrid, and Canberra. The first task of this study is the stakeholder analysis. The goal of the stakeholder analysis is to identify the main stakeholders of the SCaN system and their needs. Twenty-one main groups of stakeholders have been identified and put on a stakeholder map. Their needs are currently being elicited by means of interviews and an extensive literature review. The data will then be analyzed by applying Cameron and Crawley's stakeholder analysis theory, with a view to highlighting dominant needs and conflicting needs. The second task of this study is the architectural tradespace exploration of the next generation TDRSS. The space of possible architectures for SCaN is represented by a set of architectural decisions, each of which has a discrete set of options. A computational tool is used to automatically synthesize a very large number of possible architectures by enumerating different combinations of decisions and options. The same tool contains models to evaluate the architectures in terms of performance and cost. The performance model uses the stakeholder needs and requirements identified in the previous steps as inputs, and it is based in the VASSAR methodology presented in a companion paper. This paper summarizes the current status of the MIT SCaN architecture study. It starts by motivating the need to perform tradespace exploration studies in the context of relay data systems through a description of the history NASA's space communicati

Capillary electrophoresis fragment analysis and clone sequencing in detection of dynamic mutations of spinocerebellar ataxia

Directory of Open Access Journals (Sweden)

Yuan-yuan CHEN

2018-04-01

Full Text Available Objective To estimate the accuracy and stability of capillary electrophoresis fragment analysis and clone sequencing in detecting dynamic mutations of spinocerebellar ataxia (SCA. Methods Capillary electrophoresis fragment analysis and clone sequencing were used in detecting trinucleotide repeated sequence of 14 SCA patients (3 cases of SCA2, 2 cases of SCA7, 7 cases of SCA8 and 2 cases of SCA17. Results Capillary electrophoresis fragment analysis of 3 SCA2 cases showed the expanded cytosine-adenine-guanine (CAG repeats were 31, 30 and 32, and the copy numbers of 3 clone sequencing for 3 colonies in each case were 37/40/40, 37/38/39 and 38/39/40 respectively. Capillary electrophoresis fragment analysis of 2 SCA7 cases showed the expanded CAG repeats were 57 and 34, and the copy numbers of repeats were 69, 74, 75 in 3 colonies of one case, and was 45 in the other case. For the 7 SCA8 cases with the expanded cytosine-thymine-adenine (CTA/cytosine-thymine-guanine (CTG repeats of 99, 111, 104, 92, 89, 104 and 75, the results of clone sequencing were 97, 116, 104, 90, 90, 102 and 76 respectively. For 2 SCA17 cases with the short/expanded CAG repeats of 37/50 and 36/45, the results of clone sequencing were 51/50/52 and 45/44 for 3 and 2 colonies. Conclusions Although the higher mobility of polymerase chain reaction (PCR products containing dynamic mutation in the capillary electrophoresis fragment analysis might cause the deviation for analysis of copy numbers, the deviation was predictable and the results were repeatable. The clone sequencing results showed obvious instability, especially for SCA2 and SCA7 genes, which might owing to their simple CAG repeats. Consequently, clone sequencing is not suited for detection of dynamic mutation, not to mention the quantitative criteria of dynamic mutation sequencing. DOI: 10.3969/j.issn.1672-6731.2018.03.008

Cellulosomics, a gene-centric approach to investigating the intraspecific diversity and adaptation of Ruminococcus flavefaciens within the rumen.

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Jennifer M Brulc

Full Text Available BACKGROUND: The bovine rumen maintains a diverse microbial community that serves to break down indigestible plant substrates. However, those bacteria specifically adapted to degrade cellulose, the major structural component of plant biomass, represent a fraction of the rumen microbiome. Previously, we proposed scaC as a candidate for phylotyping Ruminococcus flavefaciens, one of three major cellulolytic bacterial species isolated from the rumen. In the present report we examine the dynamics and diversity of scaC-types both within and between cattle temporally, following a dietary switch from corn-silage to grass-legume hay. These results were placed in the context of the overall bacterial population dynamics measured using the 16S rRNA. PRINCIPAL FINDINGS: As many as 117 scaC-types were estimated, although just nineteen were detected in each of three rumens tested, and these collectively accounted for the majority of all types present. Variation in scaC populations was observed between cattle, between planktonic and fiber-associated fractions and temporally over the six-week survey, and appeared related to scaC phylogeny. However, by the sixth week no significant separation of scaC populations was seen between animals, suggesting enrichment of a constrained set of scaC-types. Comparing the amino-acid translation of each scaC-type revealed sequence variation within part of the predicted dockerin module but strong conservation in the N-terminus, where the cohesin module is located. CONCLUSIONS: The R. flavefaciens species comprises a multiplicity of scaC-types in-vivo. Enrichment of particular scaC-types temporally, following a dietary switch, and between fractions along with the phylogenetic congruence suggests that functional differences exist between types. Observed differences in dockerin modules suggest at least part of the functional heterogeneity may be conferred by scaC. The polymorphic nature of scaC enables the relative distribution of R

Modulating Leukemia-Initiating Cell Quiescence to Improve Leukemia Treatment

Science.gov (United States)

2015-09-01

T- cells and in innate immunity (Lacorazza et al., 2002). It controls the proliferation and homing of CD8+ T- cells via the Kruppel-like factors...Lin2Sca12IL7R2Kit1FccRII/ IIIhighCD34high), megakaryocyte-erythroid progenitor cell (MEP) (Lin2Sca12IL7R2Kit1FccRII/IIIlowCD34low), and common lymphoid ...to this model, the first wave gives rise exclusively to innate immune B cells in early embryonic life and may be derived from progenitor cells

Delay and Disruption Tolerant Networking MACHETE Model

Science.gov (United States)

Segui, John S.; Jennings, Esther H.; Gao, Jay L.

2011-01-01

To verify satisfaction of communication requirements imposed by unique missions, as early as 2000, the Communications Networking Group at the Jet Propulsion Laboratory (JPL) saw the need for an environment to support interplanetary communication protocol design, validation, and characterization. JPL's Multi-mission Advanced Communications Hybrid Environment for Test and Evaluation (MACHETE), described in Simulator of Space Communication Networks (NPO-41373) NASA Tech Briefs, Vol. 29, No. 8 (August 2005), p. 44, combines various commercial, non-commercial, and in-house custom tools for simulation and performance analysis of space networks. The MACHETE environment supports orbital analysis, link budget analysis, communications network simulations, and hardware-in-the-loop testing. As NASA is expanding its Space Communications and Navigation (SCaN) capabilities to support planned and future missions, building infrastructure to maintain services and developing enabling technologies, an important and broader role is seen for MACHETE in design-phase evaluation of future SCaN architectures. To support evaluation of the developing Delay Tolerant Networking (DTN) field and its applicability for space networks, JPL developed MACHETE models for DTN Bundle Protocol (BP) and Licklider/Long-haul Transmission Protocol (LTP). DTN is an Internet Research Task Force (IRTF) architecture providing communication in and/or through highly stressed networking environments such as space exploration and battlefield networks. Stressed networking environments include those with intermittent (predictable and unknown) connectivity, large and/or variable delays, and high bit error rates. To provide its services over existing domain specific protocols, the DTN protocols reside at the application layer of the TCP/IP stack, forming a store-and-forward overlay network. The key capabilities of the Bundle Protocol include custody-based reliability, the ability to cope with intermittent connectivity

Reasons for not using smoking cessation aids

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Völzke Henry

2008-04-01

Full Text Available Abstract Background Few smokers use effective smoking cessation aids (SCA when trying to stop smoking. Little is known why available SCA are used insufficiently. We therefore investigated the reasons for not using SCA and examined related demographic, smoking behaviour, and motivational variables. Methods Data were collected in two population-based studies testing smoking cessation interventions in north-eastern Germany. A total of 636 current smokers who had never used SCA and had attempted to quit or reduce smoking within the last 12 months were given a questionnaire to assess reasons for non-use. The questionnaire comprised two subscales: "Social and environmental barriers" and "SCA unnecessary." Results The most endorsed reasons for non-use of SCA were the belief to be able to quit on one's own (55.2%, the belief that help is not necessary (40.1%, and the belief that smoking does not constitute a big problem in one's life (36.5%. One quarter of all smokers reported that smoking cessation aids are not helpful in quitting and that the aids cost too much. Smokers intending to quit agreed stronger to both subscales and smokers with lower education agreed stronger to the subscale "Social and environmental barriers". Conclusion Main reasons for non-use of SCA are being overly self-confident and the perception that SCA are not helpful. Future interventions to increase the use of SCA should address these reasons in all smokers.

Superior cerebellar artery infarction in endovascular treatment for tentorial dural arteriovenous fistulas

Energy Technology Data Exchange (ETDEWEB)

Zhang Jingbo; Lv Xianli; Jiang Chuhan; Li Youxiang [Beijing Neurosurgical Institute and Beijing Tiantan Hospital, Capital Medical University, 6, Tiantan, Xili, Chongwen, 100050, Beijing (China); Wu Zhongxue, E-mail: ttyyzjb@sina.co [Beijing Neurosurgical Institute and Beijing Tiantan Hospital, Capital Medical University, 6, Tiantan, Xili, Chongwen, 100050, Beijing (China)

2010-06-15

Background: Superior cerebellar artery (SCA) syndrome shows ipsilateral cerebellar ataxia and Horner's syndrome, contralateral superficial sensory disturbance, as well as nystagmus toward the impaired side, vertigo, and nausea. Occasionally, unilateral lesions may produce bilateral hypogeusia and contralateral hypoacusia. Objective: To report 2 patients with unilateral lower midbrain ischemic lesions of the inferior colliculus level caused by transarterial embolization for tentorial dural arteriovenous fistulas (TDAVFs). Methods: Hospital records for 21 patients with TDAVFs mainly treated by endovascular techniques between 2005 and 2008 were reviewed. Two patients with MRI evidence of unilateral SCA territory infarction were investigated. Results: Of 21 patients, 2 treated transarterially with Onyx-18 (a nonahesive liquid embolic agent) developed infarctions in the territory of SCA. One patient had lateral SCA infarction characterized by ipsilateral gait ataxia, contralateral hemihypoesthesia, with additional ipsilateral ocular motor palsy and bilateral gustatory loss. And the other patient had medial SCA infarction characterized by ipsilateral ataxia contralateral hemihypoesthesia with additional contralateral hypoacusia. Conclusion: SCA infarction can be caused by transarterial injection of Onyx-18 via SCA or the posterior cerebral artery (PCA) for TDAVFs and additionally presented with gustatory loss and deafness, which is generally not a feature of the SCA syndrome.

Variation in the gut microbiota of laboratory mice is related to both genetic and environmental factors

DEFF Research Database (Denmark)

Hufeldt, Majbritt Ravn; Nielsen, Dennis Sandris; Vogensen, Finn Kvist

2010-01-01

:NMRI stock. Comparing C57BL/6 mice from 2 vendors revealed significant differences in the microbial profile, whereas the profiles of C57BL/6Sca mice raised in separate rooms within the same breeding center were not significantly different. Furthermore, housing in individually ventilated cages did not lead......During recent years, the composition of the gut microbiota (GM) has received increasing attention as a factor in the development of experimental inflammatory disease in animal models. Because increased variation in the GM might lead to increased variation in disease parameters, determining...... microbiota in 8-wk-old NMRI and C57BL/6 mice by using denaturing gradient gel electrophoresis to profile PCR-derived amplicons from bacterial 16S rRNA genes. Comparison of the cecal microbiotas revealed that the similarity index of the inbred C57BL/6Sca strain was 10% higher than that of the outbred Sca...

Heat shock proteins as potential targets for protective strategies in neurodegeneration

NARCIS (Netherlands)

Kampinga, Harm H.; Bergink, Steven

Protein aggregates are hallmarks of nearly all age-related neurodegenerative diseases, including Alzheimer's disease, Parkinson's disease, amyotrophic lateral sclerosis, and several polyglutamine diseases such as Huntington's disease and different forms of spinocerebellar ataxias (SCA; SCA1-3, SCA6,

Stem Cell Antigen-1 in Skeletal Muscle Function

OpenAIRE

Bernstein, Harold S.; Samad, Tahmina; Cholsiripunlert, Sompob; Khalifian, Saami; Gong, Wenhui; Ritner, Carissa; Aurigui, Julian; Ling, Vivian; Wilschut, Karlijn J.; Bennett, Stephen; Hoffman, Julien; Oishi, Peter

2013-01-01

Stem cell antigen-1 (Sca-1) is a member of the Ly-6 multigene family encoding highly homologous, glycosyl-phosphatidylinositol-anchored membrane proteins. Sca-1 is expressed on muscle-derived stem cells and myogenic precursors recruited to sites of muscle injury. We previously reported that inhibition of Sca-1 expression stimulated myoblast proliferation in vitro and regulated the tempo of muscle repair in vivo. Despite its function in myoblast expansion during muscle repair, a role for Sca-1...

[Spinocerebellar ataxia type 2 associated to pigmentary retinitis].

Science.gov (United States)

Jiménez-Caballero, Pedro Enrique; Serviá, Mónica

2010-07-01

Ocular disorders are useful in the characterisation of the different types of spinocerebellar ataxias (SCA); pigmentary retinitis is an alteration that is specifically associated to SCA type 7 and is characterised by night blindness, sensitivity to glare and progressive narrowing of the visual field. A 34-year-old woman with clinical symptoms of progressive ataxia and visual impairment secondary to pigmentary retinitis. The patient had a personal history with an autosomal dominant pattern of a similar disorder in her father and paternal grandmother. In the genetic study she presented a triplet expansion in the SCA type 2 gene. CONCLUSIONS; Although pigmentary retinitis belongs to the SCA type 7 phenotype, our patient presented this retinal disorder, as in other cases of SCA type 2. A genetic study for SCA type 2 must therefore be conducted in patients with a degenerative ataxic clinical picture and who present evidence of pigmentary retinitis.

Quantitative Segmentation of Fluorescence Microscopy Images of Heterogeneous Tissue: Application to the Detection of Residual Disease in Tumor Margins.

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Jenna L Mueller

Full Text Available To develop a robust tool for quantitative in situ pathology that allows visualization of heterogeneous tissue morphology and segmentation and quantification of image features.TISSUE EXCISED FROM A GENETICALLY ENGINEERED MOUSE MODEL OF SARCOMA WAS IMAGED USING A SUBCELLULAR RESOLUTION MICROENDOSCOPE AFTER TOPICAL APPLICATION OF A FLUORESCENT ANATOMICAL CONTRAST AGENT: acriflavine. An algorithm based on sparse component analysis (SCA and the circle transform (CT was developed for image segmentation and quantification of distinct tissue types. The accuracy of our approach was quantified through simulations of tumor and muscle images. Specifically, tumor, muscle, and tumor+muscle tissue images were simulated because these tissue types were most commonly observed in sarcoma margins. Simulations were based on tissue characteristics observed in pathology slides. The potential clinical utility of our approach was evaluated by imaging excised margins and the tumor bed in a cohort of mice after surgical resection of sarcoma.Simulation experiments revealed that SCA+CT achieved the lowest errors for larger nuclear sizes and for higher contrast ratios (nuclei intensity/background intensity. For imaging of tumor margins, SCA+CT effectively isolated nuclei from tumor, muscle, adipose, and tumor+muscle tissue types. Differences in density were correctly identified with SCA+CT in a cohort of ex vivo and in vivo images, thus illustrating the diagnostic potential of our approach.The combination of a subcellular-resolution microendoscope, acriflavine staining, and SCA+CT can be used to accurately isolate nuclei and quantify their density in anatomical images of heterogeneous tissue.

Development of steady-state model for MSPT and detailed analyses of receiver

Science.gov (United States)

Yuasa, Minoru; Sonoda, Masanori; Hino, Koichi

2016-05-01

Molten salt parabolic trough system (MSPT) uses molten salt as heat transfer fluid (HTF) instead of synthetic oil. The demonstration plant of MSPT was constructed by Chiyoda Corporation and Archimede Solar Energy in Italy in 2013. Chiyoda Corporation developed a steady-state model for predicting the theoretical behavior of the demonstration plant. The model was designed to calculate the concentrated solar power and heat loss using ray tracing of incident solar light and finite element modeling of thermal energy transferred into the medium. This report describes the verification of the model using test data on the demonstration plant, detailed analyses on the relation between flow rate and temperature difference on the metal tube of receiver and the effect of defocus angle on concentrated power rate, for solar collector assembly (SCA) development. The model is accurate to an extent of 2.0% as systematic error and 4.2% as random error. The relationships between flow rate and temperature difference on metal tube and the effect of defocus angle on concentrated power rate are shown.

The 3-Second Rule in Hereditary Pure Cerebellar Ataxia: A Synchronized Tapping Study

Science.gov (United States)

Matsuda, Shunichi; Matsumoto, Hideyuki; Furubayashi, Toshiaki; Hanajima, Ritsuko; Tsuji, Shoji; Ugawa, Yoshikazu; Terao, Yasuo

2015-01-01

The ‘3-second rule’ has been proposed based on miscellaneous observations that a time period of around 3 seconds constitutes the fundamental unit of time related to the neuro-cognitive machinery in normal humans. The aim of paper was to investigate the temporal processing in patients with spinocerebellar ataxia type 6 (SCA6) and SCA31, pure cerebellar types of spinocerebellar degeneration, using a synchronized tapping task. Seventeen SCA patients (11 SCA6, 6 SCA31) and 17 normal age-matched volunteers participated. The task required subjects to tap a keyboard in synchrony with sequences of auditory stimuli presented at fixed interstimulus intervals (ISIs) between 200 and 4800 ms. In this task, the subjects required non-motor components to estimate the time of forthcoming tone in addition to motor components to tap. Normal subjects synchronized their taps to the presented tones at shorter ISIs, whereas as the ISI became longer, the normal subjects displayed greater latency between the tone and the tapping (transition zone). After the transition zone, normal subjects pressed the button delayed relative to the tone. On the other hand, SCA patients could not synchronize their tapping with the tone even at shorter ISIs, although they pressed the button delayed relative to the tone earlier than normal subjects did. The earliest time of delayed tapping appearance after the transition zone was 4800 ms in normal subjects but 1800 ms in SCA patients. The span of temporal integration in SCA patients is shortened compared to that in normal subjects. This could represent non-motor cerebellar dysfunction in SCA patients. PMID:25706752

Progression of Dysphagia in Spinocerebellar Ataxia Type 6.

Science.gov (United States)

Isono, Chiharu; Hirano, Makito; Sakamoto, Hikaru; Ueno, Shuichi; Kusunoki, Susumu; Nakamura, Yusaku

2017-06-01

Spinocerebellar ataxia type 6 (SCA6), an autosomal dominant triplet repeat disease, predominantly affects the cerebellum with a late onset and generally good prognosis. Dysphagia is commonly associated with the outcomes of neurodegenerative diseases such as SCA6. Although the characteristics of dysphagia have been rarely reported in SCA6, our previous study indicated that dysphagia is generally milder in SCA6 than in SCA3, another inherited ataxia with multisystem involvement. However, abnormalities in the pharyngeal phase in SCA6 were indistinguishable from those in SCA3, with no explainable reason. To determine the reason, we repeatedly performed videofluoroscopic examinations (VF) in 14 patients with SCA6. The results showed that the gross progression of dysphagia was apparently slow, but four patients had progressive dysphagia at an early disease stage; dysphagia began within 10 years from the onset of ataxia and rapidly progressed. A common clinical feature of the four patients was a significantly older age at the onset of ataxia (74.0 vs. 60.3 years), associated with significantly shorter triplet repeats. This finding surprisingly indicated that patients who had shorter repeats and thereby later onset and potentially better prognoses were at risk for dysphagia-associated problems. Ischemic changes, homozygous mutation, and diabetes mellitus as well as aging might have contributed to the observed progressive dysphagia. We found that conventionally monitored somatosensory evoked potentials at least partly reflected progressive dysphagia. Despite the small study group, our findings suggest that clinicians should carefully monitor dysphagia in patients with SCA6 who are older at disease onset (>60 years).

Determination of Cu/Zn and Fe in human serum of patients with sickle cell anemia using radiation synchrotron

Energy Technology Data Exchange (ETDEWEB)

Canellas, C.G.L. [Nuclear Instrumentation Laboratory, COPPE/UFRJ, P.O. Box 68509, 21.941-972 Rio de Janeiro (Brazil); Carvalho, S.M.F. [State Institute of Hematology Arthur de Siqueira Cavalcanti, 20.211-030 Rio de Janeiro (Brazil); Anjos, M.J. [Nuclear Instrumentation Laboratory, COPPE/UFRJ, P.O. Box 68509, 21.941-972 Rio de Janeiro (Brazil); Physics Institute, State University of Rio de Janeiro, 20.559-900 Rio de Janeiro (Brazil); Lopes, R.T., E-mail: ricardo@lin.ufrj.br [Nuclear Instrumentation Laboratory, COPPE/UFRJ, P.O. Box 68509, 21.941-972 Rio de Janeiro (Brazil)

2012-07-15

In this work we analyzed serum samples from patients with Sickle Cell Anemia (SCA) using Total Reflection X-Ray Fluorescence using Synchrotron Radiation (SRTXRF). The SRTXRF measurements were performed at the X-Ray Fluorescence Beamline at the Brazilian National Synchrotron Light Laboratory (LNLS). We studied forty-three patients aged 18-50 suffering from SCA and sixty healthy volunteers aged 18-60. It was possible to determine the concentrations of the following elements: P, S, Cl, K, Ca, Fe, Cu, Zn, Br and Rb. Moreover, there are evidences of an association among Fe, Cu, Zn and Cu/Zn in the SCA pathogenesis process. The concentrations of Fe and Cu in the serum samples of patients with SCA were larger, 120% and 20%, respectively, when compared with the CG. The serum level Cu/Zn ratio was significantly higher (60%) in the serum samples from patients suffering from SCA than from the CG. Therefore, the Cu/Zn ratio can be used as an adjuvant index in enhancement for diagnosis of SCA. - Highlights: Black-Right-Pointing-Pointer Serum samples from patients with Sickle Cell Anemia (SCA) were analyzed by SRTXRF. Black-Right-Pointing-Pointer It was possible to determine the concentrations of the P, S, Cl, K, Ca, Fe, Cu, Zn, Br and Rb. Black-Right-Pointing-Pointer There are evidences of an association among Fe, Cu, Zn and Cu/Zn in the SCA process. Black-Right-Pointing-Pointer The results indicate that the Cu/Zn ratio can be used as an adjuvant index for diagnosis of SCA.

Determination of Cu/Zn and Fe in human serum of patients with sickle cell anemia using radiation synchrotron

International Nuclear Information System (INIS)

Canellas, C.G.L.; Carvalho, S.M.F.; Anjos, M.J.; Lopes, R.T.

2012-01-01

In this work we analyzed serum samples from patients with Sickle Cell Anemia (SCA) using Total Reflection X-Ray Fluorescence using Synchrotron Radiation (SRTXRF). The SRTXRF measurements were performed at the X-Ray Fluorescence Beamline at the Brazilian National Synchrotron Light Laboratory (LNLS). We studied forty-three patients aged 18–50 suffering from SCA and sixty healthy volunteers aged 18–60. It was possible to determine the concentrations of the following elements: P, S, Cl, K, Ca, Fe, Cu, Zn, Br and Rb. Moreover, there are evidences of an association among Fe, Cu, Zn and Cu/Zn in the SCA pathogenesis process. The concentrations of Fe and Cu in the serum samples of patients with SCA were larger, 120% and 20%, respectively, when compared with the CG. The serum level Cu/Zn ratio was significantly higher (60%) in the serum samples from patients suffering from SCA than from the CG. Therefore, the Cu/Zn ratio can be used as an adjuvant index in enhancement for diagnosis of SCA. - Highlights: ► Serum samples from patients with Sickle Cell Anemia (SCA) were analyzed by SRTXRF. ► It was possible to determine the concentrations of the P, S, Cl, K, Ca, Fe, Cu, Zn, Br and Rb. ► There are evidences of an association among Fe, Cu, Zn and Cu/Zn in the SCA process. ► The results indicate that the Cu/Zn ratio can be used as an adjuvant index for diagnosis of SCA.

Jaw Osteomyelitis as a Complication of Sickle Cell Anaemia in Three Omani Patients; Case reports and literature review

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Hilal Al-Ismaili

2017-03-01

Full Text Available Sickle cell anaemia (SCA is a common haemoglobinopathy among people from the Middle East, the Afro-Caribbean region, the Mediterranean and East India. While osteomyelitis of the long bones is a welldocumented complication of SCA, there are few documented cases of SCA patients presenting with jaw osteomyelitis. We report three SCA patients with chronic jaw osteomyelitis who presented to the Department of Oral Health, Sultan Qaboos University Hospital, Muscat, Oman, between 2009 and 2013. Two of the patients had osteomyelitis of the mandible and the third had osteomyelitis of the maxilla. In addition, a brief review of the literature is presented focusing on the clinical presentation, diagnosis and management of jaw osteomyelitis among patients with SCA.

SmallSat Database

Science.gov (United States)

Petropulos, Dolores; Bittner, David; Murawski, Robert; Golden, Bert

2015-01-01

The SmallSat has an unrealized potential in both the private industry and in the federal government. Currently over 70 companies, 50 universities and 17 governmental agencies are involved in SmallSat research and development. In 1994, the U.S. Army Missile and Defense mapped the moon using smallSat imagery. Since then Smart Phones have introduced this imagery to the people of the world as diverse industries watched this trend. The deployment cost of smallSats is also greatly reduced compared to traditional satellites due to the fact that multiple units can be deployed in a single mission. Imaging payloads have become more sophisticated, smaller and lighter. In addition, the growth of small technology obtained from private industries has led to the more widespread use of smallSats. This includes greater revisit rates in imagery, significantly lower costs, the ability to update technology more frequently and the ability to decrease vulnerability of enemy attacks. The popularity of smallSats show a changing mentality in this fast paced world of tomorrow. What impact has this created on the NASA communication networks now and in future years? In this project, we are developing the SmallSat Relational Database which can support a simulation of smallSats within the NASA SCaN Compatability Environment for Networks and Integrated Communications (SCENIC) Modeling and Simulation Lab. The NASA Space Communications and Networks (SCaN) Program can use this modeling to project required network support needs in the next 10 to 15 years. The SmallSat Rational Database could model smallSats just as the other SCaN databases model the more traditional larger satellites, with a few exceptions. One being that the smallSat Database is designed to be built-to-order. The SmallSat database holds various hardware configurations that can be used to model a smallSat. It will require significant effort to develop as the research material can only be populated by hand to obtain the unique data

Repeat interruptions in spinocerebellar ataxia type 10 expansions are strongly associated with epileptic seizures

Science.gov (United States)

McFarland, Karen N.; Liu, Jilin; Landrian, Ivette; Zeng, Desmond; Raskin, Salmo; Moscovich, Mariana; Gatto, Emilia M.; Ochoa, Adriana; Teive, Hélio A. G.; Rasmussen, Astrid; Ashizawa, Tetsuo

2014-01-01

Spinocerebellar ataxia type 10 (SCA10), an autosomal dominant neurodegenerative disorder, is the result of a non-coding, pentanucleotide repeat expansion within intron 9 of the Ataxin 10 gene. SCA10 patients present with pure cerebellar ataxia; yet, some families also have a high incidence of epilepsy. SCA10 expansions containing penta- and heptanucleotide interruption motifs, termed “ATCCT interruptions,” experience large contractions during germline transmission, particularly in paternal lineages. At the same time, these alleles confer an earlier age at onset which contradicts traditional rules of genetic anticipation in repeat expansions. Previously, ATCCT interruptions have been associated with a higher prevalence of epileptic seizures in one Mexican-American SCA10 family. In a large cohort of SCA10 families, we analyzed whether ATCCT interruptions confers a greater risk for developing seizures in these families. Notably, we find that the presence of repeat interruptions within the SCA10 expansion confers a 6.3-fold increase in the risk of an SCA10 patient developing epilepsy (6.2-fold when considering patients of Mexican ancestry only) and a 13.7-fold increase in having a positive family history of epilepsy (10.5-fold when considering patients of Mexican ancestry only). We conclude that the presence of repeat interruptions in SCA10 repeat expansion indicates a significant risk for the epilepsy phenotype and should be considered during genetic counseling. PMID:24318420

Alteration of Lymphocyte Phenotype and Function in Sickle Cell Anemia: Implications for Vaccine Responses

Science.gov (United States)

Balandya, Emmanuel; Reynolds, Teri; Obaro, Stephen; Makani, Julie

2016-01-01

Individuals with sickle cell anemia (SCA) have increased susceptibility to infections, secondary to impairment of immune function. Besides the described dysfunction in innate immunity, including impaired opsonization and phagocytosis of bacteria, evidence of dysfunction of T and B lymphocytes in SCA has also been reported. This includes reduction in the proportion of circulating CD4+ and CD8+ T cells, reduction of CD4+ helper : CD8+ suppressor T cell ratio, aberrant activation and dysfunction of regulatory T cells (Treg), skewing of CD4+ T cells towards Th2 response and loss of IgM-secreting CD27+IgMhighIgDlow memory B cells. These changes occur on the background of immune activation characterized by predominance of memory CD4+ T cell phenotypes, increased Th17 signaling and elevated levels of C-reactive protein and pro-inflammatory cytokines IL-6 and TNF-α, which may affect the immunogenicity and protective efficacy of vaccines available to prevent infections in SCA. Thus, in order to optimize the use of vaccines in SCA, a thorough understanding of T and B lymphocyte functions and vaccine reactivity among individuals with SCA is needed. Studies should be encouraged of different SCA populations, including sub-Saharan Africa where the burden of SCA is highest. This article summarizes our current understanding of lymphocyte biology in SCA, and highlights areas that warrant future research. PMID:27237467

Sheltering the self from the storm: self-construal abstractness and the stability of self-esteem.

Science.gov (United States)

Updegraff, John A; Emanuel, Amber S; Suh, Eunkook M; Gallagher, Kristel M

2010-01-01

Self-construal abstractness (SCA) refers to the degree to which people construe important bases of self-esteem in a broad, flexible, and abstract rather than a concrete and specific manner. This article hypothesized that SCA would be a unique predictor of self-esteem stability, capturing the degree to which people's most important bases of self-worth are resistant to disconfirmation. Two studies using a daily diary methodology examined relationships between SCA, daily self-esteem, and daily emotions and/or events. In Study 1, individual differences in SCA emerged as the most consistent and unique predictor of self-esteem stability. Furthermore, SCA contributed to self-esteem stability by buffering the influence of daily negative emotions on self-esteem. Study 2 manipulated SCA via a daily self-construal task and found an abstract versus concrete self-focus to buffer the influence of daily negative events on self-esteem. Implications of these findings for the study of the self and well-being are discussed.

Bone Marrow Transplantation (BMT) and Gene Replacement ...

African Journals Online (AJOL)

Abstract. Background: Sickle cell anemia (SCA) forms one of the neglected tropical disease of genetic aetiology. Unlike many complex genetic diseases inherited on a multiallelic pattern, SCA is a hemoglobinopathy of Mendelian type genetic inheritance. The SCA trait is inherited through a recessive autosomal link, with the ...

Tissue-resident Sca1+ PDGFRα+ mesenchymal progenitors are the cellular source of fibrofatty infiltration in arrhythmogenic cardiomyopathy [v1; ref status: indexed, http://f1000r.es/17s

Directory of Open Access Journals (Sweden)

Ben Paylor

2013-06-01

Full Text Available Arrhythmogenic cardiomyopathy (AC is a disease of the heart involving myocardial dystrophy leading to fibrofatty scarring of the myocardium and is associated with an increased risk of both ventricular arrhythmias and sudden cardiac death. It often affects the right ventricle but may also involve the left. Although there has been significant progress in understanding the role of underlying desmosomal genetic defects in AC, there is still a lack of data regarding the cellular processes involved in its progression. The development of cardiac fibrofatty scarring is known to be a principal pathological process associated with ventricular arrhythmias, and it is vital that we elucidate the role of various cell populations involved in the disease if targeted therapeutics are to be developed. The known role of mesenchymal progenitor cells in the reparative process of both the heart and skeletal muscle has provided inspiration for the identification of the cellular basis of fibrofatty infiltration in AC. Here we hypothesize that reparative processes triggered by myocardial degeneration lead to the differentiation of tissue-resident Sca1+ PDGFRα+ mesenchymal progenitors into adipocytes and fibroblasts, which compose the fibrofatty lesions characteristic of AC.

Application of magnetic poly(styrene-glycidyl methacrylate) microspheres for immunomagnetic separation of bone marrow cells

Energy Technology Data Exchange (ETDEWEB)

Chung, T.-H.; Chang, J.-Y. [Department of Chemical Engineering, National Chung Cheng University, Chiayi 621, Taiwan (China); Lee, W.-C. [Department of Chemical Engineering, National Chung Cheng University, Chiayi 621, Taiwan (China)], E-mail: chmwcl@ccu.edu.tw

2009-05-15

Surface-functionalized magnetic poly(styrene-glycidyl methacrylate) (PS-GMA) microspheres were prepared and coupled with Sca-1 antibody for cell selection from murine bone marrow mononuclear cells (MNCs). Biotinylated Sca-1 antibody could be directly coupled to avidin-bound magnetic microspheres. Alternatively, oxidized goat anti-mouse antibody was covalently bound onto the amino group-containing magnetic microspheres in a site-directed manner, and the resultant conjugate was coupled with non-modified Sca-1 antibody. Using the indirect antibody-bound magnetic microspheres, the purity of isolated Sca-1{sup +} cells increased with bead-to-cell ratio. Using a bead-to-cell ratio of 10 beads/cell, a purity of 85% Sca-1{sup +} cells corresponding to a 17-fold enrichment was achieved.

Accessory papillary muscles and papillary muscle hypertrophy are associated with sudden cardiac arrest of unknown cause.

Science.gov (United States)

Uhm, Jae-Sun; Youn, Jong-Chan; Lee, Hye-Jeong; Park, Junbeom; Park, Jin-Kyu; Shim, Chi Young; Hong, Geu-Ru; Joung, Boyoung; Pak, Hui-Nam; Lee, Moon-Hyoung

2015-10-15

The present study was performed for elucidating the associations between the morphology of the papillary muscles (PMs) and sudden cardiac arrest (SCA). We retrospectively reviewed history, laboratory data, electrocardiography, echocardiography, coronary angiography, and cardiac CT/MRI for 190 patients with SCA. The prevalence of accessory PMs and PM hypertrophy in patients with SCA of unknown cause was compared with that in patients with SCA of known causes and 98 age- and sex-matched patients without SCA. An accessory PM was defined as a PM with origins separated from the anterolateral and posteromedial PMs, or a PM that branched into two or three bellies at the base of the anterolateral or posteromedial PM. PM hypertrophy was defined as at least one of the two PMs having a diameter of ≥1.1cm. In 49 patients (age 49.9±15.9years; 38 men) the cause of SCA was unknown, whereas 141 (age 54.2±16.6years; 121 men) had a known cause. The prevalence of accessory PMs was significantly higher in the unknown-cause group than in the known-cause group (24.5% and 7.8%, respectively; p=0.002) or the no-SCA group (7.1%, p=0.003). The same was true for PM hypertrophy (unknown-cause 12.2%, known-cause 2.1%, p=0.010; no SCA group 1.0%, p=0.006). By logistic regression, accessory PM and PM hypertrophy were independently associated with sudden cardiac arrest of unknown cause. An accessory PM and PM hypertrophy are associated with SCA of unknown cause. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

Evaluation of Fe and Zn/Cu ratio in serum of patients with sickle cell anemia by total reflection X-ray fluorescence using synchrotron radiation

International Nuclear Information System (INIS)

Canellas, Catarine G.L.; Leitao, Roberta G.; Lopes, Ricardo T.; Bellido, Alfredo Victor B.; Anjos, Marcelino J.

2011-01-01

Sickle cell anemia (SCA) is a blood disorder that affects hemoglobin, the protein found in red blood cells that help carry oxygen throughout the body. In this work we have analyzed serum samples from patients with SCA by using total reflection X-ray fluorescence using synchrotron radiation (SRTXRF). The SRTXRF measurements were performed at the X-ray fluorescence beamline at Brazilian National Synchrotron Light Laboratory (LNLS), in Campinas, Sao Paulo using a polychromatic beam. We have studied forty-three patients aged 18-50 years old, suffering from SCA and Sixty healthy volunteers aged 18-60 years old. It was possible to determine the concentrations of the following elements: P, S, Cl, K, Ca, Fe, Cu, Zn, Br and Rb. Student's t-test was applied in order to check whether the two populations (CG x SCA) had the same mean values. It was observed that elemental concentration of P, Cl, K, Fe, Cu, Zn and Br differed significantly (α = 0.05) between groups of healthy subjects and SCA. The concentrations of K, Fe and Cu in the serum samples of patients with SCA were larger 15%, 120 % and 20 %, respectively, when compared with the CG. On the other hand, the concentrations of P (-20 %), Cl (-6 %), Zn (-25 %) and Br (-22 %) were smaller than the values determined for the control group. The serum level Cu/Zn ratio was significantly higher (60%) in the serum samples of patients with SCA group than the CG. So, the Cu/Zn ratio can be used as an adjuvant index in enhancement for diagnosis of SCA. There are evidences of an association among Fe, Cu, Zn and Cu/Zn in the SCA pathogenesis process. (author)

Evaluation of Fe and Zn/Cu ratio in serum of patients with sickle cell anemia by total reflection X-ray fluorescence using synchrotron radiation

Energy Technology Data Exchange (ETDEWEB)

Canellas, Catarine G.L.; Leitao, Roberta G; Lopes, Ricardo T., E-mail: catarine@lin.ufrj.b, E-mail: ricardo@lin.ufrj.b [Universidade Federal do Rio de Janeiro (PEN/COPPE/UFRJ), RJ (Brazil). Coordenacao dos Programas de Pos-Graduacao de Engenharia. Programa de Engenharia Nuclear. Lab. de Instrumentaco Nuclear; Carvalho, Silvia M.F., E-mail: silvia@hemorio.rj.gov.b [State Institute of Hematology Arthur de Siqueira Cavalcanti (HEMORIO), Rio de Janeiro, RJ (Brazil); Bellido, Alfredo Victor B., E-mail: alfredo@ien.gov.b [Federal Fluminense University (UFF), Niteroi, RJ (Brazil). Chemistry Inst.; Anjos, Marcelino J., E-mail: marcelin@lin.ufrj.b [State University of Rio de Janeiro (UERJ), RJ (Brazil). Physics Inst.

2011-07-01

Sickle cell anemia (SCA) is a blood disorder that affects hemoglobin, the protein found in red blood cells that help carry oxygen throughout the body. In this work we have analyzed serum samples from patients with SCA by using total reflection X-ray fluorescence using synchrotron radiation (SRTXRF). The SRTXRF measurements were performed at the X-ray fluorescence beamline at Brazilian National Synchrotron Light Laboratory (LNLS), in Campinas, Sao Paulo using a polychromatic beam. We have studied forty-three patients aged 18-50 years old, suffering from SCA and Sixty healthy volunteers aged 18-60 years old. It was possible to determine the concentrations of the following elements: P, S, Cl, K, Ca, Fe, Cu, Zn, Br and Rb. Student's t-test was applied in order to check whether the two populations (CG x SCA) had the same mean values. It was observed that elemental concentration of P, Cl, K, Fe, Cu, Zn and Br differed significantly ({alpha} = 0.05) between groups of healthy subjects and SCA. The concentrations of K, Fe and Cu in the serum samples of patients with SCA were larger 15%, 120 % and 20 %, respectively, when compared with the CG. On the other hand, the concentrations of P (-20 %), Cl (-6 %), Zn (-25 %) and Br (-22 %) were smaller than the values determined for the control group. The serum level Cu/Zn ratio was significantly higher (60%) in the serum samples of patients with SCA group than the CG. So, the Cu/Zn ratio can be used as an adjuvant index in enhancement for diagnosis of SCA. There are evidences of an association among Fe, Cu, Zn and Cu/Zn in the SCA pathogenesis process. (author)

The expression pattern of small nucleolar and small Cajal body-specific RNAs characterizes distinct molecular subtypes of multiple myeloma

International Nuclear Information System (INIS)

Ronchetti, D; Todoerti, K; Tuana, G; Agnelli, L; Mosca, L; Lionetti, M; Fabris, S; Colapietro, P; Miozzo, M; Ferrarini, M; Tassone, P; Neri, A

2012-01-01

Small nucleolar RNAs (snoRNAs) and small Cajal body-specific RNAs (scaRNAs) are non-coding RNAs involved in the maturation of other RNA molecules and generally located in the introns of host genes. It is now emerging that altered sno/scaRNAs expression may have a pathological role in cancer. This study elucidates the patterns of sno/scaRNAs expression in multiple myeloma (MM) by profiling purified malignant plasma cells from 55 MMs, 8 secondary plasma cell leukemias (sPCLs) and 4 normal controls. Overall, a global sno/scaRNAs downregulation was found in MMs and, even more, in sPCLs compared with normal plasma cells. Whereas SCARNA22 resulted the only sno/scaRNA characterizing the translocation/cyclin D4 (TC4) MM, TC2 group displayed a distinct sno/scaRNA signature overexpressing members of SNORD115 and SNORD116 families located in a region finely regulated by an imprinting center at 15q11, which, however, resulted overall hypomethylated in MMs independently of the SNORD115 and SNORD116 expression levels. Finally, integrative analyses with available gene expression and genome-wide data revealed the occurrence of significant sno/scaRNAs/host genes co-expression and the putative influence of allelic imbalances on specific snoRNAs expression. Our data extend the current view of sno/scaRNAs deregulation in cancer and add novel information to the bio-molecular complexity of plasma cell dyscrasias

Spinocerebellar ataxia type 6 in eastern India: Some new observations.

Science.gov (United States)

Bhattacharyya, Kalyan B; Pulai, Debabrata; Guin, Deb Shankar; Ganguly, Goutam; Joardar, Anindita; Roy, Sarnava; Rai, Saurabh; Biswas, Atanu; Pandit, Alok; Roy, Arijit; Senapati, Asit Kumar

2016-01-01

Spinocerebellar ataxias (SCAs) are hereditary, autosomal dominant progressive neurodegenerative disorders showing clinical and genetic heterogeneity. They are usually manifested clinically in the third to fifth decade of life although there is a wide variability in the age of onset. More than 36 different types of SCAs have been reported so far and about half of them are caused by pathological expansion of the trinucleotide, Cytosine Alanine Guanine (CAG) repeat. The global prevalence of SCA is 0.3-2 per 100,000 population, SCA3 being the commonest variety worldwide, accounting for 20-50 per cent of all cases, though SCA 2 is generally considered as the commonest one in India. However, SCA6 has not been addressed adequately from India though it is common in the eastern Asian countries like, Japan, Korea and Thailand. The present study was undertaken to identify the prevalence of SCA6 in the city of Kolkata and the eastern part of India. 83 consecutive patients were recruited for the study of possible SCAs and their clinical features and genotype were investigated. 6 of the 83 subjects turned out positive for SCA6, constituting therefore, 13.33% of the patient pool. SCA6 is prevalent in the eastern part of India, though not as frequent as the other common varieties. Further community based studies are required in order to understand the magnitude of SCA6 in the eastern part, as well as in other regions of India.

[123I]-IMP SPECT findings in spinocerebellar ataxia type 6

International Nuclear Information System (INIS)

Ito, Yasuhiko; Nakabayashi, Haruo; Iguchi, Yasuyuki; Suzuki, Masahiko; Kobayashi, Masayuki; Nakajima, Takashi

2000-01-01

To study the dynamics of metabolic function in the cerebellar hemispheres, vermis and brain stem of patients with spinocerebellar ataxia type 6 (SCA6), we used single photon emission computed tomography (SPECT) with N-isopropyl-p-[ 123 I] iodoamphetamine (IMP) to measure regional cerebral blood flow (rCBF) in six Japanese patients with SCA6 and nine normal control subjects. All patients with SCA6 were found to have expanded CAG repeats (from 22 to 24 repeats). The SPECT data were also analyzed semiquantitatively. The rCBF in the cerebellar hemisphere, vermis and brain stem was not significantly lower in patients with SCA6 than in normal controls. However, the ratio of the cerebellar hemisphere to occipital lobe (C/O ratio) was significantly lower in patients. The ratio of the vermis and brain stem to occipital lobe (V/O, P/O ratio) were not significantly lower in patients. The C/O, V/O and P/O ratio were especially sensitive indexes for regional cerebral function in patients with SCA6. Results of this study suggest that the functional decrease in SCA6 may begin in the cerebellar hemispheres. IMP SPECT was useful for evaluating rCBF in patients with SCA6. (author)

Increased risk of sudden cardiac arrest in obstructive pulmonary disease

DEFF Research Database (Denmark)

Warnier, Miriam Jacoba; Blom, Marieke Tabo; Bardai, Abdennasser

2013-01-01

BACKGROUND: We aimed to determine whether (1) patients with obstructive pulmonary disease (OPD) have an increased risk of sudden cardiac arrest (SCA) due to ventricular tachycardia or fibrillation (VT/VF), and (2) the SCA risk is mediated by cardiovascular risk-profile and/or respiratory drug use...... with electrocardiographic documentation of VT/VF were included. Conditional logistic regression analysis was used to assess the association between SCA and OPD. Pre-specified subgroup analyses were performed regarding age, sex, cardiovascular risk-profile, disease severity, and current use of respiratory drugs. RESULTS...... is associated with an increased observed risk of SCA. The most increased risk was observed in patients with a high cardiovascular risk-profile, and in those who received SABA and, possibly, those who received AC at the time of SCA....

Evaluation of Fe and Zn/Cu ratio in serum of patients with sickle cell anemia by total reflection X-ray fluorescence using synchrotron radiation

Energy Technology Data Exchange (ETDEWEB)

Canellas, Catarine G.L.; Leitao, Roberta G.; Lopes, Ricardo T., E-mail: catarine@lin.ufrj.b, E-mail: ricardo@lin.ufrj.b [Universidade Federal do Rio de Janeiro (PEN/COPPE/UFRJ), RJ (Brazil). Coordenacao dos Programas de Pos-Graduacao de Engenharia. Programa de Engenharia Nuclear. Lab. de Instrumentaco Nuclear; Carvalho, Silvia M.F., E-mail: silvia@hemorio.rj.gov.b [State Institute of Hematology Arthur de Siqueira Cavalcanti (HEMORIO), Rio de Janeiro, RJ (Brazil); Bellido, Alfredo Victor B., E-mail: alfredo@ien.gov.b [Federal Fluminense University (UFF), Niteroi, RJ (Brazil). Chemistry Inst.; Anjos, Marcelino J., E-mail: marcelin@lin.ufrj.b [State University of Rio de Janeiro (UERJ), RJ (Brazil). Physics Inst.

2011-07-01

Sickle cell anemia (SCA) is a blood disorder that affects hemoglobin, the protein found in red blood cells that help carry oxygen throughout the body. In this work we have analyzed serum samples from patients with SCA by using total reflection X-ray fluorescence using synchrotron radiation (SRTXRF). The SRTXRF measurements were performed at the X-ray fluorescence beamline at Brazilian National Synchrotron Light Laboratory (LNLS), in Campinas, Sao Paulo using a polychromatic beam. We have studied forty-three patients aged 18-50 years old, suffering from SCA and Sixty healthy volunteers aged 18-60 years old. It was possible to determine the concentrations of the following elements: P, S, Cl, K, Ca, Fe, Cu, Zn, Br and Rb. Student's t-test was applied in order to check whether the two populations (CG x SCA) had the same mean values. It was observed that elemental concentration of P, Cl, K, Fe, Cu, Zn and Br differed significantly ({alpha} = 0.05) between groups of healthy subjects and SCA. The concentrations of K, Fe and Cu in the serum samples of patients with SCA were larger 15%, 120 % and 20 %, respectively, when compared with the CG. On the other hand, the concentrations of P (-20 %), Cl (-6 %), Zn (-25 %) and Br (-22 %) were smaller than the values determined for the control group. The serum level Cu/Zn ratio was significantly higher (60%) in the serum samples of patients with SCA group than the CG. So, the Cu/Zn ratio can be used as an adjuvant index in enhancement for diagnosis of SCA. There are evidences of an association among Fe, Cu, Zn and Cu/Zn in the SCA pathogenesis process. (author)

Shaft-Condylar Angle for surgical correction in neglected and displaced lateral humeral condyle fracture in children.

Science.gov (United States)

Mulpruek, Pornchai; Angsanuntsukh, Chanika; Woratanarat, Patarawan; Sa-Ngasoongsong, Paphon; Tawonsawatruk, Tulyapruek; Chanplakorn, Pongsthorn

2015-09-01

To assess the outcome after using the Shaft-Condylar angle (SCA) as intraoperative reference for sagittal plane correction in displaced lateral humeral condyle fractures in children presented 3-weeks after injury. Ten children, with delayed presentation of a displaced lateral humeral condyle fracture and undergoing surgery during 1999-2011, were reviewed. The goal was to obtain a smooth articular surface with an intraoperative SCA of nearly 40° and nearest-anatomical carrying angle. They were allocated into two groups according to the postoperative SCA [Good-reduction group (SCA=30-50°), and Bad-reduction group (SCA50°)] and the final outcomes were then compared. All fractures united without avascular necrosis. The Good-reduction group (n=7) showed a significant improvement in final range of motion and functional outcome compared to the Bad-reduction group (n=3) (p=0.02). However, there was no significant difference in pain, carrying angle and overall outcome between both groups. SCA is a possible intraoperative reference for sagittal alignment correction in late presented displaced lateral humeral condyle fractures.

Far-infrared radiation protects viability in a cell model of Spinocerebellar Ataxia by preventing polyQ protein accumulation and improving mitochondrial function.

Science.gov (United States)

Chang, Jui-Chih; Wu, Shey-Lin; Hoel, Fredrik; Cheng, Yu-Shan; Liu, Ko-Hung; Hsieh, Mingli; Hoel, August; Tronstad, Karl Johan; Yan, Kuo-Chia; Hsieh, Ching-Liang; Lin, Wei-Yong; Kuo, Shou-Jen; Su, Shih-Li; Liu, Chin-San

2016-07-29

Far infrared radiation (FIR) is currently investigated as a potential therapeutic strategy in various diseases though the mechanism is unknown. Presently, we tested if FIR mediates beneficial effects in a cell model of the neurodegenerative disease spinocerebellar ataxia type 3 (SCA3). SCA3 is caused by a mutation leading to an abnormal polyglutamine expansion (PolyQ) in ataxin-3 protein. The consequent aggregation of mutant ataxin-3 results in disruption of vital cell functions. In this study, neuroblastoma cells (SK-N-SH) was transduced to express either non-pathogenic ataxin-3-26Q or pathogenic ataxin-3-78Q proteins. The cells expressing ataxin-3-78Q demonstrated decreased viability, and increased sensitivity to metabolic stress in the presence rotenone, an inhibitor of mitochondrial respiration. FIR exposure was found to protect against these effects. Moreover, FIR improved mitochondrial respiratory function, which was significantly compromised in ataxin-3-78Q and ataxin-3-26Q expressing cells. This was accompanied by decreased levels of mitochondrial fragmentation in FIR treated cells, as observed by fluorescence microscopy and protein expression analysis. Finally, the expression profile LC3-II, Beclin-1 and p62 suggested that FIR prevent the autophagy inhibiting effects observed in ataxin-3-78Q expressing cells. In summary, our results suggest that FIR have rescuing effects in cells expressing mutated pathogenic ataxin-3, through recovery of mitochondrial function and autophagy.

Volcanic evolution of central Basse-Terre Island revisited on the basis of new geochronology and geomorphology data

Science.gov (United States)

Ricci, J.; Quidelleur, X.; Lahitte, P.

2015-10-01

Twenty-six new and seven previous K-Ar ages obtained on groundmass separates for samples from the Axial Chain massif (Guadeloupe, F.W.I.), associated with geomorphological investigations, allow us to propose a new model of the volcanic evolution of the central part of Basse-Terre Island. The Axial Chain is composed of four edifices, Moustique, Matéliane, Capesterre, and Icaque mounts, showing coeval activity from 681 ± 12 to 509 ± 10 ka, which contradicts a previous hypothesis that flank collapse affected them successively. Our geomorphological reconstruction shows that the Axial Chain can be considered as a single large volcano, named the Southern Axial Chain volcano (SCA), rather than a succession of several smaller volcanoes. It raises questions regarding the formation of a large depression within the SCA volcano, prior to the construction of the Sans-Toucher volcano between 451 ± 13 and 412 ± 8 ka. Given presently available evidence, a slump affecting the western part of the SCA volcano is the most probable scenario to reconcile the complete age dataset and the present-day morphology of central Basse-Terre. Finally, our study shows that the SCA volcano had a post-activity volume of 90 km3, implying a construction rate of 0.5 km3/kyr. This value strongly constrains interpretations of magma generation processes throughout the Lesser Antilles arc.

Bistable Epigenetic States Explain Age-Dependent Decline in Mesenchymal Stem Cell Heterogeneity.

Science.gov (United States)

Hamidouche, Zahia; Rother, Karen; Przybilla, Jens; Krinner, Axel; Clay, Denis; Hopp, Lydia; Fabian, Claire; Stolzing, Alexandra; Binder, Hans; Charbord, Pierre; Galle, Joerg

2017-03-01

The molecular mechanisms by which heterogeneity, a major characteristic of stem cells, is achieved are yet unclear. We here study the expression of the membrane stem cell antigen-1 (Sca-1) in mouse bone marrow mesenchymal stem cell (MSC) clones. We show that subpopulations with varying Sca-1 expression profiles regenerate the Sca-1 profile of the mother population within a few days. However, after extensive replication in vitro, the expression profiles shift to lower values and the regeneration time increases. Study of the promoter of Ly6a unravels that the expression level of Sca-1 is related to the promoter occupancy by the activating histone mark H3K4me3. We demonstrate that these findings can be consistently explained by a computational model that considers positive feedback between promoter H3K4me3 modification and gene transcription. This feedback implicates bistable epigenetic states which the cells occupy with an age-dependent frequency due to persistent histone (de-)modification. Our results provide evidence that MSC heterogeneity, and presumably that of other stem cells, is associated with bistable epigenetic states and suggest that MSCs are subject to permanent state fluctuations. Stem Cells 2017;35:694-704. © The Authors Stem Cells published by Wiley Periodicals, Inc. on behalf of AlphaMed Press.

Spinocerebellar ataxia type 6 in eastern India: Some new observations

Directory of Open Access Journals (Sweden)

Kalyan B Bhattacharyya

2016-01-01

Full Text Available Introduction: Spinocerebellar ataxias (SCAs are hereditary, autosomal dominant progressive neurodegenerative disorders showing clinical and genetic heterogeneity. They are usually manifested clinically in the third to fifth decade of life although there is a wide variability in the age of onset. More than 36 different types of SCAs have been reported so far and about half of them are caused by pathological expansion of the trinucleotide, Cytosine Alanine Guanine (CAG repeat. The global prevalence of SCA is 0.3-2 per 100,000 population, SCA3 being the commonest variety worldwide, accounting for 20-50 per cent of all cases, though SCA 2 is generally considered as the commonest one in India. However, SCA6 has not been addressed adequately from India though it is common in the eastern Asian countries like, Japan, Korea and Thailand. Objective: The present study was undertaken to identify the prevalence of SCA6 in the city of Kolkata and the eastern part of India. Materials and Methods: 83 consecutive patients were recruited for the study of possible SCAs and their clinical features and genotype were investigated. Results: 6 of the 83 subjects turned out positive for SCA6, constituting therefore, 13.33% of the patient pool. Discussion: SCA6 is prevalent in the eastern part of India, though not as frequent as the other common varieties. Conclusions: Further community based studies are required in order to understand the magnitude of SCA6 in the eastern part, as well as in other regions of India.

Increased circulating fibrocytes are associated with higher reticulocyte percent in children with sickle cell anemia.

Science.gov (United States)

Karafin, Matthew S; Dogra, Shibani; Rodeghier, Mark; Burdick, Marie; Mehrad, Borna; Rose, C Edward; Strieter, Robert M; DeBaun, Michael R; Strunk, Robert C; Field, Joshua J

2016-03-01

Interstitial lung disease is common in patients with sickle cell anemia (SCA). Fibrocytes are circulating cells implicated in the pathogenesis of pulmonary fibrosis and airway remodeling in asthma. In this study, we tested the hypotheses that fibrocyte levels are: (1) increased in children with SCA compared to healthy controls, and (2) associated with pulmonary disease. Cross-sectional cohort study of children with SCA who participated in the Sleep Asthma Cohort Study. Fibrocyte levels were obtained from 45 children with SCA and 24 controls. Mean age of SCA cases was 14 years and 53% were female. In children with SCA, levels of circulating fibrocytes were greater than controls (P < 0.01). The fibrocytes expressed a hierarchy of chemokine receptors, with CXCR4 expressed on the majority of cells and CCR2 and CCR7 expressed on a smaller subset. Almost half of fibrocytes demonstrated α-smooth muscle actin activation. Increased fibrocyte levels were associated with a higher reticulocyte count (P = 0.03) and older age (P = 0.048) in children with SCA. However, children with increased levels of fibrocytes were not more likely to have asthma or lower percent predicted forced expiratory volume in 1 sec/forced vital capacity (FEV1 /FVC) or FEV1 than those with lower fibrocyte levels. Higher levels of fibrocytes in children with SCA compared to controls may be due to hemolysis. Longitudinal studies may be able to better assess the relationship between fibrocyte level and pulmonary dysfunction. © 2015 Wiley Periodicals, Inc.

Single coronary artery from right aortic sinus in a very elderly patient

Directory of Open Access Journals (Sweden)

Prashanth Panduranga

2016-10-01

Full Text Available In the absence of other associated cardiac anomalies, single coronary artery (SCA per se is a rare anomaly detected during coronary angiography or autopsy. Various types of SCA detected during coronary angiography have already been described. We herein report a type of SCA originating from the right sinus of Valsalva, with the right circumflex, left circumflex, and left anterior descending coronary arteries arising from the proximal part of the SCA in a 76-year-old female patient. She developed ventricular fibrillation during coronary angiography, which calls for caution while performing a coronary angiogram in such patients.

Model for predicting fabric filter and ESP costs

International Nuclear Information System (INIS)

Hoskins, W.; Terrill, J.K.

1992-01-01

United Engineers and Constructors (UE and C) has developed a personal computer (PC) based program (Model) for estimating capital and operating costs of fabric filters (FFs) and electrostatic precipitators (ESPs). The program contains proprietary sizing routines for both types of particulate control devices. For the FF, it determines the number of compartments, number of bags, physical dimensions and other important information. For the ESP, it determines specific collection area (SCA), number of cells, and number of TR sets. This paper reports that the program has the capability of handling a wide range of gas flows. It adjusts construction costs for the labor productivity factors in different locations. The capital costs are considered conceptual in nature with an absolute accuracy range of ±25%. The capital and operating costs are used along with economic factors to determine present worth costs. This allows site specific side-by-side comparisons of FFs and ESPs

Muscle damage induced by stretch-shortening cycle exercise.

Science.gov (United States)

Kyröläinen, H; Takala, T E; Komi, P V

1998-03-01

Strenuous stretch-shortening cycle exercise was used as a model to study the leakage of proteins from skeletal muscle. The analysis included serum levels of creatine kinase (S-CK), myoglobin (S-Mb), and carbonic anhydrase (S-CA III). Blood samples from power- (N=11) and endurance-trained (N=10) athletes were collected before, 0, and 2 h after the exercise, which consisted of a total of 400 jumps. The levels of all determined myocellular proteins increased immediately after the exercise (P exercise, and the ratio of S-CA III and S-Mb decreased (P recruitment order of motor units, and/or differences in training background.

Effet de l'amendement au carbonate de calcium (mikhart) de ...

African Journals Online (AJOL)

SARAH

31 janv. 2017 ... control substratum, SCa10 (S0 + 10 % powder of calcium carbonate), SCa20 (S0 + 20 % powder of calcium carbonate), SCa30 (S0 ..... puis montés sur un microscope électronique FEG Supra. 40 VP Zeiss à balayage et pression variable. Dès lors, ..... substrats amendés d'un apport en quantité et en qualité.

Caffeine alleviates progressive motor deficits in a transgenic mouse model of spinocerebellar ataxia.

Science.gov (United States)

Gonçalves, Nélio; Simões, Ana T; Prediger, Rui D; Hirai, Hirokazu; Cunha, Rodrigo A; Pereira de Almeida, Luís

2017-03-01

Machado-Joseph disease (MJD) is a neurodegenerative spinocerebellar ataxia (SCA) associated with an expanded polyglutamine tract within ataxin-3 for which there is currently no available therapy. We previously showed that caffeine, a nonselective adenosine receptor antagonist, delays the appearance of striatal damage resulting from expression of full-length mutant ataxin-3. Here we investigated the ability of caffeine to alleviate behavioral deficits and cerebellar neuropathology in transgenic mice with a severe ataxia resulting from expression of a truncated fragment of polyglutamine-expanded ataxin-3 in Purkinje cells. Control and transgenic c57Bl6 mice expressing in the mouse cerebella a truncated form of human ataxin-3 with 69 glutamine repeats were allowed to freely drink water or caffeinated water (1g/L). Treatments began at 7 weeks of age, when motor and ataxic phenotype emerges in MJD mice, and lasted up to 20 weeks. Mice were tested in a panel of locomotor behavioral paradigms, namely rotarod, beam balance and walking, pole, and water maze cued-platform version tests, and then sacrificed for cerebellar histology. Caffeine consumption attenuated the progressive loss of general and fine-tuned motor function, balance, and grip strength, in parallel with preservation of cerebellar morphology through decreasing the loss of Purkinje neurons and the thinning of the molecular layer in different folia. Caffeine also rescued the putative striatal-dependent executive and cognitive deficiencies in MJD mice. Our findings provide the first in vivo demonstration that caffeine intake alleviates behavioral disabilities in a severely impaired animal model of SCA. Ann Neurol 2017;81:407-418. © 2016 American Neurological Association.

Vascular Risk Factors and Clinical Progression in Spinocerebellar Ataxias

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Raymond Y. Lo

2015-02-01

Full Text Available Background: The contributions of vascular risk factors to spinocerebellar ataxia (SCA are not known.Methods: We studied 319 participants with SCA 1, 2, 3, and 6 and repeatedly measured clinical severity using the Scale for Assessment and Rating of Ataxia (SARA for 2 years. Vascular risk factors were summarized by CHA2DS2-VASc scores as the vascular risk factor index. We employed regression models to study the effects of vascular risk factors on ataxia onset and progression after adjusting for age, sex, and pathological CAG repeats. Our secondary analyses took hyperlipidemia into account.Results: Nearly 60% of SCA participants were at low vascular risks with CHA2DS2-VASc = 0, and 31% scored 2 or greater. Higher CHA2DS2-VASc scores were not associated with either earlier onset or faster progression of ataxia. These findings were not altered after accounting for hyperlipidemia. Discussion: Vascular risks are not common in SCAs and are not associated with earlier onset or faster ataxia progression.

Deriving Snow-Cover Depletion Curves for Different Spatial Scales from Remote Sensing and Snow Telemetry Data

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Fassnacht, Steven R.; Sexstone, Graham A.; Kashipazha, Amir H.; Lopez-Moreno, Juan Ignacio; Jasinski, Michael F.; Kampf, Stephanie K.; Von Thaden, Benjamin C.

2015-01-01

During the melting of a snowpack, snow water equivalent (SWE) can be correlated to snow-covered area (SCA) once snow-free areas appear, which is when SCA begins to decrease below 100%. This amount of SWE is called the threshold SWE. Daily SWE data from snow telemetry stations were related to SCA derived from moderate-resolution imaging spectro radiometer images to produce snow-cover depletion curves. The snow depletion curves were created for an 80,000 sq km domain across southern Wyoming and northern Colorado encompassing 54 snow telemetry stations. Eight yearly snow depletion curves were compared, and it is shown that the slope of each is a function of the amount of snow received. Snow-cover depletion curves were also derived for all the individual stations, for which the threshold SWE could be estimated from peak SWE and the topography around each station. A stations peak SWE was much more important than the main topographic variables that included location, elevation, slope, and modelled clear sky solar radiation. The threshold SWE mostly illustrated inter-annual consistency.

Treatment Patterns and Antipsychotic Medication Adherence Among Commercially Insured Patients With Schizoaffective Disorder in the United States

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Joshi, Kruti; Lin, Jay; Lingohr-Smith, Melissa; Fu, Dong-Jing; Muser, Erik

2016-01-01

Abstract This study assessed real-world treatment patterns and antipsychotic (AP) medication adherence among commercially insured US patients with schizoaffective disorder (SCA). Continuously insured adults aged 18 years or older with a diagnosis of SCA from January 1, 2009, to December 31, 2012, were identified from the Clinformatics Data Mart database. Patients were categorized into 2 cohorts: incident or prevalent SCA. Demographics and clinical characteristics were evaluated during the baseline period. Use of psychiatric medications and adherence to AP medications were evaluated during a 12-month follow-up period after index diagnosis of SCA. Of the overall study population (N = 2713; mean age, 40.2 y; 52.7% female), 1961 patients (72.3%) (mean age, 38.7 y; 51.3% female) had incident SCA, and 752 patients (27.7%) (mean age, 43.9 y; 56.5% female) had prevalent SCA. Antipsychotics were used by 74.8% of patients in the overall study population during the follow-up period. The most commonly prescribed oral AP was risperidone (23.9%), followed by quetiapine (21.4%) and aripiprazole (20.4%). Use of any long-acting injectable APs in the overall study population during the follow-up period was less than 3%. A total of 49.0% and 38.0% of the overall study population had medication possession ratios and proportion of days covered for APs of 80% or greater, respectively. Overall use of long-acting injectable APs for the treatment of SCA is low, and adherence to AP medications, measured by both medication possession ratio and proportion of days covered, is suboptimal among patients with SCA in the real-world setting. PMID:27525965

Novel polyglutamine model uncouples proteotoxicity from aging.

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Christie, Nakeirah T M; Lee, Amy L; Fay, Hannah G; Gray, Amelia A; Kikis, Elise A

2014-01-01

Polyglutamine expansions in certain proteins are the genetic determinants for nine distinct progressive neurodegenerative disorders and resultant age-related dementia. In these cases, neurodegeneration is due to the aggregation propensity and resultant toxic properties of the polyglutamine-containing proteins. We are interested in elucidating the underlying mechanisms of toxicity of the protein ataxin-3, in which a polyglutamine expansion is the genetic determinant for Machado-Joseph Disease (MJD), also referred to as spinocerebellar ataxia 3 (SCA3). To this end, we have developed a novel model for ataxin-3 protein aggregation, by expressing a disease-related polyglutamine-containing fragment of ataxin-3 in the genetically tractable body wall muscle cells of the model system C. elegans. Here, we demonstrate that this ataxin-3 fragment aggregates in a polyQ length-dependent manner in C. elegans muscle cells and that this aggregation is associated with cellular dysfunction. However, surprisingly, this aggregation and resultant toxicity was not influenced by aging. This is in contrast to polyglutamine peptides alone whose aggregation/toxicity is highly dependent on age. Thus, the data presented here not only describe a new polyglutamine model, but also suggest that protein context likely influences the cellular interactions of the polyglutamine-containing protein and thereby modulates its toxic properties.

Novel polyglutamine model uncouples proteotoxicity from aging.

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Nakeirah T M Christie

Full Text Available Polyglutamine expansions in certain proteins are the genetic determinants for nine distinct progressive neurodegenerative disorders and resultant age-related dementia. In these cases, neurodegeneration is due to the aggregation propensity and resultant toxic properties of the polyglutamine-containing proteins. We are interested in elucidating the underlying mechanisms of toxicity of the protein ataxin-3, in which a polyglutamine expansion is the genetic determinant for Machado-Joseph Disease (MJD, also referred to as spinocerebellar ataxia 3 (SCA3. To this end, we have developed a novel model for ataxin-3 protein aggregation, by expressing a disease-related polyglutamine-containing fragment of ataxin-3 in the genetically tractable body wall muscle cells of the model system C. elegans. Here, we demonstrate that this ataxin-3 fragment aggregates in a polyQ length-dependent manner in C. elegans muscle cells and that this aggregation is associated with cellular dysfunction. However, surprisingly, this aggregation and resultant toxicity was not influenced by aging. This is in contrast to polyglutamine peptides alone whose aggregation/toxicity is highly dependent on age. Thus, the data presented here not only describe a new polyglutamine model, but also suggest that protein context likely influences the cellular interactions of the polyglutamine-containing protein and thereby modulates its toxic properties.

The influence of socioeconomic status on the hemoglobin level and anthropometry of sickle cell anemia patients in steady state at the Lagos University Teaching Hospital.

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Animasahun, B A; Temiye, E O; Ogunkunle, O O; Izuora, A N; Njokanma, O F

2011-01-01

Sickle cell anemia (SCA) has multisystemic manifestations and is associated with severe morbidity and high mortality. It commonly affects growth leading to wasting and stunting. This study aimed to determine the influence of socioeconomic status on the nutritional status using anthropometric measurements and steady-state hemoglobin, of children with homozygous SCA, aged 1 year to 10 years in steady state at the Lagos University Teaching Hospital. This is a cross-sectional study involving 100 children with SCA and 100 age-, sex-, and social class-matched controls that fulfilled the inclusion criteria. Social class was assessed using educational attainment and occupation of parents. Hemoglobin concentration was determined using the oxy-hemoglobin method. This study demonstrated a significantly lower mean weight and weight-for-height in the SCA patients than those of controls (P hemoglobin concentration were observed from social class 1 to 4; this was statistically significant in controls (P = 0.00) but not in subjects (P > 0.1). However, SCA patients had significantly lower values than controls in each of the social classes. Poor socioeconomic status has an adverse effect on the nutritional status and hemoglobin of SCA patients.

Partial Body Weight-Supported Treadmill Training in Spinocerebellar Ataxia.

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de Oliveira, Laura Alice Santos; Martins, Camilla Polonini; Horsczaruk, Carlos Henrique Ramos; da Silva, Débora Cristina Lima; Vasconcellos, Luiz Felipe; Lopes, Agnaldo José; Meira Mainenti, Míriam Raquel; Rodrigues, Erika de Carvalho

2018-01-01

The motor impairments related to gait and balance have a huge impact on the life of individuals with spinocerebellar ataxia (SCA). Here, the aim was to assess the possibility of retraining gait, improving cardiopulmonary capacity, and challenging balance during gait in SCA using a partial body weight support (BWS) and a treadmill. Also, the effects of this training over functionality and quality of life were investigated. Eight SCA patients were engaged in the first stage of the study that focused on gait training and cardiovascular conditioning. From those, five took part in a second stage of the study centered on dynamic balance training during gait. The first and second stages lasted 8 and 10 weeks, respectively, both comprising sessions of 50 min (2 times per week). The results showed that gait training using partial BWS significantly increased gait performance, treadmill inclination, duration of exercise, and cardiopulmonary capacity in individuals with SCA. After the second stage, balance improvements were also found. Combining gait training and challenging tasks to the postural control system in SCA individuals is viable, well tolerated by patients with SCA, and resulted in changes in capacity for walking and balance.

Pregnancy Loss and Carotid Intima-Media Thickness in Mexican Women.

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Hartasanchez, Sandra A; Flores-Torres, Mario; Monge, Adriana; Yunes, Elsa; Rodriguez, Beatriz; Cantu-Brito, Carlos; Colaci, Daniela; Lamadrid-Figueroa, Hector; Lopez-Ridaura, Ruy; Lajous, Martin

2018-01-13

Cardiovascular disease in women often develops without conventional risk factors. Prenatal loss is a common pregnancy outcome that may result in physiological changes can increase the potential future risk of cardiovascular disease. Insufficient information exists regarding the impact of pregnancy loss on early markers of cardiovascular disease risk. Cross-sectional analysis of 1767 disease-free women from the MTC (Mexican Teachers' Cohort) who had been pregnant was used to evaluate the relationship between pregnancy loss and carotid intima-media thickness (IMT). Participants responded to a questionnaire regarding their reproductive history, risk factors, and medical conditions. We defined pregnancy loss as history of miscarriage and/or stillbirth. Trained neurologists measured IMT using ultrasound. We log-transformed IMT and defined subclinical carotid atherosclerosis (SCA) as IMT ≥0.8 mm and/or plaque. We used multivariable linear and logistic regression models to assess the relation of pregnancy loss, IMT, and SCA. The mean age of participants was 49.8±5.1 years. The prevalence of pregnancy loss was 22%, and we observed SCA in 23% of participants. Comparing participants who reported a pregnancy loss and those who did not, the multivariable-adjusted odds ratio for SCA was 1.52 (95% confidence interval, 1.12-2.06). Women who experienced a stillbirth had 2.32 higher odds (95% confidence interval, 1.03-5.21) of SCA than those who did not. Mean IMT appeared to be higher in women who reported a pregnancy loss relative to those who did not; nevertheless, this was not statistically significant. Pregnancy loss could be linked to cardiovascular disease later in life. The key findings of our study await confirmation and further investigation of the potential underlying mechanisms for this association is required. © 2018 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley.

Results of Study of Sulfur Oxide Reduction During Combustion of Coal-Water Slurry Fuel Through use of Sulfur Capturing Agents

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Murko Vasiliy I.

2016-01-01

Full Text Available It is shown that an effective way of burning high sulfur coal is to burn coal-water slurry fuel (CWF prepared on its basis containing a sulfur capture agent (SCA entered in the slurry at the stage of preparation. The technique of thermodynamic analysis of chemical reactions during CWF burning has been developed including burning in the presence of SCA. Using the developed calculation program, the optimal temperature conditions have been determined as required for the effective reduction of sulfur oxides in flue gases when using different types of SCA. According to the results of calculating the composition of CWF combustion products when entering various substances in the burner space as SCA it has been determined that magnesite, calcite, and dolomite are the most effective natural minerals. The analysis of calculated and experimental data proves the efficiency of SCA addition as well as validity of the obtained results.

Determinants of occurrence and survival after sudden cardiac arrest-A European perspective

DEFF Research Database (Denmark)

Empana, Jean-Philippe; Blom, Marieke T; Bӧttiger, Bernd W

2018-01-01

AIMS: The ESCAPE-NET project ("European Sudden Cardiac Arrest network- towards Prevention, Education and New Effective Treatments") aims to study: (1) risk factors and mechanisms for the occurrence of sudden cardiac arrest (SCA) in the population, and (2) risk factors and treatment strategies...... for survival after SCA on a European scale. METHODS: This is an Horizon2020 funded program of the European Union, performed by a European public-private consortium of 16 partners across 10 EU countries. There are 11 deep-phenotyped SCA cohorts for the study of risk factors and treatment strategies for survival...... ongoing efforts on SCA outside Europe and within Europe including the EuReCa project....

Parent reported sleep problems in preschool children with sickle cell anemia and controls in East London.

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Downes, Michelle; de Haan, Michelle; Kirkham, Fenella J; Telfer, Paul T

2017-06-01

Snoring and poor sleep may affect cognition, particularly in young children with chronic conditions. Parents of London preschoolers with sickle cell anemia (SCA; n = 22), matched controls (n = 24), and unselected typically developing (n = 142) preschoolers completed sleep questionnaires. Preschoolers with SCA had significantly more sleep problems when compared to matched controls and the larger population. Snoring occurred at least one to two nights a week for 79% of the SCA group. This is compared with 25% of matched controls and 33% of larger population. Randomized controlled trials to improve sleep in young children with SCA already at-risk for cognitive dysfunction should be considered. © 2016 Wiley Periodicals, Inc.

On the description of electronic final states in the K-shell ionization by protons

International Nuclear Information System (INIS)

Aashamar, O.; Kocbach, L.

1976-06-01

The choice of free electronic wave functions in the description of K-shell ionization by protons is discussed. The previously known discrepancies between PWBA and SCA results are shown to be entirely due to two different choices of electronic wave functions. Calculations in the SCA framework with Hartree-Fock-Slater wave functions are reported. Some general features of the SCA calculations are discussed. (Auth.)

Silent Corticotroph Adenomas After Stereotactic Radiosurgery: A Case–Control Study

International Nuclear Information System (INIS)

Xu, Zhiyuan; Ellis, Scott; Lee, Cheng-Chia; Starke, Robert M.; Schlesinger, David; Lee Vance, Mary; Lopes, M. Beatriz; Sheehan, Jason

2014-01-01

Purpose: To investigate the safety and effectiveness of stereotactic radiosurgery (SRS) in patients with a silent corticotroph adenoma (SCA) compared with patients with other subtypes of non–adrenocorticotropic hormone staining nonfunctioning pituitary adenoma (NFA). Methods and Materials: The clinical features and outcomes of 104 NFA patients treated with SRS in our center between September 1994 and August 2012 were evaluated. Among them, 34 consecutive patients with a confirmatory SCA were identified. A control group of 70 patients with other subtypes of NFA were selected for review based on comparable baseline features, including sex, age at the time of SRS, tumor size, margin radiation dose to the tumor, and duration of follow-up. Results: The median follow-up after SRS was 56 months (range, 6-200 months). No patients with an SCA developed Cushing disease during the follow-up. Tumor control was achieved in 21 of 34 patients (62%) in the SCA group, compared with 65 of 70 patients (93%) in the NFA group. The median progression-free survival (PFS) was 58 months in the SCA group. The actuarial PFS was 73%, 46%, and 31% in the SCA group and was 94%, 87%, and 87% in the NFA group at 3, 5, and 8 years, respectively. Silent corticotroph adenomas treated with a dose of ≥17 Gy exhibited improved PFS. New-onset loss of pituitary function developed in 10 patients (29%) in the SCA group, whereas it occurred in 18 patients (26%) in the NFA group. Eight patients (24%) in the SCA group experienced worsening of a visual field deficit or visual acuity attributed to the tumor progression, as did 6 patients (9%) in the NFA group. Conclusion: Silent corticotroph adenomas exhibited a more aggressive course with a higher progression rate than other subtypes of NFAs. Stereotactic radiosurgery is an important adjuvant treatment for control of tumor growth. Increased radiation dose may lead to improved tumor control in SCA patients

Silent Corticotroph Adenomas After Stereotactic Radiosurgery: A Case–Control Study

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Xu, Zhiyuan; Ellis, Scott; Lee, Cheng-Chia; Starke, Robert M. [Department of Neurological Surgery, University of Virginia, Charlottesville, Virginia (United States); Schlesinger, David [Department of Neurological Surgery, University of Virginia, Charlottesville, Virginia (United States); Department of Radiation Oncology, University of Virginia, Charlottesville, Virginia (United States); Lee Vance, Mary [Department of Neurological Surgery, University of Virginia, Charlottesville, Virginia (United States); Department of Internal Medicine, University of Virginia, Charlottesville, Virginia (United States); Lopes, M. Beatriz [Division of Neuropathology, University of Virginia, Charlottesville, Virginia (United States); Sheehan, Jason, E-mail: jsheehan@virginia.edu [Department of Neurological Surgery, University of Virginia, Charlottesville, Virginia (United States); Department of Radiation Oncology, University of Virginia, Charlottesville, Virginia (United States)

2014-11-15

Purpose: To investigate the safety and effectiveness of stereotactic radiosurgery (SRS) in patients with a silent corticotroph adenoma (SCA) compared with patients with other subtypes of non–adrenocorticotropic hormone staining nonfunctioning pituitary adenoma (NFA). Methods and Materials: The clinical features and outcomes of 104 NFA patients treated with SRS in our center between September 1994 and August 2012 were evaluated. Among them, 34 consecutive patients with a confirmatory SCA were identified. A control group of 70 patients with other subtypes of NFA were selected for review based on comparable baseline features, including sex, age at the time of SRS, tumor size, margin radiation dose to the tumor, and duration of follow-up. Results: The median follow-up after SRS was 56 months (range, 6-200 months). No patients with an SCA developed Cushing disease during the follow-up. Tumor control was achieved in 21 of 34 patients (62%) in the SCA group, compared with 65 of 70 patients (93%) in the NFA group. The median progression-free survival (PFS) was 58 months in the SCA group. The actuarial PFS was 73%, 46%, and 31% in the SCA group and was 94%, 87%, and 87% in the NFA group at 3, 5, and 8 years, respectively. Silent corticotroph adenomas treated with a dose of ≥17 Gy exhibited improved PFS. New-onset loss of pituitary function developed in 10 patients (29%) in the SCA group, whereas it occurred in 18 patients (26%) in the NFA group. Eight patients (24%) in the SCA group experienced worsening of a visual field deficit or visual acuity attributed to the tumor progression, as did 6 patients (9%) in the NFA group. Conclusion: Silent corticotroph adenomas exhibited a more aggressive course with a higher progression rate than other subtypes of NFAs. Stereotactic radiosurgery is an important adjuvant treatment for control of tumor growth. Increased radiation dose may lead to improved tumor control in SCA patients.

A critical appraisal of paliperidone long-acting injection in the treatment of schizoaffective disorder.

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Chue, Pierre; Chue, James

2016-01-01

Schizoaffective disorder (SCA) is a chronic and disabling mental illness that presents with mixed symptoms of schizophrenia and affective disorders. SCA is recognized as a discrete disorder, but with greater heterogeneity and symptom overlap, leading to difficulty and delay in diagnosis. Although the overall prognosis is intermediate between schizophrenia and mood disorders, SCA is associated with higher rates of suicide and hospitalization than schizophrenia. No treatment guidelines exist for SCA, and treatment is frequently complex, involving off-label use and polypharmacy (typically combinations of antipsychotics, mood stabilizers, and antidepressants). Oral paliperidone extended-release was the first agent to be approved for the treatment of SCA. As in schizophrenia and bipolar disorder, adherence to oral medications is poor, further contributing to suboptimal outcomes. The use of an antipsychotic in a long-acting injection (LAI) addresses adherence issues, thus potentially reducing relapse. Paliperidone palmitate represents the LAI formulation of paliperidone. In a long-term, double-blind, randomized, controlled trial of adult patients (n=334; intent-to-treat [ITT]) with SCA, paliperidone long-acting injection (PLAI) significantly delayed risk of relapse compared to placebo (hazard ratio 2.49, 95% confidence interval, 1.55-3.99; P<0.001). This study demonstrated the efficacy and safety of PLAI when used as either monotherapy or adjunctive therapy for the maintenance treatment of SCA. The results are consistent with a similarly designed study conducted in patients with schizophrenia, which suggests a benefit in the long-term control of not only psychotic but also affective symptoms. No new safety signals were observed. When used in monotherapy, PLAI simplifies treatment by reducing complex pharmacotherapy and obviating the necessity for daily oral medications. PLAI is the second agent, and the first LAI, to be approved for the treatment of SCA; as an LAI

Subcallosal artery stroke: infarction of the fornix and the genu of the corpus callosum. The importance of the anterior communicating artery complex. Case series and review of the literature.

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Meila, Dan; Saliou, Guillaume; Krings, Timo

2015-01-01

Despite the variable anatomy of the anterior communicating artery (AcoA) complex, three main perforating branches can be typically identified the largest of which being the subcallosal artery (ScA). We present a case series of infarction in the vascular territory of the ScA to highlight the anatomy, the clinical symptomatology, and the presumed pathophysiology as it pertains to endovascular and surgical management of vascular pathology in this region. In this retrospective multicenter case series study of patients who were diagnosed with symptomatic ScA stroke, we analyzed all available clinical records, MRI, and angiographic details. Additionally, a review of the literature is provided. We identified five different cases of ScA stroke, leading to a subsequent infarction of the fornix and the genu of the corpus callosum. The presumed pathophysiology in non-iatrogenic cases is microangiopathy, rather than embolic events; iatrogenic SCA occlusion can present after both surgical and endovascular treatment of AcoA aneurysms that may occur with or without occlusion of the AcoA. Stroke in the vascular territory of the ScA leads to a characteristic imaging and clinical pattern. Ischemia involves the anterior columns of the fornix and the genu of the corpus callosum, and patients present with a Korsakoff's syndrome including disturbances of short-term memory and cognitive changes. We conclude that despite its small size, the ScA is an important artery to watch out for during surgical or endovascular treatment of AcoA aneurysms.

Interleukin-1β and interleukin-6 gene polymorphisms are associated with manifestations of sickle cell anemia.

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Vicari, Perla; Adegoke, Samuel A; Mazzotti, Diego Robles; Cançado, Rodolfo Delfini; Nogutti, Maria Aparecida Eiko; Figueiredo, Maria Stella

2015-03-01

Sickle cell anemia (SCA), a disorder characterized by both acute and chronic inflammation, exhibits substantial phenotypic variability. Interleukin-1 beta (IL-1β) and IL-6 are important in acute and chronic diseases, and their single nucleotide polymorphisms (SNPs) have been considered as predictors of prognosis in several inflammatory conditions. This study aims at exploring possible association of IL-1β and IL-6 SNPs as potential genetic modifiers and or predictors of SCA clinical and laboratory phenotypes. This cross-sectional study involved 107 SCA patients and 110 age, sex and ethnicity-matched healthy individuals. The SNPs were identified by PCR-RFLP for IL-1β (-511C>T and +3954C>T) and IL-6 (-597G>A and -174G>C) genes. Associations between these SNPs and the clinical and laboratory profiles of patients with SCA were then determined. Allelic and genotypic frequencies of IL-1β and IL-6 SNPs between patients with SCA and controls were similar and followed HWE. IL-1β +3954C>T SNP was associated with increased risk of osteonecrosis, elevated pulmonary arterial pressure and lower absolute reticulocyte count, while IL-6 -597G>A was associated with higher likelihood of retinopathy and leg ulcer. These data indicate that IL-1β and IL-6 gene SNPs are associated with SCA complications among Brazilian patients and may act as genetic predictors of SCA clinical heterogeneity. Copyright © 2014 Elsevier Inc. All rights reserved.

Diet of bottlenose dolphins (Tursiops truncatus from the Gulf of Cadiz: Insights from stomach content and stable isotope analyses.

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Joan Giménez

Full Text Available The ecological role of species can vary among populations depending on local and regional differences in diet. This is particularly true for top predators such as the bottlenose dolphin (Tursiops truncatus, which exhibits a highly varied diet throughout its distribution range. Local dietary assessments are therefore critical to fully understand the role of this species within marine ecosystems, as well as its interaction with important ecosystem services such as fisheries. Here, we combined stomach content analyses (SCA and stable isotope analyses (SIA to describe bottlenose dolphins diet in the Gulf of Cadiz (North Atlantic Ocean. Prey items identified using SCA included European conger (Conger conger and European hake (Merluccius merluccius as the most important ingested prey. However, mass-balance isotopic mixing model (MixSIAR, using δ13C and δ15N, indicated that the assimilated diet consisted mainly on Sparidae species (e.g. seabream, Diplodus annularis and D. bellottii, rubberlip grunt, Plectorhinchus mediterraneus, and common pandora, Pagellus erythrinus and a mixture of other species including European hake, mackerels (Scomber colias, S. japonicus and S. scombrus, European conger, red bandfish (Cepola macrophthalma and European pilchard (Sardina pilchardus. These contrasting results highlight differences in the temporal and taxonomic resolution of each approach, but also point to potential differences between ingested (SCA and assimilated (SIA diets. Both approaches provide different insights, e.g. determination of consumed fish biomass for the management of fish stocks (SCA or identification of important assimilated prey species to the consumer (SIA.

Invasive Pulmonary Aspergillosis in a Sickle Cell Patient Transplant Recipient: A Successful Treatment

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Katia Paciaroni

2015-08-01

Full Text Available Sickle Cell Anaemia (SCA is the most common inherited blood disorder and is associated with severe morbidity and decreased survival. Allogeneic Haematopoietic Stem Cell Transplantation (HSCT is the only curative approach. Nevertheless the decision to perform a marrow transplant includes the risk of major complications  and mortality transplant related. The infections represent the main cause of mortality for SCA patients undergoing transplant. Invasive Pulmonary Aspergillosis (IPA is a devastating opportunistic infection and remains a significant cause of morbidity and mortality in HSCT recipients. Data regarding IPA in the setting of SCA are lacking. In the present report,  we describe a patient with SCA who developed IPA after allogeneic bone marrow transplant. The fungal infection was treated by systemic antifungal therapy in addition to the surgery, despite  mild chronic GVHD and with continuing immunosuppression therapy. This case shows that IPA occurring in bone marrow recipient with SCA can be successful treated

Accuracy and cost-analysis of placental alpha-microglobulin-1 test in the diagnosis of premature rupture of fetal membranes in resource-limited community settings.

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Eleje, George Uchenna; Ezugwu, Euzebus Chinonye; Ogunyemi, Dotun; Eleje, Lydia Ijeoma; Ikechebelu, Joseph Ifeanyichukwu; Igwegbe, Anthony Osita; Okonkwo, John E; Ikpeze, Okechukwu Christian; Udigwe, Gerald Okanandu; Onah, Hyacinth Eze; Nwosu, Betrand Obi; Ezeama, Chukwuemeka Okwudili; Ezenkwele, Eziamaka Pauline

2015-01-01

To determine accuracy and costs of placental α-microglobulin-1 (PAMG-1) test compared to standard clinical assessment (SCA) for diagnosing rupture of membranes (ROM). A multicenter double-blind study of consecutive women with symptoms and signs of ROM in Nnamdi Azikiwe University Teaching Hospital, Nnewi and University of Nigeria Teaching Hospital, Enugu, both in south-east Nigeria using SCA for ROM and the PAMG-1 test was done. ROM was diagnosed if two out of three methods from SCA (pooling, positive nitrazine test or ferning) were present and confirmed post-delivery based on presence of any two of these clinical criteria: delivery in 48 h to 7 days, evidence of chorioamnionitis, membranes overtly ruptured at delivery and adverse perinatal outcomes strongly correlated with prolonged PROM. A cost-analysis was also done. The outcome measures included sensitivity, specificity, accuracy and costs for the two tests. Accuracy, sensitivity and specificity for the PAMG-1 test were 97.2%, 97.4% and 96.7%, higher than for SCA which were 83.7%, 87.9% and 70.5%, respectively (P < 0.001). Accuracy of SCA was higher at less than 34 weeks than 34 weeks or more (88.3% vs 81.4%) while the PAMG-1 test performed equally at both gestational age categories (96.1% vs 97.7%). In women without pooling, accuracy of the PAMG-1 test was 96.7%, while it was 40.0% with SCA. Analysis showed that the overall cost of SCA was 45% higher than the PAMG-1 test. This study confirms that the PAMG-1 test has a consistently high diagnostic accuracy at all gestational ages and with equivocal cases of ROM. The PAMG-1 test appears less costly than SCA. © 2014 The Authors. Journal of Obstetrics and Gynaecology Research © 2014 Japan Society of Obstetrics and Gynecology.

Effect of curd washing on the properties of reduced-calcium and standard-calcium Cheddar cheese.

Science.gov (United States)

Hou, Jia; McSweeney, Paul L H; Beresford, Thomas P; Guinee, Timothy P

2014-10-01

Washed (W) and nonwashed (NW) variants of standard (SCa) and reduced-calcium (RCa) Cheddar cheeses were made in triplicate, ripened for a 270-d period, and analyzed for composition and changes during maturation. Curd washing was applied to cheeses to give a target level of lactose plus lactic acid in cheese moisture of 3.9 g/100 g in the W cheese, compared with a value of 5.3 g/100 g of lactose plus lactic acid in cheese moisture in the control NW cheeses. The 4 cheese types were denoted standard calcium nonwashed (SCaNW), standard calcium washed (SCaW), reduced-calcium nonwashed (RCaNW), and reduced-calcium washed (RCaW). The mean calcium level was 760 mg/100 g in the SCaNW and SCaW and 660 mg/100 g in the RCaNW and RCaW cheeses. Otherwise the gross composition of all cheeses was similar, each with protein, fat, and moisture levels of ~26, 32, and 36 g/100 g, respectively. Curd washing significantly reduced the mean level of lactic acid in the SCaW cheese and residual lactose in both SCaW and RCaW cheeses. The mean pH of the standard-calcium cheese over the 270-d ripening period increased significantly with curd washing and ripening time, in contrast to the reduced-calcium cheese, which was not affected by the latter parameters. Otherwise curd washing had little effect on changes in populations of starter bacteria or nonstarter lactic acid bacteria, proteolysis, rheology, or color of the cheese during ripening. Descriptive sensory analysis at 270 d indicated that the SCaW cheese had a nuttier, sweeter, less fruity, and less rancid taste than the corresponding SCaNW cheese. In contrast, curd washing was not as effective in discriminating between the RCaW and RCaNW cheeses. The RCaW cheese had a more buttery, caramel odor and flavor, and a more bitter, less sweet, and nutty taste than the SCaW cheese, whereas the RCaNW had a more pungent and less fruity flavor, a less fruity odor, a saltier, more-bitter, and less acidic taste, and a more astringent mouthfeel than

Systemic Cystic Angiomatosis Mimicking Metastatic Cancer: A Case Report and Review of the Literature

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Vivek Kumar

2017-01-01

Full Text Available Systemic cystic angiomatosis is a rare benign disorder due to the maldeveloped vascular and lymphatic system with less than 50 cases reported in literature so far. We report here a case of systemic cystic angiomatosis (SCA with multisystem involvement affecting the neck, thyroid, thoracic cavity, and skeletal system. The patient initially presented in her 4th decade of life with isolated lymphangioma in the neck requiring surgery. However, she experienced full-blown manifestations of SCA in her 6th decade which closely mimicked metastatic cancer. The diagnosis of SCA could only be established after multiple biopsies. The radiological and histological features of SCA with its course over 31 years in this patient have been described.

Microstructural, textural, and sensory characteristics of probiotic yogurts fortified with sodium calcium caseinate or whey protein concentrate.

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Akalın, A S; Unal, G; Dinkci, N; Hayaloglu, A A

2012-07-01

The influence of milk protein-based ingredients on the textural characteristics, sensory properties, and microstructure of probiotic yogurt during a refrigerated storage period of 28 d was studied. Milk was fortified with 2% (wt/vol) skim milk powder as control, 2% (wt/vol) sodium calcium caseinate (SCaCN), 2% (wt/vol) whey protein concentrate (WPC) or a blend of 1% (wt/vol) SCaCN and 1% (wt/vol) WPC. A commercial yogurt starter culture and Bifidobacterium lactis Bb12 as probiotic bacteria were used for the production. The fortification with SCaCN improved the firmness and adhesiveness. Higher values of viscosity were also obtained in probiotic yogurts with SCaCN during storage. However, WPC enhanced water-holding capacity more than the caseinate. Addition of SCaCN resulted in a coarse, smooth, and more compact protein network; however, WPC gave finer and bunched structures in the scanning electron microscopy micrographs. The use of SCaCN decreased texture scores in probiotic yogurt; probably due to the lower water-holding capacity and higher syneresis values in the caseinate-added yogurt sample. Therefore, the textural characteristics of probiotic yogurts improved depending on the ingredient variety. Copyright © 2012 American Dairy Science Association. Published by Elsevier Inc. All rights reserved.

[Sudden cardiac arrest in Italian sports facilities in 2015: epidemiological implications of the so-called "Balduzzi decree"].

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Zorzi, Alessandro; Susana, Angela; Spadotto, Veronica; Cacciavillani, Luisa; Corrado, Domenico

2016-11-01

Under the Italian Law "Legge Balduzzi", which was issued after the sudden cardiac death of professional athletes Pier Mario Morosini and Vigor Bovolenta in 2012, the presence of an automated external defibrillator (AED) and personnel trained to perform cardiopulmonary resuscitation must be available in every Italian sports facility from 2016. In 2015 the national and local press reported 123 cases of sudden cardiac arrests (SCA) occurring in Italian sport facilities, corresponding to an estimated ≈0.2-0.4% of all SCA and to ≈0.6-1.2% of SCA in public places. The majority of SCA victims were males (93%) and >35 years old (88%, median age 50 years). On the basis of the report of the event on the press, the rate of return of spontaneous circulation was 62% when an AED was used before emergency medical system arrival versus 9% when no bystander cardiopulmonary resuscitation or AED use by lay rescuers was mentioned. These data demonstrated that the Law has the potential to increase the survival to SCA in athletes; however, limiting the obligation of the presence of an AED only to sports facilities is not enough to decrease significantly the incidence of SCA in the general population.

Hydroxyurea for sickle cell anemia: What have we learned and what questions still remain?

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McGann, Patrick T.; Ware, Russell E.

2011-01-01

Purpose of review Sickle cell anemia (SCA) is a well-characterized severe hematological disorder with substantial morbidity and early mortality. Hydroxyurea is a potent inducer of fetal hemoglobin, and evidence over the past 25 years has documented its laboratory and clinical efficacy for both adults and children with SCA. Recent findings The Phase III study of hydroxyurea in infants (BABY HUG) has just been completed and preliminary results indicate equivocal benefits for organ protection during the two-year treatment period, but significant benefits for pain, acute chest syndrome, hospitalizations, and transfusions. Three new reports document the benefits of hydroxyurea on reducing mortality in SCA: two adult trials (LaSHS and MSH) and one pediatric study (Brazilian cohort). Recent results from the HUSTLE protocol suggest minimal genotoxicity or carcinogenicity with long-term hydroxyurea exposure. Summary The potential utility of hydroxyurea for all patients with SCA is clear and indisputable. With decades of accumulated evidence and documented efficacy with an acceptable long-term safety profile, it is time to consider hydroxyurea treatment the standard of care for all young patients with SCA. Exporting our knowledge and experience with hydroxyurea to developing nations with large medical burdens from SCA can help relieve global suffering from this condition. PMID:21372708

Which side of the balance determines the frequency of vaso-occlusive crises in children with sickle cell anemia: Blood viscosity or microvascular dysfunction?

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Charlot, Keyne; Romana, Marc; Moeckesch, Berenike; Jumet, Stéphane; Waltz, Xavier; Divialle-Doumdo, Lydia; Hardy-Dessources, Marie-Dominique; Petras, Marie; Tressières, Benoît; Tarer, Vanessa; Hue, Olivier; Etienne-Julan, Maryse; Antoine-Jonville, Sophie; Connes, Philippe

2016-01-01

Vascular resistance and tissue perfusion may be both affected by impaired vascular function and increased blood viscosity. Little is known about the effects of vascular function on the occurrence of painful vaso-occlusive crises (VOC) in children with sickle cell anemia (SCA). The aim of the present study was to determine which side of the balance (blood viscosity or vascular function) is the most deleterious in SCA and increases the risk for frequent hospitalized VOC. Microvascular function, microcirculatory oxygenation and blood viscosity were determined in a group of 22 SCA children/adolescents at steady state and a group of 13 healthy children/adolescents. Univariate analyses demonstrated blunted microvascular reactivity during local thermal heating test and decreased microcirculatory oxygenation in SCA children compared to controls. Multivariate analysis revealed that increased blood viscosity and decreased microcirculatory oxygenation were independent risk factors of frequent VOC in SCA. In contrast, the level of microvascular dysfunction does not predict VOC rate. In conclusion, increased blood viscosity is usually well supported in healthy individuals where vascular function is not impaired. However, in the context of SCA, microvascular function is impaired and any increase of blood viscosity or decrease in microcirculatory oxygenation would increase the risks for frequent VOC. Copyright © 2015 Elsevier Inc. All rights reserved.

Towards a point-of-care strip test to diagnose sickle cell anemia.

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Meaghan Bond

Full Text Available A rapid test to identify patients with sickle cell disease could have important benefits in low-resource settings. Sickle cell anemia (SCA affects about 300,000 newborns each year, the majority of whom are born in sub-Saharan Africa. Low-cost therapies are available to treat SCA, but most countries in sub-Saharan Africa lack robust neonatal screening programs needed to identify patients in need of treatment. To address this need, we developed and evaluated a competitive lateral flow assay that identifies patients with SCA (genotype HbSS in 15 minutes using undiluted whole blood. A small volume of blood (0.5 μL- 3 μL is mixed with antibody-coated blue latex beads in a tube and applied to the strip. Strips are then placed in a well of running buffer and allowed to run for 10 minutes. Laboratory evaluation with samples containing different proportions of hemoglobin A (HbA and hemoglobin S (HbS indicated that the test should enable identification of SCA patients but not persons with sickle cell trait (SCT. We evaluated the test using 41 samples from individuals with SCA, SCT, and normal blood. With visual inspection or quantitative analysis, we found a 98% accuracy when differentiating SCA from normal and SCT samples as a group (90% sensitivity and 100% specificity for identifying SCA. This work demonstrates important steps towards making a lateral flow test for hemoglobinopathies more appropriate for point-of-care use; further work is needed before the test is appropriate for clinical use.

The Iron Status of Sickle Cell Anaemia Patients in Ilorin, North Central Nigeria

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Musa A. Sani

2015-01-01

Full Text Available Objectives. Sickle cell anaemia (SCA is one of the commonest genetic disorders in the world. It is characterized by anaemia, periodic attacks of thrombotic pain, and chronic systemic organ damage. Recent studies have suggested that individuals with SCA especially from developing countries are more likely to be iron deficient rather than have iron overload. The study aims to determine the iron status of SCA patients in Ilorin, Nigeria. Methods. A cross-sectional study of 45 SCA patients in steady state and 45 non-SCA controls was undertaken. FBC, blood film, sFC, sTfR, and sTfR/log sFC index were done on all subjects. Results. The mean patients’ serum ferritin (589.33 ± 427.61 ng/mL was significantly higher than the mean serum ferritin of the controls (184.53 ± 119.74 ng/mL. The mean serum transferrin receptor of the patients (4.24 ± 0.17 μg/mL was higher than that of the controls (3.96 ± 0.17 μg/mL (p=0.290. The mean serum transferrin receptor (sTfR/log serum ferritin index of the patients (1.65 ± 0.27 μg/mL was significantly lower than that of the control (1.82 ± 0.18 μg/mL (p=0.031. Conclusion. Iron deficiency is uncommon in SCA patients and periodic monitoring of the haematological, biochemical, and clinical features for iron status in SCA patients is advised.

MODEL EVALUASI REFLEKTIF KURIKULUM PENDIDIKAN BAHASA INGGRIS DALAM PENGEMBANGAN KARAKTER BANGSA

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Monika Gultom

2014-06-01

Abstract This study aimed to develop a model of reflective curriculum evaluation at the English Education Study Program that portrayed the delivery of the values of national character in the classroom. This development study used mixed approach carried out in five phases:  initial study, defining, designing, demonstrating, and developing.  The constructs of instruments were developed based on expert judgments; the subjects were lecturers and students of English Language Education Study Program in four universities in Yogyakarta Special Territory. The composite reliability coefficient of the instruments was analyzed by SEM program of SmartPLS Version 2.0.M3 and test of fit model by GeSCA. The result of the study concluded: 1 the developed model evaluation was supported by five instruments of evaluation; 2 validity, reliability, and its implementation had been verified through FGD, Delphy techniques, and non-parametric confirmatory factor analysis; 3 the composite reliability coefficient of the five instruments was 0.93, 0.91, 0.95, 0.95, and 0.86; 4 the result of the test of fit model indicated that  model was supported by the data with GFI 0.99 and SRMR 0.06; 5 The guidelines of using the developed model consisted of background, rational, components of assessment, guidelines, and application sample.Keywords: developing model of curriculum evaluation, national character

Archetypes of Supply Chain Analytics Initiatives—An Exploratory Study

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Tino T. Herden

2018-05-01

Full Text Available While Big Data and Analytics are arguably rising stars of competitive advantage, their application is often presented and investigated as an overall approach. A plethora of methods and technologies combined with a variety of objectives creates a barrier for managers to decide how to act, while researchers investigating the impact of Analytics oftentimes neglect this complexity when generalizing their results. Based on a cluster analysis applied to 46 case studies of Supply Chain Analytics (SCA we propose 6 archetypes of initiatives in SCA to provide orientation for managers as means to overcome barriers and build competitive advantage. Further, the derived archetypes present a distinction of SCA for researchers seeking to investigate the effects of SCA on organizational performance.

A critical appraisal of paliperidone long-acting injection in the treatment of schizoaffective disorder

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Chue P

2016-01-01

Full Text Available Pierre Chue,1 James Chue2 1Department of Psychiatry, University of Alberta, 2Clinical Trials and Research Program, Edmonton, AB, Canada Abstract: Schizoaffective disorder (SCA is a chronic and disabling mental illness that presents with mixed symptoms of schizophrenia and affective disorders. SCA is recognized as a discrete disorder, but with greater heterogeneity and symptom overlap, leading to difficulty and delay in diagnosis. Although the overall prognosis is intermediate between schizophrenia and mood disorders, SCA is associated with higher rates of suicide and hospitalization than schizophrenia. No treatment guidelines exist for SCA, and treatment is frequently complex, involving off-label use and polypharmacy (typically combinations of antipsychotics, mood stabilizers, and antidepressants. Oral paliperidone extended-release was the first agent to be approved for the treatment of SCA. As in schizophrenia and bipolar disorder, adherence to oral medications is poor, further contributing to suboptimal outcomes. The use of an antipsychotic in a long-acting injection (LAI addresses adherence issues, thus potentially reducing relapse. Paliperidone palmitate represents the LAI formulation of paliperidone. In a long-term, double-blind, randomized, controlled trial of adult patients (n=334; intent-to-treat [ITT] with SCA, paliperidone long-acting injection (PLAI significantly delayed risk of relapse compared to placebo (hazard ratio 2.49, 95% confidence interval, 1.55–3.99; P<0.001. This study demonstrated the efficacy and safety of PLAI when used as either monotherapy or adjunctive therapy for the maintenance treatment of SCA. The results are consistent with a similarly designed study conducted in patients with schizophrenia, which suggests a benefit in the long-term control of not only psychotic but also affective symptoms. No new safety signals were observed. When used in monotherapy, PLAI simplifies treatment by reducing complex

Early repolarization with horizontal ST segment may be associated with aborted sudden cardiac arrest: a retrospective case control study

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Kim Sung

2012-12-01

Full Text Available Abstract Background Risk stratification of the early repolarization pattern (ERP is needed to identify malignant early repolarization. J-point elevation with a horizontal ST segment was recently suggested as a malignant feature of the ERP. In this study, the prevalence of the ERP with a horizontal ST segment was examined among survivors of sudden cardiac arrest (SCA without structural heart disease to evaluate the value of ST-segment morphology in risk stratification of the ERP. Methods We reviewed the data of 83 survivors of SCA who were admitted from August 2005 to August 2010. Among them, 25 subjects without structural heart disease were included. The control group comprised 60 healthy subjects who visited our health promotion center; all control subjects were matched for age, sex, and underlying disease (diabetes mellitus, hypertension. Early repolarization was defined as an elevation of the J point of at least 0.1 mV above the baseline in at least two continuous inferior or lateral leads that manifested as QRS slurring or notching. An ST-segment pattern of Results The SCA group included 17 men (64% with a mean age of 49.7 ± 14.5 years. The corrected QTc was not significantly different between the SCA and control groups (432.7 ± 37.96 vs. 420.4 ± 26.3, respectively; p = 0.089. The prevalence of ERP was not statistically different between the SCA and control groups (5/25, 20% vs. 4/60, 6.7%, respectively; p = 0.116. The prevalence of early repolarization with a horizontal ST segment was more frequent in the SCA than in the control group (20% vs. 3.3%, respectively; p = 0.021. Four SCA subjects (16% and one control subject (1.7% had a J-point elevation of >2 mm (p = 0.025. Four SCA subjects (16% and one (1.7% control subject had an ERP in the inferior lead (p = 0.025. Conclusion The prevalence of ERP with a horizontal ST segment was higher in patients with aborted SCA than in matched controls. This result suggests that ST morphology has

Concentrating Solar Power Projects - ISCC Duba 1 | Concentrating Solar

Science.gov (United States)

Solar Break Ground: 2016 Start Production: 2017 Participants Developer(s): Saudi Electricity Co. Owner(s ) (%): Saudi Electricity Co. EPC Contractor: Initec Energia Generation Offtaker(s): Saudi Electricity Co. Plant Configuration Solar Field SCA Manufacturer (Model): Flabeg (Ultimate Trough) HCE Manufacturer: Archimede Solar

JMBR APRIL.cdr

African Journals Online (AJOL)

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contribution of SCA related morbidities to paediatric admissions. This descriptive ... morbidities in children with. SCA were infections (44.1%), vaso-occlusive crisis (29.4%), severe anaemia .... system complications, particularly cerebrovascular ...

Two in one: report of a patient with spinocerebellar ataxia types 2 and 10.

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Kapur, Sachin S; Goldman, Jennifer G

2012-09-01

To report a rare case of the coexistence of 2 spinocerebellar ataxia (SCA) mutations in a single patient. Case report. University hospital, Movement Disorders Center. A 54-year-old man of Mexican, American Indian, and French descent with an 11-year history of gait and limb ataxia. Findings of clinical examination, magnetic resonance imaging, and video electroencephalographic monitoring. Neurologic history revealed a gradually progressive gait and limb ataxia along with muscle cramps and sensory symptoms in his distal extremities; examination revealed executive dysfunction, dysarthria, ataxia, and sensory neuronopathy. Episodes of loss of awareness were reported, but electroencephalograms were negative. Brain imaging demonstrated severe cerebellar and brainstem atrophy. Genetic evaluation of the case revealed mutations in both the SCA2 and SCA10 genes. Our patient has a unique combination of genetic mutations for 2 different SCAs, types 2 and 10, which to our knowledge, has not been previously reported. His clinical phenotype is largely consistent with SCA2, but his possible seizures and Mexican heritage suggest influences of SCA10.

Spinocerebellar ataxia-10 with paranoid schizophrenia

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Bhavesh Trikamji

2015-01-01

Full Text Available Spino-cerebellar ataxia type 10 (SCA10 is an autosomal dominant disorder that is characterized by cerebellar ataxia, seizures and nystagmus with a fragmented pursuit. Schizophrenia has been reported with SCAs 1 and 2 yet in SCA 10, psychiatric manifestations are uncommon. We report a Hispanic family involving a father and his four children with SCA10 genetic mutation. Two of his children, a 20-year-old female and a 23-year-old male, presented with gradually progressive spino-cerebellar ataxia and paranoid schizophrenia. Neurological examination revealed ocular dysmetria, dysdiadokinesia, impaired finger-to-nose exam, gait ataxia and hyperreflexia in both the cases. Additionally, they had a history of psychosis with destructive behavior, depression and paranoid delusions with auditory hallucinations. Serology and CSF studies were unremarkable and MRI brain revealed cerebellar volume loss. Ultimately, a test for ATAXIN-10 mutation was positive thus confirming the diagnosis of SCA10 in father and his four children. We now endeavor to investigate the association between schizophrenia and SCA10.

Safety critical FPGA-based NPP instrumentation and control systems: assessment, development and implementation

International Nuclear Information System (INIS)

Bakhmach, E. S.; Siora, A. A.; Tokarev, V. I.; Kharchenko, V. S.; Sklyar, V. V.; Andrashov, A. A.

2010-10-01

The stages of development, production, verification, licensing and implementation methods and technologies of safety critical instrumentation and control systems for nuclear power plants (NPP) based on FPGA (Field Programmable Gates Arrays) technologies are described. A life cycle model and multi-version technologies of dependability and safety assurance of FPGA-based instrumentation and control systems are discussed. An analysis of NPP instrumentation and control systems construction principles developed by Research and Production Corporation Radiy using FPGA-technologies and results of these systems implementation and operation at Ukrainian and Bulgarian NPP are presented. The RADIY TM platform has been designed and developed by Research and Production Corporation Radiy, Ukraine. The main peculiarity of the RADIY TM platform is the use of FPGA as programmable components for logic control operation. The FPGA-based RADIY TM platform used for NPP instrumentation and control systems development ensures sca lability of system functions types, volume and peculiarities (by changing quantity and quality of sensors, actuators, input/output signals and control algorithms); sca lability of dependability (safety integrity) (by changing a number of redundant channel, tiers, diagnostic and reconfiguration procedures); sca lability of diversity (by changing types, depth and method of diversity selection). (Author)

Inversion of Land Surface Temperature (LST Using Terra ASTER Data: A Comparison of Three Algorithms

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Milton Isaya Ndossi

2016-12-01

Full Text Available Land Surface Temperature (LST is an important measurement in studies related to the Earth surface’s processes. The Advanced Space-borne Thermal Emission and Reflection Radiometer (ASTER instrument onboard the Terra spacecraft is the currently available Thermal Infrared (TIR imaging sensor with the highest spatial resolution. This study involves the comparison of LSTs inverted from the sensor using the Split Window Algorithm (SWA, the Single Channel Algorithm (SCA and the Planck function. This study has used the National Oceanic and Atmospheric Administration’s (NOAA data to model and compare the results from the three algorithms. The data from the sensor have been processed by the Python programming language in a free and open source software package (QGIS to enable users to make use of the algorithms. The study revealed that the three algorithms are suitable for LST inversion, whereby the Planck function showed the highest level of accuracy, the SWA had moderate level of accuracy and the SCA had the least accuracy. The algorithms produced results with Root Mean Square Errors (RMSE of 2.29 K, 3.77 K and 2.88 K for the Planck function, the SCA and SWA respectively.

Movement disorders in hereditary ataxias.

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Garcia Ruiz, Pedro J; Mayo, David; Hernandez, Jaime; Cantarero, Susana; Ayuso, Carmen

2002-10-15

Movement disorders are well known features of some dominant hereditary ataxias (HA), specially SCA3/Machado-Joseph disease and dentatorubropallidolusyan atrophy. However, little is known about the existence and classification of movement disorders in other dominant and recessive ataxias. We prospectively studied the presence of movement disorders in patients referred for HA over the last 3 years. Only those patients with a confirmed family history of ataxia were included. We studied 84 cases of HA, including 46 cases of recessive and 38 cases of dominant HA. Thirty out of 46 cases of recessive HA could be classified as: Friedreich ataxia (FA), 29 cases; vitamin E deficiency, 1 case. Twenty-three out of 38 cases of dominant HA could be classified as: SCA 2, 4 cases; SCA 3, 8 cases; SCA 6, 4 cases; SCA 7, 6 cases and SCA 8, 1 case. We observed movement disorders in 20/38 (52%) patients with dominant HA and 25/46 (54%) cases with recessive HA, including 16 patients (16/29) with FA. In general, postural tremor was the most frequent observed movement disorder (27 cases), followed by dystonia (22 cases). Five patients had akinetic rigid syndrome, and in 13 cases, several movement disorders coexisted. Movement disorders are frequent findings in HA, not only in dominant HA but also in recessive HA. Copyright 2002 Elsevier Science B.V.

Colocalization and Disposition of Cellulosomes in Clostridium clariflavum as Revealed by Correlative Superresolution Imaging

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Lior Artzi

2018-02-01

Full Text Available Cellulosomes are multienzyme complexes produced by anaerobic, cellulolytic bacteria for highly efficient breakdown of plant cell wall polysaccharides. Clostridium clariflavum is an anaerobic, thermophilic bacterium that produces the largest assembled cellulosome complex in nature to date, comprising three types of scaffoldins: a primary scaffoldin, ScaA; an adaptor scaffoldin, ScaB; and a cell surface anchoring scaffoldin, ScaC. This complex can contain 160 polysaccharide-degrading enzymes. In previous studies, we proposed potential types of cellulosome assemblies in C. clariflavum and demonstrated that these complexes are released into the extracellular medium. In the present study, we explored the disposition of the highly structured, four-tiered cell-anchored cellulosome complex of this bacterium. Four separate, integral cellulosome components were subjected to immunolabeling: ScaA, ScaB, ScaC, and the cellulosome’s most prominent enzyme, GH48. Imaging of the cells by correlating scanning electron microscopy and three-dimensional (3D superresolution fluorescence microscopy revealed that some of the protuberance-like structures on the cell surface represent cellulosomes and that the components are highly colocalized and organized by a defined hierarchy on the cell surface. The display of the cellulosome on the cell surface was found to differ between cells grown on soluble or insoluble substrates. Cell growth on microcrystalline cellulose and wheat straw exhibited dramatic enhancement in the amount of cellulosomes displayed on the bacterial cell surface.

H/D exchange mass spectrometry and statistical coupling analysis reveal a role for allostery in a ferredoxin-dependent bifurcating transhydrogenase catalytic cycle.

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Berry, Luke; Poudel, Saroj; Tokmina-Lukaszewska, Monika; Colman, Daniel R; Nguyen, Diep M N; Schut, Gerrit J; Adams, Michael W W; Peters, John W; Boyd, Eric S; Bothner, Brian

2018-01-01

Recent investigations into ferredoxin-dependent transhydrogenases, a class of enzymes responsible for electron transport, have highlighted the biological importance of flavin-based electron bifurcation (FBEB). FBEB generates biomolecules with very low reduction potential by coupling the oxidation of an electron donor with intermediate potential to the reduction of high and low potential molecules. Bifurcating systems can generate biomolecules with very low reduction potentials, such as reduced ferredoxin (Fd), from species such as NADPH. Metabolic systems that use bifurcation are more efficient and confer a competitive advantage for the organisms that harbor them. Structural models are now available for two NADH-dependent ferredoxin-NADP + oxidoreductase (Nfn) complexes. These models, together with spectroscopic studies, have provided considerable insight into the catalytic process of FBEB. However, much about the mechanism and regulation of these multi-subunit proteins remains unclear. Using hydrogen/deuterium exchange mass spectrometry (HDX-MS) and statistical coupling analysis (SCA), we identified specific pathways of communication within the model FBEB system, Nfn from Pyrococus furiosus, under conditions at each step of the catalytic cycle. HDX-MS revealed evidence for allosteric coupling across protein subunits upon nucleotide and ferredoxin binding. SCA uncovered a network of co-evolving residues that can provide connectivity across the complex. Together, the HDX-MS and SCA data show that protein allostery occurs across the ensemble of iron‑sulfur cofactors and ligand binding sites using specific pathways that connect domains allowing them to function as dynamically coordinated units. Copyright © 2017 Elsevier B.V. All rights reserved.

Pharmacokinetics and bioequivalence of a liquid formulation of hydroxyurea in children with sickle cell anemia.

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Estepp, Jeremie H; Melloni, Chiara; Thornburg, Courtney D; Wiczling, Paweł; Rogers, Zora; Rothman, Jennifer A; Green, Nancy S; Liem, Robert; Brandow, Amanda M; Crary, Shelley E; Howard, Thomas H; Morris, Maurine H; Lewandowski, Andrew; Garg, Uttam; Jusko, William J; Neville, Kathleen A

2016-03-01

Hydroxyurea (HU) is a crucial therapy for children with sickle cell anemia, but its off-label use is a barrier to widespread acceptance. We found HU exposure is not significantly altered by liquid vs capsule formulation, and weight-based dosing schemes provide consistent exposure. HU is recommended for all children starting as young as 9 months of age with sickle cell anemia (SCA; HbSS and HbSβspan(0) thalassemia); however; a paucity of pediatric data exists regarding the pharmacokinetics (PK) or the exposure-response relationship of HU. This trial aimed to characterize the PK of HU in children and to evaluate and compare the bioavailability of a liquid vs capsule formulation. This multicenter; prospective; open-label trial enrolled 39 children with SCA who provided 682 plasma samples for PK analysis following administration of HU. Noncompartmental and population PK models are described. We report that liquid and capsule formulations of HU are bioequivalent; weight-based dosing schemes provide consistent drug exposure; and age-based dosing schemes are unnecessary. These data support the use of liquid HU in children unable to swallow capsules and in those whose weight precludes the use of fixed capsule formulations. Taken with existing safety and efficacy literature; these findings should encourage the use of HU across the spectrum of age and weight in children with SCA; and they should facilitate the expanded use of HU as recommended in the National Heart; Lung; and Blood Institute guidelines for individuals with SCA. © 2015, The American College of Clinical Pharmacology.

Brain derived neurotrophic factor contributes to the cardiogenic potential of adult resident progenitor cells in failing murine heart.

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Rasmita Samal

Full Text Available Resident cardiac progenitor cells show homing properties when injected into the injured but not to the healthy myocardium. The molecular background behind this difference in behavior needs to be studied to elucidate how adult progenitor cells can restore cardiac function of the damaged myocardium. Since the brain derived neurotrophic factor (BDNF moderates cardioprotection in injured hearts, we focused on delineating its regulatory role in the damaged myocardium.Comparative gene expression profiling of freshly isolated undifferentiated Sca-1 progenitor cells derived either from heart failure transgenic αMHC-CyclinT1/Gαq overexpressing mice or wildtype littermates revealed transcriptional variations. Bdnf expression was up regulated 5-fold during heart failure which was verified by qRT-PCR and confirmed at protein level. The migratory capacity of Sca-1 cells from transgenic hearts was improved by 15% in the presence of 25 ng/ml BDNF. Furthermore, BDNF-mediated effects on Sca-1 cells were studied via pulsed Stable Isotope Labeling of Amino acids in Cell Culture (pSILAC proteomics approach. After BDNF treatment significant differences between newly synthesized proteins in Sca-1 cells from control and transgenic hearts were observed for CDK1, SRRT, HDGF, and MAP2K3 which are known to regulate cell cycle, survival and differentiation. Moreover BDNF repressed the proliferation of Sca-1 cells from transgenic hearts.Comparative profiling of resident Sca-1 cells revealed elevated BDNF levels in the failing heart. Exogenous BDNF (i stimulated migration, which might improve the homing ability of Sca-1 cells derived from the failing heart and (ii repressed the cell cycle progression suggesting its potency to ameliorate heart failure.

Revascularization of the upper posterior circulation with the anterior temporal artery: an anatomical feasibility study.

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Tayebi Meybodi, Ali; Lawton, Michael T; Griswold, Dylan; Mokhtari, Pooneh; Payman, Andre; Tabani, Halima; Yousef, Sonia; Benet, Arnau

2017-09-22

OBJECTIVE In various disease processes, including unclippable aneurysms, a bypass to the upper posterior circulation (UPC) including the superior cerebellar artery (SCA) and posterior cerebral artery (PCA) may be needed. Various revascularization options exist, but the role of intracranial (IC) donors has not been scrutinized. The objective of this study was to evaluate the anatomical feasibility of utilizing the anterior temporal artery (ATA) for revascularization of the UPC. METHODS ATA-SCA and ATA-PCA bypasses were performed on 14 cadaver specimens. After performing an orbitozygomatic craniotomy and opening the basal cisterns, the ATA was divided at the M 3 -M 4 junction and mobilized to the crural cistern to complete an end-to-side bypass to the SCA and PCA. The length of the recipient artery between the anastomosis and origin was measured. RESULTS Seventeen ATAs were found. Successful anastomosis was performed in 14 (82%) of the ATAs. The anastomosis point on the PCA was 14.2 mm from its origin on the basilar artery. The SCA anastomosis point was 10.1 mm from its origin. Three ATAs did not reach the UPC region due to a common opercular origin with the middle temporal artery. The ATA-SCA bypass was also applied to the management of an incompletely coiled SCA aneurysm. CONCLUSIONS The ATA is a promising IC donor for UPC revascularization. The ATA is exposed en route to the proximal SCA and PCA through the pterional-orbitozygomatic approach. Also, the end-to-side anastomosis provides an efficient and straightforward bypass without the need to harvest a graft or perform multiple or difficult anastomoses.

Effect of additions of cerium, lanthanum, and zirconium on the state of plantinum and the activity of aluminoplatinum catalysts for the complete oxidation of hydrocarbons

International Nuclear Information System (INIS)

Drozdov, V.A.; Davydov, A.A.; Popovskii, V.V.; Tsyrul'nikov, P.G.

1986-01-01

It is shown from an analysis of the diffuse reflectance spectra that additions of cerium, lanthanum or zirconium to aluminoplatinum catalyst stabilize the platinum in an oxidized state. This leads to a change in the specific catalytic activity (SCA) towards the total oxidation of methane and butane. The SCA of modified, reduced samples is greater than the SCA of samples that were calcined in air. This is because of the greater activity of metallic platinum compared to the ionic form

Reduced cardiac {sup 123}I-metaiodobenzylguanidine uptake in patients with spinocerebellar ataxia type 2: a comparative study with Parkinson's disease

Energy Technology Data Exchange (ETDEWEB)

De Rosa, Anna; De Leva, Maria Fulvia; Maddaluno, Gennaro; Filla, Alessandro; De Michele, Giuseppe [University Federico II, Department of Neurosciences and Reproductive and Odontostomatologic Sciences, Naples (Italy); Pappata, Sabina; Pellegrino, Teresa [National Council of Research, Institute of Biostructure and Bioimaging, Naples (Italy); Fiumara, Giovanni [Institute of Diagnostic and Nuclear Development, SDN Foundation, Naples (Italy); Carotenuto, Raffaella; Cuocolo, Alberto [University Federico II, Department of Advanced Biomedical Sciences, Naples (Italy); Petretta, Mario [University Federico II, Department of Translational Medical Sciences, Naples (Italy)

2013-12-15

Spinocerebellar ataxia type 2 (SCA2) is an autosomal dominant neurodegenerative disorder characterized by cerebellar ataxia, supranuclear ophthalmoplegia, and peripheral neuropathy. Autonomic nervous system dysfunction is often present. This study evaluated the cardiac sympathetic function in patients with SCA2 using {sup 123}I-metaiodobenzylguanidine (MIBG) in comparison with patients with Parkinson's disease (PD) and control subjects. Nine patients with SCA2, nine patients with PD, and nine control subjects underwent {sup 123}I-MIBG imaging studies from which early and late heart-to-mediastinum (H/M) ratios and myocardial washout rates were calculated. Early (F = 12.3, p < 0.0001) and late (F = 16.8, p < 0.0001) H/M ratios were significantly different among groups. In controls, early and late H/M ratios (2.2 {+-} 0.12 and 2.1 {+-} 0.20) were significantly higher than in patients with SCA2 (1.9 {+-} 0.23 and 1.8 {+-} 0.20, both p < 0.05) and with patients with PD (1.7 {+-} 0.29 and 1.4 {+-} 0.35, both p < 0.001). There was also a significant difference in washout rates among groups (F = 11.7, p < 0.0001). In controls the washout rate (19.9 {+-} 9.6 %) was significantly lower (p < 0.005) than in patients with PD (51.0 {+-} 23.7 %), but not different from that in SCA2 patients (19.5 {+-} 9.4 %). In SCA2 patients, in a multivariable linear regression analysis only the Scale for the Assessment and Rating of Ataxia score was independently associated with early H/M ratio ({beta} = -0.12, p < 0.05). {sup 123}I-MIBG myocardial scintigraphy demonstrated an impairment of cardiac sympathetic function in patients with SCA2, which was less marked than in PD patients. These results suggest that {sup 123}I-MIBG cardiac imaging could become a useful tool for analysing the pathophysiology of SCA2. (orig.)

Foetal haemoglobin and disease severity in sickle cell anaemia patients in Kampala, Uganda

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Mpalampa Lena

2012-09-01

Full Text Available Abstract Background Sickle cell anaemia (SCA is a major chronic health problem in Uganda. In patients with SCA, the level of foetal haemoglobin (HbF has been found to be important in influencing the clinical course of the disease. Thus populations with high levels of HbF like those in Saudi Arabia have been described as having a milder clinical course with fewer complications as compared to populations with lower levels. Disease modifying drugs can increase the Hb F levels and modify the presentation of SCA. Methods This was a cross sectional study in which we determined foetal haemoglobin levels and examined the relationship between HbF levels and disease severity in SCA patients in Mulago Hospital, Kampala, Uganda. We consecutively enrolled 216 children aged 1 year to 18 years with SCA attending the Sickle Cell Clinic at Mulago Hospital whose guardians had given consent. The history included age at onset of initial symptoms and diagnosis, number of hospitalisations and blood transfusions and other complications of SCA (cardiovascular accidents, avascular hip necrosis and priapism. A detailed physical examination was performed to assess the current state and help describe the disease severity for each patient. Blood samples were drawn for HbF levels. HbF levels ≥10% was defined as high. Results Of the 216 children, (80 37% had HbF levels ≥10%. Significant correlations were observed between HbF level and several clinical parameters independent of age including age at diagnosis (p value 0.013, number of hospitalisations (p value 0.024 and transfusions (p value 0.018 since birth. Conclusion A third of the children with SCA attending the Sickle cell clinic in Mulago Hospital have high HbF levels. Higher HbF level is associated with later onset of symptoms and presentation, and less severe disease characterised by fewer hospitalisations and blood transfusions. We suggest HbF levels should be determined at initial contact for patients with SCA to

Bilateral subclavian origin of the bronchial arteries combined with absence of other origins.

Science.gov (United States)

Jie, Bing; Sun, Xi-Wen; Yu, Dong; Jiang, Sen

2014-08-01

There are numerous anatomical variations of the sites of origin of the bronchial arteries (BAs). A subclavian origin of a BA involves an aberrant artery that originates from the subclavian artery (SCA) or its branches. However, the aberrant artery usually originates directly from the SCA, and an SCA-origin BA arising from the branches of the SCA is rare. We herein present an extremely rare case of a right BA arising from the ipsilateral costocervical trunk, and a left BA arising from the ipsilateral thyrocervical trunk, in the absence of other origins of the BA. This anatomical variation was detected during pretherapeutic evaluation by multidetector-row computed tomography and confirmed by selective angiography. Recognition of these anatomic variations is important to surgical, diagnostic, and interventional radiologic procedures in the thorax.

Temporal Changes in FLT3 ITD Regulation of Stem Cell Self Renewal and Leukemogenesis

Science.gov (United States)

2016-11-01

AML) more frequently in adults than in children. FLT3-ITD encodes a constitutively active FLT3 tyrosine kinase. This mutation occurs in ~30% of adult...2006). FLT3ITD encodes a constitutively active tyrosine kinase 87   receptor that has been shown to activate the STAT5, MAP-kinase (MAPK), PI3-88...define cell populations: HSCs (CD150+, CD48-Lineage-, Sca1+, c-kit+), HPCs 570   (CD48-Lineage-, Sca1+, c-kit+), and GMPs (Lineage-, Sca1-, CD127-, c

Descrição de dois anos de atuação da Comissão de Ética no Uso de Animais do Setor de Ciências Agrárias da Universidade Federal do Paraná Two years description of the Animal Use Ethic Committee of the Agrarian Science Sector of the Federal University of Paraná

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Vanessa Carli Bones Silla

2009-10-01

Full Text Available A Comissão de Ética no Uso de Animais do Setor de Ciências Agrárias (CEUA-SCA da Universidade Federal do Paraná (UFPR foi criada em 2005. Este artigo teve por objetivo estudar os dois primeiros anos do trabalho dessa Comissão, principalmente quanto à redução do sofrimento animal. Foram avaliadas a composição e a metodologia de trabalho da CEUA-SCA, além da efetividade na redução do sofrimento animal, pela razão emissão de certificados por projetos protocolados (C:P e pelo estudo dos projetos que receberam ofícios. Foram avaliados 42 protocolos de pesquisa de 2006 e 24 de 2007. Animais vertebrados foram os mais usados, submetidos com maior frequência a procedimentos grau B de invasividade. Os resultados sugerem que a CEUA-SCA, no período estudado, colaborou para o controle do uso de animais nas pesquisas sob a responsabilidade do SCA-UFPR. Destaca-se a necessidade do preenchimento adequado dos formulários pelos proponentes, o aumento da pluralidade na composição da Comissão e a importância de um órgão federal que centralize informações relativas à atuação das CEUAs no Brasil.The Animal Use Ethic Committee of the Agrarian Sciences Sector (CEUA-SCA of the Federal University of Paraná (UFPR was created in 2005. The objective of this research was to describe the first two years of CEUA-SCA's work, mainly regarding to the reduction of the animal suffering. The CEUA-SCA's composition and work methodology were studied, as well as the effectiveness to reduce the animal suffering, evaluated by the ratio of certificates emitted and projects registered (C:P and by the observation of projects that received official letters. Forty two research protocols in 2006 and 24 in 2007 were evaluated. Animals used were mostly vertebrates being submitted more frequently to procedures considered B for invasiveness degree. The results show that the CEUA-SCA, in the studied period, collaborated to the control of the animal use under the

Murine bone marrow Lin⁻Sca⁻1⁺CD45⁻ very small embryonic-like (VSEL cells are heterogeneous population lacking Oct-4A expression.

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Krzysztof Szade

Full Text Available Murine very small embryonic-like (VSEL cells, defined by the Lin(-Sca-1(+CD45(- phenotype and small size, were described as pluripotent cells and proposed to be the most primitive hematopoietic precursors in adult bone marrow. Although their isolation and potential application rely entirely on flow cytometry, the immunophenotype of VSELs has not been extensively characterized. Our aim was to analyze the possible heterogeneity of Lin(-Sca(+CD45(- population and investigate the extent to which VSELs characteristics may overlap with that of hematopoietic stem cells (HSCs or endothelial progenitor cells (EPCs. The study evidenced that murine Lin(-Sca-1(+CD45(- population was heterogeneous in terms of c-Kit and KDR expression. Accordingly, the c-Kit(+KDR(-, c-Kit(-KDR(+, and c-Kit(-KDR(- subpopulations could be distinguished, while c-Kit(+KDR(+ events were very rare. The c-Kit(+KDR(- subset contained almost solely small cells, meeting the size criterion of VSELs, in contrast to relatively bigger c-Kit(-KDR(+ cells. The c-Kit(-KDR(-FSC(low subset was highly enriched in Annexin V-positive, apoptotic cells, hence omitted from further analysis. Importantly, using qRT-PCR, we evidenced lack of Oct-4A and Oct-4B mRNA expression either in whole adult murine bone marrow or in the sorted of Lin(-Sca-1(+CD45(-FSC(low population, even by single-cell qRT-PCR. We also found that the Lin(-Sca-1(+CD45(-c-Kit(+ subset did not exhibit hematopoietic potential in a single cell-derived colony in vitro assay, although it comprised the Sca-1(+c-Kit(+Lin(- (SKL CD34(-CD45(-CD105(+ cells, expressing particular HSC markers. Co-culture of Lin(-Sca-1(+CD45(-FSC(low with OP9 cells did not induce hematopoietic potential. Further investigation revealed that SKL CD45(-CD105(+ subset consisted of early apoptotic cells with fragmented chromatin, and could be contaminated with nuclei expelled from erythroblasts. Concluding, murine bone marrow Lin(-Sca-1(+CD45(-FSC(low cells are

Cardiac iron overload in chronically transfused patients with thalassemia, sickle cell anemia, or myelodysplastic syndrome.

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Mariane de Montalembert

Full Text Available The risk and clinical significance of cardiac iron overload due to chronic transfusion varies with the underlying disease. Cardiac iron overload shortens the life expectancy of patients with thalassemia, whereas its effect is unclear in those with myelodysplastic syndromes (MDS. In patients with sickle cell anemia (SCA, iron does not seem to deposit quickly in the heart. Our primary objective was to assess through a multicentric study the prevalence of cardiac iron overload, defined as a cardiovascular magnetic resonance T2*8 ECs in the past year, and age older than 6 years. We included from 9 centers 20 patients with thalassemia, 41 with SCA, and 25 with MDS in 2012-2014. Erythrocytapharesis did not consistently prevent iron overload in patients with SCA. Cardiac iron overload was found in 3 (15% patients with thalassemia, none with SCA, and 4 (16% with MDS. The liver iron content (LIC ranged from 10.4 to 15.2 mg/g dry weight, with no significant differences across groups (P = 0.29. Abnormal T2* was not significantly associated with any of the measures of transfusion or chelation. Ferritin levels showed a strong association with LIC. Non-transferrin-bound iron was high in the thalassemia and MDS groups but low in the SCA group (P<0.001. Hepcidin was low in thalassemia, normal in SCA, and markedly elevated in MDS (P<0.001. Two mechanisms may explain that iron deposition largely spares the heart in SCA: the high level of erythropoiesis recycles the iron and the chronic inflammation retains iron within the macrophages. Thalassemia, in contrast, is characterized by inefficient erythropoiesis, unable to handle free iron. Iron accumulation varies widely in MDS syndromes due to the competing influences of abnormal erythropoiesis, excess iron supply, and inflammation.

Identification and evaluation of two diagnostic markers linked to Fusarium wilt resistance (race 4) in banana (Musa spp.).

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Wang, Wei; Hu, Yulin; Sun, Dequan; Staehelin, Christian; Xin, Dawei; Xie, Jianghui

2012-01-01

Fusarium wilt caused by the fungus Fusarium oxysporum f. sp. cubense race 4 (FOC4) results in vascular tissue damage and ultimately death of banana (Musa spp.) plants. Somaclonal variants of in vitro micropropagated banana can hamper success in propagation of genotypes resistant to FOC4. Early identification of FOC4 resistance in micropropagated banana plantlets is difficult, however. In this study, we identified sequence-characterized amplified region (SCAR) markers of banana associated with resistance to FOC4. Using pooled DNA from resistant or susceptible genotypes and 500 arbitrary 10-mer oligonucleotide primers, 24 random amplified polymorphic DNA (RAPD) products were identified. Two of these RAPD markers were successfully converted to SCAR markers, called ScaU1001 (GenBank accession number HQ613949) and ScaS0901 (GenBank accession number HQ613950). ScaS0901 and ScaU1001 could be amplified in FOC4-resistant banana genotypes ("Williams 8818-1" and Goldfinger), but not in five tested banana cultivars susceptible to FOC4. The two SCAR markers were then used to identify a somaclonal variant of the genotype "Williams 8818-1", which lost resistance to FOC4. Hence, the identified SCAR markers can be applied for a rapid quality control of FOC4-resistant banana plantlets immediately after the in vitro micropropagation stage. Furthermore, ScaU1001 and ScaS0901 will facilitate marker-assisted selection of new banana cultivars resistant to FOC4.

Tracking Snow Variations in the Northern Hemisphere Using Multi-Source Remote Sensing Data (2000–2015

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Yunlong Wang

2018-01-01

Full Text Available Multi-source remote sensing data were used to generate 500-m resolution cloud-free daily snow cover images for the Northern Hemisphere. Simultaneously, the spatial and temporal dynamic variations of snow in the Northern Hemisphere were evaluated from 2000 to 2015. The results indicated that (1 the maximum, minimum, and annual average snow-covered area (SCA in the Northern Hemisphere exhibited a fluctuating downward trend; the variation of snow cover in the Northern Hemisphere had well-defined inter-annual and regional differences; (2 the average SCA in the Northern Hemisphere was the largest in January and the smallest in August; the SCA exhibited a downward trend for the monthly variations from February to April; and the seasonal variation in the SCA exhibited a downward trend in the spring, summer, and fall in the Northern Hemisphere (no pronounced variation trend in the winter was observed during the 2000–2015 period; (3 the spatial distribution of the annual average snow-covered day (SCD was related to the latitudinal zonality, and the areas exhibiting an upward trend were mainly at the mid to low latitudes with unstable SCA variations; and (4 the snow reduction was significant in the perennial SCA in the Northern Hemisphere, including high-latitude and high-elevation mountainous regions (between 35° and 50°N, such as the Tibetan Plateau, the Tianshan Mountains, the Pamir Plateau in Asia, the Alps in Europe, the Caucasus Mountains, and the Cordillera Mountains in North America.

Use of whole body CT to detect patterns of CPR-related injuries after sudden cardiac arrest.

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Dunham, Gregor M; Perez-Girbes, Alexandre; Bolster, Ferdia; Sheehan, Kellie; Linnau, Ken F

2017-11-09

We have recently implemented a dedicated sudden cardiac arrest (SCA) - whole-body computed tomography (WBCT) protocol to evaluate SCA patients with return of spontaneous circulation (ROSC) following cardiopulmonary resuscitation (CPR). The aim of this study is to evaluate the number and pattern of CPR-related injuries in ROSC patients with SCA-WBCT. Single-centre retrospective review of 39 patients (13 female; 20 male, mean age 51.8 years) with non-traumatic, out-of-hospital SCA and ROSC and evaluation with dedicated SCA-WBCT over a 10-month period. In-hospital mortality was 54%. CPR-related injuries were detected in 85% (33/39). Chest injuries were most common on WBCT: 85% (33) subjects had rib fractures (mean of 8.5 fractures/subject); 31% (12) sternal fractures; 13% (5) mediastinal haematoma; 10% (4) pneumothorax; 8% (3) pneumomediastinum and 3% (1) haemothorax. Three subjects (8%) had abdominal injuries on WBCT, including one hepatic haematoma with active haemorrhage. CPR-related injuries on WBCT after ROSC are common, with serial rib fractures detected most commonly. An unexpectedly high rate of abdominal injuries was detected on SCA-WBCT. Radiologists need to be attuned to the spectrum of CPR-related injuries in WBCT, including abdominal injuries and subtle rib fractures. • CPR frequently causes injuries. • Radiologists should be aware of the spectrum of CPR related injuries. • Rib fractures are frequent and radiologic findings often subtle. • Clinically unexpected abdominal injuries may be present.

Hematological parameters in sick cell anemia patients with and without priapism

International Nuclear Information System (INIS)

Ahmed, Sagir G.; Ibrahim, Umma A.; Hassan, Abba W.

2006-01-01

Priapism was associated with certain hematological parameters in sickle cell anemia (SCA) patients in one report but not in another. We studied differences haematological parameters between SCA patients with and without priapism. Eighteen patients with SCA who presented with acute priapism during the years 2001-2004 were compared with age-and sex-matched SCA patients without priapism with respect to hematocrit, reticulocyte count, level of irreversibly sickled cells (ISC), percentage of haemoglobin (Hb F), total leukocyte and platelet counts. SCA patients with priapism had a mean hematocrit of 0.28 L/L, which was significantly higher than mean hematocrit value of 0.24 L/L (P<0.05) in patients without priapism. The mean reticulocyte count of 8% in patients with priapism was siginificantly lower than mean reticulocyte count of 12% (P<0.05) in patients without priapism. The level of ISC of 3% in patients with priapism was significantly lower than the level of 6.5% (P<0.05) in patients without priapism. There was no statistically significant difference in the mean levels of Hb F (7% vs. 6%). Patients with priapism had a mean leukocyte count that did not significantly differ from values in patients without priapism. SCA patients with priapism had a lower rate of hemolysis resulting in a higher hematocrit and greater blood viscosity, which increased the risk of corpora cavernosal sickling and blockade. Hence, a relatively higher hematocrit is risk factor for the development priapism in patients with sickle cell anemia. (author)

Operation of a P300-based brain-computer interface by patients with spinocerebellar ataxia

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Yoji Okahara

Full Text Available Objective: We investigated the efficacy of a P300-based brain-computer interface (BCI for patients with spinocerebellar ataxia (SCA, which is often accompanied by cerebellar impairment. Methods: Eight patients with SCA and eight age- and gender-matched healthy controls were instructed to input Japanese hiragana characters using the P300-based BCI with green/blue flicker. All patients depended on some assistance in their daily lives (modified Rankin scale: mean 3.5. The chief symptom was cerebellar ataxia; no cognitive deterioration was present. A region-based, two-step P300-based BCI was used. During the P300 task, eight-channel EEG data were recorded, and a linear discriminant analysis distinguished the target from other nontarget regions of the matrix. Results: The mean online accuracy in BCI operation was 82.9% for patients with SCA and 83.2% for controls; no significant difference was detected. Conclusion: The P300-based BCI was operated successfully not only by healthy controls but also by individuals with SCA. Significance: These results suggest that the P300-based BCI may be applicable for patients with SCA. Keywords: BCI, BMI, P300, Visual stimuli, Spinocerebellar ataxia

Sickle cell anemia induces changes in peripheral lymphocytes E-NTPDase/E-ADA activities and cytokines secretion in patients under treatment.

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Castilhos, Lívia G; Doleski, Pedro H; Bertoldo, Tatiana M D; Passos, Daniela F; Bertoncheli, Claudia de M; Rezer, João F P; Schlemmer, Josiane B; Leal, Daniela B R

2015-07-01

Sickle cell anemia (SCA) is characterized by hemoglobin polymerization that results in sickle-shaped red blood cells. The vascular obstruction by sickle erythrocytes is often inflammatory, and purinergic system ecto-enzymes play an important role in modulating the inflammatory and immune response. This study aimed to evaluate the E-NTPDase and E-ADA activities in lymphocytes of SCA treated patients, as well as verify the cytokine profile in this population. Fifteen SCA treated patients and 30 health subjects (control group) were selected. The peripheral lymphocytes were isolated and E-NTPDase and E-ADA activities were determined. Serum was separated from clot formation for the cytokines quantification. E-NTPDase (ATP and ADP as substrate) and E-ADA (adenosine as substrate) activities were increased in lymphocytes from SCA patients (PADA enzymes represent an important control of purine-mediated in the SCA disease, avoiding elevated adenosine levels in the extracellular medium and consequent organ injuries in these patients. The pro-inflammatory cytokines decreased levels by use of hydroxyurea occur in attempt to reduce the pro-inflammatory response and prevent vaso-oclusive crisis. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

Prevalence of spinocerebellar ataxia 36 in a US population.

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Valera, Juliana M; Diaz, Tatyana; Petty, Lauren E; Quintáns, Beatriz; Yáñez, Zuleima; Boerwinkle, Eric; Muzny, Donna; Akhmedov, Dmitry; Berdeaux, Rebecca; Sobrido, Maria J; Gibbs, Richard; Lupski, James R; Geschwind, Daniel H; Perlman, Susan; Below, Jennifer E; Fogel, Brent L

2017-08-01

To assess the prevalence and clinical features of individuals affected by spinocerebellar ataxia 36 (SCA36) at a large tertiary referral center in the United States. A total of 577 patients with undiagnosed sporadic or familial cerebellar ataxia comprehensively evaluated at a tertiary referral ataxia center were molecularly evaluated for SCA36. Repeat primed PCR and fragment analysis were used to screen for the presence of a repeat expansion in the NOP56 gene. Fragment analysis of triplet repeat primed PCR products identified a GGCCTG hexanucleotide repeat expansion in intron 1 of NOP56 in 4 index cases. These 4 SCA36-positive families comprised 2 distinct ethnic groups: white (European) (2) and Asian (Japanese [1] and Vietnamese [1]). Individuals affected by SCA36 exhibited typical clinical features with gait ataxia and age at onset ranging between 35 and 50 years. Patients also suffered from ataxic or spastic limbs, altered reflexes, abnormal ocular movement, and cognitive impairment. In a US population, SCA36 was observed to be a rare disorder, accounting for 0.7% (4/577 index cases) of disease in a large undiagnosed ataxia cohort.

The Deadlock Principle as a Ground for the Just and Equitable Winding Up of a Solvent Company: Thunder Cats Investments 92 (Pty Ltd v Nkonjane Economic Prospecting Investment (Pty Ltd 2014 5 SA 1 (SCA

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Tumo Charles Maloka

2016-05-01

Full Text Available The question addressed by the Supreme Court of Appeal in Thunder Cats Investment 92 (Pty Ltd v Nkonjane Economic Prospecting & Investments (Pty Ltd 2014 5 SA 1 (SCA (hereafter the "Thunder Cats" provides much-needed guidance on the deadlock principle as well as the breadth and scope of the "just and equitable ground for winding up in terms of s 81(1(d(iii of the Companies Act 71 of 2008. The facts, the issues and the contextual authority of Thunder Cats also bring to fore the lacuna in the just and equitable winding up provisions of the current Companies Act which lacuna has so far received no judicial or academic consideration. This Note contends the fact that the just and equitable winding up provisions do not countenance any deviation from the statutory prescriptions once the factual grounds for just and equitable winding up have been established is not in consonance with the spirit, purport and objects of Companies Act, and, in particular those of Chapter Six of the Act which have introduced the innovative business rescue scheme into South African corporate law landscape. The facts, the issues and the contextual authority of Thunder Cats will be reviewed at length in the ensuing discussion.

Improving interoperability through gateways and cots technologies

CSIR Research Space (South Africa)

Smith, C

2013-01-01

Full Text Available simultaneously, reducing dramatically the time to deploy of the nodes. 4.2 Success History In [7] [8] it’s shown how an out-of-the-box Android smartphone can be used as an information gateway hosting JTRS SCA based public safety waveforms: AM, FM and APCO-P25... and the smartphone. Thus, the entire SCA stack, including the CRC’s Core Framework [8] and the OIS’s CORBA middleware [9] was installed in the Android smartphone. The three SCA compliant waveforms were installed also in the phone. It’s worth to mention...

Improved Estimates of Clear Sky Longwave Flux and Application to the Tropical Greenhouse Effect

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Collins, W. D.

1997-01-01

The first objective of this investigation is to eliminate the clear-sky offset introduced by the scene-identification procedures developed for the Earth Radiation Budget Experiment (ERBE). Estimates of this systematic bias range from 10 to as high as 30 W/sq m. The initial version of the ScaRaB data is being processed with the original ERBE algorithm. Since the ERBE procedure for scene identification is based upon zonal flux averages, clear scenes with longwave emission well below the zonal mean value are mistakenly classified as cloudy. The erroneous classification is more frequent in regions with deep convection and enhanced mid- and upper-tropospheric humidity. We will develop scene identification parameters with zonal and/or time dependence to reduce or eliminate the bias in the clear- sky data. The modified scene identification procedure could be used for the ScaRaB-specific version of the Earth-radiation products. The second objective is to investigate changes in the clear-sky Outgoing Longwave Radiation (OLR) associated with decadal variations in the tropical and subtropical climate. There is considerable evidence for a shift in the climate state starting in approximately 1977. The shift is accompanied by higher SSTs in the equatorial Pacific, increased tropical convection, and higher values of atmospheric humidity. Other evidence indicates that the humidity in the tropical troposphere has been steadily increasing over the last 30 years. It is not known whether the atmospheric greenhouse effect has increased during this period in response to these changes in SST and precipitable water. We will investigate the decadal-scale fluctuations in the greenhouse effect using Nimbus-7, ERBE, and ScaRaB measurements spaning 1979 to the present. The data from the different satellites will be intercalibrated by comparison with model calculations based upon ship radiosonde observations. The fluxes calculated from the radiation model will also be used for validation of the

Sustainability cost accounting - Part 2: a case study in the South African process industry

CSIR Research Space (South Africa)

Brent, AC

2007-05-01

Full Text Available and assess the SCA procedure, which considers the construction and operation of a hypothetical Gas-to-Liquid (GTL) fuel manufacturing facility at a specific location in South Africa. The SCA indicators show that the negative environmental impacts associated...

The impact of serum potassium-influencing antihypertensive drugs on the risk of out-of-hospital cardiac arrest : A case–control study

NARCIS (Netherlands)

Alharbi, Fawaz F; Souverein, Patrick C.; de Groot, Mark C.H.; Blom, Marieke T.; de Boer, Anthonius; Klungel, Olaf H.; Tan, Hanno L.

2017-01-01

Aims: Sudden cardiac arrest (SCA) is a complex multifactorial event and most commonly caused by ventricular tachycardia/ fibrillation (VT/ VF). Some antihypertensive drugs could induce hypokalaemia or hyperkalaemia, which may increase susceptibility to VT/VF and SCA. Objective: To assess the

Lodhi 5 Properties Investments CC v FirstRand Bank Limited [2015 ...

African Journals Online (AJOL)

SCA) and the enforcement of Islamic Banking Law in South Africa. ... It also looks at the SCA's approach (as a common law court) with regard to the enforcement of the principles of Islamic banking law. The judgment raises important issues ...

Development of a pharmacokinetic-guided dose individualization strategy for hydroxyurea treatment in children with sickle cell anaemia.

Science.gov (United States)

Dong, Min; McGann, Patrick T; Mizuno, Tomoyuki; Ware, Russell E; Vinks, Alexander A

2016-04-01

Hydroxyurea has emerged as the primary disease-modifying therapy for patients with sickle cell anaemia (SCA). The laboratory and clinical benefits of hydroxyurea are optimal at maximum tolerated dose (MTD), but the current empirical dose escalation process often takes up to 12 months. The purpose of this study was to develop a pharmacokinetic-guided dosing strategy to reduce the time required to reach hydroxyurea MTD in children with SCA. Pharmacokinetic (PK) data from the HUSTLE trial (NCT00305175) were used to develop a population PK model using non-linear mixed effects modelling (nonmem 7.2). A D-optimal sampling strategy was developed to estimate individual PK and hydroxyurea exposure (area under the concentration-time curve (AUC)). The initial AUC target was derived from HUSTLE clinical data and defined as the mean AUC at MTD. PK profiles were best described by a one compartment with Michaelis-Menten elimination and a transit absorption model. Body weight and cystatin C were identified as significant predictors of hydroxyurea clearance. The following clinically feasible sampling times are included in a new prospective protocol: pre-dose (baseline), 15-20 min, 50-60 min and 3 h after an initial 20 mg kg(-1) oral dose. The mean target AUC(0,∞) for initial dose titration was 115 mg l(-1)  h. We developed a PK model-based individualized dosing strategy for the prospective Therapeutic Response Evaluation and Adherence Trial (TREAT, ClinicalTrials.gov NCT02286154). This approach has the potential to optimize the dose titration of hydroxyurea therapy for children with SCA, such that the clinical benefits at MTD are achieved more quickly. © 2015 The British Pharmacological Society.

Quantitative effects of composting state variables on C/N ratio through GA-aided multivariate analysis.

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Sun, Wei; Huang, Guo H; Zeng, Guangming; Qin, Xiaosheng; Yu, Hui

2011-03-01

It is widely known that variation of the C/N ratio is dependent on many state variables during composting processes. This study attempted to develop a genetic algorithm aided stepwise cluster analysis (GASCA) method to describe the nonlinear relationships between the selected state variables and the C/N ratio in food waste composting. The experimental data from six bench-scale composting reactors were used to demonstrate the applicability of GASCA. Within the GASCA framework, GA searched optimal sets of both specified state variables and SCA's internal parameters; SCA established statistical nonlinear relationships between state variables and the C/N ratio; to avoid unnecessary and time-consuming calculation, a proxy table was introduced to save around 70% computational efforts. The obtained GASCA cluster trees had smaller sizes and higher prediction accuracy than the conventional SCA trees. Based on the optimal GASCA tree, the effects of the GA-selected state variables on the C/N ratio were ranged in a descending order as: NH₄+-N concentration>Moisture content>Ash Content>Mean Temperature>Mesophilic bacteria biomass. Such a rank implied that the variation of ammonium nitrogen concentration, the associated temperature and the moisture conditions, the total loss of both organic matters and available mineral constituents, and the mesophilic bacteria activity, were critical factors affecting the C/N ratio during the investigated food waste composting. This first application of GASCA to composting modelling indicated that more direct search algorithms could be coupled with SCA or other multivariate analysis methods to analyze complicated relationships during composting and many other environmental processes. Copyright © 2010 Elsevier B.V. All rights reserved.

Atxn2 Knockout and CAG42-Knock-in Cerebellum Shows Similarly Dysregulated Expression in Calcium Homeostasis Pathway.

Science.gov (United States)

Halbach, Melanie Vanessa; Gispert, Suzana; Stehning, Tanja; Damrath, Ewa; Walter, Michael; Auburger, Georg

2017-02-01

Spinocerebellar ataxia type 2 (SCA2) is an autosomal dominantly inherited neurodegenerative disorder with preferential affection of Purkinje neurons, which are known as integrators of calcium currents. The expansion of a polyglutamine (polyQ) domain in the RNA-binding protein ataxin-2 (ATXN2) is responsible for this disease, but the causal roles of deficient ATXN2 functions versus aggregation toxicity are still under debate. Here, we studied mouse mutants with Atxn2 knockout (KO) regarding their cerebellar global transcriptome by microarray and RT-qPCR, in comparison with data from Atxn2-CAG42-knock-in (KIN) mouse cerebellum. Global expression downregulations involved lipid and growth signaling pathways in good agreement with previous data. As a novel effect, downregulations of key factors in calcium homeostasis pathways (the transcription factor Rora, transporters Itpr1 and Atp2a2, as well as regulator Inpp5a) were observed in the KO cerebellum, and some of them also occurred subtly early in KIN cerebellum. The ITPR1 protein levels were depleted from soluble fractions of cerebellum in both mutants, but accumulated in its membrane-associated form only in the SCA2 model. Coimmunoprecipitation demonstrated no association of ITPR1 with Q42-expanded or with wild-type ATXN2. These findings provide evidence that the physiological functions and protein interactions of ATXN2 are relevant for calcium-mediated excitation of Purkinje cells as well as for ATXN2-triggered neurotoxicity. These insights may help to understand pathogenesis and tissue specificity in SCA2 and other polyQ ataxias like SCA1, where inositol regulation of calcium flux and RORalpha play a role.

In vitro phenotypic correction of hematopoietic progenitors from Fanconi anemia group A knockout mice.

Science.gov (United States)

Río, Paula; Segovia, José Carlos; Hanenberg, Helmut; Casado, José Antonio; Martínez, Jesús; Göttsche, Kerstin; Cheng, Ngan Ching; Van de Vrugt, Henri J; Arwert, Fré; Joenje, Hans; Bueren, Juan A

2002-09-15

Fanconi anemia (FA) is a rare autosomal recessive disease, characterized by bone marrow failure and cancer predisposition. So far, 8 complementation groups have been identified, although mutations in FANCA account for the disease in the majority of FA patients. In this study we characterized the hematopoietic phenotype of a Fanca knockout mouse model and corrected the main phenotypic characteristics of the bone marrow (BM) progenitors using retroviral vectors. The hematopoiesis of these animals was characterized by a modest though significant thrombocytopenia, consistent with reduced numbers of BM megakaryocyte progenitors. As observed in other FA models, the hematopoietic progenitors from Fanca(-/-) mice were highly sensitive to mitomycin C (MMC). In addition, we observed for the first time in a FA mouse model a marked in vitro growth defect of Fanca(-/-) progenitors, either when total BM or when purified Lin(-)Sca-1(+) cells were subjected to in vitro stimulation. Liquid cultures of Fanca(-/-) BM that were stimulated with stem cell factor plus interleukin-11 produced low numbers of granulocyte macrophage colony-forming units, contained a high proportion of apoptotic cells, and generated a decreased proportion of granulocyte versus macrophage cells, compared to normal BM cultures. Aiming to correct the phenotype of Fanca(-/-) progenitors, purified Lin(-)Sca-1(+) cells were transduced with retroviral vectors encoding the enhanced green fluorescent protein (EGFP) gene and human FANCA genes. Lin(-)Sca-1(+) cells from Fanca(-/-) mice were transduced with an efficiency similar to that of samples from wild-type mice. More significantly, transductions with FANCA vectors corrected both the MMC hypersensitivity as well as the impaired ex vivo expansion ability that characterized the BM progenitors of Fanca(-/-) mice.

77 FR 2606 - Unblocking of Specially Designated Nationals and Blocked Persons Pursuant to Executive Order 12978

Science.gov (United States)

2012-01-18

... OVERSEAS INC., Panama City, Panama; c/o FINANZAS DEL NORTE LUIS SAIEH Y CIA. S.C.A., Barranquilla, Colombia.... FINANZAS DEL NORTE LUIS SAIEH Y CIA. S.C.A. (f.k.a. FINANZAS DEL NORTE LTDA.), Calle 77 B No. 57-141, Ofc...

Genetics Home Reference: spinocerebellar ataxia type 3

Science.gov (United States)

... time, individuals with SCA3 may develop loss of sensation and weakness in the limbs (peripheral neuropathy), muscle cramps, muscle twitches (fasciculations), and swallowing difficulties. Individuals with SCA3 may have problems with memory, planning, and problem solving. Signs and symptoms of ...

Blood pressure indices and disease severity in patients with sickle ...

African Journals Online (AJOL)

Background: Individuals with sickle cell anaemia (SCA) have lower systemic blood pressures compared to individuals with haemoglobin Hb AA phenotype. Objective: To evaluate blood pressure indices of individuals with SCA in steady state, in comparison with haematological and clinical markers of disease severity.

The impact of serum potassium-influencing antihypertensive drugs on the risk of out-of-hospital cardiac arrest: A case-control study

NARCIS (Netherlands)

Alharbi, Fawaz F.; Souverein, Patrick C.; de Groot, Mark C. H.; Blom, Marieke T.; de Boer, Anthonius; Klungel, Olaf H.; Tan, Hanno L.

2017-01-01

AimsSudden cardiac arrest (SCA) is a complex multifactorial event and most commonly caused by ventricular tachycardia/ fibrillation (VT/ VF). Some antihypertensive drugs could induce hypokalaemia or hyperkalaemia, which may increase susceptibility to VT/VF and SCA. ObjectiveTo assess the association

Importance of methodological standardization for the ektacytometric measures of red blood cell deformability in sickle cell anemia

NARCIS (Netherlands)

Renoux, Céline; Parrow, Nermi; Faes, Camille; Joly, Philippe; Hardeman, Max; Tisdale, John; Levine, Mark; Garnier, Nathalie; Bertrand, Yves; Kebaili, Kamila; Cuzzubbo, Daniela; Cannas, Giovanna; Martin, Cyril; Connes, Philippe

2016-01-01

Red blood cell (RBC) deformability is severely decreased in patients with sickle cell anemia (SCA), which plays a role in the pathophysiology of the disease. However, investigation of RBC deformability from SCA patients demands careful methodological considerations. We assessed RBC deformability by

Safety critical FPGA-based NPP instrumentation and control systems: assessment, development and implementation

Energy Technology Data Exchange (ETDEWEB)

Bakhmach, E. S.; Siora, A. A.; Tokarev, V. I. [Research and Production Corporation Radiy, 29 Geroev Stalingrada Str., Kirovograd 25006 (Ukraine); Kharchenko, V. S.; Sklyar, V. V.; Andrashov, A. A., E-mail: marketing@radiy.co [Center for Safety Infrastructure-Oriented Research and Analysis, 37 Astronomicheskaya Str., Kharkiv 61085 (Ukraine)

2010-10-15

The stages of development, production, verification, licensing and implementation methods and technologies of safety critical instrumentation and control systems for nuclear power plants (NPP) based on FPGA (Field Programmable Gates Arrays) technologies are described. A life cycle model and multi-version technologies of dependability and safety assurance of FPGA-based instrumentation and control systems are discussed. An analysis of NPP instrumentation and control systems construction principles developed by Research and Production Corporation Radiy using FPGA-technologies and results of these systems implementation and operation at Ukrainian and Bulgarian NPP are presented. The RADIY{sup TM} platform has been designed and developed by Research and Production Corporation Radiy, Ukraine. The main peculiarity of the RADIY{sup TM} platform is the use of FPGA as programmable components for logic control operation. The FPGA-based RADIY{sup TM} platform used for NPP instrumentation and control systems development ensures sca lability of system functions types, volume and peculiarities (by changing quantity and quality of sensors, actuators, input/output signals and control algorithms); sca lability of dependability (safety integrity) (by changing a number of redundant channel, tiers, diagnostic and reconfiguration procedures); sca lability of diversity (by changing types, depth and method of diversity selection). (Author)

Gestão do Conhecimento – GC – como recurso estratégico e fonte de Vantagem Competitiva Sustentável – VCS – na perspectiva Resource-based view – RBV – Knowledge Management – KM – used as a strategic resource and source of Sustainable Competitive Advantage – SCA – in the Resource-based view – RBV –

Directory of Open Access Journals (Sweden)

Virginia Aparecida Castro

2012-06-01

Full Text Available A Gestão do Conhecimento – GC – busca integrar toda a organização ao conhecimento que pode ser criado, compartilhado e armazenado. Desta forma, este trabalho tem por objetivo evidenciar de que maneira a GC é utilizada como recurso estratégico e fonte de Vantagem Competitiva Sustentável – VCS – na perspectiva Resource based view – RBV –. É feito um levantamento na forma de censo junto aos alunos das turmas de pós-graduação lato sensu (MBA da FAGEN – Faculdade de Gestão e Negócios – da UFU – Universidade Federal de Uberlândia –, os quais atuam no mercado como gestores e possuem pelo menos um subordinado sob a sua liderança. Após uma revisão da literatura sobre GC e VCS estruturou-se o instrumento de pesquisa a partir do Diagnóstico de Gestão do Conhecimento criado por Bukowitz e Williams (2002 e do Framework VRIO proposto por Barney (1991,1995. Ao considerar o critério de desempenho global de 55%, o cálculo da média aponta um percentual de 70%. Porém, a GC é utilizada em um nível preliminar, pois nota-se que os gestores necessitam, além de reconhecerem a importância da GC, trabalharem para a sua efetiva implantação. Assim, a GC, alinhada ao plano estratégico global da empresa, poderá ser utilizada como recurso estratégico e fonte de VCS na perspectiva RBV.The Knowledge Management – KM – searches to integrate organization to the knowledge that can be created, shared and stored. This study aims to show how the KM is used as a strategic resource and source of Sustainable Competitive Advantage – SCA – in the Resource based view – RBV – perspective. We made a survey in the form of census with the students of  Master Business Administration – MBA of FAGEN – Faculdade de Gestão e Negócios – da UFU – Universidade Federal de Uberlândia –. This students are managers and have employees with your leadership. After a review of the literature on KM and SCA, we have structured the

Endovascular Management of Fusiform Superior Cerebellar Artery Aneurysms: A Series of Three Cases with Review of Literature

Directory of Open Access Journals (Sweden)

Anand Alurkar

2012-01-01

Full Text Available Distal superior cerebellar artery (SCA aneurysms are rare. Fusiform aneurysms of SCA are rarer and more challenging to treat. Parent artery occlusion by endovascular coiling is the treatment option for these cases. Presence of good collateral circulation and paucity of perforators from S1 and S2 segments makes this a feasible option. From 2007 to 2010, we treated three patients (two men and one woman between the ages of 42 to 64 years with distal fusiform SCA aneurysms using endovascular coiling. All the patients presented with symptoms of rupture and were treated in the acute phase. Informed and written high-risk consent was given by all patients prior to the procedure. Successful angiographic and clinical outcome was achieved in all three patients. Endovascular treatment of fusiform SCA aneurysms with coils is a safe and feasible option in the management of this rare entity.

Hydroxyurea Therapy for Children With Sickle Cell Anemia in Sub-Saharan Africa: Rationale and Design of the REACH Trial.

Science.gov (United States)

McGann, Patrick T; Tshilolo, Léon; Santos, Brigida; Tomlinson, George A; Stuber, Susan; Latham, Teresa; Aygun, Banu; Obaro, Stephen K; Olupot-Olupot, Peter; Williams, Thomas N; Odame, Isaac; Ware, Russell E

2016-01-01

Sickle cell anemia (SCA) is an inherited hematological disorder that causes a large but neglected global health burden, particularly in Africa. Hydroxyurea represents the only available disease-modifying therapy for SCA, and has proven safety and efficacy in high-resource countries. In sub-Saharan Africa, there is minimal use of hydroxyurea, due to lack of data, absence of evidence-based guidelines, and inexperience among healthcare providers. A partnership was established between investigators in North America and sub-Saharan Africa, to develop a prospective multicenter research protocol designed to provide data on the safety, feasibility, and benefits of hydroxyurea for children with SCA. The Realizing Effectiveness Across Continents with Hydroxyurea (REACH, ClinicalTrials.gov NCT01966731) trial is a prospective, phase I/II open-label dose escalation study of hydroxyurea that will treat a total of 600 children age 1-10 years with SCA: 150 at each of four different clinical sites within sub-Saharan Africa (Angola, Democratic Republic of Congo, Kenya, and Uganda). The primary study endpoint will be severe hematological toxicities that occur during the fixed-dose treatment phase. REACH has an adaptive statistical design that allows for careful assessment of toxicities to accurately identify a safe hydroxyurea dose. REACH will provide data that address critical gaps in knowledge for the treatment of SCA in sub-Saharan Africa. By developing local expertise with the use of hydroxyurea and helping to establish treatment guidelines, the REACH trial results will have the potential to transform care for children with SCA in Africa. © 2015 The Authors. Pediatric Blood & Cancer Published by Wiley Periodicals, Inc.

Determinants of academic performance in children with sickle cell anaemia.

Science.gov (United States)

Ezenwosu, Osita U; Emodi, Ifeoma J; Ikefuna, Anthony N; Chukwu, Barth F; Osuorah, Chidiebere D

2013-11-19

Some factors are known to influence the academic performance of children with Sickle Cell Anaemia (SCA). Information on their effects in these children is limited in Nigeria. The factors which influence academic performance of children with SCA in Enugu, Nigeria are determined in this study. Consecutive children with SCA aged 5-11 years were recruited at the weekly sickle cell clinic of the University of Nigeria Teaching Hospital (UNTH) Enugu, Nigeria. Their age- and sex- matched normal classmates were recruited as controls. The total number of days of school absence for 2009/2010 academic session was obtained for each pair of pupils from the class attendance register. Academic performance was assessed using the average of the overall scores in the three term examinations of same session. Intelligence ability was determined with Draw-A-Person Quotient (DAPQ) using the Draw-A-Person Test while socio-economic status was determined using the occupational status and educational attainment of each parent. Academic performance of children with SCA showed statistically significant association with their socio-economic status (χ2 = 9.626, p = 0.047), and significant correlation with DAPQ (r = 0.394, p = 0.000) and age (r = -0.412, p = 0.000). However, no significant relationship existed between academic performance and school absence in children with SCA (r = -0.080, p = 0.453). Academic performance of children with SCA is influenced by their intelligence ability, age and socio-economic status but not negatively affected by their increased school absenteeism.

Serum levels of copeptin, C-reactive protein and cortisol in different ...

African Journals Online (AJOL)

olayemitoyin

clinical status of an SCA patients from steady state to crisis (Ahmed ... from the Haematology Day Care Unit, Department of ... necessitates hospital admission were clinically .... Despite intensive research on sickle cell anaemia, ... increased cardiac workload, increased protein ... cerebral infarction in subjects with SCA. Our.

Only a minority of sex chromosome abnormalities are detected by a national prenatal screening program for Down syndrome

DEFF Research Database (Denmark)

Viuff, Mette Hansen; Krag, Kirstine Stochholm; Uldbjerg, Niels

2015-01-01

significantly higher NT and lower PAPP-A compared with controls (all P legal abortion rate was high for all four syndromes (47,XXX: 24%; 47,XYY: 29%; Klinefelter syndrome: 48%, TS: 84%). For SCA fetuses carried to term, only TS fetuses had consistently lower birthweights...... and placenta weights than non-SCA controls (both P = 0.0001). A few SCA cases localized in DCCR could not be found in DFMD (n = 16). LIMITATIONS, REASON FOR CAUTION: Controls were matched on sex of the fetus of cases, meaning that all electively aborted fetuses (before week 12) were excluded, possibly reducing...

Small Aircraft Transportation System Higher Volume Operations Concept

Science.gov (United States)

Abbott, Terence S.; Consiglio, Maria C.; Baxley, Brian T.; Williams, Daniel M.; Jones, Kenneth M.; Adams, Catherine A.

2006-01-01

This document defines the Small Aircraft Transportation System (SATS) Higher Volume Operations concept. The general philosophy underlying this concept is the establishment of a newly defined area of flight operations called a Self-Controlled Area (SCA). Within the SCA, pilots would take responsibility for separation assurance between their aircraft and other similarly equipped aircraft. This document also provides details for a number of off-nominal and emergency procedures which address situations that could be expected to occur in a future SCA. The details for this operational concept along with a description of candidate aircraft systems to support this concept are provided.

Ruminococcus flavefaciens 007C cellulosomes and cellulase consortium

Directory of Open Access Journals (Sweden)

Maša VODOVNIK

2015-11-01

Full Text Available Ruminococcus flavefaciens is among the most important cellulolytic bacterial species in rumen and gastrointestinal tract of monogastric herbivorous animals. Its efficiency in degradation of (hemicellulosic substrates is associated with the production of remarkably intricate extracellular multienzyme complexes, named cellulosomes. In the present work we investigated the cellulolytic system of 007C. The bioinformatic analysis of the draft genome sequence revealed identical organization of sca gene cluster as has previously been found in four other strains of R. flavefaciens. The cluster consists of five genes in the following order: scaC-scaA-scaB-cttA-scaE. The cellulases of R. flavefaciens 007C belong to four families of glycoside hydrolases, namely GH48, GH44, GH9 in GH5. Majority of these enzymes are putative endoglucanases, belonging to families GH5 and GH9, whereas only one gene encoding GH44 and GH48 was found. Apart from catalytic domains, most of these proteins also contain dockerins – signature sequences, which indicate their attachement to cellulosomes. On the other hand, carbohydrate-binding modules were only found coupled to GH9 catalytic domains. Zymogram analysis showed that larger endoglucanases were mostly constitutively expressed, wheras smaller enzymes were only detected in later phases of Avicel-grown cultures.

Progression of brain atrophy in spinocerebellar ataxia type 2: a longitudinal tensor-based morphometry study.

Science.gov (United States)

Mascalchi, Mario; Diciotti, Stefano; Giannelli, Marco; Ginestroni, Andrea; Soricelli, Andrea; Nicolai, Emanuele; Aiello, Marco; Tessa, Carlo; Galli, Lucia; Dotti, Maria Teresa; Piacentini, Silvia; Salvatore, Elena; Toschi, Nicola

2014-01-01

Spinocerebellar ataxia type 2 (SCA2) is the second most frequent autosomal dominant inherited ataxia worldwide. We investigated the capability of magnetic resonance imaging (MRI) to track in vivo progression of brain atrophy in SCA2 by examining twice 10 SCA2 patients (mean interval 3.6 years) and 16 age- and gender-matched healthy controls (mean interval 3.3 years) on the same 1.5 T MRI scanner. We used T1-weighted images and tensor-based morphometry (TBM) to investigate volume changes and the Inherited Ataxia Clinical Rating Scale to assess the clinical deficit. With respect to controls, SCA2 patients showed significant higher atrophy rates in the midbrain, including substantia nigra, basis pontis, middle cerebellar peduncles and posterior medulla corresponding to the gracilis and cuneatus tracts and nuclei, cerebellar white matter (WM) and cortical gray matter (GM) in the inferior portions of the cerebellar hemisphers. No differences in WM or GM volume loss were observed in the supratentorial compartment. TBM findings did not correlate with modifications of the neurological deficit. In conclusion, MRI volumetry using TBM is capable of demonstrating the progression of pontocerebellar atrophy in SCA2, supporting a possible role of MRI as biomarker in future trials.

Different roles of the Mre11 complex in the DNA damage response in Aspergillus nidulans.

Science.gov (United States)

Semighini, Camile P; von Zeska Kress Fagundes, Márcia Regina; Ferreira, Joseane Cristina; Pascon, Renata Castiglioni; de Souza Goldman, Maria Helena; Goldman, Gustavo Henrique

2003-06-01

The Mre11-Rad50-Nbs1 protein complex has emerged as a central player in the cellular DNA damage response. Mutations in scaANBS1, which encodes the apparent homologue of human Nbs1 in Aspergillus nidulans, inhibit growth in the presence of the anti-topoisomerase I drug camptothecin. We have used the scaANBS1 cDNA as a bait in a yeast two-hybrid screening and report the identification of the A. nidulans Mre11 homologue (mreA). The inactivated mreA strain was more sensitive to several DNA damaging and oxidative stress agents. Septation in A. nidulans is dependent not only on the uvsBATR gene, but also on the mre11 complex. scaANBS1 and mreA genes are both involved in the DNA replication checkpoint whereas mreA is specifically involved in the intra-S-phase checkpoint. ScaANBS1 also participates in G2-M checkpoint control upon DNA damage caused by MMS. In addition, the scaANBS1 gene is also important for ascospore viability, whereas mreA is required for successful meiosis in A. nidulans. Consistent with this view, the Mre11 complex and the uvsCRAD51 gene are highly expressed at the mRNA level during the sexual development.

Determining the proportion of coronary segments assessable on 16-slice CT coronary angiography: a brief report

International Nuclear Information System (INIS)

Soon, K. H.; Cox, N.; Eccleston, D.; Lim, Y.; Chaitowitz, I.; Bell, K. W.; Kelly, A-M.

2007-01-01

Computed tomography coronary angiography (CT-CA) is becoming a popular non-invasive coronary imaging method. We aimed to determine the proportion of coronary segments assessable on a 16-slice CT in comparison with conventional selective coronary angiography (SCA). We identified all patients who had both 16-slice CT-CA and recent SCA (less than 12 months) from March 2004 to July 2005. Two CT reporters blinded to SCA independently classified coronary segment assessability on CT-CA. A cardiologist blinded to CT findings classified assess-ability of coronary segments on SCA. Data were analysed using descriptive statistics and proportion of agreement. Ninety-five study pairs were included in the analysis. Of those, 1161 coronary segments were deemed assessable on SCA and 1103 segments (95%) were also assessable on CT-CA. Nonassessable segments on CT-CA were predominantly in the distal segments and branches of coronary arteries. Reasons for nonassessability were small calibre (48.3%), motion artefacts (20.7%) and poorly reconstructed segments (22.4%). The 16-slice CT was able to assess a high proportion of but not all coronary segments. Nonassessable segments were predominantly distal segments or branches of coronary arteries. Motion artefacts due to heart-rate changes, small calibre and poorly reconstructed images were main causes of nonassessability on 16-slice CT-CA

Progression of brain atrophy in spinocerebellar ataxia type 2: a longitudinal tensor-based morphometry study.

Directory of Open Access Journals (Sweden)

Mario Mascalchi

Full Text Available Spinocerebellar ataxia type 2 (SCA2 is the second most frequent autosomal dominant inherited ataxia worldwide. We investigated the capability of magnetic resonance imaging (MRI to track in vivo progression of brain atrophy in SCA2 by examining twice 10 SCA2 patients (mean interval 3.6 years and 16 age- and gender-matched healthy controls (mean interval 3.3 years on the same 1.5 T MRI scanner. We used T1-weighted images and tensor-based morphometry (TBM to investigate volume changes and the Inherited Ataxia Clinical Rating Scale to assess the clinical deficit. With respect to controls, SCA2 patients showed significant higher atrophy rates in the midbrain, including substantia nigra, basis pontis, middle cerebellar peduncles and posterior medulla corresponding to the gracilis and cuneatus tracts and nuclei, cerebellar white matter (WM and cortical gray matter (GM in the inferior portions of the cerebellar hemisphers. No differences in WM or GM volume loss were observed in the supratentorial compartment. TBM findings did not correlate with modifications of the neurological deficit. In conclusion, MRI volumetry using TBM is capable of demonstrating the progression of pontocerebellar atrophy in SCA2, supporting a possible role of MRI as biomarker in future trials.

Snow cover dynamics in Andean watersheds of Chile (32.0-39.5° S) during the years 2000-2016

Science.gov (United States)

Stehr, Alejandra; Aguayo, Mauricio

2017-10-01

Andean watersheds present important snowfall accumulation mainly during the winter, which melts during the spring and part of the summer. The effect of snowmelt on the water balance can be critical to sustain agriculture activities, hydropower generation, urban water supplies and wildlife. In Chile, 25 % of the territory between the region of Valparaiso and Araucanía comprises areas where snow precipitation occurs. As in many other difficult-to-access regions of the world, there is a lack of hydrological data of the Chilean Andes related to discharge, snow courses, and snow depths, which complicates the analysis of important hydrological processes (e.g. water availability). Remote sensing provides a promising opportunity to enhance the assessment and monitoring of the spatial and temporal variability of snow characteristics, such as the snow cover area (SCA) and snow cover dynamic (SCD). With regards to the foregoing questions, the objective of the study is to evaluate the spatiotemporal dynamics of the SCA at five watersheds (Aconcagua, Rapel, Maule, Biobío and Toltén) located in the Chilean Andes, between latitude 32.0 and 39.5° S, and to analyse its relationship with the precipitation regime/pattern and El Niño-Southern Oscillation (ENSO) events. Those watersheds were chosen because of their importance in terms of their number of inhabitants, and economic activities depending on water resources. The SCA area was obtained from MOD10A2 for the period 2000-2016, and the SCD was analysed through a number of statistical tests to explore observed trends. In order to verify the SCA for trend analysis, a validation of the MOD10A2 product was done, consisting of the comparison of snow presence predicted by MODIS with ground observations. Results indicate that there is an overall agreement of 81 to 98 % between SCA determined from ground observations and MOD10A2, showing that the MODIS snow product can be taken as a feasible remote sensing tool for SCA estimation in

Snow cover dynamics in Andean watersheds of Chile (32.0–39.5° S during the years 2000–2016

Directory of Open Access Journals (Sweden)

A. Stehr

2017-10-01

Full Text Available Andean watersheds present important snowfall accumulation mainly during the winter, which melts during the spring and part of the summer. The effect of snowmelt on the water balance can be critical to sustain agriculture activities, hydropower generation, urban water supplies and wildlife. In Chile, 25 % of the territory between the region of Valparaiso and Araucanía comprises areas where snow precipitation occurs. As in many other difficult-to-access regions of the world, there is a lack of hydrological data of the Chilean Andes related to discharge, snow courses, and snow depths, which complicates the analysis of important hydrological processes (e.g. water availability. Remote sensing provides a promising opportunity to enhance the assessment and monitoring of the spatial and temporal variability of snow characteristics, such as the snow cover area (SCA and snow cover dynamic (SCD. With regards to the foregoing questions, the objective of the study is to evaluate the spatiotemporal dynamics of the SCA at five watersheds (Aconcagua, Rapel, Maule, Biobío and Toltén located in the Chilean Andes, between latitude 32.0 and 39.5° S, and to analyse its relationship with the precipitation regime/pattern and El Niño–Southern Oscillation (ENSO events. Those watersheds were chosen because of their importance in terms of their number of inhabitants, and economic activities depending on water resources. The SCA area was obtained from MOD10A2 for the period 2000–2016, and the SCD was analysed through a number of statistical tests to explore observed trends. In order to verify the SCA for trend analysis, a validation of the MOD10A2 product was done, consisting of the comparison of snow presence predicted by MODIS with ground observations. Results indicate that there is an overall agreement of 81 to 98 % between SCA determined from ground observations and MOD10A2, showing that the MODIS snow product can be taken as a feasible remote sensing

Flash-flood potential assessment and mapping by integrating the weights-of-evidence and frequency ratio statistical methods in GIS environment - case study: Bâsca Chiojdului River catchment (Romania)

Science.gov (United States)

Costache, Romulus; Zaharia, Liliana

2017-06-01

Given the significant worldwide human and economic losses caused due to floods annually, reducing the negative consequences of these hazards is a major concern in development strategies at different spatial scales. A basic step in flood risk management is identifying areas susceptible to flood occurrences. This paper proposes a methodology allowing the identification of areas with high potential of accelerated surface run-off and consequently, of flash-flood occurrences. The methodology involves assessment and mapping in GIS environment of flash flood potential index (FFPI), by integrating two statistical methods: frequency ratio and weights-of-evidence. The methodology was applied for Bâsca Chiojdului River catchment (340 km2), located in the Carpathians Curvature region (Romania). Firstly, the areas with torrential phenomena were identified and the main factors controlling the surface run-off were selected (in this study nine geographical factors were considered). Based on the features of the considered factors, many classes were set for each of them. In the next step, the weights of each class/category of the considered factors were determined, by identifying their spatial relationships with the presence or absence of torrential phenomena. Finally, the weights for each class/category of geographical factors were summarized in GIS, resulting the FFPI values for each of the two statistical methods. These values were divided into five classes of intensity and were mapped. The final results were used to estimate the flash-flood potential and also to identify the most susceptible areas to this phenomenon. Thus, the high and very high values of FFPI characterize more than one-third of the study catchment. The result validation was performed by (i) quantifying the rate of the number of pixels corresponding to the torrential phenomena considered for the study (training area) and for the results' testing (validating area) and (ii) plotting the ROC (receiver operating

Induced pluripotent stem cell - derived neurons for the study of spinocerebellar ataxia type 3

DEFF Research Database (Denmark)

Hansen, Susanne Kofoed; Stummann, Tina C.; Madsen, Helena Borland

2016-01-01

The neurodegenerative disease spinocerebellar ataxia type 3 (SCA3) is caused by a CAG-repeat expansion in the ATXN3 gene. In this study, induced pluripotent stem cell (iPSC) lines were established from two SCA3 patients. Dermal fibroblasts were reprogrammed using an integration-free method...

Clonal structure of Staphylococcus aureus colonizing children with sickle cell anaemia and healthy controls

NARCIS (Netherlands)

Schaumburg, F.; Biallas, B.; Alabi, A. S.; Grobusch, M. P.; Feugap, E. N.; Lell, B.; Mellmann, A.; Peters, G.; Kremsner, P. G.; Becker, K.; Adegnika, A. A.

2013-01-01

Children with sickle cell anaemia (SCA) might carry hospital-associated bacterial lineages due to frequent hospital stays and antibiotic treatments. In this study we compared Staphylococcus aureus from SCA patients (n=73) and healthy children (n=143) in a cross-sectional study in Gabon. S. aureus

Circumstances and causes of out-of-hospital cardiac arrest in sudden death survivors

NARCIS (Netherlands)

de Vreede-Swagemakers, J. J.; Gorgels, A. P.; Dubois-Arbouw, W. I.; Dalstra, J.; Daemen, M. J.; van Ree, J. W.; Stijns, R. E.; Wellens, H. J.

1998-01-01

To study the circumstances and medical profile of out-of-hospital sudden cardiac arrest (SCA) patients in whom resuscitation was attempted by the ambulance service, and to identify causes of SCA in survivors and factors that influence resuscitation success rate. During a five year period (1991-95)

Sudden Cardiac Arrest

Science.gov (United States)

... increases the risk of arrhythmia and SCA. Physical stress, which can cause the heart’s electrical system to stop working. In people who already have heart problems, intense physical activity or exercise can lead to SCA because the release of the hormone adrenalin acts as a trigger ...

Language Impairment in Cerebellar Ataxia

NARCIS (Netherlands)

van Gaalen, Judith; de Swart, Bert J. M.; Oostveen, Judith; Knuijt, Simone; van de Warrenburg, Bart P. C.; Kremer, Berry (H. ) P. H.

Background: Several studies have suggested that language impairment can be observed in patients with cerebellar pathology. The aim of this study was to investigate language performance in patients with spinocerebellar ataxia type 6 (SCA6). Methods: We assessed speech and language in 29 SCA6 patients

Linguistic Polyphony

DEFF Research Database (Denmark)

Nølke, Henning

on the Scandinavian variant of polyphony, ScaPoLine. ScaPoLine is a formal linguistic theory whose main purpose is to specify the instructions conveyed through linguistic form for the creation of polyphonic meaning. The theoretical introduction is followed by polyphonic analyses of linguistic phenomena...

The prevalence and factors associated with musculoskeletal ...

African Journals Online (AJOL)

Background: Sickle cell anaemia (SCA) is a condition characterized by a predominance of haemoglobin S (hbss) in the red blood cells. Musculoskeletal involvement is one of the commonest clinical manifestations of SCA. There is limited information on the prevalence, patterns and factors associated with Musculoskeletal ...

Depression as the Primary Cause of Insomnia and Excessive Daytime Sleepiness in a Family with Multiple Cases of Spinocerebellar Ataxia.

Science.gov (United States)

Hsu, Chun-Hsien; Chen, Yen-Lin; Pei, Dee; Yu, Shu-Man; Liu, I-Chao

2016-07-15

Spinocerebellar ataxia (SCA) is a hereditary disease characterized by central nervous system-related motor dysfunctions. Sleep disorders and frequent non-motor manifestations are commonly comorbid with SCA. To elucidate this relationship, we present three cases in a family that included multiple SCA type 2 patients with various sleep disorders. Complete physical examination, and genetic and imaging studies were performed. Anti-parkinsonism medications were prescribed after neurological examination. Clonazepam and/or quetiapine were administered for sleep disorders but failed to resolve insomnia and excessive daytime sleepiness (EDS). Based on DSM-5 criteria, all cases were diagnosed with depression. After treatment with serotonin-norepinephrine reuptake inhibitors and noradrenergic and specific serotonergic antidepressants, symptoms of insomnia and EDS, which are strongly associated with depression in SCA type 2 patients, improved significantly. It is crucial to recognize insomnia and EDS in neurodegenerative diseases, not only for earlier diagnosis, but also to improve quality of life. © 2016 American Academy of Sleep Medicine.

Influence of lime juice on the severity of sickle cell anemia.

Science.gov (United States)

Adegoke, Samuel Ademola; Shehu, Umar Abdullahi; Mohammed, Lasisi Oluwafemi; Sanusi, Yunusa; Oyelami, Oyeku Akibu

2013-06-01

The pain in sickle cell anemia (SCA) is often triggered by dehydration, acidosis, and fever that are usually due to malaria. Intake of lime juice was recently demonstrated to facilitate clearance of the malaria parasite. It was therefore sought to determine whether regular intake of lime juice will ameliorate crisis, especially recurrent bone pain. In this preliminary, open-labeled, randomized study, the effects of lime juice on the clinical and some laboratory characteristics of children with SCA were tested. Among the 113 children with SCA studied in two hospitals, the 58 receiving lime treatment had lower rates of significant painful episodes than the 55 without lime (37 versus 83 crises in 6 months, and 0.64±0.11 versus 1.51±0.34 average rates per child, plime did not cause any significant side-effect. Regular intake of lime juice may be of great therapeutic and nutritional relevance in children with SCA.

Identification of IFRD1 variant in a Han Chinese family with autosomal dominant hereditary spastic paraplegia associated with peripheral neuropathy and ataxia.

Science.gov (United States)

Lin, Pengfei; Zhang, Dong; Xu, Guangrun; Yan, Chuanzhu

2018-04-01

Spinocerebellar ataxias (SCAs) are a group of autosomal dominant, clinically heterogeneous neurodegenerative disorders. SCA18 is a rare autosomal dominant sensory/motor neuropathy with ataxia (OMIM#607458) associated with a single missense variant c.514 A>G in the interferon related developmental regulator 1 (IFRD1) gene previously reported in a five-generation American family of Irish origin. However, to date, there have been no other reports of the IFRD1 mutation to confirm its role in SCA. Here, we report a Han Chinese family with SCA18; the family members presented with a slowly progressing gait ataxia, pyramidal tract signs, and peripheral neuropathy. We identified a missense variant (c.514 A>G, p.I172V) in IFRD1 gene in the family using targeted next-generation sequencing and Sanger direct sequencing with specific primers. Our results suggest that the IFRD1 gene may be the causative allele for SCA18.

Architecture of a general purpose embedded Slow-Control Adapter ASIC for future high-energy physics experiments

International Nuclear Information System (INIS)

Gabrielli, Alessandro; Loddo, Flavio; Ranieri, Antonio; De Robertis, Giuseppe

2008-01-01

This work is aimed at defining the architecture of a new digital ASIC, namely Slow-Control Adapter (SCA), which will be designed in a commercial 130-nm CMOS technology. This chip will be embedded within a high-speed data acquisition optical link (GBT) to control and monitor the front-end electronics in future high-energy physics experiments. The GBT link provides a transparent transport layer between the SCA and control electronics in the counting room. The proposed SCA supports a variety of common bus protocols to interface with end-user general-purpose electronics. Between the GBT and the SCA a standard 100 Mb/s IEEE-802.3 compatible protocol will be implemented. This standard protocol allows off-line tests of the prototypes using commercial components that support the same standard. The project is justified because embedded applications in modern large HEP experiments require particular care to assure the lowest possible power consumption, still offering the highest reliability demanded by very large particle detectors.

Architecture of a general purpose embedded Slow-Control Adapter ASIC for future high-energy physics experiments

Science.gov (United States)

Gabrielli, Alessandro; Loddo, Flavio; Ranieri, Antonio; De Robertis, Giuseppe

2008-10-01

This work is aimed at defining the architecture of a new digital ASIC, namely Slow-Control Adapter (SCA), which will be designed in a commercial 130-nm CMOS technology. This chip will be embedded within a high-speed data acquisition optical link (GBT) to control and monitor the front-end electronics in future high-energy physics experiments. The GBT link provides a transparent transport layer between the SCA and control electronics in the counting room. The proposed SCA supports a variety of common bus protocols to interface with end-user general-purpose electronics. Between the GBT and the SCA a standard 100 Mb/s IEEE-802.3 compatible protocol will be implemented. This standard protocol allows off-line tests of the prototypes using commercial components that support the same standard. The project is justified because embedded applications in modern large HEP experiments require particular care to assure the lowest possible power consumption, still offering the highest reliability demanded by very large particle detectors.

Yeast Sgf73/Ataxin-7 serves to anchor the deubiquitination module into both SAGA and Slik(SALSA HAT complexes

Directory of Open Access Journals (Sweden)

Lee Kenneth K

2009-02-01

Full Text Available Abstract Spinocerebellar ataxia (SCA is a physically devastating, genetically inherited disorder characterized by abnormal brain function that results in the progressive loss of the ability to coordinate movements. There are many types of SCAs as there are various gene mutations that can cause this disease. SCA types 1–3, 6–10, 12, and 17 result from a trinucleotide repeat expansion in the DNA-coding sequence. Intriguingly, recent work has demonstrated that increased trinucleotde expansions in the SCA7 gene result in defect in the function of the SAGA histone acetyltransferase complex. The SCA7 gene encodes a subunit of the SAGA complex. This subunit is conserved in yeast as the SGF73 gene. We demonstrate that Sgf73 is required to recruit the histone deubiquitination module into both SAGA and the related SliK(SALSA complex, and to maintain levels of histone ubiquitination, which is necessary for regulation of transcription at a number of genes.

From mild ataxia to huntington disease phenocopy: the multiple faces of spinocerebellar ataxia 17.

Science.gov (United States)

Koutsis, Georgios; Panas, Marios; Paraskevas, George P; Bougea, Anastasia M; Kladi, Athina; Karadima, Georgia; Kapaki, Elisabeth

2014-01-01

Introduction. Spinocerebellar ataxia 17 (SCA 17) is a rare autosomal dominant cerebellar ataxia (ADCA) caused by a CAG/CAA expansion in the TBP gene, reported from a limited number of countries. It is a very heterogeneous ADCA characterized by ataxia, cognitive decline, psychiatric symptoms, and involuntary movements, with some patients presenting with Huntington disease (HD) phenocopies. The SCA 17 expansion is stable during parent-child transmission and intrafamilial phenotypic homogeneity has been reported. However, significant phenotypic variability within families has also been observed. Report of the Family. We presently report a Greek family with a pathological expansion of 54 repeats at the SCA 17 locus that displayed remarkable phenotypic variability. Among 3 affected members, one presented with HD phenocopy; one with progressive ataxia, dementia, chorea, dystonia, and seizures, and one with mild slowly progressive ataxia with minor cognitive and affective symptoms. Conclusions. This is the first family with SCA 17 identified in Greece and highlights the multiple faces of this rare disorder, even within the same family.

Atmospheric River Development and Effects on Southern California

Science.gov (United States)

Harris, S. M.; Carvalho, L. V.

2014-12-01

Throughout most of southern California (SCA) annual precipitation totals occur from relatively few storms per season. Any changes to storm frequency or intensity may dramatically impact the region, as its landscapes are prone to various rainfall-induced hazards including landslides and floods. These hazards become more frequent following drought or fire events, conditions also reliant on precipitation and common in SCA. Rainfall forecasts are especially difficult to determine as regional precipitation is affected by numerous phenomena. On synoptic timescales, atmospheric rivers (ARs) are one such phenomenon known to impact SCA rainfall. ARs are channels of high water vapor content found within the lower atmosphere that transport moisture towards midlatitudes. In areas with varying topography, ARs often produce high-intensity precipitation due to orographic forcing. Although much insight has been gained in understanding AR climatology affecting North America's western coast, the spatiotemporal characteristics and atmospheric forcings driving ARs to SCA need to be further addressed. The goal of this work is to understand the characteristics of ARs that impact SCA and to distinguish them from ARs that impact northern latitudes. We investigate AR characteristics as well as atmospheric features prior to plume initiation for ARs impacting different landfall regions along North America's western coast between 1998-2008. Dates of AR events are organized according to landfall region using total precipitable water (TPW) fields from the National Oceanic and Atmospheric Administration's Climate Forecast System Reanalysis (CFSR). Additional CFSR fields are used to create anomaly composites of moist static energy, geopotential height, as well as upper-level zonal and low-level meridional winds for each landfall region on the day of and prior to AR occurrence. ARs that impact SCA display different TPW plume characteristics as well as wave train patterns throughout the AR

Trajectories of Change in Students' Self-Concepts of Ability and Values in Math and College Major Choice

Science.gov (United States)

Musu-Gillette, Lauren E.; Wigfield, Allan; Harring, Jeffrey R.; Eccles, Jacquelynne S.

2015-01-01

This study extends previous research on the long-term connections between motivation constructs in expectancy-value theory and achievement outcomes. Using growth mixture modelling, we examined trajectories of change for 421 students from 4th grade through college in their self-concept of ability (SCA) in math, interest in math, and perceived…

The eXtraordinarY Kids Clinic: an interdisciplinary model of care for children and adolescents with sex chromosome aneuploidy

Directory of Open Access Journals (Sweden)

Tartaglia N

2015-07-01

Full Text Available Nicole Tartaglia,1,2 Susan Howell,1,2 Rebecca Wilson,2 Jennifer Janusz,1,2 Richard Boada,1,2 Sydney Martin,2 Jacqueline B Frazier,2 Michelle Pfeiffer,2 Karen Regan,2 Sarah McSwegin,2 Philip Zeitler1,2 1Department of Pediatrics, University of Colorado School of Medicine, 2Child Development Unit, Children's Hospital Colorado, Aurora, CO, USA Purpose: Individuals with sex chromosome aneuploidies (SCAs are born with an atypical number of X and/or Y chromosomes, and present with a range of medical, developmental, educational, behavioral, and psychological concerns. Rates of SCA diagnoses in infants and children are increasing, and there is a need for specialized interdisciplinary care to address associated risks. The eXtraordinarY Kids Clinic was established to provide comprehensive and experienced care for children and adolescents with SCA, with an interdisciplinary team composed of developmental–behavioral pediatrics, endocrinology, genetic counseling, child psychology, pediatric neuropsychology, speech–language pathology, occupational therapy, nursing, and social work. The clinic model includes an interdisciplinary approach to care, where assessment results by each discipline are integrated to develop unified diagnostic impressions and treatment plans individualized for each patient. Additional objectives of the eXtraordinarY Kids Clinic program include prenatal genetic counseling, research, education, family support, and advocacy. Methods: Satisfaction surveys were distributed to 496 patients, and responses were received from 168 unique patients. Results: Satisfaction with the overall clinic visit was ranked as “very satisfied” in 85%, and as “satisfied” in another 9.8%. Results further demonstrate specific benefits from the clinic experience, the importance of a knowledgeable clinic coordinator, and support the need for similar clinics across the country. Three case examples of the interdisciplinary approach to assessment and

Erythrocyte aging in sickle cell disease.

NARCIS (Netherlands)

Bosman, G.J.C.G.M.

2004-01-01

Physiological removal of old erythrocytes from the circulation by macrophages is initiated by binding of autologous IgG to senescent cell antigen (SCA). SCA is generated from the anion exchanger band 3. This process is accompanied by a number of alterations in the function and structure of band 3.

Protein C and antithrombin levels in patients with sickle cell anemia ...

African Journals Online (AJOL)

Background: Alterations in the components of hemostasis, namely platelet function, the procoagulant, anticoagulant, and the fibrinolytic systems, are observed in sickle cell anemia (SCA) and are in favor of a procoagulant phenotype. Therefore, study of protein C and antithrombin (AT) levels in patients with SCA in steady ...

High-resolution LIDAR and ground observations of snow cover in a complex forested terrain in the Sierra Nevada - implications for optical remote sensing of seasonal snow.

Science.gov (United States)

Kostadinov, T. S.; Harpold, A.; Hill, R.; McGwire, K.

2017-12-01

Seasonal snow cover is a key component of the hydrologic regime in many regions of the world, especially those in temperate latitudes with mountainous terrain and dry summers. Such regions support large human populations which depend on the mountain snowpack for their water supplies. It is thus important to quantify snow cover accurately and continuously in these regions. Optical remote-sensing methods are able to detect snow and leverage space-borne spectroradiometers with global coverage such as MODIS to produce global snow cover maps. However, snow is harder to detect accurately in mountainous forested terrain, where topography influences retrieval algorithms, and importantly - forest canopies complicate radiative transfer and obfuscate the snow. Current satellite snow cover algorithms assume that fractional snow-covered area (fSCA) under the canopy is the same as the fSCA in the visible portion of the pixel. In-situ observations and first principles considerations indicate otherwise, therefore there is a need for improvement of the under-canopy correction of snow cover. Here, we leverage multiple LIDAR overflights and in-situ observations with a distributed fiber-optic temperature sensor (DTS) to quantify snow cover under canopy as opposed to gap areas at the Sagehen Experimental Forest in the Northern Sierra Nevada, California, USA. Snow-off LIDAR overflights from 2014 are used to create a baseline high-resolution digital elevation model and classify pixels at 1 m resolution as canopy-covered or gap. Low canopy pixels are excluded from the analysis. Snow-on LIDAR overflights conducted by the Airborne Snow Observatory in 2016 are then used to classify all pixels as snow-covered or not and quantify fSCA under canopies vs. in gap areas over the Sagehen watershed. DTS observations are classified as snow-covered or not based on diel temperature fluctuations and used as validation for the LIDAR observations. LIDAR- and DTS-derived fSCA is also compared with

Hydroxyurea Therapy for Children With Sickle Cell Anemia in Sub‐Saharan Africa: Rationale and Design of the REACH Trial

Science.gov (United States)

Tshilolo, Léon; Santos, Brigida; Tomlinson, George A.; Stuber, Susan; Latham, Teresa; Aygun, Banu; Obaro, Stephen K.; Olupot‐Olupot, Peter; Williams, Thomas N.; Odame, Isaac; Ware, Russell E.

2015-01-01

Background Sickle cell anemia (SCA) is an inherited hematological disorder that causes a large but neglected global health burden, particularly in Africa. Hydroxyurea represents the only available disease‐modifying therapy for SCA, and has proven safety and efficacy in high‐resource countries. In sub‐Saharan Africa, there is minimal use of hydroxyurea, due to lack of data, absence of evidence‐based guidelines, and inexperience among healthcare providers. Procedure A partnership was established between investigators in North America and sub‐Saharan Africa, to develop a prospective multicenter research protocol designed to provide data on the safety, feasibility, and benefits of hydroxyurea for children with SCA. Results The Realizing Effectiveness Across Continents with Hydroxyurea (REACH, ClinicalTrials.gov NCT01966731) trial is a prospective, phase I/II open‐label dose escalation study of hydroxyurea that will treat a total of 600 children age 1–10 years with SCA: 150 at each of four different clinical sites within sub‐Saharan Africa (Angola, Democratic Republic of Congo, Kenya, and Uganda). The primary study endpoint will be severe hematological toxicities that occur during the fixed‐dose treatment phase. REACH has an adaptive statistical design that allows for careful assessment of toxicities to accurately identify a safe hydroxyurea dose. Conclusions REACH will provide data that address critical gaps in knowledge for the treatment of SCA in sub‐Saharan Africa. By developing local expertise with the use of hydroxyurea and helping to establish treatment guidelines, the REACH trial results will have the potential to transform care for children with SCA in Africa. Pediatr Blood Cancer © 2015 Wiley Periodicals, Inc. PMID:26275071

Assessment of kidney function in sickle cell anemia patients in Zaria, Nigeria

Directory of Open Access Journals (Sweden)

Rasheed Yusuf

2017-01-01

Full Text Available Introduction: Sickle cell anemia (SCA patients are prone to kidney injury by various mechanisms including reduced blood flow, ischemia, and papillary necrosis. Sickle cell nephropathy may progress to end-stage renal disease with increased morbidity and mortality. Objective: To assess renal function tests and their relationship with kidney length in steady state SCA patients. Subjects and Methods: Seventy-four adult SCA patients in steady state and 20 hemoglobin AA controls were enrolled into the study. Serum urea, electrolytes, creatinine, and uric acid were assayed while estimated glomerular filtration rate (eGFR was calculated. Renal scan was also performed to assess the kidney length. Results: Serum potassium, phosphate, and uric acid were statistically significantly higher while sodium, chloride, bicarbonate, calcium, and eGFR were significantly lower in SCA patient than in controls (P < 0.05. eGFR of < 90 ml/min was found in 50 (67.6% of SCA patients out of which 7 (9.5% had Stage 3 chronic kidney disease (CKD (<60 ml/min and one patient with Stage 4 CKD who also had shrunken kidneys with elevated serum creatinine (203 μmol/L and urea (11.7 mmol/L concentration. Renal ultrasonography revealed reduced renal size in 20 (27.1% of the patients while 2 (2.7% had a renal enlargement. There was no correlation between renal length and serum electrolytes, urea, creatinine, and eGFR. Conclusion: The majority of steady state SCA patients in Zaria have reduced eGFR and dyselectrolytemia. However, there was no association between the kidney length and the biochemical parameters. We, thus, recommend renal function tests to be routinely requested for proper management of these patients.

Are sickle cell anaemia and sickle cell trait predictive factors for periodontal disease? A cohort study.

Science.gov (United States)

de Carvalho, H L C C; Thomaz, E B A F; Alves, C M C; Souza, S F C

2016-10-01

Periodontal diseases are associated with bacterial challenge and the host immune response, and are also modulated by genetic factors. There is evidence that sickle cell anaemia (SCA) does not represent a risk factor for periodontal diseases. However, it is still unclear whether the heterozygous condition [sickle cell trait (SCT)] is associated with periodontal diseases. SCT is a genetic condition that can cause vaso-occlusive events, which may be associated with a propensity to bacterial infections. The aim of this study was to investigate the association of SCA and SCT with periodontal diseases by evaluating clinical and radiographic characteristics. The sample (n = 369) was selected and divided into two groups: exposed groups [HbSS (SCA genotype) and HbAS (SCT genotype) = 246] and a nonexposed group (HbAA = 123). HbAA consisted of individuals without SCA and SCT. The clinical parameters evaluated were plaque index, gingival index, calculus index, clinical probing depth, clinical attachment level, gingival recession, tooth mobility and furcation involvement. The percentage of alveolar bone loss was measured using a Schei ruler. Binomial and Poisson regressions were used to estimate correlations of interest (α = 0.05). None of the periodontal parameters was associated with SCA. SCT was associated with gingivitis (p = 0.041) and periodontitis (p = 0.002). Individuals with SCT had a lower plaque index (p = 0.044) but a higher calculus index (p = 0.003) and greater alveolar bone loss (p = 0.010) compared with subjects in the HbAA group. SCT can act as a predictor for establishment of periodontal diseases. There was no correlation between SCA and periodontal diseases. © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

A single-cell analysis platform for electrochemiluminescent detection of platelets adhesion to endothelial cells based on Au@DL-ZnCQDs nanoprobes.

Science.gov (United States)

Long, Dongping; Shang, Yunfei; Qiu, Youyi; Zhou, Bin; Yang, Peihui

2018-04-15

A novel single-cell analysis platform (SCA) was developed for the investigation of platelets adhesion to single human umbilical vein endothelial cell (HUVEC) via using the adhesion molecule (E-selectin) on the damaged HUVEC as the marker site, and integrating electrochemiluminescence (ECL) with the ultrasensitive Au@DL-ZnCQDs nanoprobes. The Au@DL-ZnCQDs nanocomposite, a kind of double layer zinc-coadsorbed carbon quantum dot (ZnCQDs) core-shell nanoprobe, was firstly constructed by using gold nanoparticles (AuNPs) as the core to load with ZnCQDs and then the citrate-modified silver nanoparticles (AgNPs) as the bridge to link AuNPs-ZnCQDs with ZnCQDs to form the core-shell with double layer ZnCQDs (DL-ZnCQDs) nanoprobe, revealed a 10-fold signal amplification. The H 2 O 2 -induced oxidative damage HUVECs were utilized as the cellular model on which anti-E-selectin functionalized nanoprobes specially recognized E-selectin, the SCA showed that the ECL signals decreased with platelets adhesion to single HUVEC. The proposed SCA could effectively and dynamically monitor the adhesion between single HUVEC and platelets in the absence and presence of collagen activation, moreover, be able to quantitatively detect the number of platelets adhesion to single HUVEC, and show a good analytical performance with linear range from 1 to 15 platelets. In contrast, the HUVEC was down-regulated the expression of adhesion molecules by treating with quercetin inhibitor, and the SCA also exhibited the feasibility for analysis of platelets adhesion to single HUVEC. Therefore, the single-cell analysis platform provided a novel and promising protocol for analysis of the single intercellular adhesion, and it will be beneficial to elucidate the pathogenesis of cardiovascular diseases. Copyright © 2017 Elsevier B.V. All rights reserved.

Value of Frequency Domain Resting-State Functional Magnetic Resonance Imaging Metrics Amplitude of Low-Frequency Fluctuation and Fractional Amplitude of Low-Frequency Fluctuation in the Assessment of Brain Tumor-Induced Neurovascular Uncoupling.

Science.gov (United States)

Agarwal, Shruti; Lu, Hanzhang; Pillai, Jay J

2017-08-01

The aim of this study was to explore whether the phenomenon of brain tumor-related neurovascular uncoupling (NVU) in resting-state blood oxygen level-dependent functional magnetic resonance imaging (BOLD fMRI) (rsfMRI) may also affect the resting-state fMRI (rsfMRI) frequency domain metrics the amplitude of low-frequency fluctuation (ALFF) and fractional ALFF (fALFF). Twelve de novo brain tumor patients, who underwent clinical fMRI examinations, including task-based fMRI (tbfMRI) and rsfMRI, were included in this Institutional Review Board-approved study. Each patient displayed decreased/absent tbfMRI activation in the primary ipsilesional (IL) sensorimotor cortex in the absence of a corresponding motor deficit or suboptimal task performance, consistent with NVU. Z-score maps for the motor tasks were obtained from general linear model analysis (reflecting motor activation vs. rest). Seed-based correlation analysis (SCA) maps of sensorimotor network, ALFF, and fALFF were calculated from rsfMRI data. Precentral and postcentral gyri in contralesional (CL) and IL hemispheres were parcellated using an automated anatomical labeling template for each patient. Region of interest (ROI) analysis was performed on four maps: tbfMRI, SCA, ALFF, and fALFF. Voxel values in the CL and IL ROIs of each map were divided by the corresponding global mean of ALFF and fALFF in the cortical brain tissue. Group analysis revealed significantly decreased IL ALFF (p = 0.02) and fALFF (p = 0.03) metrics compared with CL ROIs, consistent with similar findings of significantly decreased IL BOLD signal for tbfMRI (p = 0.0005) and SCA maps (p = 0.0004). The frequency domain metrics ALFF and fALFF may be markers of lesion-induced NVU in rsfMRI similar to previously reported alterations in tbfMRI activation and SCA-derived resting-state functional connectivity maps.

Estimating Rates of Psychosocial Problems in Urban and Poor Children with Sickle Cell Anemia.

Science.gov (United States)

Barbarin, Oscar A.; And Others

1994-01-01

Examined adjustment problems for children and adolescents with sickle cell anemia (SCA). Parents provided information on social, emotional, academic, and family adjustment of 327 children with SCA. Over 25% of children had emotional adjustment problems in form of internalizing symptoms (anxiety and depression); at least 20% had problems related to…

Development of a pharmacokinetic‐guided dose individualization strategy for hydroxyurea treatment in children with sickle cell anaemia

Science.gov (United States)

Dong, Min; McGann, Patrick T.; Mizuno, Tomoyuki; Ware, Russell E.

2016-01-01

AIMS Hydroxyurea has emerged as the primary disease‐modifying therapy for patients with sickle cell anaemia (SCA). The laboratory and clinical benefits of hydroxyurea are optimal at maximum tolerated dose (MTD), but the current empirical dose escalation process often takes up to 12 months. The purpose of this study was to develop a pharmacokinetic‐guided dosing strategy to reduce the time required to reach hydroxyurea MTD in children with SCA. Methods Pharmacokinetic (PK) data from the HUSTLE trial (NCT00305175) were used to develop a population PK model using non‐linear mixed effects modelling (nonmem 7.2). A D‐optimal sampling strategy was developed to estimate individual PK and hydroxyurea exposure (area under the concentration–time curve (AUC)). The initial AUC target was derived from HUSTLE clinical data and defined as the mean AUC at MTD. Results PK profiles were best described by a one compartment with Michaelis–Menten elimination and a transit absorption model. Body weight and cystatin C were identified as significant predictors of hydroxyurea clearance. The following clinically feasible sampling times are included in a new prospective protocol: pre‐dose (baseline), 15–20 min, 50–60 min and 3 h after an initial 20 mg kg–1 oral dose. The mean target AUC(0,∞) for initial dose titration was 115 mg l–1 h. Conclusion We developed a PK model‐based individualized dosing strategy for the prospective Therapeutic Response Evaluation and Adherence Trial (TREAT, ClinicalTrials.gov NCT02286154). This approach has the potential to optimize the dose titration of hydroxyurea therapy for children with SCA, such that the clinical benefits at MTD are achieved more quickly. PMID:26615061

A study on the association between parvovirus B19 infection, serum tumour necrosis factor and C-reactive protein levels among Nigerian patients with sickle cell anaemia.

Science.gov (United States)

Iwalokun, Bamidele Abiodun; Iwalokun, Senapon Olusola; Hodonu, Semande Olufunmilayo; Aina, Olugbenga Ayoola; Omilabu, Sunday

2012-11-01

Microbial burden involving parvovirus B19 infection has been recognised as a major cause of morbidity and mortality in sickle cell anaemia (SCA) patients. Given the recent reports of parvovirus B19 infection in Nigeria and the role of inflammation in sickle cell crisis, knowledge of the relationship between the two may be essential for deploying appropriate interventions in infected patients. This study determined the serum levels of tumour necrosis factor alpha (TNF-α) and C-reactive protein (CRP) as inflammatory markers in Nigerian SCA patients with and without parvovirus B19 infections. A total of 64 SCA patients aged 5-25 years and 41 age-matched apparently healthy volunteers with haemoglobin genotypes AA or AS were enrolled with consent into the study. Parvovirus B19 infection and serum levels of TNF-α and CRP were determined by the ELISA method. The overall prevalence rate of parvovirus B19 infection in the study subjects was 13.3%. This rate further showed gender variation and negative correlation with age. Significant (p Parvovirus B19 infection was found to elicit greater increases in these inflammatory markers than in infected non-SCA controls. We conclude that parvovirus B19 infection is common in this environment, and that serum TNF-α and CRP are predictors of clinical inflammatory episodes in infected SCA patients.

Engaging Key Stakeholders in Climate Change: A Community-Based Project for Youth-Led Participatory Climate Action

Science.gov (United States)

Trott, Carlie D.

Few studies have examined how youth think about, and take action on climate change and far fewer have sought to facilitate their engagement using participatory methods. This dissertation evaluated the impacts of Science, Camera, Action! (SCA), a novel after-school program that combined climate change education with participatory action through photovoice. The specific aims of this study were to: (1) Evaluate the impacts of SCA on youth participants' climate change knowledge, attitudes, and behaviors; (2) Examine how SCA participation served to empower youth agency; and (3) Explore SCA's influence on youths' science engagement. Participants were 55 youths (ages 10 to 12) across three Boys and Girls Club sites in Northern Colorado. SCA's Science component used interactive activities to demonstrate the interrelationships between Earth's changing climate, ecosystems, and sustainable actions within communities. Photovoice, SCA's Camera component, was used to explore youths' climate change perspectives and to identify opportunities for their active engagement. Finally, SCA's Action component aimed to cultivate youth potential as agents of change in their families and communities through the development and implementation of youth-led action projects. Action projects included local policy advocacy, a tree-planting campaign, a photo gallery opening, development of a website, and the establishment of a Boys and Girls Club community garden. To evaluate SCA impacts, a combination of survey and focus group methods were used. Following the program, youth demonstrated increased knowledge of the scientific and social dimensions of the causes and consequences of climate change, as well as its solutions through human action. Though participants expressed a mix of positive (e.g., hope) and negative (e.g., sadness) emotions about climate change, they left the program with an increased sense of respect for nature, an enhanced sense of environmental responsibility, and a greater sense

An RFI Detection Algorithm for Microwave Radiometers Using Sparse Component Analysis

Science.gov (United States)

Mohammed-Tano, Priscilla N.; Korde-Patel, Asmita; Gholian, Armen; Piepmeier, Jeffrey R.; Schoenwald, Adam; Bradley, Damon

2017-01-01

Radio Frequency Interference (RFI) is a threat to passive microwave measurements and if undetected, can corrupt science retrievals. The sparse component analysis (SCA) for blind source separation has been investigated to detect RFI in microwave radiometer data. Various techniques using SCA have been simulated to determine detection performance with continuous wave (CW) RFI.

Body Mass Index and Sexual Maturation in Adolescent Patients with ...

African Journals Online (AJOL)

Background: Sickle cell anaemia (SCA) is associated with delayed sexual maturation. The Body Mass Index (BMI) or Quetelets Index is closely linked to events of puberty in normal children. We have so far, found no reports on studies on the relationship between BMI and puberty in patients with SCA. Objectives: To evaluate ...

Line X tester analysis in pea (Pisum sativum L.): Identification of ...

African Journals Online (AJOL)

STORAGESEVER

2008-08-18

Aug 18, 2008 ... parents together with the information regarding nature and magnitude of gene effects controlling quantitative traits (Basbag et al., 2007). GCA and SCA which identify the hybrids with high yield are the most important criteria in breeding programs (Ceyhan, 2003). High GCA and. SCA for seed yield and yield ...

76 FR 53719 - Nondisplacement of Qualified Workers Under Service Contracts

Science.gov (United States)

2011-08-29

.... Subpart A--General Executive Order 13495 does not establish wage or fringe benefit rates. The minimum wage... misplaced. As noted, the SCA establishes the minimum wage rates and fringe benefits to be paid to service employees on a contract for services. These minimum wage rates and fringe benefits can result from the SCA...

Assessment of biochemical liver function tests in relation to age ...

African Journals Online (AJOL)

Background and Objective: Multiorgan failure including liver dysfunction is a common finding in sickle cell anemia (SCA) patients, the cause of which is multifactorial with advancing age said to be a major determinant. There is a paucity of data on liver function among SCA patients in relation to age in northern Nigerian ...

Stable-isotope analysis of a deep-sea benthic-fish assemblage: evidence of an enriched benthic food web.

Science.gov (United States)

Boyle, M D; Ebert, D A; Cailliet, G M

2012-04-01

In this study, fishes and invertebrates collected from the continental slope (1000 m) of the eastern North Pacific Ocean were analysed using stable-isotope analysis (SIA). Resulting trophic positions (T(P) ) were compared to known diets and habitats from the literature. Dual isotope plots indicated that most species groups (invertebrates and fishes) sorted as expected along the carbon and nitrogen axes, with less intraspecific variability than interspecific variability. Results also indicated an isotopically distinct benthic and pelagic food web, as the benthic food web was more enriched in both nitrogen and carbon isotopes. Trophic positions from SIA supported this finding, resulting in the assignment of fishes to different trophic positions from those expected based on published dietary information. These differences can be explained largely by the habitat of the prey and the percentage of the diet that was scavenged. A mixing model estimated dietary contributions of prey similar to those of the known diet of Bathyraja trachura from stomach-content analysis (SCA). Linear regressions indicated that trophic positions calculated from SIA and SCA, when plotted against B. trachura total length for 32 individuals, exhibited similar variation and patterns. Only the T(P) from SCA yielded significant results (stomach content: P 0·05). © 2012 The Authors. Journal of Fish Biology © 2012 The Fisheries Society of the British Isles.

Snow Cover Monitoring Using MODIS Data in Liaoning Province, Northeastern China

Directory of Open Access Journals (Sweden)

Yu Lu

2010-03-01

Full Text Available This paper presents the results of snow cover monitoring studies in Liaoning Province, northeastern China, using MODIS data. Snow cover plays an important role in both the regional water balance and soil moisture properties during the early spring in northeastern China. In addition, heavy snowfalls commonly trigger hazards such as flooding, caused by rapid snow melt, or crop failure, resulting from fluctuations in soil temperature associated with changes in the snow cover. The latter is a function of both regional, or global, climatic changes, as well as fluctuations in the albedo resulting from variations in the Snow Covered Area (SCA. These impacts are crucial to human activities, especially to those living in middle-latitude areas such as Liaoning Province. Thus, SCA monitoring is currently an important tool in studies of global climate change, particularly because satellite remote sensing data provide timely and efficient snow cover information for large areas. In this study, MODIS L1B data, MODIS Daily Snow Products (MOD10A1 and MODIS 8-day Snow Products (MOD10A2 were used to monitor the SCA of Liaoning Province over the winter months of November–April, 2006–2008. The effects of cloud masking and forest masking on the snow monitoring results were also assessed. The results show that the SCA percentage derived from MODIS L1B data is relatively consistent, but slightly higher than that obtained from MODIS Snow Products. In situ data from 25 snow stations were used to assess the accuracy of snow cover monitoring from the SCA compared to the results from MODIS Snow Products. The studies found that the SCA results were more reliable than MODIS Snow Products in the study area.

Association of classical markers and establishment of the dyslipidemic sub-phenotype of sickle cell anemia.

Science.gov (United States)

Aleluia, Milena Magalhães; da Guarda, Caroline Conceição; Santiago, Rayra Pereira; Fonseca, Teresa Cristina Cardoso; Neves, Fábia Idalina; de Souza, Regiana Quinto; Farias, Larissa Alves; Pimenta, Felipe Araújo; Fiuza, Luciana Magalhães; Pitanga, Thassila Nogueira; Ferreira, Júnia Raquel Dutra; Adorno, Elisângela Vitória; Cerqueira, Bruno Antônio Veloso; Gonçalves, Marilda de Souza

2017-04-11

Sickle cell anemia (SCA) patients exhibit sub-phenotypes associated to hemolysis and vaso-occlusion. The disease has a chronic inflammatory nature that has been also associated to alterations in the lipid profile. This study aims to analyze hematological and biochemical parameters to provide knowledge about the SCA sub-phenotypes previously described and suggest a dyslipidemic sub-phenotype. A cross-sectional study was conducted from 2013 to 2014, and 99 SCA patients in steady state were enrolled. We assessed correlations and associations with hematological and biochemical data and investigated the co-inheritance of -α 3.7Kb -thalassemia (-α 3.7Kb -thal). Correlation analyses were performed using Spearman and Pearson coefficient. The median of quantitative variables between two groups was compared using t-test and Mann-Whitney. P-values <0.05 were considered statistically significant. We found significant association of high lactate dehydrogenase levels with decreased red blood cell count and hematocrit as well as high levels of total and indirect bilirubin. SCA patients with low nitric oxide metabolites had high total cholesterol, high-density lipoprotein cholesterol, and low-density lipoprotein cholesterol and reduced very low-density cholesterol, triglycerides, direct bilirubin level and reticulocyte counts. In SCA patients with high-density lipoprotein cholesterol greater than 40 mg/dL, we observed increased red blood cell count, hemoglobin, hematocrit, and fetal hemoglobin and decreased nitric oxide metabolites levels. The presence of -α 3.7Kb -thal was associated with high red blood cell count and low mean corpuscular volume, mean corpuscular hemoglobin, platelet count and total and indirect bilirubin levels. Our results provide additional information about the association between biomarkers and co-inheritance of -α 3.7Kb -thal in SCA, and suggest the role of dyslipidemia and nitric oxide metabolites in the characterization of this sub-phenotype.

Spinocerebellar Ataxia Type 6 Protein Aggregates Cause Deficits in Motor Learning and Cerebellar Plasticity

NARCIS (Netherlands)

Mark, Melanie D; Krause, Martin; Boele, Henk-Jan; Kruse, Wolfgang; Pollok, Stefan; Kuner, Thomas; Dalkara, Deniz; Koekkoek, Sebastiaan; De Zeeuw, Chris I; Herlitze, Stefan

2015-01-01

Spinocerebellar ataxia type 6 (SCA6) is linked to poly-glutamine (polyQ) within the C terminus (CT) of the pore-forming subunits of P/Q-type Ca(2+) channels (Cav2.1) and is characterized by CT protein aggregates found in cerebellar Purkinje cells (PCs). One hypothesis regarding SCA6 disease is that

Occurrence of haemolysin antibodies among sickle cell anaemia ...

African Journals Online (AJOL)

SERVER

2007-05-16

May 16, 2007 ... A total of 50 normal controls and 54 SCA patients attending the sickle cell clinic of the. University of Calabar Teaching Hospital, Cross River State of Nigeria, were screened for haemolysins and quantitated using standard techniques. The distribution of haemolysins in the SCA patients was α. (16.7), β (11.1) ...

Sudden cardiac arrest in sports - need for uniform registration: A Position Paper from the Sport Cardiology Section of the European Association for Cardiovascular Prevention and Rehabilitation.

Science.gov (United States)

Solberg, E E; Borjesson, M; Sharma, S; Papadakis, M; Wilhelm, M; Drezner, J A; Harmon, K G; Alonso, J M; Heidbuchel, H; Dugmore, D; Panhuyzen-Goedkoop, N M; Mellwig, K-P; Carre, F; Rasmusen, H; Niebauer, J; Behr, E R; Thiene, G; Sheppard, M N; Basso, C; Corrado, D

2016-04-01

There are large variations in the incidence, registration methods and reported causes of sudden cardiac arrest/sudden cardiac death (SCA/SCD) in competitive and recreational athletes. A crucial question is to which degree these variations are genuine or partly due to methodological incongruities. This paper discusses the uncertainties about available data and provides comprehensive suggestions for standard definitions and a guide for uniform registration parameters of SCA/SCD. The parameters include a definition of what constitutes an 'athlete', incidence calculations, enrolment of cases, the importance of gender, ethnicity and age of the athlete, as well as the type and level of sporting activity. A precise instruction for autopsy practice in the case of a SCD of athletes is given, including the role of molecular samples and evaluation of possible doping. Rational decisions about cardiac preparticipation screening and cardiac safety at sport facilities requires increased data quality concerning incidence, aetiology and management of SCA/SCD in sports. Uniform standard registration of SCA/SCD in athletes and leisure sportsmen would be a first step towards this goal. © The European Society of Cardiology 2015.

[Effects of a physical training program on quantitative neurological indices in mild stage type 2 spinocerebelar ataxia patients].

Science.gov (United States)

Pérez-Avila, I; Fernández-Vieitez, J A; Martínez-Góngora, E; Ochoa-Mastrapa, R; Velázquez-Manresa, M G

Type 2 spinocerebelar ataxia (SCA2) is a neurodegenerative disease with higher prevalence and incidence in Holguín province, Cuba. At present, there is not any drug to counteract the loss of coordinative motor capacities of these patients. Thus physical training seems to be the only way to attenuate the course of disease. To evaluate the effectiveness of a physical training program on quantitative neurological indices in SCA2 patients. A samples of 87 SCA2 patients were studied. All subjects underwent a six month physical exercise program based on coordination, balance and muscular conditioning exercises. Quantitative tests were applied to all patients both before and after the application of the exercise program. Comparisons between pretest versus posttest values were made to evaluate the improvement in neurological indices. All neurological indices both with open eyes and closed eyes significantly improved from pretest to posttest. Static balance, evaluated by Romberg test, also enhanced with training. The exercise training program significantly improved the neurological indices in SCA2 patient with mild stage of disease.

From Mild Ataxia to Huntington Disease Phenocopy: The Multiple Faces of Spinocerebellar Ataxia 17

Directory of Open Access Journals (Sweden)

Georgios Koutsis

2014-01-01

Full Text Available Introduction. Spinocerebellar ataxia 17 (SCA 17 is a rare autosomal dominant cerebellar ataxia (ADCA caused by a CAG/CAA expansion in the TBP gene, reported from a limited number of countries. It is a very heterogeneous ADCA characterized by ataxia, cognitive decline, psychiatric symptoms, and involuntary movements, with some patients presenting with Huntington disease (HD phenocopies. The SCA 17 expansion is stable during parent-child transmission and intrafamilial phenotypic homogeneity has been reported. However, significant phenotypic variability within families has also been observed. Report of the Family. We presently report a Greek family with a pathological expansion of 54 repeats at the SCA 17 locus that displayed remarkable phenotypic variability. Among 3 affected members, one presented with HD phenocopy; one with progressive ataxia, dementia, chorea, dystonia, and seizures, and one with mild slowly progressive ataxia with minor cognitive and affective symptoms. Conclusions. This is the first family with SCA 17 identified in Greece and highlights the multiple faces of this rare disorder, even within the same family.

Thought and language disorders in very early onset schizophrenia, schizoaffective disorder and bipolar disorder

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Telma Pantano

Full Text Available Abstract Background Thought and language disorders are main features of adults with schizophrenia and bipolar disorders however studies on such abnormalities are scant in young patients with very early onset psychosis (VEOS. The aim of the present study is to assess the relationship between language and thought disorders in patients with very early onset schizophrenia (SCZ, schizoaffective disorders (SCA and bipolar disorders (BD. Method Forty-one patients (18 SCZ, 16 BD, and 7 SCA with mean age less than 15 years old were assessed through a series of neurocognitive and psycholinguistic tests, including the Thought, Language and Communication Scale (TLC. Results SCZ group performed worse in all tests as well as the TLC, followed by SCA and BD groups respectively. Thought disorders were related to deficits in executive functioning and semantic processing, and the metaphors’ test was the best predictor of TLC functioning. Discussion TD in SCZ, SCA and BD are one of the most important features in patients with VEOS and that the evaluation of metaphor comprehension can be an important instrument in the early detection of this disorder.

Cerebral vasculopathy in children with sickle cell anemia.

Science.gov (United States)

Fasano, Ross M; Meier, Emily R; Hulbert, Monica L

2015-01-01

Sickle cell anemia (SCA)-associated cerebral vasculopathy and moyamoya is a unique entity reflecting the abnormal interactions between sickled red blood cells (RBCs) and the cerebral arterial endothelium. Endothelial injury, coagulation activation, and the inflammatory response generated by sickled RBCs are implicated in the development of cerebral vasculopathy, but the pathophysiology remains incompletely understood. SCA-specific screening and treatment guidelines have successfully reduced the incidence of overt strokes in this high-risk population. However, despite aggressive hematological management, many children with cerebral vasculopathy due to SCA have progressive vasculopathy and recurrent strokes; therefore, more effective therapies, such as revascularization surgery and curative hematopoietic stem cell transplant, are urgently needed. Copyright © 2014 Elsevier Inc. All rights reserved.

Assessing the Accuracy of Passive Microwave Estimates of Snow Water Equivalent in Data-Scarce Regions for Use in Water Resource Applications: A Case Study in the Upper Helmand Watershed, Afghanistan

Science.gov (United States)

2011-03-01

the HMS model was de- veloped using the spatial hydrology tool, GeoHMS, within ArcGIS . Basin characteristics were derived from the USGS DEM for each...snowpack. Still, it has been successfully used in hydrolog - ic applications. The snow runoff model (SRM) uses SCA and a basin - specific snow depletion curve...three sections. The first section describes the preliminary analysis of the study data and methods. An overview of the basin hydrology is given. The

The effect of knowledge based view on sustainable competitive advantage

Directory of Open Access Journals (Sweden)

Fatemeh Rezaee

2015-11-01

Full Text Available This study investigates the quantitative relationship between knowledge based view (i.e. empowering employees, promoting confidence, coding rules and sustainability competitive advantage (i.e. market, customer, financial within the banking industry of Iran. A valid research instrument was utilized to conduct a survey of 150 top- and middle-level managers from Mellat bank of Iran. With a response rate of 81.3 percent, 122 questionnaires are returned; the number of valid and usable questionnaires was 101. In order to determine validity of questionnaire, the content validity was used and Cronbach's alpha was used to determine the reliability of the questionnaire (KBV questionnaire 0.886, SCA questionnaire 0.843. Utilizing the structural equation modeling, and after a series of exploratory and confirmatory factor analyses, it was found that KBV had the greatest effect on the market centered SCA, while it had the least influence on the customer centered.

Evaluation of an assimilation scheme to estimate snow water equivalent in the High Atlas of Morocco.

Science.gov (United States)

Baba, W. M.; Baldo, E.; Gascoin, S.; Margulis, S. A.; Cortés, G.; Hanich, L.

2017-12-01

The snow melt from the Atlas mountains represents a crucial water resource for crop irrigation in Morocco. Due to the paucity of in situ measurements, and the high spatial variability of the snow cover in this semi-arid region, assimilation of snow cover area (SCA) from high resolution optical remote sensing into a snowpack energy-balance model is considered as a promising method to estimate the snow water equivalent (SWE) and snow melt at catchment scales. Here we present a preliminary evaluation of an uncalibrated particle batch smoother data assimilation scheme (Margulis et al., 2015, J. Hydrometeor., 16, 1752-1772) in the High-Atlas Rheraya pilot catchment (225 km2) over a snow season. This approach does not require in situ data since it is based on MERRA-2 reanalyses data and satellite fractional snow cover area data. We compared the output of this prior/posterior ensemble data assimilation system to output from the distributed snowpack evolution model SnowModel (Liston and Elder, 2006, J. Hydrometeor. 7, 1259-1276). SnowModel was forced with in situ meteorological data from five automatic weather stations (AWS) and some key parameters (precipitation correction factor and rain-snow phase transition parameters) were calibrated using a time series of 8-m resolution SCA maps from Formosat-2. The SnowModel simulation was validated using a continuous snow height record at one high elevation AWS. The results indicate that the open loop simulation was reasonably accurate (compared to SnowModel results) in spite of the coarse resolution of the MERRA-2 forcing. The assimilation of Formosat-2 SCA further improved the simulation in terms of the peak SWE and SWE evolution over the melt season. During the accumulation season, the differences between the modeled and estimated (posterior) SWE were more substantial. The differences appear to be due to some observed precipitation events not being captured in MERRA-2. Further investigation will determine whether additional

Pyuria as a diagnostic test for urinary tract infection in children with ...

African Journals Online (AJOL)

Objective: This study aimed at determining the significance of pyuria as a screening test for UTI in children with sickle cell anaemia (SCA). Methods: Two hundred and seventy-two children with SCA, aged 6 months to 15 years, were studied out of which 185 (68.0%) were in stable state and 87 (32.0%) were in crises.

The demise of the Roman-Dutch 'kommer-recht': Interpretation of ...

African Journals Online (AJOL)

The Supreme Court of Appeal (in a judgment dated 23.11.07) in the case of. Bid Industrial Holdings v Strang (2007) SCA 144 (RSA) (now cited as 2008(3) SA 355 (SCA)) held that the common-law requirement of arrest to found or confirm jurisdiction where an incola plaintiff wishes to sue a foreign peregrinus, which ...

SCaN Transportable Communication Platform (STCP)

Science.gov (United States)

Haddad, George; Tanger, Thomas; Pleva, David; Schoenholz, Bryan; Nam, Connor

2017-01-01

NASA Glenn Research Center required a satellite communication trailer that served dual purposes; 24/7 Emergency Communication Services (ECS) in the event of a natural or manmade disaster that disrupted conventional communications, and a Ka Band NASA TDRS capability providing a research capability for over the air evaluations/characterizations. The trailer was to be field deployable, environmentally controlled and self-contained providing local area networks (LANs) and Wide Area Networks (WAN's) with user access both wired (Ethernet) and wireless (802.11) supporting VoIP, Internet Web access and Email. The TDRSS terminal included a 200W TWT amplifier mounted on the feed boom, individual up and down converters, and custom integrated waveguide and a supporting feed system. Other features such as a mast, generator, electrical, lighting, surveillance, and storage capabilities were also required. The Trailer was developed and demonstrated these original requested capabilities. New Requirements are defined and the trailer is now being evolved and upgraded to be a backup for the Near- Earth Network (NEN) Stations at KSC that will support the launch phase of EM-1. This paper presents the current and future capabilities of the trailer and additional options that will make it a valuable deployable asset to support remote operations from any launch from location.

Evaluating soil moisture retrievals from ESA’s SMOS and NASA’s SMAP brightness temperature datasets

Science.gov (United States)

Al-Yaari, A.; Wigneron, J.-P.; Kerr, Y.; Rodriguez-Fernandez, N.; O’Neill, P. E.; Jackson, T. J.; De Lannoy, G.J.M.; Al Bitar, A; Mialon, A.; Richaume, P.; Walker, JP; Mahmoodi, A.; Yueh, S.

2018-01-01

Two satellites are currently monitoring surface soil moisture (SM) using L-band observations: SMOS (Soil Moisture and Ocean Salinity), a joint ESA (European Space Agency), CNES (Centre national d’études spatiales), and CDTI (the Spanish government agency with responsibility for space) satellite launched on November 2, 2009 and SMAP (Soil Moisture Active Passive), a National Aeronautics and Space Administration (NASA) satellite successfully launched in January 2015. In this study, we used a multilinear regression approach to retrieve SM from SMAP data to create a global dataset of SM, which is consistent with SM data retrieved from SMOS. This was achieved by calibrating coefficients of the regression model using the CATDS (Centre Aval de Traitement des Données) SMOS Level 3 SM and the horizontally and vertically polarized brightness temperatures (TB) at 40° incidence angle, over the 2013 – 2014 period. Next, this model was applied to SMAP L3 TB data from Apr 2015 to Jul 2016. The retrieved SM from SMAP (referred to here as SMAP_Reg) was compared to: (i) the operational SMAP L3 SM (SMAP_SCA), retrieved using the baseline Single Channel retrieval Algorithm (SCA); and (ii) the operational SMOSL3 SM, derived from the multiangular inversion of the L-MEB model (L-MEB algorithm) (SMOSL3). This inter-comparison was made against in situ soil moisture measurements from more than 400 sites spread over the globe, which are used here as a reference soil moisture dataset. The in situ observations were obtained from the International Soil Moisture Network (ISMN; https://ismn.geo.tuwien.ac.at/) in North of America (PBO_H2O, SCAN, SNOTEL, iRON, and USCRN), in Australia (Oznet), Africa (DAHRA), and in Europe (REMEDHUS, SMOSMANIA, FMI, and RSMN). The agreement was analyzed in terms of four classical statistical criteria: Root Mean Squared Error (RMSE), Bias, Unbiased RMSE (UnbRMSE), and correlation coefficient (R). Results of the comparison of these various products with in

Surface planarization effect of siloxane derivatives in organic semiconductor layers

Energy Technology Data Exchange (ETDEWEB)

Sakanoue, Kei [Center for Organic Photonics and Electronics Research, Kyushu University, Nishi-ku, Fukuoka 819-0395 (Japan); Harada, Hironobu; Ando, Kento [Department of Chemical Engineering, Graduate School of Engineering, Kyushu University, Nishi-ku, Fukuoka 819-0395 (Japan); Yahiro, Masayuki [Institute of Systems, Information Technologies and Nanotechnologies, 2-1-22, Sawara-ku, Fukuoka 814-0001 (Japan); Fukai, Jun, E-mail: jfukai@chem-eng.kyushu-u.ac.jp [Department of Chemical Engineering, Graduate School of Engineering, Kyushu University, Nishi-ku, Fukuoka 819-0395 (Japan)

2015-12-31

The ability of siloxane surface control additives (SCAs) to planarize organic semiconductor films with a thickness of tens of nanometers printed on indium tin oxide (ITO) surfaces with stripe-patterned bank structures using a liquid-phase method is demonstrated. Three types of SCAs with different molecular structures are examined in organic solutions of toluene, anisole and tetralin containing N,N′-bis(3-methylphenyl)-N,N′-bis(phenyl)-benzidine as a solute and typical organic semiconductor. While there is an optimum SCA and concentration for each solution, one type of SCA is comprehensively effective for all solutions. This SCA increased contact angle, which is contrary to the typical behavior of SCAs. Scanning electron microscope images of the thin films near the banks reveal that this SCA did not change the contact area between the film and substrate surface, which is related to the effectiveness of the SCA. SCAs did not affect the current–voltage characteristics of green organic light-emitting diodes, but did increase external quantum efficiencies, suggesting that SCAs can be used to improve the quality of solution-deposited films for use in optical devices. - Highlights: • Surface control additives planarize organic semiconductor films coated on surfaces. • The most effective additive increases the contact angle of solutions during drying. • The effect of additives is deduced from solutal Marangoni forces. • Additives have little effect on organic light-emitting diode performance.

Scandoside Exerts Anti-Inflammatory Effect Via Suppressing NF-κB and MAPK Signaling Pathways in LPS-Induced RAW 264.7 Macrophages

Directory of Open Access Journals (Sweden)

Jingyu He

2018-02-01

Full Text Available The iridoids of Hedyotis diffusa Willd play an important role in the anti-inflammatory process, but the specific iridoid with anti-inflammatory effect and its mechanism has not be thoroughly studied. An iridoid compound named scandoside (SCA was isolated from H. diffusa and its anti-inflammatory effect was investigated in lipopolysaccharide (LPS-induced RAW 264.7 macrophages. Its anti-inflammatory mechanism was confirmed by in intro experiments and molecular docking analyses. As results, SCA significantly decreased the productions of nitric oxide (NO, prostaglandin E2 (PGE2, tumor necrosis factor-α (TNF-α and interleukin-6 (IL-6 and inhibited the levels of inducible nitric oxide synthase (iNOS, cyclooxygenase-2 (COX-2, TNF-α and IL-6 messenger RNA (mRNA expression in LPS-induced RAW 264.7 macrophages. SCA treatment suppressed the phosphorylation of inhibitor of nuclear transcription factor kappa-B alpaha (IκB-α, p38, extracellular signal-regulated kinase (ERK and c-Jun N-terminal kinase (JNK. The docking data suggested that SCA had great binding abilities to COX-2, iNOS and IκB. Taken together, the results indicated that the anti-inflammatory effect of SCA is due to inhibition of pro-inflammatory cytokines and mediators via suppressing the nuclear transcription factor kappa-B (NF-κB and mitogen-activated protein kinase (MAPK signaling pathways, which provided useful information for its application and development.

Surface planarization effect of siloxane derivatives in organic semiconductor layers

International Nuclear Information System (INIS)

Sakanoue, Kei; Harada, Hironobu; Ando, Kento; Yahiro, Masayuki; Fukai, Jun

2015-01-01

The ability of siloxane surface control additives (SCAs) to planarize organic semiconductor films with a thickness of tens of nanometers printed on indium tin oxide (ITO) surfaces with stripe-patterned bank structures using a liquid-phase method is demonstrated. Three types of SCAs with different molecular structures are examined in organic solutions of toluene, anisole and tetralin containing N,N′-bis(3-methylphenyl)-N,N′-bis(phenyl)-benzidine as a solute and typical organic semiconductor. While there is an optimum SCA and concentration for each solution, one type of SCA is comprehensively effective for all solutions. This SCA increased contact angle, which is contrary to the typical behavior of SCAs. Scanning electron microscope images of the thin films near the banks reveal that this SCA did not change the contact area between the film and substrate surface, which is related to the effectiveness of the SCA. SCAs did not affect the current–voltage characteristics of green organic light-emitting diodes, but did increase external quantum efficiencies, suggesting that SCAs can be used to improve the quality of solution-deposited films for use in optical devices. - Highlights: • Surface control additives planarize organic semiconductor films coated on surfaces. • The most effective additive increases the contact angle of solutions during drying. • The effect of additives is deduced from solutal Marangoni forces. • Additives have little effect on organic light-emitting diode performance.

The effect of UGT1A1 promoter polymorphism in the development of hyperbilirubinemia and cholelithiasis in hemoglobinopathy patients.

Directory of Open Access Journals (Sweden)

Suad AlFadhli

Full Text Available Present study was aimed to explore the effect of (TAn UGT1A1 gene promoter polymorphism on bilirubin metabolism, bilirubinaemia, predisposition to cholelithiasis and subsequent cholecystectomy, in Sickle-Cell Anemia (SCA and beta-Thalasemia major (bTH in Kuwaiti subjects compared to other population. This polymorphism was analyzed and correlated to total bilirubin and cholelithiasis in 270 age, gender, ethnically matched subjects (92 bTH, 116 SCA and 62 Controls using PCR, dHPLC, fragment analysis and direct sequencing. Four genotypes of UGT1A1 were detected in this study (TA6/6, TA6/7, TA6/8 and TA7/7. (TA6/8 was found only in four individuals; hence it was not included in the analysis. There was a statistically significant association of genotypes with serum total bilirubin levels in both bTH and SCA groups (p<0.001. Subjects with (TA7/7 had the highest total serum bilirubin level (178.7 ± 3.5 µmole/l. A significant association was observed between allele (TA7 and cholelithiasis development (p = 0.0001. The 40%, 67.5% and 100% of SCA with (TA6/6, (TA6/7 and (TA7/7 respectively developed cholelithiasis and were subsequently cholecystectomized. Our results confirm UGT1A1 (TA7 allele as one of the factors accounting for the hyperbilirubinemia and cholelithiasis observed in SCA and bTH.

Autosomal dominant hereditary ataxia in Sri Lanka

OpenAIRE

Sumathipala, Dulika S; Abeysekera, Gayan S; Jayasekara, Rohan W; Tallaksen, Chantal ME; Dissanayake, Vajira HW

2013-01-01

Background Spinocerebellar ataxias (SCA) are a group of hereditary neurodegenerative disorders. Prevalence of SCA subtypes differ worldwide. Autosomal dominant ataxias are the commonest types of inherited ataxias seen in Sri Lanka. The aim of the study is to determine the genetic etiology of patients with autosomal dominant ataxia in Sri Lanka and to describe the clinical features of each genetic subtype. Methods ...

Estatinas para el síndrome coronario agudo

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Noah Vale

2012-11-01

Conclusiones de los autores: Según las pruebas disponibles, el inicio del tratamiento con estatinas en el transcurso de 14 días después del SCA no reduce la muerte, el infarto de miocardio ni el accidente cerebrovascular hasta los cuatro meses, pero reduce la ocurrencia de angina inestable a los cuatro meses después del SCA.

Combining ability analysis in Brassica juncea L. for oil quality traits

African Journals Online (AJOL)

USER

2010-06-28

Jun 28, 2010 ... the SCA effects for glucosinolate, erucic acid and protein content. The parental genotypes NUM009,. NUM123, NUM105 and NUM117 and their hybrids NUM009x NUM123, NUM103x NUM105, NUM113x. NUM124 and NUM103x NUM120 had high GCA and SCA effects, respectively and therefore these ...

Design of the Front-End Detector Control System of the ATLAS New Small Wheels

CERN Document Server

Koulouris, Aimilianos; The ATLAS collaboration

2017-01-01

The ATLAS experiment will be upgraded during the next LHC Long Shutdown (LS2). The flagship upgrade is the New Small Wheel (NSW), which consists of 2 disks of Muon Gas detectors. The detector technologies used are Micromegas (MM) and sTGC, providing a total of 16 layers of tracking and trigger. The Slow Control Adapter (SCA) is part of the GigaBit Transceiver (GBT) - “Radiation Hard Optical Link Project” family of chips designed at CERN, EP-ESE department, which will be used at the NSW upgrade. The SCA offers several interfaces to read analog and digital inputs, and configure front-end Readout ASICs, FPGAs, or other chips. This poster gives an overview of the system, data flow, and software developed for communicating with the SCA.

Spinocerebellar ataxia type 7: Report of an Indian family

Directory of Open Access Journals (Sweden)

Gurusidheshwar M Wali

2013-01-01

Full Text Available Spinocerebellar ataxia type 7 (SCA7 is a form of autosomal dominant cerebellar ataxia which is associated with pigmentary retinal degeneration. It is known for its world-wide rarity except in the Scandinavian countries. It is very rarely reported from India and the neighbouring Asian countries . The present report describes the neurogenetic findings of a family of SCA7, from the northern part of Karnataka in South India. It documents the wide intrafamilial phenotypic variability, which could be correlated with the CAG repeat counts and phenomenon of anticipation. Genotype phenotype correlation highlighted certain disparities in comparison with the previous studies. The report highlights the need for multiethnic population studies and the role of genetic counseling and prenatal testing in SCA7 patients.

Anomalous Origin of the Right Coronary Artery from the Left Anterior Descending Coronary Artery in a Patient with Ascending Aortic Aneurysm

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Ufuk Gürkan

2012-04-01

Full Text Available The incidence of coronary artery anomalies has been reported between 0.6 to 1.3% in angiographic series and 0.3% in autopsy series. An isolated single coronary artery (SCA is even a rarer congenital anomaly occurring in approximately 0.02% of the population. The ectopic origin of the right coronary artery (RCA from the left anterior descending (LAD artery is relatively rare and more benign than other types of anomalous origin of the RCAs. We report a case of an adult male patient with SCA anomaly in which the RCA takes off from the mid LAD. To the best of our knowledge, SCA anomaly coinciding with ascending aortic aneurysm which was treated with Bentall operation has never been described before.

Spinocerebellar Ataxia Type 2: Clinicogenetic Aspects, Mechanistic Insights, and Management Approaches

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Luis C. Velázquez-Pérez

2017-09-01

Full Text Available Spinocerebellar ataxia type 2 (SCA2 is an autosomal dominant cerebellar ataxia that occurs as a consequence of abnormal CAG expansions in the ATXN2 gene. Progressive clinical features result from the neurodegeneration of cerebellum and extra-cerebellar structures including the pons, the basal ganglia, and the cerebral cortex. Clinical, electrophysiological, and imaging approaches have been used to characterize the natural history of the disease, allowing its classification into four distinct stages, with special emphasis on the prodromal stage, which is characterized by a plethora of motor and non-motor features. Neuropathological investigations of brain tissue from SCA2 patients reveal a widespread involvement of multiple brain systems, mainly cerebellar and brainstem systems. Recent findings linking ataxin-2 intermediate expansions to other neurodegenerative diseases such as amyotrophic lateral sclerosis have provided insights into the ataxin-2-related toxicity mechanism in neurodegenerative diseases and have raised new ethical challenges to molecular predictive diagnosis of SCA2. No effective neuroprotective therapies are currently available for SCA2 patients, but some therapeutic options such as neurorehabilitation and some emerging neuroprotective drugs have shown palliative benefits.

Automated external defibrillators in the Australian fitness industry.

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Norton, Kevin I; Norton, Lynda H

2008-04-01

Sudden cardiac arrest (SCA) occurs in many thousands of Australians each year. Scientific evidence shows an increased survival rate for individuals who receive electrical defibrillation in the first few minutes after SCA. In the last decade automated (rhythm-detecting) external defibrillators (AEDs) have become available that are portable and affordable. Although still relatively rare, there is still the potential that SCA may occur when a person undertakes physical activity. Consequently, health/fitness centres are increasingly recognised as higher risk sites that may benefit from placement of AEDs. There are no laws in Australia requiring health/fitness centres to install AEDs. However, several international and professional organisations have "strongly encouraged" larger centres to install AEDs. Guidelines and algorithms are presented to help estimate the risk of SCA in fitness centres. Fitness centre placement is particularly important if the clientele is older or has a 'high-risk' profile, for example, clients with cardiovascular, respiratory or metabolic disease. International negligence case law and duty of care principles suggests the standard of care required in health/fitness centres may be increasing. Therefore, it may be prudent to install AEDs in larger centres and those in which higher risk groups are physically active.

Paliperidone for the treatment of schizoaffective disorder.

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Alphs, Larry; Fu, Dong-Jing; Turkoz, Ibrahim

2016-01-01

Schizoaffective disorder (SCA) is a complex mental illness characterized by psychosis and affective symptoms. Treatment usually involves concomitant therapy with antipsychotics, mood stabilizers, and/or antidepressants. Effective treatment must address acute symptoms, maintain long-term stability, promote recovery, and improve patient functioning. Data from 3 pivotal studies evaluating the acute and maintenance treatment of SCA with paliperidone are reviewed. Two formulations of paliperidone have been studied for these indications: an extended-release oral formulation (NCT00397033, NCT00412373) and long-acting injectable once-monthly paliperidone palmitate (NCT01193153). The reported effects of these formulations on psychotic, depressive, and manic symptoms are discussed. Both formulations were found to be safe and effective for the acute and maintenance treatment of SCA. Of critical importance for this treatment population is that rapid improvement was seen in all major symptoms of SCA, including psychosis, depression, and mania. Mediation analyses suggest that the known antipsychotic effects of paliperidone occur independently of its antidepressant effects. Both formulations of the drug are effective when used as monotherapy or adjunctively with antidepressants or mood stabilizers. Beyond symptom control, both formulations improved patient functioning and increased patient satisfaction.

Chewing ability as a parameter for evaluating the disability of patients with temporomandibular disorders.

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Kurita, H; Ohtsuka, A; Kurashina, K; Kopp, S

2001-05-01

Restoration of chewing ability is an important aspect of the treatment for temporomandibular disorders (TMDs). However, too little attention has been paid to it. We have used a questionnaire to evaluate and score the chewing ability of TMD patients. The questionnaire includes 19 kinds of food and a chewing task. The patient was asked if she/he experiences difficulty in enjoying eating. The aim of this study was to evaluate correlations between score of chewing ability (SCA) and other symptoms/signs of TMD. Four hundred and seventy-three consecutive TMD patients were evaluated for SCA and other symptoms/signs including temporomandibular joint (TMJ) pain, TMJ and muscle tenderness, TMJ noise (clicking and crepitus), and maximum mouth opening. The relationship between SCA and other symptoms/signs were analysed by multiple regression analysis. Score of chewing ability correlated significantly with TMJ pain and mouth opening capacity but not with TMJ noise and muscle tenderness. Age was a background factor but sex was not. The result of this study suggests that SCA correlated with dysfunction of the TMD patients. This method could be used to evaluate the ability of chewing in assessment of TMD.

Trace elements in sickle cell disease

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Durosinmi, M.A.; Ojo, J.O.; Oluwole, A.F.; Akanle, O.A.; Arshed, W.; Spyrou, N.M.

1993-01-01

Instrumental Neutron Activation Analysis (INAA) and Proton-Induced X-ray Emission (PIXE) analysis (employed as a complementary technique) have been used to determine the concentration of 11 elements in blood samples and its components erythrocytes and plasma obtained from three groups of subjects in Nigeria viz: sickle cell anaemia (SCA) subjects, subjects with sickle cell trait and normal control subjects. The results suggest that SCA subjects have significantly higher concentrations of Na, Cl, Ca and Cu in their whole blood and erythrocytes and a higher concentration of Cl and Cu in their plasma relative to control subjects. Furthermore, a significantly lower concentration of K, Fe, Zn, Se, Br and Rb were found in the whole blood and erythrocytes of the SCA subjects as compared to the controls while the concentration of K and Fe in the plasma of the SCA subjects were however, found to be significantly higher than that of the control group. The study also shows that there were no significant differences between the concentration of these 11 elements in the group with sickle cell trait and the normal control group. (author) 20 refs.; 4 tabs

The transplantation of neural stem cells and predictive factors in hematopoietic recovery in irradiated mice.

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Filip, S; Mokrý, J; Karbanová, J; Vávrová, J; Vokurková, J; Bláha, M; English, D

2005-04-01

A number of surprising observations have shown that stem cells, in suitable conditions, have the ability to produce a whole spectrum of cell types, regardless, whether these tissues are derived from the same germ layer or not. This phenomenon is called stem cell plasticity, which means that tissue-specific stem cells are mutually interchangeable. In our experiments, as a model, we used neural stem cells (NSCs) harvested from fetal (E14-15) neocortex and beta-galactosidase positive. In the first experiment we found that on days 12 and 30 after sub-lethal irradiation (LD 8.5 Gy) and (beta-galactosidase(+)) NSCs transplantation all mice survived, just as the group with bone marrow transplantation. Moreover, the bone marrow of mice transplanted NSCs contained the number of CFU-GM colonies with beta-galactosidase(+) cells which was as much as 50% higher. These differences were statistically significant, pthird experiment, we verified the mutual interchange of Sca-1 surface antigen in the bone marrow cells and NSCs before transplantation. Analysis of this antigen showed 24.8% Sca-1 positive cells among the bone marrow cells, while NSCs were Sca-1 negative. Our experiments show that NSCs share hemopoietic identity and may significantly influence the recovery of damaged hematopoiesis but do not have typical superficial markers as HSCs. This result is important for the determination of predictive factors for hemopoiesis recovery, for stem cell plasticity and for their use in the cell therapy.

Motor Deficits and Cerebellar Atrophy in Elovl5 Knock Out Mice.

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Hoxha, Eriola; Gabriele, Rebecca M C; Balbo, Ilaria; Ravera, Francesco; Masante, Linda; Zambelli, Vanessa; Albergo, Cristian; Mitro, Nico; Caruso, Donatella; Di Gregorio, Eleonora; Brusco, Alfredo; Borroni, Barbara; Tempia, Filippo

2017-01-01

Spino-Cerebellar-Ataxia type 38 (SCA38) is caused by missense mutations in the very long chain fatty acid elongase 5 gene, ELOVL5 . The main clinical findings in this disease are ataxia, hyposmia and cerebellar atrophy. Mice in which Elovl5 has been knocked out represent a model of the loss of function hypothesis of SCA38. In agreement with this hypothesis, Elovl5 knock out mice reproduced the main symptoms of patients, motor deficits at the beam balance test and hyposmia. The cerebellar cortex of Elovl5 knock out mice showed a reduction of thickness of the molecular layer, already detectable at 6 months of age, confirmed at 12 and 18 months. The total perimeter length of the Purkinje cell (PC) layer was also reduced in Elovl5 knock out mice. Since Elovl5 transcripts are expressed by PCs, whose dendrites are a major component of the molecular layer, we hypothesized that an alteration of their dendrites might be responsible for the reduced thickness of this layer. Reconstruction of the dendritic tree of biocytin-filled PCs, followed by Sholl analysis, showed that the distribution of distal dendrites was significantly reduced in Elovl5 knock out mice. Dendritic spine density was conserved. These results suggest that Elovl5 knock out mice recapitulate SCA38 symptoms and that their cerebellar atrophy is due, at least in part, to a reduced extension of PC dendritic arborization.

[Blood serum level of primary bile acids in cattle, horses, swine and dogs].

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Karsai, F; Szaniszló, F; Pethes, G

1991-02-01

The levels of the two primary bile acids, cholic acid (CA) and chenodeoxycholic acid (CDCA), were determined by radioimmunoassay in cattle, horse, pig and dog serum. The mean serum cholic acid (SCA) and deoxycholic acid (SCDCA) levels of cows varied with their reproductive status, being 7.8 (+/- 3.3) and 1.5 (+/- 1.0) mumol/l in dry cows, 17.8 (+/- 6.9) and 2.3 (+/- 1.0) mumol/l in freshly calved dams, and 15.8 (+/- 5.7) and 2.3 (+/- 0.8) mumol/l, respectively, in lactating cows. The SCA level found in the immediate prepartal period and also on the day of calving corresponded to those found during the dry period, then, they tended to rise 2 days after calving and attained the peak characteristic for freshly calved dams on day 3 or 4 post partum. Feed consumption had no influence on the serum levels of primary bile acids, and circadian variations of SCA and SCDCA were also negligible. Suckling calves had much lower SCA levels (2.3 (+/- 1.0) mumol/l before feeding than cows. This initial concentration rose to 10.3 (+/- 2.9) mumol/l 1 h after feeding and returned to 5.0 (+/- 2.1) mumol/l 3 h later. Like cows, horses showed no appreciate difference between pre- and post-feeding levels of SCA (2.2 (+/- 1.2) mumol/l) and SCDCA (1.1 (+/- 0.3) mumol/l). Unlike bovines, pigs and dogs showed a considerable increase in the serum levels of the primary bile acids after feeding.(ABSTRACT TRUNCATED AT 250 WORDS)

Genetic determinants and stroke in children with sickle cell disease,

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Daniela O.W. Rodrigues

Full Text Available Abstract Objective: To verify genetic determinants associated with stroke in children with sickle cell disease (SCD. Methods: Prospective cohort with 110 children submitted to neonatal screening by the Neonatal Screening Program, between 1998 and 2007, with SCD diagnosis, followed at a regional reference public service for hemoglobinopathies. The analyzed variables were type of hemoglobinopathy, gender, coexistence with alpha thalassemia (α-thal, haplotypes of the beta globin chain cluster, and stroke. The final analysis was conducted with 66 children with sickle cell anemia (SCA, using the chi-squared test in the program SPSS® version 14.0. Results: Among children with SCD, 60% had SCA. The prevalence of coexistence with α-thal was 30.3% and the Bantu haplotype (CAR was identified in 89.2%. The incidence of stroke was significantly higher in those with SCA (27.3% vs. 2.3%; p = 0.001 and males (24.1% vs. 9.6%; p = 0.044. The presence of α-thal (p = 0.196, the CAR haplotype (p = 0.543, and socioeconomic factors were not statistically significant in association with the occurrence of stroke. Conclusion: There is a high incidence of stroke in male children and in children with SCA. Coexistence with α-thal and haplotypes of the beta globin chain cluster did not show any significant association with stroke. The heterogeneity between previously evaluated populations, the non-reproducibility between studies, and the need to identify factors associated with stroke in patients with SCA indicate the necessity of conducting further research to demonstrate the relevance of genetic factors in stroke related to SCD.

PARN and TOE1 Constitute a 3′ End Maturation Module for Nuclear Non-coding RNAs

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Ahyeon Son

2018-04-01

Full Text Available Summary: Poly(A-specific ribonuclease (PARN and target of EGR1 protein 1 (TOE1 are nuclear granule-associated deadenylases, whose mutations are linked to multiple human diseases. Here, we applied mTAIL-seq and RNA sequencing (RNA-seq to systematically identify the substrates of PARN and TOE1 and elucidate their molecular functions. We found that PARN and TOE1 do not modulate the length of mRNA poly(A tails. Rather, they promote the maturation of nuclear small non-coding RNAs (ncRNAs. PARN and TOE1 act redundantly on some ncRNAs, most prominently small Cajal body-specific RNAs (scaRNAs. scaRNAs are strongly downregulated when PARN and TOE1 are compromised together, leading to defects in small nuclear RNA (snRNA pseudouridylation. They also function redundantly in the biogenesis of telomerase RNA component (TERC, which shares sequence motifs found in H/ACA box scaRNAs. Our findings extend the knowledge of nuclear ncRNA biogenesis, and they provide insights into the pathology of PARN/TOE1-associated genetic disorders whose therapeutic treatments are currently unavailable. : By analyzing the 3′ termini of transcriptome, Son et al. reveal the targets of PARN and TOE1, two nuclear deadenylases with disease associations. Both deadenylases are involved in nuclear small non-coding RNA maturation, but not in mRNA deadenylation. Their combined activity is particularly important for biogenesis of scaRNAs and TERC. Keywords: PARN, TOE1, CAF1Z, deadenylase, 3′ end maturation, adenylation, deadenylation, scaRNA, TERC

A Cost-Effectiveness Analysis of a Pilot Neonatal Screening Program for Sickle Cell Anemia in the Republic of Angola.

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McGann, Patrick T; Grosse, Scott D; Santos, Brigida; de Oliveira, Vysolela; Bernardino, Luis; Kassebaum, Nicholas J; Ware, Russell E; Airewele, Gladstone E

2015-12-01

To assess the cost-effectiveness of a pilot newborn screening (NBS) and treatment program for sickle cell anemia (SCA) in Luanda, Angola. In July 2011, a pilot NBS and treatment program was implemented in Luanda, Angola. Infants identified with SCA were enrolled in a specialized SCA clinic in which they received preventive care and sickle cell education. In this analysis, the World Health Organization (WHO) and generalized cost-effectiveness analysis methods were used to estimate gross intervention costs of the NBS and treatment program. To determine healthy life-years (HLYs) gained by screening and treatment, we assumed NBS reduced mortality to that of the Angolan population during the first 5 years based upon WHO and Global Burden of Diseases Study 2010 estimates, but provided no significant survival benefit for children who survive through age 5 years. A secondary sensitivity analysis with more conservative estimates of mortality benefits also was performed. The costs of downstream medical costs, including acute care, were not included. Based upon the costs of screening 36,453 infants and treating the 236 infants with SCA followed after NBS in the pilot project, NBS and treatment program is projected to result in the gain of 452-1105 HLYs, depending upon the discounting rate and survival assumptions used. The corresponding estimated cost per HLY gained is $1380-$3565, less than the gross domestic product per capita in Angola. These data demonstrate that NBS and treatment for SCA appear to be highly cost-effective across all scenarios for Angola by the WHO criteria. Copyright © 2015 Elsevier Inc. All rights reserved.

Modifications of resting state networks in spinocerebellar ataxia type 2.

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Cocozza, Sirio; Saccà, Francesco; Cervo, Amedeo; Marsili, Angela; Russo, Cinzia Valeria; Giorgio, Sara Maria Delle Acque; De Michele, Giuseppe; Filla, Alessandro; Brunetti, Arturo; Quarantelli, Mario

2015-09-01

We aimed to investigate the integrity of the Resting State Networks in spinocerebellar ataxia type 2 (SCA2) and the correlations between the modification of these networks and clinical variables. Resting-state functional magnetic resonance imaging (RS-fMRI) data from 19 SCA2 patients and 29 healthy controls were analyzed using an independent component analysis and dual regression, controlling at voxel level for the effect of atrophy by co-varying for gray matter volume. Correlations between the resting state networks alterations and disease duration, age at onset, number of triplets, and clinical score were assessed by Spearman's coefficient, for each cluster which was significantly different in SCA2 patients compared with healthy controls. In SCA2 patients, disruption of the cerebellar components of all major resting state networks was present, with supratentorial involvement only for the default mode network. When controlling at voxel level for gray matter volume, the reduction in functional connectivity in supratentorial regions of the default mode network, and in cerebellar regions within the default mode, executive and right fronto-parietal networks, was still significant. No correlations with clinical variables were found for any of the investigated resting state networks. The SCA2 patients show significant alterations of the resting state networks, only partly explained by the atrophy. The default mode network is the only resting state network that shows also supratentorial changes, which appear unrelated to the cortical gray matter volume. Further studies are needed to assess the clinical significance of these changes. © 2015 International Parkinson and Movement Disorder Society.

Deep Brain Stimulation for Tremor Associated with Underlying Ataxia Syndromes: A Case Series and Discussion of Issues

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Genko Oyama

2014-07-01

Full Text Available Background: Deep brain stimulation (DBS has been utilized to treat various symptoms in patients suffering from movement disorders such as Parkinson's disease, dystonia, and essential tremor. Though ataxia syndromes have not been formally or frequently addressed with DBS, there are patients with ataxia and associated medication refractory tremor or dystonia who may potentially benefit from therapy.Methods: A retrospective database review was performed, searching for cases of ataxia where tremor and/or dystonia were addressed by utilizing DBS at the University of Florida Center for Movement Disorders and Neurorestoration between 2008 and 2011. Five patients were found who had DBS implantation to address either medication refractory tremor or dystonia. The patient's underlying diagnoses included spinocerebellar ataxia type 2 (SCA2, fragile X associated tremor ataxia syndrome (FXTAS, a case of idiopathic ataxia (ataxia not otherwise specified [NOS], spinocerebellar ataxia type 17 (SCA17, and a senataxin mutation (SETX.Results: DBS improved medication refractory tremor in the SCA2 and the ataxia NOS patients. The outcome for the FXTAS patient was poor. DBS improved dystonia in the SCA17 and SETX patients, although dystonia did not improve in the lower extremities of the SCA17 patient. All patients reported a transient gait dysfunction postoperatively, and there were no reports of improvement in ataxia‐related symptoms.Discussion: DBS may be an option to treat tremor, inclusive of dystonic tremor in patients with underlying ataxia; however, gait and other symptoms may possibly be worsened.Erratum published on July 27, 2016

Airway disease in highway and tunnel construction workers exposed to silica.

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Oliver, L Christine; Miracle-McMahill, Heidi

2006-12-01

Construction workers employed in a unique type of tunnel construction known as tunnel jacking were exposed over an 18-month period to respirable crystalline silica at concentrations that exceeded the OSHA permissible exposure limit. The present study examines workplace exposures and occurrence of airway disease in these workers. Medical and occupational histories and chest radiographs were obtained on 343 active construction workers who had worked on the site during the period in question. Chest radiographs were interpreted according to the ILO-1980 system of classification. Standardized questions were used to develop an algorithm to define symptoms consistent with asthma (SCA) and to determine these respiratory outcomes: chronic bronchitis, shortness of breath (SOB), and physician-diagnosed asthma (current vs. not current). Relationships with each of three work activities were examined: slurry wall breakthrough (SWB), chipping caisson overpour, and tunneling/mining. Participants included laborers, carpenters, tunnel workers, ironworkers, operating engineers, and electricians. No cases of silicosis were found on chest X-ray. Overall prevalence of chronic bronchitis, SCA, SOB, and physician-diagnosed asthma was 10.7%, 25%, 29%, and 6.6%, respectively. Odds ratios (OR) for carpenters compared to laborers were significantly elevated for chronic bronchitis, SCA, and SOB. SWB was associated with chronic bronchitis and SCA (OR 4.93, 95% CI = 1.01, 24.17; OR 3.32, 95% CI = 1.25, 8.84, respectively). The interaction between SWB, SCA, and trade was significant for carpenters (OR 6.87, 95% CI = 1.66, 28.39). Inverse trends were observed for months on the site and chronic bronchitis, SCA, and SOB (P = 0.0374, 0.0006, and 0.0307, respectively). Tunnel construction workers exposed to respirable crystalline silica and cement dust are at increased risk for airway disease. Extent of risk varies by trade and work activity. Our data indicate the importance of bystander exposures and

Deregulation of the actin cytoskeleton and macropinocytosis in response to phorbol ester by the mutant protein kinase C gamma that causes spinocerebellar ataxia type 14

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Kazuhiro eYamamoto

2014-04-01

Full Text Available Several missense mutations in the protein kinase Cγ (γPKC gene have been found to cause spinocerebellar ataxia type 14 (SCA14, an autosomal dominant neurodegenerative disease. γPKC is a neuron-specific member of the classical PKCs and is activated and translocated to subcellular regions as a result of various stimuli, including diacylglycerol synthesis, increased intracellular Ca2+ and phorbol esters. We investigated whether SCA14 mutations affect the γPKC-related functions by stimulating HeLa cells with TPA (12-O-tetradecanoylpholbol 13-acetate, a type of phorbol ester. Wild-type (WT γPKC-GFP was translocated to the plasma membrane within 10 min of TPA stimulation, followed by its perinuclear translocation and cell shrinkage, in a PKC kinase activity- and microtubule-dependent manner. On the other hand, although SCA14 mutant γPKC-GFP exhibited a similar translocation to the plasma membrane, the subsequent perinuclear translocation and cell shrinkage were significantly impaired in response to TPA. Translocated WT γPKC colocalized with F-actin and formed large vesicular structures in the perinuclear region. The uptake of FITC-dextran, a marker of macropinocytosis, was promoted by TPA stimulation in cells expressing WT γPKC, and FITC-dextran was surrounded by γPKC-positive vesicles. Moreover, TPA induced the phosphorylation of MARCKS, which is a membrane-substrate of PKC, resulting in the translocation of phosphorylated MARCKS to the perinuclear region, suggesting that TPA induces macropinocytosis via γPKC activation. However, TPA failed to activate macropinocytosis and trigger the translocation of phosphorylated MARCKS in cells expressing the SCA14 mutant γPKC. These findings suggest that γPKC is involved in the regulation of the actin cytoskeleton and macropinocytosis in HeLa cells, while SCA14 mutant γPKC fails to regulate these processes due to its reduced kinase activity at the plasma membrane. This property might be involved in

Realizing effectiveness across continents with hydroxyurea: Enrollment and baseline characteristics of the multicenter REACH study in Sub-Saharan Africa.

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McGann, Patrick T; Williams, Thomas N; Olupot-Olupot, Peter; Tomlinson, George A; Lane, Adam; Luís Reis da Fonseca, José; Kitenge, Robert; Mochamah, George; Wabwire, Ham; Stuber, Susan; Howard, Thad A; McElhinney, Kathryn; Aygun, Banu; Latham, Teresa; Santos, Brígida; Tshilolo, Léon; Ware, Russell E

2018-08-01

Despite its well-described safety and efficacy in the treatment of sickle cell anemia (SCA) in high-income settings, hydroxyurea remains largely unavailable in sub-Saharan Africa, where more than 75% of annual SCA births occur and many comorbidities exist. Realizing Effectiveness Across Continents with Hydroxyurea (REACH, ClinicalTrials.gov NCT01966731) is a prospective, Phase I/II open-label trial of hydroxyurea designed to evaluate the feasibility, safety, and benefits of hydroxyurea treatment for children with SCA in four sub-Saharan African countries. Following comprehensive training of local research teams, REACH was approved by local Ethics Committees and achieved full enrollment ahead of projections with 635 participants enrolled over a 30-month period, despite half of families living >12 km from their clinical site. At enrollment, study participants (age 5.4 ± 2.4 years) had substantial morbidity, including a history of vaso-occlusive pain (98%), transfusion (68%), malaria (85%), and stroke (6%). Significant differences in laboratory characteristics were noted across sites, with lower hemoglobin concentrations (P < .01) in Angola (7.2 ± 1.0 g/dL) and the DRC (7.0 ± 0.9 g/dL) compared to Kenya (7.4 ± 1.1 g/dL) and Uganda (7.5 ± 1.1 g/dL). Analysis of known genetic modifiers of SCA demonstrated a high frequency of α-thalassemia (58.4% with at least a single α-globin gene deletion) and G6PD deficiency (19.7% of males and 2.4% of females) across sites. The CAR β-globin haplotype was present in 99% of participants. The full enrollment to REACH confirms the feasibility of conducting high-quality SCA research in Africa; this study will provide vital information to guide safe and effective dosing of hydroxyurea for children with SCA living in Africa. © 2018 Wiley Periodicals, Inc.

Quantification of spatial temporal variability of snow cover and hydro-climatic variables based on multi-source remote sensing data in the Swat watershed, Hindukush Mountains, Pakistan

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Anjum, Muhammad Naveed; Ding, Yongjian; Shangguan, Donghui; Liu, Junguo; Ahmad, Ijaz; Ijaz, Muhammad Wajid; Khan, Muhammad Imran

2018-02-01

The northern part of Hindukush Mountains has a perplexing environment due to the influence of adjacent mountains of Himalaya, Karakoram, and Tibetan Plateau. Although reliable evidences of climate change are available; however, a clear knowledge of snow cover dynamics in the context of climate change is missing for this region. In this study, we used various remotely sensed (TRMM precipitation product, while MODIS temperature and snow cover products) and gauge-based datasets to quantify the spatiotemporal variability of climatic variables and their turn effects over the snow cover area (SCA) and river discharge in the Swat watershed, northern Hindukush Mountains, Pakistan. The Mann-Kendall method and Sen's slope estimator were used to estimate the trends in SCA and hydro-climatic variables, at 5% significant level (P = 0.05). Results show that the winter and springs temperatures have increased (at the rate of 0.079 and 0.059 °C year-1, respectively), while decreasing in the summer and autumn (at the rate of 0.049 and 0.070 °C year-1, respectively). Basin-wide increasing tendency of precipitation was identified with a highest increasing rate of 3.563 mm year-1 in the spring season. A decreasing trend in the winter SCA (at the rate of -0.275% year-1) and increasing trends in other seasons were identified. An increasing tendency of river discharge on annual and seasonal scales was also witnessed. The seasonal variations in discharge showed significant positive and negative relationships with temperature and SCA, respectively. We conclude that the future variations in the temperature and SCA in the higher altitudes of the Swat watershed could substantially affect the seasonality of the river discharge. Moreover, it implies that the effect of ongoing global warming on the SCA in the snowmelt-dominated river basins needs to be considered for sustainable regional planning and management of water resources, hydropower production, and downstream irrigation scheduling.

The joint space-time statistics of macroweather precipitation, space-time statistical factorization and macroweather models

International Nuclear Information System (INIS)

Lovejoy, S.; Lima, M. I. P. de

2015-01-01

Over the range of time scales from about 10 days to 30–100 years, in addition to the familiar weather and climate regimes, there is an intermediate “macroweather” regime characterized by negative temporal fluctuation exponents: implying that fluctuations tend to cancel each other out so that averages tend to converge. We show theoretically and numerically that macroweather precipitation can be modeled by a stochastic weather-climate model (the Climate Extended Fractionally Integrated Flux, model, CEFIF) first proposed for macroweather temperatures and we show numerically that a four parameter space-time CEFIF model can approximately reproduce eight or so empirical space-time exponents. In spite of this success, CEFIF is theoretically and numerically difficult to manage. We therefore propose a simplified stochastic model in which the temporal behavior is modeled as a fractional Gaussian noise but the spatial behaviour as a multifractal (climate) cascade: a spatial extension of the recently introduced ScaLIng Macroweather Model, SLIMM. Both the CEFIF and this spatial SLIMM model have a property often implicitly assumed by climatologists that climate statistics can be “homogenized” by normalizing them with the standard deviation of the anomalies. Physically, it means that the spatial macroweather variability corresponds to different climate zones that multiplicatively modulate the local, temporal statistics. This simplified macroweather model provides a framework for macroweather forecasting that exploits the system's long range memory and spatial correlations; for it, the forecasting problem has been solved. We test this factorization property and the model with the help of three centennial, global scale precipitation products that we analyze jointly in space and in time

Quantitative effects of composting state variables on C/N ratio through GA-aided multivariate analysis

International Nuclear Information System (INIS)

Sun Wei; Huang, Guo H.; Zeng Guangming; Qin Xiaosheng; Yu Hui

2011-01-01

It is widely known that variation of the C/N ratio is dependent on many state variables during composting processes. This study attempted to develop a genetic algorithm aided stepwise cluster analysis (GASCA) method to describe the nonlinear relationships between the selected state variables and the C/N ratio in food waste composting. The experimental data from six bench-scale composting reactors were used to demonstrate the applicability of GASCA. Within the GASCA framework, GA searched optimal sets of both specified state variables and SCA's internal parameters; SCA established statistical nonlinear relationships between state variables and the C/N ratio; to avoid unnecessary and time-consuming calculation, a proxy table was introduced to save around 70% computational efforts. The obtained GASCA cluster trees had smaller sizes and higher prediction accuracy than the conventional SCA trees. Based on the optimal GASCA tree, the effects of the GA-selected state variables on the C/N ratio were ranged in a descending order as: NH 4 + -N concentration > Moisture content > Ash Content > Mean Temperature > Mesophilic bacteria biomass. Such a rank implied that the variation of ammonium nitrogen concentration, the associated temperature and the moisture conditions, the total loss of both organic matters and available mineral constituents, and the mesophilic bacteria activity, were critical factors affecting the C/N ratio during the investigated food waste composting. This first application of GASCA to composting modelling indicated that more direct search algorithms could be coupled with SCA or other multivariate analysis methods to analyze complicated relationships during composting and many other environmental processes. - Research Highlights: → A genetic algorithm aided stepwise cluster analysis method in food waste composting. → Nonlinear relationships between the selected state variables and the C/N ratio. → Introduced proxy tables save around 70% computational

Quantitative effects of composting state variables on C/N ratio through GA-aided multivariate analysis

Energy Technology Data Exchange (ETDEWEB)

Sun Wei [Faculty of Engineering and Applied Science, University of Regina, Regina, Saskatchewan, S4S 0A2 (Canada); Huang, Guo H., E-mail: huangg@iseis.org [Faculty of Engineering and Applied Science, University of Regina, Regina, Saskatchewan, S4S 0A2 (Canada); MOE Key Laboratory of Regional Energy Systems Optimization, Sino-Canada Energy and Environmental Research Academy, North China Electric Power University, Beijing, 102206 (China); Zeng Guangming [MOE Key Laboratory of Environmental Biology and Pollution Control, College of Environmental Science and Engineering, Hunan University, Changsha, Hunan, 410082 (China); Qin Xiaosheng [School of Civil and Environmental Engineering, Nanyang Technological University, 50 Nanyang Avenue, Singapore 639798 (Singapore); Yu Hui [Faculty of Engineering and Applied Science, University of Regina, Regina, Saskatchewan, S4S 0A2 (Canada)

2011-03-01

It is widely known that variation of the C/N ratio is dependent on many state variables during composting processes. This study attempted to develop a genetic algorithm aided stepwise cluster analysis (GASCA) method to describe the nonlinear relationships between the selected state variables and the C/N ratio in food waste composting. The experimental data from six bench-scale composting reactors were used to demonstrate the applicability of GASCA. Within the GASCA framework, GA searched optimal sets of both specified state variables and SCA's internal parameters; SCA established statistical nonlinear relationships between state variables and the C/N ratio; to avoid unnecessary and time-consuming calculation, a proxy table was introduced to save around 70% computational efforts. The obtained GASCA cluster trees had smaller sizes and higher prediction accuracy than the conventional SCA trees. Based on the optimal GASCA tree, the effects of the GA-selected state variables on the C/N ratio were ranged in a descending order as: NH{sub 4}{sup +}-N concentration > Moisture content > Ash Content > Mean Temperature > Mesophilic bacteria biomass. Such a rank implied that the variation of ammonium nitrogen concentration, the associated temperature and the moisture conditions, the total loss of both organic matters and available mineral constituents, and the mesophilic bacteria activity, were critical factors affecting the C/N ratio during the investigated food waste composting. This first application of GASCA to composting modelling indicated that more direct search algorithms could be coupled with SCA or other multivariate analysis methods to analyze complicated relationships during composting and many other environmental processes. - Research Highlights: {yields} A genetic algorithm aided stepwise cluster analysis method in food waste composting. {yields} Nonlinear relationships between the selected state variables and the C/N ratio. {yields} Introduced proxy tables

Estimation of Heterosis and Combining Ability in F1 Hybrids of Upland Cotton for Yield and Fiber Traits

International Nuclear Information System (INIS)

Arain, B.T.; Baloch, M.J.; Bughio, Q.U.A.; Sial, P.; Arain, M.A.; Baloch, A.

2015-01-01

The experimental research was conducted so as to determine the general combining ability (GCA) and specific combining ability (SCA) estimates and heterotic effects for seed cotton yield and fibre traits in 5 x 5 half diallel crosses of upland cotton (Gossypium hirsutum L.). The parental genotypes studied were; CRIS-134, IR-3701, IR-1524, FH-113 and MG-6. The characters such as bolls/plant, sympodial branches/plant, boll weight (g), plant height (cm), fibre length (mm), seed cotton yield/plant (g), seed index (g) and ginning out turn percentage were studied. The experiment was laid-out in a randomized complete block design with four replications at experimental field of the Department of Plant Breeding and Genetics, Sindh Agriculture University Tandojam, Pakistan during 2013. The results revealed that, parents and hybrids differed significantly for their mean performance regarding all the traits studied. The importance of heterotic effects was evident from the significance of parents vs. hybrids performance. The variances due to GCA and SCA were significant for all the traits except that GCA was non-significant for boll weight only whereas, SCA was non-significant for boll weight, seed index and ginning out turn percentage. The significance of GCA indicated the importance of additive genes advocating the traits while, the involvement of non-additive genes was evident from the significance of SCA variances. The GCA variances were greater than SCA for bolls per plant, plant height, seed cotton yield and lint percentage while, SCA variances were higher than GCA for sympodial branches/plant and fibre length. Parents IR-3701, FH-113 and MG-6 displayed higher positive GCA effects for bolls/plant, sympodial branches/plant, fibre length, seed cotton yield, seed index and ginning out turn percentage. The per se performance of these three parents was exactly reflected in their GCA effects and such happenings are exceptional. Such results suggested that, all three parents were

Impact parameter dependence of inner-shell vacancy production in fast ion--atom collisions

International Nuclear Information System (INIS)

Randall, R.R.

1975-01-01

The impact parameter dependence of the probability for production of K x rays has been measured for oxygen projectiles on copper, chlorine projectiles on aluminum, titanium and copper, and carbon and fluorine projectiles on argon at scaled velocities near 0.5. The O + Cu data was taken at incident energies of 1.56, 1.88 and 2.69 MeV/amu for the O bombardment of thin Cu foils. The Cl ions had incident energies of 0.6 and 0.85 MeV/amu upon thin foils of Al, Ti, and Cu. A thin Ar gas target was used for 1.58 MeV/amu C and F beams, permitting measurements to be made for charge-pure C 4+ , C 6+ , F 5+ and F 9+ projectiles. Cu, Cl and Ar K x rays were observed with a Si(Li) detector and scattered particles were counted using a masked surface-barrier detector. Comparison of the shapes of the measured probability curves with predictions of the semiclassical Coulomb approximation (SCA) shows adequate agreement for the O + Cu system. For the higher ratio of projectile to target nuclear charge (Z 1 /Z 2 ) of the Cl + Al, Ti, Cu and C, F + Ar systems, the SCA and Brinkman--Kramers (BK) model for charge transfer fail to predict the measured curves. In particular, the SCA and BK fail to account for large vacancy production probabilities at large impact parameters (larger than the Slater-screened Bohr radii of the K electrons). Further, the dependence of the shapes of the measured curves on the charge state of the incident projectile is pronounced for the cases having the larger Z 1 /Z 2 values. Alternative models are discussed in an attempt to account for the observed behavior

On the need for a time- and location-dependent estimation of the NDSI threshold value for reducing existing uncertainties in snow cover maps at different scales

Science.gov (United States)

Härer, Stefan; Bernhardt, Matthias; Siebers, Matthias; Schulz, Karsten

2018-05-01

Knowledge of current snow cover extent is essential for characterizing energy and moisture fluxes at the Earth's surface. The snow-covered area (SCA) is often estimated by using optical satellite information in combination with the normalized-difference snow index (NDSI). The NDSI thereby uses a threshold for the definition if a satellite pixel is assumed to be snow covered or snow free. The spatiotemporal representativeness of the standard threshold of 0.4 is however questionable at the local scale. Here, we use local snow cover maps derived from ground-based photography to continuously calibrate the NDSI threshold values (NDSIthr) of Landsat satellite images at two European mountain sites of the period from 2010 to 2015. The Research Catchment Zugspitzplatt (RCZ, Germany) and Vernagtferner area (VF, Austria) are both located within a single Landsat scene. Nevertheless, the long-term analysis of the NDSIthr demonstrated that the NDSIthr at these sites are not correlated (r = 0.17) and different than the standard threshold of 0.4. For further comparison, a dynamic and locally optimized NDSI threshold was used as well as another locally optimized literature threshold value (0.7). It was shown that large uncertainties in the prediction of the SCA of up to 24.1 % exist in satellite snow cover maps in cases where the standard threshold of 0.4 is used, but a newly developed calibrated quadratic polynomial model which accounts for seasonal threshold dynamics can reduce this error. The model minimizes the SCA uncertainties at the calibration site VF by 50 % in the evaluation period and was also able to improve the results at RCZ in a significant way. Additionally, a scaling experiment shows that the positive effect of a locally adapted threshold diminishes using a pixel size of 500 m or larger, underlining the general applicability of the standard threshold at larger scales.

Small Aircraft Transportation System, Higher Volume Operations Concept: Off-Nominal Operations

Science.gov (United States)

Abbott, Terence S.; Consiglio, Maria C.; Baxley, Brian T.; Williams, Daniel M.; Conway, Sheila R.

2005-01-01

This document expands the Small Aircraft Transportation System, (SATS) Higher Volume Operations (HVO) concept to include off-nominal conditions. The general philosophy underlying the HVO concept is the establishment of a newly defined area of flight operations called a Self-Controlled Area (SCA). During periods of poor weather, a block of airspace would be established around designated non-towered, non-radar airports. Aircraft flying enroute to a SATS airport would be on a standard instrument flight rules flight clearance with Air Traffic Control providing separation services. Within the SCA, pilots would take responsibility for separation assurance between their aircraft and other similarly equipped aircraft. Previous work developed the procedures for normal HVO operations. This document provides details for off-nominal and emergency procedures for situations that could be expected to occur in a future SCA.

Supported Conversation for hospital staff

DEFF Research Database (Denmark)

Forchhammer, Hysse B; Løvholt, Annelise P.; Mathiesen, Lone Lundbak

in communication and interaction, Supported Conversation for Adults with Aphasia (SCA) was adapted and implemented in a large neurological department at Rigshospitalet-Glostrup in Copenhagen. Method 152 staff members representing different health professionals were assigned to one of eleven courses during a six...... month period. Each course had 10-12 participants and lasted 6 hours, including instruction in the SCA principles, video analysis, interdisciplinary group work, and practice sessions with PWAs. Self-assessed learning outcomes were evaluated with a brief questionnaire filled out by staff members...... in communication, also showed significant improvements across all staff groups. After the course, more time to spend with patients was perceived as the most important factor to further increase communication success with PWA. Conclusion The results show that interdisciplinary SCA-courses successfully increase...

Real forms of non-linear superconformal and quasi-superconformal algebras and their unified realization

International Nuclear Information System (INIS)

Bina, B.; Guenaydin, M.

1997-01-01

We give a complete classification of the real forms of simple non-linear superconformal algebras (SCA) and quasi-superconformal algebras (QSCA) and present a unified realization of these algebras with simple symmetry groups. This classification is achieved by establishing a correspondence between simple non-linear QSCA's and SCA's and quaternionic and super-quaternionic symmetric spaces of simple Lie groups and Lie supergroups, respectively. The unified realization we present involves a dimension zero scalar field (dilaton), dimension-1 symmetry currents, and dimension-1/2 free bosons for QSCA's and dimension-1/2 free fermions for SCA's. The free bosons and fermions are associated with the quaternionic and super-quaternionic symmetric spaces of corresponding Lie groups and Lie supergroups, respectively. We conclude with a discussion of possible applications of our results. (orig.)

Hormonal and echocardiographic abnormalities in adult patients with sickle-cell anemia in Bahrain

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Garadah TS

2016-12-01

Full Text Available Taysir S Garadah,1,2 Ahmed A Jaradat,3 Mohammed E Alalawi,1 Adla B Hassan2 1Cardiac Unit, Salmaniya Medical Complex, Ministry of Health, 2Department of Internal Medicine, College of Medicine and Medical Sciences, Arabian Gulf University, Manama, 3Department of Family and Community Medicine, Arabian Gulf University, Manama, Bahrain Background: Adrenal, thyroid, and parathyroid gland hormonal changes are recognized in children with homozygous (HbSS sickle-cell anemia (SCA, but are not clear in adult patients with SCA. Aim: To assess the metabolic and endocrine abnormalities in adult patients with SCA and evaluate left ventricular (LV systolic and diastolic functions compared with patients with no SCA and further study the relationship between serum levels of cortisol, free thyroxine (T4, and testosterone with serum ferritin. Materials and methods: The study was conducted on 82 patients with adult HbSS SCA compared with a sex- and age-matched control group. The serum levels of cortisol, parathyroid hormone (PTH, testosterone, thyroid-stimulating hormone (TSH, and free T4 were compared. Blood levels of hemoglobin, reticulocyte count, lactate dehydrogenase (LDH, calcium, alkaline phosphatase (ALP, vitamin D3, and ferritin were also compared. Pulsed Doppler echo was performed to evaluate the LV mass, wall thickness, and cavity dimensions with diastolic filling velocities of early (E and atria (A waves. Biometric data were analyzed as mean ± standard deviation between the two groups. Multiple regression analysis was performed between serum levels of ferritin as independent variable and testosterone, cortisol, and thyroid hormones. Results: A total of 82 adult patients with HbSS SCA were enrolled who had a mean age of 21±5.7 years, with 51 males (62%. Patients with SCA compared with the control group had significantly lower hemoglobin, body mass index, cortisol, vitamin D3, testosterone, and T4. Furthermore, there were significantly high levels of

Germ-line CAG repeat instability causes extreme CAG repeat expansion with infantile-onset spinocerebellar ataxia type 2

DEFF Research Database (Denmark)

Vinther-Jensen, Tua; Ek, Jakob; Duno, Morten

2013-01-01

The spinocerebellar ataxias (SCA) are a genetically and clinically heterogeneous group of diseases, characterized by dominant inheritance, progressive cerebellar ataxia and diverse extracerebellar symptoms. A subgroup of the ataxias is caused by unstable CAG-repeat expansions in their respective ...... of paternal germ-line repeat sequence instability of the expanded SCA2 locus.European Journal of Human Genetics advance online publication, 10 October 2012; doi:10.1038/ejhg.2012.231....

Estimation of malondialdehyde levels in serum and saliva of children affected with sickle cell anemia

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Sudhindra Baliga

2018-01-01

Full Text Available Background: Sickle cell anemia (SCA is an inherited disorder of hemoglobin synthesis characterized by deformed erythrocytes. Hemoglobin S present in sickle-shaped erythrocytes exhibits an enhanced rate of auto-oxidation compared with normal hemoglobin A. It produces more of reactive oxygen species (ROS which promotes oxidatively stressed environment. ROS degrade the membranes of sickle cell erythrocytes composed of polyunsaturated lipids and form malondialdehyde (MDA as a by-product. Aim: The aim of the study is to evaluate and compare the MDA levels of serum and saliva in SCA patients. Design: A total of 150 children aged 4–12 years were divided into two groups: Group A (n = 75 consisting of children suffering from SCA and Group B (n = 75 consisting of healthy children. Blood and saliva samples were collected aseptically from both the groups, and they were subjected to thiobarbituric acid assay. Absorbance was evaluated spectrophotometrically at 531 nm, and the values of concentration of MDA were derived. Results: The mean MDA levels in serum and saliva were 8.9825 ± 1.04 and 0.5152 ± 0.28, respectively, in Group A and they were found to be higher than mean MDA levels of serum (5.87 ± 0.92 and saliva (0.2929 ± 0.06 of Group B and the difference of their mean was found to be statistically significant. Conclusion: A significant correlation of the MDA was found in saliva and serum of the patients with SCA. This finding suggests that saliva can be effectively used as a noninvasive alternative for assessing the oxidative stress in patients with SCA.

Mammalian small nucleolar RNAs are mobile genetic elements.

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Michel J Weber

2006-12-01

Full Text Available Small nucleolar RNAs (snoRNAs of the H/ACA box and C/D box categories guide the pseudouridylation and the 2'-O-ribose methylation of ribosomal RNAs by forming short duplexes with their target. Similarly, small Cajal body-specific RNAs (scaRNAs guide modifications of spliceosomal RNAs. The vast majority of vertebrate sno/scaRNAs are located in introns of genes transcribed by RNA polymerase II and processed by exonucleolytic trimming after splicing. A bioinformatic search for orthologues of human sno/scaRNAs in sequenced mammalian genomes reveals the presence of species- or lineage-specific sno/scaRNA retroposons (sno/scaRTs characterized by an A-rich tail and an approximately 14-bp target site duplication that corresponds to their insertion site, as determined by interspecific genomic alignments. Three classes of snoRTs are defined based on the extent of intron and exon sequences from the snoRNA parental host gene they contain. SnoRTs frequently insert in gene introns in the sense orientation at genomic hot spots shared with other genetic mobile elements. Previously characterized human snoRNAs are encoded in retroposons whose parental copies can be identified by phylogenic analysis, showing that snoRTs can be faithfully processed. These results identify snoRNAs as a new family of mobile genetic elements. The insertion of new snoRNA copies might constitute a safeguard mechanism by which the biological activity of snoRNAs is maintained in spite of the risk of mutations in the parental copy. I furthermore propose that retroposition followed by genetic drift is a mechanism that increased snoRNA diversity during vertebrate evolution to eventually acquire new RNA-modification functions.

Double-blind crossover trial of trimethoprim-sulfamethoxazole in spinocerebellar ataxia type 3/Machado-Joseph disease.

Science.gov (United States)

Schulte, T; Mattern, R; Berger, K; Szymanski, S; Klotz, P; Kraus, P H; Przuntek, H; Schöls, L

2001-09-01

To evaluate the efficiency of a combination of trimethoprim and sulfamethoxazole in patients with spinocerebellar ataxia type 3/Machado-Joseph disease (SCA3/MJD). Placebo-controlled, double-blind crossover trial in 22 patients with genetically confirmed SCA3/MJD. Study phases of 6 months were separated by a washout period of 4 weeks. Dosages were a combination of trimethoprim, 160 mg, and sulfamethoxazole, 800 mg, twice daily for 2 weeks, followed by a combination of trimethoprim, 80 mg, and sulfamethoxazole, 400 mg, twice daily for 5.5 months. Outpatient department of the Neurological Clinic, Ruhr-University, Bochum, Germany. Ataxia ranking scale, self-assessment score, static posturography, and results of motor performance testing. Effects on the visual system were studied using the achromatic Vision Contrast Test System and the Farnsworth-Munsell 100-hue test for color discrimination. Physical and mental health were documented using the Medical Outcomes Study 36-Item Short-Form Health Survey. Subgroup analyses assessed the influence of age, sex, age at onset, duration of the disease, phenotype, and CAG repeat length on test performance. Twenty of 22 patients completed the study. Dropouts were due to a rash (placebo phase) and an attempted suicide in a family conflict. Trimethoprim-sulfamethoxazole therapy had no significant effect in SCA3/MJD patients in the short-term analysis (2 weeks) or in the long-term interval (6 months). In contrast to previous reports that studied smaller groups of patients, treatment with trimethoprim-sulfamethoxazole did not improve the diverse and complex movement disorders caused by SCA3/MJD. Trimethoprim-sulfamethoxazole had no effect on the visual system and cannot be recommended as a continuous treatment for SCA3/MJD patients.

Mangifera indica L. extract (Vimang improves the aversive memory in spinocerebellar ataxia type 2 transgenic mice.

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Natasha Maurmann

2014-06-01

Full Text Available Context: The spinocerebellar ataxia type 2 (SCA-2 is a progressive neurodegenerative disorder without specific therapy identified, and it is related to the loss of function in the cerebellum, mitochondrial dysfunction, oxidative stress and neurotoxic processes. Scientific evidence indicates that Mangifera indica L. aqueous extract (MiE and its major constituent (mangiferin display antioxidant, anti-inflammatory and neuroprotective actions. Aims: To investigate the MiE and mangiferin effects on behavioral outcomes of neurological function in SCA-2 transgenic mice. Methods: The SCA-2 transgenic mice were daily and orally administered during 12 months with MiE (10, 50, and 100 mg/kg, mangiferin (10 mg/kg or vehicle. It was evaluated locomotion (open-field, aversive memory (inhibitory avoidance and declarative memory (object recognition. To explore possible cellular mechanisms underlying the in vivo effects was also evaluated their effects on nerve grow factor (NGF and tumor necrosis factor-α (TNF-α levels in the human glioblastoma cell line U138-MG supernatant. Results: MiE administration did not affect the object recognition memory, but mangiferin did. The natural extract improved selectively the aversive memory in SCA-2 mice, indicating that MiE can affect behavioral parameters regarding fear-related memory. MiE also induced a significant increase in supernatant levels of NGF and TNF-α in vitro in human U138-MG glioblastoma cells. Conclusions: The results suggest that MiE enhances the aversive memory through a mechanism that might involve an increase in neurotrophin and cytokine levels. These findings constitute the basis for the use of the natural extract in the prevention/treatment of memory deficits in SCA-2.

Etiology of Sudden Cardiac Arrest and Death in US Competitive Athletes: A 2-Year Prospective Surveillance Study.

Science.gov (United States)

Peterson, Danielle F; Siebert, David M; Kucera, Kristen L; Thomas, Leah Cox; Maleszewski, Joseph J; Lopez-Anderson, Martha; Suchsland, Monica Z; Harmon, Kimberly G; Drezner, Jonathan A

2018-04-09

To determine the etiology of sudden cardiac arrest and death (SCA/D) in competitive athletes through a prospective national surveillance program. Sudden cardiac arrest and death cases in middle school, high school, college, and professional athletes were identified from July 2014 to June 2016 through traditional and social media searches, reporting to the National Center for Catastrophic Sports Injury Research, communication with state and national high school associations, review of the Parent Heart Watch database, and search of student-athlete deaths on the NCAA Resolutions List. Autopsy reports and medical records were reviewed by a multidisciplinary panel to determine the underlying cause. US competitive athletes with SCA/D. Etiology of SCA/D. A total of 179 cases of SCA/D were identified (74 arrests with survival, 105 deaths): average age 16.6 years (range 11-29), 149 (83.2%) men, 94 (52.5%) whites, and 54 (30.2%) African American. One hundred seventeen (65.4%) had an adjudicated diagnosis, including 83 deaths and 34 survivors. The most common etiologies included hypertrophic cardiomyopathy (19, 16.2%), coronary artery anomalies (16, 13.7%), idiopathic left ventricular hypertrophy/possible cardiomyopathy (13, 11.1%), autopsy-negative sudden unexplained death (8, 6.8%), Wolff-Parkinson-White (8, 6.8%), and long QT syndrome (7, 6.0%). Hypertrophic cardiomyopathy was more common in male basketball (23.3%), football (25%), and African American athletes (30.3%). An estimated 56.4% of cases would likely demonstrate abnormalities on an electrocardiogram. The etiology of SCA/D in competitive athletes involves a wide range of clinical disorders. More robust reporting mechanisms, standardized autopsy protocols, and accurate etiology data are needed to better inform prevention strategies.

HYDROXYUREA TREATMENT DECREASES GLOMERULAR HYPERFILTRATION IN CHILDREN WITH SICKLE CELL ANEMIA

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Aygun, Banu; Mortier, Nicole A.; Smeltzer, Matthew P.; Shulkin, Barry L.; Hankins, Jane S.; Ware, Russell E.

2015-01-01

Background Glomerular hyperfiltration and microalbuminuria/proteinuria are early manifestations of sickle nephropathy. The effects of hydroxyurea therapy on these renal manifestations of sickle cell anemia (SCA) are not well defined. Objective To investigate the effects of hydroxyurea on glomerular filtration rate (GFR) measured by 99mTc-DTPA clearance, and on microalbuminuria/proteinuria in children with SCA. Study Design Hydroxyurea Study of Long-Term Effects (HUSTLE) is a prospective study (NCT00305175) with the goal of describing the long-term cellular, molecular, and clinical effects of hydroxyurea therapy in SCA. Glomerular filtration rate, urine microalbumin, and serum cystatin C were measured before initiating hydroxyurea therapy and then repeated after 3 years. Baseline and Year 3 values for HUSTLE subjects were compared using the Wilcoxon Signed Rank test. Associations between continuous variables were evaluated using Spearman correlation coefficient. Results Twenty-three children with SCA (median age 7.5 years, range 2.5–14.0 years) received hydroxyurea at maximum tolerated dose (MTD, 24.4 ± 4.5 mg/kg/day, range 15.3–30.6 mg/kg/day). After three years of treatment, GFR measured by 99mTc-DTPA decreased significantly from 167 ± 46 mL/min/1.73m2 to 145 ± 27 mL/min/1.73m2 (p=0.016). This decrease in GFR was significantly associated with increase in fetal hemoglobin (p= 0.042) and decrease in lactate dehydrogenase levels (p=0.035). Urine microalbumin and cystatin C levels did not change significantly. Conclusions Hydroxyurea at MTD is associated with a decrease in hyperfiltration in young children with SCA. PMID:23255310

Cardiac events in football and strategies for first-responder treatment on the field.

Science.gov (United States)

Schmied, Christian; Drezner, Jonathan; Kramer, Efraim; Dvorak, Jiri

2013-12-01

The incidence and outcomes of sudden cardiac arrest (SCA) and global strategies for prevention of sudden cardiac death (SCD) in football are not known. The aim of this study was to estimate the occurrence of cardiac events in football and to investigate the preventive measures taken among the Fédération International de Football Association (FIFA) member associations internationally. A questionnaire was sent to the member associations of FIFA. The first section addressed the previous events of SCA, SCD or unexplained sports-related sudden death within the last 10 years. Further questions focused on football player medical screening strategies and SCA resuscitation response protocols on the field. 126 of 170 questionnaires were returned (response rate 74.1%), and 103 questionnaires (60.6%) were completed sufficiently to include in further analysis. Overall, 107 cases of SCA/SCD and 5 unexplained football-associated sudden deaths were reported. These events occurred in 52 of 103 responding associations (50.5%). 23 of 112 (20.5%) footballers survived. 12 of 22 (54.5%) players treated with an available automated external defibrillators (AED) on the pitch survived. A national registry to monitor cardiac events was established in only 18.4% of the associations. Most associations (85.4%) provide regular cardiac screening for their national teams while 75% screen teams of the national leagues. An AED is available at all official matches in 68% of associations. National registries to accurately measure SCA/SCD in football are rare and greatly needed. Deficiencies in emergency preparations, undersupply of AEDs on the field during matches, and variability in resuscitation response protocols and training of team-staff members should be addressed to effectively prevent SCD in football.

Genetic determinants and stroke in children with sickle cell disease.

Science.gov (United States)

Rodrigues, Daniela O W; Ribeiro, Luiz C; Sudário, Lysla C; Teixeira, Maria T B; Martins, Marina L; Pittella, Anuska M O L; Junior, Irtis de O Fernandes

To verify genetic determinants associated with stroke in children with sickle cell disease (SCD). Prospective cohort with 110 children submitted to neonatal screening by the Neonatal Screening Program, between 1998 and 2007, with SCD diagnosis, followed at a regional reference public service for hemoglobinopathies. The analyzed variables were type of hemoglobinopathy, gender, coexistence with alpha thalassemia (α-thal), haplotypes of the beta globin chain cluster, and stroke. The final analysis was conducted with 66 children with sickle cell anemia (SCA), using the chi-squared test in the program SPSS ® version 14.0. Among children with SCD, 60% had SCA. The prevalence of coexistence with α-thal was 30.3% and the Bantu haplotype (CAR) was identified in 89.2%. The incidence of stroke was significantly higher in those with SCA (27.3% vs. 2.3%; p=0.001) and males (24.1% vs. 9.6%; p=0.044). The presence of α-thal (p=0.196), the CAR haplotype (p=0.543), and socioeconomic factors were not statistically significant in association with the occurrence of stroke. There is a high incidence of stroke in male children and in children with SCA. Coexistence with α-thal and haplotypes of the beta globin chain cluster did not show any significant association with stroke. The heterogeneity between previously evaluated populations, the non-reproducibility between studies, and the need to identify factors associated with stroke in patients with SCA indicate the necessity of conducting further research to demonstrate the relevance of genetic factors in stroke related to SCD. Copyright © 2016 Sociedade Brasileira de Pediatria. Published by Elsevier Editora Ltda. All rights reserved.

COMPARISON OF REAL-TIME MICROVASCULAR ABNORMALITIES IN PEDIATRIC AND ADULT SICKLE CELL ANEMIA PATIENTS

Science.gov (United States)

Cheung, Anthony T.W.; Miller, Joshua W.; Craig, Sarah M.; To, Patricia L.; Lin, Xin; Samarron, Sandra L.; Chen, Peter C.Y.; Zwerdling, Theodore; Wun, Ted; Li, Chin-Shang; Green, Ralph

2010-01-01

The conjunctival microcirculation in 14 pediatric and 8 adult sickle cell anemia (SCA) patients was studied using computer-assisted intravital microscopy. The bulbar conjunctiva in SCA patients in both age groups exhibited a blanched/avascular appearance characterized by decreased vascularity. SCA patients from both age groups had many of the same abnormal morphometric {vessel diameter, vessel distribution, morphometry (shape), tortuosity, arteriole:venule (A:V) ratio, and hemosiderin deposits} and dynamic {vessel sludging/sludged flow, boxcar blood (trickled) flow and abnormal flow velocity} abnormalities. A severity index (SI) was computed to quantify the degree of vasculopathy for comparison between groups. The severity of vasculopathy differed significantly between the pediatric and adult patients (SI: 4.2 ± 1.8 vs 6.6 ± 2.4; p=0.028), indicative of a lesser degree of overall severity in the pediatric patients. Specific abnormalities that were less prominent in the pediatric patients included abnormal vessel morphometry and tortuosity. Sludged flow, abnormal vessel distribution, abnormal A:V ratio, and boxcar flow, appeared in high prevalence in both age groups. The results indicate that SCA microvascular abnormalities develop in childhood and the severity of vasculopathy likely progresses with age. Intervention and effective treatment/management modalities should target pediatric patients to ameliorate, slow down or prevent progressive microvascular deterioration. PMID:20872552

Dietary risk factors for colorectal cancer in Brazil: a case control study.

Science.gov (United States)

Angelo, Sandro Nunes; Lourenço, Gustavo J; Magro, Daniéla O; Nascimento, Helvia; Oliveira, Rogério A; Leal, Raquel F; Ayrizono, Maria de Lourdes S; Fagundes, João J; Coy, Claudio S R; Lima, Carmen S P

2016-02-27

High meat intake and low consumption of vegetables, fruits and whole grains have been associated with increased risk of colorectal cancer in some relevant cohort studies conducted in distinct ethnic populations. The role of the dietary pattern on the risk of sporadic colorectal adenocarcinoma (SCA) in Brazil is unknown; therefore, it was the aim of the present study. The dietary patterns of 169 patients with SCA and 101 controls were analysed by food frequency recall. Crude odds ratios were calculated and given within 95 % confidence intervals. Patients reported higher average intakes of beef (32.0 ± 1.8 versus 23.7 ± 1.6, P = 0.0069), chicken (18.1 ± 0.9 versus 12.2 ± 0.8, P = 0.0002), and pork (8.9 ± 0.9 versus 3.4 ± 0.5, P < 0.0001). These individuals had a 1.025, 1.069, and 1.121-fold increased risk of SCA. Similar consumption of fish, vegetables, fruits and whole grains was reported by patients and controls. Meat consumption is greater in patients with SCA in the Brazilian population. Considering the study population - characterized by ethnic heterogeneity -, the environmental factor related to food habits may be associated with higher incidence of this disease in Brazil.

Molecular genetics of a Chinese family with spinocerebellar ataxia

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Dan-dan WU

2015-10-01

Full Text Available Objective　To study the genotype of the members of a Chinese family with spinocerebellar ataxia (SCA. Methods　The peripheral blood samples of 6 patients and 40 asymptomatic people belonged to the family were collected. Referring to the clinical manifestations of the proband and second-generation sequencing results, the CAG trinucleotide repeats of the pathogenic gene ATXN2 were amplified by polymerase chain reaction (PCR. The repeated times of the trinucleotide in normally and abnormally amplified alleles were defined by agarose gel electrophoresis and PCR products sequencing. Results　Autosomal dominant heredity was the cause of the SCA in this family. Six out of 46 in the fourth-generation were SCA2 patients, 7 were the carriers of pathogenic allele. The repeated times of CAG trinucleotide were within the normal range in one of the two alleles of ATXN2, but they were in abnormal range in the another one. The repeated times of CAG trinucleotide were 40-46 in abnormal alleles of patients. Conclusion　Autosomal dominant heredity SCA2 has been diagnosed in this family caused by the dynamic nutation of CAG trinucleotide repeats, and 7 pathogenic allele carriers in this family were confirmed by genetic diagnosis. DOI: 10.11855/j.issn.0577-7402.2015.08.07

How I use hydroxyurea to treat young patients with sickle cell anemia

Science.gov (United States)

2010-01-01

Hydroxyurea has many characteristics of an ideal drug for sickle cell anemia (SCA) and provides therapeutic benefit through multiple mechanisms of action. Over the past 25 years, substantial experience has accumulated regarding its safety and efficacy for patients with SCA. Early proof-of-principle studies were followed by prospective phase 1/2 trials demonstrating efficacy in affected adults, then adolescents and children, and more recently infants and toddlers. The phase 3 National Heart, Lung and Blood Institute–sponsored Multicenter Study of Hydroxyurea trial proved clinical efficacy for preventing acute vaso-occlusive events in severely affected adults. Based on this cumulative experience, hydroxyurea has emerged as an important therapeutic option for children and adolescents with recurrent vaso-occlusive events; recent evidence documents sustained long-term benefits with prevention or reversal of chronic organ damage. Despite abundant evidence for its efficacy, however, hydroxyurea has not yet translated into effective therapy for SCA. Because many healthcare providers have inadequate knowledge about hydroxyurea, patients and families are not offered treatment or decline because of unrealistic fears. Limited support for hydroxyurea by lay organizations and inconsistent medical delivery systems also contribute to underuse. Although questions remain regarding its long-term risks and benefits, current evidence suggests that many young patients with SCA should receive hydroxyurea treatment. PMID:20223921

Tensor exchange amplitudes in K +- N charge exchange reactions

International Nuclear Information System (INIS)

Svec, M.

1979-01-01

Tensor (A 2 ) exchange amplitudes in K +- N charge exchange (CEX) are constructed from the K +- N CEX data supplemented by information on the vector (rho) exchange amplitudes from πN sca tering. We observed new features in the t-structure of A 2 exchange amplitudes which contradict the t-de pendence anticipated by most of the Regge models. The results also provide evidence for violation of weak exchange degeneracy

Interview with Warren Wiscombe on scientific programing and his contributions to atmospheric science tool making

OpenAIRE

Flatau, Piotr J.

2013-01-01

On March 11, 2013 I talked with Warren Wiscombe about his contributions to scientific computer programming, atmospheric science and radiative transfer. Our conversation is divided into three parts related to light scattering, radiative transfer and his general thoughts about scientific programming. There are some reflections on how radiative transfer parameterizations gradually sneaked in to modern climate and atmospheric Global Circulation Models. Why some software programs such as light sca...

Recent Successes and Future Plans for NASA's Space Communications and Navigation Testbed on the International Space Station

Science.gov (United States)

Reinhart, Richard C.; Sankovic, John M.; Johnson, Sandra K.; Lux, James P.; Chelmins, David T.

2014-01-01

Flexible and extensible space communications architectures and technology are essential to enable future space exploration and science activities. NASA has championed the development of the Space Telecommunications Radio System (STRS) software defined radio (SDR) standard and the application of SDR technology to reduce the costs and risks of using SDRs for space missions, and has developed an on-orbit testbed to validate these capabilities. The Space Communications and Navigation (SCaN) Testbed (previously known as the Communications, Navigation, and Networking reConfigurable Testbed (CoNNeCT)) is advancing SDR, on-board networking, and navigation technologies by conducting space experiments aboard the International Space Station. During its first year(s) on-orbit, the SCaN Testbed has achieved considerable accomplishments to better understand SDRs and their applications. The SDR platforms and software waveforms on each SDR have over 1500 hours of operation and are performing as designed. The Ka-band SDR on the SCaN Testbed is NASAs first space Ka-band transceiver and is NASA's first Ka-band mission using the Space Network. This has provided exciting opportunities to operate at Ka-band and assist with on-orbit tests of NASA newest Tracking and Data Relay Satellites (TDRS). During its first year, SCaN Testbed completed its first on-orbit SDR reconfigurations. SDR reconfigurations occur when implementing new waveforms on an SDR. SDR reconfigurations allow a radio to change minor parameters, such as data rate, or complete functionality. New waveforms which provide new capability and are reusable across different missions provide long term value for reconfigurable platforms such as SDRs. The STRS Standard provides guidelines for new waveform development by third parties. Waveform development by organizations other than the platform provider offers NASA the ability to develop waveforms itself and reduce its dependence and costs on the platform developer. Each of these

Applying Model Based Systems Engineering to NASA's Space Communications Networks

Science.gov (United States)

Bhasin, Kul; Barnes, Patrick; Reinert, Jessica; Golden, Bert

2013-01-01

System engineering practices for complex systems and networks now require that requirement, architecture, and concept of operations product development teams, simultaneously harmonize their activities to provide timely, useful and cost-effective products. When dealing with complex systems of systems, traditional systems engineering methodology quickly falls short of achieving project objectives. This approach is encumbered by the use of a number of disparate hardware and software tools, spreadsheets and documents to grasp the concept of the network design and operation. In case of NASA's space communication networks, since the networks are geographically distributed, and so are its subject matter experts, the team is challenged to create a common language and tools to produce its products. Using Model Based Systems Engineering methods and tools allows for a unified representation of the system in a model that enables a highly related level of detail. To date, Program System Engineering (PSE) team has been able to model each network from their top-level operational activities and system functions down to the atomic level through relational modeling decomposition. These models allow for a better understanding of the relationships between NASA's stakeholders, internal organizations, and impacts to all related entities due to integration and sustainment of existing systems. Understanding the existing systems is essential to accurate and detailed study of integration options being considered. In this paper, we identify the challenges the PSE team faced in its quest to unify complex legacy space communications networks and their operational processes. We describe the initial approaches undertaken and the evolution toward model based system engineering applied to produce Space Communication and Navigation (SCaN) PSE products. We will demonstrate the practice of Model Based System Engineering applied to integrating space communication networks and the summary of its

Baicalein Rescues Delayed Cooling via Preservation of Akt Activation and Akt-Mediated Phospholamban Phosphorylation

Directory of Open Access Journals (Sweden)

Zuohui Shao

2018-03-01

Full Text Available Cooling reduces the ischemia/reperfusion (I/R injury seen in sudden cardiac arrest (SCA by decreasing the burst of reactive oxygen species (ROS. Its cardioprotection is diminished when delay in reaching the target temperature occurs. Baicalein, a flavonoid derived from the root of Scutellaria baicalensis Georgi, possesses antioxidant properties. Therefore, we hypothesized that baicalein can rescue cooling cardioprotection when cooling is delayed. Two murine cardiomyocyte models, an I/R model (90 min ischemia/3 h reperfusion and stunning model (30 min ischemia/90 min reperfusion, were used to assess cell survival and contractility, respectively. Cooling (32 °C was initiated either during ischemia or during reperfusion. Cell viability and ROS generation were measured. Cell contractility was evaluated by real-time phase-contrast imaging. Our results showed that cooling reduced cell death and ROS generation, and this effect was diminished when cooling was delayed. Baicalein (25 µM, given either at the start of reperfusion or start of cooling, resulted in a comparable reduction of cell death and ROS production. Baicalein improved phospholamban phosphorylation, contractility recovery, and cell survival. These effects were Akt-dependent. In addition, no synergistic effect was observed with the combined treatments of cooling and baicalein. Our data suggest that baicalein may serve as a novel adjunct therapeutic strategy for SCA resuscitation.

A combined static-dynamic single-dose imaging protocol to compare quantitative dynamic SPECT with static conventional SPECT.

Science.gov (United States)

Sciammarella, Maria; Shrestha, Uttam M; Seo, Youngho; Gullberg, Grant T; Botvinick, Elias H

2017-08-03

SPECT myocardial perfusion imaging (MPI) is a clinical mainstay that is typically performed with static imaging protocols and visually or semi-quantitatively assessed for perfusion defects based upon the relative intensity of myocardial regions. Dynamic cardiac SPECT presents a new imaging technique based on time-varying information of radiotracer distribution, which permits the evaluation of regional myocardial blood flow (MBF) and coronary flow reserve (CFR). In this work, a preliminary feasibility study was conducted in a small patient sample designed to implement a unique combined static-dynamic single-dose one-day visit imaging protocol to compare quantitative dynamic SPECT with static conventional SPECT for improving the diagnosis of coronary artery disease (CAD). Fifteen patients (11 males, four females, mean age 71 ± 9 years) were enrolled for a combined dynamic and static SPECT (Infinia Hawkeye 4, GE Healthcare) imaging protocol with a single dose of 99m Tc-tetrofosmin administered at rest and a single dose administered at stress in a one-day visit. Out of 15 patients, eleven had selective coronary angiography (SCA), 8 within 6 months and the rest within 24 months of SPECT imaging, without intervening symptoms or interventions. The extent and severity of perfusion defects in each myocardial region was graded visually. Dynamically acquired data were also used to estimate the MBF and CFR. Both visually graded images and estimated CFR were tested against SCA as a reference to evaluate the validity of the methods. Overall, conventional static SPECT was normal in ten patients and abnormal in five patients, dynamic SPECT was normal in 12 patients and abnormal in three patients, and CFR from dynamic SPECT was normal in nine patients and abnormal in six patients. Among those 11 patients with SCA, conventional SPECT was normal in 5, 3 with documented CAD on SCA with an overall accuracy of 64%, sensitivity of 40% and specificity of 83%. Dynamic SPECT image

Pathogenesis of chronic rhinosinusitis in patients affected by β-thalassemia major and sickle cell anaemia post allogenic bone marrow transplant.

Science.gov (United States)

Martino, F; Di Mauro, R; Paciaroni, K; Gaziev, J; Alfieri, C; Greco, L; Floris, R; Di Girolamo, S; Di Girolamo, M

2018-03-01

Sickle cell anemia (SCA) and β -thalassemia major are well-recognized beta-globin gene disorders of red blood cells associated to mortality and morbidity included bone morbidities due to ineffective erythropoiesis and bone marrow expansion, which affect every part of the skeleton. While there are an abundance of described disease manifestations of the head and neck, the manner of paranasal sinuses involvement and its relations to β-thalassemia and SCA process was not studied yet. Therefore, the aim of this study was to investigate a possible increased risk of rhinosinusitis and the real pathogenetic mechanism of it, comparing these two hematological diseases using msCT, gold standard for paranasal sinuses evaluation. A retrospective analysis of 90 patients affected by β-thalassemia major or SCA (respectively 59 and 31) underwent allogeneic bone marrow transplantation (BMT), and 44 control subjects was performed. Both patient categories and control group have been subjected to hematological and radiological evaluation using 64-multidetector-row CT scanner without contrast injection. Statistical analysis reveals that patients of the two study groups exhibit a significantly increased risk of sinusitis in comparison with the normal controls (RR: 3.55 for β-thalassemic pediatric subjects; RR: 3.35 for SCA pediatric subjects). A significant difference (p < 0,5) was found between the β -thalassemic patients on the one side, and SCA and control group on the other side, with regard to the evaluation of the typical anatomic alteration of maxillary sinus: β-thalassemic children had significant increase in the bone thickness of anterior and lateral sinus walls and significant reduction in volume and density compared to SCA patients and control group, with normal conditions of these parameters. In these hematological patients, there is an increased incidence of sinonasal infections due their therapy-induced immunosuppression post transplantation. In �

Análise em longo prazo na síndrome coronariana aguda: existem diferenças na morbimortalidade?

Directory of Open Access Journals (Sweden)

Adolfo Alexandre Farah de Aguiar

Full Text Available FUNDAMENTO: A insuficiência cardíaca (IC tem grande importância como preditor de morbimortalidade em pacientes com síndrome coronariana aguda (SCA. OBJETIVO: Avaliar os preditores de morbimortalidade na SCA em longo prazo. MÉTODOS: Foi um estudo de coorte de 403 pacientes consecutivos com queixas de dor torácica. Descreveram-se dados demográficos, clínicos, laboratoriais e terapêuticos, sendo avaliados durante a internação e até oito anos após alta, em relação à presença ou ausência de eventos cardiovasculares e óbitos. RESULTADOS: Foram 403 pacientes com queixas de dor torácica, em que 65,8% apresentavam diagnóstico de SCA sem supra de ST, 27,8% SCA com supra de ST e 6,5% sem SCA. Destes, foram avaliados os 377 pacientes com SCA, em que 37,9% eram do sexo feminino, e a média de idade foi de 62,2 ± 11,6 anos. A presença de IC antes ou durante a hospitalização influenciou a mortalidade. Dos fatores prognósticos, a creatinina inicial merece destaque, sendo o ponto de corte de 1,4 mg/dl (acurácia = 62,1%; HR = 3,27; p < 0,001. Notamos pior prognóstico para cada acréscimo de dez anos de idade (HR = 1,37; p < 0,001 e para cada incremento de 10 bpm na frequência cardíaca (HR = 1,22 p < 0,001. Quanto às terapias utilizadas antes e após 2002, houve aumento de uso de betabloqueadores, inibidores da enzima conversora da angiotensina (IECAs, estatinas e antiplaquetários, tendo impacto na mortalidade. CONCLUSÃO: Presença de IC admissional, creatinina, idade e FC foram preditores independentes de mortalidade. Observou-se que pacientes com IC atendidos antes de 2002 apresentaram pior sobrevida em relação aos atendidos após 2002 e que a mudança na terapia foi a responsável por isso.

O escore de risco de sangramento como preditor de mortalidade em pacientes com síndromes coronarianas agudas

Directory of Open Access Journals (Sweden)

José Carlos Nicolau

2013-12-01

Full Text Available FUNDAMENTO: A ocorrência de sangramento aumenta a mortalidade intra-hospitalar em pacientes com síndromes coronarianas agudas (SCAs, e há uma boa correlação entre os escores de risco de sangramento e a incidência de eventos hemorrágicos. No entanto, o papel dos escores de risco de sangramento como fatores preditivos de mortalidade é pouco estudado. OBJETIVO: Analisar o papel do escore de risco de sangramento como fator preditivo de mortalidade intra-hospitalar numa coorte de pacientes com SCA tratados num centro terciário de cardiologia. MÉTODOS: Dos 1.655 pacientes com SCA (547 com SCA com supra de ST e 1.118 com SCA sem supra de ST, calculou-se o escore de risco de sangramento ACUITY/HORIZONS prospectivamente em 249 pacientes e retrospectivamente nos demais 1.416. Informações sobre mortalidade e complicações hemorrágicas também foram obtidas. RESULTADOS: A idade média da população estudada foi 64,3 ± 12,6 anos e o escore de risco de sangramento médio foi 18 ± 7,7. A correlação entre sangramento e mortalidade foi altamente significativa (p < 0,001; OR = 5,29, assim como a correlação entre escore de sangramento e hemorragia intra-hospitalar (p < 0,001; OR = 1,058, e entre escore de sangramento e mortalidade intra-hospitalar (OR ajustado = 1,121, p < 0,001, área sob a curva ROC 0,753; p < 0,001. O OR ajustado e a área sob a curva ROC para a população com SCA com supra de ST foram 1,046 (p = 0,046 e 0,686 ± 0,040 (p < 0,001, respectivamente, e para SCA sem supra de ST foram 1,150 (p < 0,001 e 0,769 ± 0,036 (p < 0,001, respectivamente. CONCLUSÃO: O escore de risco de sangramento é um fator preditivo muito útil e altamente confiável para mortalidade intra-hospitalar em uma grande variedade de pacientes com SCAs, especialmente aqueles com angina instável ou infarto agudo do miocárdio sem supra de ST.

Augmenting SCA project management and automation framework

Science.gov (United States)

Iyapparaja, M.; Sharma, Bhanupriya

2017-11-01

In our daily life we need to keep the records of the things in order to manage it in more efficient and proper way. Our Company manufactures semiconductor chips and sale it to the buyer. Sometimes it manufactures the entire product and sometimes partially and sometimes it sales the intermediary product obtained during manufacturing, so for the better management of the entire process there is a need to keep the track record of all the entity involved in it. Materials and Methods: Therefore to overcome with the problem the need raised to develop the framework for the maintenance of the project and for the automation testing. Project management framework provides an architecture which supports in managing the project by marinating the records of entire requirements, the test cases that were created for testing each unit of the software, defect raised from the past years. So through this the quality of the project can be maintained. Results: Automation framework provides the architecture which supports the development and implementation of the automation test script for the software testing process. Conclusion: For implementing project management framework the product of HP that is Application Lifecycle management is used which provides central repository to maintain the project.

Review of the Probable Maximum Flood (PMF) Snowmelt Analysis for Success Dam

Science.gov (United States)

2015-11-01

watershed; and the USACE reports (1998, 2013) further describe the watershed characteris- tics, vegetation , climate, precipitation, and flooding. The...developed SCA product, MOD10A1, the MODIS/ Terra Snow Cover Daily L3 Global 500 m Grid (Hall et al. 2006), which provides a daily estimate of the fSCA of...find mean cloud persistence, or the average time between cloud-free scenes, to be less than a day at Terra MODIS’s temporal scale (approxi- mately 1

Studies on Molecular Mechanisms Underlying Spinocerebellar Ataxia Type 3

DEFF Research Database (Denmark)

Kristensen, Line Vildbrad

. Even though a range of mechanisms contributing to polyQ diseases have been uncovered, there is still no treatment available. One of the more common polyQ diseases is SCA3, which is caused by a polyQ expansion in the ataxin-3 protein that normally functions as a deubiquitinating enzyme involved...... in protein quality control. In SCA3 patients polyQ expanded ataxin-3 forms intranuclear inclusions in various brain areas, but why the polyQ expansion of ataxin-3 leads to neuronal dysfunction is still not well understood. This thesis describes molecular biological investigations of ataxin-3 biology, aimed...... at furthering our understanding of SCA3 disease mechanisms. In manuscript I, we investigated if post-translational modifications of ataxin-3 were changed by the polyQ expansion. The ubiquitin chain topology and ubiquitination pattern of ataxin-3 were unaltered by the polyQ expansion. In contrast...

Hydroxyurea therapy contributes to infertility in adult men with sickle cell disease: a review.

Science.gov (United States)

DeBaun, Michael R

2014-12-01

Hydroxyurea therapy, a chemotherapeutic agent, is the only US FDA approved therapy for the prevention of vaso-occlusive pain in sickle cell disease (SCD). The National Institutes of Health has sponsored two Phase III randomized, placebo-controlled trials, initially in adults, and subsequently in children with sickle cell anemia (SCA). Despite the overwhelming evidence that hydroxyurea therapy is beneficial to children and adults with SCA, individuals with SCA and their families express reservations about its use, in part because of the concerns about fertility, particularly in men. As adolescent boys with SCD are now expected to reach their reproductive years, a new concern is emerging about the role of hydroxyurea therapy as a barrier to their progeny. This review will systemically evaluate compromised fertility in men with SCD, and the evidence that hydroxyurea therapy is associated with further decreasing fertility in men with SCD.

Hematopoietic Stem Cell Transplantation in Thalassemia and Sickle Cell Anemia

Science.gov (United States)

Lucarelli, Guido; Isgrò, Antonella; Sodani, Pietro; Gaziev, Javid

2012-01-01

The globally widespread single-gene disorders β-thalassemia and sickle cell anemia (SCA) can only be cured by allogeneic hematopoietic stem cell transplantation (HSCT). HSCT treatment of thalassemia has substantially improved over the last two decades, with advancements in preventive strategies, control of transplant-related complications, and preparative regimens. A risk class–based transplantation approach results in disease-free survival probabilities of 90%, 84%, and 78% for class 1, 2, and 3 thalassemia patients, respectively. Because of disease advancement, adult thalassemia patients have a higher risk for transplant-related toxicity and a 65% cure rate. Patients without matched donors could benefit from haploidentical mother-to-child transplantation. There is a high cure rate for children with SCA who receive HSCT following myeloablative conditioning protocols. Novel non-myeloablative transplantation protocols could make HSCT available to adult SCA patients who were previously excluded from allogeneic stem cell transplantation. PMID:22553502

Evaluating the influence of motor control on selective attention through a stochastic model: the paradigm of motor control dysfunction in cerebellar patient.

Science.gov (United States)

Veneri, Giacomo; Federico, Antonio; Rufa, Alessandra

2014-01-01

Attention allows us to selectively process the vast amount of information with which we are confronted, prioritizing some aspects of information and ignoring others by focusing on a certain location or aspect of the visual scene. Selective attention is guided by two cognitive mechanisms: saliency of the image (bottom up) and endogenous mechanisms (top down). These two mechanisms interact to direct attention and plan eye movements; then, the movement profile is sent to the motor system, which must constantly update the command needed to produce the desired eye movement. A new approach is described here to study how the eye motor control could influence this selection mechanism in clinical behavior: two groups of patients (SCA2 and late onset cerebellar ataxia LOCA) with well-known problems of motor control were studied; patients performed a cognitively demanding task; the results were compared to a stochastic model based on Monte Carlo simulations and a group of healthy subjects. The analytical procedure evaluated some energy functions for understanding the process. The implemented model suggested that patients performed an optimal visual search, reducing intrinsic noise sources. Our findings theorize a strict correlation between the "optimal motor system" and the "optimal stimulus encoders."

Hydroxyurea lowers transcranial Doppler flow velocities in children with sickle cell anaemia in a Nigerian cohort.

Science.gov (United States)

Lagunju, IkeOluwa; Brown, Biobele J; Sodeinde, Olugbemiro

2015-09-01

Sickle cell anaemia (SCA) is the leading genetic disorder in Nigeria. Elevated velocities ≥170 cm/sec occur in about a third of Nigerian children with SCA. Chronic blood transfusion for stroke prevention is faced with a myriad of challenges in our practice. To evaluate the effectiveness of hydroxyurea (HU) in reducing flow velocities in a cohort of Nigerian children with SCA and elevated velocities treated with HU. An observational study was carried out on a cohort of Nigerian children with SCA and elevated velocities identified on routine transcranial Doppler (TCD) screening. HU was recommended in those with TCD velocities ≥ 170cm/sec as stipulated in our hospital protocol. Outcomes were compared after ≥12 months of observation. Fifty children with elevated TCD velocities were studied; 31 consented to HU therapy and 19 declined. Children on HU showed a statistically significant decline in mean velocities from 199.7 [17.1] cm/sec to 165.8 [20.7] cm/sec (P < 0.001) with a significant increase in mean packed cell volume from 21.1 [3.4] to 25.0 [2.8]%. Children without treatment had a significant rise in mean velocities from 190.2 [10.8] cm/sec to 199.7 [14.9] cm/sec (P = 0.003). Children with conditional risk velocities on HU were less likely to convert to abnormal risk (P < 0.001). Two stroke events occurred, one in each group. No adverse effects of HU were recorded in the cohort. HU appears to significantly reduce TCD velocities in Nigerian children with SCA and elevated velocities ≥170 cm/sec with beneficial effect on the haematological profile. HU may provide an effective approach to primary stroke prevention, particularly in Africa. © 2015 Wiley Periodicals, Inc.

Food web functioning of the benthopelagic community in a deep-sea seamount based on diet and stable isotope analyses

Science.gov (United States)

Preciado, Izaskun; Cartes, Joan E.; Punzón, Antonio; Frutos, Inmaculada; López-López, Lucía; Serrano, Alberto

2017-03-01

Trophic interactions in the deep-sea fish community of the Galicia Bank seamount (NE Atlantic) were inferred by using stomach contents analyses (SCA) and stable isotope analyses (SIA) of 27 fish species and their main prey items. Samples were collected during three surveys performed in 2009, 2010 and 2011 between 625 and 1800 m depth. Three main trophic guilds were determined using SCA data: pelagic, benthopelagic and benthic feeders, respectively. Vertically migrating macrozooplankton and meso-bathypelagic shrimps were identified to play a key role as pelagic prey for the deep sea fish community of the Galicia Bank. Habitat overlap was hardly detected; as a matter of fact, when species coexisted most of them evidenced a low dietary overlap, indicating a high degree of resource partitioning. A high potential competition, however, was observed among benthopelagic feeders, i.e.: Etmopterus spinax, Hoplostethus mediterraneus and Epigonus telescopus. A significant correlation was found between δ15N and δ13C for all the analysed species. When calculating Trophic Levels (TLs) for the main fish species, using both the SCA and SIA approaches, some discrepancies arose: TLs calculated from SIA were significantly higher than those obtained from SCA, probably indicating a higher consumption of benthic-suprabenthic prey in the previous months. During the summer, food web functioning in the Galicia Bank was more influenced by the assemblages dwelling in the water column than by deep-sea benthos, which was rather scarce in the summer samples. These discrepancies demonstrate the importance of using both approaches, SCA (snapshot of diet) and SIA (assimilated food in previous months), when attempting trophic studies, if an overview of food web dynamics in different compartments of the ecosystem is to be obtained.

Doppler echocardiographic study in adolescents and young adults with sickle cell anemia

Directory of Open Access Journals (Sweden)

Wolney de Andrade Martins

1999-12-01

Full Text Available OBJECTIVE: Anatomical and functional assessment of the heart through Doppler and echocardiography in patients with cell anemia (SCA. METHODS: Twenty-five patients with SCA and ages ranging from 14 to 45 years were prospectively studied in a comparison with 25 healthy volunteers. All of them underwent clinical and laboratory evaluation and Doppler echocardiography as well.The measurements were converted into body surface indices. RESULTS: There were increases in all chamber diameters and left ventricle (LV mass of the SCA patients. It was characterised an eccentric hypertrophy of the left ventricle. The preload was increased (left ventricle end-diastolic volume and the afterload was decreased (diastolic blood pressure, peripheral vascular resistance and end-systolic parietal stress ESPS. The cardiac index was increased due to the stroke volume. The ejection fraction and the percentage of the systolic shortening , as well as the systolic time intervals of the LV were equivalent. The isovolumetric contraction period of the LV was increased. The mitral E-septum distance and the end-systolic volume index (ESVi were increased. The ESPS/ESVi ratio,a loading independent parameter, was decreased in SCA, suggesting systolic dysfunction. No significant differences in the diastolic function or in the pulmonary pressure occurred. CONCLUSION: Chamber dilations, eccentric hypertrophy and systolic dysfunction confirm the evidence of the literature in characterizing a sickle cell anemia cardiomyopathy.

Traditional Herbal Management of Sickle Cell Anemia: Lessons from Nigeria

Directory of Open Access Journals (Sweden)

Sunday J. Ameh

2012-01-01

Full Text Available Background. Patients in West Africa where sickle cell anemia (SCA is endemic have for ages been treated with natural products, especially herbs, as, is still the case in rural communities. Objective. In this paper we look closely at some of these herbs to see if there are any lessons to be learnt or clues to be found for optimizing the treatments based on them, as had been done in the case of NIPRISAN, which was developed from herbs in Nigeria based on Yoruba Medicine. Methods. Select publications on SCA, its molecular biology and pathology, and actual and experimental cases of herbal treatment were perused in search of molecular clues that can be linked to chemical constituents of the herbs involved. Results. The study revealed that during the last 2-3 decades, much progress was made in several aspects of SCA pharmacology, especially the approval of hydroxyurea. As for SCA herbalism, this paper revealed that antisickling herbs abound in West Africa and that the most promising may yet be found. Three new antisickling herbs (Entandrophragma utile, Chenopodium ambrosioides, and Petiveria alliacea were reported in May 2011. At NIPRD, where NIPRISAN was developed, three other recipes are currently awaiting development. Conclusion. The study raised the hope that the search in the Tropics for more effective herbal recipes for managing sickle cell anaemia will be more fruitful with time and effort.

Improvements to Integrated Tradespace Analysis of Communications Architectures (ITACA) Network Loading Analysis Tool

Science.gov (United States)

Lee, Nathaniel; Welch, Bryan W.

2018-01-01

NASA's SCENIC project aims to simplify and reduce the cost of space mission planning by replicating the analysis capabilities of commercially licensed software which are integrated with relevant analysis parameters specific to SCaN assets and SCaN supported user missions. SCENIC differs from current tools that perform similar analyses in that it 1) does not require any licensing fees, 2) will provide an all-in-one package for various analysis capabilities that normally requires add-ons or multiple tools to complete. As part of SCENIC's capabilities, the ITACA network loading analysis tool will be responsible for assessing the loading on a given network architecture and generating a network service schedule. ITACA will allow users to evaluate the quality of service of a given network architecture and determine whether or not the architecture will satisfy the mission's requirements. ITACA is currently under development, and the following improvements were made during the fall of 2017: optimization of runtime, augmentation of network asset pre-service configuration time, augmentation of Brent's method of root finding, augmentation of network asset FOV restrictions, augmentation of mission lifetimes, and the integration of a SCaN link budget calculation tool. The improvements resulted in (a) 25% reduction in runtime, (b) more accurate contact window predictions when compared to STK(Registered Trademark) contact window predictions, and (c) increased fidelity through the use of specific SCaN asset parameters.

Characteristic signal changes in the pontine base on T2- and multishot diffusion-weighted images in spinocerebellar ataxia type 1

International Nuclear Information System (INIS)

Adachi, M.; Kawanami, T.; Ohshima, H.; Hosoya, T.

2006-01-01

The aim of the present study was to demonstrate characteristic signal changes of the pontine base on T2-weighted images of patients with SCA 1, and to elucidate the relationship between abnormal high-intensities of the pontine base on T2-weighted images and the findings on multishot diffusion-weighted images. We assessed abnormal signals of the pontine base on T2-weighted images from 50 controls and six patients with SCA 1 diagnosed by genetic analysis. At the same time, we evaluated the degeneration of the transverse pontine fibers in the pontine base by multishot diffusion-weighted imaging. A midline high-intensity was seen in the pontine base on T2-weighted images in two of the 50 controls and five of the six patients with SCA 1. The midline high-intensity had a sensitivity of 83.3% for patients and a specificity of 96.0% for controls. Multishot diffusion-weighted imaging demonstrated the degeneration - the amorphous-pattern signal - of the transverse pontine fibers in four (66.7%) of the six patients. In the other two patients, the zebra-pattern signal was seen in the pontine base. The midline high-intensity on T2-weighted images appears to be one of characteristic MRI findings of SCA 1. Multishot diffusion-weighted imaging suggested that the midline high-intensity should reflect the degeneration of the transverse pontine fibers. (orig.)

Precisión diagnóstica de un modelo de redes bayesianas en los síndromes coronarios agudos

Directory of Open Access Journals (Sweden)

John Sprockel

2015-08-01

Full Text Available Introducción: La caracterización diagnóstica del dolor torácico, con énfasis en los síndromes coronarios agudos (SCA es un requerimiento primordial para los médicos del área de urgencias. Objetivos: En el presente estudio se busca diseñar y evaluar el desempeño de las redes bayesianas en el apoyo al diagnóstico de los SCA. Metodología: Se trata de un estudio de pruebas diagnósticas en el cual se diseñaron dos modelos de redes bayesianas entrenadas en el framework OpenMarkov, a partir de las variables de la escala de probabilidad de Braunwald de angina en un grupo de 159 pacientes que luego se validó en una cohorte de 108 pacientes adultos hospitalizados con sospecha de un SCA en un hospital de tercer nivel de atención. Resultados: Se obtuvo una sensibilidad baja aunque con especificidad y valor predictivo positivo adecuados (62, 86 y 87% respectivamente. El rendimiento fue mejor en los casos que tuvieron electrocardiograma y biomarcadores negativos. Conclusiones: Un modelo de redes Bayesianas entrenado a partir de las variables de la escala de probabilidad de angina inestable de Braunwald, presenta un rendimiento aceptable para el diagnóstico de los SCA.

Evaluation of maize S2 lines in test cross combinations I : flowering and morphological traits

International Nuclear Information System (INIS)

Rahman, H.; Shah, A.Z.; Shah, S.M.A.; Khalil, I.H.

2010-01-01

This research work was conducted at N.W.F.P. Agricultural University, Peshawar to test 24 maize S2 lines using line x tester analysis. Data were recorded on days to 50% pollen shedding and silking, anthesis silking interval (ASI), ear height and plant height. Results revealed highly significant differences among the testcrosses for all these parameters. Maximum days to silking (62) were observed for TC-13, using WD 2x8 as a tester, while SCA for day to silking was recorded 3.15. Maximum days to anthesis were observed (60) for TC-36, using WD 3x6 as a tester. Maximum value (3 days) for anthesis silking interval was observed for TC-13, using WD 2x8 as a tester, while SCA for anthesis silking interval was recorded to be 1.60 days. Maximum ear height (82cm) was observed for the TC-21, using WD 3x6 as a tester, while SCA for ear height was 1.5 cm. Maximum plant eight (161 cm) was observed for TC-21, using WD 3x6 as a tester, while SCA for plant height was found to be -17.8 cm. These testcrosses are recommended to be included in further breeding programs for developing maize germplasm with earlier flowering and desirable plant and ear height attributes. (author)

Traditional herbal management of sickle cell anemia: lessons from Nigeria.

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Ameh, Sunday J; Tarfa, Florence D; Ebeshi, Benjamin U

2012-01-01

Background. Patients in West Africa where sickle cell anemia (SCA) is endemic have for ages been treated with natural products, especially herbs, as, is still the case in rural communities. Objective. In this paper we look closely at some of these herbs to see if there are any lessons to be learnt or clues to be found for optimizing the treatments based on them, as had been done in the case of NIPRISAN, which was developed from herbs in Nigeria based on Yoruba Medicine. Methods. Select publications on SCA, its molecular biology and pathology, and actual and experimental cases of herbal treatment were perused in search of molecular clues that can be linked to chemical constituents of the herbs involved. Results. The study revealed that during the last 2-3 decades, much progress was made in several aspects of SCA pharmacology, especially the approval of hydroxyurea. As for SCA herbalism, this paper revealed that antisickling herbs abound in West Africa and that the most promising may yet be found. Three new antisickling herbs (Entandrophragma utile, Chenopodium ambrosioides, and Petiveria alliacea) were reported in May 2011. At NIPRD, where NIPRISAN was developed, three other recipes are currently awaiting development. Conclusion. The study raised the hope that the search in the Tropics for more effective herbal recipes for managing sickle cell anaemia will be more fruitful with time and effort.

Serum Copper Level Significantly Influences Platelet Count, Lymphocyte Count and Mean Cell Hemoglobin in Sickle Cell Anemia

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Okocha Chide

2015-12-01

Full Text Available Background Changes in serum micro nutrients levels affect a number of critically important metabolic processes; these could potentially influence blood counts and ultimately disease presentation in patients with sickle cell anemia (SCA. Objectives To evaluate the influence of serum micro-nutrients levels; zinc, copper, selenium and magnesium on blood counts in steady state SCA patients. Methods A cross sectional study that involved 28 steady state adult SCA subjects. Seven milliliters (mls of blood was collected; 3 mls was for hemoglobin electrophoresis and full blood count determination while 4 mls was for measurement of serum micro nutrients levels, by the atomic absorption spectrophotometry. Correlation between serum micro-nutrient levels and blood counts was done by the Pearson’s linear regression. Ethical approval was obtained from the institutional review board and each participant gave informed consent. All data was analyzed by SPSS software version 20. Results There was a significant correlation between serum copper levels and mean cell hemoglobin (MCH, platelet and lymphocyte counts (r = 0.418; P = 0.02, r = -0.376; P = 0.04 and r = -0.383; P = 0.04, respectively. There were no significant correlations between serum levels of other micro nutrients (selenium, zinc and magnesium and blood counts. Conclusions Copper influences blood count in SCA patients probably by inducing red cell haemolysis, oxidant tissue damage and stimulating the immune system.

Origin of Matrix-Producing Cells That Contribute to Aortic Fibrosis in Hypertension.

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Wu, Jing; Montaniel, Kim Ramil C; Saleh, Mohamed A; Xiao, Liang; Chen, Wei; Owens, Gary K; Humphrey, Jay D; Majesky, Mark W; Paik, David T; Hatzopoulos, Antonis K; Madhur, Meena S; Harrison, David G

2016-02-01

Various hypertensive stimuli lead to exuberant adventitial collagen deposition in large arteries, exacerbating blood pressure elevation and end-organ damage. Collagen production is generally attributed to resident fibroblasts; however, other cells, including resident and bone marrow-derived stem cell antigen positive (Sca-1(+)) cells and endothelial and vascular smooth muscle cells, can produce collagen and contribute to vascular stiffening. Using flow cytometry and immunofluorescence, we found that adventitial Sca-1(+) progenitor cells begin to produce collagen and acquire a fibroblast-like phenotype in hypertension. We also found that bone marrow-derived cells represent more than half of the matrix-producing cells in hypertension, and that one-third of these are Sca-1(+). Cell sorting and lineage-tracing studies showed that cells of endothelial origin contribute to no more than one fourth of adventitial collagen I(+) cells, whereas those of vascular smooth muscle lineage do not contribute. Our findings indicate that Sca-1(+) progenitor cells and bone marrow-derived infiltrating fibrocytes are major sources of arterial fibrosis in hypertension. Endothelial to mesenchymal transition likely also contributes, albeit to a lesser extent and pre-existing resident fibroblasts represent a minority of aortic collagen-producing cells in hypertension. This study shows that vascular stiffening represents a complex process involving recruitment and transformation of multiple cells types that ultimately elaborate adventitial extracellular matrix. © 2015 American Heart Association, Inc.

Measurements of red cell deformability and hydration reflect HbF and HbA2 in blood from patients with sickle cell anemia.

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Parrow, Nermi L; Tu, Hongbin; Nichols, James; Violet, Pierre-Christian; Pittman, Corinne A; Fitzhugh, Courtney; Fleming, Robert E; Mohandas, Narla; Tisdale, John F; Levine, Mark

2017-06-01

Decreased erythrocyte deformability, as measured by ektacytometry, may be associated with disease severity in sickle cell anemia (SCA). Heterogeneous populations of rigid and deformable cells in SCA blood result in distortions of diffraction pattern measurements that correlate with the concentration of hemoglobin S (HbS) and the percentage of irreversibly sickled cells. We hypothesize that red cell heterogeneity, as well as deformability, will also be influenced by the concentration of alternative hemoglobins such as fetal hemoglobin (HbF) and the adult variant, HbA 2 . To test this hypothesis, we investigate the relationship between diffraction pattern distortion, osmotic gradient ektacytometry parameters, and the hemoglobin composition of SCA blood. We observe a correlation between the extent of diffraction pattern distortions and percentage of HbF and HbA 2 . Osmotic gradient ektacytometry data indicate that minimum elongation in the hypotonic region is positively correlated with HbF, as is the osmolality at which it occurs. The osmolality at both minimum and maximum elongation is inversely correlated with HbS and HbA 2 . These data suggest that HbF may effectively improve surface-to-volume ratio and osmotic fragility in SCA erythrocytes. HbA 2 may be relatively ineffective in improving these characteristics or cellular hydration at the levels found in this patient cohort. Copyright © 2017. Published by Elsevier Inc.

Factors Associated with Growth Retardation in Children Suffering from Sickle Cell Anemia: First Report from Central Africa

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Aimé Lukusa Kazadi

2017-01-01

Full Text Available Background. The aim of this study was to investigate and determine the risk factors associated with poor growth among SCA children. Methods. A cross-sectional study was conducted in Kinshasa, the capital’s country. The nutritional status was assessed using the Z scores of the anthropometric indices. Results. We gathered data on the 256 patients, 138 females (53.9%, who entered the study. The mean age at presentation was 8.4 ± 4.9 years of age. Underweight, stunting, and wasting were found, respectively, in 47.7%, 10.5%, and 50.3% of SCA children. A history of hand-foot syndrome, more than 3 blood transfusions, being less than 12 months of age when receiving the first transfusion, more than two severe sickle crises per year, a medical history of severe infections, and the presence of hepatomegaly were associated with poor growth. When comparing sickle cell patients under 12 years of age (n=159 to a group of 296 age-matched children with normal Hb-AA, a significantly higher proportion of subjects with stunting and underweight were found among SCA. Conclusion. Nutritional status encountered in Congolese sickle cell children has been described for the first time in this study. A high prevalence of poor growth in SCA children was found in our study.

Traditional Herbal Management of Sickle Cell Anemia: Lessons from Nigeria

Science.gov (United States)

Ameh, Sunday J.; Tarfa, Florence D.; Ebeshi, Benjamin U.

2012-01-01

Background. Patients in West Africa where sickle cell anemia (SCA) is endemic have for ages been treated with natural products, especially herbs, as, is still the case in rural communities. Objective. In this paper we look closely at some of these herbs to see if there are any lessons to be learnt or clues to be found for optimizing the treatments based on them, as had been done in the case of NIPRISAN, which was developed from herbs in Nigeria based on Yoruba Medicine. Methods. Select publications on SCA, its molecular biology and pathology, and actual and experimental cases of herbal treatment were perused in search of molecular clues that can be linked to chemical constituents of the herbs involved. Results. The study revealed that during the last 2-3 decades, much progress was made in several aspects of SCA pharmacology, especially the approval of hydroxyurea. As for SCA herbalism, this paper revealed that antisickling herbs abound in West Africa and that the most promising may yet be found. Three new antisickling herbs (Entandrophragma utile, Chenopodium ambrosioides, and Petiveria alliacea) were reported in May 2011. At NIPRD, where NIPRISAN was developed, three other recipes are currently awaiting development. Conclusion. The study raised the hope that the search in the Tropics for more effective herbal recipes for managing sickle cell anaemia will be more fruitful with time and effort. PMID:23198140

Genetic determinants and stroke in children with sickle cell disease

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Daniela O.W. Rodrigues

2016-11-01

Conclusion: There is a high incidence of stroke in male children and in children with SCA. Coexistence with α‐thal and haplotypes of the beta globin chain cluster did not show any significant association with stroke. The heterogeneity between previously evaluated populations, the non‐reproducibility between studies, and the need to identify factors associated with stroke in patients with SCA indicate the necessity of conducting further research to demonstrate the relevance of genetic factors in stroke related to SCD.

The co-inheritance of alpha-thalassemia and sickle cell anemia is associated with better hematological indices and lower consultations rate in Cameroonian patients and could improve their survival.

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Maryam Bibi Rumaney

Full Text Available BACKGROUND: Co-inheritance of α-thalassemia was reported to be associated with a delayed age of disease onset among Cameroonian Sickle Cell Anemia (SCA patients. The present study aimed to explore the correlation between α-thalassemia, hematological indices, and clinical events in these patients. METHODS AND FINDINGS: We studied 161 Cameroonian SCA patients and 103 controls (59.1% HbAA with median ages of 17.5 and 23 years. RFLP-PCR was used to confirm SCA genotype and to describe haplotypes in the HBB-like genes cluster. Multiplex Gap-PCR was performed to investigate the 3.7 kb α-globin gene deletions. SNaPshot PCR, capillary electrophoresis and cycle sequencing were used for the genotyping of 10 SNPs in BCL11A, HMIP1/2, OR51B5/6 and HBG loci, known to influence HbF levels. Generalised linear regression models adjusted for age, sex and SNPs genotypes was used to investigate effects of α-thalassemia on clinical and hematological indices. The median rate of vaso-occlusive painful crisis and hospitalisations was two and one per year, respectively. Stroke was reported in eight cases (7.4%. Benin haplotype was the most prevalent (66.3%; n = 208 chromosomes. Among patients, 37.3% (n = 60 had at least one 3.7 kb deletion, compared to 10.9% (n = 6 among HbAA controls (p<0.001. Among patients, the median RBC count increased with the number of 3.7 kb deletions [2.6, 3.0 and 3.4 million/dl, with no, one and two deletions (p = 0.01]. The median MCV decreased with the number of 3.7 kb deletion [86, 80, and 68fl, with no, one and two deletions (p<0.0001], as well as median WBC counts [13.2, 10.5 and 9.8×109/L (p<0.0001. The co-inheritance of α-thalassemia was associated with lower consultations rate (p = 0.038. CONCLUSION: The co-inheritance of α-thalassemia and SCA is associated with improved hematological indices, and lower consultations rate in this group of patients. This could possibly improve their survival and explain the

The Co-Inheritance of Alpha-Thalassemia and Sickle Cell Anemia Is Associated with Better Hematological Indices and Lower Consultations Rate in Cameroonian Patients and Could Improve Their Survival

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Rumaney, Maryam Bibi; Ngo Bitoungui, Valentina Josiane; Vorster, Anna Alvera; Ramesar, Raj; Kengne, Andre Pascal; Ngogang, Jeanne; Wonkam, Ambroise

2014-01-01

Background Co-inheritance of α-thalassemia was reported to be associated with a delayed age of disease onset among Cameroonian Sickle Cell Anemia (SCA) patients. The present study aimed to explore the correlation between α-thalassemia, hematological indices, and clinical events in these patients. Methods and Findings We studied 161 Cameroonian SCA patients and 103 controls (59.1% HbAA) with median ages of 17.5 and 23 years. RFLP-PCR was used to confirm SCA genotype and to describe haplotypes in the HBB-like genes cluster. Multiplex Gap-PCR was performed to investigate the 3.7 kb α-globin gene deletions. SNaPshot PCR, capillary electrophoresis and cycle sequencing were used for the genotyping of 10 SNPs in BCL11A, HMIP1/2, OR51B5/6 and HBG loci, known to influence HbF levels. Generalised linear regression models adjusted for age, sex and SNPs genotypes was used to investigate effects of α-thalassemia on clinical and hematological indices. The median rate of vaso-occlusive painful crisis and hospitalisations was two and one per year, respectively. Stroke was reported in eight cases (7.4%). Benin haplotype was the most prevalent (66.3%; n = 208 chromosomes). Among patients, 37.3% (n = 60) had at least one 3.7 kb deletion, compared to 10.9% (n = 6) among HbAA controls (p<0.001). Among patients, the median RBC count increased with the number of 3.7 kb deletions [2.6, 3.0 and 3.4 million/dl, with no, one and two deletions (p = 0.01)]. The median MCV decreased with the number of 3.7 kb deletion [86, 80, and 68fl, with no, one and two deletions (p<0.0001)], as well as median WBC counts [13.2, 10.5 and 9.8×109/L (p<0.0001. The co-inheritance of α-thalassemia was associated with lower consultations rate (p = 0.038). Conclusion The co-inheritance of α-thalassemia and SCA is associated with improved hematological indices, and lower consultations rate in this group of patients. This could possibly improve their survival and explain the higher

Targeting the CACNA1A IRES as a Treatment for Spinocerebellar Ataxia Type 6.

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Pastor, Parviz Daniel Hejazi; Du, Xiaofei; Fazal, Sarah; Davies, Andre N; Gomez, Christopher M

2018-02-01

We have discovered that the P/Q-type voltage-gated Ca 2+ channel (VGCC) gene, CACNA1A, encodes both the α1A (Cav2.1) subunit and a newly recognized transcription factor, α1ACT, by means of a novel internal ribosomal entry site (IRES) within the α1A C-terminal coding region. α1ACT, when mutated with an expansion of the polyglutamine tract in the C-terminus, gives rise to spinocerebellar ataxia type 6 (SCA6). Because silencing of the entire CACNA1A gene would result in the loss of the essential Cav2.1 channel, the IRES controlling α1ACT expression is an excellent target for selective silencing of α1ACT as a therapeutic intervention for SCA6. We performed a high-throughput screen of FDA-approved small molecules using a dual luciferase reporter system and identified ten hits able to selectively inhibit the IRES. We identified four main candidates that showed selective suppression of α1ACT relative to α1A in HEK cells expressing a native CACNA1A vector. We previously pursued another avenue of molecular intervention through miRNA silencing. We studied three human miRNAs (miRNA-711, -3191-5p, -4786) that would potentially bind to sequences within the CACNA1A IRES region, based on an miRNA prediction program. Only miRNA-3191-5p was found to selectively inhibit the translation of α1ACT in cells. We developed a hyperacute model of SCA6 in mice by injecting a pathogenic form of the IRES-mediated α1ACT (AAV9-α1ACTQ33). Finally, we tested the effectiveness of the miRNA therapy by co-expressing either control miRNA or miRNA-3191-5p and found that miRNA-3191-5p decreased the levels of α1ACTQ33 and prevented the hyperacute disease in mice. These studies provide the proof of principle that a therapy directed at selectively preventing α1ACT expression could be used to treat SCA6.

A Study on the Rheological and Mechanical Properties of Photo-Curable Ceramic/Polymer Composites with Different Silane Coupling Agents for SLA 3D Printing Technology.

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Song, Se Yeon; Park, Min Soo; Lee, Jung Woo; Yun, Ji Sun

2018-02-07

Silane coupling agents (SCAs) with different organofunctional groups were coated on the surfaces of Al₂O₃ ceramic particles through hydrolysis and condensation reactions, and the SCA-coated Al₂O₃ ceramic particles were dispersed in a commercial photopolymer based on interpenetrating networks (IPNs). The organofunctional groups that have high radical reactivity and are more effective in UV curing systems are usually functional groups based on acryl, such as acryloxy groups, methacrloxy groups, and acrylamide groups, and these silane coupling agents seem to improve interfacial adhesion and dispersion stability. The coating morphology and the coating thickness distribution of SCA-coated Al₂O₃ ceramic particles according to the different organofunctional groups were observed by FE-TEM. The initial dispersibility and dispersion stability of the SCA-coated Al₂O₃/High-temp composite solutions were investigated by relaxation NMR and Turbiscan. The rheological properties of the composite solutions were investigated by viscoelastic analysis and the mechanical properties of 3D-printed objects were observed with a nanoindenter.

Tuberculous spondylodiscitis in a patient with a sickle-cell disease: CT findings

International Nuclear Information System (INIS)

Krupniewski, Leszek; Palczewski, Piotr; Gołębiowski, Marek; Kosińska-Kaczyńska, Katarzyna

2012-01-01

Although sickle-cell anemia (SCA) is common in black Americans, Sub-Saharan Africa and in the Mediterranean area, the disease is rare in the temperate climate zone. The manifestations of the disease are related mainly to the production of abnormal hemoglobin that leads to organ ischemia and increased susceptibility to infection caused by functional asplenia. The authors present CT findings in a 39-year-old black woman diagnosed due to abdominal pain, lymphadenopathy and fever. CT of the thorax and abdomen demonstrated changes in the liver, spleen, and skeletal system suggestive of SCA complicated with spondylodiscitis in the thoracic spine. Hepatomegaly and small calcified spleen are typical findings in older homozygotic patients with SCA. The lesions in the skeleton may be related either to intramedullary hematopoiesis or osteonecrosis and osteomyelitis. In the latter case, diffuse osteosclerosis and H-shaped vertebrae are most typical. Tuberculous spondylodiscitis is characterized by the location in the thoracic region, preferential involvement of anterior elements, relative sparing of intervertebral discs, and cold abscesses

Tuberculous spondylodiscitis in a patient with a sickle-cell disease: CT findings.

Science.gov (United States)

Krupniewski, Leszek; Palczewski, Piotr; Gołębiowski, Marek; Kosińska-Kaczyńska, Katarzyna

2012-01-01

Although sickle-cell anemia (SCA) is common in black Americans, Sub-Saharan Africa and in the Mediterranean area, the disease is rare in the temperate climate zone. The manifestations of the disease are related mainly to the production of abnormal hemoglobin that leads to organ ischemia and increased susceptibility to infection caused by functional asplenia. The authors present CT findings in a 39-year-old black woman diagnosed due to abdominal pain, lymphadenopathy and fever. CT of the thorax and abdomen demonstrated changes in the liver, spleen, and skeletal system suggestive of SCA complicated with spondylodiscitis in the thoracic spine. Hepatomegaly and small calcified spleen are typical findings in older homozygotic patients with SCA. The lesions in the skeleton may be related either to intramedullary hematopoiesis or osteonecrosis and osteomyelitis. In the latter case, diffuse osteosclerosis and H-shaped vertebrae are most typical. Tuberculous spondylodiscitis is characterized by the location in the thoracic region, preferential involvement of anterior elements, relative sparing of intervertebral discs, and cold abscesses.

L-subshell ionization studies of Au for α-particle and lithium-ion bombardment

International Nuclear Information System (INIS)

Dhal, B.B.; Nandi, T.; Padhi, H.C.; Trautmann, D.

1995-01-01

L-subshell ionization of Au has been investigated for α-particle and lithium-ion bombardments with energies 0.54-1.74 MeV u -1 and 0.65-1.44 MeV u -1 , respectively. Comparison of experimental x-ray production cross sections with the predictions of the ECPSSR and SCA theories shows reasonably good agreement for L α and L β x-rays, whereas for L γ and L γ1+5 the ECPSSR theory underestimates the cross sections by about 60% for both α-particle and lithium-ion impact, and the SCA theory agrees reasonably well. The ECPSSR theory underestimates the L 1 - and L 2 -subshell ionization cross sections and gives good agreement for the L 3 -subshell, whereas the SCA theory overestimates the L 3 -subshell ionization cross sections and gives good agreement for L 1 and L 2 . The experimental data for the total-ionization cross sections are within 25% of the predictions of both the theories. (Author)

Muscle synergy analysis in children with cerebral palsy

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Tang, Lu; Li, Fei; Cao, Shuai; Zhang, Xu; Wu, De; Chen, Xiang

2015-08-01

Objective. To explore the mechanism of lower extremity dysfunction of cerebral palsy (CP) children through muscle synergy analysis. Approach. Twelve CP children were involved in this study, ten adults (AD) and eight typically developed (TD) children were recruited as a control group. Surface electromyographic (sEMG) signals were collected bilaterally from eight lower limb muscles of the subjects during forward walking at a comfortable speed. A nonnegative matrix factorization algorithm was used to extract muscle synergies. In view of muscle synergy differences in number, structure and symmetry, a model named synergy comprehensive assessment (SCA) was proposed to quantify the abnormality of muscle synergies. Main results. There existed larger variations between the muscle synergies of the CP group and the AD group in contrast with the TD group. Fewer mature synergies were recruited in the CP group, and many abnormal synergies specific to the CP group appeared. Specifically, CP children were found to recruit muscle synergies with a larger difference in structure and symmetry between two legs of one subject and different subjects. The proposed SCA scale demonstrated its great potential to quantitatively assess the lower-limb motor dysfunction of CP children. SCA scores of the CP group (57.00 ± 16.78) were found to be significantly less (p < 0.01) than that of the control group (AD group: 95.74 ± 2.04; TD group: 84.19 ± 11.76). Significance. The innovative quantitative results of this study can help us to better understand muscle synergy abnormality in CP children, which is related to their motor dysfunction and even the physiological change in their nervous system.

The risk of acute coronary syndrome in Ramadan.

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Sriha Belguith, Asma; Baccouche, Houda; Grissa, Mohamed Habib; Boubaker, Hamdi; Bouida, Wahid; Beltaief, Kaouthar; Sekma, Adel; Fredj, Nizar; Bzeouiche, Nasri; Zina, Zied; Boukef, Riadh; Soltani, Mohamed; Nouira, Semir

2016-10-01

Data on the effect of fasting on coronary disease are rare and controversial. The aim of our study was to investigate the influence of Ramadan on the prevalence of acute coronary syndrome among chest pain patients in the emergency department of Monastir. It was a prospective study, performed in the emergency department of Fattouma Bourguiba University Hospital of Monastir, during the 3 months before, during and after Ramadan from 2012 to 2014. We included all patients with non-traumatic chest pain during the study period. Data were collected using a standardized form. The uniformity chi 2 test, ANOVA test, Kruskal-Wallis test were performed at the 5% level. Binary logistic regression model was used for multivariate analysis. The SCA prevalence was 17% a month before Ramadan, 22% during Ramadan and 28% one month after Ramadan (p = 0.007). According to the results of the multivariate analysis, the period of Ramadan is not associated with increase of risk of SCA whereas the risk doubles after Ramadan in all group (p = 0.001). In subgroups analysis, the period of R was associated with an amplification of risk in men aged more than 55 years and women older than 65 years (OR: 2.1; p = 0.020) and among subjects with hypertension (OR: 2.4, p = 0.007). Ramadan and Shawwal were not associated with an increase of risk among subjects without CVX risk factor. We have demonstrated that the risk of SCA has increased in Ramadan only among the elderly and patient with hypertension. The increased risk in Shawwal can be explained by the lifting of dietary restriction.

Therapeutic strategies in Sickle Cell Anemia: The past present and future.

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Fernandes, Queenie

2017-06-01

Sickle Cell Anemia (SCA) was one of the first hemoglobinopathies to be discovered. It is distinguished by the mutation-induced expression of a sickle cell variant of hemoglobin (HbS) that triggers erythrocytes to take a characteristic sickled conformation. The complex physiopathology of the disease and its associated clinical complications has initiated multi-disciplinary research within its field. This review attempts to lay emphasis on the evolution, current standpoint and future scope of therapeutic strategies in SCA. Copyright © 2017 Elsevier Inc. All rights reserved.

Acoustic noise improves motor learning in spontaneously hypertensive rats, a rat model of attention deficit hyperactivity disorder.

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Söderlund, Göran B W; Eckernäs, Daniel; Holmblad, Olof; Bergquist, Filip

2015-03-01

The spontaneously hypertensive (SH) rat model of ADHD displays impaired motor learning. We used this characteristic to study if the recently described acoustic noise benefit in learning in children with ADHD is also observed in the SH rat model. SH rats and a Wistar control strain were trained in skilled reach and rotarod running under either ambient noise or in 75 dBA white noise. In other animals the effect of methylphenidate (MPH) on motor learning was assessed with the same paradigms. To determine if acoustic noise influenced spontaneous motor activity, the effect of acoustic noise was also determined in the open field activity paradigm. We confirm impaired motor learning in the SH rat compared to Wistar SCA controls. Acoustic noise restored motor learning in SH rats learning the Montoya reach test and the rotarod test, but had no influence on learning in Wistar rats. Noise had no effect on open field activity in SH rats, but increased corner time in Wistar. MPH completely restored rotarod learning and performance but did not improve skilled reach in the SH rat. It is suggested that the acoustic noise benefit previously reported in children with ADHD is shared by the SH rat model of ADHD, and the effect is in the same range as that of stimulant treatment. Acoustic noise may be useful as a non-pharmacological alternative to stimulant medication in the treatment of ADHD. Copyright © 2014 The Authors. Published by Elsevier B.V. All rights reserved.

Chronic inflammatory state in sickle cell anemia patients is associated with HBB(*)S haplotype.

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Bandeira, Izabel C J; Rocha, Lillianne B S; Barbosa, Maritza C; Elias, Darcielle B D; Querioz, José A N; Freitas, Max Vitor Carioca; Gonçalves, Romélia P

2014-02-01

The chronic inflammatory state in sickle cell anemia (SCA) is associated with several factors such as the following: endothelial damage; increased production of reactive oxygen species; hemolysis; increased expression of adhesion molecules by leukocytes, erythrocytes, and platelets; and increased production of proinflammatory cytokines. Genetic characteristics affecting the clinical severity of SCA include variations in the hemoglobin F (HbF) level, coexistence of alpha-thalassemia, and the haplotype associated with the HbS gene. The different haplotypes of SCA are Bantu, Benin, Senegal, Cameroon, and Arab-Indian. These haplotypes are associated with ethnic groups and also based on the geographical origin. Studies have shown that the Bantu haplotype is associated with higher incidence of clinical complications than the other haplotypes and is therefore considered to have the worst prognosis. This study aimed to evaluate the profile of the proinflammatory cytokines interleukin-6, tumor necrosis factor-α, and interleukin-17 in patients with SCA and also to assess the haplotypes associated with beta globin cluster S (HBB(*)S). We analyzed a total of 62 patients who had SCA and had been treated with hydroxyurea; they had received a dose ranging between 15 and 25 (20.0±0.6)mg/kg/day for 6-60 (18±3.4)months; their data were compared with those for 30 normal individuals. The presence of HbS was detected and the haplotypes of the beta S gene cluster were analyzed by polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP). Our study demonstrated that SCA patients have increased inflammatory profile when compared to the healthy individuals. Further, analysis of the association between the haplotypes and inflammatory profile showed that the levels of IL-6 and TNF-α were greater in subjects with the Bantu/Bantu haplotype than in subjects with the Benin/Benin haplotype. The Bantu/Benin haplotype individuals had lower levels of cytokines than those with

On Modeling Large-Scale Multi-Agent Systems with Parallel, Sequential and Genuinely Asynchronous Cellular Automata

International Nuclear Information System (INIS)

Tosic, P.T.

2011-01-01

We study certain types of Cellular Automata (CA) viewed as an abstraction of large-scale Multi-Agent Systems (MAS). We argue that the classical CA model needs to be modified in several important respects, in order to become a relevant and sufficiently general model for the large-scale MAS, and so that thus generalized model can capture many important MAS properties at the level of agent ensembles and their long-term collective behavior patterns. We specifically focus on the issue of inter-agent communication in CA, and propose sequential cellular automata (SCA) as the first step, and genuinely Asynchronous Cellular Automata (ACA) as the ultimate deterministic CA-based abstract models for large-scale MAS made of simple reactive agents. We first formulate deterministic and nondeterministic versions of sequential CA, and then summarize some interesting configuration space properties (i.e., possible behaviors) of a restricted class of sequential CA. In particular, we compare and contrast those properties of sequential CA with the corresponding properties of the classical (that is, parallel and perfectly synchronous) CA with the same restricted class of update rules. We analytically demonstrate failure of the studied sequential CA models to simulate all possible behaviors of perfectly synchronous parallel CA, even for a very restricted class of non-linear totalistic node update rules. The lesson learned is that the interleaving semantics of concurrency, when applied to sequential CA, is not refined enough to adequately capture the perfect synchrony of parallel CA updates. Last but not least, we outline what would be an appropriate CA-like abstraction for large-scale distributed computing insofar as the inter-agent communication model is concerned, and in that context we propose genuinely asynchronous CA. (author)

Comparative study of Newtonian physiological blood flow through normal and stenosed carotid artery

Science.gov (United States)

Rahman, Mohammad Matiur; Hossain, Md. Anwar; Mamun, Khairuzzaman; Akhter, Most. Nasrin

2017-06-01

A numerical simulation is performed to investigate Newtonian physiological flows behavior on three dimensional idealized carotid artery (CA) and single stenosed (75% by area) carotid artery(SCA). The wall vessel is set as rigid during simulation. Bifurcated blood vessel are simulated by using three-dimensional flow analysis. Physiological and parabolic velocity profiles are set out to fix the conditions of inlet boundaries of artery. In other hand, physiological waveform is an important part of compilation and it is successfully done by utilization of Fourier series having sixteen harmonics. The investigation has a Reynolds number range of 94 to 1120. Low Reynolds number k — ω model has been used as governing equation. The investigation has been carried out to characterize the flow behavior of blood in two geometry, namely, (i) Normal carotid artery (CA) and (ii) Stenosed carotid artery (SCA). The Newtonian model has been used to study the physics of fluid. The findings of the two models are thoroughly compared in order to observe there behavioral sequence of flows. The numerical results were presented in terms of velocity, pressure, wall shear stress distributions and cross sectional velocities as well as the streamlines contour. Stenosis disturbs the normal pattern of blood flow through the artery as reduced area. At stenosis region velocity and peak Reynolds number rapidly increase and Reynolds number reach transitional and turbulent region. These flow fluctuation and turbulence have bad effect to the blood vessel which makes to accelerate the progress of stenosis.

Multitemporal Snow Cover Mapping in Mountainous Terrain for Landsat Climate Data Record Development

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Crawford, Christopher J.; Manson, Steven M.; Bauer, Marvin E.; Hall, Dorothy K.

2013-01-01

A multitemporal method to map snow cover in mountainous terrain is proposed to guide Landsat climate data record (CDR) development. The Landsat image archive including MSS, TM, and ETM+ imagery was used to construct a prototype Landsat snow cover CDR for the interior northwestern United States. Landsat snow cover CDRs are designed to capture snow-covered area (SCA) variability at discrete bi-monthly intervals that correspond to ground-based snow telemetry (SNOTEL) snow-water-equivalent (SWE) measurements. The June 1 bi-monthly interval was selected for initial CDR development, and was based on peak snowmelt timing for this mountainous region. Fifty-four Landsat images from 1975 to 2011 were preprocessed that included image registration, top-of-the-atmosphere (TOA) reflectance conversion, cloud and shadow masking, and topographic normalization. Snow covered pixels were retrieved using the normalized difference snow index (NDSI) and unsupervised classification, and pixels having greater (less) than 50% snow cover were classified presence (absence). A normalized SCA equation was derived to independently estimate SCA given missing image coverage and cloud-shadow contamination. Relative frequency maps of missing pixels were assembled to assess whether systematic biases were embedded within this Landsat CDR. Our results suggest that it is possible to confidently estimate historical bi-monthly SCA from partially cloudy Landsat images. This multitemporal method is intended to guide Landsat CDR development for freshwaterscarce regions of the western US to monitor climate-driven changes in mountain snowpack extent.

Misclassification of Patients with Spinocerebellar Ataxia as having Psychogenic Postural Instability based on Computerized Dynamic Posturography

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Susan J Herdman

2011-04-01

Full Text Available Specific criteria have been developed based on computerized dynamic posturography (CDP to assist clinicians in identifying patients with psychogenic balance problems1-4. Patients with known Spinocerebellar Ataxia (SCA meet several of the criteria for psychogenic balance problem and risk being misclassified as having imbalance of psychogenic origin. However, our research shows that patients with SCA may be distinguished from patients with psychogenic balance problems in several ways. We compared test performance on CDP and the observation of specific behaviors that are associated with psychogenic balance problems in patients with SCA (n = 43 and patients with known psychogenic balance problems (n = 40. Chi square analysis was used to determine if there were significant differences between the groups for the frequency of each criterion for psychogenic CDP and Observed Behaviors. Level of significance was Bonferroni corrected for multiple comparisons. Sensitivity, specificity, and positive likelihood ratios were calculated for each criterion. Hierarchical cluster analysis was used to examine whether the two patient groups demonstrated similar groupings of criteria. Comparison of the results of these analyses identified two criteria that were significantly more frequent in the Psychogenic group than in the SCA group: Regular Periodicity of sway and Circular Sway. Sensitivity, specificity and positive likelihood ratios identified two additional criteria, Inconsistent Motor Responses and Large lateral Sway that also seem to suggest a psychogenic component to a person’s imbalance. Prospective studies are needed to validate the usefulness of these findings.

Sparse Contextual Activation for Efficient Visual Re-Ranking.

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Bai, Song; Bai, Xiang

2016-03-01

In this paper, we propose an extremely efficient algorithm for visual re-ranking. By considering the original pairwise distance in the contextual space, we develop a feature vector called sparse contextual activation (SCA) that encodes the local distribution of an image. Hence, re-ranking task can be simply accomplished by vector comparison under the generalized Jaccard metric, which has its theoretical meaning in the fuzzy set theory. In order to improve the time efficiency of re-ranking procedure, inverted index is successfully introduced to speed up the computation of generalized Jaccard metric. As a result, the average time cost of re-ranking for a certain query can be controlled within 1 ms. Furthermore, inspired by query expansion, we also develop an additional method called local consistency enhancement on the proposed SCA to improve the retrieval performance in an unsupervised manner. On the other hand, the retrieval performance using a single feature may not be satisfactory enough, which inspires us to fuse multiple complementary features for accurate retrieval. Based on SCA, a robust feature fusion algorithm is exploited that also preserves the characteristic of high time efficiency. We assess our proposed method in various visual re-ranking tasks. Experimental results on Princeton shape benchmark (3D object), WM-SRHEC07 (3D competition), YAEL data set B (face), MPEG-7 data set (shape), and Ukbench data set (image) manifest the effectiveness and efficiency of SCA.

In vitro studies of Rickettsia-host cell interactions: Confocal laser scanning microscopy of Rickettsia helvetica-infected eukaryotic cell lines.

Science.gov (United States)

Speck, Stephanie; Kern, Tanja; Aistleitner, Karin; Dilcher, Meik; Dobler, Gerhard; Essbauer, Sandra

2018-02-01

Rickettsia (R.) helvetica is the most prevalent rickettsia found in Ixodes ricinus ticks in Germany. Several studies reported antibodies against R. helvetica up to 12.5% in humans investigated, however, fulminant clinical cases are rare indicating a rather low pathogenicity compared to other rickettsiae. We investigated growth characteristics of R. helvetica isolate AS819 in two different eukaryotic cell lines with focus on ultra-structural changes of host cells during infection determined by confocal laser scanning microscopy. Further investigations included partially sequencing of rickA, sca4 and sca2 genes, which have been reported to encode proteins involved in cell-to-cell spread and virulence in some rickettsiae. R. helvetica grew constantly but slowly in both cell lines used. Confocal laser scanning microscopy revealed that the dissemination of R. helvetica AS819 in both cell lines was rather mediated by cell break-down and bacterial release than cell-to-cell spread. The cytoskeleton of both investigated eukaryotic cell lines was not altered. R. helvetica possesses rickA, but its expression is not sufficient to promote actin-based motility as demonstrated by confocal laser scanning microscopy. Hypothetical Sca2 and Sca4 proteins were deduced from nucleotide gene sequences but the predicted amino acid sequences were disrupted or truncated compared to other rickettsiae most likely resulting in non-functional proteins. Taken together, these results might give a first hint to the underlying causes of the reduced virulence and pathogenicity of R. helvetica.

Asymptomatic bacteriuria in children with sickle cell anemia at The University of Nigeria teaching hospital, Enugu, South East, Nigeria

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Ikefuna Anthony N

2011-09-01

Full Text Available Abstract Background Urinary tract infection (UTI is a common cause of childhood morbidity and mortality in the tropics. Children with sickle cell anemia (SCA may have compromised kidney function arising from repeated vaso-occlusive episodes and recurrent symptomatic or asymptomatic UTI. Objectives This study aims at determining the prevalence of asymptomatic bacteriuria and sensitivity pattern in children with homozygous sickle haemoglobin compared to children with normal haemoglobin. Methods One hundred children with SCA in stable state and 100 children with normal haemoglobin aged 2-12 years were screened for asymptomatic bacteriuria using midstream urine samples. The samples were incubated aerobically at 37°C for 24 hours within one hour of collection. Children whose urine samples yielded significant bacteriuria (≥105cfu/ml on two consecutive cultures were regarded as having asymptomatic bacteriuria. Results Asymptomatic bacteriuria was noted in 6% of children with SCA and occurred more in females than males (F: M = 5:1 when compared to 2% in children with normal haemoglobin. Escherichia coli was the commonest organism isolated (33.3%. All the organisms were resistant to co-trimoxazole and ampicillin while most were sensitive to gentamicin, ceftriaxone and the quinolones. Conclusion The risk of asymptomatic bacteriuria is three times more common in children with sickle cell anemia than in children with normal haemoglobin. It is therefore important to screen SCA patients, especially the females for UTI and should be treated according to the sensitivity result of the cultured organisms.

Decreased hematocrit-to-viscosity ratio and increased lactate dehydrogenase level in patients with sickle cell anemia and recurrent leg ulcers.

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Philippe Connes

Full Text Available Leg ulcer is a disabling complication in patients with sickle cell anemia (SCA but the exact pathophysiological mechanisms are unknown. The aim of this study was to identify the hematological and hemorheological alterations associated with recurrent leg ulcers. Sixty-two SCA patients who never experienced leg ulcers (ULC- and 13 SCA patients with a positive history of recurrent leg ulcers (ULC+--with no leg ulcers at the time of the study--were recruited. All patients were in steady state condition. Blood was sampled to perform hematological, biochemical (hemolytic markers and hemorheological analyses (blood viscosity, red blood cell deformability and aggregation properties. The hematocrit-to-viscosity ratio (HVR, which reflects the red blood cell oxygen transport efficiency, was calculated for each subject. Patients from the ULC+ group were older than patients from the ULC- group. Anemia (red blood cell count, hematocrit and hemoglobin levels was more pronounced in the ULC+ group. Lactate dehydrogenase level was higher in the ULC+ group than in the ULC- group. Neither blood viscosity, nor RBC aggregation properties differed between the two groups. HVR was lower and RBC deformability tended to be reduced in the ULC+ group. Our study confirmed increased hemolytic rate and anemia in SCA patients with leg ulcers recurrence. Furthermore, our data suggest that although systemic blood viscosity is not a major factor involved in the pathophysiology of this complication, decreased red blood cell oxygen transport efficiency (i.e., low hematocrit/viscosity ratio may play a role.

Combining Ability of Pod Yield and Related Traits of Groundnut (Arachis hypogaea L. under Salinity Stress

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Md. Abul Kalam Azad

2014-01-01

Full Text Available A study was performed using 6×6 F1 diallel population without reciprocals to assess the mode of inheritance of pod yield and related traits in groundnut with imposed salinity stress. Heterosis was found for pod number and yield. Data on general and specific combining ability (gca and sca indicated additive and nonadditive gene actions. The gca: sca ratios were much less than unity suggesting predominant role of nonadditive gene effects. Cultivars “Binachinabadam-2” and “Dacca-1” and mutant M6/25/64-82 had the highest, second highest, and third highest pod number, as well as gca values, respectively. These two cultivars and another mutant M6/15/70-19 also had the highest, second highest, and third highest pod yield, as well as gca values, respectively. Therefore, “Dacca-1”, “Binachinabadam-2”, M6/25/64-82, and M6/15/70-19 could be used as source of salinity tolerance. Cross combinations showing high sca effects arising from parents with high and low gca values for any trait indicate the influence of nonadditive genes on their expression. Parents of these crosses can be used for biparental mating or reciprocal recurrent selection for developing high yielding varieties. Crosses with high sca effects having both parents with good gca effects could be exploited by pedigree breeding to get transgressive segregants.

Sudden cardiac arrest in sports - need for uniform registration

DEFF Research Database (Denmark)

Solberg, E E; Borjesson, M; Sharma, S

2016-01-01

, the importance of gender, ethnicity and age of the athlete, as well as the type and level of sporting activity. A precise instruction for autopsy practice in the case of a SCD of athletes is given, including the role of molecular samples and evaluation of possible doping. Rational decisions about cardiac...... preparticipation screening and cardiac safety at sport facilities requires increased data quality concerning incidence, aetiology and management of SCA/SCD in sports. Uniform standard registration of SCA/SCD in athletes and leisure sportsmen would be a first step towards this goal....

Heat treatment of long pulse operation for the JAERI ERL-FEL

International Nuclear Information System (INIS)

Sawamura, Masaru; Nagai, Ryoji; Kikuzawa, Nobuhiro; Hajima, Ryoichi; Minehara, Eisuke

2005-01-01

RF power sources are replaced from all-solid-state amplifiers to IOT amplifiers for the superconducting accelerators (SCAs) and a vacuum tube amplifier for the SHB of the JAERI ERL-FEL. A long pulse operation increased the pressure in the cryostat of the SCA. The single-cell SCA can be operated in 9% duty according to the time constant of the pressure decay in the cryostat. SHB can be operated in 4% duty which is limited by the frequency range of the tuners. The result of the ABAQUS calculation shows the more duty operation. (author)

Novel use Of Hydroxyurea in an African Region with Malaria (NOHARM): a trial for children with sickle cell anemia.

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Opoka, Robert O; Ndugwa, Christopher M; Latham, Teresa S; Lane, Adam; Hume, Heather A; Kasirye, Phillip; Hodges, James S; Ware, Russell E; John, Chandy C

2017-12-14

Hydroxyurea treatment is recommended for children with sickle cell anemia (SCA) living in high-resource malaria-free regions, but its safety and efficacy in malaria-endemic sub-Saharan Africa, where the greatest sickle-cell burden exists, remain unknown. In vitro studies suggest hydroxyurea could increase malaria severity, and hydroxyurea-associated neutropenia could worsen infections. NOHARM (Novel use Of Hydroxyurea in an African Region with Malaria) was a randomized, double-blinded, placebo-controlled trial conducted in malaria-endemic Uganda, comparing hydroxyurea to placebo at 20 ± 2.5 mg/kg per day for 12 months. The primary outcome was incidence of clinical malaria. Secondary outcomes included SCA-related adverse events (AEs), clinical and laboratory effects, and hematological toxicities. Children received either hydroxyurea (N = 104) or placebo (N = 103). Malaria incidence did not differ between children on hydroxyurea (0.05 episodes per child per year; 95% confidence interval [0.02, 0.13]) vs placebo (0.07 episodes per child per year [0.03, 0.16]); the hydroxyurea/placebo malaria incidence rate ratio was 0.7 ([0.2, 2.7]; P = .61). Time to infection also did not differ significantly between treatment arms. A composite SCA-related clinical outcome (vaso-occlusive painful crisis, dactylitis, acute chest syndrome, splenic sequestration, or blood transfusion) was less frequent with hydroxyurea (45%) than placebo (69%; P = .001). Children receiving hydroxyurea had significantly increased hemoglobin concentration and fetal hemoglobin, with decreased leukocytes and reticulocytes. Serious AEs, sepsis episodes, and dose-limiting toxicities were similar between treatment arms. Three deaths occurred (2 hydroxyurea, 1 placebo, and none from malaria). Hydroxyurea treatment appears safe for children with SCA living in malaria-endemic sub-Saharan Africa, without increased severe malaria, infections, or AEs. Hydroxyurea provides SCA-related laboratory and clinical

[PM2.5 pollution and aerosol optical properties in fog and haze days during autumn and winter in Beijing area].

Science.gov (United States)

Zhao, Xiu-Juan; Pu, Wei-Wei; Meng, Wei; Ma, Zhi-Qiang; Dong, Fan; He, Di

2013-02-01

A study on the PM2.5 pollution and aerosol optical properties in haze-fog days was carried out from Sep. 1st to Dec. 7th, 2011 in Beijing area by using PM2.5 concentration, aerosol scattering coefficient (sigma sca) and absorption coefficient (sigma abs) measured under urban and rural environment. The effect of weather condition on the PM25 pollution and aerosol optical properties was discussed as well. The results showed that the PM2.5 concentration, sigma sca and sigma abs, were evidently higher in haze-fog days than those in non-haze-fog days. The average PM2.5 concentrations in haze-fog days with values of 97.6 microg m-3 and 64.4 microg.m-3 were as 3.3 and 4.8 times as those in non-haze-fog days at urban and rural stations, respectively. The higher PM2.5 concentration in urban area resulted in the more frequent fog and haze phenomena than that in rural area. The PM25 concentration, sigma sca, and sigma abs were significantly higher in urban area than that in rural area in mist days, while relatively close in mist-haze days. This difference suggested that the effect of regional transport of pollution was relatively evident in mist-haze days but weak in mist day. In fog days the sigma sca showed no evident difference between urban and rural area, and was the highest in all types of fog and haze weather. The scattering property of aerosol was the strongest in fog days. The different weather conditions resulted in various characteristics of spatial distribution of PM2.5 concentration, sigma sca and sigma abs, as well as the strength of PM2,5 pollution and aerosol extinction. The pollutants transported by the strong southwest wind above the boundary layer and subsided in the boundary layer companying with the local accumulation of pollutants due to the weak diffusion resulted in the most serious haze-fog episode with the strongest PM2.5 pollution and aerosol extinction.

Spectrum of musculo-skeletal disorders in sickle cell disease in Lagos, Nigeria

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Ogo Chidiebere N

2010-01-01

Full Text Available Abstract Background Sickle cell anemia (SCA is a common genetic disease in Nigeria. Past studies from West Africa focused on isolated aspects of its medical and surgical presentations. To the best of our knowledge, the musculo-skeletal presentations amongst Nigerians with SCA have not been documented in a single all encompassing study. This work aims to prospectively document the musculo-skeletal disease burden among SCA patients. Methods In a prospective study of 318 consecutive patients with genotype-confirmed SCA at the Lagos University Teaching Hospital (LUTH, the musculo-skeletal pathologies, anatomic sites, grade of disease, age at presentation and management outcome were recorded over a one-year period. Data obtained were analyzed using Epi-Info software version 6.0. Data are presented as frequencies (% and mean values (SD as appropriate. Results The HbSS genotype occurred in 296 (93.0%, while 22 (7.0% were HbSC. 100 (31.4% patients with average presenting haemoglobin concentration of 8.2 g/100 ml in the study group, presented with 131 musculo-skeletal pathologies in 118 anatomic sites. Osteomyelitis 31 (31% and septic arthritis 19 (19% were most commonly observed in children less than 10 years. Skin ulcers and avascular necrosis (AVN occurred predominantly in the older age groups, with frequencies of 13 (13.0% and 26 (26.0% respectively. 20 (71.5% of diagnosed cases of AVN presented with radiological grade 4 disease. The lower limbs were involved in 84 (71.1% of sites affected. Lesions involving the spine were rare 11 (0.9%. Multiple presentations occurred in 89 (28.0% of patients; 62 (69.7% of which were children below 10 years. Conclusions Musculo-skeletal complications are common features of sickle cell anaemia seen in 31.4%. Infectious aetiologies predominate with long bones and joints of lower limbs more commonly affected by osteomyelitis and septic arthritis. Healthcare providers managing SCA should be aware of the potential

Renal disease in adult Nigerians with sickle cell anemia: A report of prevalence, clinical features and risk factors

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R A Bolarinwa

2012-01-01

Full Text Available Renal abnormalities in adult Nigerians with sickle cell anemia (SCA have not been extensively studied. To determine the prevalence, pattern and the associated risk factors of renal disease, 72 subjects with SCA from two centers in the southwestern Nigeria were investigated. Socio-demographic data, body mass index and clinical findings were documented. The urine analysis, serum bio-chemistry, hemogram and renal factors attributable to SCA were determined. Presence of albuminuria of at least 1+ or microalbuminuria in those negative with dipstick; and the estimated glomerular filtration rate (eGFR using the Cockcroft-Gault formula categorized subjects to various stages of chronic kidney disease (CKD. Subjects with and without albuminuria were compared to determine the relative risk associated with renal disease. Four (5.6% subjects had macro-albuminuria, while 32 (44.4% had micro-albuminuria and 30 (41.7% had hemoglobinuria. In the subjects with albuminuria, age, hematocrit, systolic blood pressure, serum creatinine, urea and creatinine clearance were numerically higher while the eGFR was numerically lower. There was no significant difference in the clinical parameters studied in the two groups of subjects. The diastolic blood pressure was significantly higher in the albuminuric group. Based on eGFR, 22 (30.6% subjects had hyperfiltration (GFR > 140 mL/min/1.73 m2, of whom 36.4% had albuminuria, 18 (25.0% had stage 1 CKD, 30 (41.7% had stage 2 CKD and two (2.7% subjects had stage 3 CKD with albuminuria. None had stage 4 and 5 CKD. We conclude that renal abnormalities, importantly albuminuria, is common in adult Nigerians with SCA and the pattern and incidence are similar to those reported from other parts of the world. Regular blood pressure monitoring, early diagnosis and active intervention are advocated to delay progression to end-stage kidney disease in view of poor outcomes of renal replacement therapy in SCA patients with nephropathy.

Evaluating the Influence of Motor Control on Selective Attention through a Stochastic Model: The Paradigm of Motor Control Dysfunction in Cerebellar Patient

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Giacomo Veneri

2014-01-01

Full Text Available Attention allows us to selectively process the vast amount of information with which we are confronted, prioritizing some aspects of information and ignoring others by focusing on a certain location or aspect of the visual scene. Selective attention is guided by two cognitive mechanisms: saliency of the image (bottom up and endogenous mechanisms (top down. These two mechanisms interact to direct attention and plan eye movements; then, the movement profile is sent to the motor system, which must constantly update the command needed to produce the desired eye movement. A new approach is described here to study how the eye motor control could influence this selection mechanism in clinical behavior: two groups of patients (SCA2 and late onset cerebellar ataxia LOCA with well-known problems of motor control were studied; patients performed a cognitively demanding task; the results were compared to a stochastic model based on Monte Carlo simulations and a group of healthy subjects. The analytical procedure evaluated some energy functions for understanding the process. The implemented model suggested that patients performed an optimal visual search, reducing intrinsic noise sources. Our findings theorize a strict correlation between the “optimal motor system” and the “optimal stimulus encoders.”

A Study on the Rheological and Mechanical Properties of Photo-Curable Ceramic/Polymer Composites with Different Silane Coupling Agents for SLA 3D Printing Technology

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Se Yeon Song

2018-02-01

Full Text Available Silane coupling agents (SCAs with different organofunctional groups were coated on the surfaces of Al2O3 ceramic particles through hydrolysis and condensation reactions, and the SCA-coated Al2O3 ceramic particles were dispersed in a commercial photopolymer based on interpenetrating networks (IPNs. The organofunctional groups that have high radical reactivity and are more effective in UV curing systems are usually functional groups based on acryl, such as acryloxy groups, methacrloxy groups, and acrylamide groups, and these silane coupling agents seem to improve interfacial adhesion and dispersion stability. The coating morphology and the coating thickness distribution of SCA-coated Al2O3 ceramic particles according to the different organofunctional groups were observed by FE-TEM. The initial dispersibility and dispersion stability of the SCA-coated Al2O3/High-temp composite solutions were investigated by relaxation NMR and Turbiscan. The rheological properties of the composite solutions were investigated by viscoelastic analysis and the mechanical properties of 3D-printed objects were observed with a nanoindenter.

Sickle cell anaemia and the experiences of young people living with the condition.

Science.gov (United States)

Foster, Nicole; Ellis, Michelle

2018-04-26

Sickle cell anaemia (SCA) is a life-threatening haemoglobin disorder acknowledged for its unpredictability and painful episodes. The aim of this qualitative literature review was to explore the experiences of young people living with SCA and its effect on their lives. The objective was to critically review selected primary research and make recommendations for practice, education and research. After reviewing potential articles using EBSCOhost, inclusion and exclusion criteria were devised and six appropriate studies were found with most participants in the 10-25 years age range. These studies were conducted in the UK and the United States. The Critical Appraisal Skills Programme qualitative research checklist was used to evaluate the articles. Thematic analysis identified three themes: acceptance, support and unpredictability, with sub-themes of spirituality and discrimination. It was clear that SCA affected multiple areas of young people's lives. Recommendations are made for practice, education and research. © 2018 RCN Publishing Company Ltd. All rights reserved. Not to be copied, transmitted or recorded in any way, in whole or part, without prior permission of the publishers.

Engraftment and reconstitution of hematopoiesis is dependent on VEGFR2 mediated regeneration of sinusoidal endothelial cells

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Hooper, Andrea T.; Butler, Jason M.; Nolan, Daniel J; Kranz, Andrea; Iida, Kaoruko; Kobayashi, Mariko; Kopp, Hans-Georg; Shido, Koji; Petit, Isabelle; Yanger, Kilangsungla; James, Daylon; Witte, Larry; Zhu, Zhenping; Wu, Yan; Pytowski, Bronislaw; Rosenwaks, Zev; Mittal, Vivek; Sato, Thomas N.; Rafii, Shahin

2011-01-01

SUMMARY The phenotypic attributes and molecular determinants for the regeneration of bone marrow (BM) sinusoidal endothelial cells (SECs) and their contribution to hematopoiesis are unknown. We show that after myelosuppression VEGFR2 activation promotes reassembly of regressed SECs, reconstituting hematopoietic stem and progenitor cells (HSPCs). VEGFR2 and VEGFR3 expression are restricted to BM vasculature, demarcating a continuous network of VEGFR2+VEGFR3+Sca1− SECs and VEGFR2+VEGFR3−Sca1+ arterioles. While chemotherapy (5FU) and sublethal irradiation (650 rad) induce minor SEC regression, lethal irradiation (950 rad) induces severe regression of SECs requiring BM transplantation (BMT) for regeneration. Conditional deletion of VEGFR2 in adult mice blocks regeneration of SECs in sublethally irradiated animals, preventing hematopoietic reconstitution. Inhibition of VEGFR2 signaling in lethally irradiated wild type mice rescued with BMT severely impairs SEC reconstruction, preventing engraftment and reconstitution of HSPCs. Therefore, activation of VEGFR2 is critical for regeneration of VEGFR3+Sca1− SECs that are essential for engraftment and restoration of HSPCs and hematopoiesis. PMID:19265665

G6PD deficiency and absence of α-thalassemia increase the risk for cerebral vasculopathy in children with sickle cell anemia.

Science.gov (United States)

Joly, Philippe; Garnier, Nathalie; Kebaili, Kamila; Renoux, Céline; Dony, Arthur; Cheikh, Nathalie; Renard, Cécile; Ceraulo, Antony; Cuzzubbo, Daniela; Pondarré, Corinne; Martin, Cyril; Pialoux, Vincent; Francina, Alain; Bertrand, Yves; Connes, Philippe

2016-04-01

The aim of this study was to test the association between hematological/genetic factors and cerebral vasculopathy in children with sickle cell anemia (SCA). A group with cerebral vasculopathy (VASC) was composed of children who had stroke (n = 6), silent infarct (n = 11), or an abnormal transcranial Doppler (n = 5). Eighty-four patients had neither positive history of stroke or silent infarct, nor abnormal transcranial Doppler (NORM group). An intermediate group (COND; n = 15) was composed of SCA children with a conditional transcranial Doppler. Biological analyses were performed on samples obtained at steady state and before the beginning of any chronic treatment. The comparisons of the three groups demonstrated a protective effect of α-thalassemia against cerebral vasculopathy through its effects on hemoglobin and reticulocyte levels. Moreover, we observed higher frequency of G6PD deficiency in the VASC group compared with the other groups. Our study confirms the key role of α-thalassemia and G6PD status in the pathophysiology of cerebral vasculopathy in SCA children. © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Heterosis and combining ability: a diallel cross of three geographically isolated populations of Pacific abalone Haliotis discus hannai Ino

Science.gov (United States)

Deng, Yuewen; Liu, Xiao; Zhang, Guofan; Wu, Fucun

2010-11-01

We conducted a complete diallel cross among three geographically isolated populations of Pacific abalone Haliotis discus hannai Ino to determine the heterosis and the combining ability of growth traits at the spat stage. The three populations were collected from Qingdao (Q) and Dalian (D) in China, and Miyagi (M) in Japan. We measured the shell length, shell width, and total weight. The magnitude of the general combining ability (GCA) variance was more pronounced than the specific combining ability (SCA) variance, which is evidenced by both the ratio of the genetic component in total variation and the GCA/SCA values. The component variances of GCA and SCA were significant for all three traits ( P<0.05), indicating the importance of additive and non-additive genetic effects in determining the expression of these traits. The reciprocal maternal effects (RE) were also significant for these traits ( P<0.05). Our results suggest that population D was the best general combiner in breeding programs to improve growth traits. The DM cross had the highest heterosis values for all three traits.

Damping in nuclear collective modes in a semiclassical fluid-dynamical approximation

International Nuclear Information System (INIS)

Vignolo, C.E.; Hernandez, Susana

1989-01-01

A semiclassical fluiddynamical model based on an usual scaling approximation (SCA) was extended to investigate the role of one and two-body dissipation in the widths of nuclear collective modes. The competition between one and two-body viscosity in: i) the collisionless (elastic) limit; ii) the hydrodynamical case and iii) the general viscoelastic regime is examined over the whole range of nuclear collision time scales. Numerical solutions are investigated for the first magnetic 2 - twist mode in 208 Pb. (Author) [es

Effect of exogenous apelin-13 on cardiac stem cell mobilization in rats with myocardial infarction

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Nan ZHENG

2013-11-01

Full Text Available Objective　To explore the protective effect of exogenous apelin-13 on heart after acute myocardial infarction (AMI in rats and its mechanism. Methods　SD rats were randomly divided into 3 groups: sham-operated group (n=6, control group (AMI + saline solution, n=12, experimental group (AMI + apelin-13, n=12. Four rats died in the control group, and five in the experimental group. The rest rats of both control and experimental groups underwent intramyocardial injection with saline solution 20μl and apelin-13 0.2μg/20μl within 5min after coronary artery ligation, respectively. The rats of sham-operated group underwent thoracic surgery without both coronary artery ligation and drug injection. Echocardiography was performed and myocardial infarct size was measured to evaluate the changes of cardiac function. Immunohistochemical staining method was used to detect the positive expression of C-kit, Flk1 and Sca1 in myocardial tissue. Western blotting and RT-PCR were used to quantitatively examine the expression levels of C-kit, Flk1 and Sca1 protein and mRNA in myocardial tissue. Results　The results of echocardiography and myocardial infarct size measurement showed that cardiac function of rats was improved more significantly in experimental group than in control group (EF: 68.43%±2.06% in experimental group and 46.40%±15.18% in control group; FS: 33.70%±1.55% in experimental group and 20.73%±8.14% in control group; infarction myocardial area percentage: 16.10%±3.08% in experimental group and 33.83%±5.64% in control group; P<0.05. Immunohistochemical staining of C-kit, Flk1 and Sca1 was negative in sham-operated group and positive or strong positive both in experimental group and control group. Western blotting and RT-PCR showed that the protein and mRNA expression of C-kit, Flk1 and Sca1 were significantly higher in experimental group than in control group (protein level: C-kit 0.48±0.17 vs 1.05±0.08, Flk1 0.40±0.26 vs 0.88±0.10, Sca1

Delayed hemolytic transfusion reaction presenting as a painful crisis in a patient with sickle cell anemia.

Science.gov (United States)

Fabron, A; Moreira, G; Bordin, J O

1999-01-07

Patients with sickle cell anemia (SCA) are frequently transfused with red blood cells (RBC). Recently we reported that the calculated risk of RBC alloimmunization per transfussed unit in Brazilian patients with SCA is 1.15%. We describe a delayed hemolytic transfusion reaction (DHTR) presenting as a painful crisis in a patient with SCA. A 35-year-old Brazilian female with homozygous SCA was admitted for a program of partial exchange transfusion prior to cholecystectomy. Her blood group was O RhD positive and no atypical RBC alloantibody was detected using the indirect antiglobulin technique. Pre-transfusional hemoglobin (Hb) was 8.7 g/dL and isovolumic partial exchange transfusion was performed using 4 units of ABO compatible packed RBC. Five days after the last transfusion she developed generalized joint pain and fever of 39 degrees C. Her Hb level dropped from 12.0 g/dL to 9.3 g/dL and the unconjugated bilirrubin level rose to 27 mmol/L. She was jaundiced and had hemoglobinuria. Hemoglobin electrophoresis showed 48.7% HbS, 46.6% HbA1, 2.7% HbA2, and 2.0% HbF. The patient's extended RBC phenotype was CDe, K-k+, Kp(a-b+), Fy(a-b-), M+N+s+, Le(a+b-), Di(a-). An RBC alloantibody with specificity to the Rh system (anti-c, titer 1:16.384) was identified by the indirect antiglobulin test. The Rh phenotype of the RBC used in the last packed RBC transfusion was CcDEe. The patient was discharged, asymptomatic, 7 days after admission.

SCaN Testbed Software Development and Lessons Learned

Science.gov (United States)

Kacpura, Thomas J.; Varga, Denise M.

2012-01-01

National Aeronautics and Space Administration (NASA) has developed an on-orbit, adaptable, Software Defined Radio (SDR)Space Telecommunications Radio System (STRS)-based testbed facility to conduct a suite of experiments to advance technologies, reduce risk, and enable future mission capabilities on the International Space Station (ISS). The SCAN Testbed Project will provide NASA, industry, other Government agencies, and academic partners the opportunity to develop and field communications, navigation, and networking technologies in the laboratory and space environment based on reconfigurable, SDR platforms and the STRS Architecture.The SDRs are a new technology for NASA, and the support infrastructure they require is different from legacy, fixed function radios. SDRs offer the ability to reconfigure on-orbit communications by changing software for new waveforms and operating systems to enable new capabilities or fix any anomalies, which was not a previous option. They are not stand alone devices, but required a new approach to effectively control them and flow data. This requires extensive software to be developed to utilize the full potential of these reconfigurable platforms. The paper focuses on development, integration and testing as related to the avionics processor system, and the software required to command, control, monitor, and interact with the SDRs, as well as the other communication payload elements. An extensive effort was required to develop the flight software and meet the NASA requirements for software quality and safety. The flight avionics must be radiation tolerant, and these processors have limited capability in comparison to terrestrial counterparts. A big challenge was that there are three SDRs onboard, and interfacing with multiple SDRs simultaneously complicatesd the effort. The effort also includes ground software, which is a key element for both the command of the payload, and displaying data created by the payload. The verification of the software was an extensive effort. The challenges of specifying a suitable test matrix with reconfigurable systems that offer numerous configurations is highlighted. Since the flight system testing requires methodical, controlled testing that limits risk, a nearly identical ground system to the on-orbit flight system was required to develop the software and write verification procedures before it was installed and tested on the flight system. The development of the SCAN testbed was an accelerated effort to meet launch constraints, and this paper discusses tradeoffs made to balance needed software functionality and still maintain the schedule. Future upgrades are discussed that optimize the avionics and allow experimenters to utilize the SCAN testbed potential.

Large bandwidth RGC transimpedance preamplifier design in SCA

International Nuclear Information System (INIS)

Wang Ke; Wang Zheng; Liu Zhen'an; Wei Wei; Lu Weiguo; Gary Varner

2009-01-01

A Large Bandwidth RGC Transimpedance Preamplifier is designed for amplifying the high-fidelity timing signal in Switch Capacitance Array chip application. This amplifier have characteristics of low input impedance, large bandwidth, high transimpedance. It will be made under TSMC 0.25μm CMOS technology, and the supply voltage is single 2.5 V. Simulation results indicate: the transimpedance is 5000 ohm, -3dB BW is 953 MHz, and the detector output capacitance have litter effect on the bandwidth in some range. (authors)

Distributed snow modeling suitable for use with operational data for the American River watershed.

Science.gov (United States)

Shamir, E.; Georgakakos, K. P.

2004-12-01

The mountainous terrain of the American River watershed (~4300 km2) at the Western slope of the Northern Sierra Nevada is subject to significant variability in the atmospheric forcing that controls the snow accumulation and ablations processes (i.e., precipitation, surface temperature, and radiation). For a hydrologic model that attempts to predict both short- and long-term streamflow discharges, a plausible description of the seasonal and intermittent winter snow pack accumulation and ablation is crucial. At present the NWS-CNRFC operational snow model is implemented in a semi distributed manner (modeling unit of about 100-1000 km2) and therefore lump distinct spatial variability of snow processes. In this study we attempt to account for the precipitation, temperature, and radiation spatial variability by constructing a distributed snow accumulation and melting model suitable for use with commonly available sparse data. An adaptation of the NWS-Snow17 energy and mass balance that is used operationally at the NWS River Forecast Centers is implemented at 1 km2 grid cells with distributed input and model parameters. The input to the model (i.e., precipitation and surface temperature) is interpolated from observed point data. The surface temperature was interpolated over the basin based on adiabatic lapse rates using topographic information whereas the precipitation was interpolated based on maps of climatic mean annual rainfall distribution acquired from PRISM. The model parameters that control the melting rate due to radiation were interpolated based on aspect. The study was conducted for the entire American basin for the snow seasons of 1999-2000. Validation of the Snow Water Equivalent (SWE) prediction is done by comparing to observation from 12 snow Sensors. The Snow Cover Area (SCA) prediction was evaluated by comparing to remotely sensed 500m daily snow cover derived from MODIS. The results that the distribution of snow over the area is well captured and the

Maxillary sinus marrow hyperplasia in sickle cell anemia