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  1. Neuroimaging of Lipid Storage Disorders

    Science.gov (United States)

    Rieger, Deborah; Auerbach, Sarah; Robinson, Paul; Gropman, Andrea

    2013-01-01

    Lipid storage diseases, also known as the lipidoses, are a group of inherited metabolic disorders in which there is lipid accumulation in various cell types, including the central nervous system, because of the deficiency of a variety of enzymes. Over time, excessive storage can cause permanent cellular and tissue damage. The brain is particularly…

  2. Lysosomal Storage Disorders and Malignancy

    Directory of Open Access Journals (Sweden)

    Gregory M. Pastores

    2017-02-01

    Full Text Available Lysosomal storage disorders (LSDs are infrequent to rare conditions caused by mutations that lead to a disruption in the usual sequential degradation of macromolecules or their transit within the cell. Gaucher disease (GD, a lipidosis, is among the most common LSD, with an estimated incidence of 1 in 40,000 among the Caucasian, non-Jewish population. Studies have indicated an increased frequency of polyclonal and monoclonal gammopathy among patients with GD. It has been shown that two major sphingolipids that accumulate in GD, namely, β-glucosylceramide 22:0 (βGL1-22 and glucosylsphingosine (LGL1, can be recognized by a distinct subset of CD1d-restricted human and murine type II natural killer T (NKT cells. Investigations undertaken in an affected mouse model revealed βGL1-22- and LGL1-specific NKT cells were present and constitutively promoted the expression of a T-follicular helper (TFH phenotype; injection of these lipids led to downstream induction of germinal center B cells, hypergammaglobulinemia, and the production of antilipid antibodies. Subsequent studies have found clonal immunoglobulin in 33% of sporadic human monoclonal gammopathies is also specific for the lysolipids LGL1 and lysophosphatidylcholine (LPC. Furthermore, substrate reduction ameliorated GD-associated gammopathy in mice. It had been hypothesized that chronic antigenic stimulation by the abnormal lipid storage and associated immune dysregulation may be the underlying mechanism for the increased incidence of monoclonal and polyclonal gammopathies, as well as an increased incidence of multiple myeloma in patients with GD. Current observations support this proposition and illustrate the value of investigations into rare diseases, which as ‘experiments of nature’ may provide insights into conditions found in the general population that continue to remain incompletely understood.

  3. Red blood cell storage duration and long-term mortality in patients undergoing cardiac intervention

    DEFF Research Database (Denmark)

    Dencker, D; Pedersen, F; Engstrøm, T

    2017-01-01

    OBJECTIVES: To study the effect of red blood cell (RBC) storage duration on long-term mortality in patients undergoing cardiac intervention. BACKGROUND: RBCs undergo numerous structural and functional changes during storage. Observational studies have assessed the association between RBC storage...... duration and patient outcomes with conflicting results. METHODS: Between January 2006 and December 2014, 82 408 patients underwent coronary angiography. Of these, 1856 patients received one to four RBC units within 30 days after this procedure. Patients were allocated according to length of RBC storage...

  4. Substrate reduction therapy of glycosphingolipid storage disorders

    NARCIS (Netherlands)

    Aerts, Johannes M. F. G.; Hollak, Carla E. M.; Boot, Rolf G.; Groener, Johanna E. M.; Maas, Mario

    2006-01-01

    In the last 15 years enormous progress has been made regarding therapy of type I Gaucher disease, a severely disabling disorder characterized by intralysosomal storage of glucosylceramide in tissue macrophages. Effective enzyme replacement therapy of type I Gaucher disease, based on chronic

  5. Attention-deficit/hyperactivity disorder in children undergoing peritoneal dialysis.

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    Yousefichaijan, Parsa; Sharafkhah, Mojtaba; Vazirian, Shams; Seyedzadeh, Abolhasan; Rafeie, Mohammad; Salehi, Bahman; Amiri, Mohammad; Ebrahimimonfared, Mohsen

    2015-03-01

    Attention-deficit/hyperactivity disorder (ADHD) is the most common childhood psychiatric disorder. This disorder is more prevalent in some chronic disease. The aim of this study was to investigate ADHD in children with end-stage renal disease (ESRD) undergoing continuous ambulatory peritoneal dialysis (CAPD) and to compare the results with those of healthy children. This case-control study was conducted for six months (December 22, 2013 to June 21, 2014) on five to 16-year-old children, visiting the Pediatric Dialysis Unit of Amirkabir Hospital, Arak, Iran, and Taleghani Hospital, Kermanshah, Iran. A total of 100 children with ESRD who had undergone CAPD for at least six months and 100 healthy children were included in this study as case and control groups, respectively. ADHD was diagnosed by Conner's Parent Rating Scale-48 (CPRS-48) and DSM-IV-TR criteria, and was confirmed through consultation by psychologist. Data were analyzed by Binomial test in SPSS 18. The ADHD inattentive type was observed in 16 cases (16%) with CAPD and five controls (5%) (P = 0.01). Moreover, ADHD hyperactive-impulsive type was observed in 27 cases (27%) with CAPD and seven controls (9%) (P = 0.002). Despite these significant differences, no children were diagnosed with ADHD combined type among all subjects. Inattentive type and hyperactive-impulsive type of ADHD are more prevalent in children with ESRD undergoing CAPD. Therefore screening methods for ADHD is necessary in these patients.

  6. Autophagy, lipophagy and lysosomal lipid storage disorders.

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    Ward, Carl; Martinez-Lopez, Nuria; Otten, Elsje G; Carroll, Bernadette; Maetzel, Dorothea; Singh, Rajat; Sarkar, Sovan; Korolchuk, Viktor I

    2016-04-01

    Autophagy is a catabolic process with an essential function in the maintenance of cellular and tissue homeostasis. It is primarily recognised for its role in the degradation of dysfunctional proteins and unwanted organelles, however in recent years the range of autophagy substrates has also been extended to lipids. Degradation of lipids via autophagy is termed lipophagy. The ability of autophagy to contribute to the maintenance of lipo-homeostasis becomes particularly relevant in the context of genetic lysosomal storage disorders where perturbations of autophagic flux have been suggested to contribute to the disease aetiology. Here we review recent discoveries of the molecular mechanisms mediating lipid turnover by the autophagy pathways. We further focus on the relevance of autophagy, and specifically lipophagy, to the disease mechanisms. Moreover, autophagy is also discussed as a potential therapeutic target in several key lysosomal storage disorders. Copyright © 2016 The Authors. Published by Elsevier B.V. All rights reserved.

  7. Lysosomal exocytosis and lipid storage disorders.

    Science.gov (United States)

    Samie, Mohammad Ali; Xu, Haoxing

    2014-06-01

    Lysosomes are acidic compartments in mammalian cells that are primarily responsible for the breakdown of endocytic and autophagic substrates such as membranes, proteins, and lipids into their basic building blocks. Lysosomal storage diseases (LSDs) are a group of metabolic disorders caused by genetic mutations in lysosomal hydrolases required for catabolic degradation, mutations in lysosomal membrane proteins important for catabolite export or membrane trafficking, or mutations in nonlysosomal proteins indirectly affecting these lysosomal functions. A hallmark feature of LSDs is the primary and secondary excessive accumulation of undigested lipids in the lysosome, which causes lysosomal dysfunction and cell death, and subsequently pathological symptoms in various tissues and organs. There are more than 60 types of LSDs, but an effective therapeutic strategy is still lacking for most of them. Several recent in vitro and in vivo studies suggest that induction of lysosomal exocytosis could effectively reduce the accumulation of the storage materials. Meanwhile, the molecular machinery and regulatory mechanisms for lysosomal exocytosis are beginning to be revealed. In this paper, we first discuss these recent developments with the focus on the functional interactions between lipid storage and lysosomal exocytosis. We then discuss whether lysosomal exocytosis can be manipulated to correct lysosomal and cellular dysfunction caused by excessive lipid storage, providing a potentially general therapeutic approach for LSDs. Copyright © 2014 by the American Society for Biochemistry and Molecular Biology, Inc.

  8. Lysosomal exocytosis and lipid storage disorders

    Science.gov (United States)

    Samie, Mohammad Ali; Xu, Haoxing

    2014-01-01

    Lysosomes are acidic compartments in mammalian cells that are primarily responsible for the breakdown of endocytic and autophagic substrates such as membranes, proteins, and lipids into their basic building blocks. Lysosomal storage diseases (LSDs) are a group of metabolic disorders caused by genetic mutations in lysosomal hydrolases required for catabolic degradation, mutations in lysosomal membrane proteins important for catabolite export or membrane trafficking, or mutations in nonlysosomal proteins indirectly affecting these lysosomal functions. A hallmark feature of LSDs is the primary and secondary excessive accumulation of undigested lipids in the lysosome, which causes lysosomal dysfunction and cell death, and subsequently pathological symptoms in various tissues and organs. There are more than 60 types of LSDs, but an effective therapeutic strategy is still lacking for most of them. Several recent in vitro and in vivo studies suggest that induction of lysosomal exocytosis could effectively reduce the accumulation of the storage materials. Meanwhile, the molecular machinery and regulatory mechanisms for lysosomal exocytosis are beginning to be revealed. In this paper, we first discuss these recent developments with the focus on the functional interactions between lipid storage and lysosomal exocytosis. We then discuss whether lysosomal exocytosis can be manipulated to correct lysosomal and cellular dysfunction caused by excessive lipid storage, providing a potentially general therapeutic approach for LSDs. PMID:24668941

  9. Clinical neurogenetics: neuropathic lysosomal storage disorders.

    Science.gov (United States)

    Pastores, Gregory M; Maegawa, Gustavo H B

    2013-11-01

    The lysosomal storage disorders are a clinically heterogeneous group of inborn errors of metabolism, associated with the accumulation of incompletely degraded macromolecules within several cellular sites. Affected individuals present with a broad range of clinical problems, including hepatosplenomegaly and skeletal dysplasia. Onset of symptoms may range from birth to adulthood. Most are associated with neurologic features. Later-onset forms are often misdiagnosed as symptoms, which might include psychiatric manifestations, are slowly progressive, and may precede other neurologic or systemic features. Symptomatic care, which remains the mainstay for most subtypes, can lead to significant improvement in quality of life. Copyright © 2013 Elsevier Inc. All rights reserved.

  10. Mitochondrial Dysfunction in Lysosomal Storage Disorders

    Directory of Open Access Journals (Sweden)

    Mario de la Mata

    2016-10-01

    Full Text Available Lysosomal storage diseases (LSDs describe a heterogeneous group of rare inherited metabolic disorders that result from the absence or loss of function of lysosomal hydrolases or transporters, resulting in the progressive accumulation of undigested material in lysosomes. The accumulation of substances affects the function of lysosomes and other organelles, resulting in secondary alterations such as impairment of autophagy, mitochondrial dysfunction, inflammation and apoptosis. LSDs frequently involve the central nervous system (CNS, where neuronal dysfunction or loss results in progressive neurodegeneration and premature death. Many LSDs exhibit signs of mitochondrial dysfunction, which include mitochondrial morphological changes, decreased mitochondrial membrane potential (ΔΨm, diminished ATP production and increased generation of reactive oxygen species (ROS. Furthermore, reduced autophagic flux may lead to the persistence of dysfunctional mitochondria. Gaucher disease (GD, the LSD with the highest prevalence, is caused by mutations in the GBA1 gene that results in defective and insufficient activity of the enzyme β-glucocerebrosidase (GCase. Decreased catalytic activity and/or instability of GCase leads to accumulation of glucosylceramide (GlcCer and glucosylsphingosine (GlcSph in the lysosomes of macrophage cells and visceral organs. Mitochondrial dysfunction has been reported to occur in numerous cellular and mouse models of GD. The aim of this manuscript is to review the current knowledge and implications of mitochondrial dysfunction in LSDs.

  11. Lysosomal storage disorders: A review of the musculoskeletal features.

    Science.gov (United States)

    James, Rebecca A; Singh-Grewal, Davinder; Lee, Senq-J; McGill, Jim; Adib, Navid

    2016-03-01

    The lysosomal storage disorders are a collection of progressive, multisystem disorders that frequently present in childhood. Their timely diagnosis is paramount as they are becoming increasingly treatable. Musculoskeletal manifestations often occur early in the disease course, hence are useful as diagnostics clues. Non-inflammatory joint stiffness or pain, carpal tunnel syndrome, trigger fingers, unexplained pain crises and short stature should all prompt consideration of a lysosomal storage disorder. Recurrent ENT infections, hepatosplenomegaly, recurrent hernias and visual/hearing impairment - especially when clustered together - are important extra-skeletal features. As diagnostic and therapeutic options continue to evolve, children with lysosomal storage disorders and their families are facing more sophisticated options for screening and treatment. The aim of this article is to highlight the paediatric presentations of lysosomal storage disorders, with an emphasis on the musculoskeletal features. © 2016 Paediatrics and Child Health Division (The Royal Australasian College of Physicians).

  12. Is Menkes Syndrome a Copper Storage Disorder

    DEFF Research Database (Denmark)

    Heydorn, Kaj; Damsgaard, Else; Horn, N.

    1978-01-01

    is not a simple deficiency syndrome. There is strong evidence that the fetus is affected and that the abnormal Cu distribution is a manifestation of this. Some data demonstrating that significant extra-hepatic storage of Cu is also present in affected males during postnatal life is reported. Cu was determined...

  13. Gaucher disease and other storage disorders.

    Science.gov (United States)

    Grabowski, Gregory A

    2012-01-01

    In 1882, Philippe Gaucher described a 32-year-old woman with massive splenomegaly and unusually large cells in the spleen, which he called a "primary epithelioma of the spleen." The systemic nature and inheritance of the disease and its variants involving the viscera and CNS were described over the next century. The delineation of the causal enzymatic defects, genetics, molecular pathology, and genomics have provided pathogenic insights into the phenotypic spectrum and the bases for development of specific therapies for what is now known as Gaucher disease. As a prototype, the clinically and economically successful intracellular enzyme therapy provided the impetus for the expansion of similar research and therapeutic developments for other lysosomal storage diseases (LSDs) and orphan diseases, including Fabry, Pompe, and Niemann-Pick diseases, as well as several mucopolysaccharidoses. Continuing studies of such LSDs, which occur as a group in more than 7000 live births, have revealed the complex molecular interdigitation with the autophagy and apoptotic pathways and proteostasis and the impact of disruptions of the lysosomal/autophagy and proteostasis systems on more common diseases has been recognized. Examples include age-related neurodegenerative diseases (eg, Parkinson disease and Gaucher disease), idiopathic hypertrophic myocardiopathies, stroke and renal failure (eg, Fabry disease), and Nonalcoholic Fatty Liver Disease/Nonalcoholic SteatoHepatitis (NAFLD/NASH) and atherosclerosis (eg, lysosomal acid lipase deficiencies). Although perceived as rare, the availability of treatment and the impact of the LSDs on more common diseases require their integration into routine clinical practice.

  14. Cerebrotendinous xanthomatosis (a rare lipid storage disorder): a case report

    OpenAIRE

    Razi, Syed Mohd; Gupta, Abhinav Kumar; Gupta, Deepak Chand; Gutch, Manish; Gupta, Keshav Kumar; Usman, Syeda Iqra

    2016-01-01

    Background Cerebrotendinous xanthomatosis is a very rare autosomal recessive lipid storage disorder affecting bile acid biosynthesis. It is manifested by subtle neurological and non-neurological symptoms due to abnormal tissue lipid deposition. Diagnosis is usually delayed but early diagnosis and replacement therapy can prevent devastating neurological sequelae. Case presentation We present a case of a 25-year-old Asian Indian woman who presented with gait difficulty, fusiform swellings of bi...

  15. Prevalence of mood disorders and utility of the PRIME-MD in patients undergoing radiation therapy

    International Nuclear Information System (INIS)

    Leopold, Kenneth A.; Ahles, Tim A.; Walch, Susan; Amdur, Robert J.; Mott, Leila A.; Wiegand-Packard, Linda; Oxman, Thomas E.

    1998-01-01

    Purpose: To validate a short, structured interview procedure that allows practicing oncologists to quickly and reliably identify mood disorders in their patients, and to estimate the prevalence and types of mood disorders in a radiation therapy patient setting, noting relationships between mood disorders and patient characteristics. Methods: Consecutive, eligible adult patients from the practices of two radiation oncologists were administered the Primary Care Evaluation of Mental Disorders (PRIME-MD) by the treating physician. A subset of these patients was also evaluated with the SCID, administered by trained mental health care personnel. Agreement between the two instruments was examined using the kappa statistic. Prevalence of mood disorders was determined from the PRIME-MD. The significance of relationships between patient characteristics and mood disorders was examined by chi-square and ANOVA analysis, and subsequently by multivariate logistic regression analysis. Results: One hundred twenty-two patients were studied. Fifty-three of these were administered the SCID. Agreement between the two instruments was very good (kappa = 0.70). A diagnosis of a depressive or anxiety disorder by the PRIME-MD was made in 59 of the 122 patients (48%, 95% confidence interval = 39%, 58%). Multivariate analysis showed that a diagnosis of a depressive mood disorder was significantly related to pain intensity and prior history of depression. Conclusion: We have demonstrated the validity and feasibility of the PRIME-MD administered by oncologists in making diagnoses of mood disorders. The prevalence of mood disorders in our set of patients undergoing a course of RT was nearly 50%. Future studies should describe the natural history of these disorders, and determine optimal intervention strategies

  16. Assessment of Quality of Life in Patients With Skin Disorders Undergoing Ayurvedic Panchakarma (Biopurification) as Management.

    Science.gov (United States)

    Deshpande, Harish; Shivakumar; Kavita, M B; Tripathy, T B; Chaturvedi, Ashutosh

    2016-07-01

    Chronic skin conditions can have a negative impact on one's quality of life, affecting their physical, functional, and emotional well-being. Whereas biopurifactory measures (panchakarma) of Ayurveda claims to provide better quality of life after treatment. Hence current study is planned to provide evidence in patients with skin disorders, undergoing Ayurvedic treatment. Sixty patients with skin disorder, who underwent purification therapies like therapeutic emesis and therapeutic purgation, were randomly placed in 2 groups to assess quality of life. Quality of life assessment was done with the help of Skindex-29 among the patients before and after Ayurvedic purification therapy. Thereafter, the quality of life assessment was done on the first follow-up. A statistically significant improvement in the quality of life domains-emotions, functioning, and symptoms-after the Ayurvedic management was observed with P value Ayurveda purification therapies. © The Author(s) 2015.

  17. JCL Roundtable: enzyme replacement therapy for lipid storage disorders.

    Science.gov (United States)

    Brown, W Virgil; Desnick, Robert J; Grabowski, Gregory A

    2014-01-01

    There are several inherited disorders that involve abnormal storage of lipids in tissues leading to severe compromise of organs. Sadly, these are often accompanied by lifelong morbidity and early mortality. Disorders such as Gaucher, Fabry, and lysosomal acid lipase deficiencies (Wolman and cholesteryl ester storage diseases) have been known for many years, and provide a difficult and frustrating set of problems for patients, their families, and their physicians. With recombinant methods of protein synthesis, it is now possible to literally replace the defective enzymes that underlie the basic pathophysiology of many such disorders. The delivery of these enzymes into the affected cells is possible because of their location in the lysosomes where the natural degradation of their lipid substrates occurs. I have asked 2 well-known investigators to join us for this Roundtable. These are professors who have been involved with the research that has made this type of therapy possible and who have participated in the clinical trials that demonstrated the value of enzyme replacement therapy. They are Dr. Robert Desnick, dean of Genetic and Genomic Medicine and professor and chairman emeritus of the Department of Genetics and Genomic Sciences at the Icahn School of Medicine at Mount Sinai in New York City, and Dr. Gregory Grabowski, professor of Microbiology, Biochemistry, and Pediatrics, at the University of Cincinnati College of Medicine. Dr. Grabowski recently retired from that school to become the chief science officer of Synageva, a company involved in producing enzymes for this type of therapy. Copyright © 2014 National Lipid Association. Published by Elsevier Inc. All rights reserved.

  18. Treatment for preventing bleeding in people with haemophilia or other congenital bleeding disorders undergoing surgery.

    Science.gov (United States)

    Coppola, Antonio; Windyga, Jerzy; Tufano, Antonella; Yeung, Cindy; Di Minno, Matteo Nicola Dario

    2015-02-09

    In people with haemophilia or other congenital bleeding disorders undergoing surgical interventions, haemostatic treatment is needed in order to correct the underlying coagulation abnormalities and minimise the bleeding risk. This treatment varies according to the specific haemostatic defect, its severity and the type of surgical procedure. The aim of treatment is to ensure adequate haemostatic coverage for as long as the bleeding risk persists and until wound healing is complete. To assess the effectiveness and safety of different haemostatic regimens (type, dose and duration, modality of administration and target haemostatic levels) administered in people with haemophilia or other congenital bleeding disorders for preventing bleeding complications during and after surgical procedures. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group's Coagulopathies Trials Register, compiled from electronic database searches and handsearching of journals and conference abstract books. We also searched the reference lists of relevant articles and reviews.Date of the last search: 20 November 2014. Randomised and quasi-randomised controlled trials comparing any hemostatic treatment regimen to no treatment or to another active regimen in children and adults with haemophilia or other congenital bleeding disorders undergoing any surgical intervention. Two authors independently assessed trials (eligibility and risks of bias) and extracted data. Meta-analyses were performed on available and relevant data. Of the 16 identified trials, four (112 participants) were eligible for inclusion.Two trials evaluated 59 people with haemophilia A and B undergoing 63 dental extractions. Trials compared the use of a different type (tranexamic acid or epsilon-aminocaproic acid) and regimen of antifibrinolytic agents as haemostatic support to the initial replacement treatment. Neither trial specifically addressed mortality (one of this review's primary outcomes); however, in the frame

  19. Orofacial Disorders of Patients with End Stage Renal Disease Undergoing Haemodialysis

    Directory of Open Access Journals (Sweden)

    Yohana Gowara

    2015-05-01

    Full Text Available Several orofacial disorders in patients with end stage renal disease (ESRD undergoing hemodialysis have been reported. However, up to the present, particularly in Indonesia, such data still limited. Objective: the purpose of this study was to assess the orofacial disorders in patients with ESDR undergoing hemodialysis at Cipto Mangunkusumo Hospital, Indonesia. Methods: The study was conducted through observation using a cross-sectional design. The subjects were selected by consecutive sampling. Ninety-three patients fulfilled the inclusion criteria and enrolled in this study. They participated in the structural interview-using questionnaire assessing subjective complaints; clinical examinations; and salivary measurements. Results: Xerostomia (82.8% dysgeusia (66.7%, metal taste (57%, perioral anesthesia (24.7% were the common symptoms. Clinical findings consisted of tongue coating (100%, calculus deposits (97.8%, pallor of oral mucous (94.6%, sialosis (75.3%, uremic odor (40,9%, haemorrhagic spot (39.8%, angular cheilitis (37.7%, gingival bleeding (15.1%, and oral candidiasis (3.2% were also found. Salivary changes showed the increase of salivary viscosity (86%, pH (80.6%, buffer capacity (76.3% whereas decrease of mucous hydration level (79.6% and the flow rates of unstimulated (22.6% and stimulated (31.2% whole saliva were observed. Conclusion: The findings of orofacial disorders required attention and further comprehensive management to enhance the quality of life of patients with ESDR.DOI: 10.14693/jdi.v21i3.262

  20. The clinical spectrum and pathophysiology of skeletal complications in lysosomal storage disorders

    NARCIS (Netherlands)

    Clarke, Lorne A.; Hollak, Carla E. M.

    2015-01-01

    Lysosomal storage disorders affect multiple organs including the skeleton. Disorders with prominent skeletal symptoms are type 1 and 3 Gaucher disease, the mucopolysaccharidoses, the glycoproteinoses and pycnodysostosis. Clinical manifestations range from asymptomatic radiographical evidence of bone

  1. Cerebrotendinous xanthomatosis (a rare lipid storage disorder): a case report.

    Science.gov (United States)

    Razi, Syed Mohd; Gupta, Abhinav Kumar; Gupta, Deepak Chand; Gutch, Manish; Gupta, Keshav Kumar; Usman, Syeda Iqra

    2016-04-19

    Cerebrotendinous xanthomatosis is a very rare autosomal recessive lipid storage disorder affecting bile acid biosynthesis. It is manifested by subtle neurological and non-neurological symptoms due to abnormal tissue lipid deposition. Diagnosis is usually delayed but early diagnosis and replacement therapy can prevent devastating neurological sequelae. We present a case of a 25-year-old Asian Indian woman who presented with gait difficulty, fusiform swellings of bilateral tendo-Achilles and infrapatellar tendons, along with history of bilateral cataract surgery 1 year earlier. The diagnosis was made on the basis of clinical, biochemical, imaging, and histopathological analysis and replacement therapy was started. The peculiarity of the present case is the absence of any neurological manifestations which are usually the early clues to the diagnosis of cerebrotendinous xanthomatosis. The present case report emphasizes the fact that early age bilateral cataracts along with bilateral tendo-Achilles xanthomas can be early pointers toward the diagnosis of cerebrotendinous xanthomatosis.

  2. A novel transgenic mouse model of lysosomal storage disorder.

    Science.gov (United States)

    Ortiz-Miranda, Sonia; Ji, Rui; Jurczyk, Agata; Aryee, Ken-Edwin; Mo, Shunyan; Fletcher, Terry; Shaffer, Scott A; Greiner, Dale L; Bortell, Rita; Gregg, Ronald G; Cheng, Alan; Hennings, Leah J; Rittenhouse, Ann R

    2016-11-01

    Knockout technology has proven useful for delineating functional roles of specific genes. Here we describe and provide an explanation for striking pathology that occurs in a subset of genetically engineered mice expressing a rat Ca V β2a transgene under control of the cardiac α-myosin heavy chain promoter. Lesions were limited to mice homozygous for transgene and independent of native Cacnb2 genomic copy number. Gross findings included an atrophied pancreas; decreased adipose tissue; thickened, orange intestines; and enlarged liver, spleen, and abdominal lymph nodes. Immune cell infiltration and cell engulfment by macrophages were associated with loss of pancreatic acinar cells. Foamy macrophages diffusely infiltrated the small intestine's lamina propria, while similar macrophage aggregates packed liver and splenic red pulp sinusoids. Periodic acid-Schiff-positive, diastase-resistant, iron-negative, Oil Red O-positive, and autofluorescent cytoplasm was indicative of a lipid storage disorder. Electron microscopic analysis revealed liver sinusoids distended by clusters of macrophages containing intracellular myelin "swirls" and hepatocytes with enlarged lysosomes. Additionally, build up of cholesterol, cholesterol esters, and triglycerides, along with changes in liver metabolic enzyme levels, were consistent with a lipid processing defect. Because of this complex pathology, we examined the transgene insertion site. Multiple transgene copies inserted into chromosome 19; at this same site, an approximate 180,000 base pair deletion occurred, ablating cholesterol 25-hydroxylase and partially deleting lysosomal acid lipase and CD95 Loss of gene function can account for the altered lipid processing, along with hypertrophy of the immune system, which define this phenotype, and serendipitously provides a novel mouse model of lysosomal storage disorder. Copyright © 2016 the American Physiological Society.

  3. Less Is More: Substrate Reduction Therapy for Lysosomal Storage Disorders

    Directory of Open Access Journals (Sweden)

    Maria Francisca Coutinho

    2016-07-01

    Full Text Available Lysosomal storage diseases (LSDs are a group of rare, life-threatening genetic disorders, usually caused by a dysfunction in one of the many enzymes responsible for intralysosomal digestion. Even though no cure is available for any LSD, a few treatment strategies do exist. Traditionally, efforts have been mainly targeting the functional loss of the enzyme, by injection of a recombinant formulation, in a process called enzyme replacement therapy (ERT, with no impact on neuropathology. This ineffectiveness, together with its high cost and lifelong dependence is amongst the main reasons why additional therapeutic approaches are being (and have to be investigated: chaperone therapy; gene enhancement; gene therapy; and, alternatively, substrate reduction therapy (SRT, whose aim is to prevent storage not by correcting the original enzymatic defect but, instead, by decreasing the levels of biosynthesis of the accumulating substrate(s. Here we review the concept of substrate reduction, highlighting the major breakthroughs in the field and discussing the future of SRT, not only as a monotherapy but also, especially, as complementary approach for LSDs.

  4. Radiological and clinical characterization of the lysosomal storage disorders: non-lipid disorders.

    Science.gov (United States)

    Parker, E I; Xing, M; Moreno-De-Luca, A; Harmouche, E; Terk, M R

    2014-01-01

    Lysosomal storage diseases (LSDs) are a large group of genetic metabolic disorders that result in the accumulation of abnormal material, such as mucopolysaccharides, glycoproteins, amino acids and lipids, within cells. Since many LSDs manifest during infancy or early childhood, with potentially devastating consequences if left untreated, timely identification is imperative to prevent irreversible damage and early death. In this review, the key imaging features of the non-lipid or extralipid LSDs are examined and correlated with salient clinical manifestations and genetic information. Disorders are stratified based on the type of excess material causing tissue or organ dysfunction, with descriptions of the mucopolysaccharidoses, mucolipidoses, alpha-mannosidosis, glycogen storage disorder II and cystinosis. In addition, similarities and differences in radiological findings between each of these LSDs are highlighted to facilitate further recognition. Given the rare and extensive nature of the LSDs, mastery of their multiple clinical and radiological traits may seem challenging. However, an understanding of the distinguishing imaging characteristics of LSDs and their clinical correlates may allow radiologists to play a key role in the early diagnosis of these progressive and potentially fatal disorders.

  5. Autophagic dysfunction in a lysosomal storage disorder due to impaired proteolysis

    OpenAIRE

    Elrick, Matthew J.; Lieberman, Andrew P.

    2013-01-01

    Alterations in macroautophagy (hereafter referred to as “autophagy”) are a common feature of lysosomal storage disorders, and have been hypothesized to play a major role in the pathogenesis of these diseases. We have recently reported multiple defects in autophagy contributing to the lysosomal storage disorder Niemann-Pick type C (NPC). These include increased formation of autophagosomes, slowed turnover of autophagosomes secondary to impaired lysosomal proteolysis, and delivery of stored lip...

  6. Autophagic dysfunction in a lysosomal storage disorder due to impaired proteolysis.

    Science.gov (United States)

    Elrick, Matthew J; Lieberman, Andrew P

    2013-02-01

    Alterations in macroautophagy (hereafter referred to as "autophagy") are a common feature of lysosomal storage disorders, and have been hypothesized to play a major role in the pathogenesis of these diseases. We have recently reported multiple defects in autophagy contributing to the lysosomal storage disorder Niemann-Pick type C (NPC). These include increased formation of autophagosomes, slowed turnover of autophagosomes secondary to impaired lysosomal proteolysis, and delivery of stored lipids to the lysosome via autophagy. The study summarized here describes novel methods for the interrogation of individual stages of the autophagic pathway, and suggests mechanisms by which lipid storage may result in broader lysosomal dysfunction.

  7. Semantic Dementia Shows both Storage and Access Disorders of Semantic Memory

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    Yumi Takahashi

    2014-01-01

    Full Text Available Objective. Previous studies have shown that some patients with semantic dementia (SD have memory storage disorders, while others have access disorders. Here, we report three SD cases with both disorders. Methods. Ten pictures and ten words were prepared as visual stimuli to determine if the patients could correctly answer names and select pictures after hearing the names of items (Card Presentation Task, assessing memory storage disorder. In a second task, the viewing time was set at 20 or 300 msec (Momentary Presentation Task, evaluating memory access disorder using items for which correct answers were given in the first task. The results were compared with those for 6 patients with Alzheimer’s disease (AD. Results. The SD patients had lower scores than the AD group for both tasks, suggesting both storage and access disorders. The AD group had almost perfect scores on the Card Presentation Task but showed impairment on the Momentary Presentation Task, although to a lesser extent than the SD cases. Conclusions. These results suggest that SD patients have both storage and access disorders and have more severe access disorder than patients with AD.

  8. Analysis of the frequency and degree of temporomandibular disorder in patients with head and neck cancer undergoing radiotherapy

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    Douglas Roberto Pegoraro

    Full Text Available Abstract Introduction: Head and neck cancer is responsible for an increasing incidence of primary malignant neoplasm cases worldwide. Radiotherapy is one of the treatments of choice for this type of cancer, but it can cause adverse effects, such as temporomandibular disorder. The objective of this study was to characterize the degree and frequency of temporomandibular disorder in patients with head and neck cancer undergoing radiotherapy. Method: This research was quantitative, descriptive and exploratory. The sample consisted of 22 patients that answered assessment questions and the Helkimo anamnestic questionnaire, modified by Fonseca (1992. The data were collected from May to October 2014, and statistically analyzed using the Chi-square test, with a significance level of p ≤ 0.05. Results: Of the 22 patients, 86.4 % were male, with a mean age of 58.86 ± 9.41 years. Temporomandibular disorder was present in 31.8% of the subjects, based on the assessment prior to radiotherapy, and in 59.1% in the post-treatment assessment. Among all questions, the most frequent was "Do you use only one side of the mouth to chew?" with 22.7% "yes" answers, both at the first assessment and at the post treatment. Conclusion: According to the results of this study, temporomandibular disorder is a disease that is present with a high prevalence in people diagnosed with head and neck cancer undergoing radiotherapy.

  9. Response to "Treatment compliance and effectiveness in complex PTSD patients with co-morbid personality disorder undergoing stabilizing cognitive behavioral group treatment: a preliminary study"

    NARCIS (Netherlands)

    de Jongh, A.; ten Broeke, E.

    2014-01-01

    Last November, the European Journal of Psychotraumatology published an interesting paper entitled "Treatment compliance and effectiveness in complex PTSD patients with co-morbid personality disorder undergoing stabilizing cognitive behavioral group treatment: a preliminary study". This article

  10. PEG-lipid micelles enable cholesterol efflux in Niemann-Pick Type C1 disease-based lysosomal storage disorder

    Science.gov (United States)

    Brown, Anna; Patel, Siddharth; Ward, Carl; Lorenz, Anna; Ortiz, Mauren; Duross, Allison; Wieghardt, Fabian; Esch, Amanda; Otten, Elsje G.; Heiser, Laura M.; Korolchuk, Viktor I.; Sun, Conroy; Sarkar, Sovan; Sahay, Gaurav

    2016-08-01

    2-Hydroxy-propyl-β-cyclodextrin (HPβCD), a cholesterol scavenger, is currently undergoing Phase 2b/3 clinical trial for treatment of Niemann Pick Type C-1 (NPC1), a fatal neurodegenerative disorder that stems from abnormal cholesterol accumulation in the endo/lysosomes. Unfortunately, the extremely high doses of HPβCD required to prevent progressive neurodegeneration exacerbates ototoxicity, pulmonary toxicity and autophagy-based cellular defects. We present unexpected evidence that a poly (ethylene glycol) (PEG)-lipid conjugate enables cholesterol clearance from endo/lysosomes of Npc1 mutant (Npc1-/-) cells. Herein, we show that distearyl-phosphatidylethanolamine-PEG (DSPE-PEG), which forms 12-nm micelles above the critical micelle concentration, accumulates heavily inside cholesterol-rich late endosomes in Npc1-/- cells. This potentially results in cholesterol solubilization and leakage from lysosomes. High-throughput screening revealed that DSPE-PEG, in combination with HPβCD, acts synergistically to efflux cholesterol without significantly aggravating autophagy defects. These well-known excipients can be used as admixtures to treat NPC1 disorder. Increasing PEG chain lengths from 350 Da-30 kDa in DSPE-PEG micelles, or increasing DSPE-PEG content in an array of liposomes packaged with HPβCD, improved cholesterol egress, while Pluronic block copolymers capable of micelle formation showed slight effects at high concentrations. We postulate that PEG-lipid based nanocarriers can serve as bioactive drug delivery systems for effective treatment of lysosomal storage disorders.

  11. Association Between Allergies and Psychiatric Disorders in Patients Undergoing Invasive Procedures.

    Science.gov (United States)

    Aberle, Dwight; Wu, Stephanie E; Oklu, Rahmi; Erinjeri, Joseph; Deipolyi, Amy R

    Associations between allergies and psychiatric disorders have been reported in the context of depression and suicide; psychiatric disorders may affect pain perception. To investigate the relationship of allergies with psychiatric disorders and pain perception in the context of invasive procedures, specifically during tunneled hemodialysis catheter placement. We identified 89 patients (51 men, 38 women), mean age 66 years (range: 23-96), who underwent tunneled hemodialysis catheter placement (1/2014-2/2015), recording numeric rating scale pain scores, medications, psychiatric history, allergies, and smoking status. Of 89 patients, 47 patients had no allergies, and 42 had ≥1 allergy. Patients with allergies were more likely to have a pre-existing psychiatric disorder compared to those without allergies, odds ratio 2.6 (95% CI: 1.0-6.8). Having allergies did not affect procedural sedation or postprocedural pain scores. Multiple logistic regression with age, sex, smoking, presence of allergies, psychiatric history, inpatient/outpatient status, procedure time, and procedural sedation administration as inputs and postprocedural pain as the outcome showed that the only independent predictor was receiving procedural sedation (P = 0.005). Findings corroborate anecdotal reports of allergies as a marker for psychiatric history. However, having allergies was not associated with increased pain or need for more sedation. Further studies could prospectively assess whether allergies and psychiatric disorders affect patient/doctor perceptions beyond pain during invasive procedures. Copyright © 2017 The Academy of Psychosomatic Medicine. Published by Elsevier Inc. All rights reserved.

  12. Excessive burden of lysosomal storage disorder gene variants in Parkinson's disease

    NARCIS (Netherlands)

    Robak, L.A.; Jansen, I.E.; Rooij, J van; Uitterlinden, A.G.; Kraaij, R.; Jankovic, J.; Heutink, P.; Shulman, J.M.; Bloem, B.; Post, B.; Scheffer, H.; Warrenburg, B.P.C. van de; et al.,

    2017-01-01

    Mutations in the glucocerebrosidase gene (GBA), which cause Gaucher disease, are also potent risk factors for Parkinson's disease. We examined whether a genetic burden of variants in other lysosomal storage disorder genes is more broadly associated with Parkinson's disease susceptibility. The

  13. Statistical study on the thyroid disorders on Sudanese female undergoing in vitro investigations in Khartoum state

    International Nuclear Information System (INIS)

    Albaba, O. S. A.

    2002-10-01

    In this study 711 Sudanese female have been analyzed for thyroid function. Thyroid related hormones were measured thyroxine (T4), triiodothyronine (T3) and thyroid stimulating hormone (TSH). The study had been held during one complete year. The female subjects were referred to Sudan Atomic Energy Commission radioimmunoassay laboratory from different hospitals in Khartoum state. The age of females varied from less than one year up to 70 years. The age was divided into 10 years interval in order to study the dominants thyroid disorder in each interval. Statistical package for social science (SPSS) program was used in the study as data analysis tool, the clear observation from this study was the high incidence of disorders among the age between 20 up to 40 years. (Author)

  14. Body dysmorphic disorder in patients undergoing septorhinoplasty surgery: should we be performing routine screening?

    Science.gov (United States)

    Joseph, J; Randhawa, P; Hannan, S A; Long, J; Goh, S; O'Shea, N; Saleh, H; Hansen, E; Veale, D; Andrews, P

    2017-06-01

    Body dysmorphic disorder (BDD) is defined as having a preoccupation with a perceived flaw in one's appearance, which appears slight to others and significantly interferes with a person's functioning. When undetected in septorhinoplasty patients, it will often lead to poor outcomes. We performed a prospective cohort study to determine the prevalence of BDD in our patients and whether surgical correction could be considered. We recruited 34 patients being considered for septorhinoplasty in a tertiary referral rhinology clinic and a control group of 50 from the otology clinic giving a total of 84. Participants completed the Body Dysmorphic Disorder Questionnaire (BDDQ), the sino-nasal outcome test-23 (SNOT-23) and underwent nasal inspiratory peak flow (NIPF). Those found to be at high risk for BDD were referred to a clinical psychologist. Of the septorhinoplasty patients, 11 (32%) were high risk for BDD. Following psychological assessment, 7 (63%) patients were felt to be unsuitable for surgery and were offered psychological therapy. SNOT-23 scores were significantly higher in the BDD group indicating a negative impact on quality of life. NIPF readings were not significantly different in the BDD group compared to the control group. The BDDQ is a valid tool for identifying patients at risk of BDD. A close working relationship with clinical psychology has been advantageous to help the selection process of candidates for surgery when there is a high risk of BDD. © 2016 John Wiley & Sons Ltd.

  15. Motivational stage of change in young patients undergoing day treatment for eating disorders.

    Science.gov (United States)

    Bustin, Lisa A; Lane-Loney, Susan E; Hollenbeak, Christopher S; Ornstein, Rollyn M

    2013-01-01

    The objective was to determine whether motivation to change is significantly altered over the course of partial hospitalization in children and adolescents with eating disorders (EDs). This study was a retrospective chart review of 30 sets of adolescents and their parents who completed the Motivational Stage of Change for Adolescents Recovering from an Eating Disorder (MSCARED) at both intake and discharge from partial hospitalization. The main outcome variables included change in stage of change (SOC) for patients and their parents. Secondary outcomes included correlations between SOC and other baseline variables, as well as changes in SOC and psychological test scores. The SOC was significantly higher at discharge than at intake in both the patients and parents, but the two groups were not in agreement at discharge. The change in the SOC was correlated with change in Children's Eating Attitudes Test scores. Assessment of decisional balance showed correlations with SOC. Age, change in weight, and psychiatric diagnoses did not correlate with initial SOC. The MSCARED may be a useful tool for monitoring young ED patients' psychological improvements with day treatment. Initial SOC is not predictive of treatment outcomes.

  16. Risk profiles of treatment noncompletion for inpatients and outpatients undergoing alcohol disorder rehabilitation treatment

    Directory of Open Access Journals (Sweden)

    Preuss UW

    2012-05-01

    Full Text Available Ulrich W Preuss,1 Jörg Zimmermann,2,3 Gabriele Schultz,2 Anna Watzke,2 Peggy Schmidt,4 Bärbel Löhnert,5 Michael Soyka2,61Department of Psychiatry, Psychotherapy and Psychosomatics, University of Halle-Wittenberg, Halle, Germany; 2Ev Krankenhaus Bethanien GmbH, Fachklinik Gristower Wiek, Johanna-Odebrecht-Stiftung, Germany; 3Karl-Jaspers-Klinik, Fachkrankenhaus für Psychiatrie und Psychotherapie, Psychiatrieverbund Oldenburger Land, Germany; 4Department of Psychiatry, Ludwig-Maximilians-Universität Munich, Germany; 5Klientenzentrierte Problemberatung, Dachau/Munich, Germany; 6Privatklinik Meiringen, Meiringen, Switzerland Background: Rehabilitation treatment noncompletion is considered a risk factor for long term relapse in alcohol-dependent individuals. The aim of this analysis of in- and outpatients in alcohol dependence rehabilitation in Germany is to identify social, mental, and somatic risk profiles for treatment noncompletion.Methods: A total of 92 individuals from an outpatient program and 303 individuals from two inpatient rehabilitation treatment units in three different locations in Germany were recruited and assessed with a structured interview and several measures of psychopathology (personality disorders, anxiety, depression, and impulsivity at treatment admission, with termination at 12 months follow-up. Participants were subdivided into treatment completers and noncompleters for any reason.Results: A total of 10.2% of inpatients and 16.1% of outpatients did not complete treatment. Compared with treatment completers, noncompleters had a significantly lower rate of continuous abstinence at 1-year follow-up, more recent alcohol consumption before admission, and a higher rate of borderline personality disorders. Among inpatients, an elevated rate of lifetime mental disorders, depression, and suicide attempts was found among treatment noncompleters; among outpatients, treatment noncompleters were more often than completers to be

  17. Family accommodation of anxiety symptoms in youth undergoing intensive multimodal treatment for anxiety disorders and obsessive-compulsive disorder: Nature, clinical correlates, and treatment response.

    Science.gov (United States)

    La Buissonnière-Ariza, Valérie; Schneider, Sophie C; Højgaard, Davíð; Kay, Brian C; Riemann, Bradley C; Eken, Stephanie C; Lake, Peter; Nadeau, Joshua M; Storch, Eric A

    2018-01-01

    Family accommodation is associated with a range of clinical features including symptom severity, functional impairment, and treatment response. However, most previous studies in children and adolescents investigated family accommodation in samples of youth with obsessive-compulsive disorder (OCD) or anxiety disorders receiving non-intensive outpatient services. In this study, we aimed to investigate family accommodation of anxiety symptoms in a sample of youth with clinical anxiety levels undergoing an intensive multimodal intervention for anxiety disorders or OCD. We first assessed the internal consistency of the Family Accommodation Scale - Anxiety (FASA). We next examined family accommodation presentation and correlates. The FASA showed high internal consistency for all subscales and total score, and good item and subscale correlations with the total score. All parents reported at least mild accommodation, and the mean levels of family accommodation were particularly high. Child age, anxiety severity, and comorbid depressive symptoms predicted baseline accommodation. However, the association between anxiety severity and family accommodation no longer remained significant after adding the other factors to the model. In addition, family accommodation partially mediated the relationship between anxiety severity and functional impairment. Finally, post-treatment changes in family accommodation predicted changes in symptom severity and functional impairment. These findings suggest the FASA is an appropriate tool to assess family accommodation in intensive treatment samples. Further, they underline the importance of addressing family accommodation in this population given the particularly high levels of accommodating behaviors and the evidence for adverse outcomes associated with this feature. Copyright © 2017 Elsevier Inc. All rights reserved.

  18. Impaired Autophagy in the Lipid-Storage Disorder Niemann-Pick Type C1 Disease

    OpenAIRE

    Sarkar, Sovan; Carroll, Bernadette; Buganim, Yosef; Maetzel, Dorothea; Ng, Alex H.M.; Cassady, John P.; Cohen, Malkiel A.; Chakraborty, Souvik; Wang, Haoyi; Spooner, Eric; Ploegh, Hidde; Gsponer, Joerg; Korolchuk, Viktor I.; Jaenisch, Rudolf

    2013-01-01

    Autophagy dysfunction has been implicated in misfolded protein accumulation and cellular toxicity in several diseases. Whether alterations in autophagy also contribute to the pathology of lipid-storage disorders is not clear. Here, we show defective autophagy in Niemann-Pick type C1 (NPC1) disease associated with cholesterol accumulation, where the maturation of autophagosomes is impaired because of defective amphisome formation caused by failure in SNARE machinery, whereas the lysosomal prot...

  19. PEG-lipid micelles enable cholesterol efflux in Niemann-Pick Type C1 disease-based lysosomal storage disorder

    Science.gov (United States)

    Brown, Anna; Patel, Siddharth; Ward, Carl; Lorenz, Anna; Ortiz, Mauren; DuRoss, Allison; Wieghardt, Fabian; Esch, Amanda; Otten, Elsje G.; Heiser, Laura M.; Korolchuk, Viktor I.; Sun, Conroy; Sarkar, Sovan; Sahay, Gaurav

    2016-01-01

    2-Hydroxy-propyl-β-cyclodextrin (HPβCD), a cholesterol scavenger, is currently undergoing Phase 2b/3 clinical trial for treatment of Niemann Pick Type C-1 (NPC1), a fatal neurodegenerative disorder that stems from abnormal cholesterol accumulation in the endo/lysosomes. Unfortunately, the extremely high doses of HPβCD required to prevent progressive neurodegeneration exacerbates ototoxicity, pulmonary toxicity and autophagy-based cellular defects. We present unexpected evidence that a poly (ethylene glycol) (PEG)-lipid conjugate enables cholesterol clearance from endo/lysosomes of Npc1 mutant (Npc1−/−) cells. Herein, we show that distearyl-phosphatidylethanolamine-PEG (DSPE-PEG), which forms 12-nm micelles above the critical micelle concentration, accumulates heavily inside cholesterol-rich late endosomes in Npc1−/− cells. This potentially results in cholesterol solubilization and leakage from lysosomes. High-throughput screening revealed that DSPE-PEG, in combination with HPβCD, acts synergistically to efflux cholesterol without significantly aggravating autophagy defects. These well-known excipients can be used as admixtures to treat NPC1 disorder. Increasing PEG chain lengths from 350 Da-30 kDa in DSPE-PEG micelles, or increasing DSPE-PEG content in an array of liposomes packaged with HPβCD, improved cholesterol egress, while Pluronic block copolymers capable of micelle formation showed slight effects at high concentrations. We postulate that PEG-lipid based nanocarriers can serve as bioactive drug delivery systems for effective treatment of lysosomal storage disorders. PMID:27572704

  20. Hepatocellular adenoma in a woman who was undergoing testosterone treatment for gender identity disorder.

    Science.gov (United States)

    Kato, Keizo; Abe, Hiroshi; Hanawa, Noriko; Fukuzawa, Junya; Matsuo, Ryota; Yonezawa, Takeshi; Itoh, Sadahiro; Sato, Yoshiyuki; Ika, Makiko; Shimizu, Shohei; Endo, Shinji; Hano, Hiroshi; Izu, Asami; Sugitani, Masahiko; Tsubota, Akihito

    2018-03-27

    A 32-year-old Japanese woman was admitted to our hospital for the diagnosis and treatment of multiple liver tumors. She had been receiving 125 mg testosterone enanthate every 2 weeks following female-to-male gender identity disorder (GID) diagnosis at 20 years of age. Ultrasonography, computed tomography, and magnetic resonance imaging showed 11 hepatic nodular tumors with a maximum diameter of 28 mm. Liver tumors with hepatocellular adenoma (HCA) were diagnosed with needle biopsy. Segmentectomy of the left lateral lobe including two lesions, subsegmentectomy of S6 including two lesions, enucleation of each tumor in S5 and S7, and open surgical radiofrequency ablation for each tumor in S4 and S7 were performed. Immunohistochemical specimens showed that the tumor cells were diffusely and strongly positive for glutamine synthetase and that the nuclei were ectopically positive for β-catenin. Thus, the tumors were diagnosed as β-catenin-activated HCA (b-HCA). Transcatheter arterial chemoembolization plus subsequent radiofrequency ablation was performed for the 3 residual lesions in S4 and S8. Although testosterone enanthate was being continued for GID, no recurrence was observed until at least 22 months after the intensive treatments. HCA development in such patients receiving testosterone should be closely monitored using image inspection.

  1. Mitochondrial dysfunction in human skeletal muscle biopsies of lipid storage disorder.

    Science.gov (United States)

    Debashree, Bandopadhyay; Kumar, Manish; Keshava Prasad, Thottethodi Subrahmanya; Natarajan, Archana; Christopher, Rita; Nalini, Atchayaram; Bindu, Parayil Sankaran; Gayathri, Narayanappa; Srinivas Bharath, Muchukunte Mukunda

    2018-02-09

    Mitochondria regulate the balance between lipid metabolism and storage in the skeletal muscle. Altered lipid transport, metabolism and storage influence the bioenergetics, redox status and insulin signalling, contributing to cardiac and neurological diseases. Lipid storage disorders (LSDs) are neurological disorders which entail intramuscular lipid accumulation and impaired mitochondrial bioenergetics in the skeletal muscle causing progressive myopathy with muscle weakness. However, the mitochondrial changes including molecular events associated with impaired lipid storage have not been completely understood in the human skeletal muscle. We carried out morphological and biochemical analysis of mitochondrial function in muscle biopsies of human subjects with LSDs (n = 7), compared to controls (n = 10). Routine histology, enzyme histochemistry and ultrastructural analysis indicated altered muscle cell morphology and mitochondrial structure. Protein profiling of the muscle mitochondria from LSD samples (n = 5) (vs. control, n = 5) by high-throughput mass spectrometric analysis revealed that impaired metabolic processes could contribute to mitochondrial dysfunction and ensuing myopathy in LSDs. We propose that impaired fatty acid and respiratory metabolism along with increased membrane permeability, elevated lipolysis and altered cristae entail mitochondrial dysfunction in LSDs. Some of these mechanisms were unique to LSD apart from others that were common to dystrophic and inflammatory muscle pathologies. Many differentially regulated mitochondrial proteins in LSD are linked with other human diseases, indicating that mitochondrial protection via targeted drugs could be a treatment modality in LSD and related metabolic diseases. © 2018 International Society for Neurochemistry.

  2. Lack of cortisol response in patients with posttraumatic stress disorder (PTSD undergoing a diagnostic interview

    Directory of Open Access Journals (Sweden)

    de Quervain Dominique JF

    2007-10-01

    Full Text Available Abstract Background According to DSM-IV, the diagnosis of posttraumatic stress disorder (PTSD requires the experience of a traumatic event during which the person's response involved intense fear, helplessness, or horror. In order to diagnose PTSD, clinicians must interview the person in depth about his/her previous experiences and determine whether the individual has been traumatized by a specific event or events. However, asking questions about traumatic experiences can be stressful for the traumatized individual and it has been cautioned that subsequent "re-traumatization" could occur. This study investigated the cortisol response in traumatized refugees with PTSD during a detailed and standardized interview about their personal war and torture experiences. Methods Participants were male refugees with severe PTSD who solicited an expert opinion in the Psychological Research Clinic for Refugees of the University of Konstanz. 17 patients were administered the Vivo Checklist of War, Detention, and Torture Events, a standardized interview about traumatic experiences, and 16 subjects were interviewed about absorption behavior. Self-reported measures of affect and arousal, as well as saliva cortisol were collected at four points. Before and after the experimental intervention, subjects performed a Delayed Matching-to-Sample (DMS task for distraction. They also rated the severity of selected PTSD symptoms, as well as the level of intrusiveness of traumatic memories at that time. Results Cortisol excretion diminished in the course of the interview and showed the same pattern for both groups. No specific response was detectable after the supposed stressor. Correspondingly, ratings of subjective well-being, memories of the most traumatic event(s and PTSD symptoms did not show any significant difference between groups. Those in the presumed stress condition did not perform worse than persons in the control condition after the stressor. However, both

  3. Rapid screening for lipid storage disorders using biochemical markers Expert center data and review of the literature

    NARCIS (Netherlands)

    Voorink-Moret, M.; Goorden, S. M. I.; van Kuilenburg, A. B. P.; Wijburg, F. A.; Ghauharali-van der Vlugt, J. M. M.; Beers-Stet, F. S.; Zoetekouw, A.; Kulik, W.; Hollak, C. E. M.; Vaz, F. M.

    2018-01-01

    Background: In patients suspected of a lipid storage disorder (sphingolipidoses, lipidoses), confirmation of the diagnosis relies predominantly on the measurement of specific enzymatic activities and genetic studies. New UPLC-MS/MS methods have been developed to measure lysosphingolipids and

  4. A lipid storage-like disorder contributes to cognitive decline in HIV-infected subjects.

    Science.gov (United States)

    Bandaru, Veera Venkata Ratnam; Mielke, Michelle M; Sacktor, Ned; McArthur, Justin C; Grant, Igor; Letendre, Scott; Chang, Linda; Wojna, Valerie; Pardo, Carlos; Calabresi, Peter; Munsaka, Sody; Haughey, Norman J

    2013-10-22

    In this multicenter cohort study, we sought to identify prognostic and associative metabolic indicators for HIV-associated neurocognitive disorders (HAND). A quantitative lipidomic analysis was conducted on 524 longitudinal CSF samples collected from 7 different performance sites across the mainland United States, Hawaii, and Puerto Rico. Subjects included HIV-infected individuals with longitudinal clinical and cognitive testing data and cognitively normal HIV-negative healthy controls. At baseline, HIV+ subjects could be differentiated from HIV- controls by reductions in a single ceramide species and increases in multiple forms of cholesterol. Perturbations in cholesterol metabolism and ceramide were influenced by combined antiretroviral therapy (cART) use. There were no cross-sectional baseline differences in any lipid metabolite when HIV+ subjects were grouped according to cognitive status. However, a single sphingolipid metabolite and reduced levels of esterified cholesterols were prognostic indicators of incident cognitive decline. Longitudinal patterns of these disturbances in sphingolipid and sterol metabolism suggest that a progressive disorder of lipid metabolism that is similar to disorders of lipid storage may contribute to the pathogenesis of HAND. These findings suggest that HIV infection and cART are independently associated with a CNS metabolic disturbance, identify surrogate markers that are prognostic for cognitive decline, and implicate a lipid storage-like disorder in the progression of HAND.

  5. Predictors of premenstrual impairment among women undergoing prospective assessment for premenstrual dysphoric disorder: a cycle-level analysis.

    Science.gov (United States)

    Schmalenberger, K M; Eisenlohr-Moul, T A; Surana, P; Rubinow, D R; Girdler, S S

    2017-07-01

    Women who experience significant premenstrual symptoms differ in the extent to which these symptoms cause cyclical impairment. This study clarifies the type and number of symptoms that best predict premenstrual impairment in a sample of women undergoing prospective assessment for premenstrual dysphoric disorder (PMDD) in a research setting. Central research goals were to determine (1) which emotional, psychological, and physical symptoms of PMDD are uniquely associated with premenstrual impairment, and (2) how many cyclical symptoms optimally predict the presence of a clinically significant premenstrual elevation of impairment. A total of 267 naturally cycling women recruited for retrospective report of premenstrual emotional symptoms completed daily symptom reports using the Daily Record of Severity of Problems (DRSP) and occupational, recreational, and relational impairment for 1-4 menstrual cycles (N = 563 cycles). Multilevel regression revealed that emotional, psychological, and physical symptoms differ in their associations with impairment. The core emotional symptoms of PMDD were predictors of impairment, but not after accounting for secondary psychological symptoms, which were the most robust predictors. The optimal number of premenstrual symptoms for predicting clinically significant premenstrual impairment was four. Results enhance our understanding of the type and number of premenstrual symptoms associated with premenstrual impairment among women being evaluated for PMDD in research contexts. Additional work is needed to determine whether cognitive symptoms should receive greater attention in the study of PMDD, and to revisit the usefulness of the five-symptom diagnostic threshold.

  6. Biomarkers in the diagnosis of lysosomal storage disorders: proteins, lipids, and inhibodies.

    Science.gov (United States)

    Aerts, Johannes M F G; Kallemeijn, Wouter W; Wegdam, Wouter; Joao Ferraz, Maria; van Breemen, Marielle J; Dekker, Nick; Kramer, Gertjan; Poorthuis, Ben J; Groener, Johanna E M; Cox-Brinkman, Josanne; Rombach, Saskia M; Hollak, Carla E M; Linthorst, Gabor E; Witte, Martin D; Gold, Henrik; van der Marel, Gijs A; Overkleeft, Herman S; Boot, Rolf G

    2011-06-01

    A biomarker is an analyte indicating the presence of a biological process linked to the clinical manifestations and outcome of a particular disease. In the case of lysosomal storage disorders (LSDs), primary and secondary accumulating metabolites or proteins specifically secreted by storage cells are good candidates for biomarkers. Clinical applications of biomarkers are found in improved diagnosis, monitoring disease progression, and assessing therapeutic correction. These are illustrated by reviewing the discovery and use of biomarkers for Gaucher disease and Fabry disease. In addition, recently developed chemical tools allowing specific visualization of enzymatically active lysosomal glucocerebrosidase are described. Such probes, coined inhibodies, offer entirely new possibilities for more sophisticated molecular diagnosis, enzyme replacement therapy monitoring, and fundamental research.

  7. Alterations in membrane trafficking and pathophysiological implications in lysosomal storage disorders.

    Science.gov (United States)

    Kuech, Eva-Maria; Brogden, Graham; Naim, Hassan Y

    2016-11-01

    Lysosomal storage disorders are a heterogeneous group of more than 50 distinct inborn metabolic diseases affecting about 1 in 5000 to 7000 live births. The diseases often result from mutations followed by functional deficiencies of enzymes or transporters within the acidic environment of the lysosome, which mediate the degradation of a wide subset of substrates, including glycosphingolipids, glycosaminoglycans, cholesterol, glycogen, oligosaccharides, peptides and glycoproteins, or the export of the respective degradation products from the lysosomes. The progressive accumulation of uncleaved substrates occurs in multiple organs and finally causes a broad spectrum of different pathologies including visceral, neurological, skeletal and hematologic manifestations. Besides deficient lysosomal enzymes and transporters other defects may lead to lysosomal storage disorders, including activator defects, membrane defects or defects in modifier proteins. In this review we concentrate on four different lysosomal storage disorders: Niemann-Pick type C, Fabry disease, Gaucher disease and Pompe disease. While the last three are caused by defective lysosomal hydrolases, Niemann-Pick type C is caused by the inability to export LDL-derived cholesterol out of the lysosome. We want to emphasise potential implications of membrane trafficking defects on the pathology of these diseases, as many mutations interfere with correct lysosomal protein trafficking and alter cellular lipid homeostasis. Current therapeutic strategies are summarised, including substrate reduction therapy as well as pharmacological chaperone therapy which directly aim to improve folding and lysosomal transport of misfolded mutant proteins. Copyright © 2016 Elsevier B.V. and Société Française de Biochimie et Biologie Moléculaire (SFBBM). All rights reserved.

  8. Impaired cholesterol esterification in primary brain cultures of the lysosomal cholesterol storage disorder (LCSD) mouse mutant

    International Nuclear Information System (INIS)

    Patel, S.C.; Suresh, S.; Weintroub, H.; Brady, R.O.; Pentchev, P.G.

    1987-01-01

    Esterification of cholesterol was investigated in primary neuroglial cultures obtained from newborn lysosomal cholesterol storage disorder (LCSD) mouse mutants. An impairment in 3 H-oleic acid incorporation into cholesteryl esters was demonstrated in cultures of homozygous LCSD brain. Primary cultures derived from other phenotypically normal pups of the carrier breeders esterified cholesterol at normal levels or at levels which were intermediary between normal and deficient indicating a phenotypic expression of the LCSD heterozygote genotype. These observations on LCSD mutant brain cells indicate that the defect in cholesterol esterification is closely related to the primary genetic defect and is expressed in neuroglial cells in culture

  9. Neuronopathic lysosomal storage disorders: Approaches to treat the central nervous system.

    Science.gov (United States)

    Scarpa, Maurizio; Bellettato, Cinzia Maria; Lampe, Christina; Begley, David J

    2015-03-01

    Pharmacological research has always focused on developing new therapeutic strategies capable of modifying a disease's natural history and improving patients' quality of life. Despite recent advances within the fields of medicine and biology, some diseases still represent a major challenge for successful therapy. Neuronopathic lysosomal storage disorders, in particular, have high rates of morbidity and mortality and a devastating socio-economic effect. Many of the available therapies, such as enzyme replacement therapy, can reverse the natural history of the disease in peripheral organs but, unfortunately, are still unable to reach the central nervous system effectively because they cannot cross the blood-brain barrier that surrounds and protects the brain. Moreover, many lysosomal storage disorders are characterized by a number of blood-brain barrier dysfunctions, which may further contribute to disease neuropathology and accelerate neuronal cell death. These issues, and their context in the development of new therapeutic strategies, will be discussed in detail in this chapter. Copyright © 2014 Elsevier Ltd. All rights reserved.

  10. Wolman's disease and cholesteryl ester storage disorder: the phenotypic spectrum of lysosomal acid lipase deficiency.

    Science.gov (United States)

    Pericleous, Marinos; Kelly, Claire; Wang, Tim; Livingstone, Callum; Ala, Aftab

    2017-09-01

    Lysosomal acid lipase deficiency is a rare, autosomal recessive condition caused by mutations in the gene encoding lysosomal acid lipase (LIPA) that result in reduced or absent activity of this essential enzyme. The severity of the resulting disease depends on the nature of the underlying mutation and magnitude of its effect on enzymatic function. Wolman's disease is a severe disorder that presents during infancy, resulting in failure to thrive, hepatomegaly, and hepatic failure, and an average life expectancy of less than 4 months. Cholesteryl ester storage disorder arises later in life and is less severe, although the two diseases share many common features, including dyslipidaemia and transaminitis. The prevalence of these diseases has been estimated at one in 40 000 to 300 000, but many cases are undiagnosed and unreported, and awareness among clinicians is low. Lysosomal acid lipase deficiency-which can be diagnosed using dry blood spot testing-is often misdiagnosed as non-alcoholic fatty liver disease (NAFLD), non-alcoholic steatohepatitis (NASH), hereditary dyslipidaemia, or cryptogenic cirrhosis. There are no formal guidelines for treatment of these patients, and treatment options are limited. In this Review we appraise the existing literature on Wolman's disease and cholesteryl ester storage disease, and discuss available treatments, including enzyme replacement therapy, oral lipid-lowering therapy, stem-cell transplantation, and liver transplantation. Copyright © 2017 Elsevier Ltd. All rights reserved.

  11. The Role of Next-Generation Sequencing in the Diagnosis of Lysosomal Storage Disorders

    Directory of Open Access Journals (Sweden)

    Katalin Komlosi MD, PhD

    2016-10-01

    Full Text Available Next-generation sequencing (NGS panels are used widely in clinical diagnostics to identify genetic causes of various monogenic disease groups including neurometabolic disorders and, more recently, lysosomal storage disorders (LSDs. Many new challenges have been introduced through these new technologies, both at the laboratory level and at the bioinformatics level, with consequences including new requirements for interpretation of results, and for genetic counseling. We review some recent examples of the application of NGS technologies, with purely diagnostic and with both diagnostic and research aims, for establishing a rapid genetic diagnosis in LSDs. Given that NGS can be applied in a way that takes into account the many issues raised by international consensus guidelines, it can have a significant role even early in the course of the diagnostic process, in combination with biochemical and clinical data. Besides decreasing the delay in diagnosis for many patients, a precise molecular diagnosis is extremely important as new therapies are becoming available within the LSD spectrum for patients who share specific types of mutations. A genetic diagnosis is also the prerequisite for genetic counseling, family planning, and the individual choice of reproductive options in affected families.

  12. PEG-lipid micelles enable cholesterol efflux in Niemann-Pick Type C1 disease-based lysosomal storage disorder

    OpenAIRE

    Brown, Anna; Patel, Siddharth; Ward, Carl; Lorenz, Anna; Ortiz, Mauren; DuRoss, Allison; Wieghardt, Fabian; Esch, Amanda; Otten, Elsje G.; Heiser, Laura M.; Korolchuk, Viktor I.; Sun, Conroy; Sarkar, Sovan; Sahay, Gaurav

    2016-01-01

    2-Hydroxy-propyl-?-cyclodextrin (HP?CD), a cholesterol scavenger, is currently undergoing Phase 2b/3 clinical trial for treatment of Niemann Pick Type C-1 (NPC1), a fatal neurodegenerative disorder that stems from abnormal cholesterol accumulation in the endo/lysosomes. Unfortunately, the extremely high doses of HP?CD required to prevent progressive neurodegeneration exacerbates ototoxicity, pulmonary toxicity and autophagy-based cellular defects. We present unexpected evidence that a poly (e...

  13. Rapid screening for lipid storage disorders using biochemical markers. Expert center data and review of the literature.

    Science.gov (United States)

    Voorink-Moret, M; Goorden, S M I; van Kuilenburg, A B P; Wijburg, F A; Ghauharali-van der Vlugt, J M M; Beers-Stet, F S; Zoetekouw, A; Kulik, W; Hollak, C E M; Vaz, F M

    2018-02-01

    In patients suspected of a lipid storage disorder (sphingolipidoses, lipidoses), confirmation of the diagnosis relies predominantly on the measurement of specific enzymatic activities and genetic studies. New UPLC-MS/MS methods have been developed to measure lysosphingolipids and oxysterols, which, combined with chitotriosidase activity may represent a rapid first tier screening for lipid storage disorders. A lysosphingolipid panel consisting of lysoglobotriaosylceramide (LysoGb3), lysohexosylceramide (LysoHexCer: both lysoglucosylceramide and lysogalactosylceramide), lysosphingomyelin (LysoSM) and its carboxylated analogue lysosphingomyelin-509 (LysoSM-509) was measured in control subjects and plasma samples of predominantly untreated patients affected with lipid storage disorders (n=74). In addition, the oxysterols cholestane-3β,5α,6β-triol and 7-ketocholesterol were measured in a subset of these patients (n=36) as well as chitotriosidase activity (n=43). A systematic review of the literature was performed to assess the usefulness of these biochemical markers. Specific elevations of metabolites, i.e. without overlap between controls and other lipid storage disorders, were found for several lysosomal storage diseases: increased LysoSM levels in acid sphingomyelinase deficiency (Niemann-Pick disease type A/B), LysoGb3 levels in males with classical phenotype Fabry disease and LysoHexCer (i.e. lysoglucosylceramide/lysogalactosylceramide) in Gaucher and Krabbe diseases. While elevated levels of LysoSM-509 and cholestane-3β,5α,6β-triol did not discriminate between Niemann Pick disease type C and acid sphingomyelinase deficiency, LysoSM-509/LysoSM ratio was specifically elevated in Niemann-Pick disease type C. In Gaucher disease type I, mild increases in several lysosphingolipids were found including LysoGb3 with levels in the range of non-classical Fabry males and females. Chitotriosidase showed specific elevations in symptomatic Gaucher disease, and was mildly

  14. Impaired Autophagy in the Lipid-Storage Disorder Niemann-Pick Type C1 Disease

    Directory of Open Access Journals (Sweden)

    Sovan Sarkar

    2013-12-01

    Full Text Available Autophagy dysfunction has been implicated in misfolded protein accumulation and cellular toxicity in several diseases. Whether alterations in autophagy also contribute to the pathology of lipid-storage disorders is not clear. Here, we show defective autophagy in Niemann-Pick type C1 (NPC1 disease associated with cholesterol accumulation, where the maturation of autophagosomes is impaired because of defective amphisome formation caused by failure in SNARE machinery, whereas the lysosomal proteolytic function remains unaffected. Expression of functional NPC1 protein rescues this defect. Inhibition of autophagy also causes cholesterol accumulation. Compromised autophagy was seen in disease-affected organs of Npc1 mutant mice. Of potential therapeutic relevance is that HP-β-cyclodextrin, which is used for cholesterol-depletion treatment, impedes autophagy, whereas stimulating autophagy restores its function independent of amphisome formation. Our data suggest that a low dose of HP-β-cyclodextrin that does not perturb autophagy, coupled with an autophagy inducer, may provide a rational treatment strategy for NPC1 disease.

  15. Impaired autophagy in the lipid-storage disorder Niemann-Pick type C1 disease.

    Science.gov (United States)

    Sarkar, Sovan; Carroll, Bernadette; Buganim, Yosef; Maetzel, Dorothea; Ng, Alex H M; Cassady, John P; Cohen, Malkiel A; Chakraborty, Souvik; Wang, Haoyi; Spooner, Eric; Ploegh, Hidde; Gsponer, Joerg; Korolchuk, Viktor I; Jaenisch, Rudolf

    2013-12-12

    Autophagy dysfunction has been implicated in misfolded protein accumulation and cellular toxicity in several diseases. Whether alterations in autophagy also contribute to the pathology of lipid-storage disorders is not clear. Here, we show defective autophagy in Niemann-Pick type C1 (NPC1) disease associated with cholesterol accumulation, where the maturation of autophagosomes is impaired because of defective amphisome formation caused by failure in SNARE machinery, whereas the lysosomal proteolytic function remains unaffected. Expression of functional NPC1 protein rescues this defect. Inhibition of autophagy also causes cholesterol accumulation. Compromised autophagy was seen in disease-affected organs of Npc1 mutant mice. Of potential therapeutic relevance is that HP-β-cyclodextrin, which is used for cholesterol-depletion treatment, impedes autophagy, whereas stimulating autophagy restores its function independent of amphisome formation. Our data suggest that a low dose of HP-β-cyclodextrin that does not perturb autophagy, coupled with an autophagy inducer, may provide a rational treatment strategy for NPC1 disease. Copyright © 2013 The Authors. Published by Elsevier Inc. All rights reserved.

  16. Molecular diagnosis of glycogen storage disease and disorders with overlapping clinical symptoms by massive parallel sequencing.

    Science.gov (United States)

    Vega, Ana I; Medrano, Celia; Navarrete, Rosa; Desviat, Lourdes R; Merinero, Begoña; Rodríguez-Pombo, Pilar; Vitoria, Isidro; Ugarte, Magdalena; Pérez-Cerdá, Celia; Pérez, Belen

    2016-10-01

    Glycogen storage disease (GSD) is an umbrella term for a group of genetic disorders that involve the abnormal metabolism of glycogen; to date, 23 types of GSD have been identified. The nonspecific clinical presentation of GSD and the lack of specific biomarkers mean that Sanger sequencing is now widely relied on for making a diagnosis. However, this gene-by-gene sequencing technique is both laborious and costly, which is a consequence of the number of genes to be sequenced and the large size of some genes. This work reports the use of massive parallel sequencing to diagnose patients at our laboratory in Spain using either a customized gene panel (targeted exome sequencing) or the Illumina Clinical-Exome TruSight One Gene Panel (clinical exome sequencing (CES)). Sequence variants were matched against biochemical and clinical hallmarks. Pathogenic mutations were detected in 23 patients. Twenty-two mutations were recognized (mostly loss-of-function mutations), including 11 that were novel in GSD-associated genes. In addition, CES detected five patients with mutations in ALDOB, LIPA, NKX2-5, CPT2, or ANO5. Although these genes are not involved in GSD, they are associated with overlapping phenotypic characteristics such as hepatic, muscular, and cardiac dysfunction. These results show that next-generation sequencing, in combination with the detection of biochemical and clinical hallmarks, provides an accurate, high-throughput means of making genetic diagnoses of GSD and related diseases.Genet Med 18 10, 1037-1043.

  17. Severe steatohepatitis in a patient with a rare neutral lipid storage disorder due to ABHD5 mutation.

    Science.gov (United States)

    Ronchetti, Anna; Prati, Daniele; Pezzotta, Maria Grazia; Tavian, Daniela; Colombo, Roberto; Callea, Francesco; Colli, Agostino

    2008-09-01

    Fatty liver disease is mainly caused by alcohol consumption, excessive body weight, dyslipidemia and impaired glucose tolerance, but inherited disorders can sometimes be involved. We report the case of a 40-year-old woman with steatohepatitis and severe portal hypertension, associated with ichthyosis, cataract and hypoacusia. The clinical, pathological and genetic findings were consistent with a diagnosis of Chanarin-Dorfman syndrome (CDS), a rare autosomal recessive inherited neutral lipid storage disorder, and genetic analysis showed that a novel ABHD5 mutation is responsible.

  18. A non-conserved miRNA regulates lysosomal function and impacts on a human lysosomal storage disorder

    DEFF Research Database (Denmark)

    Frankel, Lisa B; Di Malta, Chiara; Wen, Jiayu

    2014-01-01

    Sulfatases are key enzymatic regulators of sulfate homeostasis with several biological functions including degradation of glycosaminoglycans (GAGs) and other macromolecules in lysosomes. In a severe lysosomal storage disorder, multiple sulfatase deficiency (MSD), global sulfatase activity...... of proteoglycan catabolism and lysosomal function. This blocks autophagy-mediated degradation, causing cytoplasmic accumulation of autophagosomes and autophagic substrates. By targeting miR-95 in cells from MSD patients, we can effectively increase residual SUMF1 expression, allowing for reactivation of sulfatase...

  19. 'Scarlett Spur Red Delicious' apple volatile production accompanying physiological disorder development during low pO2 controlled atmosphere storage.

    Science.gov (United States)

    Lumpkin, Christie; Fellman, John K; Rudell, David R; Mattheis, James

    2014-02-19

    Apple (Malus domestica Borkh.) fruit volatile production is regulated by a variety of factors including low oxygen storage conditions. This study examined the impact of low pO2 controlled atmospheres on 'Scarlett Spur Red Delicious' apple volatile production and disorder development. Accumulation of apple volatile compounds was characterized during long-term cold storage at 0.5 °C in air or low pO2 (0.3, 0.8, or 1.5 kPa) with 1 kPa CO2. Volatile accumulation differed quantitatively with pO2 as acetaldehyde, ethanol, and ethyl ester accumulation increased with decreased pO2 during the first weeks in storage. Differences in volatile accumulation among atmospheres were evident through 6 months. The rate of ethanol accumulation increased with decreased pO2 and could potentially be used to monitor low O2 stress. Incidence of low oxygen disorders after 9 months was highest in fruit held at the lowest pO2. The sesquiterpene α-farnesene was not detected throughout the storage period.

  20. Inborn Errors of Metabolism with Hypoglycemia Glycogen Storage Diseases and Inherited Disorders of Gluconeogenesis : Glycogen Storage Diseases and Inherited Disorders of Gluconeogenesis

    NARCIS (Netherlands)

    Weinstein, David A.; Steuerwald, Ulrike; De Souza, Carolina F. M.; Derks, Terry G. J.

    Although hyperinsulinism is the predominant inherited cause of hypoglycemia in the newborn period, inborn errors of metabolism are the primary etiologies after 1 month of age. Disorders of carbohydrate metabolism often present with hypoglycemia when fasting occurs. The presentation, diagnosis, and

  1. Continuous solid-state phase transitions in energy storage materials with orientational disorder – Computational and experimental approach

    International Nuclear Information System (INIS)

    Singh, Harpreet; Talekar, Anjali; Chien, Wen-Ming; Shi, Renhai; Chandra, Dhanesh; Mishra, Amrita; Tirumala, Muralidhar; Nelson, Daryl J.

    2015-01-01

    We report on TES (thermal energy storage) in new CT (continuous phase transitions) in multicomponent tetrahederally configured (orientationally disordered) crystals of NPG-neopentylglycol-C 5 H 12 O 2 , PG-pentaglycerine-C 5 H 12 O 3 , and PE-pentaerythritol-C 5 H 12 O 4 . This discovery is applicable in thermal energy storage in many systems which do not require conventional isothermal first-order phase transition energy storage. The above compounds exhibit polymorphs of orientationally disordered phases in which O–H…O bond rotation around the C–C bond stores significant amount of energy; for example, in PE 41.26 kJ/mol are absorbed isothermally during solid–solid transitions. In this paper we show, anisothermal continuous phase transitions (CT), due to compositional changes with changes in temperature, associated with a measurable amount of energy, not reported earlier. The correlation of phase stability regions in pseudo-binaries, calculated from ternary NPG–PG–PE phase diagrams, is validated by experimental ternary DSC (differential scanning calorimetry) and in-situ x-ray diffraction data. We established equations for determining the CT in a temperature range, and their respective enthalpies of transitions for any composition of the ternaries. Thermodynamic calculations of the Gibbs energies of the solution phases are modeled as substitutional solid solutions, in which the excess Gibbs energies are expressed by the Redlich–Kister–Muggianu polynomial. There is excellent agreement between the experimental and CALPHAD calculated data. - Highlights: • Continuous phase transition (CT) thermal energy storage in organic ternary system. • Anisothermal temperature ramping leads to CT transitions as per lever rule. • Orientationally disordered phases store energy in O–H…O bond rotation/oscillation. • Validated calculated data with measured thermodynamic properties in ternary system. • Used CALPHAD methodology to calculate Gibbs energies of

  2. Pharmacotherapy in the Management of Voiding and Storage Disorders, Including Enuresis and Encopresis

    Science.gov (United States)

    Reiner, William G.

    2008-01-01

    Enuresis and encopresis are disorders of the bladder and rectum, and this article helps in understanding the neurobiology of lower urinary tract and anorectal function to help in the treatment of these disorders. Treatment for children with these disorders emphasizes either a psychological or pharmacological approach.

  3. Pulmonary functions and sleep-related breathing disorders in lipid storage disease.

    Science.gov (United States)

    Bingöl, Züleyha; Tekce, Hacer Durmuş; Sağcan, Gülseren; Serdaroğlu, Piraye; Kıyan, Esen

    2018-03-01

    Pulmonary function abnormalities and sleep-related breathing disorders (SRBD) are frequent in subjects with several neuromuscular diseases but there is no data about lipid storage diseases (LSD). Therefore, we aimed to evaluate pulmonary functions and SRBD in adults with LSD. Pulmonary functions (forced expiratory volume (FEV 1 ), forced vital capacity (FVC), supine FVC, upright-supine FVC% change, maximal inspiratory pressure (MIP), maximal expiratory pressure (MEP), peak cough flow (PCF)), arterial blood gases, and polysomnographic data of all subjects were evaluated. Twenty-five subjects with LSD were evaluated [17 males, 8 females; age 34.9 ± 15 years; BMI 26.5 ± 3.4 kg/m 2 ]. MIP was - 72.2 ± 32.7 cmH 2 O ( 45 mmHg). REM sleep had decreased in all subjects (10.2% ± 6.1). Obstructive sleep apnea (OSA) was found in 80% of the subjects (n = 20; 9 mild, 9 moderate, 2 severe). For subjects with OSA, apnea-hypopnea index (AHI) was 20.8 ± 15.9/h, oxygen desaturation index (ODI) was 11.9 ± 15.4/h, AHI REM was 30.6 ± 19.7/h, AHI NREM was 19.7 ± 16.6/h, ODI REM was 27.2 ± 26.1/h, and ODI NREM was 11.4 ± 15/h. Five subjects (20%) diagnosed as REM-related OSA. Nocturnal mean SpO 2 was 94.9% ± 1.7, lowest SpO 2 was 73.3% ± 13.9, and time spent with SpO 2 < 90% was 2.4% ± 7.2. In subjects with LSD, pulmonary function impairment, daytime hypercapnia and hypoxemia, and OSA, especially REM-related OSA, are frequent. Therefore, pulmonary functions and polysomnography should be performed routinely.

  4. The impact of failing to identify suspect effort in patients undergoing adult attention-deficit/hyperactivity disorder (ADHD) assessment.

    Science.gov (United States)

    Marshall, Paul S; Hoelzle, James B; Heyerdahl, Danielle; Nelson, Nathaniel W

    2016-10-01

    [Correction Notice: An Erratum for this article was reported in Vol 28(10) of Psychological Assessment (see record 2016-22725-001). In the article, the penultimate sentence of the abstract should read “These results suggest that a significant percentage of those making a suspect effort will be diagnosed with ADHD using the most commonly employed assessment methods: an interview alone (71%); an interview and ADHD behavior rating scales combined (65%); and an interview, behavior rating scales, and most continuous performance tests combined (62%).” All versions of this article have been corrected.] This retrospective study examines how many adult patients would plausibly receive a diagnosis of attention-deficit/hyperactivity disorder (ADHD) if performance and symptom validity measures were not administered during neuropsychological evaluations. Five hundred fifty-four patients were extracted from an archival clinical dataset. A total of 102 were diagnosed with ADHD based on cognitive testing, behavior rating scales, effort testing, and clinical interview; 115 were identified as putting forth suspect effort in accordance with the Slick, Sherman, and Iverson (1999) criteria. From a clinical decision-making perspective, suspect effort and ADHD groups were nearly indistinguishable on ADHD behavior, executive function, and functional impairment rating scales, as well as on cognitive testing and key clinical interview questions. These results suggest that a significant percentage of those making a suspect effort will be diagnosed with ADHD using the most commonly employed assessment methods: an interview alone (71%); an interview and ADHD behavior rating scales combined (65%); and an interview, behavior rating scales, and most continuous performance tests combined (62%) [corrected]. This research makes clear that it is essential to evaluate task engagement and possible symptom amplification during clinical evaluations. PsycINFO Database Record (c) 2016 APA, all rights

  5. A Comparative Study on the Alterations of Endocytic Pathways in Multiple Lysosomal Storage Disorders.

    Science.gov (United States)

    Rappaport, Jeff; Manthe, Rachel L; Solomon, Melani; Garnacho, Carmen; Muro, Silvia

    2016-02-01

    Many cellular activities and pharmaceutical interventions involve endocytosis and delivery to lysosomes for processing. Hence, lysosomal processing defects can cause cell and tissue damage, as in lysosomal storage diseases (LSDs) characterized by lysosomal accumulation of undegraded materials. This storage causes endocytic and trafficking alterations, which exacerbate disease and hinder treatment. However, there have been no systematic studies comparing different endocytic routes in LSDs. Here, we used genetic and pharmacological models of four LSDs (type A Niemann-Pick, type C Niemann-Pick, Fabry, and Gaucher diseases) and evaluated the pinocytic and receptor-mediated activity of the clathrin-, caveolae-, and macropinocytic routes. Bulk pinocytosis was diminished in all diseases, suggesting a generic endocytic alteration linked to lysosomal storage. Fluid-phase (dextran) and ligand (transferrin) uptake via the clathrin route were lower for all LSDs. Fluid-phase and ligand (cholera toxin B) uptake via the caveolar route were both affected but less acutely in Fabry or Gaucher diseases. Epidermal growth factor-induced macropinocytosis was altered in Niemann-Pick cells but not other LSDs. Intracellular trafficking of ligands was also distorted in LSD versus wild-type cells. The extent of these endocytic alterations paralleled the level of cholesterol storage in disease cell lines. Confirming this, pharmacological induction of cholesterol storage in wild-type cells disrupted endocytosis, and model therapeutics restored uptake in proportion to their efficacy in attenuating storage. This suggests a proportional and reversible relationship between endocytosis and lipid (cholesterol) storage. By analogy, the accumulation of biological material in other diseases, or foreign material from drugs or their carriers, may cause similar deficits, warranting further investigation.

  6. "The impact of failing to identify suspect effort in patients undergoing adult attention-deficit/hyperactivity disorder (ADHD) assessment": Correction to Marshall et al. (2016).

    Science.gov (United States)

    2016-10-01

    Reports an error in "The Impact of Failing to Identify Suspect Effort in Patients Undergoing Adult Attention-Deficit/Hyperactivity Disorder (ADHD) Assessment" by Paul S. Marshall, James B. Hoelzle, Danielle Heyerdahl and Nathaniel W. Nelson ( Psychological Assessment , Advanced Online Publication, Jan 11, 2016, np). In the article, the penultimate sentence of the abstract should read “These results suggest that a significant percentage of those making a suspect effort will be diagnosed with ADHD using the most commonly employed assessment methods: an interview alone (71%); an interview and ADHD behavior rating scales combined (65%); and an interview, behavior rating scales, and most continuous performance tests combined (62%).” All versions of this article have been corrected. (The following abstract of the original article appeared in record 2016-00618-001.) This retrospective study examines how many adult patients would plausibly receive a diagnosis of attention-deficit/hyperactivity disorder (ADHD) if performance and symptom validity measures were not administered during neuropsychological evaluations. Five hundred fifty-four patients were extracted from an archival clinical dataset. A total of 102 were diagnosed with ADHD based on cognitive testing, behavior rating scales, effort testing, and clinical interview; 115 were identified as putting forth suspect effort in accordance with the Slick, Sherman, and Iverson (1999) criteria. From a clinical decision-making perspective, suspect effort and ADHD groups were nearly indistinguishable on ADHD behavior, executive function, and functional impairment rating scales, as well as on cognitive testing and key clinical interview questions. These results suggest that a significant percentage of those making a suspect effort will be diagnosed with ADHD using the most commonly employed assessment methods: an interview alone (71%); an interview and ADHD behavior rating scales combined (65%); and an interview, behavior

  7. Psychosocial and socio-demographic factors associated with outcomes for patients undergoing rehabilitation for chronic whiplash associated disorders: a pilot study.

    Science.gov (United States)

    Baltov, Petko; Côte, Julie; Truchon, Manon; Feldman, Debbie Ehrmann

    2008-01-01

    Identify psychosocial and socio-demographic factors (measured prior to treatment) that were associated with post-treatment self-perceived pain and disability and two secondary outcomes: psychological distress, and return to work in patients undergoing multidisciplinary rehabilitation for chronic whiplash associated disorders (WAD). Interviews were conducted with 28 patients with chronic WAD at entry to and completion of an intensive rehabilitation program, and a telephone interview was carried out three months later. Participants completed pain and disability, and psychological distress questionnaires, at baseline and at both follow-ups. They also completed psychosocial questionnaires and provided socio-demographic information. The effect of each of the independent variables on the outcomes was first evaluated by simple regressions, and then subsequently by multiple regression analysis. Higher baseline pain and disability predicted higher pain and disability at both follow-ups (p factor that affected pain and disability post-rehabilitation. Psychosocial factors played a role in the prognosis of psychological distress and return to work.

  8. Restarting stalled autophagy a potential therapeutic approach for the lipid storage disorder, Niemann-Pick type C1 disease.

    Science.gov (United States)

    Sarkar, Sovan; Maetzel, Dorothea; Korolchuk, Viktor I; Jaenisch, Rudolf

    2014-06-01

    Autophagy is essential for cellular homeostasis and its dysfunction in human diseases has been implicated in the accumulation of misfolded protein and in cellular toxicity. We have recently shown impairment in autophagic flux in the lipid storage disorder, Niemann-Pick type C1 (NPC1) disease associated with abnormal cholesterol sequestration, where maturation of autophagosomes is impaired due to defective amphisome formation caused by failure in SNARE machinery. Abrogation of autophagy also causes cholesterol accumulation, suggesting that defective autophagic flux in NPC1 disease may act as a primary causative factor not only by imparting its deleterious effects, but also by increasing cholesterol load. However, cholesterol depletion treatment with HP-β-cyclodextrin impedes autophagy, whereas pharmacologically stimulating autophagy restores its function independent of amphisome formation. Of potential therapeutic relevance is that a low dose of HP-β-cyclodextrin that does not perturb autophagy, coupled with an autophagy inducer, may rescue both the cholesterol and autophagy defects in NPC1 disease.

  9. Disordering fantasies of coal and technology: Carbon capture and storage in Australia

    International Nuclear Information System (INIS)

    Marshall, Jonathan Paul

    2016-01-01

    One of the main ways that continued use of coal is justified, and compensated for, is through fantasies of technology. This paper explores the politics of 'Carbon Capture and Storage' (CCS) technologies in Australia. These technologies involve capturing CO 2 emissions, usually to store them 'safely' underground in a process called 'geo-sequestration'. In Australia the idea of 'clean coal' has been heavily promoted, and is a major part of CO 2 emissions reduction plans, despite the technological difficulties, the lack of large scale working prototypes, the lack of coal company investment in such research, and the current difficulties in detecting leaks. This paper investigates the ways that the politics of 'clean coal' have functioned as psycho-social defence mechanisms, to prolong coal usage, assuage political discomfort and anxiety, and increase the systemic disturbance produced by coal power. - Highlights: • Clean coal and geological sequestration is part of Australian climate policy. • Governments have offered much to carbon capture and storage (CCS) projects. • Coal, and coal power, industries have been relatively uninterested. • Progress with CCS is problematic and has not lived up to expectations. • CCS defends against tackling the connection between coal and climate.

  10. Lysosome lipid storage disorder in NCTR-BALB/c mice. II. Morphologic and cytochemical studies.

    OpenAIRE

    Shio, H.; Fowler, S.; Bhuvaneswaran, C.; Morris, M. D.

    1982-01-01

    Electron-microscopic and cytochemical studies were carried out on tissues of NCTR-BALB/c mice. These mice are affected with a neurovisceral genetic disorder involving excessive tissue accumulation of lipid. Distinctive polymorphic intracellular inclusions, bounded by a membrane and containing lamellated bodies, were found in many cells of liver, spleen, lung, kidney, intestine, lymph nodes, and brain. The inclusions transformed reticuloendothelial cells into massive foam cells. Acid phosphata...

  11. 'Doctor Google' ending the diagnostic odyssey in lysosomal storage disorders: parents using internet search engines as an efficient diagnostic strategy in rare diseases.

    Science.gov (United States)

    Bouwman, Machtelt G; Teunissen, Quirine G A; Wijburg, Frits A; Linthorst, Gabor E

    2010-08-01

    The expansion of the internet has resulted in widespread availability of medical information for both patients and physicians. People increasingly spend time on the internet searching for an explanation, diagnosis or treatment for their symptoms. Regarding rare diseases, the use of the internet may be an important tool in the diagnostic process. The authors present two cases in which concerned parents made a correct diagnosis of a lysosomal storage disorder in their child by searching the internet after a long doctor's delay. These cases illustrate the utility of publicly available internet search engines in diagnosing rare disorders and in addition illustrate the lengthy diagnostic odyssey which is common in these disorders.

  12. Neuronal and epithelial cell rescue resolves chronic systemic inflammation in the lipid storage disorder Niemann-Pick C.

    Science.gov (United States)

    Lopez, Manuel E; Klein, Andrés D; Hong, Jennifer; Dimbil, Ubah J; Scott, Matthew P

    2012-07-01

    Chronic systemic inflammation is thought to be a major contributor to metabolic and neurodegenerative diseases. Since inflammatory components are shared among different disorders, targeting inflammation is an attractive option for mitigating disease. To test the significance of inflammation in the lipid storage disorder (LSD) Niemann-Pick C (NPC), we deleted the macrophage inflammatory gene Mip1a/Ccl3 from NPC diseased mice. Deletion of Ccl3 had been reported to delay neuronal loss in Sandhoff LSD mice by inhibiting macrophage infiltration. For NPC mice, in contrast, deleting Ccl3 did not retard neurodegeneration and worsened the clinical outcome. Depletion of visceral tissue macrophages also did not alter central nervous system (CNS) pathology and instead increased liver injury, suggesting a limited macrophage infiltration response into the CNS and a beneficial role of macrophage activity in visceral tissue. Prevention of neuron loss or liver injury, even at late stages in the disease, was achieved through specific rescue of NPC disease in neurons or in liver epithelial cells, respectively. Local epithelial cell correction was also sufficient to reduce the macrophage-associated pathology in lung tissue. These results demonstrate that elevated inflammation and macrophage activity does not necessarily contribute to neurodegeneration and tissue injury, and LSD defects in immune cells may not preclude an appropriate inflammatory response. We conclude that inflammation remains secondary to neuronal and epithelial cell dysfunction and does not irreversibly contribute to the pathogenic cascade in NPC disease. Without further exploration of possible beneficial roles of inflammatory mediators, targeting inflammation may not be therapeutically effective at ameliorating disease severity.

  13. The impact of the immune system on the safety and efficiency of enzyme replacement therapy in lysosomal storage disorders.

    Science.gov (United States)

    Broomfield, A; Jones, S A; Hughes, S M; Bigger, B W

    2016-07-01

    In the light of clinical experience in infantile onset Pompe patients, the immunological impact on the tolerability and long-term efficacy of enzyme replacement therapy (ERT) for lysosomal storage disorders has come under renewed scrutiny. This article details the currently proposed immunological mechanisms involved in the development of anti-drug antibodies and the current therapies used in their treatment. Given the current understanding of the adaptive immune response, it focuses particularly on T cell dependent mechanisms and the paradigm of using lymphocytic negative selection as a predictor of antibody formation. This concept originally postulated in the 1970s, stipulated that the genotypically determined lack of production or production of a variant protein determines an individual's lymphocytic repertoire. This in turn is the key factor in determining the potential severity of an individual's immunological response to ERT. It also highlights the need for immunological assay standardization particularly those looking at describing the degree of functional impact, robust biochemical or clinical endpoints and detailed patient subgroup identification if the true evaluations of impact are to be realised.

  14. Biochemical Basis of CO2-Related Internal Browning Disorders in Pears (Pyrus communis L. cv. Rocha) during Long-Term Storage

    NARCIS (Netherlands)

    Deuchande, Teresa; Larrigaudière, Christian; Giné-bordonaba, Jordi; Pinto de Carvalho, Susana; Vasconcelos, Marta W.

    2016-01-01

    This study aimed at understanding the biochemical basis of internal browning disorders (IBDs) in ‘Rocha’ pear. For this purpose, the effects of storage under normal controlled atmosphere (CA) (3 kPa of O2 + 0.5 kPa of CO2) and IBD-inducing CA (1 kPa of O2 + 10 kPa of CO2) on the antioxidant and

  15. Lipid metabolic perturbation is an early-onset phenotype in adult spinster mutants: a Drosophila model for lysosomal storage disorders.

    Science.gov (United States)

    Hebbar, Sarita; Khandelwal, Avinash; Jayashree, R; Hindle, Samantha J; Chiang, Yin Ning; Yew, Joanne Y; Sweeney, Sean T; Schwudke, Dominik

    2017-12-15

    Intracellular accumulation of lipids and swollen dysfunctional lysosomes are linked to several neurodegenerative diseases, including lysosomal storage disorders (LSD). Detailed characterization of lipid metabolic changes in relation to the onset and progression of neurodegeneration is currently missing. We systematically analyzed lipid perturbations in spinster (spin) mutants, a Drosophila model of LSD-like neurodegeneration. Our results highlight an imbalance in brain ceramide and sphingosine in the early stages of neurodegeneration, preceding the accumulation of endomembranous structures, manifestation of altered behavior, and buildup of lipofuscin. Manipulating levels of ceramidase and altering these lipids in spin mutants allowed us to conclude that ceramide homeostasis is the driving force in disease progression and is integral to spin function in the adult nervous system. We identified 29 novel physical interaction partners of Spin and focused on the lipid carrier protein, Lipophorin (Lpp). A subset of Lpp and Spin colocalize in the brain and within organs specialized for lipid metabolism (fat bodies and oenocytes). Reduced Lpp protein was observed in spin mutant tissues. Finally, increased levels of lipid metabolites produced by oenocytes in spin mutants allude to a functional interaction between Spin and Lpp, underscoring the systemic nature of lipid perturbation in LSD. © 2017 Hebbar et al. This article is distributed by The American Society for Cell Biology under license from the author(s). Two months after publication it is available to the public under an Attribution–Noncommercial–Share Alike 3.0 Unported Creative Commons License (http://creativecommons.org/licenses/by-nc-sa/3.0).

  16. Gene-wise association of variants in four lysosomal storage disorder genes in neuropathologically confirmed Lewy body disease.

    Science.gov (United States)

    Clark, Lorraine N; Chan, Robin; Cheng, Rong; Liu, Xinmin; Park, Naeun; Parmalee, Nancy; Kisselev, Sergey; Cortes, Etty; Torres, Paola A; Pastores, Gregory M; Vonsattel, Jean P; Alcalay, Roy; Marder, Karen; Honig, Lawrence L; Fahn, Stanley; Mayeux, Richard; Shelanski, Michael; Di Paolo, Gilbert; Lee, Joseph H

    2015-01-01

    Variants in GBA are associated with Lewy Body (LB) pathology. We investigated whether variants in other lysosomal storage disorder (LSD) genes also contribute to disease pathogenesis. We performed a genetic analysis of four LSD genes including GBA, HEXA, SMPD1, and MCOLN1 in 231 brain autopsies. Brain autopsies included neuropathologically defined LBD without Alzheimer Disease (AD) changes (n = 59), AD without significant LB pathology (n = 71), Alzheimer disease and lewy body variant (ADLBV) (n = 68), and control brains without LB or AD neuropathology (n = 33). Sequencing of HEXA, SMPD1, MCOLN1 and GBA followed by 'gene wise' genetic association analysis was performed. To determine the functional effect, a biochemical analysis of GBA in a subset of brains was also performed. GCase activity was measured in a subset of brain samples (n = 64) that included LBD brains, with or without GBA mutations, and control brains. A lipidomic analysis was also performed in brain autopsies (n = 67) which included LBD (n = 34), ADLBV (n = 3), AD (n = 4), PD (n = 9) and control brains (n = 17), comparing GBA mutation carriers to non-carriers. In a 'gene-wise' analysis, variants in GBA, SMPD1 and MCOLN1 were significantly associated with LB pathology (p range: 0.03-4.14 x10(-5)). Overall, the mean levels of GCase activity were significantly lower in GBA mutation carriers compared to non-carriers (plipid classes, ceramides and sphingolipids, was observed in LBD brains carrying GBA mutations compared to controls (p range: p<0.05-p<0.01). Our study indicates that variants in GBA, SMPD1 and MCOLN1 are associated with LB pathology. Biochemical data comparing GBA mutation carrier to non-carriers support these findings, which have important implications for biomarker development and therapeutic strategies.

  17. Gene-wise association of variants in four lysosomal storage disorder genes in neuropathologically confirmed Lewy body disease.

    Directory of Open Access Journals (Sweden)

    Lorraine N Clark

    Full Text Available Variants in GBA are associated with Lewy Body (LB pathology. We investigated whether variants in other lysosomal storage disorder (LSD genes also contribute to disease pathogenesis.We performed a genetic analysis of four LSD genes including GBA, HEXA, SMPD1, and MCOLN1 in 231 brain autopsies. Brain autopsies included neuropathologically defined LBD without Alzheimer Disease (AD changes (n = 59, AD without significant LB pathology (n = 71, Alzheimer disease and lewy body variant (ADLBV (n = 68, and control brains without LB or AD neuropathology (n = 33. Sequencing of HEXA, SMPD1, MCOLN1 and GBA followed by 'gene wise' genetic association analysis was performed. To determine the functional effect, a biochemical analysis of GBA in a subset of brains was also performed. GCase activity was measured in a subset of brain samples (n = 64 that included LBD brains, with or without GBA mutations, and control brains. A lipidomic analysis was also performed in brain autopsies (n = 67 which included LBD (n = 34, ADLBV (n = 3, AD (n = 4, PD (n = 9 and control brains (n = 17, comparing GBA mutation carriers to non-carriers.In a 'gene-wise' analysis, variants in GBA, SMPD1 and MCOLN1 were significantly associated with LB pathology (p range: 0.03-4.14 x10(-5. Overall, the mean levels of GCase activity were significantly lower in GBA mutation carriers compared to non-carriers (p<0.001. A significant increase and accumulation of several species for the lipid classes, ceramides and sphingolipids, was observed in LBD brains carrying GBA mutations compared to controls (p range: p<0.05-p<0.01.Our study indicates that variants in GBA, SMPD1 and MCOLN1 are associated with LB pathology. Biochemical data comparing GBA mutation carrier to non-carriers support these findings, which have important implications for biomarker development and therapeutic strategies.

  18. Lysosomal storage disorders

    CERN Document Server

    Cabrera-Salazar, Mario A; Cabrera-Salazar, Mario

    2007-01-01

    This book describes the nature of the lysosomal dysfunction and diseases as well as potential future treatments and therapies. This is an invaluable resource for researchers in biochemical and molecular genetics, enzyme therapy, and gene transfer.

  19. Working memory deficits in adults with attention-deficit/hyperactivity disorder (ADHD): an examination of central executive and storage/rehearsal processes.

    Science.gov (United States)

    Alderson, R Matt; Hudec, Kristen L; Patros, Connor H G; Kasper, Lisa J

    2013-05-01

    The current study was the first to use a regression approach to examine the unique contributions of central executive (CE) and storage/rehearsal processes to working memory (WM) deficits in adults with ADHD. Thirty-seven adults (ADHD = 21, HC = 16) completed phonological (PH) and visuospatial (VS) working memory tasks. While both groups performed significantly better during the PH task relative to the VS task, adults with ADHD exhibited significant deficits across both working memory modalities. Further, the ADHD group recalled disproportionately fewer PH and VS stimuli as set-size demands increased. Overall, the CE and PH storage/rehearsal processes of adults with ADHD were both significantly impaired relative to those of the healthy control adults; however, the magnitude of the CE effect size was much smaller compared to previous studies of children with the disorder. Collectively, results provide support for a lifelong trajectory of WM deficits in ADHD. © 2013 American Psychological Association

  20. Sedation using 5% lidocaine patches, midazolam and propofol in a combative, obese adolescent with severe autistic disorder undergoing brain magnetic resonance imaging: a case report.

    Science.gov (United States)

    Seo, Kwon Hui; Jung, Hong Soo; Kang, Eu Gene; Kim, Change Jae; Rhee, Ho Young; Jeon, Yeon Soo

    2014-12-01

    We present a 17-year-old man who underwent brain magnetic resonance imaging and laboratory exams for uncontrolled seizure. Patients with an autistic disorder require deep sedation or, occasionally, general anesthesia even for radiologic exams or simple procedures. The anesthetic management of an obese, violent patient with a severe autistic disorder and mental retardation can be challenging to anesthesiologists and requires a more careful approach in selecting adequate anesthetics and doses. This case emphasizes the importance of having a detailed plan to ensure the smooth process of premedication, anesthetic induction, maintenance, emergence and safe discharge of incorporated patients in the event of unexpected situations. A 5% lidocaine patch to relieve the pain from the intramuscular injection and intravenous cannulation, intramuscular midazolam as premedication, and propofol for the maintenance of sedation can be a good sedation protocol in incorporated patients.

  1. Energy storage

    International Nuclear Information System (INIS)

    Anon.

    1992-01-01

    This chapter discusses the role that energy storage may have on the energy future of the US. The topics discussed in the chapter include historical aspects of energy storage, thermal energy storage including sensible heat storage, latent heat storage, thermochemical heat storage, and seasonal heat storage, electricity storage including batteries, pumped hydroelectric storage, compressed air energy storage, and superconducting magnetic energy storage, and production and combustion of hydrogen as an energy storage option

  2. Analysis of the influence of respiratory disorders observed in preoperative spirometry on the dynamics of early inflammatory response in patients undergoing isolated coronary artery bypass grafting.

    Science.gov (United States)

    Szylińska, Aleksandra; Listewnik, Mariusz J; Rotter, Iwona; Rył, Aleksandra; Biskupski, Andrzej; Brykczyński, Mirosław

    2017-01-01

    Preoperative spirometry provides measurable information about the occurrence of respiratory disorders. The aim of this study was to assess the association between preoperative spirometry abnormalities and the intensification of early inflammatory responses in patients following coronary artery bypass graft in extracorporeal circulation. The study involved 810 patients (625 men and 185 women) aged 65.4±7.9 years who were awaiting isolated coronary artery bypass surgery. On the basis of spirometry performed on the day of admittance to the hospital, the patients were divided into three groups. Patients without respiratory problems constituted 78.8% of the entire group. Restricted breathing was revealed by spirometry in 14.9% and obstructive breathing in 6.3% of patients. Inter-group analysis showed statistically significant differences in C-reactive protein (CRP) between patients with restrictive spirometry abnormalities and patients without any pulmonary dysfunction. CRP concentrations differed before surgery ( P =0.006) and on the second ( P spirometry results from restrictive respiratory disorders have an elevated level of generalized inflammatory response both before and after the isolated coronary artery bypass surgery. Therefore, this group of patients should be given special postoperative monitoring and, in particular, intensive respiratory rehabilitation immediately after reconstitution.

  3. Treatment compliance and effectiveness in complex PTSD patients with co-morbid personality disorder undergoing stabilizing cognitive behavioral group treatment: a preliminary study

    Directory of Open Access Journals (Sweden)

    Ethy Dorrepaal

    2013-11-01

    Full Text Available Background: In the empirical and clinical literature, complex posttraumatic stress disorder (PTSD and personality disorders (PDs are suggested to be predictive of drop-out or reduced treatment effectiveness in trauma-focused PTSD treatment. Objective: In this study, we aimed to investigate if personality characteristics would predict treatment compliance and effectiveness in stabilizing complex PTSD treatment. Method: In a randomized controlled trial on a 20-week stabilizing group cognitive behavioral treatment (CBT for child-abuse-related complex PTSD, we included 71 patients of whom 38 were randomized to a psycho-educational and cognitive behavioral stabilizing group treatment. We compared the patients with few PD symptoms (adaptive (N=14 with the non-adaptive patients (N=24 as revealed by a cluster analysis. Results: We found that non-adaptive patients compared to the adaptive patients showed very low drop-out rates. Both non-adaptive patients, classified with highly different personality profiles “withdrawn” and “aggressive,” were equally compliant. With regard to symptom reduction, we found no significant differences between subtypes. Post-hoc, patients with a PD showed lower drop-out rates and higher effect sizes in terms of complex PTSD severity, especially on domains that affect regulation and interpersonal problems. Conclusion: Contrary to our expectations, these preliminary findings indicate that this treatment is well tolerated by patients with a variety of personality pathology. Larger sample sizes are needed to study effectiveness for subgroups of complex PTSD patients.

  4. Myasthenia gravis in a patient affected by glycogen storage disease type Ib: a further manifestation of an increased risk for autoimmune disorders?

    Science.gov (United States)

    Melis, D; Balivo, F; Della Casa, R; Romano, A; Taurisano, R; Capaldo, B; Riccardi, G; Monsurrò, M R; Parenti, G; Andria, G

    2008-12-01

    Glycogen storage disease type Ib (GSD Ib, OMIM 232220) is an inborn disorder of glucose metabolism, caused by mutations in the G6PT gene, encoding a glucose 6-phosphate transporter (G6PT). GSD Ib is mainly associated with fasting hypoglycaemia and hepatomegaly. Most GSD Ib patients also show neutropenia and neutrophil dysfunction and therefore are at risk of developing severe infections and inflammatory bowel disease (IBD). An increased risk for autoimmune disorders, such as thyroid autoimmunity and Crohn-like disease, has also been demonstrated, but no systematic study on the prevalence of autoimmune disorders in GSD Ib patients has ever been performed. We describe a 25-year-old patient affected by GSD Ib who developed 'seronegative' myasthenia gravis (MG), presenting with bilateral eyelid ptosis, diplopia, dysarthria, severe dysphagia, dyspnoea and fatigue. The repetitive stimulation of peripheral nerves test showed signs of exhaustion of neuromuscular transmission, particularly evident in the cranial area. Even in the absence of identifiable anti-acetylcholine receptor antibodies, seronegative MG is considered an autoimmune disorder and may be related to the disturbed immune function observed in GSD Ib patients.

  5. CNS penetration of intrathecal-lumbar idursulfase in the monkey, dog and mouse: implications for neurological outcomes of lysosomal storage disorder.

    Directory of Open Access Journals (Sweden)

    Pericles Calias

    Full Text Available A major challenge for the treatment of many central nervous system (CNS disorders is the lack of convenient and effective methods for delivering biological agents to the brain. Mucopolysaccharidosis II (Hunter syndrome is a rare inherited lysosomal storage disorder resulting from a deficiency of iduronate-2-sulfatase (I2S. I2S is a large, highly glycosylated enzyme. Intravenous administration is not likely to be an effective therapy for disease-related neurological outcomes that require enzyme access to the brain cells, in particular neurons and oligodendrocytes. We demonstrate that intracerebroventricular and lumbar intrathecal administration of recombinant I2S in dogs and nonhuman primates resulted in widespread enzyme distribution in the brain parenchyma, including remarkable deposition in the lysosomes of both neurons and oligodendrocytes. Lumbar intrathecal administration also resulted in enzyme delivery to the spinal cord, whereas little enzyme was detected there after intraventricular administration. Mucopolysaccharidosis II model is available in mice. Lumbar administration of recombinant I2S to enzyme deficient animals reduced the storage of glycosaminoglycans in both superficial and deep brain tissues, with concurrent morphological improvements. The observed patterns of enzyme transport from cerebrospinal fluid to the CNS tissues and the resultant biological activity (a warrant further investigation of intrathecal delivery of I2S via lumbar catheter as an experimental treatment for the neurological symptoms of Hunter syndrome and (b may have broader implications for CNS treatment with biopharmaceuticals.

  6. Outcome predictors in autism spectrum disorders preschoolers undergoing treatment as usual: insights from an observational study using artificial neural networks

    Directory of Open Access Journals (Sweden)

    Narzisi A

    2015-06-01

    Full Text Available Antonio Narzisi,1 Filippo Muratori,1,2 Massimo Buscema,3,4 Sara Calderoni,1 Enzo Grossi3,5 1Department of Developmental Neuroscience, IRCCS Stella Maris Foundation, 2Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy; 3Semeion Research Centre of Sciences of Communication, Rome, Italy; 4Department of Mathematical and Statistical Sciences, University of Colorado Denver, Denver, CO, USA; 5Autism Research Unit, Villa Santa Maria Institute, Tavernerio, Italy Background: Treatment as usual (TAU for autism spectrum disorders (ASDs includes eclectic treatments usually available in the community and school inclusion with an individual support teacher. Artificial neural networks (ANNs have never been used to study the effects of treatment in ASDs. The Auto Contractive Map (Auto-CM is a kind of ANN able to discover trends and associations among variables creating a semantic connectivity map. The matrix of connections, visualized through a minimum spanning tree filter, takes into account nonlinear associations among variables and captures connection schemes among clusters. Our aim is to use Auto-CM to recognize variables to discriminate between responders versus no responders at TAU.Methods: A total of 56 preschoolers with ASDs were recruited at different sites in Italy. They were evaluated at T0 and after 6 months of treatment (T1. The children were referred to community providers for usual treatments. Results: At T1, the severity of autism measured through the Autism Diagnostic Observation Schedule decreased in 62% of involved children (Response, whereas it was the same or worse in 37% of the children (No Response. The application of the Semeion ANNs overcomes the 85% of global accuracy (Sine Net almost reaching 90%. Consequently, some of the tested algorithms were able to find a good correlation between some variables and TAU outcome. The semantic connectivity map obtained with the application of the Auto-CM system showed

  7. Patients undergoing long-term treatment with antihypertensive eye drops responded positively with respect to their ocular surface disorder to oral supplementation with antioxidants and essential fatty acids

    Directory of Open Access Journals (Sweden)

    Galbis-Estrada C

    2013-06-01

    Full Text Available Carmen Galbis-Estrada,1,* Maria D Pinazo-Durán,1,* Jorge Cantú-Dibildox,2 Carla Marco-Ramírez,1 Manuel Díaz-Llópis,1,3 Javier Benítez-del-Castillo21Ophthalmic Research Unit Santiago Grisolia, Department of Surgery/Ophthalmology, Faculty of Medicine, University of Valencia, Valencia, Spain; 2Department of Ophthalmology, Hospital of Jerez, Jerez de la Frontera, Cádiz, Spain; 3University and Polytechnic Hospital La Fe, Valencia, Spain*These authors contributed equally to this workBackground: Glaucoma and dry eye disorders (DEDs are frequent comorbidities. The antioxidant and anti-inflammatory properties of essential polyunsaturated fatty acids have been extensively studied in relation to eye diseases.Objective: Our objective was to determine the effects of oral supplementation with a combined formulation of antioxidants and essential polyunsaturated fatty acids on expression of cytokines and chemokines in tears from patients with DEDs or primary open-angle glaucoma (POAG.Methods: Participants (n = 97 were distributed into three groups: (1 individuals with nonsevere DEDs (DEDG, (2 individuals with nonadvanced POAG (POAGG, and (3 healthy controls. These groups were randomized into two subgroups: one received a daily antioxidant and essential polyunsaturated fatty acid supplement (two pills for 3 months (+S, and the other did not (−NS. Participants were interviewed and ophthalmologically examined. Concentrations of specific cytokines and chemokines in reflex tears were determined by multiplexed particle-based flow cytometry. The data were analyzed statistically (SPSS version 15.0.Results: Comparison of the results from the DEDG and POAGG patients showed significant differences in tear expression of granulocyte-macrophage colony-stimulating factor (P = 0.008, tumor necrosis factor α (P = 0.005, vascular endothelial growth factor (P = 0.038, interleukin-4 (P = 0.030, and interleukin-6 (P = 0.044. The main signs and symptoms of dry eyes such

  8. Long-Term Evaluation of Patients Undergoing Genitoplasty due to Disorders of Sex Development: Results from a 14-Year Follow-Up

    Directory of Open Access Journals (Sweden)

    Heng Zhang

    2013-01-01

    Full Text Available Purpose. To summarize the experience in treating patients with genitoplasty due to disorders of sex development in China. Methods. The operative procedures, gender of rearing, surgical outcome, and psychosocial and family adjustments of 262 patients were reviewed retrospectively. Results. At initial diagnosis, the mean age was years (range: 2–38 years. There were 96 children, 133 adolescents, and 33 adults. Follow-up was done every 6 months. Patients with female sex assignment had no urinary incontinence or voiding difficulty. Five patients underwent the second surgery (3%; vaginal dilation was performed in 35 patients with postoperative vaginal stenosis; 12 patients (7.4% were unsatisfactory with the outcome. For patients with male sex assignment, the median length of penis was 2.2 cm in prepubertal patients, 4.2 cm in pubertal patients, and 5.0 cm in adults; 39 patients developed postvoid dribbling (39%; 21 patients underwent a second surgery (21%; urethral dilation was done in 28 patients (28% due to urethral stricture; 38 patients were unsatisfactory with the outcome (38%. In addition, 136 patients (83% with female sex assignment and 54 (54% with male sex assignment had favorable psychosocial adjustment. Conclusions. Patients with male sex assignment have more surgical complications and difficulties in psychosocial adjustment as compared to those with female sex assignment.

  9. Patients undergoing long-term treatment with antihypertensive eye drops responded positively with respect to their ocular surface disorder to oral supplementation with antioxidants and essential fatty acids.

    Science.gov (United States)

    Galbis-Estrada, Carmen; Pinazo-Durán, Maria D; Cantú-Dibildox, Jorge; Marco-Ramírez, Carla; Díaz-Llópis, Manuel; Benítez-del-Castillo, Javier

    2013-01-01

    Glaucoma and dry eye disorders (DEDs) are frequent comorbidities. The antioxidant and anti-inflammatory properties of essential polyunsaturated fatty acids have been extensively studied in relation to eye diseases. Our objective was to determine the effects of oral supplementation with a combined formulation of antioxidants and essential polyunsaturated fatty acids on expression of cytokines and chemokines in tears from patients with DEDs or primary open-angle glaucoma (POAG). Participants (n = 97) were distributed into three groups: (1) individuals with nonsevere DEDs (DEDG), (2) individuals with nonadvanced POAG (POAGG), and (3) healthy controls. These groups were randomized into two subgroups: one received a daily antioxidant and essential polyunsaturated fatty acid supplement (two pills) for 3 months (+S), and the other did not (-NS). Participants were interviewed and ophthalmologically examined. Concentrations of specific cytokines and chemokines in reflex tears were determined by multiplexed particle-based flow cytometry. The data were analyzed statistically (SPSS version 15.0). Comparison of the results from the DEDG and POAGG patients showed significant differences in tear expression of granulocyte-macrophage colony-stimulating factor (P = 0.008), tumor necrosis factor α (P = 0.005), vascular endothelial growth factor (P = 0.038), interleukin-4 (P = 0.030), and interleukin-6 (P = 0.044). The main signs and symptoms of dry eyes such as dryness, burning, photophobia, eye heaviness, and blurred vision, as well as positive changes in eyelashes, hair, nails and skin, were significantly improved in DEDG +S and POAGG +S patients relative to unsupplemented patients. Inflammation biomarkers were differentially expressed in glaucomatous tears, but the differences changed upon antioxidant/essential polyunsaturated fatty acid supplementation. Chronic instillation of antihypertensive eye drops must be considered for integrating protocols to glaucoma standards of care.

  10. Perceived quality of life in partners of patients undergoing treatment in somatic health, mental health, or substance use disorder units: a cross-sectional study.

    Science.gov (United States)

    Birkeland, Bente; Weimand, Bente M; Ruud, Torleif; Høie, Magnhild M; Vederhus, John-Kåre

    2017-08-30

    This study explores (1) differences in socio-demographic, social/familial, and health variables and perceived quality of life (QoL) among partners of patients with somatic illness, mental illness, or substance use disorder (SUD); and (2) identifies factors associated with QoL. Participants (N = 213) in this cross-sectional study were recruited from inpatient or outpatient services in five hospitals in Norway, 2013-2014. QoL was measured by the QoL-5, a generic five-item questionnaire. Differences between groups were examined using Chi-square for categorical variables and Kruskal-Wallis for contiuous variables. Multiple linear regression analyses were used to examine factors associated with QoL. The mean QoL score was similar to that of a general population sample, and 13% of the sample had a markedly low QoL. Partners in the SUD group experienced worse socio-demographic conditions in terms of occupation and income, but QoL did not differ significantly among the three groups. In a regression model, perceived family cohesion was positively associated with QoL while psychological distress (Symptom Checklist-10) was negatively related to it. The model explained 56% of the variance in QoL. When patients are ill, clinicians should consider the partners' QoL, and brief QoL tools can be used to identify those who are struggling most. Reduced QoL is associated with higher psychological distress and lower family cohesion. Treatment initiatives focusing on these themes may serve as preventive measures to help the most vulnerable families cope with their difficult life situation.

  11. The quality of life of children and adolescents with ADHD undergoing outpatient psychiatric treatment: simple disorders of activity and attention and hyperkinetic conduct disorders in comparison with each other and with other diagnostic groups.

    Science.gov (United States)

    Remschmidt, Helmut; Mattejat, Fritz

    2010-12-01

    (1) How does the quality of life of patients with ADHD treated in an ambulatory care setting compare to that of other patient groups in child and adolescent psychiatry? (2) Can differences in the quality of life be demonstrated between patients with simple disorders of activity and attention and those with hyperkinetic conduct disorders? (3) How does the quality of life in these patient groups change over one year of treatment? The Inventory for the Assessment of Life Quality in Children and Adolescents (Inventar zur Untersuchung der Lebensqualität von Kindern und Jugendlichen, ILK) was applied to a sample of 726 patients derived from nine different outpatient practices for child and adolescent psychiatry. Among them were 196 patients with a simple disorder of activity and attention and 64 with a hyperkinetic conduct disorder. A comparison between these two groups was the main aim of the study. The mean age of the patients in the sample (all diagnoses) was 8.7 ± 3 years. The two groups of hyperkinetic patients made up 35% of the overall sample, and both of them showed a marked male predominance. The hyperkinetic patients tended to have lower quality-of-life scores than patients in the other diagnostic groups. Longitudinal observation revealed improvements in the quality of life across all patient groups, but the patients with hyperkinetic disorders (both groups) improved the least. The parents of the hyperkinetic patients, too, reported suffering greater stress because of their children's condition than the parents of children with other types of disorders. The ILK instrument has test-metrical qualities that render it usable and capable of holding its own among other, comparable instruments. It can be used to assess the quality of life of children with various diagnoses. Children with ADHD tend to have the least favorable quality-of-life scores, yet they do show some degree of improvement in their quality of life after a year of treatment.

  12. Energy Storage.

    Science.gov (United States)

    Eaton, William W.

    Described are technological considerations affecting storage of energy, particularly electrical energy. The background and present status of energy storage by batteries, water storage, compressed air storage, flywheels, magnetic storage, hydrogen storage, and thermal storage are discussed followed by a review of development trends. Included are…

  13. Cloud storage based mobile assessment facility for patients with post-traumatic stress disorder using integrated signal processing algorithm

    Science.gov (United States)

    Balbin, Jessie R.; Pinugu, Jasmine Nadja J.; Basco, Abigail Joy S.; Cabanada, Myla B.; Gonzales, Patrisha Melrose V.; Marasigan, Juan Carlos C.

    2017-06-01

    The research aims to build a tool in assessing patients for post-traumatic stress disorder or PTSD. The parameters used are heart rate, skin conductivity, and facial gestures. Facial gestures are recorded using OpenFace, an open-source face recognition program that uses facial action units in to track facial movements. Heart rate and skin conductivity is measured through sensors operated using Raspberry Pi. Results are stored in a database for easy and quick access. Databases to be used are uploaded to a cloud platform so that doctors have direct access to the data. This research aims to analyze these parameters and give accurate assessment of the patient.

  14. Amnestic Disorders

    NARCIS (Netherlands)

    Kessels, R.P.C.; Savage, G.; Cautin, R.L.; Lilienfeld, S.O.

    2015-01-01

    Amnestic disorders may involve deficits in the encoding or storage of information in memory, or in retrieval of information from memory. Etiologies vary and include traumatic brain injury, neurodegenerative disease, and psychiatric illness. Different forms of amnesia can be distinguished:

  15. Newborn screening for six lysosomal storage disorders in a cohort of Mexican patients: Three-year findings from a screening program in a closed Mexican health system.

    Science.gov (United States)

    Navarrete-Martínez, Juana Inés; Limón-Rojas, Ana Elena; Gaytán-García, Maria de Jesús; Reyna-Figueroa, Jesús; Wakida-Kusunoki, Guillermo; Delgado-Calvillo, Ma Del Rocío; Cantú-Reyna, Consuelo; Cruz-Camino, Héctor; Cervantes-Barragán, David Eduardo

    2017-05-01

    To evaluate the results of a lysosomal newborn screening (NBS) program in a cohort of 20,018 Mexican patients over the course of 3years in a closed Mexican Health System (Petróleos Mexicanos [PEMEX] Health Services). Using dried blood spots (DBS), we performed a multiplex tandem mass spectrometry enzymatic assay for six lysosomal storage disorders (LSDs) including Pompe disease, Fabry disease, Gaucher disease, mucopolysaccharidosis type I (MPS-I), Niemann-Pick type A/B, and Krabbe disease. Screen-positive cases were confirmed using leukocyte enzymatic activity and DNA molecular analysis. From July 2012 to April 2016, 20,018 patients were screened; 20 patients were confirmed to have an LSD phenotype (99.9 in 100,000 newborns). Final distributions include 11 Pompe disease, five Fabry disease, two MPS-I, and two Niemann-Pick type A/B patients. We did not find any Gaucher or Krabbe patients. A final frequency of 1 in 1001 LSD newborn phenotypes was established. NBS is a major public health achievement that has decreased the morbidity and mortality of inborn errors of metabolism. The introduction of NBS for LSD presents new challenges. This is the first multiplex Latin-American study of six LSDs detected through NBS. Copyright © 2017 Elsevier Inc. All rights reserved.

  16. Clinical utility of neuronal cells directly converted from fibroblasts of patients for neuropsychiatric disorders: studies of lysosomal storage diseases and channelopathy

    Science.gov (United States)

    Kano, Shin-ichi; Yuan, Ming; Cardarelli, Ross A.; Maegawa, Gustavo; Higurashi, Norimichi; Gaval-Cruz, Meriem; Wilson, Ashley M.; Tristan, Carlos; Kondo, Mari A.; Chen, Yian; Koga, Minori; Obie, Cassandra; Ishizuka, Koko; Seshadri, Saurav; Srivastava, Rupali; Kato, Takahiro A.; Horiuchi, Yasue; Sedlak, Thomas W.; Lee, Yohan; Rapoport, Judith L.; Hirose, Shinichi; Okano, Hideyuki; Valle, David; O'Donnell, Patricio; Sawa, Akira; Kai, Mihoko

    2015-01-01

    Methodologies for generating functional neuronal cells directly from human fibroblasts [induced neuronal (iN) cells] have been recently developed, but the research so far has only focused on technical refinements or recapitulation of known pathological phenotypes. A critical question is whether this novel technology will contribute to elucidation of novel disease mechanisms or evaluation of therapeutic strategies. Here we have addressed this question by studying Tay-Sachs disease, a representative lysosomal storage disease, and Dravet syndrome, a form of severe myoclonic epilepsy in infancy, using human iN cells with feature of immature postmitotic glutamatergic neuronal cells. In Tay-Sachs disease, we have successfully characterized canonical neuronal pathology, massive accumulation of GM2 ganglioside, and demonstrated the suitability of this novel cell culture for future drug screening. In Dravet syndrome, we have identified a novel functional phenotype that was not suggested by studies of classical mouse models and human autopsied brains. Taken together, the present study demonstrates that human iN cells are useful for translational neuroscience research to explore novel disease mechanisms and evaluate therapeutic compounds. In the future, research using human iN cells with well-characterized genomic landscape can be integrated into multidisciplinary patient-oriented research on neuropsychiatric disorders to address novel disease mechanisms and evaluate therapeutic strategies. PMID:25732146

  17. Tritium storage

    International Nuclear Information System (INIS)

    Hircq, B.

    1990-01-01

    This document represents a synthesis relative to tritium storage. After indicating the main storage particularities as regards tritium, storages under gaseous and solid form are after examined before establishing choices as a function of the main criteria. Finally, tritium storage is discussed regarding tritium devices associated to Fusion Reactors and regarding smaller devices [fr

  18. Impact of lysosomal storage disorders on biology of mesenchymal stem cells: Evidences from in vitro silencing of glucocerebrosidase (GBA) and alpha-galactosidase A (GLA) enzymes.

    Science.gov (United States)

    Squillaro, Tiziana; Antonucci, Ivana; Alessio, Nicola; Esposito, Anna; Cipollaro, Marilena; Melone, Mariarosa Anna Beatrice; Peluso, Gianfranco; Stuppia, Liborio; Galderisi, Umberto

    2017-12-01

    Lysosomal storage disorders (LDS) comprise a group of rare multisystemic diseases resulting from inherited gene mutations that impair lysosomal homeostasis. The most common LSDs, Gaucher disease (GD), and Fabry disease (FD) are caused by deficiencies in the lysosomal glucocerebrosidase (GBA) and alpha-galactosidase A (GLA) enzymes, respectively. Given the systemic nature of enzyme deficiency, we hypothesized that the stem cell compartment of GD and FD patients might be also affected. Among stem cells, mesenchymal stem cells (MSCs) are a commonly investigated population given their role in hematopoiesis and the homeostatic maintenance of many organs and tissues. Since the impairment of MSC functions could pose profound consequences on body physiology, we evaluated whether GBA and GLA silencing could affect the biology of MSCs isolated from bone marrow and amniotic fluid. Those cell populations were chosen given the former's key role in organ physiology and the latter's intriguing potential as an alternative stem cell model for human genetic disease. Our results revealed that GBA and GLA deficiencies prompted cell cycle arrest along with the impairment of autophagic flux and an increase of apoptotic and senescent cell percentages. Moreover, an increase in ataxia-telangiectasia-mutated staining 1 hr after oxidative stress induction and a return to basal level at 48 hr, along with persistent gamma-H2AX staining, indicated that MSCs properly activated DNA repair signaling, though some damages remained unrepaired. Our data therefore suggest that MSCs with reduced GBA or GLA activity are prone to apoptosis and senescence due to impaired autophagy and DNA repair capacity. © 2017 Wiley Periodicals, Inc.

  19. Energy storage

    Science.gov (United States)

    Kaier, U.

    1981-04-01

    Developments in the area of energy storage are characterized, with respect to theory and laboratory, by an emergence of novel concepts and technologies for storing electric energy and heat. However, there are no new commercial devices on the market. New storage batteries as basis for a wider introduction of electric cars, and latent heat storage devices, as an aid for solar technology applications, with satisfactory performance standards are not yet commercially available. Devices for the intermediate storage of electric energy for solar electric-energy systems, and for satisfying peak-load current demands in the case of public utility companies are considered. In spite of many promising novel developments, there is yet no practical alternative to the lead-acid storage battery. Attention is given to central heat storage for systems transporting heat energy, small-scale heat storage installations, and large-scale technical energy-storage systems.

  20. Hydrogen storage in planetary physics

    International Nuclear Information System (INIS)

    Baltensperger, W.

    1984-01-01

    Hydrogen in contact with most substances undergoes first order phase transitions with increasing pressure during which hydrides are formed. This applies to the core of hydrogen rich planets. It is speculated that a partial hydrogen storage in the early history of the earth could have lead to the formation of continents. Primordial carbon hydrides are synthesized during this process. (Author) [pt

  1. Energy storage

    International Nuclear Information System (INIS)

    2012-01-01

    After having outlined the importance of energy storage in the present context, this document outlines that it is an answer to economic, environmental and technological issues. It proposes a brief overview of the various techniques of energy storage: under the form of chemical energy (hydrocarbons, biomass, hydrogen production), thermal energy (sensitive or latent heat storage), mechanical energy (potential energy by hydraulic or compressed air storage, kinetic energy with flywheels), electrochemical energy (in batteries), electric energy (super-capacitors, superconductor magnetic energy storage). Perspectives are briefly evoked

  2. Dermatillomania: In patient undergoing orthodontic treatment

    Directory of Open Access Journals (Sweden)

    Adit

    2014-01-01

    Full Text Available Dermatillomania is a disorder in which a person habitually picks their skin, and this is a form of self-injury. It can involve any part of the body, but usually involves the face, neck, arms and shoulders. Symptoms often follow an event that has caused severe emotional distress. A dermatillomania or compulsive skin picking episode may be a conscious response to anxiety or depression but is frequently done as an unconscious habit. In this case report, a patient undergoing orthodontic treatment was found to be suffering from dermatillomania and was treated using psychological counseling.

  3. Neutron storage

    International Nuclear Information System (INIS)

    Strelkov, A.V.

    2004-01-01

    The report is devoted to neutron storage (NS) and describes the history of experiments on the NS development. Great attention is paid to ultracold neutron (UCN) storage. The experiments on the UCN generation, transport, spectroscopy, storage and detection are described. Experiments on searching the UCN electric-dipole moment and electric charge are continued. Possible using of UCN for studying the nanoparticles is discussed [ru

  4. Energy storage

    International Nuclear Information System (INIS)

    Odru, P.

    2010-01-01

    This book proposes a broad overview of the technologies developed in the domains of on-board electricity storage (batteries, super-capacitors, flywheels), stationary storage (hydraulic dams, compressed air, batteries and hydrogen), and heat storage (sensible, latent and sorption) together with their relative efficiency, their expected developments and what advantages they can offer. Eminent specialists of this domain have participated to the redaction of this book, all being members of the Tuck's Foundation 'IDees' think tank. (J.S.)

  5. Energy storage

    CERN Document Server

    Brunet, Yves

    2013-01-01

    Energy storage examines different applications such as electric power generation, transmission and distribution systems, pulsed systems, transportation, buildings and mobile applications. For each of these applications, proper energy storage technologies are foreseen, with their advantages, disadvantages and limits. As electricity cannot be stored cheaply in large quantities, energy has to be stored in another form (chemical, thermal, electromagnetic, mechanical) and then converted back into electric power and/or energy using conversion systems. Most of the storage technologies are examined: b

  6. Tritium storage

    International Nuclear Information System (INIS)

    Hircq, B.

    1989-01-01

    A general synthesis about tritium storage is achieved in this paper and a particular attention is given to practical application in the Fusion Technology Program. Tritium, storage under gaseous form and solid form are discussed (characteristics, advantages, disadvantages and equipments). The way of tritium storage is then discussed and a choice established as a function of a logic which takes into account the main working parameters

  7. Coagulation management in patients undergoing neurosurgical procedures.

    Science.gov (United States)

    Robba, Chiara; Bertuetti, Rita; Rasulo, Frank; Bertuccio, Alessando; Matta, Basil

    2017-10-01

    Management of coagulation in neurosurgical procedures is challenging. In this contest, it is imperative to avoid further intracranial bleeding. Perioperative bleeding can be associated with a number of factors, including anticoagulant drugs and coagulation status but is also linked to the characteristic and the site of the intracranial disorder. The aim of this review will be to focus primarily on the new evidence regarding the management of coagulation in patients undergoing craniotomy for neurosurgical procedures. Antihemostatic and anticoagulant drugs have shown to be associated with perioperative bleeding. On the other hand, an increased risk of venous thromboembolism and hypercoagulative state after elective and emergency neurosurgery, in particular after brain tumor surgery, has been described in several patients. To balance the risk between thrombosis and bleeding, it is important to be familiar with the perioperative changes in coagulation and with the recent management guidelines for anticoagulated patients undergoing neurosurgical procedures, in particular for those taking new direct anticoagulants. We have considered the current clinical trials and literature regarding both safety and efficacy of deep venous thrombosis prophylaxis in the neurosurgical population. These were mainly trials concerning both elective surgical and intensive care patients with a poor grade intracranial bleed or multiple traumas with an associated severe traumatic brain injury (TBI). Coagulation management remains a major issue in patients undergoing neurosurgical procedures. However, in this field of research, literature quality is poor and further studies are necessary to identify the best strategies to minimize risks in this group of patients.

  8. Probe Storage

    NARCIS (Netherlands)

    Gemelli, Marcellino; Abelmann, Leon; Engelen, Johannes Bernardus Charles; Khatib, M.G.; Koelmans, W.W.; Zaboronski, Olog; Campardo, Giovanni; Tiziani, Federico; Laculo, Massimo

    2011-01-01

    This chapter gives an overview of probe-based data storage research over the last three decades, encompassing all aspects of a probe recording system. Following the division found in all mechanically addressed storage systems, the different subsystems (media, read/write heads, positioning, data

  9. Speech profile of patients undergoing primary palatoplasty.

    Science.gov (United States)

    Menegueti, Katia Ignacio; Mangilli, Laura Davison; Alonso, Nivaldo; Andrade, Claudia Regina Furquim de

    2017-10-26

    To characterize the profile and speech characteristics of patients undergoing primary palatoplasty in a Brazilian university hospital, considering the time of intervention (early, before two years of age; late, after two years of age). Participants were 97 patients of both genders with cleft palate and/or cleft and lip palate, assigned to the Speech-language Pathology Department, who had been submitted to primary palatoplasty and presented no prior history of speech-language therapy. Patients were divided into two groups: early intervention group (EIG) - 43 patients undergoing primary palatoplasty before 2 years of age and late intervention group (LIG) - 54 patients undergoing primary palatoplasty after 2 years of age. All patients underwent speech-language pathology assessment. The following parameters were assessed: resonance classification, presence of nasal turbulence, presence of weak intraoral air pressure, presence of audible nasal air emission, speech understandability, and compensatory articulation disorder (CAD). At statistical significance level of 5% (p≤0.05), no significant difference was observed between the groups in the following parameters: resonance classification (p=0.067); level of hypernasality (p=0.113), presence of nasal turbulence (p=0.179); presence of weak intraoral air pressure (p=0.152); presence of nasal air emission (p=0.369), and speech understandability (p=0.113). The groups differed with respect to presence of compensatory articulation disorders (p=0.020), with the LIG presenting higher occurrence of altered phonemes. It was possible to assess the general profile and speech characteristics of the study participants. Patients submitted to early primary palatoplasty present better speech profile.

  10. Energy storage

    Energy Technology Data Exchange (ETDEWEB)

    1962-07-01

    The papers on energy storage problems, given to the United Nations Conference on New Sources of Energy, Rome, 1961, are reviewed. Many aspects of the subject are discussed: comparisons between the costs of storing energy in batteries and in fuel cells; the use, efficiency and expected improvement of fuel cells; the principles involved in the chemical conversion of solar energy to chemical energy; the use of metal hydride fuel cells; the chemical conversion and storage of concentrated solar energy for which the solar furnace is used for photochemical reactions. Finally, the general costs of storing energy in any form and delivering it are analyzed with particular reference to storage batteries and fuel cells.

  11. Understanding phonological memory deficits in boys with attention-deficit/hyperactivity disorder (ADHD): dissociation of short-term storage and articulatory rehearsal processes.

    Science.gov (United States)

    Bolden, Jennifer; Rapport, Mark D; Raiker, Joseph S; Sarver, Dustin E; Kofler, Michael J

    2012-08-01

    The current study dissociated and examined the two primary components of the phonological working memory subsystem--the short-term store and articulatory rehearsal mechanism--in boys with ADHD (n = 18) relative to typically developing boys (n = 15). Word lists of increasing length (2, 4, and 6 words per trial) were presented to and recalled by children following a brief (3 s) interval to assess their phonological short-term storage capacity. Children's ability to utilize the articulatory rehearsal mechanism to actively maintain information in the phonological short-term store was assessed using word lists at their established memory span but with extended rehearsal times (12 s and 21 s delays). Results indicate that both phonological shortterm storage capacity and articulatory rehearsal are impaired or underdeveloped to a significant extent in boys with ADHD relative to typically developing boys, even after controlling for age, SES, IQ, and reading speed. Larger magnitude deficits, however, were apparent in short-term storage capacity (ES = 1.15 to 1.98) relative to articulatory rehearsal (ES = 0.47 to 1.02). These findings are consistent with previous reports of deficient phonological short-term memory in boys with ADHD, and suggest that future attempts to develop remedial cognitive interventions for children with ADHD will need to include active components that require children to hold increasingly more information over longer time intervals.

  12. Reduction of suicidal ideation in patients undergoing psychotherapy in the day hospital for the treatment of neurotic and behavioral disorders and neurotic symptoms reported by them before the hospitalization.

    Science.gov (United States)

    Rodziński, Paweł; Rutkowski, Krzysztof; Sobański, Jerzy A; Murzyn Białas, Agnieszka; Cyranka, Katarzyna; Grządziel, Karolina; Smiatek-Mazgaj, Bogna; Klasa, Katarzyna; Müldner-Nieckowski, Łukasz; Dembińska, Edyta; Mielimąka, Michał

    2015-01-01

    Analysis of associations between symptoms reported before the beginning of the hospitalization and reduction of suicidal ideation - or its lack - obtained until the end of the hospitalization in patients of the day hospital for the treatment of neurotic and behavioral disorders. Symptoms Checklist KO"O" and Life Inventory completed by 461 women and 219 men treated with intensive integrative psychotherapy with predominance of psychodynamic approach in the day hospital due to neurotic, behavioral and personality disorders between 2005-2013. Percentages of patients reporting SI initially and at the end of the treatment were 29.1% and 10.2% respectively in women and 36.5% and 13.7% in men. The improvement in terms of initially reported SI was obtained by 84.3% of women and 77.5% of men. Among patients, those initially reporting SI were characterized by greater intensity of neurotic symptoms (ppsychotherapy. As such, those subgroups of women require special attention and diligent selection of the therapeutic methods.

  13. Pair Distribution Function Analysis of Structural Disorder by Nb5+ Inclusion in Ceria: Evidence for Enhanced Oxygen Storage Capacity from Under-Coordinated Oxide.

    Science.gov (United States)

    Hiley, Craig I; Playford, Helen Y; Fisher, Janet M; Felix, Noelia Cortes; Thompsett, David; Kashtiban, Reza J; Walton, Richard I

    2018-02-07

    Partial substitution of Ce 4+ by Nb 5+ is possible in CeO 2 by coinclusion of Na + to balance the charge, via hydrothermal synthesis in sodium hydroxide solution. Pair distribution function analysis using reverse Monte Carlo refinement reveals that the small pentavalent substituent resides in irregular coordination positions in an average fluorite lattice, displaced away from the ideal cubic coordination toward four oxygens. This results in under-coordinated oxygen, which explains significantly enhanced oxygen storage capacity of the materials of relevance to redox catalysis used in energy and environmental applications.

  14. Investigation of parenting stress in primary care-givers with children undergoing autistic disorder%孤独症儿童主要照顾者亲职压力现状的调查分析

    Institute of Scientific and Technical Information of China (English)

    邵国琼; 田征文

    2016-01-01

    Objective To investigate the status of parenting stress in the primary care-givers of children diagnosed with autistic disorder and sum up the nursing strategies. Methods Seventy-eight primary care-givers of children diagnosed with autistic disorder were interviewed using the parenting stress index-short form (PSI-SF). Results The mean score of parenting stress was 104.08 ± 18.32, which was at a high level. The 3 subscales score from high to low:parenting anxiety(36.22 ± 8.46);disabled children (35.64 ± 6.41);parent-child interation disorder(32.01 ± 7.15). Conclusion Medical staff should pay more attertion to these care-givers in order to enhance their ability to cope with various problems in the parenting process and hence reduce the level of parenting pressure.%目的:了解孤独症儿童主要照顾者亲职压力现状,并提出相应的对策。方法采用亲职压力指标简表(parenting stress index-short form, PSI-SF)对78名孤独症儿童主要照顾者进行调查。结果孤独症儿童主要照顾者PSI-SF总分为(104.08±18.32)分,处于较高的水平,其3个子量表得分由高到低依次为亲职愁苦(36.22±8.46)分、困难儿童(35.64±6.41)分、亲子互动失调(32.01±7.15)分。结论医护工作者应加强对孤独症儿童主要照顾者亲职压力的评估,提供孤独症相关知识和情感支持和提高其创伤后成长水平,进而降低其亲职压力水平。

  15. Reversible Li storage for nanosize cation/anion-disordered rocksalt-type oxyfluorides: LiMoO2 - x LiF (0 ≤ x ≤ 2) binary system

    Science.gov (United States)

    Takeda, Nanami; Hoshino, Satoshi; Xie, Lixin; Chen, Shuo; Ikeuchi, Issei; Natsui, Ryuichi; Nakura, Kensuke; Yabuuchi, Naoaki

    2017-11-01

    A binary system of LiMoO2 - x LiF (0 ≤ x ≤ 2), Li1+xMoO2Fx, is systematically studied as potential positive electrode materials for rechargeable Li batteries. Single phase and nanosized samples on this binary system are successfully prepared by using a mechanical milling route. Crystal structures and Li storage properties on the binary system are also examined. Li2MoO2F (x = 1), which is classified as a cation-/anion-disordered rocksalt-type structure and is a thermodynamically metastable phase, delivers a large reversible capacity of over 300 mAh g-1 in Li cells with good reversibility. Highly reversible Li storage is realized for Li2MoO2F consisting of nanosized particles based on Mo3+/Mo5+ two-electron redox as evidenced by ex-situ X-ray absorption spectroscopy coupled with ex-situ X-ray diffractometry. Moreover, the presence of the most electronegative element in the framework structure effectively increases the electrode potential of Mo redox through an inductive effect. From these results, potential of nanosized lithium molybdenum oxyfluorides for high-capacity positive electrode materials of rechargeable Li batteries are discussed.

  16. Storage of long lived solid waste

    International Nuclear Information System (INIS)

    Ozarde, P.D.; Agarwal, K.; Gupta, R.K.; Gandhi, K.G.

    2009-01-01

    Long lived solid waste, generated during the fuel cycle mainly includes high level vitrified waste product, high level cladding hulls and low and intermediate level alpha wastes. These wastes require storage in specially designed engineered facilities before final disposal into deep geological repository. Since high-level vitrified waste contain heat generating radionuclides, the facility for their storage is designed for continuous cooling. High level cladding hulls undergo volume reduction by compaction and will be subsequently stored. (author)

  17. Lipid storage myopathies.

    Science.gov (United States)

    Bruno, Claudio; Dimauro, Salvatore

    2008-10-01

    The aim of this review is to provide an update on disorders of lipid metabolism affecting skeletal muscle exclusively or predominantly and to summarize recent clinical, genetic, and therapeutic studies in this field. Over the past 5 years, new clinical phenotypes and genetic loci have been described, unusual pathogenic mechanisms have been elucidated, and novel pharmacological approaches have been developed. At least one genetic defect responsible for the myopathic form of CoQ10 deficiency has been identified, causing a disorder that is allelic with the late-onset riboflavine-responsive form of multiple acyl-coenzyme A dehydrogenation deficiency. Novel mechanisms involved in the lipolytic breakdown of cellular lipid depots have been described and have led to the identification of genes and mutations responsible for multisystemic neutral lipid storage disorders, characterized by accumulation of triglyceride in multiple tissues, including muscle. Defects in lipid metabolism can affect either the mitochondrial transport and oxidation of exogenous fatty acid or the catabolism of endogenous triglycerides. These disorders impair energy production and almost invariably involve skeletal muscle, causing progressive myopathy with muscle weakness, or recurrent acute episodes of rhabdomyolysis triggered by exercise, fasting, or infections. Clinical and genetic characterization of these disorders has important implications both for accurate diagnostic approach and for development of therapeutic strategies.

  18. Determination of the Oswestry Disability Index score equivalent to a "satisfactory symptom state" in patients undergoing surgery for degenerative disorders of the lumbar spine-a Spine Tango registry-based study.

    Science.gov (United States)

    van Hooff, Miranda L; Mannion, Anne F; Staub, Lukas P; Ostelo, Raymond W J G; Fairbank, Jeremy C T

    2016-10-01

    The achievement of a given change score on a valid outcome instrument is commonly used to indicate whether a clinically relevant change has occurred after spine surgery. However, the achievement of such a change score can be dependent on baseline values and does not necessarily indicate whether the patient is satisfied with the current state. The achievement of an absolute score equivalent to a patient acceptable symptom state (PASS) may be a more stringent measure to indicate treatment success. This study aimed to estimate the score on the Oswestry Disability Index (ODI, version 2.1a; 0-100) corresponding to a PASS in patients who had undergone surgery for degenerative disorders of the lumbar spine. This is a cross-sectional study of diagnostic accuracy using follow-up data from an international spine surgery registry. The sample includes 1,288 patients with degenerative lumbar spine disorders who had undergone elective spine surgery, registered in the EUROSPINE Spine Tango Spine Surgery Registry. The main outcome measure was the ODI (version 2.1a). Surgical data and data from the ODI and Core Outcome Measures Index (COMI) were included to determine the ODI threshold equivalent to PASS at 1 year (±1.5 months; n=780) and 2 years (±2 months; n=508) postoperatively. The symptom-specific well-being item of the COMI was used as the external criterion in the receiver operating characteristic (ROC) analysis to determine the ODI threshold equivalent to PASS. Separate sensitivity analyses were performed based on the different definitions of an "acceptable state" and for subgroups of patients. JF is a copyright holder of the ODI. The ODI threshold for PASS was 22, irrespective of the time of follow-up (area under the curve [AUC]: 0.89 [sensitivity {Se}: 78.3%, specificity {Sp}: 82.1%] and AUC: 0.91 [Se: 80.7%, Sp: 85.6] for the 1- and 2-year follow-ups, respectively). Sensitivity analyses showed that the absolute ODI-22 threshold for the two follow-up time-points were

  19. Lysosomal storage disease 2 - Pompe's disease

    NARCIS (Netherlands)

    van der Ploeg, Ans T.; Reuser, Arnold J. J.

    2008-01-01

    Pompe's disease, glycogen-storage disease type II, and acid maltase deficiency are alternative names for the same metabolic disorder. It is a pan-ethnic autosomal recessive trait characterised by acid alpha-glucosidase deficiency leading to lysosomal glycogen storage. Pompe's disease is also

  20. Reduction of suicidal ideation in patients undergoing psychotherapy in the day hospital for the treatment of neurotic and behavioral disorders and their neurotic personality traits measured before the hospitalization.

    Science.gov (United States)

    Rodziński, Paweł; Rutkowski, Krzysztof; Sobański, Jerzy A; Murzyn, Agnieszka; Mielimąka, Michał; Smiatek-Mazgaj, Bogna; Cyranka, Katarzyna; Dembińska, Edyta; Grządziel, Karolina; Klasa, Katarzyna; Müldner-Nieckowski, Łukasz

    2015-01-01

    Analysis of associations between initial neurotic personality traits and subsequent reduction of suicidal ideation (SI) - or lack of such reduction - obtained until the end of hospitalization in patients who underwent the course of intensive psychotherapy conducted in integrative approach with predominance of psychodynamic approach. Symptom Checklist KO"O", Neurotic Personality Questionnaire KON-2006 and Life Inventory completed by 461 women and 219 men hospitalized in the day-hospital due to neurotic, behavioral and personality disorders between 2005-2013. At the stage of qualification 134 women and 80 men reported SI, of whom subsequently 84.3% and 77.5% respectively improved. Women with prominent Tendency to risk-taking (p=0.002) and Impulsiveness (p=0.038) constituted subgroups with decreased chances of improvement in terms of SI, while men with prominently elevated level of Envy (p=0.041) and women who seemed to have difficulties in expressing anger adequately (ppsychotherapy. Thus, those subgroups require special attention and diligent selection of therapeutic methods. Also, it is probable that focusing therapy at the above-mentioned personality components may increase effectiveness of SI treatment. Reducing SI during psychotherapy appears to be highly effective especially in women with difficulties in expressing anger adequately and in men with prominently elevated level of Envy, which suggests adequacy of this treatment choice and of targeting those difficulties during psychotherapy.

  1. Energy Storage

    CSIR Research Space (South Africa)

    Bladergroen, B

    2015-10-01

    Full Text Available In commercial arena, the most recent developments in EES are in electrochemical storage, singling out Li-ion batteries and Vanadium Redox flow batteries, while power-to-gas/-fuels (electrolysis of water into hydrogen and subsequent methanisation...

  2. Liver Storage

    African Journals Online (AJOL)

    1971-10-23

    Oct 23, 1971 ... The need for whole-organ preservation has become in- cre.asingly important ... ideally fulfil the same purpose as the circulation of blood through the body, ... Hepatic hypothermia produced by the introduction of cold electrolyte ... Recently, we reported successful hypothermic immersion storage for up to 8 ...

  3. Dry storage

    International Nuclear Information System (INIS)

    Arnott, Don.

    1985-01-01

    The environmental movement has consistently argued against disposal of nuclear waste. Reasons include its irretrievability in the event of leakage, the implication that reprocessing will continue and the legitimacy attached to an expanding nuclear programme. But there is an alternative. The author here sets out the background and a possible future direction of a campaign based on a call for dry storage. (author)

  4. Sobrevida e complicações em idosos com doenças neurológicas em nutrição enteral Occurrence of complications and survival rates in elderly with neurological disorders undergoing enteral nutrition therapy

    Directory of Open Access Journals (Sweden)

    Aline Stangherlin Martins

    2012-12-01

    Full Text Available OBJETIVO: Avaliar a sobrevida e complicações de pacientes idosos com doenças neurológicas em uso de nutrição enteral (NE. MÉTODOS: Avaliaram-se pacientes acima de 60 anos acompanhados pelo serviço de atenção domiciliar de um plano de saúde de Belo Horizonte, MG, Brasil. A avaliação ocorreu no domicílio após a alta hospitalar com NE, após três e seis meses e ao término do estudo. Foram realizadas avaliação nutricional, coleta de dados em prontuários e entrevistas com familiares ou cuidadores. RESULTADOS: Foram avaliados 79 pacientes, idade 82,9 ± 10,4 anos, 49,4% com demência e 50,6% com outros diagnósticos neurológicos, 100% com elevado grau de dependência avaliada pelo índice de Katz. A maioria dos pacientes (91,2% apresentou complicações (pneumonia, perda da sonda, diarreia, constipação, vômito, extravasamento periostomia, obstrução da sonda, refluxo e miíase. Pneumonia foi a mais frequente, ocorrendo em 55,9%. A mortalidade foi de 15,2% aos três meses, 22,8% aos 6 meses e 43% ao término do estudo. A mediana de sobrevida após iniciada a NE foi de 364 dias. Não se observaram diferenças entre mortalidade e diagnóstico neurológico, vias de acesso de NE e complicações. A sobrevida foi menor em pacientes com estado nutricional inadequado e albumina OBJECTIVE: To evaluate the occurrence of complications, as well as the survival rates, in elderly people having neurological diseases and undergoing enteral nutrition therapy (ENT. METHODS: Patients aged over 60 years, assisted by a home medical service from a healthcare plan in the city of Belo Horizonte, MG, Brazil, were thoroughly evaluated. The mentioned evaluation occurred at their homes after hospital discharge with enteral nutrition (EN after a three-month period, a six-month period, and at the end of the study. A nutritional assessment was performed along with data collection performed on the patients' electronic medical records, and interviews

  5. Storage Rings

    International Nuclear Information System (INIS)

    Fischer, W.

    2010-01-01

    Storage rings are circular machines that store particle beams at a constant energy. Beams are stored in rings without acceleration for a number of reasons (Tab. 1). Storage rings are used in high-energy, nuclear, atomic, and molecular physics, as well as for experiments in chemistry, material and life sciences. Parameters for storage rings such as particle species, energy, beam intensity, beam size, and store time vary widely depending on the application. The beam must be injected into a storage ring but may not be extracted (Fig. 1). Accelerator rings such as synchrotrons are used as storage rings before and after acceleration. Particles stored in rings include electrons and positrons; muons; protons and anti-protons; neutrons; light and heavy, positive and negative, atomic ions of various charge states; molecular and cluster ions, and neutral polar molecules. Spin polarized beams of electrons, positrons, and protons were stored. The kinetic energy of the stored particles ranges from 10 -6 eV to 3.5 x 10 12 eV (LHC, 7 x 10 12 eV planned), the number of stored particles from one (ESR) to 1015 (ISR). To store beam in rings requires bending (dipoles) and transverse focusing (quadrupoles). Higher order multipoles are used to correct chromatic aberrations, to suppress instabilities, and to compensate for nonlinear field errors of dipoles and quadrupoles. Magnetic multipole functions can be combined in magnets. Beams are stored bunched with radio frequency systems, and unbunched. The magnetic lattice and radio frequency system are designed to ensure the stability of transverse and longitudinal motion. New technologies allow for better storage rings. With strong focusing the beam pipe dimensions became much smaller than previously possible. For a given circumference superconducting magnets make higher energies possible, and superconducting radio frequency systems allow for efficient replenishment of synchrotron radiation losses of large current electron or positron beams

  6. Superconducting energy storage magnet

    Science.gov (United States)

    Boom, Roger W. (Inventor); Eyssa, Yehia M. (Inventor); Abdelsalam, Mostafa K. (Inventor); Huang, Xianrui (Inventor)

    1993-01-01

    A superconducting magnet is formed having composite conductors arrayed in coils having turns which lie on a surface defining substantially a frustum of a cone. The conical angle with respect to the central axis is preferably selected such that the magnetic pressure on the coil at the widest portion of the cone is substantially zero. The magnet structure is adapted for use as an energy storage magnet mounted in an earthen trench or tunnel where the strength the surrounding soil is lower at the top of the trench or tunnel than at the bottom. The composite conductor may be formed having a ripple shape to minimize stresses during charge up and discharge and has a shape for each ripple selected such that the conductor undergoes a minimum amount of bending during the charge and discharge cycle. By minimizing bending, the working of the normal conductor in the composite conductor is minimized, thereby reducing the increase in resistance of the normal conductor that occurs over time as the conductor undergoes bending during numerous charge and discharge cycles.

  7. The use of port-a-caths in adult patients with Lysosomal Storage Disorders receiving Enzyme Replacement Therapy-one centre experience

    Directory of Open Access Journals (Sweden)

    Mairead McLoughlin

    2017-12-01

    Full Text Available Port-a-cath is a widely used device in patients with long-term venous access demand such as frequent or continuous administration of medications such as Enzyme Replacement Therapy (ERT, chemotherapy delivery, blood transfusions, blood products, and fluids. Patients with Lysosomal Storage Diseases (LSDs often require recurrent courses of ERT. We reviewed our experience of using port-a-caths in patients with LSDs with the focus on challenges and complications associated with these catheters. Among 245 adult patients who were treated with ERT, twenty patients (8.2% had a port-a-cath inserted due to poor venous access. Six patients were using their first port whereas five other patients had their port-a-caths replaced at least once. The remaining six patients had inactive port-a-caths. The majority of patients with active port-a-caths never missed more than one consecutive infusion, although one patient missed 2 consecutive infusions whilst on holiday. We identified significant gaps in patients' and their families' understanding of the management of port-a-caths and risks associated with them. It resulted in producing a leaflet and designing an educational program for our LSD patients.

  8. Underground Storage Tanks - Storage Tank Locations

    Data.gov (United States)

    NSGIC Education | GIS Inventory — A Storage Tank Location is a DEP primary facility type, and its sole sub-facility is the storage tank itself. Storage tanks are aboveground or underground, and are...

  9. What's Up with the Storage Hierarchy?

    DEFF Research Database (Denmark)

    Bonnet, Philippe

    2017-01-01

    Ten years ago, Jim Gray observed that flash was about to replace magnetic disks. He also predicted that the need for low latency would make main memory databases commonplace. Most of his predictions have proven accurate. Today, who can make predictions about the future of the storage hierarchy......? Both main memory and storage systems are undergoing profound transformations. First, their design goals are increasingly complex (reconfigurable infrastructure at low latency, high resource utilization and stable energy footprint). Second, the status quo is not an option due to the shortcomings...... of existing solutions (memory bandwidth gap, inefficiency of generic memory/storage controllers). Third, new technologies are emerging (hybrid memories, non-volatile memories still under non-disclosure agreements, near-data processing in memory and storage). The impact of these transformations on the storage...

  10. Fuel storage

    International Nuclear Information System (INIS)

    Palacios, C.; Alvarez-Miranda, A.

    2009-01-01

    ENSA is a well known manufacturer of multi-system primary components for the nuclear industry and is totally prepared to satisfy future market requirements in this industry. At the same time that ENSA has been gaining a reputation world wider for the supply of primary components, has been strengthening its commitment and experience in supplying spent fuel components, either pool racks or storage and transportation casks, and offers not only fabrication but also design capabilities for its products. ENSA has supplied Spent Fuel Pool Racks, in spain, Finland, Taiwan, Korea, China, and currently it is in the process of licensing its own rack design in the United States of America for the ESBWR along with Ge-Hitachi. ENSA has supplied racks for 20 pools and 22 different reactors and it has also manufactured racks under all available technologies and developed a design known as Interlock Cell Matrix whose main features are outlined in this article. Another ENSA achievement in rack technology is the use of remote control for re-racking activities instead of using divers, which improves the ALARA requirements. Regarding casks for storage and transportation, ENSA also has al leading worldwide position, with exports prevailing over the Spanish market where ENSA has supplied 16 storage and transportation casks to the Spanish nuclear power Trillo. In some cases, ENSA acts as subcontractor for other clients. Foreign markets are still a major challenge for ENSA. ENSA-is well known for its manufacturing capabilities in the nuclear industry, but has been always involved in design activities through its engineering division, which carries out different tasks: components Design; Tooling Design; Engineering and Documentation; Project Engineering; Calculations, Design and Development Engineering. (Author)

  11. Underground storage

    Energy Technology Data Exchange (ETDEWEB)

    1965-06-10

    A procedure is described for making an underground storage cavity in a soluble formation. Two holes are drilled, and fluid is pumped into the first hole. This fluid is a non-solute for the formation material. Then pressure is applied to the fluid until the formation is fractured in the direction of the second hole. More non-solute fluid is injected to complete the fracture between the 2 holes. A solute fluid is then circulated between the 2 holes, which results in removal of that part of the formation next to the fracture and the forming of a chamber.

  12. Pumped storage

    International Nuclear Information System (INIS)

    Strauss, P.L.

    1991-01-01

    The privately financed 1,000 MW Rocky Point Pumped Storage Project located in central Colorado, USA, will be one of the world's highest head, 2,350 feet reversible pump/turbine projects. The project will offer an economical supply of peaking power and spinning reserve power to Colorado and other southwestern states. This paper describes how the project will be made compatible with the environmental conditions in the project area and the type of terrestrial mitigation measures that are being proposed for those situations where the project impacts the environment, either temporarily or permanently

  13. Safety and potential efficacy of gemfibrozil as a supportive treatment for children with late infantile neuronal ceroid lipofuscinosis and other lipid storage disorders.

    Science.gov (United States)

    Kim, Kyeongsoon; Kleinman, Hynda K; Lee, Hahn-Jun; Pahan, Kalipada

    2017-06-17

    Neuronal Ceroid Lipofuscinosis (NCL), also known as Batten disease, is a group of genetically distinct lysosomal disorders that mainly affect the central nervous system, resulting in progressive motor and cognitive decline primarily in children. Multiple distinct genes involved in the metabolism of lipids have been identified to date with various mutations in this family of diseases. There is no cure for these diseases but some new therapeutic approaches have been tested that offer more hope than the standard palliative care. Many of the therapeutic advances require invasive procedures but some progress in slowing the disease has been found and more options can be expected in the future. We also review the literature on children with disease/conditions other than NCL for the non-invasive use, safety, and tolerability of a lipid-lowering drug, gemfibrozil, as a potential treatment for NCLs. Gemfibrozil has shown efficacy in an animal model of NCL known as CLN2 (late infantile classic juvenile) and has been shown to be safe for lowering lipids in children. Among the 200 non-NCL children found in the published literature who were treated with gemfibrozil for NCL-related problems, only 3 experienced adverse events, including 2 with muscle pain and 1 with localized linear IgA bullous dermatitis. We conclude that gemfibrozil is safe for long-term use in children, causes minimal adverse events, is well tolerated, and may delay the progression of NCLs. Gemfibrozil may potentially be an alternative to more invasive therapeutic approaches currently under investigation and has the potential to be used in combination with other therapeutic approaches.

  14. Gas storage materials, including hydrogen storage materials

    Science.gov (United States)

    Mohtadi, Rana F; Wicks, George G; Heung, Leung K; Nakamura, Kenji

    2013-02-19

    A material for the storage and release of gases comprises a plurality of hollow elements, each hollow element comprising a porous wall enclosing an interior cavity, the interior cavity including structures of a solid-state storage material. In particular examples, the storage material is a hydrogen storage material such as a solid state hydride. An improved method for forming such materials includes the solution diffusion of a storage material solution through a porous wall of a hollow element into an interior cavity.

  15. Geological storage of radioactive waste

    International Nuclear Information System (INIS)

    Barthoux, A.

    1983-01-01

    Certain radioactive waste contains substances which present, although they disappear naturally in a progressive manner, a potential risk which can last for very long periods, of over thousands of years. To ensure a safe long-term handling, provision has been made to bury it deep in stable geological structures which will secure its confinement. Radioactive waste is treated and conditioned to make it insoluble and is then encased in matrices which are to immobilize them. The most radioactive waste is thus incorporated in a matrix of glass which will ensure the insulation of the radioactive substances during the first thousands of years. Beyond that time, the safety will be ensured by the properties of the storage site which must be selected from now on. Various hydrogeological configurations have been identified. They must undergo detailed investigations, including even the creation of an underground laboratory. This document also presents examples of underground storage installations which are due to be built [fr

  16. Lysosomal Storage Diseases To date

    OpenAIRE

    HOFFMANN, Björn; MAYATEPEK, Ertan

    2011-01-01

    New therapeutic options and progress of approved therapies have made Lysosomal Storage Diseases (LSDs) one of the most exciting group of diseases. This review aims to summarize current achievements in these particular disorders and to give an outlook towards possible future treatment options. Enzyme replacement therapy is the gold standard for Gaucher disease, Fabry disease, Mucopolysaccharidosis type I, II, and VI, and for Pompe disease. Besides this, substrate reduction has been approved fo...

  17. Lysosomal storage diseases

    Science.gov (United States)

    Ferreira, Carlos R.; Gahl, William A.

    2016-01-01

    Lysosomes are cytoplasmic organelles that contain a variety of different hydrolases. A genetic deficiency in the enzymatic activity of one of these hydrolases will lead to the accumulation of the material meant for lysosomal degradation. Examples include glycogen in the case of Pompe disease, glycosaminoglycans in the case of the mucopolysaccharidoses, glycoproteins in the cases of the oligosaccharidoses, and sphingolipids in the cases of Niemann-Pick disease types A and B, Gaucher disease, Tay-Sachs disease, Krabbe disease, and metachromatic leukodystrophy. Sometimes, the lysosomal storage can be caused not by the enzymatic deficiency of one of the hydrolases, but by the deficiency of an activator protein, as occurs in the AB variant of GM2 gangliosidosis. Still other times, the accumulated lysosomal material results from failed egress of a small molecule as a consequence of a deficient transporter, as in cystinosis or Salla disease. In the last couple of decades, enzyme replacement therapy has become available for a number of lysosomal storage diseases. Examples include imiglucerase, taliglucerase and velaglucerase for Gaucher disease, laronidase for Hurler disease, idursulfase for Hunter disease, elosulfase for Morquio disease, galsulfase for Maroteaux-Lamy disease, alglucosidase alfa for Pompe disease, and agalsidase alfa and beta for Fabry disease. In addition, substrate reduction therapy has been approved for certain disorders, such as eliglustat for Gaucher disease. The advent of treatment options for some of these disorders has led to newborn screening pilot studies, and ultimately to the addition of Pompe disease and Hurler disease to the Recommended Uniform Screening Panel (RUSP) in 2015 and 2016, respectively. PMID:29152458

  18. Lysosomal lipid storage diseases.

    Science.gov (United States)

    Schulze, Heike; Sandhoff, Konrad

    2011-06-01

    Lysosomal lipid storage diseases, or lipidoses, are inherited metabolic disorders in which typically lipids accumulate in cells and tissues. Complex lipids, such as glycosphingolipids, are constitutively degraded within the endolysosomal system by soluble hydrolytic enzymes with the help of lipid binding proteins in a sequential manner. Because of a functionally impaired hydrolase or auxiliary protein, their lipid substrates cannot be degraded, accumulate in the lysosome, and slowly spread to other intracellular membranes. In Niemann-Pick type C disease, cholesterol transport is impaired and unesterified cholesterol accumulates in the late endosome. In most lysosomal lipid storage diseases, the accumulation of one or few lipids leads to the coprecipitation of other hydrophobic substances in the endolysosomal system, such as lipids and proteins, causing a "traffic jam." This can impair lysosomal function, such as delivery of nutrients through the endolysosomal system, leading to a state of cellular starvation. Therapeutic approaches are currently restricted to mild forms of diseases with significant residual catabolic activities and without brain involvement.

  19. Search for electric dipole moments in storage rings

    Directory of Open Access Journals (Sweden)

    Lenisa Paolo

    2016-01-01

    Full Text Available The JEDI collaboration aims at making use of storage ring to provide the most precise measurement of the electric dipole moments of hadrons. The method makes exploits a longitudinal polarized beam. The existence an electric dipole moment would generate a torque slowly twisting the particle spin out of plan of the storage ring into the vertical direction. The observation of non zero electric dipole moment would represent a clear sign of new physics beyond the Standard Model. Feasiblity tests are presently undergoing at the COSY storage ring Forschungszentrum Jülich (Germany, to develop the novel techniques to be implemented in a future dedicated storage ring.

  20. Biomarker for Glycogen Storage Diseases

    Science.gov (United States)

    2017-07-03

    Fructose Metabolism, Inborn Errors; Glycogen Storage Disease; Glycogen Storage Disease Type I; Glycogen Storage Disease Type II; Glycogen Storage Disease Type III; Glycogen Storage Disease Type IV; Glycogen Storage Disease Type V; Glycogen Storage Disease Type VI; Glycogen Storage Disease Type VII; Glycogen Storage Disease Type VIII

  1. Anxiety Disorders

    Science.gov (United States)

    ... Registry Residents & Medical Students Residents Medical Students Patients & Families Mental Health Disorders/Substance Use Find a Psychiatrist Addiction and Substance Use Disorders ADHD Anxiety Disorders Autism Spectrum Disorder Bipolar Disorders Depression Eating Disorders Obsessive-Compulsive ...

  2. Mental Disorders

    Science.gov (United States)

    Mental disorders include a wide range of problems, including Anxiety disorders, including panic disorder, obsessive-compulsive disorder, ... disorders, including schizophrenia There are many causes of mental disorders. Your genes and family history may play ...

  3. Electrochemical hydrogen Storage Systems

    Energy Technology Data Exchange (ETDEWEB)

    Dr. Digby Macdonald

    2010-08-09

    described in the previous literature for electrochemical reduction of spent fuels, have been attempted. A quantitative analytical method for measuring the concentration of sodium borohydride in alkaline aqueous solution has been developed as part of this work and is described herein. Finally, findings from stability tests for sodium borohydride in aqueous solutions of several different compositions are reported. For aminoborane, other research institutes have developed regeneration schemes involving tributyltin hydride. In this report, electrochemical reduction experiments attempting to regenerate tributyltin hydride from tributyltin chloride (a representative by-product of the regeneration scheme) are described. These experiments were performed in the non-aqueous solvents acetonitrile and 1,2-dimethoxyethane. A non-aqueous reference electrode for electrolysis experiments in acetonitrile was developed and is described. One class of boron hydrides, called polyhedral boranes, became of interest to the DOE due to their ability to contain a sufficient amount of hydrogen to meet program goals and because of their physical and chemical safety attributes. Unfortunately, the research performed here has shown that polyhedral boranes do not react in such a way as to allow enough hydrogen to be released, nor do they appear to undergo hydrogenation from the spent fuel form back to the original hydride. After the polyhedral boranes were investigated, the project goals remained the same but the hydrogen storage material was switched by the DOE to ammonia borane. Ammonia borane was found to undergo an irreversible hydrogen release process, so a direct hydrogenation was not able to occur. To achieve the hydrogenation of the spent ammonia borane fuel, an indirect hydrogenation reaction is possible by using compounds called organotin hydrides. In this process, the organotin hydrides will hydrogenate the spent ammonia borane fuel at the cost of their own oxidation, which forms organotin

  4. Electrochemical hydrogen Storage Systems

    International Nuclear Information System (INIS)

    Macdonald, Digby

    2010-01-01

    previous literature for electrochemical reduction of spent fuels, have been attempted. A quantitative analytical method for measuring the concentration of sodium borohydride in alkaline aqueous solution has been developed as part of this work and is described herein. Finally, findings from stability tests for sodium borohydride in aqueous solutions of several different compositions are reported. For aminoborane, other research institutes have developed regeneration schemes involving tributyltin hydride. In this report, electrochemical reduction experiments attempting to regenerate tributyltin hydride from tributyltin chloride (a representative by-product of the regeneration scheme) are described. These experiments were performed in the non-aqueous solvents acetonitrile and 1,2-dimethoxyethane. A non-aqueous reference electrode for electrolysis experiments in acetonitrile was developed and is described. One class of boron hydrides, called polyhedral boranes, became of interest to the DOE due to their ability to contain a sufficient amount of hydrogen to meet program goals and because of their physical and chemical safety attributes. Unfortunately, the research performed here has shown that polyhedral boranes do not react in such a way as to allow enough hydrogen to be released, nor do they appear to undergo hydrogenation from the spent fuel form back to the original hydride. After the polyhedral boranes were investigated, the project goals remained the same but the hydrogen storage material was switched by the DOE to ammonia borane. Ammonia borane was found to undergo an irreversible hydrogen release process, so a direct hydrogenation was not able to occur. To achieve the hydrogenation of the spent ammonia borane fuel, an indirect hydrogenation reaction is possible by using compounds called organotin hydrides. In this process, the organotin hydrides will hydrogenate the spent ammonia borane fuel at the cost of their own oxidation, which forms organotin halides. To enable a

  5. Schizoaffective disorder

    Science.gov (United States)

    ... or do not improve with treatment Thoughts of suicide or of harming others Alternative Names Mood disorder - schizoaffective disorder; Psychosis - schizoaffective disorder Images Schizoaffective disorder ...

  6. Characteristics of storage capacity of working memory in children with attention-deficit/hyperactivity disorder%注意缺陷多动障碍儿童的工作记忆存储特征

    Institute of Scientific and Technical Information of China (English)

    郭耀光; 陈强; 周翔; 静进

    2012-01-01

    Objective: To analyze the characteristics of storage capacity of working memory in children with (attention-deficit/hyperactivity disorder, ADHD). Methods: Twenty-six children with ADHD were selected as the case group who were diagnosed according to the Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition (DSM-IV), and 39 age- and sex-matched normal children were selected as the control group. All the subjects were assessed with the China Version-Wechsler Intelligence Scale for Children (C-W1SC), modified version of number recall, modified version of lapping test, spatial memory and hand movement in Kaufman Assessment Battery for Children (K-ABC), to examine their storage capacity of working memory. Results: The ADHD group got lower scores in the items of orthodromic number recall (10 s later) [(5,5± 1.3) vs. (6.8 ± 1.5) ], reverse number recall [(3.3 ± 1. 1) vs. (4. 1 ±1.0)], orthodromic click [(4.4 ±1.1) vs. (5. 1 ±0.8)], orthodromic click (10 s later) [(3.4 ±1.3) vs. (4. 1 ±1.1)] in tapping test, and hand movement test [(8.5 ± 3.3) vs. (10.4 ± 3.1), P 0.05). Multiple linear regression model indicated that the lower scores of orthodromic number recall (10 s later) (β = 0. 31), orthodromic click (β = 0.29) and orthodromic click (10 s later) score (β = 0.24) in tapping test were related to ADHD (Ps < 0.05). Conclusion: It suggests that the children with ADHD might have the deficiency of visuo-spatial sketch pad with remembering orthodromic information and low capacity to keep information in phonological loop.%目的:探讨注意缺陷多动障碍(ADHD)儿童工作记忆存储能力特征.方法:对26名符合美国精神障碍诊断与统计手册第4版(DSM-Ⅳ)诊断标准的ADHD儿童和39名年龄及性别与之匹配的正常儿童,进行中国修订-韦氏儿童智力量表(C-WISC)、背数测验变式、方块图敲击试验变式和Kaufman儿童能力评估成套测验的寻找位置和手动作分测验测试,测查言语工

  7. A Comprehensive Investigation of Memory Impairment in Attention Deficit Hyperactivity Disorder and Oppositional Defiant Disorder

    Science.gov (United States)

    Rhodes, Sinead M.; Park, Joanne; Seth, Sarah; Coghill, David R.

    2012-01-01

    Background: We conducted a comprehensive and systematic assessment of memory functioning in drug-naive boys with attention deficit hyperactivity disorder (ADHD) and oppositional defiant disorder (ODD). Methods: Boys performed verbal and spatial working memory (WM) component (storage and central executive) and verbal and spatial storage load tasks,…

  8. Specialised care in patients undergoing pancreatoduodenectomy

    NARCIS (Netherlands)

    Tol, J.A.M.G.

    2014-01-01

    This thesis studies the controversies in the management of patients with pancreatic cancer undergoing pancreatoduodenectomy and determines different factors that will improve this management and thereby the postoperative outcomes. The studies were performed in both the pre-, peri- and postoperative

  9. Antibiotic prophylaxis for patients undergoing elective endoscopic ...

    African Journals Online (AJOL)

    Antibiotic prophylaxis for patients undergoing elective endoscopic retrograde cholangiopancreatography. M Brand, D Bisoz. Abstract. Background. Antibiotic prophylaxis for endoscopic retrograde cholangiopancreatography (ERCP) is controversial. We set out to assess the current antibiotic prescribing practice among ...

  10. Fuel assembly storage pool

    International Nuclear Information System (INIS)

    Hiranuma, Hiroshi.

    1976-01-01

    Object: To remove limitation of the number of storage of fuel assemblies to increase the number of storage thereof so as to relatively reduce the water depth required for shielding radioactive rays. Structure: Fuel assembly storage rack containers for receiving a plurality of spent fuel assembly racks are stacked in multi-layer fashion within a storage pool filled with water for shielding radioactive rays and removing heat. (Furukawa, Y.)

  11. Heat transport and storage

    International Nuclear Information System (INIS)

    Despois, J.

    1977-01-01

    Recalling the close connections existing between heat transport and storage, some general considerations on the problem of heat distribution and transport are presented 'in order to set out the problem' of storage in concrete form. This problem is considered in its overall plane, then studied under the angle of the different technical choices it involves. The two alternatives currently in consideration are described i.e.: storage in a mined cavity and underground storage as captive sheet [fr

  12. Storage in Europe

    International Nuclear Information System (INIS)

    Cabanes, J.M.; Rottenberg, J.; Abiad, A.; Caudron, S.; Girault, Ph.

    2007-01-01

    Storage represents one of the key elements among the different modulation tools. How the problem of storage is put forward in Europe in front of the increasing uncertainty of the gas demand and prices? What are the policies implemented by storage facility operators? To what extend storage can amortize gas prices volatility or allow the market actors to take the best profit of this volatility? These are the questions debated at this workshop by four specialists of this domain. (J.S.)

  13. The proteome of red cell membranes and vesicles during storage in blood bank conditions.

    NARCIS (Netherlands)

    Bosman, G.J.C.G.M.; Lasonder, E.; Luten, M.; Roerdinkholder-Stoelwinder, B.; Novotny, V.M.J.; Bos, H.; Grip, W.J. de

    2008-01-01

    BACKGROUND: During storage of red cells (RBCs) for transfusion, RBCs undergo a number of biochemical and morphologic changes. To be able to identify the mechanisms underlying these storage lesions, a proteomic analysis of the membranes of RBCs and their vesicles was performed during various periods

  14. Pit Water Storage Ottrupgaard

    DEFF Research Database (Denmark)

    Heller, Alfred

    2000-01-01

    The pit water storage, a seasonal thermal storage, was built in 1993 with floating lid and hybrid clay-polymer for pit lining. The storage was leaking severe and solutions were to be found. In the paper solutions for pit lining and floating lids are discussed, cost estimations given and coming...

  15. Wind turbine storage systems

    International Nuclear Information System (INIS)

    Ibrahim, H.; Ilinca, A.; Perron, J.

    2005-01-01

    Electric power is often produced in locations far from the point of utilization which creates a challenge in stabilizing power grids, particularly since electricity cannot be stored. The production of decentralized electricity by renewable energy sources offers a greater security of supply while protecting the environment. Wind power holds the greatest promise in terms of environmental protection, competitiveness and possible applications. It is known that wind energy production is not always in phase with power needs because of the uncertainty of wind. For that reason, energy storage is the key for the widespread integration of wind energy into the power grids. This paper proposed various energy storage methods that can be used in combination with decentralized wind energy production where an imbalance exists between electricity production and consumption. Energy storage can play an essential role in bringing value to wind energy, particularly if electricity is to be delivered during peak hours. Various types of energy storage are already in use or are being developed. This paper identified the main characteristics of various electricity storage techniques and their applications. They include stationary or embarked storage for long or short term applications. A comparison of characteristics made it possible to determine which types of electricity storage are best suited for wind energy. These include gravity energy; thermal energy; compressed air energy; coupled storage with natural gas; coupled storage with liquefied gas; hydrogen storage for fuel cells; chemical energy storage; storage in REDOX batteries; storage by superconductive inductance; storage in supercondensers; and, storage as kinetic energy. 21 refs., 21 figs

  16. Fuel storage tank

    International Nuclear Information System (INIS)

    Peehs, M.; Stehle, H.; Weidinger, H.

    1979-01-01

    The stationary fuel storage tank is immersed below the water level in the spent fuel storage pool. In it there is placed a fuel assembly within a cage. Moreover, the storage tank has got a water filling and a gas buffer. The water in the storage tank is connected with the pool water by means of a filter, a surge tank and a water purification facility, temperature and pressure monitoring being performed. In the buffer compartment there are arranged catalysts a glow plugs for recombination of radiolysis products into water. The supply of water into the storage tank is performed through the gas buffer compartment. (DG) [de

  17. Assessing storage adequacy

    International Nuclear Information System (INIS)

    Amirault, P.

    2004-01-01

    Government policy encourages the use of natural gas. It is expected that liquefied natural gas (LNG) and Arctic gas will make up 20 to 25 per cent of supply. This presentation provided an outlook of storage value based on a technical analysis by the National Petroleum Counsel (NPC) report. A moderately robust growth is expected in the residential and commercial load which may be partially offset by robust growth in electricity. The net result is an increase in storage requirements. It was concluded that there is a strong case for growth in storage demand but a lack of good sites for additional capacity. This will lead to higher storage values. The NPC sees the need for 1 Tcf more storage use by 2025, of which 700 Bcf will need to come from new storage. In particular, current storage levels may not be sufficient to meet a colder than normal winter, and deliverability is affected by field inventory. Most storage capacity was built before 1985, mostly by regulated entities. It is expected that only 250 to 400 Bcf will be added over the next 25 years in North America. If storage becomes scarce, prices will move to the marginal cost of new additions, and the upper limit on price will be determined by salt cavern storage. An increase of $1.00 in the price of leasing storage would add about $0.11 to the average price of consumed gas. tabs., figs

  18. Spent fuel storage rack

    International Nuclear Information System (INIS)

    Morikawa, Matsuo; Uchiyama, Yuichi.

    1983-01-01

    Purpose: To improve the safety and facilitate the design by limiting the relative displacement in a storage rack. Constitution: The outer wall of a storage rack disposed in water within a fuel pool, the pool wall opposing to the storage rack and the structure between the opposing storages racks are made as a space for confining the pool water or a structure formed with a slight gap, for example, a combination of a recessed structure and a protruded structure. In such a constitution, a space for confirming the pool water is established and the pool water thus confined forms a flow resistance when the storage rack vibrates upon earthquakes, serves as a damper and significantly reduces the responsivity. Furthermore, the relative displacement in the storage rack is limited to inhibit excess earthquake forces to exert on setting bolts and rack clamping bolts of the storage rack. (Sekiya, K.)

  19. [Thyroid and cardiovascular disorders].

    Science.gov (United States)

    Zyśko, Dorota; Gajek, Jacek

    2004-05-01

    In this study three problems concerning interactions between thyroid and cardiovascular system are discussed. Cardiac arrhythmias, congestive heart failure, pleural effusion, hyperlipidaemia, arterial hypertension may be consequences of thyroid disorders leading to inappropriate hormone secretion. During such illnesses as heart failure, myocardial infarction and in patients undergoing coronary artery bypass surgery profound changes may occur in thyroid hormone metabolism known as sick euthyroid syndrome. Treatment with amiodarone may lead to changes in thyroid tests results and to development of hypothyroidism or thyrotoxicosis.

  20. Pulmonary hypertenstion ad leading factor in patients undergoing dialysis

    International Nuclear Information System (INIS)

    Rehman, I.U.; Sumera, A.; Idrees, M.K.; Tanweer, A.

    2014-01-01

    Objective: To determine the frequency and leading factors of pulmonary hypertension among chronic hemodialysis patients. Study Design: Case series. Place and Duration of Study: Hemodialysis Unit, Department of Nephrology, Sindh Institute of Urology and Transplantation, Karachi, from September 2011 to March 2012. Methodology: Patients of either gender aged between 16 to 60 years of age undergoing hemodialysis for at least 3 months not having pre-existing valvular heart disease, chronic lung disease or connective tissue disorder were included. Pulmonary hypertension was prospectively estimated by Doppler echocardiogram on patients undergoing dialysis. Pulmonary artery pressure was calculated on the post-dialysis day and leading factors were compared between patients with and without pulmonary hypertension. Results: A total of 178 patients were included in study with male to female ratio120/58 (2.06:1). The mean age was 33.84 +- 11.9 years. The mean duration of hemodialysis was 23.85 +- 22.48 months. Pulmonary hypertension was found in 76 (42.7%) patients. Out of the studied factors, low serum albumin ( 3.4 mg/dl, p = 0.01) was found to be statistically significant in patients with pulmonary hypertension. Conclusion: Pulmonary hypertension was frequently present in dialysis population (42.7%). This subset of patients had significantly lower albumin levels in serum. More research is needed in its pathogenesis to arrest its course. (author)

  1. PC-Cluster based Storage System Architecture for Cloud Storage

    OpenAIRE

    Yee, Tin Tin; Naing, Thinn Thu

    2011-01-01

    Design and architecture of cloud storage system plays a vital role in cloud computing infrastructure in order to improve the storage capacity as well as cost effectiveness. Usually cloud storage system provides users to efficient storage space with elasticity feature. One of the challenges of cloud storage system is difficult to balance the providing huge elastic capacity of storage and investment of expensive cost for it. In order to solve this issue in the cloud storage infrastructure, low ...

  2. Large mass storage facility

    International Nuclear Information System (INIS)

    Peskin, A.M.

    1978-01-01

    The report of a committee to study the questions surrounding possible acquisition of a large mass-storage device is presented. The current computing environment at BNL and justification for an online large mass storage device are briefly discussed. Possible devices to meet the requirements of large mass storage are surveyed, including future devices. The future computing needs of BNL are prognosticated. 2 figures, 4 tables

  3. Benchmarking Cloud Storage Systems

    OpenAIRE

    Wang, Xing

    2014-01-01

    With the rise of cloud computing, many cloud storage systems like Dropbox, Google Drive and Mega have been built to provide decentralized and reliable file storage. It is thus of prime importance to know their features, performance, and the best way to make use of them. In this context, we introduce BenchCloud, a tool designed as part of this thesis to conveniently and efficiently benchmark any cloud storage system. First, we provide a study of six commonly-used cloud storage systems to ident...

  4. Patients Undergoing Dacryocystorhinostomy Surgery in Northern ...

    African Journals Online (AJOL)

    the excess overflow of tears called Epiphora.[1] A disorder of the tear drainage is either functional or anatomical abnormalities. In terms of functional disorder, pumping dysfunction is the problem, which may be due to poor function of lacrimal punctum, eyelid laxity or orbicularis muscle weakness or facial nerve palsy. In the ...

  5. A new storage-ring light source

    Energy Technology Data Exchange (ETDEWEB)

    Chao, Alex [SLAC National Accelerator Lab., Menlo Park, CA (United States)

    2015-06-01

    A recently proposed technique in storage ring accelerators is applied to provide potential high-power sources of photon radiation. The technique is based on the steady-state microbunching (SSMB) mechanism. As examples of this application, one may consider a high-power DUV photon source for research in atomic and molecular physics or a high-power EUV radiation source for industrial lithography. A less challenging proof-of-principle test to produce IR radiation using an existing storage ring is also considered.

  6. 303-K Storage Facility closure plan

    International Nuclear Information System (INIS)

    1993-01-01

    Recyclable scrap uranium with zircaloy-2 and copper silicon alloy, uranium-titanium alloy, beryllium/zircaloy-2 alloy, and zircaloy-2 chips and fines were secured in concrete billets (7.5-gallon containers) in the 303-K Storage Facility, located in the 300 Area. The beryllium/zircaloy-2 alloy and zircaloy-2 chips and fines are designated as mixed waste with the characteristic of ignitability. The concretion process reduced the ignitability of the fines and chips for safe storage and shipment. This process has been discontinued and the 303-K Storage Facility is now undergoing closure as defined in the Resource Conservation and Recovery Act (RCRA) of 1976 and the Washington Administrative Code (WAC) Dangerous Waste Regulations, WAC 173-303-040. This closure plan presents a description of the 303-K Storage Facility, the history of materials and waste managed, and the procedures that will be followed to close the 303-K Storage Facility. The 303-K Storage Facility is located within the 300-FF-3 (source) and 300-FF-5 (groundwater) operable units, as designated in the Hanford Federal Facility Agreement and Consent Order (Tri-Party Agreement) (Ecology et al. 1992). Contamination in the operable units 300-FF-3 and 300-FF-5 is scheduled to be addressed through the Comprehensive Environmental Response, Compensation, and Liability Act (CERCLA) of 1980 remedial action process. Therefore, all soil remedial action at the 304 Facility will be conducted as part of the CERCLA remedial action of operable units 300-FF-3 and 300-FF-5

  7. Water Storage: Quo Vadis?

    Science.gov (United States)

    Smakhtin, V.

    2017-12-01

    Humans stored water - in various forms - for ages, coping with water resources variability, and its extremes - floods and droughts. Storage per capita, and other storage-related indicators, have essentially become one way of reflecting the progress of economic development. Massive investments went into large surface water reservoirs that have become the characteristic feature of the earth's landscapes, bringing both benefits and controversy. As water variability progressively increases with changing climate, globally, on one hand, and the idea of sustainable development receives strong traction, on another - it may be worth the while to comprehensively examine current trends and future prospects for water storage development. The task is surely big, to say the least. The presentation will aim to initiate a structured discussion on this multi-facet issue and identify which aspects and trends of water storage development may be most important in the context of Sustainable Development Goals, Sendai Framework for Disaster Risk Reduction, Paris Agreement on Climate Change, and examine how, where and to what extent water storage planning can be improved. It will cover questions like i) aging of large water storage infrastructure, the current extent of this trend in various geographical regions, and possible impacts on water security and security of nations; ii) improved water storage development planning overall in the context of various water development alternatives and storage options themselves and well as their combinations iii) prospects for another "storage revolution" - speed increase in dam numbers, and where, if at all this is most likely iv) recent events in storage development, e.g. is dam decommissioning a trend that picks pace, or whether some developing economies in Asia can do without going through the period of water storage construction, with alternatives, or suggestions for alleviation of negative impacts v) the role of subsurface storage as an

  8. Energy Storage Economics

    Energy Technology Data Exchange (ETDEWEB)

    Elgqvist, Emma M [National Renewable Energy Laboratory (NREL), Golden, CO (United States)

    2017-09-07

    This presentation provides an overview on energy storage economics including recent market trends, battery terminology and concepts, value streams, challenges, and an example of how photovoltaics and storage can be used to lower demand charges. It also provides an overview of the REopt Lite web tool inputs and outputs.

  9. System for secure storage

    NARCIS (Netherlands)

    2005-01-01

    A system (100) comprising read means (112) for reading content data and control logic data from a storage medium (101), the control logic data being uniquely linked to the storage medium (101), processing means (113-117), for processing the content data and feeding the processed content data to an

  10. Grain Handling and Storage.

    Science.gov (United States)

    Harris, Troy G.; Minor, John

    This text for a secondary- or postecondary-level course in grain handling and storage contains ten chapters. Chapter titles are (1) Introduction to Grain Handling and Storage, (2) Elevator Safety, (3) Grain Grading and Seed Identification, (4) Moisture Control, (5) Insect and Rodent Control, (6) Grain Inventory Control, (7) Elevator Maintenance,…

  11. Wind-energy storage

    Science.gov (United States)

    Gordon, L. H.

    1980-01-01

    Program SIMWEST can model wind energy storage system using any combination of five types of storage: pumped hydro, battery, thermal, flywheel, and pneumatic. Program is tool to aid design of optional system for given application with realistic simulation for further evaluation and verification.

  12. Optical storage networking

    Science.gov (United States)

    Mohr, Ulrich

    2001-11-01

    For efficient business continuance and backup of mission- critical data an inter-site storage network is required. Where traditional telecommunications costs are prohibitive for all but the largest organizations, there is an opportunity for regional carries to deliver an innovative storage service. This session reveals how a combination of optical networking and protocol-aware SAN gateways can provide an extended storage networking platform with the lowest cost of ownership and the highest possible degree of reliability, security and availability. Companies of every size, with mainframe and open-systems environments, can afford to use this integrated service. Three mayor applications are explained; channel extension, Network Attached Storage (NAS), Storage Area Networks (SAN) and how optical networks address the specific requirements. One advantage of DWDM is the ability for protocols such as ESCON, Fibre Channel, ATM and Gigabit Ethernet, to be transported natively and simultaneously across a single fiber pair, and the ability to multiplex many individual fiber pairs over a single pair, thereby reducing fiber cost and recovering fiber pairs already in use. An optical storage network enables a new class of service providers, Storage Service Providers (SSP) aiming to deliver value to the enterprise by managing storage, backup, replication and restoration as an outsourced service.

  13. Cell therapy for pediatric disorders of glia

    DEFF Research Database (Denmark)

    Albuquerque Osório, Maria Joana; Goldman, Steven A.

    2016-01-01

    The childhood disorders of glia comprise a group of diseases that include the pediatric leukodystrophies and lysosomal storage disorders, cerebral palsies and perinatal hypoxic ischemic encephalopathies, and selected neurodevelopmental disorders of glial origin. Essentially, all of these disorders...... (GPCs) and their derivatives, the glial disorders may be uniquely attractive targets for cell-based therapeutic strategies, and the pediatric disorders especially so. As a result, GPCs, which can distribute throughout the neuraxis and give rise to new astrocytes and myelinogenic oligodendrocytes, have...... become of great interest as candidates for the therapeutic restoration of normal glial architecture and function, as well as new myelin, to the pediatric brain....

  14. ERDA's Chemical Energy Storage Program

    Science.gov (United States)

    Swisher, J. H.; Kelley, J. H.

    1977-01-01

    The Chemical Energy Storage Program is described with emphasis on hydrogen storage. Storage techniques considered include pressurized hydrogen gas storage, cryogenic liquid hydrogen storage, storage in hydride compounds, and aromatic-alicyclic hydrogen storage. Some uses of energy storage are suggested. Information on hydrogen production and hydrogen use is also presented. Applications of hydrogen energy systems include storage of hydrogen for utilities load leveling, industrial marketing of hydrogen both as a chemical and as a fuel, natural gas supplementation, vehicular applications, and direct substitution for natural gas.

  15. Genetic variants associated with sleep disorders

    OpenAIRE

    Kripke, Daniel F.; Kline, Lawrence E.; Nievergelt, Caroline M.; Murray, Sarah S.; Shadan, Farhad F.; Dawson, Arthur; Poceta, J. Steven; Cronin, John; Jamil, Shazia M.; Tranah, Gregory J.; Loving, Richard T.; Grizas, Alexandra P.; Hahn, Elizabeth K.

    2015-01-01

    © 2014 The Authors. Objective: The diagnostic boundaries of sleep disorders are under considerable debate. The main sleep disorders are partly heritable therefore, defining heritable pathophysiologic mechanisms could delineate diagnoses and suggest treatment. We collected clinical data and DNA from consenting patients scheduled to undergo clinical polysomnograms, to expand our understanding of the polymorphisms associated with the phenotypes of particular sleep disorders. Methods: Patients at...

  16. Electricity Storage. Technology Brief

    Energy Technology Data Exchange (ETDEWEB)

    Simbolotti, G. [Italian National Agency for New Technologies, Energy and Sustainable Economic Development ENEA, Rome (Italy); Kempener, R. [International Renewable Energy Agency IRENA, Bonn (Germany)

    2012-04-15

    Electricity storage is a key technology for electricity systems with a high share of renewables as it allows electricity to be generated when renewable sources (i.e. wind, sunlight) are available and to be consumed on demand. It is expected that the increasing price of fossil fuels and peak-load electricity and the growing share of renewables will result in electricity storage to grow rapidly and become more cost effective. However, electricity storage is technically challenging because electricity can only be stored after conversion into other forms of energy, and this involves expensive equipment and energy losses. At present, the only commercial storage option is pumped hydro power where surplus electricity (e.g. electricity produced overnight by base-load coal or nuclear power) is used to pump water from a lower to an upper reservoir. The stored energy is then used to produce hydropower during daily high-demand periods. Pumped hydro plants are large-scale storage systems with a typical efficiency between 70% and 80%, which means that a quarter of the energy is lost in the process. Other storage technologies with different characteristics (i.e. storage process and capacity, conversion back to electricity and response to power demand, energy losses and costs) are currently in demonstration or pre-commercial stages and discussed in this brief report: Compressed air energy storage (CAES) systems, Flywheels; Electrical batteries; Supercapacitors; Superconducting magnetic storage; and Thermal energy storage. No single electricity storage technology scores high in all dimensions. The technology of choice often depends on the size of the system, the specific service, the electricity sources and the marginal cost of peak electricity. Pumped hydro currently accounts for 95% of the global storage capacity and still offers a considerable expansion potential but does not suit residential or small-size applications. CAES expansion is limited due to the lack of suitable

  17. Plutonium storage criteria

    Energy Technology Data Exchange (ETDEWEB)

    Chung, D. [Scientech, Inc., Germantown, MD (United States); Ascanio, X. [Dept. of Energy, Germantown, MD (United States)

    1996-05-01

    The Department of Energy has issued a technical standard for long-term (>50 years) storage and will soon issue a criteria document for interim (<20 years) storage of plutonium materials. The long-term technical standard, {open_quotes}Criteria for Safe Storage of Plutonium Metals and Oxides,{close_quotes} addresses the requirements for storing metals and oxides with greater than 50 wt % plutonium. It calls for a standardized package that meets both off-site transportation requirements, as well as remote handling requirements from future storage facilities. The interim criteria document, {open_quotes}Criteria for Interim Safe Storage of Plutonium-Bearing Solid Materials{close_quotes}, addresses requirements for storing materials with less than 50 wt% plutonium. The interim criteria document assumes the materials will be stored on existing sites, and existing facilities and equipment will be used for repackaging to improve the margin of safety.

  18. Compact nuclear fuel storage

    International Nuclear Information System (INIS)

    Kiselev, V.V.; Churakov, Yu.A.; Danchenko, Yu.V.; Bylkin, B.K.; Tsvetkov, S.V.

    1983-01-01

    Different constructions of racks for compact storage of spent fuel assemblies (FA) in ''coolin''g pools (CP) of NPPs with the BWR and PWR type reactors are described. Problems concerning nuclear and radiation safety and provision of necessary thermal conditions arising in such rack design are discussed. It is concluded that the problem of prolonged fuel storage at NPPs became Very actual for many countries because of retapdation of the rates of fuel reprocessing centers building. Application of compact storage racks is a promising solution of the problem of intermediate FA storage at NPPs. Such racks of stainless boron steel and with neutron absorbers in the from of boron carbide panels enable to increase the capacity of the present CP 2-2.6 times, and the period of FA storage in them up to 5-10 years

  19. Mesoporous nanocrystalline film architecture for capacitive storage devices

    Science.gov (United States)

    Dunn, Bruce S.; Tolbert, Sarah H.; Wang, John; Brezesinski, Torsten; Gruner, George

    2017-05-16

    A mesoporous, nanocrystalline, metal oxide construct particularly suited for capacitive energy storage that has an architecture with short diffusion path lengths and large surface areas and a method for production are provided. Energy density is substantially increased without compromising the capacitive charge storage kinetics and electrode demonstrates long term cycling stability. Charge storage devices with electrodes using the construct can use three different charge storage mechanisms immersed in an electrolyte: (1) cations can be stored in a thin double layer at the electrode/electrolyte interface (non-faradaic mechanism); (2) cations can interact with the bulk of an electroactive material which then undergoes a redox reaction or phase change, as in conventional batteries (faradaic mechanism); or (3) cations can electrochemically adsorb onto the surface of a material through charge transfer processes (faradaic mechanism).

  20. Toward flexible polymer and paper-based energy storage devices

    Energy Technology Data Exchange (ETDEWEB)

    Nyholm, Leif [Department of Materials Chemistry, The Aangstroem Laboratory, Uppsala University, Box 538, SE-751 21 Uppsala (Sweden); Nystroem, Gustav; Mihranyan, Albert; Stroemme, Maria [Nanotechnology and Functional Materials, Department of Engineering Sciences, The Aangstroem Laboratory, Uppsala University, Box 534, SE-751 21 Uppsala (Sweden)

    2011-09-01

    All-polymer and paper-based energy storage devices have significant inherent advantages in comparison with many currently employed batteries and supercapacitors regarding environmental friendliness, flexibility, cost and versatility. The research within this field is currently undergoing an exciting development as new polymers, composites and paper-based devices are being developed. In this report, we review recent progress concerning the development of flexible energy storage devices based on electronically conducting polymers and cellulose containing composites with particular emphasis on paper-based batteries and supercapacitors. We discuss recent progress in the development of the most commonly used electronically conducting polymers used in flexible device prototypes, the advantages and disadvantages of this type of energy storage devices, as well as the two main approaches used in the manufacturing of paper-based charge storage devices. (Copyright copyright 2011 WILEY-VCH Verlag GmbH and Co. KGaA, Weinheim)

  1. Electricity storage using a thermal storage scheme

    Energy Technology Data Exchange (ETDEWEB)

    White, Alexander, E-mail: ajw36@cam.ac.uk [Hopkinson Laboratory, Cambridge University Engineering Department, Trumpington Street, Cambridge. CB2 1PZ (United Kingdom)

    2015-01-22

    The increasing use of renewable energy technologies for electricity generation, many of which have an unpredictably intermittent nature, will inevitably lead to a greater demand for large-scale electricity storage schemes. For example, the expanding fraction of electricity produced by wind turbines will require either backup or storage capacity to cover extended periods of wind lull. This paper describes a recently proposed storage scheme, referred to here as Pumped Thermal Storage (PTS), and which is based on “sensible heat” storage in large thermal reservoirs. During the charging phase, the system effectively operates as a high temperature-ratio heat pump, extracting heat from a cold reservoir and delivering heat to a hot one. In the discharge phase the processes are reversed and it operates as a heat engine. The round-trip efficiency is limited only by process irreversibilities (as opposed to Second Law limitations on the coefficient of performance and the thermal efficiency of the heat pump and heat engine respectively). PTS is currently being developed in both France and England. In both cases, the schemes operate on the Joule-Brayton (gas turbine) cycle, using argon as the working fluid. However, the French scheme proposes the use of turbomachinery for compression and expansion, whereas for that being developed in England reciprocating devices are proposed. The current paper focuses on the impact of the various process irreversibilities on the thermodynamic round-trip efficiency of the scheme. Consideration is given to compression and expansion losses and pressure losses (in pipe-work, valves and thermal reservoirs); heat transfer related irreversibility in the thermal reservoirs is discussed but not included in the analysis. Results are presented demonstrating how the various loss parameters and operating conditions influence the overall performance.

  2. Aspirin in patients undergoing noncardiac surgery

    DEFF Research Database (Denmark)

    Devereaux, P J; Mrkobrada, Marko; Sessler, Daniel I

    2014-01-01

    BACKGROUND: There is substantial variability in the perioperative administration of aspirin in patients undergoing noncardiac surgery, both among patients who are already on an aspirin regimen and among those who are not. METHODS: Using a 2-by-2 factorial trial design, we randomly assigned 10......,010 patients who were preparing to undergo noncardiac surgery and were at risk for vascular complications to receive aspirin or placebo and clonidine or placebo. The results of the aspirin trial are reported here. The patients were stratified according to whether they had not been taking aspirin before...... the study (initiation stratum, with 5628 patients) or they were already on an aspirin regimen (continuation stratum, with 4382 patients). Patients started taking aspirin (at a dose of 200 mg) or placebo just before surgery and continued it daily (at a dose of 100 mg) for 30 days in the initiation stratum...

  3. Efficacy and safety of tacrolimus treatment for rheumatoid arthritis patients undergoing hemodialysis.

    Science.gov (United States)

    Yamashita, Misuzu; Natsumeda, Masamitsu; Takasugi, Koji; Ueno, Akiko; Ezawa, Kayo; Ezawa, Kazuhiko

    2008-01-01

    Rheumatoid arthritis (RA) is an autoimmune disorder characterized by progressive joint destruction that requires aggressive treatment using appropriate disease-modifying antirheumatic drugs (DMARDs). RA patients with renal failure, however, are intolerant to most DMARDs due to the potential toxicity. In Japan, tacrolimus was approved for the treatment of RA in 2005. Based on its pharmacokinetics, tacrolimus may be administered to the patients undergoing hemodialysis. We report two cases of RA patients on hemodialysis treated effectively and safely with tacrolimus.

  4. Liver fibrosis in type I Gaucher disease: magnetic resonance imaging, transient elastography and parameters of iron storage.

    Directory of Open Access Journals (Sweden)

    Anneloes E Bohte

    Full Text Available Long term liver-related complications of type-1 Gaucher disease (GD, a lysosomal storage disorder, include fibrosis and an increased incidence of hepatocellular carcinoma. Splenectomy has been implicated as a risk factor for the development of liver pathology in GD. High ferritin concentrations are a feature of GD and iron storage in Gaucher cells has been described, but iron storage in the liver in relation to liver fibrosis has not been studied. Alternatively, iron storage in GD may be the result of iron supplementation therapy or regular blood transfusions in patients with severe cytopenia. In this pilot study, comprising 14 type-1 GD patients (7 splenectomized, 7 non-splenectomized and 7 healthy controls, we demonstrate that liver stiffness values, measured by Transient Elastography and MR-Elastography, are significantly higher in splenectomized GD patients when compared with non-splenectomized GD patients (p = 0.03 and p = 0.01, respectively. Liver iron concentration was elevated (>60±30 µmol/g in 4 GD patients of whom 3 were splenectomized. No relationship was found between liver stiffness and liver iron concentration. HFE gene mutations were more frequent in splenectomized (6/7 than in non-splenectomized (2/7 participants (p = 0.10. Liver disease appeared more advanced in splenectomized than in non-splenectomized patients. We hypothesize a relationship with excessive hepatic iron accumulation in splenectomized patients. We recommend that all splenectomized patients, especially those with evidence of substantial liver fibrosis undergo regular screening for HCC, according to current guidelines.

  5. Platelet activation in outpatients undergoing esophagogastroduodenoscopy

    International Nuclear Information System (INIS)

    Sagripanti, A.; Polloni, A.; Materazzi, F.; Ferdeghini, M.; Pinori, E.; Bianchi, R.

    1989-01-01

    To evaluate the influence of emotional stress on platelet function mesured by radioimmunoassay in plasma two platelet factor 4, in a series of outpatients undergoing esophagogastroduodenoscopy for upper digestive complaints has been measured. The plasma levels of β-thromboglobulin and platelet factor 4, determined just before the instrumental examination, were significantly more elevated as compared to basal values, checked a week later. These results provide evidence of enhanced in vivo platelet release reaction during emotional stress

  6. Ultrafine hydrogen storage powders

    Science.gov (United States)

    Anderson, Iver E.; Ellis, Timothy W.; Pecharsky, Vitalij K.; Ting, Jason; Terpstra, Robert; Bowman, Robert C.; Witham, Charles K.; Fultz, Brent T.; Bugga, Ratnakumar V.

    2000-06-13

    A method of making hydrogen storage powder resistant to fracture in service involves forming a melt having the appropriate composition for the hydrogen storage material, such, for example, LaNi.sub.5 and other AB.sub.5 type materials and AB.sub.5+x materials, where x is from about -2.5 to about +2.5, including x=0, and the melt is gas atomized under conditions of melt temperature and atomizing gas pressure to form generally spherical powder particles. The hydrogen storage powder exhibits improved chemcial homogeneity as a result of rapid solidfication from the melt and small particle size that is more resistant to microcracking during hydrogen absorption/desorption cycling. A hydrogen storage component, such as an electrode for a battery or electrochemical fuel cell, made from the gas atomized hydrogen storage material is resistant to hydrogen degradation upon hydrogen absorption/desorption that occurs for example, during charging/discharging of a battery. Such hydrogen storage components can be made by consolidating and optionally sintering the gas atomized hydrogen storage powder or alternately by shaping the gas atomized powder and a suitable binder to a desired configuration in a mold or die.

  7. Storage of radioactive wastes

    International Nuclear Information System (INIS)

    1992-07-01

    Even if the best waste minimization measures are undertaken throughout radioisotope production or usage, significant radioactive wastes arise to make management measures essential. For developing countries with low isotope usage and little or no generation of nuclear materials, it may be possible to handle the generated waste by simply practicing decay storage for several half-lives of the radionuclides involved, followed by discharge or disposal without further processing. For those countries with much larger facilities, longer lived isotopes are produced and used. In this situation, storage is used not only for decay storage but also for in-process retention steps and for the key stage of interim storage of conditioned wastes pending final disposal. The report will serve as a technical manual providing reference material and direct step-by-step know-how to staff in radioisotope user establishments and research centres in the developing Member States without nuclear power generation. Considerations are limited to the simpler storage facilities. The restricted quantities and low activity associated with the relevant wastes will generally permit contact-handling and avoid the need for shielding requirements in the storage facilities or equipment used for handling. A small quantity of wastes from some radioisotope production cells and from reactor cooling water treatment may contain sufficient short lived activity from activated corrosion products to require some separate decay storage before contact-handling is suitable. 16 refs, 12 figs, 8 tabs

  8. Aflatoxins & Safe Storage

    Directory of Open Access Journals (Sweden)

    Philippe eVillers

    2014-04-01

    Full Text Available The paper examines both field experience and research on the prevention of the exponential growth of aflatoxins during multi-month post harvest storage in hot, humid countries. The approach described is the application of modern safe storage methods using flexible, Ultra Hermetic™ structures that create an unbreatheable atmosphere through insect and microorganism respiration alone, without use of chemicals, fumigants, or pumps. Laboratory and field data are cited and specific examples are given describing the uses of Ultra Hermetic storage to prevent the growth of aflatoxins with their significant public health consequences. Also discussed is the presently limited quantitative information on the relative occurrence of excessive levels of aflatoxin (>20 ppb before versus after multi-month storage of such crops as maize, rice and peanuts when under high humidity, high temperature conditions and, consequently, the need for further research to determine the frequency at which excessive aflatoxin levels are reached in the field versus after months of post-harvest storage. The significant work being done to reduce aflatoxin levels in the field is mentioned, as well as its probable implications on post harvest storage. Also described is why, with some crops such as peanuts, using Ultra Hermetic storage may require injection of carbon dioxide or use of an oxygen absorber as an accelerant. The case of peanuts is discussed and experimental data is described.

  9. LRRK2 mediated Rab8a phosphorylation promotes lipid storage.

    Science.gov (United States)

    Yu, Miao; Arshad, Muhammad; Wang, Wenmin; Zhao, Dongyu; Xu, Li; Zhou, Linkang

    2018-02-27

    Several mutations in leucine rich repeat kinase 2 (LRRK2) gene have been associated with pathogenesis of Parkinson's disease (PD), a neurodegenerative disorder marked by resting tremors, and rigidity, leading to Postural instability. It has been revealed that mutations that lead to an increase of kinase activity of LRRK2 protein are significantly associated with PD pathogenesis. Recent studies have shown that some Rab GTPases, especially Rab8, serve as substrates of LRRK2 and undergo phosphorylation in its switch II domain upon interaction. Current study was performed in order to find out the effects of the phosphorylation of Rab8 and its mutants on lipid metabolism and lipid droplets growth. The phosphorylation status of Rab8a was checked by phos-tag gel. Point mutant construct were generated to investigate the function of Rab8a. 3T3L1 cells were transfected with indicated plasmids and the lipid droplets were stained with Bodipy. Fluorescent microscopy experiments were performed to examine the sizes of lipid droplets. The interactions between Rab8a and Optineurin were determined by immunoprecipitation and western blot. Our assays demonstrated that Rab8a was phosphorylated by mutated LRRK2 that exhibits high kinase activity. Phosphorylation of Rab8a on amino acid residue T72 promoted the formation of large lipid droplets. T72D mutant of Rab8a had higher activity to promote the formation of large lipid droplets compared with wild type Rab8a, with increase in average diameter of lipid droplets from 2.10 μm to 2.46 μm. Moreover, phosphorylation of Rab8a weakened the interaction with its effector Optineurin. Y1699C mutated LRRK2 was able to phosphorylate Rab8a and phosphorylation of Rab8a on site 72 plays important role in the fusion and enlargement of lipid droplets. Taken together, our study suggests an indirect relationship between enhanced lipid storage capacity and PD pathogenesis.

  10. Spent-fuel-storage alternatives

    International Nuclear Information System (INIS)

    1980-01-01

    The Spent Fuel Storage Alternatives meeting was a technical forum in which 37 experts from 12 states discussed storage alternatives that are available or are under development. The subject matter was divided into the following five areas: techniques for increasing fuel storage density; dry storage of spent fuel; fuel characterization and conditioning; fuel storage operating experience; and storage and transport economics. Nineteen of the 21 papers which were presented at this meeting are included in this Proceedings. These have been abstracted and indexed

  11. Magnox waste storage complex

    International Nuclear Information System (INIS)

    Anon.

    1990-01-01

    This article looks at the design and construction of British Nuclear Fuel Limited's (BNFL) Magnox waste storage complex by Costain Engineering Limited. Magnox swarf from fuel decanning is stored underwater in specially designed silos. Gas processing capabilities from Costain Engineering Limited and the experience of BNFL combined in this project to provide the necessary problem-solving skills necessary for this waste storage upgrading and extension project. A retrofitted inerting facility was fitted to an existing building and a new storage extension was fitted, both without interrupting reprocessing operations at Sellafield. (UK)

  12. Tiered Storage For LHC

    CERN Multimedia

    CERN. Geneva; Hanushevsky, Andrew

    2012-01-01

    For more than a year, the ATLAS Western Tier 2 (WT2) at SLAC National Accelerator has been successfully operating a two tiered storage system based on Xrootd's flexible cross-cluster data placement framework, the File Residency Manager. The architecture allows WT2 to provide both, high performance storage at the higher tier to ATLAS analysis jobs, as well as large, low cost disk capacity at the lower tier. Data automatically moves between the two storage tiers based on the needs of analysis jobs and is completely transparent to the jobs.

  13. Next generation storage facility

    International Nuclear Information System (INIS)

    Schlesser, J.A.

    1994-01-01

    With diminishing requirements for plutonium, a substantial quantity of this material requires special handling and ultimately, long-term storage. To meet this objective, we at Los Alamos, have been involved in the design of a storage facility with the goal of providing storage capabilities for this and other nuclear materials. This paper presents preliminary basic design data, not for the structure and physical plant, but for the container and arrays which might be configured within the facility, with strong emphasis on criticality safety features

  14. Monitored retrievable storage design

    International Nuclear Information System (INIS)

    Woods, W.D.

    1985-01-01

    The Nuclear Waste Policy Act of 1982 (NWPA) established a national policy for the safe storage and disposal of spent nuclear fuel and high-level radioactive waste. The NWPA requires that DOE... ''submit a proposal to Congress on the need for and feasibility of one or more Monitored Retrievable Storage (MRS) Facilities''... In subsequent evaluations of the commercial nuclear waste management system, DOE has identified important advantages in providing an MRS Facility as an integral part of the total system. The integral MRS Facility serves as an independent, centralized spent nuclear fuel and high-level waste handling and packaging facility with a safe temporary storage capacity

  15. Storage-ring FEL for the vuv

    International Nuclear Information System (INIS)

    Peterson, J.M.; Bisognano, J.J.; Garren, A.A.; Halbach, K.; Kim, K.J.; Sah, R.C.

    1984-09-01

    A free-electron laser for the vuv operating in a storage ring requires an electron beam of high density and low energy spread and a short wavelength, narrow-gap undulator. These conditions tend to produce longitudinal and transverse beam instabilities, excessive beam growth through multiple intrabeam scattering, and a short gas-scattering lifetime. Passing the beam only occasionally through the undulator in a by-pass straight section, as proposed by Murphy and Pellegrini, allows operation in a high-gain, single-pass mode and a long gas-scattering lifetime. Several storage ring designs have been considered to see how best to satisfy the several requirements. Each features a by-pass, a low-emittance lattice, and built-in wigglers for enhanced damping to counteract the intra-beam scattering. 15 references, 3 figures, 2 tables

  16. The significance of peripartum fever in women undergoing vaginal deliveries.

    Science.gov (United States)

    Bensal, Adi; Weintraub, Adi Y; Levy, Amalia; Holcberg, Gershon; Sheiner, Eyal

    2008-10-01

    We investigated whether patients undergoing vaginal delivery who developed peripartum fever (PPF) had increased rates of other gestational complications. A retrospective study was undertaken comparing pregnancy complications of patients who developed PPF with those who did not. A multivariable logistic regression model was constructed to control for confounders. To avoid ascertainment bias, the year of birth was included in the model. Women who underwent cesarean delivery and those with multiple pregnancies were excluded from the study. During the study period, there were 169,738 singleton vaginal deliveries, and 0.4% of the women suffered from PPF. Hypertensive disorders, induction of labor, dystocia of labor in the second stage, suspected fetal distress, meconium-stained amniotic fluid, postpartum hemorrhage, manual lysis of a retained placenta, and revision of the uterine cavity and cervix were found to be independently associated with PPF by multivariable analysis. Year of birth was found to be a risk factor for fever. Apgar scores lower than 7 at 1 but not 5 minutes were significantly higher in the PPF group. Perinatal mortality rates were significantly higher among women with PPF (6.7% versus 1.3%, odds ratio [OR] = 5.4; 95% confidence interval [CI] 3.9 to 7.3; P < 0.001). Using another multivariable analysis, with perinatal mortality as the outcome variable, PPF was found as an independent risk factor for perinatal mortality (OR = 2.9; 95% CI 1.9 to 4.6; P < 0.001). PPF in women undergoing vaginal deliveries is associated with adverse perinatal outcomes and specifically is an independent risk factor for perinatal mortality.

  17. Sleep-related movement disorders.

    Science.gov (United States)

    Merlino, Giovanni; Gigli, Gian Luigi

    2012-06-01

    Several movement disorders may occur during nocturnal rest disrupting sleep. A part of these complaints is characterized by relatively simple, non-purposeful and usually stereotyped movements. The last version of the International Classification of Sleep Disorders includes these clinical conditions (i.e. restless legs syndrome, periodic limb movement disorder, sleep-related leg cramps, sleep-related bruxism and sleep-related rhythmic movement disorder) under the category entitled sleep-related movement disorders. Moreover, apparently physiological movements (e.g. alternating leg muscle activation and excessive hypnic fragmentary myoclonus) can show a high frequency and severity impairing sleep quality. Clinical and, in specific cases, neurophysiological assessments are required to detect the presence of nocturnal movement complaints. Patients reporting poor sleep due to these abnormal movements should undergo non-pharmacological or pharmacological treatments.

  18. Storage of strawberry pollen

    Directory of Open Access Journals (Sweden)

    Rafet Aslantaş

    2013-12-01

    Full Text Available This study was carried out to determine storage ability of strawberry pollen at different temperatures for three different strawberry cultivars 'Aliso', 'Brio', and 'Cruz'. Strawberry pollen was stored at room temperature (22 ±2°C, +4°C, -4°C and -18°C in stabile humidity conditions. Strawberry poIlen was germinated using the hanging drop method in a 20% sucrose solution. Pollen germination rate increased because of low temperature storage. Pollen stored at room temperature and +4°C, -4°C, and -18°C was kept for 8 months, about one year, and 20 months, respectively. Pollen germination rates decreased as the length of storage period increased. The reaction of all cultivars tested on the duration and temperature of storage was similar.

  19. Hydrogen storage compositions

    Science.gov (United States)

    Li, Wen; Vajo, John J.; Cumberland, Robert W.; Liu, Ping

    2011-04-19

    Compositions for hydrogen storage and methods of making such compositions employ an alloy that exhibits reversible formation/deformation of BH.sub.4.sup.- anions. The composition includes a ternary alloy including magnesium, boron and a metal and a metal hydride. The ternary alloy and the metal hydride are present in an amount sufficient to render the composition capable of hydrogen storage. The molar ratio of the metal to magnesium and boron in the alloy is such that the alloy exhibits reversible formation/deformation of BH.sub.4.sup.- anions. The hydrogen storage composition is prepared by combining magnesium, boron and a metal to prepare a ternary alloy and combining the ternary alloy with a metal hydride to form the hydrogen storage composition.

  20. Memory mass storage

    CERN Document Server

    Campardo, Giovanni; Iaculo, Massimo

    2011-01-01

    Covering all the fundamental storage technologies such as semiconductor, magnetic, optical and uncommon, this volume details their core characteristics. In addition, it includes an overview of the 'biological memory' of the human brain and its organization.

  1. Cryptography from noisy storage.

    Science.gov (United States)

    Wehner, Stephanie; Schaffner, Christian; Terhal, Barbara M

    2008-06-06

    We show how to implement cryptographic primitives based on the realistic assumption that quantum storage of qubits is noisy. We thereby consider individual-storage attacks; i.e., the dishonest party attempts to store each incoming qubit separately. Our model is similar to the model of bounded-quantum storage; however, we consider an explicit noise model inspired by present-day technology. To illustrate the power of this new model, we show that a protocol for oblivious transfer is secure for any amount of quantum-storage noise, as long as honest players can perform perfect quantum operations. Our model also allows us to show the security of protocols that cope with noise in the operations of the honest players and achieve more advanced tasks such as secure identification.

  2. Center for Hydrogen Storage.

    Science.gov (United States)

    2013-06-01

    The main goals of this project were to (1) Establish a Center for Hydrogen Storage Research at Delaware State University for the preparation and characterization of selected complex metal hydrides and the determination their suitability for hydrogen ...

  3. Hydrogen storage using borohydrides

    International Nuclear Information System (INIS)

    Bernard BONNETOT; Laetitia LAVERSENNE

    2006-01-01

    The possibilities of hydrogen storage using borohydrides are presented and discussed specially in regard of the recoverable hydrogen amount and related to the recovering conditions. A rapid analysis of storage possibilities is proposed taking in account the two main ways for hydrogen evolution: the dehydrogenation obtained through thermal decomposition or the hydrolysis of solids or solutions. The recoverable hydrogen is related to the dehydrogenation conditions and the real hydrogen useful percentage is determined for each case of use. The high temperature required for dehydrogenation even when using catalyzed compounds lead to poor outlooks for this storage way. The hydrolysis conditions direct the chemical yield of the water consuming, and this must be related to the experimental conditions which rule the storage capacity of the 'fuel' derived from the borohydride. (authors)

  4. Wet storage integrity update

    International Nuclear Information System (INIS)

    Bailey, W.J.; Johnson, A.B. Jr.

    1983-09-01

    This report includes information from various studies performed under the Wet Storage Task of the Spent Fuel Integrity Project of the Commercial Spent Fuel Management (CSFM) Program at Pacific Northwest Laboratory. An overview of recent developments in the technology of wet storage of spent water reactor fuel is presented. Licensee Event Reports pertaining to spent fuel pools and the associated performance of spent fuel and storage components during wet storage are discussed. The current status of fuel that was examined under the CSFM Program is described. Assessments of the effect of boric acid in spent fuel pool water on the corrosion and stress corrosion cracking of stainless steel and the stress corrosion cracking of stainless steel piping containing stagnant water at spent fuel pools are discussed. A list of pertinent publications is included. 84 references, 21 figures, 11 tables

  5. Spent fuel storage requirements

    International Nuclear Information System (INIS)

    Fletcher, J.

    1982-06-01

    Spent fuel storage requirements, as projected through the year 2000 for U.S. LWRs, were calculated using information supplied by the utilities reflecting plant status as of December 31, 1981. Projections through the year 2000 combined fuel discharge projections of the utilities with the assumed discharges of typical reactors required to meet the nuclear capacity of 165 GWe projected by the Energy Information Administration (EIA) for the year 2000. Three cases were developed and are summarized. A reference case, or maximum at-reactor (AR) capacity case, assumes that all reactor storage pools are increased to their maximum capacities as estimated by the utilities for spent fuel storage utilizing currently licensed technologies. The reference case assumes no transshipments between pools except as currently licensed by the Nuclear Regulatory Commission (NRC). This case identifies an initial requirement for 13 MTU of additional storage in 1984, and a cumulative requirement for 14,490 MTU additional storage in the year 2000. The reference case is bounded by two alternative cases. One, a current capacity case, assumes that only those pool storage capacity increases currently planned by the operating utilities will occur. The second, or maximum capacity with transshipment case, assumes maximum development of pool storage capacity as described above and also assumes no constraints on transshipment of spent fuel among pools of reactors of like type (BWR, PWR) within a given utility. In all cases, a full core discharge capability (full core reserve or FCR) is assumed to be maintained for each reactor, except that only one FCR is maintained when two reactors share a common pool. For the current AR capacity case the indicated storage requirements in the year 2000 are indicated to be 18,190 MTU; for the maximum capacity with transshipment case they are 11,320 MTU

  6. Flywheel energy storage; Schwungmassenspeicher

    Energy Technology Data Exchange (ETDEWEB)

    Bornemann, H.J. [Forschungszentrum Karlsruhe GmbH Technik und Umwelt (Germany)

    1996-12-31

    Energy storages may be chemical systems such as batteries, thermal systems such as hot-water tanks, electromagnetic systems such as capacitors and coils, or mechanical systems such as pumped storage power systems or flywheel energy storages. In flywheel energy storages the energy is stored in the centrifugal mass in the form of kinetic energy. This energy can be converted to electricity via a motor/generator unit and made available to the consumer. The introduction of magnetic bearings has greatly enhanced the potential of flywheel energy storages. As there is no contact between the moving parts of magnetic bearings, this technology provides a means of circumventing the engineering and operational problems involved in the we of conventional bearings (ball, roller, plain, and gas bearings). The advantages of modern flywheel energy storages over conventional accumulators are an at least thousandfold longer service life, low losses during long-time storage, greater power output in the case of short-time storage, and commendable environmental benignity. (orig./HW) [Deutsch] Als Enegiespeicher kommen chemische Systeme, z.B. Batterien, thermische Systeme, z.B. Warmwassertanks, elektromagnetische Systeme, z.B. Kondensatoren und Spulen, sowie mechanische Systeme, z.B. Pumpspeicherwerke und Schwungmassenspeicher in Frage. In einem Schwungmassenspeicher wird Energie in Form von kinetischer Energie in der Schwungmasse gespeichert. Ueber eine Moter/Generator Einheit wird diese Energie in elektrischen Strom umgewandelt und dem Verbraucher zugefuehrt. Mit der Einfuehrung von magnetischen Lagern konnte die Leistungsfaehigkeit von Schwungmassenspeichern erheblich gesteigert werden. Da in einem Magnetlager keine Beruehrung zwischen sich bewegenden Teilen besteht, wird ein Grossteil der mit dem Einsatz konventioneller Lager (Kugel- und Rollenlager, Gleitlager und Gaslager) verbundenen ingenieurtechnischen und betriebstechnischen Probleme vermieden. Die Vorteile von modernen

  7. Optimization of Munitions Storage.

    Science.gov (United States)

    1979-12-01

    zones of land around each facility place a significant economic cost on the storage of munitions (Schreyer, 1970: 1). Munitions storage is a subject...ADDTOT, BTOTAL 353 REAL MPH,MW ,MPD, MPU ,4,MPNEW,MCD 354 IF (SW2.NE.0) GO TO 1 355 SW2 - 1 356 WRITE (6,2) 357 GO TO 3 358 1 IF (REC.EQ.0) GO TO 4 359 IF

  8. Spent fuel storage

    International Nuclear Information System (INIS)

    Huppert

    1976-01-01

    To begin with, the author explains the reasons for intermediate storage of fuel elements in nuclear power stations and in a reprocessing plant and gives the temperature and radioactivity curves of LWR fuel elements after removal from the reactor. This is followed by a description of the facilities for fuel element storage in a reprocessing plant and of their functions. Futher topics are criticality and activity control, the problem of cooling time and safety systems. (HR) [de

  9. Analog storage integrated circuit

    Science.gov (United States)

    Walker, J.T.; Larsen, R.S.; Shapiro, S.L.

    1989-03-07

    A high speed data storage array is defined utilizing a unique cell design for high speed sampling of a rapidly changing signal. Each cell of the array includes two input gates between the signal input and a storage capacitor. The gates are controlled by a high speed row clock and low speed column clock so that the instantaneous analog value of the signal is only sampled and stored by each cell on coincidence of the two clocks. 6 figs.

  10. Annual Report: Carbon Storage

    Energy Technology Data Exchange (ETDEWEB)

    Strazisar, Brian [National Energy Technology Lab. (NETL), Pittsburgh, PA, (United States); Guthrie, George [National Energy Technology Lab. (NETL), Pittsburgh, PA, (United States)

    2012-09-30

    Activities include laboratory experimentation, field work, and numerical modeling. The work is divided into five theme areas (or first level tasks) that each address a key research need: Flow Properties of Reservoirs and Seals, Fundamental Processes and Properties, Estimates of Storage Potential, Verifying Storage Performance, and Geospatial Data Resources. The project also includes a project management effort which coordinates the activities of all the research teams.

  11. Eating Disorders

    Science.gov (United States)

    ... of-control eating Women are more likely than men to have eating disorders. They usually start in the teenage years and often occur along with depression, anxiety disorders, and substance abuse. Eating disorders can ...

  12. Eating Disorders

    Science.gov (United States)

    ... Application Process Managing Grants Clinical Research Training Small Business Research Labs at NIMH Labs at NIMH Home Research ... About Eating Disorders More Publications About Eating Disorders Research Results PubMed: Journal Articles about Eating Disorders Contact Us The National ...

  13. Personality Disorders

    Science.gov (United States)

    ... Disorders in Adults Data Sources Share Personality Disorders Definitions Personality disorders represent “an enduring pattern of inner ... MSC 9663 Bethesda, MD 20892-9663 Follow Us Facebook Twitter YouTube Google Plus NIMH Newsletter NIMH RSS ...

  14. Schizoaffective Disorder

    Science.gov (United States)

    ... variations in brain chemistry and structure. Risk factors Factors that increase the risk of developing schizoaffective disorder include: Having a close blood relative who has schizoaffective disorder, schizophrenia or bipolar disorder Stressful events that trigger symptoms ...

  15. Storage of nuclear wastes

    International Nuclear Information System (INIS)

    Ahlstroem, P.E.

    1988-01-01

    The Swedish system of handling and storage of nuclear wastes is well-developed. Existing plants and systems provide great freedom of action and flexibility regarding future development and decisions of ultimate storage of the spent fuel. The interim storage in CLAB - Central interim storage facility for spent nuclear fuel - could continue without any safety related problems for more than 40 years. In practice the choice of ultimate treatment system is not locked until the encapsulation of the fuel starts. At the same time it is of importance that the generation benefiting by the nuclear power production also be responsible for the development of the ultimate storage system and not unnecessarily postpones important decisions. The ultimate storage system for spent fuel could and should be developed within existing schedule. At the same time is should be worked out to provide coming generations with possibilities to do the type of supervision they like without maintenance and supervision requiring to become a prerequisite for a safe function. (O.S.)

  16. Energy Storage Systems

    Science.gov (United States)

    Elliott, David

    2017-07-01

    As renewable energy use expands there will be a need to develop ways to balance its variability. Storage is one of the options. Presently the main emphasis is for systems storing electrical power in advanced batteries (many of them derivatives of parallel developments in the electric vehicle field), as well as via liquid air storage, compressed air storage, super-capacitors and flywheels, and, the leader so far, pumped hydro reservoirs. In addition, new systems are emerging for hydrogen generation and storage, feeding fuel cell power production. Heat (and cold) is also a storage medium and some systems exploit thermal effects as part of wider energy management activity. Some of the more exotic ones even try to use gravity on a large scale. This short book looks at all the options, their potentials and their limits. There are no clear winners, with some being suited to short-term balancing and others to longer-term storage. The eventual mix adopted will be shaped by the pattern of development of other balancing measures, including smart-grid demand management and super-grid imports and exports.

  17. Superconducting magnetic energy storage

    International Nuclear Information System (INIS)

    Rogers, J.D.; Boenig, H.J.

    1978-01-01

    Superconducting inductors provide a compact and efficient means of storing electrical energy without an intermediate conversion process. Energy storage inductors are under development for diurnal load leveling and transmission line stabilization in electric utility systems and for driving magnetic confinement and plasma heating coils in fusion energy systems. Fluctuating electric power demands force the electric utility industry to have more installed generating capacity than the average load requires. Energy storage can increase the utilization of base-load fossil and nuclear power plants for electric utilities. Superconducting magnetic energy storage (SMES) systems, which will store and deliver electrical energy for load leveling, peak shaving, and the stabilization of electric utility networks are being developed. In the fusion area, inductive energy transfer and storage is also being developed by LASL. Both 1-ms fast-discharge theta-pinch and 1-to-2-s slow tokamak energy transfer systems have been demonstrated. The major components and the method of operation of an SMES unit are described, and potential applications of different size SMES systems in electric power grids are presented. Results are given for a 1-GWh reference design load-leveling unit, for a 30-MJ coil proposed stabilization unit, and for tests with a small-scale, 100-kJ magnetic energy storage system. The results of the fusion energy storage and transfer tests are also presented. The common technology base for the systems is discussed

  18. Secure Storage Architectures

    Energy Technology Data Exchange (ETDEWEB)

    Aderholdt, Ferrol [Tennessee Technological University; Caldwell, Blake A [ORNL; Hicks, Susan Elaine [ORNL; Koch, Scott M [ORNL; Naughton, III, Thomas J [ORNL; Pogge, James R [Tennessee Technological University; Scott, Stephen L [Tennessee Technological University; Shipman, Galen M [ORNL; Sorrillo, Lawrence [ORNL

    2015-01-01

    The purpose of this report is to clarify the challenges associated with storage for secure enclaves. The major focus areas for the report are: - review of relevant parallel filesystem technologies to identify assets and gaps; - review of filesystem isolation/protection mechanisms, to include native filesystem capabilities and auxiliary/layered techniques; - definition of storage architectures that can be used for customizable compute enclaves (i.e., clarification of use-cases that must be supported for shared storage scenarios); - investigate vendor products related to secure storage. This study provides technical details on the storage and filesystem used for HPC with particular attention on elements that contribute to creating secure storage. We outline the pieces for a a shared storage architecture that balances protection and performance by leveraging the isolation capabilities available in filesystems and virtualization technologies to maintain the integrity of the data. Key Points: There are a few existing and in-progress protection features in Lustre related to secure storage, which are discussed in (Chapter 3.1). These include authentication capabilities like GSSAPI/Kerberos and the in-progress work for GSSAPI/Host-keys. The GPFS filesystem provides native support for encryption, which is not directly available in Lustre. Additionally, GPFS includes authentication/authorization mechanisms for inter-cluster sharing of filesystems (Chapter 3.2). The limitations of key importance for secure storage/filesystems are: (i) restricting sub-tree mounts for parallel filesystem (which is not directly supported in Lustre or GPFS), and (ii) segregation of hosts on the storage network and practical complications with dynamic additions to the storage network, e.g., LNET. A challenge for VM based use cases will be to provide efficient IO forwarding of the parallel filessytem from the host to the guest (VM). There are promising options like para-virtualized filesystems to

  19. Spent nuclear fuel storage

    International Nuclear Information System (INIS)

    Romanato, Luiz Sergio

    2005-01-01

    When a country becomes self-sufficient in part of the nuclear cycle, as production of fuel that will be used in nuclear power plants for energy generation, it is necessary to pay attention for the best method of storing the spent fuel. Temporary storage of spent nuclear fuel is a necessary practice and is applied nowadays all over the world, so much in countries that have not been defined their plan for a definitive repository, as well for those that already put in practice such storage form. There are two main aspects that involve the spent fuels: one regarding the spent nuclear fuel storage intended to reprocessing and the other in which the spent fuel will be sent for final deposition when the definitive place is defined, correctly located, appropriately characterized as to several technical aspects, and licentiate. This last aspect can involve decades of studies because of the technical and normative definitions at a given country. In Brazil, the interest is linked with the storage of spent fuels that will not be reprocessed. This work analyses possible types of storage, the international panorama and a proposal for future construction of a spent nuclear fuel temporary storage place in the country. (author)

  20. Thromboprophylaxis for women undergoing caesarean section.

    LENUS (Irish Health Repository)

    Kennedy, C

    2012-02-01

    Thromboprophylaxis for women undergoing caesarean section (CS) was introduced in the hospital in 1995. This study audited the use of tinzaparin prophylaxis in a nested cohort of women who screened negative for diabetes mellitus at 28 weeks gestation. All the women had their weight measured and BMI calculated at the first antenatal visit. Of the 284 women, 68 (24%) had a CS and all received tinzaparin. Of the 68, however, 94% received a dose lower than recommended. Compliance with prophylaxis was complete but compliance with the recommended dosage was suboptimal, which may result in venous thromboembolism after CS despite thromboprophylaxis.

  1. Care of patients undergoing external radiotherapy

    International Nuclear Information System (INIS)

    Lang, C.

    1977-01-01

    The anxiety and associated depression suffered by most patients undergoing radiotherapy is discussed and the possibilities open to the nurse to encourage and reassure patients thus facilitating physical care are considered. The general symptoms of anorexia, nausea, tiredness, skin problems, alopecia, bonemarrow depresssion and rapid tumour destruction are described and nursing care prescribed. The side-effects which may occur following radiation of the brain, head and neck region, eyes, oesophagus, lung, abdomen, pelvis, bones, skin, spine, and spinal cord are considered from the nursing standpoint. The specialised subject of radiotherapy in children is discussed briefly. (U.K.)

  2. Neutrino Signals in Electron-Capture Storage-Ring Experiments

    Directory of Open Access Journals (Sweden)

    Avraham Gal

    2016-06-01

    Full Text Available Neutrino signals in electron-capture decays of hydrogen-like parent ions P in storage-ring experiments at GSI are reconsidered, with special emphasis placed on the storage-ring quasi-circular motion of the daughter ions D in two-body decays P → D + ν e . It is argued that, to the extent that daughter ions are detected, these detection rates might exhibit modulations with periods of order seconds, similar to those reported in the GSI storage-ring experiments for two-body decay rates. New dedicated experiments in storage rings, or using traps, could explore these modulations.

  3. SOLID-STATE STORAGE DEVICE WITH PROGRAMMABLE PHYSICAL STORAGE ACCESS

    DEFF Research Database (Denmark)

    2017-01-01

    a storage device action request, and the storage device evaluating a first rule of the one or more rules by determining if the received request fulfills request conditions comprised in the first rule, and in the affirmative the storage device performing request actions comprised in the first rule......Embodiments of the present invention includes a method of operating a solid-state storage device, comprising a storage device controller in the storage device receiving a set of one or more rules, each rule comprising (i) one or more request conditions to be evaluated for a storage device action...... request received from a host computer, and (ii) one or more request actions to be performed on a physical address space of a non-volatile storage unit in the solid-state storage device in case the one or more request conditions are fulfilled; the method further comprises: the storage device receiving...

  4. Developing new transportable storage casks for interim dry storage

    International Nuclear Information System (INIS)

    Hayashi, K.; Iwasa, K.; Araki, K.; Asano, R.

    2004-01-01

    Transportable storage metal casks are to be consistently used during transport and storage for AFR interim dry storage facilities planning in Japan. The casks are required to comply with the technical standards of regulations for both transport (hereinafter called ''transport regulation'') and storage (hereafter called ''storage regulation'') to maintain safety functions (heat transfer, containment, shielding and sub-critical control). In addition to these requirements, it is not planned in normal state to change the seal materials during storage at the storage facility, therefore it is requested to use same seal materials when the casks are transported after storage period. The dry transportable storage metal casks that satisfy the requirements have been developed to meet the needs of the dry storage facilities. The basic policy of this development is to utilize proven technology achieved from our design and fabrication experience, to carry out necessary verification for new designs and to realize a safe and rational design with higher capacity and efficient fabrication

  5. Molecular solar thermal energy storage in photoswitch oligomers increases energy densities and storage times.

    Science.gov (United States)

    Mansø, Mads; Petersen, Anne Ugleholdt; Wang, Zhihang; Erhart, Paul; Nielsen, Mogens Brøndsted; Moth-Poulsen, Kasper

    2018-05-16

    Molecular photoswitches can be used for solar thermal energy storage by photoisomerization into high-energy, meta-stable isomers; we present a molecular design strategy leading to photoswitches with high energy densities and long storage times. High measured energy densities of up to 559 kJ kg -1 (155 Wh kg -1 ), long storage lifetimes up to 48.5 days, and high quantum yields of conversion of up to 94% per subunit are demonstrated in norbornadiene/quadricyclane (NBD/QC) photo-/thermoswitch couples incorporated into dimeric and trimeric structures. By changing the linker unit between the NBD units, we can at the same time fine-tune light-harvesting and energy densities of the dimers and trimers so that they exceed those of their monomeric analogs. These new oligomers thereby meet several of the criteria to be met for an optimum molecule to ultimately enter actual devices being able to undergo closed cycles of solar light-harvesting, energy storage, and heat release.

  6. Dorfman-Chanarin syndrome: A rare neutral lipid storage disease

    OpenAIRE

    Mitra Souvik; Samanta Moumita; Sarkar Mihir; Chatterjee Sukanta

    2010-01-01

    Dorfman-Chanarin syndrome is a rare neutral lipid storage disorder characterized by ichthyosis, lipid vacuolations in peripheral leucocytes, and multisystem involvement. It is an autosomal recessive disorder caused by mutations in the CGI-58 gene. A total of 42 cases have been reported worldwide till February 2009 out of which 4 have been previously reported from India. We report a case of a 20-month-old male with congenital ichthyosis, organomegaly, and bilateral cryptorchidism. Examination ...

  7. Somatic symptom disorder

    Science.gov (United States)

    ... related disorders; Somatization disorder; Somatiform disorders; Briquet syndrome; Illness anxiety disorder References American Psychiatric Association. Somatic symptom disorder. Diagnostic and Statistical Manual of Mental Disorders . ...

  8. Medicamentos de alto custo para doenças raras no Brasil: o exemplo das doenças lisossômicas High cost drugs for rare diseases in Brazil: the case of lysosomal storage disorders

    Directory of Open Access Journals (Sweden)

    Mônica Vinhas de Souza

    2010-11-01

    Full Text Available Este artigo aborda, de forma crítica, aspectos das políticas públicas brasileiras para medicamentos, com ênfase nos de alto custo dirigidos às doenças raras. As doenças lisossômicas foram utilizadas como exemplo pela sua raridade e pela tendência mundial para o desenvolvimento de novos fármacos para seu tratamento. Três doenças foram abordadas: doença de Gaucher, doença de Fabry e mucopolissacaridose tipo I. Embora todas tenham medicamentos registrados no Brasil, a doença de Gaucher é a única com protocolo clínico e diretrizes de tratamento balizadas pelo Ministério da Saúde. Os autores almejam, com este artigo, fomentar a discussão sobre o papel da avaliação de tecnologias em saúde para o tratamento das doenças raras no Brasil, enfatizando a necessidade de políticas legitimadas dirigidas especialmente a elas. A despeito das dificuldades de se estabelecer uma política de saúde específica para cada doença rara, é possível o estabelecimento de modelos racionais para lidar com esse crescente desafio.This paper approaches in a critical way aspects of Brazilian public policies for drugs, emphasizing those classified as high cost and for rare diseases. The lysosomal storage diseases was taken as an example because of their rarity and the international trend for the development of new drugs for their treatment, all at high costs. Three lysosomal storage diseases were approached: Gaucher disease, Fabry disease and mucopolysaccharidosis type I. Gaucher disease has its treatment drug licensed in Brazil and guidelines for its use are established through a clinical protocol by the Ministry of Health. The others have their drug treatments registered in Brazil; however, no treatment guidelines for them have been developed by the government. The objective of the paper was to foster the discussion on the role of health technology assessment for high-cost drugs for rare diseases in Brazil, emphasizing the need for establishing

  9. GAS STORAGE TECHNOLOGY CONSORTIUM

    Energy Technology Data Exchange (ETDEWEB)

    Robert W. Watson

    2004-10-18

    Gas storage is a critical element in the natural gas industry. Producers, transmission and distribution companies, marketers, and end users all benefit directly from the load balancing function of storage. The unbundling process has fundamentally changed the way storage is used and valued. As an unbundled service, the value of storage is being recovered at rates that reflect its value. Moreover, the marketplace has differentiated between various types of storage services, and has increasingly rewarded flexibility, safety, and reliability. The size of the natural gas market has increased and is projected to continue to increase towards 30 trillion cubic feet (TCF) over the next 10 to 15 years. Much of this increase is projected to come from electric generation, particularly peaking units. Gas storage, particularly the flexible services that are most suited to electric loads, is critical in meeting the needs of these new markets. In order to address the gas storage needs of the natural gas industry, an industry-driven consortium was created--the Gas Storage Technology Consortium (GSTC). The objective of the GSTC is to provide a means to accomplish industry-driven research and development designed to enhance operational flexibility and deliverability of the Nation's gas storage system, and provide a cost effective, safe, and reliable supply of natural gas to meet domestic demand. To accomplish this objective, the project is divided into three phases that are managed and directed by the GSTC Coordinator. The first phase, Phase 1A, was initiated on September 30, 2003, and was completed on March 31, 2004. Phase 1A of the project included the creation of the GSTC structure, development and refinement of a technical approach (work plan) for deliverability enhancement and reservoir management. This report deals with Phase 1B and encompasses the period July 1, 2004, through September 30, 2004. During this time period there were three main activities. First was the

  10. Aboveground storage tanks

    International Nuclear Information System (INIS)

    Rizzo, J.A.

    1992-01-01

    With the 1988 promulgation of the comprehensive Resource Conservation and Recovery Act (RCRA) regulations for underground storage of petroleum and hazardous substances, many existing underground storage tank (UST) owners have been considering making the move to aboveground storage. While on the surface, this may appear to be the cure-all to avoiding the underground leakage dilemma, there are many other new and different issues to consider with aboveground storage. The greatest misconception is that by storing materials above ground, there is no risk of subsurface environmental problems. it should be noted that with the aboveground storage tank (AGST) systems, there is still considerable risk of environmental contamination, either by the failure of onground tank bottoms or the spillage of product onto the ground surface where it subsequently finds its way to the ground water. In addition, there are added safety concerns that must be addressed. So what are the other specific areas of concern besides environmental to be addressed when making the decision between underground and aboveground tanks? The primary issues that will be addressed in this paper are: Safety, Product Losses, Cost Comparison of USTs vs AGSTs, Space Availability/Accessibility, Precipitation Handling, Aesthetics and Security, Pending and Existing Regulations

  11. Radioactive waste storage issues

    International Nuclear Information System (INIS)

    Kunz, D.E.

    1994-01-01

    In the United States we generate greater than 500 million tons of toxic waste per year which pose a threat to human health and the environment. Some of the most toxic of these wastes are those that are radioactively contaminated. This thesis explores the need for permanent disposal facilities to isolate radioactive waste materials that are being stored temporarily, and therefore potentially unsafely, at generating facilities. Because of current controversies involving the interstate transfer of toxic waste, more states are restricting the flow of wastes into - their borders with the resultant outcome of requiring the management (storage and disposal) of wastes generated solely within a state's boundary to remain there. The purpose of this project is to study nuclear waste storage issues and public perceptions of this important matter. Temporary storage at generating facilities is a cause for safety concerns and underscores, the need for the opening of permanent disposal sites. Political controversies and public concern are forcing states to look within their own borders to find solutions to this difficult problem. Permanent disposal or retrievable storage for radioactive waste may become a necessity in the near future in Colorado. Suitable areas that could support - a nuclear storage/disposal site need to be explored to make certain the health, safety and environment of our citizens now, and that of future generations, will be protected

  12. High temperature storage loop :

    Energy Technology Data Exchange (ETDEWEB)

    Gill, David Dennis; Kolb, William J.

    2013-07-01

    A three year plan for thermal energy storage (TES) research was created at Sandia National Laboratories in the spring of 2012. This plan included a strategic goal of providing test capability for Sandia and for the nation in which to evaluate high temperature storage (>650ÀC) technology. The plan was to scope, design, and build a flow loop that would be compatible with a multitude of high temperature heat transfer/storage fluids. The High Temperature Storage Loop (HTSL) would be reconfigurable so that it was useful for not only storage testing, but also for high temperature receiver testing and high efficiency power cycle testing as well. In that way, HTSL was part of a much larger strategy for Sandia to provide a research and testing platform that would be integral for the evaluation of individual technologies funded under the SunShot program. DOEs SunShot program seeks to reduce the price of solar technologies to 6/kWhr to be cost competitive with carbon-based fuels. The HTSL project sought to provide evaluation capability for these SunShot supported technologies. This report includes the scoping, design, and budgetary costing aspects of this effort

  13. Energy storage connection system

    Science.gov (United States)

    Benedict, Eric L.; Borland, Nicholas P.; Dale, Magdelena; Freeman, Belvin; Kite, Kim A.; Petter, Jeffrey K.; Taylor, Brendan F.

    2012-07-03

    A power system for connecting a variable voltage power source, such as a power controller, with a plurality of energy storage devices, at least two of which have a different initial voltage than the output voltage of the variable voltage power source. The power system includes a controller that increases the output voltage of the variable voltage power source. When such output voltage is substantially equal to the initial voltage of a first one of the energy storage devices, the controller sends a signal that causes a switch to connect the variable voltage power source with the first one of the energy storage devices. The controller then causes the output voltage of the variable voltage power source to continue increasing. When the output voltage is substantially equal to the initial voltage of a second one of the energy storage devices, the controller sends a signal that causes a switch to connect the variable voltage power source with the second one of the energy storage devices.

  14. Technology Roadmap: Energy Storage

    Energy Technology Data Exchange (ETDEWEB)

    NONE

    2014-03-01

    Energy storage technologies are valuable components in most energy systems and could be an important tool in achieving a low-carbon future. These technologies allow for the decoupling of energy supply and demand, in essence providing a valuable resource to system operators. There are many cases where energy storage deployment is competitive or near-competitive in today's energy system. However, regulatory and market conditions are frequently ill-equipped to compensate storage for the suite of services that it can provide. Furthermore, some technologies are still too expensive relative to other competing technologies (e.g. flexible generation and new transmission lines in electricity systems). One of the key goals of this new roadmap is to understand and communicate the value of energy storage to energy system stakeholders. This will include concepts that address the current status of deployment and predicted evolution in the context of current and future energy system needs by using a ''systems perspective'' rather than looking at storage technologies in isolation.

  15. Nuclear fuel storage facility

    International Nuclear Information System (INIS)

    Matsumoto, Takashi; Isaka, Shinji.

    1987-01-01

    Purpose: To increase the spent fuel storage capacity and reduce the installation cost in a nuclear fuel storage facility. Constitution: Fuels handled in the nuclear fuel storage device of the present invention include the following four types: (1) fresh fuels, (2) 100 % reactor core charged fuels, (3) spent fuels just after taking out and (4) fuels after a certain period (for example one half-year) from taking out of the reactor. Reactivity is high for the fuels (1), and some of fuels (2), while low in the fuels (3) (4), Source intensity is strong for the fuels (3) and some of the fuels (2), while it is low for the fuels (1) and (4). Taking notice of the fact that the reactivity, radioactive source intensity and generated after heat are different in the respective fuels, the size of the pool and the storage capacity are increased by the divided storage control. While on the other hand, since the division is made in one identical pool, the control method becomes important, and the working range is restricted by means of a template, interlock, etc., the operation mode of the handling machine is divided into four, etc. for preventing errors. (Kamimura, M.)

  16. WWER spent fuel storage

    Energy Technology Data Exchange (ETDEWEB)

    Bower, C C; Lettington, C [GEC Alsthom Engineering Systems Ltd., Whetstone (United Kingdom)

    1994-12-31

    Selection criteria for PAKS NPP dry storage system are outlined. They include the following: fuel temperature in storage; sub-criticality assurance (avoidance of criticality for fuel in the unirradiated condition without having to take credit for burn-up); assurance of decay heat removal; dose uptake to the operators and public; protection of environment; volume of waste produced during operation and decommissioning; physical protection of stored irradiated fuel assemblies; IAEA safeguards assurance; storage system versus final disposal route; cost of construction and extent of technology transfer to Hungarian industry. Several available systems are evaluated against these criteria, and as a result the GEC ALSTHOM Modular Vault Dry Store (MVDS) system has been selected. The MVDS is a passively cooled dry storage facility. Its most important technical, safety, licensing and technology transfer characteristics are outlined. On the basis of the experience gained some key questions and considerations related to the East European perspective in the field of spent fuel storage are discussed. 8 figs.

  17. WWER spent fuel storage

    International Nuclear Information System (INIS)

    Bower, C.C.; Lettington, C.

    1994-01-01

    Selection criteria for PAKS NPP dry storage system are outlined. They include the following: fuel temperature in storage; sub-criticality assurance (avoidance of criticality for fuel in the unirradiated condition without having to take credit for burn-up); assurance of decay heat removal; dose uptake to the operators and public; protection of environment; volume of waste produced during operation and decommissioning; physical protection of stored irradiated fuel assemblies; IAEA safeguards assurance; storage system versus final disposal route; cost of construction and extent of technology transfer to Hungarian industry. Several available systems are evaluated against these criteria, and as a result the GEC ALSTHOM Modular Vault Dry Store (MVDS) system has been selected. The MVDS is a passively cooled dry storage facility. Its most important technical, safety, licensing and technology transfer characteristics are outlined. On the basis of the experience gained some key questions and considerations related to the East European perspective in the field of spent fuel storage are discussed. 8 figs

  18. The methods of hydrogen storage

    International Nuclear Information System (INIS)

    Joubert, J.M.; Cuevas, F.; Latroche, M.; Percheron-Guegan, A.

    2005-01-01

    Hydrogen may be an excellent energy vector owing to its high specific energy. Its low density is however a serious drawback for its storage. Three techniques exist to store hydrogen. Storage under pressure is now performed in composite tanks under pressures around 700 bar. Liquid storage is achieved at cryogenic temperatures. Solid storage is possible in reversible metal hydrides or on high surface area materials. The three storage means are compared in terms of performance, energetic losses and risk. (authors)

  19. Plasma magnesium concentration in patients undergoing coronary artery bypass grafting.

    Science.gov (United States)

    Kotlinska-Hasiec, Edyta; Makara-Studzinska, Marta; Czajkowski, Marek; Rzecki, Ziemowit; Olszewski, Krzysztof; Stadnik, Adam; Pilat, Jacek; Rybojad, Beata; Dabrowski, Wojciech

    2017-05-11

    [b]Introduction[/b]. Magnesium (Mg) plays a crucial role in cell physiology and its deficiency may cause many disorders which often require intensive treatment. The aim of this study was to analyse some factors affecting preoperative plasma Mg concentration in patients undergoing coronary artery bypass grafting (CABG). [b]Materials and method[/b]. Adult patients scheduled for elective CABG with cardio-pulmonary bypass (CPB) under general anaesthesia were studied. Plasma Mg concentration was analysed before surgery in accordance with age, domicile, profession, tobacco smoking and preoperative Mg supplementation. Blood samples were obtained from the radial artery just before the administration of anaesthesia. [b]Results. [/b]150 patients were studied. Mean preoperative plasma Mg concentration was 0.93 ± 0.17 mmol/L; mean concentration in patients - 1.02 ± 0.16; preoperative Mg supplementation was significantly higher than in patients without such supplementation. Moreover, intellectual workers supplemented Mg more frequently and had higher plasma Mg concentration than physical workers. Plasma Mg concentration decreases in elderly patients. Patients living in cities, on average, had the highest plasma Mg concentration. Smokers had significantly lower plasma Mg concentration than non-smokers. [b]Conclusions. [/b]1. Preoperative magnesium supplementation increases its plasma concentration. 2. Intellectual workers frequently supplement magnesium. 3. Smoking cigarettes decreases plasma magnesium concentration.

  20. Federal Interim Storage program

    International Nuclear Information System (INIS)

    Johnson, E.R.; McBride, J.A.

    1984-01-01

    The DOE has developed a program for providing Federal Interim Storage servies for spent nuclear fuel which complies with the requirements of the Nuclear Waste Policy Act of 1982. Although very little constructive activity in providing storage facilities can be undertaken by DOE until fuel has been certified by NRC as eligible for FIS, DOE planning and background information is such as to provide reasonable assurance that its obligations can be fulfilled when the required certifications have been issued. A fee structure providing fuel recovery of all costs associated with the FIS program, as required by the Act, has been developed. It provides for an equitable distribution of costs among users, based on the quantity of fuel requiring storage

  1. Evolution of clustered storage

    CERN Multimedia

    CERN. Geneva; Van de Vyvre, Pierre

    2007-01-01

    The session actually featured two presentations: * Evolution of clustered storage by Lance Hukill, Quantum Corporation * ALICE DAQ - Usage of a Cluster-File System: Quantum StorNext by Pierre Vande Vyvre, CERN-PH the second one prepared at short notice by Pierre (thanks!) to present how the Quantum technologies are being used in the ALICE experiment. The abstract to Mr Hukill's follows. Clustered Storage is a technology that is driven by business and mission applications. The evolution of Clustered Storage solutions starts first at the alignment between End-users needs and Industry trends: * Push-and-Pull between managing for today versus planning for tomorrow * Breaking down the real business problems to the core applications * Commoditization of clients, servers, and target devices * Interchangeability, Interoperability, Remote Access, Centralized control * Oh, and yes, there is a budget and the "real world" to deal with This presentation will talk through these needs and trends, and then ask the question, ...

  2. Costs of Archival Storage

    DEFF Research Database (Denmark)

    Nielsen, Anders Bo; Thirifays, Alex; Kejser, Ulla Bøgvad

    2012-01-01

    to determine the costs of establishing and maintaining a preservation solution destined for long-term preservation of digital materials and to develop a tool capable of doing this operation. In order to fulfill the purposes, the project employed a combination of own and external experience as well as the OAIS......This paper presents an analysis of the cost of archival storage. The study is part of a project conducted by The Danish National Archives, The Royal Library, and The State and University Library to develop a generic cost model for digital preservation (CMDP). The purposes of the study were...... Reference Model as a framework to fully understand and identify the cost critical activities of bit-preservation as described in Archival Storage. We found that the costs of Archival Storage are obviously closely linked to the data volume, but also to the required preservation quality, especially...

  3. Plutonium storage phenomenology

    International Nuclear Information System (INIS)

    Szempruch, R.

    1995-12-01

    Plutonium has been produced, handled, and stored at Department of Energy (DOE) facilities since the 1940s. Many changes have occurred during the last 40 years in the sources, production demands, and end uses of plutonium. These have resulted in corresponding changes in the isotopic composition as well as the chemical and physical forms of the processed and stored plutonium. Thousands of ordinary food pack tin cans have been used successfully for many years to handle and store plutonium. Other containers have been used with equal success. This paper addressees the exceptions to this satisfactory experience. To aid in understanding the challenges of handling plutonium for storage or immobilization the lessons learned from past storage experience and the necessary countermeasures to improve storage performance are discussed

  4. Hormone profiles and their relation with menstrual cycles in patients undergoing hemodialysis

    Directory of Open Access Journals (Sweden)

    Deniz Cemgil Arıkan

    2011-03-01

    Full Text Available Objective: To investigate the etiology of menstrual disorders among patients undergoing hemodialysis due to chronic renal failure by assessing menstrual history, serum hormone levels, and other biochemical factors. Material and methods: Thirty patients undergoing hemodialysis and 30 healthy women at reproductive age were enrolled in our study. Demographic characteristics, hormonal and biochemical data, and sonographically measured endometrial thickness values of the subjects were compared. In addition, the present and the pre-hemodialysis menstrual pattern of the patients undergoing hemodialysis were recorded. The hormonal, hematological, and biochemical data of the patients were compared according to their menstrual patterns. Results: No statistical significance was seen between age, BMI, gravida, parity, abortion, and curettage among groups (p>0.05. Hemoglobin and hematocrit levels were significantly lower in the hemodialysis group than in the control (p0.05. Mean serum LH and prolactin levels were significantly higher in the hemodialysis group compared to the control (p0.05. Serum LH and prolactin levels were higher, and serum FSH, estradiol and TSH levels were lower in patients who developed amenorrhea after hemodialysis treatment when compared to non-amenorrheic subjects. However, these differences were not statistically significant (p>0.05. Discussion: The most important factor in the etiology of menstrual disorders seen in chronic renal failure patients was high serum LH and prolactin levels. Hemodialysis is a successful treatment that extends life expectancy and ameliorates the hypothalamo-pituitary-ovarian axis in chronic renal failure patients.

  5. Extended storage of spent fuel

    International Nuclear Information System (INIS)

    1992-10-01

    This document is the final report on the IAEA Co-ordinated Research Programme on the Behaviour of Spent Fuel and Storage Facility Components during Long Term Storage (BEFAST-II, 1986-1991). It contains the results on wet and dry spent fuel storage technologies obtained from 16 organizations representing 13 countries who participated in the co-ordinated research programme. Considerable quantities of spent fuel continue to arise and accumulate. Many countries are investigating the option of extended spent fuel storage prior to reprocessing or fuel disposal. Wet storage continues to predominate as an established technology with the construction of additional away-from-reactor storage pools. However, dry storage is increasingly used with most participants considering dry storage concepts for the longer term. Depending on the cladding type options of dry storage in air or inert gas are proposed. Dry storage is becoming widely used as a supplement to wet storage for zirconium alloy clad oxide fuels. Storage periods as long as under wet conditions appear to be feasible. Dry storage will also continue to be used for Al clad and Magnox type fuel. Enhancement of wet storage capacity will remain an important activity. Rod consolidation to increase wet storage capacity will continue in the UK and is being evaluated for LWR fuel in the USA, and may start in some other countries. High density storage racks have been successfully introduced in many existing pools and are planned for future facilities. For extremely long wet storage (≥50 years), there is a need to continue work on fuel integrity investigations and LWR fuel performance modelling. it might be that pool component performance in some cases could be more limiting than the FA storage performance. It is desirable to make concerted efforts in the field of corrosion monitoring and prediction of fuel cladding and poll component behaviour in order to maintain good experience of wet storage. Refs, figs and tabs

  6. Electrochemical energy storage

    CERN Document Server

    Tarascon, Jean-Marie

    2015-01-01

    The electrochemical storage of energy has become essential in assisting the development of electrical transport and use of renewable energies. French researchers have played a key role in this domain but Asia is currently the market leader. Not wanting to see history repeat itself, France created the research network on electrochemical energy storage (RS2E) in 2011. This book discusses the launch of RS2E, its stakeholders, objectives, and integrated structure that assures a continuum between basic research, technological research and industries. Here, the authors will cover the technological

  7. Nuclear fuel storage

    International Nuclear Information System (INIS)

    Bevilacqua, F.

    1979-01-01

    A method and apparatus for the storage of fuel in a stainless steel egg crate structure within a storage pool are described. Fuel is initially stored in a checkerboard pattern or in each opening if the fuel is of low enrichment. Additional fuel (or fuel of higher enrichment) is later stored by adding stainless steel angled plates within each opening, thereby forming flux traps between the openings. Still higher enrichment fuel is later stored by adding poison plates either with or without the stainless steel angles. 8 claims

  8. Proton storage rings

    International Nuclear Information System (INIS)

    Rau, R.R.

    1978-04-01

    A discussion is given of proton storage ring beam dynamic characteristics. Topics considered include: (1) beam energy; (2) beam luminosity; (3) limits on beam current; (4) beam site; (5) crossing angle; (6) beam--beam interaction; (7) longitudinal instability; (8) effects of scattering processes; (9) beam production; and (10) high magnetic fields. Much of the discussion is related to the design parameters of ISABELLE, a 400 x 400 GeV proton---proton intersecting storage accelerator to be built at Brookhaven National Laboratory

  9. Attenuation of Red Blood Cell Storage Lesions with Vitamin C

    Directory of Open Access Journals (Sweden)

    Kimberly Sanford

    2017-07-01

    Full Text Available Stored red blood cells (RBCs undergo oxidative stress that induces deleterious metabolic, structural, biochemical, and molecular changes collectively referred to as “storage lesions”. We hypothesized that vitamin C (VitC, reduced or oxidized would reduce red cell storage lesions, thus prolonging their storage duration. Whole-blood-derived, leuko-reduced, SAGM (saline-adenine-glucose-mannitol-preserved RBC concentrates were equally divided into four pediatric storage bags and the following additions made: (1 saline (saline; (2 0.3 mmol/L reduced VitC (Lo VitC; (3 3 mmol/L reduced VitC (Hi VitC; or (4 0.3 mmol/L oxidized VitC (dehydroascorbic acid, DHA as final concentrations. Biochemical and rheological parameters were serially assessed at baseline (prior to supplementation and Days 7, 21, 42, and 56 for RBC VitC concentration, pH, osmotic fragility by mechanical fragility index, and percent hemolysis, LDH release, glutathione depletion, RBC membrane integrity by scanning electron microscopy, and Western blot for β-spectrin. VitC exposure (reduced and oxidized significantly increased RBC antioxidant status with varying dynamics and produced trends in reduction in osmotic fragility and increases in membrane integrity. Conclusion: VitC partially protects RBC from oxidative changes during storage. Combining VitC with other antioxidants has the potential to improve long-term storage of RBC.

  10. Hepatic MR imaging for in vivo differentiation of steatosis, iron deposition and combined storage disorder: Single-ratio in/opposed phase analysis vs. dual-ratio Dixon discrimination

    International Nuclear Information System (INIS)

    Bashir, Mustafa R.; Merkle, Elmar M.; Smith, Alastair D.; Boll, Daniel T.

    2012-01-01

    Objective: To assess whether in vivo dual-ratio Dixon discrimination can improve detection of diffuse liver disease, specifically steatosis, iron deposition and combined disease over traditional single-ratio in/opposed phase analysis. Methods: Seventy-one patients with biopsy-proven (17.7 ± 17.0 days) hepatic steatosis (n = 16), iron deposition (n = 11), combined deposition (n = 3) and neither disease (n = 41) underwent MR examinations. Dual-echo in/opposed-phase MR with Dixon water/fat reconstructions were acquired. Analysis consisted of: (a) single-ratio hepatic region-of-interest (ROI)-based assessment of in/opposed ratios; (b) dual-ratio hepatic ROI assessment of in/opposed and fat/water ratios; (c) computer-aided dual-ratio assessment evaluating all hepatic voxels. Disease-specific thresholds were determined; statistical analyses assessed disease-dependent voxel ratios, based on single-ratio (a) and dual-ratio (b and c) techniques. Results: Single-ratio discrimination succeeded in identifying iron deposition (I/O Ironthreshold Fatthreshold>1.15 ) from normal parenchyma, sensitivity 70.0%; it failed to detect combined disease. Dual-ratio discrimination succeeded in identifying abnormal hepatic parenchyma (F/W Normalthreshold > 0.05), sensitivity 96.7%; logarithmic functions for iron deposition (I/O Iron d iscriminator (0.01−F/W Iron )/0.48 ) and for steatosis (I/O Fatdiscriminator > e (F/W Fat −0.01)/0.48 ) differentiated combined from isolated diseases, sensitivity 100.0%; computer-aided dual-ratio analysis was comparably sensitive but less specific, 90.2% vs. 97.6%. Conclusion: MR two-point-Dixon imaging using dual-ratio post-processing based on in/opposed and fat/water ratios improved in vivo detection of hepatic steatosis, iron deposition, and combined storage disease beyond traditional in/opposed analysis.

  11. Transcriptomic events associated with internal browning of apple during postharvest storage

    OpenAIRE

    Mellidou, Ifigeneia; Buts, Kim; Hatoum, Darwish; Ho, Quang Tri; Johnston, Jason W; Watkins, Christopher B; Schaffer, Robert J; Gapper, Nigel E; Giovannoni, Jim J; Rudell, David R; Hertog, Maarten LATM; Nicolai, Bart M

    2014-01-01

    Background Postharvest ripening of apple (Malus x domestica) can be slowed down by low temperatures, and a combination of low O2 and high CO2 levels. While this maintains the quality of most fruit, occasionally storage disorders such as flesh browning can occur. This study aimed to explore changes in the apple transcriptome associated with a flesh browning disorder related to controlled atmosphere storage using RNA-sequencing techniques. Samples from a browning-susceptible cultivar (‘Braeburn...

  12. Symptomatic lipid storage in carriers for the PNPLA2 gene

    NARCIS (Netherlands)

    Janssen, M.C.H.; Engelen, B.G.M. van; Kapusta, L.; Lammens, M.M.; Dijk, M.; Fischer, J.; Graaf, M. van der; Wevers, R.A.; Fahrleitner, M.; Zimmermann, R.; Morava, E.

    2013-01-01

    Neutral lipid storage disease comprises a heterogeneous group of inherited disorders characterized by severe accumulation of cytoplasmic triglyceride droplets in several tissues and neutrophils. A novel type of autosomal recessive lipid myopathy due to PNPLA2 mutations was recently described with

  13. CHEMICAL STORAGE: MYTHS VERSUS REALITY

    International Nuclear Information System (INIS)

    Simmons, F.

    2007-01-01

    A large number of resources explaining proper chemical storage are available. These resources include books, databases/tables, and articles that explain various aspects of chemical storage including compatible chemical storage, signage, and regulatory requirements. Another source is the chemical manufacturer or distributor who provides storage information in the form of icons or color coding schemes on container labels. Despite the availability of these resources, chemical accidents stemming from improper storage, according to recent reports (1) (2), make up almost 25% of all chemical accidents. This relatively high percentage of chemical storage accidents suggests that these publications and color coding schemes although helpful, still provide incomplete information that may not completely mitigate storage risks. This manuscript will explore some ways published storage information may be incomplete, examine the associated risks, and suggest methods to help further eliminate chemical storage risks

  14. Storage potential of ‘SCS426 Venice’ apples under different storage technologies

    Directory of Open Access Journals (Sweden)

    Mariuccia Schlichting de Martin

    2018-04-01

    Full Text Available Abstract This study aimed to evaluate the storage potential of SCS426 Venice apples under different storage technologies. Fruits were harvested in an experimental orchard located in Fraiburgo, SC and stored for up to eight and ten months in 2013 and 2014, respectively. Apples were treated or not with methylcyclopropene (1-MCP and stored under air atmosphere (AA, 0.5±0.5 °C/RH 85±5% or controlled atmosphere (CA; 1.5 kPa of O2 and 1.5 kPa of CO2 at 0.7±0.5 °C/RH of 93±3%. Fruits were evaluated every two months of storage, after one and seven days of shelf life (23 ± 0.3 °C/RH 93±3%. The storage period of ‘SCS426 Venice’ apples under AA without 1-MCP application should not extend beyond six months. Under this storage condition, fruits had higher incidence of decay, ethylene production and respiratory rates, higher skin degreening, lower flesh firmness, titratable acidity and soluble solids content than fruits stored under CA or AA with 1-MCP. ‘SCS426 Venice’ apples develop flesh browning and superficial scald after long-term storage. ‘SCS426 Venice’ apples under AA treated with 1-MCP or under CA (regardless of 1-MCP application can be stored for more than eight months, keeping flesh firmness above 14 lb and low incidence of physiological disorders even after ten months of storage.

  15. Recent development and gene therapy for glycogen storage disease type Ia.

    Science.gov (United States)

    Chou, Janice Y; Kim, Goo-Young; Cho, Jun-Ho

    2017-09-01

    Glycogen storage disease type Ia (GSD-Ia) is an autosomal recessive metabolic disorder caused by a deficiency in glucose-6-phosphatase-α (G6Pase-α or G6PC) that is expressed primarily in the liver, kidney, and intestine. G6Pase-α catalyzes the hydrolysis of glucose-6-phosphate (G6P) to glucose and phosphate in the terminal step of gluconeogenesis and glycogenolysis, and is a key enzyme for endogenous glucose production. The active site of G6Pase-α is inside the endoplasmic reticulum (ER) lumen. For catalysis, the substrate G6P must be translocated from the cytoplasm into the ER lumen by a G6P transporter (G6PT). The functional coupling of G6Pase-α and G6PT maintains interprandial glucose homeostasis. Dietary therapies for GSD-Ia are available, but cannot prevent the long-term complication of hepatocellular adenoma that may undergo malignant transformation to hepatocellular carcinoma. Animal models of GSD-Ia are now available and are being exploited to both delineate the disease more precisely and develop new treatment approaches, including gene therapy.

  16. Kinetic energy storage system

    Energy Technology Data Exchange (ETDEWEB)

    Jaeggi, M.; Folini, P.

    1983-09-03

    A flywheel system for the purpose of energy storage in decentral solar- or wind energy plants is introduced. The system comprises a rotor made out of plastic fibre, a motor/generator serving as electro-mechanical energy converter and a frequency-voltage transformer serving as electric adapter. The storable energy quantity amounts to several kWh.

  17. Solar Energy: Heat Storage.

    Science.gov (United States)

    Knapp, Henry H., III

    This module on heat storage is one of six in a series intended for use as supplements to currently available materials on solar energy and energy conservation. Together with the recommended texts and references (sources are identified), these modules provide an effective introduction to energy conservation and solar energy technologies. The module…

  18. Benchmarking personal cloud storage

    NARCIS (Netherlands)

    Drago, Idilio; Bocchi, Enrico; Mellia, Marco; Slatman, Herman; Pras, Aiko

    2013-01-01

    Personal cloud storage services are data-intensive applications already producing a significant share of Internet traffic. Several solutions offered by different companies attract more and more people. However, little is known about each service capabilities, architecture and - most of all -

  19. DPM: Future Proof Storage

    CERN Document Server

    Alvarez, Alejandro; Furano, Fabrizio; Hellmich, Martin; Keeble, Oliver; Rocha, Ricardo; CERN. Geneva. IT Department

    2012-01-01

    The Disk Pool Manager (DPM) is a lightweight solution for grid enabled disk storage management. Operated at more than 240 sites it has the widest distribution of all grid storage solutions in the WLCG infrastructure. It provides an easy way to manage and configure disk pools, and exposes multiple interfaces for data access (rfio, xroot, nfs, gridftp and http/dav) and control (srm). During the last year we have been working on providing stable, high performant data access to our storage system using standard protocols, while extending the storage management functionality and adapting both configuration and deployment procedures to reuse commonly used building blocks. In this contribution we cover in detail the extensive evaluation we have performed of our new HTTP/WebDAV and NFS 4.1 frontends, in terms of functionality and performance. We summarize the issues we faced and the solutions we developed to turn them into valid alternatives to the existing grid protocols - namely the additional work required to prov...

  20. Energy Storage and Retrieval

    Indian Academy of Sciences (India)

    Annual Meetings · Mid Year Meetings · Discussion Meetings · Public Lectures · Lecture Workshops · Refresher Courses · Symposia · Live Streaming. Home; Journals; Resonance – Journal of Science Education; Volume 1; Issue 6. Energy Storage and Retrieval The Secondary Battery Route. A K Shukla P Vishnu Kamath.

  1. Tuber storage proteins.

    Science.gov (United States)

    Shewry, Peter R

    2003-06-01

    A wide range of plants are grown for their edible tubers, but five species together account for almost 90 % of the total world production. These are potato (Solanum tuberosum), cassava (Manihot esculenta), sweet potato (Ipomoea batatus), yams (Dioscorea spp.) and taro (Colocasia, Cyrtosperma and Xanthosoma spp.). All of these, except cassava, contain groups of storage proteins, but these differ in the biological properties and evolutionary relationships. Thus, patatin from potato exhibits activity as an acylhydrolase and esterase, sporamin from sweet potato is an inhibitor of trypsin, and dioscorin from yam is a carbonic anhydrase. Both sporamin and dioscorin also exhibit antioxidant and radical scavenging activity. Taro differs from the other three crops in that it contains two major types of storage protein: a trypsin inhibitor related to sporamin and a mannose-binding lectin. These characteristics indicate that tuber storage proteins have evolved independently in different species, which contrasts with the highly conserved families of storage proteins present in seeds. Furthermore, all exhibit biological activities which could contribute to resistance to pests, pathogens or abiotic stresses, indicating that they may have dual roles in the tubers.

  2. Fuel storage rack

    International Nuclear Information System (INIS)

    Mollon, L.

    1977-01-01

    Disclosed is a storage rack for spent nuclear fuel elements comprising a multiplicity of elongated hollow containers of uniform cross-section, preferably square,some of said containers having laterally extending continuous flanges extending between adjacent containers and defining continuous elongated chambers therebetween for the reception of neutron absorbing panels. 18 claims, 7 figures

  3. Carbon Capture and Storage

    NARCIS (Netherlands)

    Benson, S.M.; Bennaceur, K.; Cook, P.; Davison, J.; Coninck, H. de; Farhat, K.; Ramirez, C.A.; Simbeck, D.; Surles, T.; Verma, P.; Wright, I.

    2012-01-01

    Emissions of carbon dioxide, the most important long-lived anthropogenic greenhouse gas, can be reduced by Carbon Capture and Storage (CCS). CCS involves the integration of four elements: CO 2 capture, compression of the CO2 from a gas to a liquid or a denser gas, transportation of pressurized CO 2

  4. Storage of radioactive waste

    International Nuclear Information System (INIS)

    Pittman, F.K.

    1974-01-01

    Four methods for managing radioactive waste in order to protect man from its potential hazards include: transmutation to convert radioisotopes in waste to stable isotopes; disposal in space; geological disposal; and surface storage in shielded, cooled, and monitored containers. A comparison of these methods shows geologic disposal in stable formations beneath landmasses appears to be the most feasible with today's technology. (U.S.)

  5. NGLW RCRA Storage Study

    International Nuclear Information System (INIS)

    Waters, R.J.; Ochoa, R.; Fritz, K.D.; Craig, D.W.

    2000-01-01

    The Idaho Nuclear Technology and Engineering Center (INTEC) at the Idaho National Engineering and Environmental Laboratory contains radioactive liquid waste in underground storage tanks at the INTEC Tank Farm Facility (TFF). INTEC is currently treating the waste by evaporation to reduce the liquid volume for continued storage, and by calcination to reduce and convert the liquid to a dry waste form for long-term storage in calcine bins. Both treatment methods and activities in support of those treatment operations result in Newly Generated Liquid Waste (NGLW) being sent to TFF. The storage tanks in the TFF are underground, contained in concrete vaults with instrumentation, piping, transfer jets, and managed sumps in case of any liquid accumulation in the vault. The configuration of these tanks is such that Resource Conservation and Recovery Act (RCRA) regulations apply. The TFF tanks were assessed several years ago with respect to the RCRA regulations and they were found to be deficient. This study considers the configuration of the current tanks and the RCRA deficiencies identified for each. The study identifies four potential methods and proposes a means of correcting the deficiencies. The cost estimates included in the study account for construction cost; construction methods to minimize work exposure to chemical hazards, radioactive contamination, and ionizing radiation hazards; project logistics; and project schedule. The study also estimates the tank volumes benefit associated with each corrective action to support TFF liquid waste management planning

  6. NGLW RCRA Storage Study

    Energy Technology Data Exchange (ETDEWEB)

    R. J. Waters; R. Ochoa; K. D. Fritz; D. W. Craig

    2000-06-01

    The Idaho Nuclear Technology and Engineering Center (INTEC) at the Idaho National Engineering and Environmental Laboratory contains radioactive liquid waste in underground storage tanks at the INTEC Tank Farm Facility (TFF). INTEC is currently treating the waste by evaporation to reduce the liquid volume for continued storage, and by calcination to reduce and convert the liquid to a dry waste form for long-term storage in calcine bins. Both treatment methods and activities in support of those treatment operations result in Newly Generated Liquid Waste (NGLW) being sent to TFF. The storage tanks in the TFF are underground, contained in concrete vaults with instrumentation, piping, transfer jets, and managed sumps in case of any liquid accumulation in the vault. The configuration of these tanks is such that Resource Conservation and Recovery Act (RCRA) regulations apply. The TFF tanks were assessed several years ago with respect to the RCRA regulations and they were found to be deficient. This study considers the configuration of the current tanks and the RCRA deficiencies identified for each. The study identifies four potential methods and proposes a means of correcting the deficiencies. The cost estimates included in the study account for construction cost; construction methods to minimize work exposure to chemical hazards, radioactive contamination, and ionizing radiation hazards; project logistics; and project schedule. The study also estimates the tank volumes benefit associated with each corrective action to support TFF liquid waste management planning.

  7. Spent-fuel-storage alternatives

    Energy Technology Data Exchange (ETDEWEB)

    1980-01-01

    The Spent Fuel Storage Alternatives meeting was a technical forum in which 37 experts from 12 states discussed storage alternatives that are available or are under development. The subject matter was divided into the following five areas: techniques for increasing fuel storage density; dry storage of spent fuel; fuel characterization and conditioning; fuel storage operating experience; and storage and transport economics. Nineteen of the 21 papers which were presented at this meeting are included in this Proceedings. These have been abstracted and indexed. (ATT)

  8. Economics of dry storage systems

    International Nuclear Information System (INIS)

    Moore, G.R.; Winders, R.C.

    1980-01-01

    This paper postulates a dry storage application suitable as a regional away-from-reactor storage (AFR), develops an economical system design concept and estimates system costs. The system discussed uses the experience gained in the dry storage research activities and attempts to present a best foot forward system concept. The major element of the system is the Receiving and Packaging Building. In this building fuel assemblies are removed from transportation casks and encapsulated for storage. This facility could be equally applicable to silo, vault, or caisson storage. However the caisson storage concept has been chosen for discussion purposes

  9. Silo Storage Preconceptual Design

    Energy Technology Data Exchange (ETDEWEB)

    Stephanie L. Austad; Patrick W. Bragassa; Kevin M Croft; David S Ferguson; Scott C Gladson; Annette L Shafer; John H Weathersby

    2012-09-01

    The National Nuclear Security Administration (NNSA) has a need to develop and field a low-cost option for the long-term storage of a variety of radiological material. The storage option’s primary requirement is to provide both environmental and physical protection of the materials. Design criteria for this effort require a low initial cost and minimum maintenance over a 50-year design life. In 1999, Argonne National Laboratory-West was tasked with developing a dry silo storage option for the BN-350 Spent Fuel in Aktau Kazakhstan. Argon’s design consisted of a carbon steel cylinder approximately 16 ft long, 18 in. outside diameter and 0.375 in. wall thickness. The carbon steel silo was protected from corrosion by a duplex coating system consisting of zinc and epoxy. Although the study indicated that the duplex coating design would provide a design life well in excess of the required 50 years, the review board was concerned because of the novelty of the design and the lack of historical use. In 2012, NNSA tasked Idaho National Laboratory (INL) with reinvestigating the silo storage concept and development of alternative corrosion protection strategies. The 2012 study, “Silo Storage Concepts, Cathodic Protection Options Study” (INL/EST-12-26627), concludes that the option which best fits the design criterion is a passive cathotic protection scheme, consisting of a carbon steel tube coated with zinc or a zinc-aluminum alloy encapsulated in either concrete or a cement grout. The hot dipped zinc coating option was considered most efficient, but the flame-sprayed option could be used if a thicker zinc coating was determined to be necessary.

  10. Isotope selection for patients undergoing prostate brachytherapy

    International Nuclear Information System (INIS)

    Cha, Christine M.; Potters, Louis; Ashley, Richard; Freeman, Katherine; Wang Xiaohong; Waldbaum, Robert; Leibel, Steven

    1999-01-01

    Purpose: Ultrasound-guided trans perineal interstitial permanent prostate brachytherapy (TIPPB) is generally performed with either 103 Pd or 125 I. The use of 125 I for low Gleason score tumors and 103 Pd for higher Gleason scores has been suggested based on isotope dose rate and cell doubling time observed in in vitro studies. While many centers follow these isotope selection criteria, other centers have elected to use only a single isotope, regardless of Gleason score. No clinical data have been published comparing these isotopes. This study was undertaken to compare outcomes between 125 I and 103 Pd in a matched pair analysis for patients undergoing prostate brachytherapy. Methods and Materials: Six hundred forty-eight consecutively treated patients with clinically confined prostate cancer underwent TIPPB between June 1992 and February 1997. Five hundred thirty-two patients underwent TIPPB alone, whereas 116 received pelvic external beam irradiation and TIPPB. Ninety-three patients received androgen deprivation therapy prior to TIPPB. The prescribed doses for TIPPB were 160 Gy for 125 I (pre-TG43) and 120 Gy for 103 Pd. Patients treated with combination therapy received 41.4 or 45 Gy (1.8 Gy/fraction) external beam irradiation followed by a 3- to 5-week break and then received either a 120-Gy 125 I or a 90-Gy 103 Pd implant. Until November 1994, all patients underwent an 125 I implant after which the isotope selection was based on either Gleason score (Gleason score 2-5: 125 I; Gleason 5-8: 103 Pd) or isotope availability. A matched pair analysis was performed to assess any difference between isotopes. Two hundred twenty-two patients were matched according to Gleason score, prostate-specific antigen (PSA), and stage. PSA relapse-free survival (PSA-RFS) was calculated based on the American Society for Therapeutic Radiology and Oncology (ASTRO) Consensus Group definition of failure. Kaplan-Meier actuarial survival curves were compared to assess differences in

  11. NV Energy Electricity Storage Valuation

    Energy Technology Data Exchange (ETDEWEB)

    Ellison, James F.; Bhatnagar, Dhruv; Samaan, Nader A.; Jin, Chunlian

    2013-06-30

    This study examines how grid-level electricity storage may benet the operations of NV Energy in 2020, and assesses whether those benets justify the cost of the storage system. In order to determine how grid-level storage might impact NV Energy, an hourly production cost model of the Nevada Balancing Authority (\\BA") as projected for 2020 was built and used for the study. Storage facilities were found to add value primarily by providing reserve. Value provided by the provision of time-of-day shifting was found to be limited. If regulating reserve from storage is valued the same as that from slower ramp rate resources, then it appears that a reciprocating engine generator could provide additional capacity at a lower cost than a pumped storage hydro plant or large storage capacity battery system. In addition, a 25-MW battery storage facility would need to cost $650/kW or less in order to produce a positive Net Present Value (NPV). However, if regulating reserve provided by storage is considered to be more useful to the grid than that from slower ramp rate resources, then a grid-level storage facility may have a positive NPV even at today's storage system capital costs. The value of having storage provide services beyond reserve and time-of-day shifting was not assessed in this study, and was therefore not included in storage cost-benefit calculations.

  12. Storage Policies and Optimal Shape of a Storage System

    NARCIS (Netherlands)

    Zaerpour, N.; De Koster, René; Yu, Yugang

    2013-01-01

    The response time of a storage system is mainly influenced by its shape (configuration), the storage assignment and retrieval policies, and the location of the input/output (I/O) points. In this paper, we show that the optimal shape of a storage system, which minimises the response time for single

  13. l-carnitine as a Potential Additive in Blood Storage Solutions: A Study on Erythrocytes

    OpenAIRE

    Soumya, R.; Carl, H.; Vani, R.

    2015-01-01

    Erythrocytes undergo various changes during storage (storage lesion) that in turn reduces their functioning and survival. Oxidative stress plays a major role in the storage lesion and antioxidants can be used to combat this stress. This study elucidates the effects of l-carnitine (LC) on erythrocytes of stored blood. Blood was obtained from male Wistar rats and stored (4 °C) for 20 days in CPDA-1 (citrate phosphate dextrose adenine) solution. Samples were divided into–(i) controls (ii) LC 10 ...

  14. Plasma fibronectin in patients undergoing major surgery

    International Nuclear Information System (INIS)

    Sallam, M.H.M.

    2003-01-01

    Plasma fibronectin in patients undergoing major surgery had been determined before and after operation. The study was done on 15 patients and 15 normal healthy individuals. The study revealed that patients subjected to major operation, their fibronectin level was normal before operation followed by reduction one day post-operation. After one week, fibronectin level raised again nearly to the pre-operations levels. The probable mechanisms of fibronectin in healing processes were discussed. Fibronectin (FN) is a family of structurally and immunologically related high molecular weight glycoproteins that are present in many cell surfaces, in extracellular fluids, in connective tissues and in most membranes. Interaction with certain discrete extracellular substances, such as a glucosaminoglycans (e.g. heparin), fibrin and collagen and with cell surface structure seem to account for many of its biological activities, among which are regulation of adhesion, spreading and locomotion (Mosesson and amrani, 1980). The concentration of Fn in human plasma decreases after extensive destruction such as that occurs in major surgery, burns or other trauma. This decrease has been generally though to be due to increased consumption of soluble plasma Fn in opsonization of particulate and soluble debris from circulation by the reticuloendothelial (RE) system. Fn rapidly appears in injury areas, in experimentally induced blisters, wounded and epithelium tissues (Petersen et al., 1985). Fn accumulates at times of increased vascular permeability and it is produced by cell of blood vessels in response to injury

  15. Helicobacter pylori infection in patients undergoing appendectomy.

    Science.gov (United States)

    Pavlidis, T E; Atmatzidis, K S; Papaziogas, B T; Souparis, A; Koutelidakis, I M; Papaziogas, T B

    2002-01-01

    Helicobacter pylori has been found in the upper gastrointestinal tract; it is incriminated as aetiological factor in various pathological conditions. This prospective study assesses the presence of this microorganism in the appendix flora and the possible role of its infection in the pathogenesis of acute appendicitis. H. pylori was investigated in 46 consecutive patients undergoing emergent appendectomy for presumed acute appendicitis. Blood sample for serological test of H. pylori infection was drawn before operation. The removed appendix specimen was stained for H. pylori; confirmation was made by PCR (Polymerase Chain Reaction) analysis. The intensity of inflammation was determined pathologically grading from no inflammation to gangrenous appendicitis. Statistical analysis was made using the chi-square test. Seropositivity for H. pylori infection was found in 18 patients (39%), but the microbe was detected in just two appendix specimens (4%). In all seropositive patients acute appendicitis was confirmed by the pathology study; serous (33%) and purulent or gangrenous (67%). The latter incidence in the seronegative patients was 50%. There were found eight specimens (17%) negative for inflammation dealing all with seronegative patients. It seems that H. pylori colonizes the appendix in small proportion and is unlikely to be associated in direct correlation with acute appendicitis. However, seropositive patients with acute inflammation are likely to suffer from purulent or gangrenous form.

  16. Constipation Risk in Patients Undergoing Abdominal Surgery

    Science.gov (United States)

    Celik, Sevim; Atar, Nurdan Yalcin; Ozturk, Nilgun; Mendes, Guler; Kuytak, Figen; Bakar, Esra; Dalgiran, Duygu; Ergin, Sumeyra

    2015-01-01

    Background: Problems regarding bowel elimination are quite common in patients undergoing abdominal surgery. Objectives: To determine constipation risk before the surgery, bowel elimination during postoperative period, and the factors affecting bowel elimination. Patients and Methods: This is a cross-sectional study. It was conducted in a general surgery ward of a university hospital in Zonguldak, Turkey between January 2013 and May 2013. A total of 107 patients were included in the study, who were selected by convenience sampling. Constipation Risk Assessment Scale (CRAS), patient information form, medical and nursing records were used in the study. Results: The mean age of the patients was found to be 55.97 ± 15.74 (year). Most of the patients have undergone colon (37.4%) and stomach surgeries (21.5%). Open surgical intervention (83.2%) was performed on almost all patients (96.3%) under general anesthesia. Patients were at moderate risk for constipation with average scores of 11.71 before the surgery. A total of 77 patients (72%) did not have bowel elimination problem during postoperative period. The type of the surgery (P < 0.05), starting time for oral feeding after the surgery (P < 0.05), and mobilization (P < 0.05) were effective on postoperative bowel elimination. Conclusions: There is a risk for constipation after abdominal surgery. Postoperative practices are effective on the risk of constipation. PMID:26380107

  17. Synthetic methane for power storage

    NARCIS (Netherlands)

    Botta, G.; Barankin, Michael; Walspurger, S.

    2013-01-01

    With increased share of energy generated from variable renewable sources, storage becomes a critical issue to ensure constantly balanced supply/demand. Methane is a promising vector for energy storage and transport.

  18. Hydrogen Storage Technical Team Roadmap

    Energy Technology Data Exchange (ETDEWEB)

    None

    2013-06-01

    The mission of the Hydrogen Storage Technical Team is to accelerate research and innovation that will lead to commercially viable hydrogen-storage technologies that meet the U.S. DRIVE Partnership goals.

  19. Underground Storage Tanks in Iowa

    Data.gov (United States)

    Iowa State University GIS Support and Research Facility — Underground storage tank (UST) sites which store petroleum in Iowa. Includes sites which have been reported to DNR, and have active or removed underground storage...

  20. Anxiety Disorders

    Science.gov (United States)

    ... the death of a loved one or parents' divorce) and major life transitions (like moving to a ... Ways to Deal With Anxiety Dealing With Difficult Emotions Anxiety Disorders Posttraumatic Stress Disorder Fears and Phobias ...

  1. Bipolar Disorder

    Science.gov (United States)

    Bipolar disorder is a serious mental illness. People who have it go through unusual mood changes. They go ... The down feeling is depression. The causes of bipolar disorder aren't always clear. It runs in families. ...

  2. Mathematics disorder

    Science.gov (United States)

    ... this page: //medlineplus.gov/ency/article/001534.htm Mathematics disorder To use the sharing features on this page, please enable JavaScript. Mathematics disorder is a condition in which a child's ...

  3. Personality Disorders

    Science.gov (United States)

    Personality disorders are a group of mental illnesses. They involve long-term patterns of thoughts and behaviors ... serious problems with relationships and work. People with personality disorders have trouble dealing with everyday stresses and ...

  4. Cephalic Disorders

    Science.gov (United States)

    ... destructive lesions, but are sometimes the result of abnormal development. The disorder can occur before or after birth. Porencephaly most ... decade of life. SCHIZENCEPHALY is a rare developmental disorder characterized by abnormal slits, or clefts, in the cerebral hemispheres. Schizencephaly ...

  5. Oppositional defiant disorder

    Science.gov (United States)

    ... as possibilities: Anxiety disorders Attention-deficit/hyperactivity disorder (ADHD) Bipolar disorder Depression Learning disorders Substance abuse disorders Treatment The best treatment for the child is to ...

  6. Panic Disorder and Women

    Science.gov (United States)

    ... health illnesses Alcoholism, substance abuse, and addictive behavior Anxiety disorders Attention deficit hyperactivity disorder Bipolar disorder (manic depressive illness) Borderline personality disorder Depression Eating disorders Post-traumatic ...

  7. Eating Disorders

    OpenAIRE

    Gucciardi, Enza; Celasun, Nalan; Ahmad, Farah; Stewart, Donna E

    2004-01-01

    Abstract Health Issue Eating disorders are an increasing public health problem among young women. Anorexia and bulimia may give rise to serious physical conditions such as hypothermia, hypotension, electrolyte imbalance, endocrine disorders, and kidney failure. Key Issues Eating disorders are primarily a problem among women. In Ontario in 1995, over 90% of reported hospitalized cases of anorexia and bulimia were women. In addition to eating disorders, preoccupation with weight, body image and...

  8. Data storage as a service

    OpenAIRE

    Tomšič, Jan

    2016-01-01

    The purpose of the thesis was comparison of interfaces to network attached file systems and object storage. The thesis describes network file system and mounting procedure in Linux operating system. Object storage and distributed storage systems are explained with examples of usage. Amazon S3 is an example of object store with access trough REST interface. Ceph, a system for distributed object storage, is explained in detail, and a Ceph cluster was deployed for the purpose of this thesis. Cep...

  9. Mass storage for microprocessor farms

    International Nuclear Information System (INIS)

    Areti, H.

    1990-01-01

    Experiments in high energy physics require high density and high speed mass storage. Mass storage is needed for data logging during the online data acquisition, data retrieval and storage during the event reconstruction and data manipulation during the physics analysis. This paper examines the storage and speed requirements at the first two stages of the experiments and suggests a possible starting point to deal with the problem. 3 refs., 3 figs

  10. Guidebook on spent fuel storage

    International Nuclear Information System (INIS)

    1984-01-01

    The Guidebook summarizes the experience and information in various areas related to spent fuel storage: technological aspects, the transport of spent fuel, economical, regulatory and institutional aspects, international safeguards, evaluation criteria for the selection of a specific spent fuel storage concept, international cooperation on spent fuel storage. The last part of the Guidebook presents specific problems on the spent fuel storage in the United Kingdom, Sweden, USSR, USA, Federal Republic of Germany and Switzerland

  11. Radiation exposure from diagnostic imaging among patients with gastrointestinal disorders.

    LENUS (Irish Health Repository)

    Desmond, Alan N

    2012-03-01

    There are concerns about levels of radiation exposure among patients who undergo diagnostic imaging for inflammatory bowel disease (IBD), compared with other gastrointestinal (GI) disorders. We quantified imaging studies and estimated the cumulative effective dose (CED) of radiation received by patients with organic and functional GI disorders. We also identified factors and diagnoses associated with high CEDs.

  12. Bipolar Disorder.

    Science.gov (United States)

    Spearing, Melissa

    Bipolar disorder, a brain disorder that causes unusual shifts in a person's mood, affects approximately one percent of the population. It commonly occurs in late adolescence and is often unrecognized. The diagnosis of bipolar disorder is made on the basis of symptoms, course of illness, and when possible, family history. Thoughts of suicide are…

  13. Concrete storage cask for interim storage of spent nuclear fuel

    International Nuclear Information System (INIS)

    Nabemoto, Toyonobu; Fujiwara, Hiroaki; Kobayashi, Shunji; Shionaga, Ryosuke

    2004-01-01

    Experiments and analytical evaluation of the fabrication, non-destructive inspection and structural integrity of reinforced concrete body for storage casks were carried out to demonstrate the concrete storage cask for spent fuel generated from nuclear power plants. Analytical survey on the type of concrete material and fabrication method of the storage cask was performed and the most suitable fabrication method for the concrete body was identified to reduce concrete cracking. The structural integrity of the concrete body of the storage cask under load conditions during storage was confirmed and the long term integrity of concrete body against degradation dependent on environmental factors was evaluated. (author)

  14. Normal matter storage of antiprotons

    International Nuclear Information System (INIS)

    Campbell, L.J.

    1987-01-01

    Various simple issues connected with the possible storage of anti p in relative proximity to normal matter are discussed. Although equilibrium storage looks to be impossible, condensed matter systems are sufficiently rich and controllable that nonequilibrium storage is well worth pursuing. Experiments to elucidate the anti p interactions with normal matter are suggested. 32 refs

  15. Digital device for synchronous storage

    International Nuclear Information System (INIS)

    Kobzar', Yu.M.; Kovtun, V.G.; Pashechko, N.I.

    1991-01-01

    Synchronous storage digital device for IR electron-photon emission spectrometer operating with analogue-to-digital converter F4223 or monocrystal converter K572PV1 is described. The device accomplished deduction of noise-background in each storage cycle. Summation and deduction operational time equals 90 ns, device output code discharge - 20, number of storages -2 23

  16. Volatile liquid storage system

    International Nuclear Information System (INIS)

    Laverman, R.J.; Winters, P.J.; Rinehart, J.K.

    1992-01-01

    This patent describes a method of collecting and abating emission from a volatile liquid in an above ground storage tank. It comprises the liquid storage tank having a bottom, a vertical cylindrical circular wall having a lower edge portion joined to the bottom, and an external fixed roof, the tank having an internal floating roof floating on a volatile liquid stored in the tank, and air vent means in the tank in communication with a vapor space in the tank constituting at least the space above the floating roof when the floating roof floats on a predetermined maximum volume of volatile liquid in the tank; permitting ambient air; pumping emission laden air from the tank vapor space above the floating roof; and by means of the emissions abatement apparatus eliminating most of the emission from the emissions laden air with formation of a gaseous effluent and then discharging the resulting gaseous effluent to the atmosphere

  17. Superconducting magnetic energy storage

    International Nuclear Information System (INIS)

    Rogers, J.D.

    1976-01-01

    Fusion power production requires energy storage and transfer on short time scales to create confining magnetic fields and for heating plasmas. The theta-pinch Scyllac Fusion Test Reactor (SFTR) requires 480 MJ of energy to drive the 5-T compression field with a 0.7-ms rise time. Tokamak Experimental Power Reactors (EPR) require 1 to 2 GJ of energy with a 1 to 2-s rise time for plasma ohmic heating. The design, development, and testing of four 300-kJ energy storage coils to satisfy the SFTR needs are described. Potential rotating machinery and homopolar energy systems for both the Reference Theta-Pinch Reactor (RTPR) and tokamak ohmic-heating are presented

  18. Superconducting Magnetic Energy Storage

    International Nuclear Information System (INIS)

    Hassenzahl, W.

    1989-01-01

    Recent programmatic developments in Superconducting Magnetic Energy Storage (SMES) have prompted renewed and widespread interest in this field. In mid 1987 the Defense Nuclear Agency, acting for the Strategic Defense Initiative Office issued a request for proposals for the design and construction of SMES Engineering Test Model (ETM). Two teams, one led by Bechtel and the other by Ebasco, are now engaged in the first phase of the development of a 10 to 20 MWhr ETM. This report presents the rationale for energy storage on utility systems, describes the general technology of SMES, and explains the chronological development of the technology. The present ETM program is outlined; details of the two projects for ETM development are described in other papers in these proceedings. The impact of high Tc materials on SMES is discussed

  19. Entangled Cloud Storage

    DEFF Research Database (Denmark)

    Ateniese, Giuseppe; Dagdelen, Özgür; Damgård, Ivan Bjerre

    2012-01-01

    keeps the files in it private but still lets each client P_i recover his own data by interacting with S; no cooperation from other clients is needed. At the same time, the cloud provider is discouraged from altering or overwriting any significant part of c as this will imply that none of the clients can......Entangled cloud storage enables a set of clients {P_i} to “entangle” their files {f_i} into a single clew c to be stored by a (potentially malicious) cloud provider S. The entanglement makes it impossible to modify or delete significant part of the clew without affecting all files in c. A clew...... recover their files. We provide theoretical foundations for entangled cloud storage, introducing the notion of an entangled encoding scheme that guarantees strong security requirements capturing the properties above. We also give a concrete construction based on privacy-preserving polynomial interpolation...

  20. Optical information storage

    International Nuclear Information System (INIS)

    Woike, T.

    1996-01-01

    In order to increase storage capacity and data transfer velocity by about three orders of magnitude compared to CD or magnetic disc it is necessary to work with optical techniques, especially with holography. About 100 TByte can be stored in a waver of an area of 50 cm 2 via holograms which corresponds to a density of 2.10 9 Byte/mm 2 . Every hologram contains data of 1 MByte, so that parallel-processing is possible for read-out. Using high-speed CCD-arrays a read-out velocity of 1 MByte/μsec can be reached. Further, holographic technics are very important in solid state physics. We will discuss the existence of a space charge field in Sr 1-x Ba x Nb 2 O 6 doped with cerium and the physical properties of metastable states, which are suited for information storage. (author) 19 figs., 9 refs

  1. Speech disorders - children

    Science.gov (United States)

    ... disorder; Voice disorders; Vocal disorders; Disfluency; Communication disorder - speech disorder; Speech disorder - stuttering ... evaluation tools that can help identify and diagnose speech disorders: Denver Articulation Screening Examination Goldman-Fristoe Test of ...

  2. Alternatives for water basin spent fuel storage using pin storage

    International Nuclear Information System (INIS)

    Viebrock, J.M.; Carlson, R.W.

    1979-09-01

    The densest tolerable form for storing spent nuclear fuel is storage of only the fuel rods. This eliminates the space between the fuel rods and frees the hardware to be treated as non-fuel waste. The storage density can be as much as 1.07 MTU/ft 2 when racks are used that just satisfy the criticality and thermal limitations. One of the major advantages of pin storage is that it is compatible with existing racks; however, this reduces the storage density to 0.69 MTU/ft 2 . Even this is a substantial increase over the 0.39 MTU/ft 2 that is achievable with current high capacity stainless steel racks which have been selected as the bases for comparison. Disassembly requires extensive operation on the fuel assembly to remove the upper end fitting and to extract the fuel rods from the assembly skeleton. These operations will be performed with the aid of an elevator to raise the assembly where each fuel rod is grappled. Lowering the elevator will free the fuel rod for transfer to the storage canister. A storage savings of $1510 per MTU can be realized if the pin storage concept is incorporated at a new away-from-reactor facility. The storage cost ranges from $3340 to $7820 per MTU of fuel stored with the lower cost applying to storage at an existing away-from-reactor storage facility and the higher cost applying to at-reactor storage

  3. Energy Storage Project

    Science.gov (United States)

    Mercer, Carolyn R.; Jankovsky, Amy L.; Reid, Concha M.; Miller, Thomas B.; Hoberecht, Mark A.

    2011-01-01

    NASA's Exploration Technology Development Program funded the Energy Storage Project to develop battery and fuel cell technology to meet the expected energy storage needs of the Constellation Program for human exploration. Technology needs were determined by architecture studies and risk assessments conducted by the Constellation Program, focused on a mission for a long-duration lunar outpost. Critical energy storage needs were identified as batteries for EVA suits, surface mobility systems, and a lander ascent stage; fuel cells for the lander and mobility systems; and a regenerative fuel cell for surface power. To address these needs, the Energy Storage Project developed advanced lithium-ion battery technology, targeting cell-level safety and very high specific energy and energy density. Key accomplishments include the development of silicon composite anodes, lithiated-mixed-metal-oxide cathodes, low-flammability electrolytes, and cell-incorporated safety devices that promise to substantially improve battery performance while providing a high level of safety. The project also developed "non-flow-through" proton-exchange-membrane fuel cell stacks. The primary advantage of this technology set is the reduction of ancillary parts in the balance-of-plant--fewer pumps, separators and related components should result in fewer failure modes and hence a higher probability of achieving very reliable operation, and reduced parasitic power losses enable smaller reactant tanks and therefore systems with lower mass and volume. Key accomplishments include the fabrication and testing of several robust, small-scale nonflow-through fuel cell stacks that have demonstrated proof-of-concept. This report summarizes the project s goals, objectives, technical accomplishments, and risk assessments. A bibliography spanning the life of the project is also included.

  4. DPM: Future Proof Storage

    Science.gov (United States)

    Alvarez, Alejandro; Beche, Alexandre; Furano, Fabrizio; Hellmich, Martin; Keeble, Oliver; Rocha, Ricardo

    2012-12-01

    The Disk Pool Manager (DPM) is a lightweight solution for grid enabled disk storage management. Operated at more than 240 sites it has the widest distribution of all grid storage solutions in the WLCG infrastructure. It provides an easy way to manage and configure disk pools, and exposes multiple interfaces for data access (rfio, xroot, nfs, gridftp and http/dav) and control (srm). During the last year we have been working on providing stable, high performant data access to our storage system using standard protocols, while extending the storage management functionality and adapting both configuration and deployment procedures to reuse commonly used building blocks. In this contribution we cover in detail the extensive evaluation we have performed of our new HTTP/WebDAV and NFS 4.1 frontends, in terms of functionality and performance. We summarize the issues we faced and the solutions we developed to turn them into valid alternatives to the existing grid protocols - namely the additional work required to provide multi-stream transfers for high performance wide area access, support for third party copies, credential delegation or the required changes in the experiment and fabric management frameworks and tools. We describe new functionality that has been added to ease system administration, such as different filesystem weights and a faster disk drain, and new configuration and monitoring solutions based on the industry standards Puppet and Nagios. Finally, we explain some of the internal changes we had to do in the DPM architecture to better handle the additional load from the analysis use cases.

  5. Tuber Storage Proteins

    OpenAIRE

    SHEWRY, PETER R.

    2003-01-01

    A wide range of plants are grown for their edible tubers, but five species together account for almost 90 % of the total world production. These are potato (Solanum tuberosum), cassava (Manihot esculenta), sweet potato (Ipomoea batatus), yams (Dioscorea spp.) and taro (Colocasia, Cyrtosperma and Xanthosoma spp.). All of these, except cassava, contain groups of storage proteins, but these differ in the biological properties and evolutionary relationships. Thus, patatin from potato exhibits act...

  6. Large mass storage facility

    Energy Technology Data Exchange (ETDEWEB)

    Peskin, Arnold M.

    1978-08-01

    This is the final report of a study group organized to investigate questions surrounding the acquisition of a large mass storage facility. The programatic justification for such a system at Brookhaven is reviewed. Several candidate commercial products are identified and discussed. A draft of a procurement specification is developed. Some thoughts on possible new directions for computing at Brookhaven are also offered, although this topic was addressed outside of the context of the group's deliberations. 2 figures, 3 tables.

  7. Storage ring group summary

    International Nuclear Information System (INIS)

    King, N.M.

    1980-01-01

    The Storage Ring Group set out to identify and pursue salient problems in accelerator physics for heavy ion fusion, divorced from any particular reference design concept. However, it became apparent that some basic parameter framework was required to correlate the different study topics. As the Workshop progressed, ring parameters were modified and updated. Consequently, the accompanying papers on individual topics will be found to refer to slightly varied parameters, according to the stage at which the different problems were tackled

  8. Compact Holographic Data Storage

    Science.gov (United States)

    Chao, T. H.; Reyes, G. F.; Zhou, H.

    2001-01-01

    NASA's future missions would require massive high-speed onboard data storage capability to Space Science missions. For Space Science, such as the Europa Lander mission, the onboard data storage requirements would be focused on maximizing the spacecraft's ability to survive fault conditions (i.e., no loss in stored science data when spacecraft enters the 'safe mode') and autonomously recover from them during NASA's long-life and deep space missions. This would require the development of non-volatile memory. In order to survive in the stringent environment during space exploration missions, onboard memory requirements would also include: (1) survive a high radiation environment (1 Mrad), (2) operate effectively and efficiently for a very long time (10 years), and (3) sustain at least a billion write cycles. Therefore, memory technologies requirements of NASA's Earth Science and Space Science missions are large capacity, non-volatility, high-transfer rate, high radiation resistance, high storage density, and high power efficiency. JPL, under current sponsorship from NASA Space Science and Earth Science Programs, is developing a high-density, nonvolatile and rad-hard Compact Holographic Data Storage (CHDS) system to enable large-capacity, high-speed, low power consumption, and read/write of data in a space environment. The entire read/write operation will be controlled with electrooptic mechanism without any moving parts. This CHDS will consist of laser diodes, photorefractive crystal, spatial light modulator, photodetector array, and I/O electronic interface. In operation, pages of information would be recorded and retrieved with random access and high-speed. The nonvolatile, rad-hard characteristics of the holographic memory will provide a revolutionary memory technology meeting the high radiation challenge facing the Europa Lander mission. Additional information is contained in the original extended abstract.

  9. Disk Storage Server

    CERN Multimedia

    This model was a disk storage server used in the Data Centre up until 2012. Each tray contains a hard disk drive (see the 5TB hard disk drive on the main disk display section - this actually fits into one of the trays). There are 16 trays in all per server. There are hundreds of these servers mounted on racks in the Data Centre, as can be seen.

  10. Hoarding: From a Symptom to a Disorder

    Directory of Open Access Journals (Sweden)

    Suheyla Dogan Bulut

    2015-09-01

    Full Text Available Hoarding is a psychological disorder characterized by excessive collecting, storage and inability to discard large quantities of the objects, usually accompanying a severe level of distress or dysfunctionality. Despite the concept has been known for more than a century, it used to be conceptualized as a component of obsessive compulsive disorder. However, hoarding disorder appears as a distinct psychiatric disorder in the last updated version of the Diagnostic and Statistical Manual of Mental Disorders (DSM-5. Accordingly, in this review we aimed to make a general framework in understanding of hoarding disorder which is an attention-grabbing diagnosis in these days. In this regard, we addressed the etiology, clinical features of, and treatment approaches to hoarding disorder. [Psikiyatride Guncel Yaklasimlar - Current Approaches in Psychiatry 2015; 7(3.000: 319-332

  11. SXLS storage ring design

    International Nuclear Information System (INIS)

    Anon.

    1991-01-01

    X-ray lithography has emerged as a strong candidate to meet the demands of ever finer linewidths on integrated circuits, particularly for linewidths less than .25 microns. Proximity printing X-ray lithography makes use of soft X-rays to shadow print an image of a mask onto a semiconductor wafer to produce integrated circuits. To generate the required X-rays in sufficient quantities to make commercial production viable, electron storage rings have been proposed as the soft X-ray sources. Existing storage rings have been used to do the initial development work and the success of these efforts has led the lithographers to request that new rings be constructed that are dedicated to X-ray lithography. As a result of a series of workshops held at BNL [10.3] which were attended by both semiconductor and accelerator scientists, the following set of zeroth order specifications' on the light and electron beam of a storage ring for X-ray lithography were developed: critical wavelength of light: λ c = 6 to 10 angstroms, white light power: P = 0.25 to 2.5 watts/mrad, horizontal collection angle per port: θ = 10 to 50 mrad, electron beam sizes: σ x ∼ σ y y ' < 1 mrad

  12. Maui energy storage study.

    Energy Technology Data Exchange (ETDEWEB)

    Ellison, James; Bhatnagar, Dhruv; Karlson, Benjamin

    2012-12-01

    This report investigates strategies to mitigate anticipated wind energy curtailment on Maui, with a focus on grid-level energy storage technology. The study team developed an hourly production cost model of the Maui Electric Company (MECO) system, with an expected 72 MW of wind generation and 15 MW of distributed photovoltaic (PV) generation in 2015, and used this model to investigate strategies that mitigate wind energy curtailment. It was found that storage projects can reduce both wind curtailment and the annual cost of producing power, and can do so in a cost-effective manner. Most of the savings achieved in these scenarios are not from replacing constant-cost diesel-fired generation with wind generation. Instead, the savings are achieved by the more efficient operation of the conventional units of the system. Using additional storage for spinning reserve enables the system to decrease the amount of spinning reserve provided by single-cycle units. This decreases the amount of generation from these units, which are often operated at their least efficient point (at minimum load). At the same time, the amount of spinning reserve from the efficient combined-cycle units also decreases, allowing these units to operate at higher, more efficient levels.

  13. Underground storage tanks

    International Nuclear Information System (INIS)

    Anon.

    1993-01-01

    Environmental contamination from leaking underground storage tanks poses a significant threat to human health and the environment. An estimated five to six million underground storage tanks containing hazardous substances or petroleum products are in use in the US. Originally placed underground as a fire prevention measure, these tanks have substantially reduced the damages from stored flammable liquids. However, an estimated 400,000 underground tanks are thought to be leaking now, and many more will begin to leak in the near future. Products released from these leaking tanks can threaten groundwater supplies, damage sewer lines and buried cables, poison crops, and lead to fires and explosions. As required by the Hazardous and Solid Waste Amendments (HSWA), the EPA has been developing a comprehensive regulatory program for underground storage tanks. The EPA proposed three sets of regulations pertaining to underground tanks. The first addressed technical requirements for petroleum and hazardous substance tanks, including new tank performance standards, release detection, release reporting and investigation, corrective action, and tank closure. The second proposed regulation addresses financial responsibility requirements for underground petroleum tanks. The third addressed standards for approval of state tank programs

  14. Underground storage of heat

    International Nuclear Information System (INIS)

    Despois, J.; Nougarede, F.

    1976-01-01

    The interest laying in heat storage is envisaged taking account of the new energy context, with a view to optimizing the use of production means of heat sources hardly modulated according to the demand. In such a way, a natural medium, without any constructions cost but only an access cost is to be used. So, porous and permeable rocky strata allowing the use of a pressurized water flow as a transfer fluid are well convenient. With such a choice high temperatures (200 deg C) may be obtained, that are suitable for long transmissions. A mathematical model intended for solving the conservation equations in the case of heat storage inside a confined water layer is discussed. An approach of the operating conditions of storage may involve either a line-up arrangement (with the hot drilling at the center, the cold drillings being aligned on both sides) or a radial arrangement (with cold drillings at the peripheral edge encircling the hot drilling at the center of the layer). The three principal problems encountered are: starting drilling, and the circuit insulation and control [fr

  15. Mass storage at NSA

    Science.gov (United States)

    Shields, Michael F.

    1993-01-01

    The need to manage large amounts of data on robotically controlled devices has been critical to the mission of this Agency for many years. In many respects this Agency has helped pioneer, with their industry counterparts, the development of a number of products long before these systems became commercially available. Numerous attempts have been made to field both robotically controlled tape and optical disk technology and systems to satisfy our tertiary storage needs. Custom developed products were architected, designed, and developed without vendor partners over the past two decades to field workable systems to handle our ever increasing storage requirements. Many of the attendees of this symposium are familiar with some of the older products, such as: the Braegen Automated Tape Libraries (ATL's), the IBM 3850, the Ampex TeraStore, just to name a few. In addition, we embarked on an in-house development of a shared disk input/output support processor to manage our every increasing tape storage needs. For all intents and purposes, this system was a file server by current definitions which used CDC Cyber computers as the control processors. It served us well and was just recently removed from production usage.

  16. The Effects of Partial Turbinectomy on Patients Undergoing Rhinoplasty

    Directory of Open Access Journals (Sweden)

    Behrooz Gandomi

    2011-03-01

    Full Text Available Introduction: A reduction or cessation of the ability to breathe through the nose can affect a person's overall health. Nasal passage blockage can also result in snoring. Difficulty in breathing through the nose after rhinoplasty is a serious problem and patient dissatisfaction can be significant even when cosmetic results are excellent. Long-term impacts on the quality of life and contributions to the pathophysiology of sleep-related breathing disorders have both been documented. This study compares nasal obstruction and other prevalent respiratory side effects of rhinoplasty in the presence and absence of partial turbinectomy. Methods and Materials: This is an experimental case study conducted in 2007 on patients undergoing rhinoplastic surgery in Shahid Dastgheyb Hospital of Shiraz, Iran. Sampling was done on all admitted patients during this year in the mentioned hospital. For the first group, rhinoplasty plus partial turbinectomy was performed while taking into consideration the Helsinki criteria septorhinoplasty without partial turbinectomy was performed on the second group. P-value less than 0.05 was considered as statistically significant. Results: There was no significant difference between the case and control group in terms of their mean age and gender distribution. Respiratory mean scores before and after rhinoplasty in both groups were significantly different in some issues of Nasal Scale (i.e.: Nasal blockage or obstruction, trouble sleeping, inability to get enough air through the nose during exercise or exertion, trouble breathing through nose.  Conclusion: Achieving cosmetic goals while preserving such necessary functions is a great concern for rhinologists and this makes different complexes. To create a balance between the maximum physiologic function and the best cosmetic feature is an art.

  17. Adapting immunisation schedules for children undergoing chemotherapy.

    Science.gov (United States)

    Fernández-Prada, María; Rodríguez-Martínez, María; García-García, Rebeca; García-Corte, María Dolores; Martínez-Ortega, Carmen

    2018-02-01

    Children undergoing chemotherapy for cancer have special vaccination needs after completion of the treatment. The aim of this study was to evaluate the adaptation of post-chemotherapy vaccination schedules. An observational study was performed on a retrospective cohort that included all children aged from 0 to 14 years, who completed chemotherapy in a tertiary hospital between 2009 and 2015. Inclusion and exclusion criteria were applied. Immunisation was administered in accordance with the guidelines of the Vaccine Advisory Committee of the Spanish Association of Paediatrics. Primary Care immunisation and clinical records of the Preventive Medicine and Public Health Department were reviewed. Of the 99 children who had received chemotherapy, 51 (70.6% males) were included in the study. As regards the type of tumour, 54.9% had a solid organ tumour, and 45.1% had a haematological tumour. Post-chemotherapy immunisation was administered to 70.6%. The most common vaccines received were: diphtheria-tetanus-pertussis or diphtheria-tetanus (54.9%), meningococcus C (41.2%), and seasonal influenza (39.2%). The rate of adaptation of the immunisation schedule after chemotherapy was 9.8%. The pneumococcal conjugate vaccine against 7v or 13v was administered to 21.6% of study subjects. However, only 17.6% received polysaccharide 23v. None received vaccination against hepatitis A. No statistically significant differences were observed between adherence to immunisation schedules and type of tumour (P=.066), gender (P=.304), or age (P=.342). Post-chemotherapy immunisation of children with cancer is poor. The participation of health professionals in training programs and referral of paediatric cancer patients to Vaccine Units could improve the rate of schedule adaptation and proper immunisation of this population. Copyright © 2016 Elsevier España, S.L.U. and Sociedad Española de Enfermedades Infecciosas y Microbiología Clínica. All rights reserved.

  18. Preoperative information needs of children undergoing tonsillectomy.

    LENUS (Irish Health Repository)

    Buckley, Aoife

    2012-02-01

    AIMS AND OBJECTIVES: To identify the information needs of children undergoing tonsillectomy with reference to content of information, method of delivery, information providers and timing of information provision. BACKGROUND: Tonsillectomy can be anxiety provoking for children and preoperative preparation programmes are long recognised to reduce anxiety. However, few have been designed from the perspectives of children and to date little is known about how best to prepare children in terms of what to tell them, how to convey information to them, who can best provide information and what is the best timing for information provision. DESIGN: A qualitative descriptive study. METHOD: Data were collected from nine children (aged 6-9) using interviews supported by a write and draw technique. Data were coded and categorised into themes reflecting content, method, providers and timing of information. RESULTS: Children openly communicated their information needs especially on what to tell them to expect when facing a tonsillectomy. Their principal concerns were about operation procedures, experiencing \\'soreness\\' and discomfort postoperatively and parental presence. Mothers were viewed as best situated to provide them with information. Children were uncertain about what method of information and timing would be most helpful to them. CONCLUSION: Preoperative educational interventions need to take account of children\\'s information needs so that they are prepared for surgery in ways that are meaningful and relevant to them. Future research is needed in this area. RELEVANCE TO CLINICAL PRACTICE: Practical steps towards informing children about having a tonsillectomy include asking them what they need to know and addressing their queries accordingly. Child-centred information leaflets using a question and answer format could also be helpful to children.

  19. Bipolar disorders

    DEFF Research Database (Denmark)

    Vieta, Eduard; Berk, Michael; Schulze, Thomas G

    2018-01-01

    Bipolar disorders are chronic and recurrent disorders that affect >1% of the global population. Bipolar disorders are leading causes of disability in young people as they can lead to cognitive and functional impairment and increased mortality, particularly from suicide and cardiovascular disease...... and accurate diagnosis is difficult in clinical practice as the onset of bipolar disorder is commonly characterized by nonspecific symptoms, mood lability or a depressive episode, which can be similar in presentation to unipolar depression. Moreover, patients and their families do not always understand...... a bipolar disorder from other conditions. Optimal early treatment of patients with evidence-based medication (typically mood stabilizers and antipsychotics) and psychosocial strategies is necessary....

  20. Ripening, storage temperature, ethylene action, and oxidative stress alter apple peel phytosterol metabolism

    Science.gov (United States)

    The chilling conditions of apple cold storage can provoke an economically significant necrotic peel disorder called superficial scald (scald) in susceptible cultivars. Disorder development can be reduced by inhibiting ethylene action or oxidative stress. We found previously that scald is preceded b...

  1. SRS K-area material storage. Expanding capabilities

    International Nuclear Information System (INIS)

    Koenig, R.

    2013-01-01

    In support of the Department of Energy’s continued plans to de-inventory and reduce the footprint of Cold War era weapons’ material production sites, the K-Area Material Storage (KAMS) facility, located in the K-Area Complex (KAC) at the Savannah River Site reservation, has expanded since its startup authorization in 2000 to accommodate DOE’s material consolidation mission. During the facility’s growth and expansion, KAMS will have expanded its authorization capability of material types and storage containers to allow up to 8200 total shipping containers once the current expansion effort completes in 2014. Recognizing the need to safely and cost effectively manage other surplus material across the DOE Complex, KAC is constantly evaluating the storage of different material types within K area. When modifying storage areas in KAC, the Documented Safety Analysis (DSA) must undergo extensive calculations and reviews; however, without an extensive and proven security posture the possibility for expansion would not be possible. The KAC maintains the strictest adherence to safety and security requirements for all the SNM it handles. Disciplined Conduct of Operations and Conduct of Projects are demonstrated throughout this historical overview highlighting various improvements in capability, capacity, demonstrated cost effectiveness and utilization of the KAC as the DOE Center of Excellence for safe and secure storage of surplus SNM.

  2. Scalable cloud without dedicated storage

    Science.gov (United States)

    Batkovich, D. V.; Kompaniets, M. V.; Zarochentsev, A. K.

    2015-05-01

    We present a prototype of a scalable computing cloud. It is intended to be deployed on the basis of a cluster without the separate dedicated storage. The dedicated storage is replaced by the distributed software storage. In addition, all cluster nodes are used both as computing nodes and as storage nodes. This solution increases utilization of the cluster resources as well as improves fault tolerance and performance of the distributed storage. Another advantage of this solution is high scalability with a relatively low initial and maintenance cost. The solution is built on the basis of the open source components like OpenStack, CEPH, etc.

  3. Holographic Optical Data Storage

    Science.gov (United States)

    Timucin, Dogan A.; Downie, John D.; Norvig, Peter (Technical Monitor)

    2000-01-01

    Although the basic idea may be traced back to the earlier X-ray diffraction studies of Sir W. L. Bragg, the holographic method as we know it was invented by D. Gabor in 1948 as a two-step lensless imaging technique to enhance the resolution of electron microscopy, for which he received the 1971 Nobel Prize in physics. The distinctive feature of holography is the recording of the object phase variations that carry the depth information, which is lost in conventional photography where only the intensity (= squared amplitude) distribution of an object is captured. Since all photosensitive media necessarily respond to the intensity incident upon them, an ingenious way had to be found to convert object phase into intensity variations, and Gabor achieved this by introducing a coherent reference wave along with the object wave during exposure. Gabor's in-line recording scheme, however, required the object in question to be largely transmissive, and could provide only marginal image quality due to unwanted terms simultaneously reconstructed along with the desired wavefront. Further handicapped by the lack of a strong coherent light source, optical holography thus seemed fated to remain just another scientific curiosity, until the field was revolutionized in the early 1960s by some major breakthroughs: the proposition and demonstration of the laser principle, the introduction of off-axis holography, and the invention of volume holography. Consequently, the remainder of that decade saw an exponential growth in research on theory, practice, and applications of holography. Today, holography not only boasts a wide variety of scientific and technical applications (e.g., holographic interferometry for strain, vibration, and flow analysis, microscopy and high-resolution imagery, imaging through distorting media, optical interconnects, holographic optical elements, optical neural networks, three-dimensional displays, data storage, etc.), but has become a prominent am advertising

  4. Fuel performance in water storage

    International Nuclear Information System (INIS)

    Hoskins, A.P.; Scott, J.G.; Shelton-Davis, C.V.; McDannel, G.E.

    1993-11-01

    Westinghouse Idaho Nuclear Company operates the Idaho Chemical Processing Plant (ICPP) at the Idaho National Engineering Laboratory (INEL) for the Department of Energy (DOE). A variety of different types of fuels have been stored there since the 1950's prior to reprocessing for uranium recovery. In April of 1992, the DOE decided to end fuel reprocessing, changing the mission at ICPP. Fuel integrity in storage is now viewed as long term until final disposition is defined and implemented. Thus, the condition of fuel and storage equipment is being closely monitored and evaluated to ensure continued safe storage. There are four main areas of fuel storage at ICPP: an original underwater storage facility (CPP-603), a modern underwater storage facility (CPP-666), and two dry fuel storage facilities. The fuels in storage are from the US Navy, DOE (and its predecessors the Energy Research and Development Administration and the Atomic Energy Commission), and other research programs. Fuel matrices include uranium oxide, hydride, carbide, metal, and alloy fuels. In the underwater storage basins, fuels are clad with stainless steel, zirconium, and aluminum. Also included in the basin inventory is canned scrap material. The dry fuel storage contains primarily graphite and aluminum type fuels. A total of 55 different fuel types are currently stored at the Idaho Chemical Processing Plant. The corrosion resistance of the barrier material is of primary concern in evaluating the integrity of the fuel in long term water storage. The barrier material is either the fuel cladding (if not canned) or the can material

  5. Dry storage of Magnox fuel

    International Nuclear Information System (INIS)

    1986-09-01

    This work, commissioned by the CEGB, studies the feasibility of a combination of short-term pond storage and long-term dry storage of Magnox spent fuel as a cheaper alternative to reprocessing. Storage would be either at the reactor site or a central site. Two designs are considered, based on existing design work done by GEC-ESL and NNC; the capsule design developed by NNC and with storage in passive vaults for up to 100 yrs and the GEC-ESL tube design developed at Wylfa for the interim storage of LWR. For the long-term storage of Magnox spent fuel the GEC-ESL tubed vault all-dry storage method is recommended and specifications for this method are given. (U.K.)

  6. Developing new transportable storage casks for interim dry storage

    Energy Technology Data Exchange (ETDEWEB)

    Hayashi, K.; Iwasa, K.; Araki, K.; Asano, R. [Hitachi Zosen Diesel and Engineering Co., Ltd., Tokyo (Japan)

    2004-07-01

    Transportable storage metal casks are to be consistently used during transport and storage for AFR interim dry storage facilities planning in Japan. The casks are required to comply with the technical standards of regulations for both transport (hereinafter called ''transport regulation'') and storage (hereafter called ''storage regulation'') to maintain safety functions (heat transfer, containment, shielding and sub-critical control). In addition to these requirements, it is not planned in normal state to change the seal materials during storage at the storage facility, therefore it is requested to use same seal materials when the casks are transported after storage period. The dry transportable storage metal casks that satisfy the requirements have been developed to meet the needs of the dry storage facilities. The basic policy of this development is to utilize proven technology achieved from our design and fabrication experience, to carry out necessary verification for new designs and to realize a safe and rational design with higher capacity and efficient fabrication.

  7. Soil Quality in Mining Areas Undergoing Ecological Restoration

    Science.gov (United States)

    Dinarowski, Marcela; Casagrande, José Carlos; Bizuti, Denise T. G.; Silva, Luiz Gabriel; Soares, Marcio Roberto; Brancalion, Pedro H. S.

    2014-05-01

    Mining is one of the anthropogenic activities most impactful to natural resources, and can profoundly affect the resilience of ecosystems depending on the level of soil degradation. Ecological restoration has generated promising results even in situations of degradation as intense as those of mining. The aim of this study was to evaluate the quality of the soil in areas explored by the bauxite extraction undergoing restoration: recently mined, seven years, 20 years and native forest. The studied areas are located in the municipality of Poços de Caldas-MG, belonging to ALCOA Alumínio. The mined-out areas for seven and twenty years were uncompressed and received topsoil, liming and fertilization with nitrogen, phosphorus and potassium. Samples for chemical analyses of soil fertility were carried out at depths of 0-5, 5-10, 10-20, 20-40 and 40-60 cm. Soil quality was evaluated by pondered additive model. The parameters were considered organic matter (0.6) and bases saturation (0.4) for soil fertility function (0.6) and calcium (0.5) and aluminum saturation (0.5) for the function root development (0.4) - (the numbers in parentheses represent the weights attributed). Despite the high content, only the organic matter was not a parameter enough to classify the soil quality, once the native forest has very low base saturation (7%). The soil quality index(SQI) obtained allowed to classify the areas, being the first restored 20 years ago with SQI equal to 0.7 followed of the restored 7 years ago, native forest and newly mined with SQIs equal to 0.6, 04 and 0.3, respectively. The native tropical forests have low soil fertility, keeping by the cycling of nutrients. This demonstrates the need for the degraded areas, especially the mined, are uncompressed to allow storage of water and root development, in addition to the replacement of nutrients and soil acidity correction, especially high levels of aluminum saturation (66%) and low calcium (3 mmolcdm-3).

  8. Inflammation and metabolic disorders.

    Science.gov (United States)

    Navab, Mohamad; Gharavi, Nima; Watson, Andrew D

    2008-07-01

    Poor nutrition, overweight and obesity have increasingly become a public health concern as they affect many metabolic disorders, including heart disease, diabetes, digestive system disorders, and renal failure. Study of the effects of life style including healthy nutrition will help further elucidate the mechanisms involved in the adverse effects of poor nutrition. Unhealthy life style including poor nutrition can result in imbalance in our oxidation/redox systems. Lipids can undergo oxidative modification by lipoxygenases, cyclooxygenases, myeloperoxidase, and other enzymes. Oxidized phospholipids can induce inflammatory molecules in the liver and other organs. This can contribute to inflammation, leading to coronary heart disease, stroke, renal failure, inflammatory bowl disease, metabolic syndrome, bone and joint disorders, and even certain types of cancer. Our antioxidant and antiinflammatory defense mechanisms contribute to a balance between the stimulators and the inhibitors of inflammation. Beyond a point, however, these systems might be overwhelmed and eventually fail. High-density lipoprotein is a potent inhibitor of the formation of toxic oxidized lipids. High-density lipoprotein is also an effective system for stimulating the genes whose products are active in the removal, inactivation, and elimination of toxic lipids. Supporting the high-density lipoprotein function should help maintain the balance in these systems. It is hoped that the present report would elucidate some of the ongoing work toward this goal.

  9. Psychopathological profile and prevalence of dual pathology on patients with alcoholic dependence undergoing outpatient treatment.

    Science.gov (United States)

    García-Carretero, Miguel A; Novalbos-Ruiz, José P; Robles-Martínez, María; Jordán-Quintero, María A; O'Ferrall-González, Cristina

    2017-01-01

    Assess the prevalence of dual pathology in patients with alcohol dependence and describe the psychopathological profile of mental disorders, impulsiveness, ADHD presence and craving. It is a cross-sectional study about dual pathology, carried out on 102 patients undergoing outpatient treatment. The presence of dual pathology is established by means of the MINI-5 interview and the MCMI-III test; DSM-IV being used as the alcohol abuse criteria. Impulsiveness, ADHD presence, craving and quality of life were measured through SIS, ASRSv1, MACS and SF-36. The prevalence of dual pathology ranges from 45.1% to 80.4% according to MCMI-III and MINI-5, respectively. The most frequent pathologies are current major depressive episodes, followed by current generalized anxiety disorders, suicide risk and current dysthymia disorders; 73.2% of dual patients present a moderate and intense global score according to MACS, 56.1% got a meaningful score in impulsiveness according to SIS and 41.5% has highly consistent symptoms with ADHD. As regards quality of life, 53.7% of the sample had bad mental health. In the case of dual patients consuming other substances, 30% had a history of bipolar disorders and 10% had a high suicide risk. The prevalence of psychiatric comorbidity in patients with alcohol dependence undergoing outpatient treatment varies depending on the detection method, MINI being the one identifying a greater number of cases. More than half of dual patients present impulsive behavior, a bad mental health state and high craving levels. Special attention should be paid to dual patients consuming other substances.

  10. INTESTINAL MALROTATION IN PATIENTS UNDERGOING BARIATRIC SURGERY.

    Science.gov (United States)

    Vidal, Eduardo Arevalo; Rendon, Francisco Abarca; Zambrano, Trino Andrade; García, Yudoco Andrade; Viteri, Mario Ferrin; Campos, Josemberg Marins; Ramos, Manoela Galvão; Ramos, Almino Cardoso

    Intestinal malrotation is a rare congenital anomaly. In adults is very difficult to recognize due to the lack of symptoms. Diagnosis is usually incidental during surgical procedures or at autopsy. To review the occurrence and recognition of uneventful intestinal malrotation discovered during regular cases of bariatric surgeries. Were retrospectively reviewed the medical registry of 20,000 cases undergoing bariatric surgery, from January 2002 to January 2016, looking for the occurrence of intestinal malrotation and consequences in the intraoperative technique and immediate evolution of the patients. Five cases (0,025%) of intestinal malrotation were found. All of them were males, aging 45, 49, 37,52 and 39 years; BMI 35, 42, 49, 47 and 52 kg/m2, all of them with a past medical history of morbid obesity. The patient with BMI 35 kg/m2 suffered from type 2 diabetes also. All procedures were completed by laparoscopic approach, with no conversions. In one patient was not possible to move the jejunum to the upper abdomen in order to establish the gastrojejunostomy and a sleeve gastrectomy was performed. In another patient was not possible to fully recognize the anatomy due to bowel adhesions and a single anastomosis gastric bypass was preferred. No leaks or bleeding were identified. There were no perioperative complications. All patients were discharged 72 h after the procedure and no immediate 30-day complications were reported. Patients with malrotation can successfully undergo laparoscopic bariatric surgery. May be necessary changes in the surgical original strategy regarding the malrotation. Surgeons must check full abdominal anatomical condition prior to start the division of the stomach. Má-rotação intestinal é rara anomalia congênita em adultos de difícil reconhecimento devido à falta de sintomas. O diagnóstico é feito geralmente incidentalmente durante procedimentos cirúrgicos ou durante autópsia. Verificar a ocorrência e reconhecimento não eventual

  11. Mental disorders, brain disorders, neurodevelopmental disorders ...

    African Journals Online (AJOL)

    . Amongst DSM's most vocal 'insider' critics has been Thomas Insel, Director of the US National Institute of Mental Health. Insel has publicly criticised DSM's adherence to a symptom-based classification of mental disorder, and used the weight ...

  12. Energy Storage System

    Science.gov (United States)

    1996-01-01

    SatCon Technology Corporation developed the drive train for use in the Chrysler Corporation's Patriot Mark II, which includes the Flywheel Energy Storage (FES) system. In Chrysler's experimental hybrid- electric car, the hybrid drive train uses an advanced turboalternator that generates electricity by burning a fuel; a powerful, compact electric motor; and a FES that eliminates the need for conventional batteries. The FES system incorporates technology SatCon developed in more than 30 projects with seven NASA centers, mostly for FES systems for spacecraft attitude control and momentum recovery. SatCon will continue to develop the technology with Westinghouse Electric Corporation.

  13. Interim storage study report

    Energy Technology Data Exchange (ETDEWEB)

    Rawlins, J.K.

    1998-02-01

    High-level radioactive waste (HLW) stored at the Idaho Chemical Processing Plant (ICPP) in the form of calcine and liquid and liquid sodium-bearing waste (SBW) will be processed to provide a stable waste form and prepare the waste to be transported to a permanent repository. Because a permanent repository will not be available when the waste is processed, the waste must be stored at ICPP in an Interim Storage Facility (ISF). This report documents consideration of an ISF for each of the waste processing options under consideration.

  14. CLOUD STORAGE SERVICES

    OpenAIRE

    Yan, Cheng

    2017-01-01

    Cloud computing is a hot topic in recent research and applications. Because it is widely used in various fields. Up to now, Google, Microsoft, IBM, Amazon and other famous co partnership have proposed their cloud computing application. Look upon cloud computing as one of the most important strategy in the future. Cloud storage is the lower layer of cloud computing system which supports the service of the other layers above it. At the same time, it is an effective way to store and manage heavy...

  15. Compact electron storage rings

    International Nuclear Information System (INIS)

    Williams, G.P.

    1987-01-01

    There have been many recent developments in the area of compact storage rings. Such rings would have critical wavelengths of typically 10 A, achieved with beam energies of several hundreds of MeV and superconducting dipole fields of around 5 Tesla. Although the primary motivation for progress in this area is that of commercial x-ray lithography, such sources might be an attractive source for college campuses to operate. They would be useful for many programs in materials science, solid state, x-ray microscopy and other biological areas. We discuss the properties of such sources and review developments around the world, primarily in the USA, japan and W. Germany

  16. SALES, STORAGE AND SALVAGE

    CERN Multimedia

    Division SPL, groupe logistique; A. Notar

    2000-01-01

    From 3 January 2000 there will be a security barrier in front of the storage-recycling area in bldg 133, which will be accessible only to authorised staff and contractors.You are reminded that the equipment delivered to this area must be unpolluted and non-radioactive. The cost of recycling the equipment will be debited to the budget code of the Division concerned, with the prior approval of the Group Leader.Reminder relating to equipment salesThe Sales Section is open on Thursdays from 13.30 to 15.00 hours only.SPL DivisionLogistics GroupA. Notari

  17. Energy storage financing :

    Energy Technology Data Exchange (ETDEWEB)

    Baxter, Richard

    2016-08-01

    Project financing is emerging as the linchpin for the future health, direction, and momentum of the energy storage industry. Market leaders have so far relied on selffunding or captive lending arrangements to fund projects. New lenders are proceeding hesitantly as they lack a full understanding of the technology, business, and credit risks involved in this rapidly changing market. The U.S. Department of Energy is poised to play a critical role in expanding access to capital by reducing the barriers to entry for new lenders, and providing trusted analytical benchmarks to better judge and price the risk in systematic ways.

  18. Interim storage report

    International Nuclear Information System (INIS)

    Rawlins, J.K.

    1998-02-01

    High-level radioactive waste (HLW) stored at the Idaho Chemical Processing Plant (ICPP) in the form of calcine and liquid and liquid sodium-bearing waste (SBW) will be processed to provide a stable waste form and prepare the waste to be transported to a permanent repository. Because a permanent repository will not be available when the waste is processed, the waste must be stored at ICPP in an Interim Storage Facility (ISF). This report documents consideration of an ISF for each of the waste processing options under consideration

  19. TEXT Energy Storage System

    International Nuclear Information System (INIS)

    Weldon, W.F.; Rylander, H.G.; Woodson, H.H.

    1977-01-01

    The Texas Experimental Tokamak (TEXT) Enery Storage System, designed by the Center for Electromechanics (CEM), consists of four 50 MJ, 125 V homopolar generators and their auxiliaries and is designed to power the toroidal and poloidal field coils of TEXT on a two-minute duty cycle. The four 50 MJ generators connected in series were chosen because they represent the minimum cost configuration and also represent a minimal scale up from the successful 5.0 MJ homopolar generator designed, built, and operated by the CEM

  20. Magnesium for Hydrogen Storage

    DEFF Research Database (Denmark)

    Pedersen, Allan Schrøder; Kjøller, John; Larsen, B.

    1983-01-01

    A study of the hydrogenation characteristics of fine magnesium powder during repeated cycling has been performed using a high-pressure microbalance facility. No effect was found from the cycling regarding kinetics and storage capacity. The reaction rate of the absorption process was fast...... at temperatures around 600 K and above, but the reversed reaction showed somewhat slower kinetics around 600 K. At higher temperatures the opposite was found. The enthalpy and entropy change by the hydrogenation, derived from pressure-concentration isotherms, agree fairly well with those reported earlier....

  1. Plutonium storage study

    International Nuclear Information System (INIS)

    1979-01-01

    This Spanish study gives a more detailed analysis of a possible store for plutonium oxide. The capacity of the store is assumed to be 30 t Pu and the minimum storage time 2 years. The study includes a general description of the store and its design philosophy; comments on the quality and properties of the material stored; a detailed criticality study and comments on gas and heat generation and shielding requirements; and a brief cost evaluation. Costs are estimated to be about $110/kg PuO 2 /year

  2. Gas Storage Technology Consortium

    Energy Technology Data Exchange (ETDEWEB)

    Joel Morrison; Elizabeth Wood; Barbara Robuck

    2010-09-30

    The EMS Energy Institute at The Pennsylvania State University (Penn State) has managed the Gas Storage Technology Consortium (GSTC) since its inception in 2003. The GSTC infrastructure provided a means to accomplish industry-driven research and development designed to enhance the operational flexibility and deliverability of the nation's gas storage system, and provide a cost-effective, safe, and reliable supply of natural gas to meet domestic demand. The GSTC received base funding from the U.S. Department of Energy's (DOE) National Energy Technology Laboratory (NETL) Oil & Natural Gas Supply Program. The GSTC base funds were highly leveraged with industry funding for individual projects. Since its inception, the GSTC has engaged 67 members. The GSTC membership base was diverse, coming from 19 states, the District of Columbia, and Canada. The membership was comprised of natural gas storage field operators, service companies, industry consultants, industry trade organizations, and academia. The GSTC organized and hosted a total of 18 meetings since 2003. Of these, 8 meetings were held to review, discuss, and select proposals submitted for funding consideration. The GSTC reviewed a total of 75 proposals and committed co-funding to support 31 industry-driven projects. The GSTC committed co-funding to 41.3% of the proposals that it received and reviewed. The 31 projects had a total project value of $6,203,071 of which the GSTC committed $3,205,978 in co-funding. The committed GSTC project funding represented an average program cost share of 51.7%. Project applicants provided an average program cost share of 48.3%. In addition to the GSTC co-funding, the consortium provided the domestic natural gas storage industry with a technology transfer and outreach infrastructure. The technology transfer and outreach were conducted by having project mentoring teams and a GSTC website, and by working closely with the Pipeline Research Council International (PRCI) to

  3. Solar energy storage

    CERN Document Server

    Sorensen, Bent

    2015-01-01

    While solar is the fastest-growing energy source in the world, key concerns around solar power's inherent variability threaten to de-rail that scale-up . Currently, integration of intermittent solar resources into the grid creates added complication to load management, leading some utilities to reject it altogether, while other operators may penalize the producers via rate increases or force solar developers to include storage devices on-site to smooth out power delivery at the point of production. However these efforts at mitigation unfold, it is increasingly clear to parties on all sides th

  4. Monitored retrievable storage status

    International Nuclear Information System (INIS)

    Carlson, J.H.

    1985-01-01

    The Department of Energy (DOE) is preparing a proposal for Congressional consideration of the inclusion of a monitored retrievable storage (MRS) facility as part of the Federal Nuclear Waste Management System. The DOE plans to submit the proposal package to the Congress by January 15, 1986. The proposed preferred location of the MRS facility is the former Clinch River Breeder site in the state of Tennessee. If the Congress approves implementation of the MRS proposal, it is estimated that the facility could be licensed and operational in ten years

  5. Regulated underground storage tanks

    International Nuclear Information System (INIS)

    1992-06-01

    This guidance package is designed to assist DOE Field operations by providing thorough guidance on the underground storage tank (UST) regulations. [40 CFR 280]. The guidance uses tables, flowcharts, and checklists to provide a ''roadmap'' for DOE staff who are responsible for supervising UST operations. This package is tailored to address the issues facing DOE facilities. DOE staff should use this guidance as: An overview of the regulations for UST installation and operation; a comprehensive step-by-step guidance for the process of owning and operating an UST, from installation to closure; and a quick, ready-reference guide for any specific topic concerning UST ownership or operation

  6. Heavy ion storage rings

    International Nuclear Information System (INIS)

    Schuch, R.

    1987-01-01

    A brief overview of synchrotron storage rings for heavy ions, which are presently under construction in different accelerator laboratories is given. Ions ranging from protons up to uranium ions at MeV/nucleon energies will be injected into these rings using multiturn injection from the accelerators available or being built in these laboratories. After injection, it is planned to cool the phase space distribution of the ions by merging them with cold electron beams or laser beams, or by using stochastic cooling. Some atomic physics experiments planned for these rings are presented. 35 refs

  7. Storage array reflection considerations

    International Nuclear Information System (INIS)

    Haire, M.J.; Jordan, W.C.; Taylor, R.G.

    1997-01-01

    The assumptions used for reflection conditions of single containers are fairly well established and consistently applied throughout the industry in nuclear criticality safety evaluations. Containers are usually considered to be either fully water-reflected (i.e. surrounded by 6 to 12 in. of water) for safety calculations or reflected by 1 in. of water for nominal (structural material and air) conditions. Tables and figures are usually available for performing comparative evaluations of containers under various loading conditions. Reflection considerations used for evaluating the safety of storage arrays of fissile material are not as well established

  8. Femtoslicing in Storage Rings

    CERN Document Server

    Khan, Shaukat

    2005-01-01

    The generation of ultrashort synchrotron radiation pulses by laser-induced energy modulation of electrons and their subsequent transverse displacement, now dubbed "femtoslicing," was demonstrated at the Advanced Light Source in Berkeley. More recently, a femtoslicing user facility was commissioned at the BESSY storage ring in Berlin, and another project is in progress at the Swiss Light Source. The paper reviews the principle of femtoslicing, its merits and shortcomings, as well as the variations of its technical implementation. Various diagnostics techniques to detect successful laser-electron interaction are discussed and experimental results are presented.

  9. 78 FR 35272 - Agency Forms Undergoing Paperwork Reduction Act Review

    Science.gov (United States)

    2013-06-12

    ...); sickle cell disease (SCD); attention-deficit/hyperactivity disorder (ADHD); and Tourette syndrome. Birth... blood disorders. Identified priority diseases, disorders, and conditions included in this information collection activity include but are not limited to preconception health; autism spectrum disorders (ASDs) and...

  10. [Eating disorders].

    Science.gov (United States)

    Miyake, Yoshie; Okamoto, Yuri; Jinnin, Ran; Shishida, Kazuhiro; Okamoto, Yasumasa

    2015-02-01

    Eating disorders are characterized by aberrant patterns of eating behavior, including such symptoms as extreme restriction of food intake or binge eating, and severe disturbances in the perception of body shape and weight, as well as a drive for thinness and obsessive fears of becoming fat. Eating disorder is an important cause for physical and psychosocial morbidity in young women. Patients with eating disorders have a deficit in the cognitive process and functional abnormalities in the brain system. Recently, brain-imaging techniques have been used to identify specific brain areas that function abnormally in patients with eating disorders. We have discussed the clinical and cognitive aspects of eating disorders and summarized neuroimaging studies of eating disorders.

  11. Cephalic Disorders

    Science.gov (United States)

    ... information sheet compiled by the National Institute of Neurological Disorders and Stroke (NINDS). Patient Organizations Birth Defect Research for Children, Inc. 976 Lake Baldwin Lane Suite 104 Orlando ...

  12. Radioactive gas storage device

    International Nuclear Information System (INIS)

    Seki, Eiji; Kobayashi, Yoshihiro.

    1989-01-01

    The present invention concerns a device of ionizing radioactive gases to be processed in gaseous nuclear fission products in nuclear fuel reprocessing plants, etc., and injecting them into metal substrates for storage. The device comprises a vessel for a tightly closed type outer electrode in which gases to be processed are introduced, an electrode disposed to the inside of the vessel and the target material, a high DC voltage power source for applying high voltage to the electrodes, etc. There are disposed a first electric discharging portion for preparting discharge plasma for ion injection of different electrode distance and a second electric discharging portion for causing stable discharge between the vessel and the electrode. The first electric discharging portion for the ion injection provides an electrode distance suitable to acceleration sputtering and the second electric discharging portion is used for stable discharge. Accordingly, if the gas pressure in the radioactive gas storage device is reduced by the external disturbance, etc., since the second electric discharging portion satisfies the electric discharging conditions, the device can continue electric discharge. (K.M.)

  13. Flywheel energy storage workshop

    Energy Technology Data Exchange (ETDEWEB)

    O`Kain, D.; Carmack, J. [comps.

    1995-12-31

    Since the November 1993 Flywheel Workshop, there has been a major surge of interest in Flywheel Energy Storage. Numerous flywheel programs have been funded by the Advanced Research Projects Agency (ARPA), by the Department of Energy (DOE) through the Hybrid Vehicle Program, and by private investment. Several new prototype systems have been built and are being tested. The operational performance characteristics of flywheel energy storage are being recognized as attractive for a number of potential applications. Programs are underway to develop flywheels for cars, buses, boats, trains, satellites, and for electric utility applications such as power quality, uninterruptible power supplies, and load leveling. With the tremendous amount of flywheel activity during the last two years, this workshop should again provide an excellent opportunity for presentation of new information. This workshop is jointly sponsored by ARPA and DOE to provide a review of the status of current flywheel programs and to provide a forum for presentation of new flywheel technology. Technology areas of interest include flywheel applications, flywheel systems, design, materials, fabrication, assembly, safety & containment, ball bearings, magnetic bearings, motor/generators, power electronics, mounting systems, test procedures, and systems integration. Information from the workshop will help guide ARPA & DOE planning for future flywheel programs. This document is comprised of detailed viewgraphs.

  14. Stationary flywheel energy storage

    Energy Technology Data Exchange (ETDEWEB)

    Gilhaus, A; Hau, E; Gassner, G; Huss, G; Schauberger, H

    1981-01-01

    The aim of this system study is to find out industrial applications of stationary flywheel energy accumulators. The economic value for the consumer and the effects on the power supply grid are investigated. Up to now, stationary flywheel energy accumulators have only been used in a small range. The main reason for thinking of the application in a wider range was the hope that those could be used economically for lowering the maximum output demand of the power supply grid. The possible savings in energy costs, however, proved to be too small for paying back the investment costs. Further benefits are necessary for advantageous application. As to overall economy, compensation of short time maximum power output seems to be more favorable at the power stations. An additional possibility for energy storage by flywheels is given where otherwise lost energy can be used effectively, according to the successful brake energy storage in vehicles. Under this aspect the future use of flywheels in wind-power-plants seems to be promising. Attractive savings of energy can be obtained by introducing modern flywheel technology for emergency power supply units which are employed for instance in telecommunication systems. Especially the application for emergency power supply, in power stations and in combustion with wind energy converters need further investigation.

  15. Nuclear fuel storage

    International Nuclear Information System (INIS)

    Bevilacqua, F.

    1981-01-01

    A nuclear fuel storage apparatus for use in a water-filled pool is fabricated of a material such as stainless steel in the form of an egg crate structure having vertically extending openings. Fuel may be stored in this basic structure in a checkerboard pattern with high enrichment fuel, or in all openings when the fuel is of low effective enrichment. Inserts of a material such as stainless steel are adapted to fit within these openings so that a water gap and, therefore, a flux trap is formed between adjacent fuel storage locations. These inserts may be added at a later time and fuel of a higher enrichment may be stored in each opening. When it is desired to store fuel of still greater enrichment, poison plates may be added to the water gap formed by the installed insert plates, or substituted for the insert plates. Alternately, or in addition, fuel may be installed in high neutron absorption poison boxes which surround the fuel assembly. The stainless steel inserts and the poison plates are each not required until the capacity of the basic egg crate structure is approached. Purchase of these items can, therefore, be deferred for many years. Should the fuel to be stored be of higher enrichment than initially forecast, the deferred decision on the poison plates makes it possible to obtain increased poison in the plates to satisfy the newly discovered requirement

  16. Storage array reflection considerations

    International Nuclear Information System (INIS)

    Haire, M.J.; Jordan, W.C.; Taylor, R.G.

    1997-01-01

    The assumptions used for reflection conditions of single containers are fairly well established and consistently applied throughout the industry in nuclear criticality safety evaluations. Containers are usually considered to be either fully water reflected (i.e., surrounded by 6 to 12 in. of water) for safety calculations or reflected by 1 in. of water for nominal (structural material and air) conditions. Tables and figures are usually available for performing comparative evaluations of containers under various loading conditions. Reflection considerations used for evaluating the safety of storage arrays of fissile material are not as well established. When evaluating arrays, it has become more common for analysts to use calculations to demonstrate the safety of the array configuration. In performing these calculations, the analyst has considerable freedom concerning the assumptions made for modeling the reflection of the array. Considerations are given for the physical layout of the array with little or no discussion (or demonstration) of what conditions are bounded by the assumed reflection conditions. For example, an array may be generically evaluated by placing it in a corner of a room in which the opposing walls are far away. Typically, it is believed that complete flooding of the room is incredible, so the array is evaluated for various levels of water mist interspersed among array containers. This paper discusses some assumptions that are made regarding storage array reflection

  17. Spent fuel interim storage

    International Nuclear Information System (INIS)

    Bilegan, Iosif C.

    2003-01-01

    The official inauguration of the spent fuel interim storage took place on Monday July 28, 2003 at Cernavoda NNP. The inaugural event was attended by local and central public authority representatives, a Canadian Government delegation as well as newsmen from local and central mass media and numerous specialists from Cernavoda NPP compound. Mr Andrei Grigorescu, State Secretary with the Economy and Commerce Ministry, underlined in his talk the importance of this objective for the continuous development of nuclear power in Romania as well as for Romania's complying with the EU practice in this field. Also the excellent collaboration between the Canadian contractor AECL and the Romanian partners Nuclear Montaj, CITON, UTI, General Concret in the accomplishment of this unit at the planned terms and costs. On behalf of Canadian delegation, spoke Minister Don Boudria. He underlined the importance which the Canadian Government affords to the cooperation with Romania aiming at specific objectives in the field of nuclear power such as the Cernavoda NPP Unit 2 and spent fuel interim storage. After traditional cutting of the inaugural ribbon by the two Ministers the festivities continued on the Cernavoda NPP Compound with undersigning the documents regarding the project completion and a press conference

  18. Optical information storage

    Energy Technology Data Exchange (ETDEWEB)

    Woike, T [Koeln Univ., Inst. fuer Kristallography, Koeln (Germany)

    1996-11-01

    In order to increase storage capacity and data transfer velocity by about three orders of magnitude compared to CD or magnetic disc it is necessary to work with optical techniques, especially with holography. About 100 TByte can be stored in a waver of an area of 50 cm{sup 2} via holograms which corresponds to a density of 2.10{sup 9} Byte/mm{sup 2}. Every hologram contains data of 1 MByte, so that parallel-processing is possible for read-out. Using high-speed CCD-arrays a read-out velocity of 1 MByte/{mu}sec can be reached. Further, holographic technics are very important in solid state physics. We will discuss the existence of a space charge field in Sr{sub 1-x}Ba{sub x}Nb{sub 2}O{sub 6} doped with cerium and the physical properties of metastable states, which are suited for information storage. (author) 19 figs., 9 refs.

  19. Spent fuel storage rack

    International Nuclear Information System (INIS)

    Kurokawa, Hideaki; Kumagaya, Naomi; Oda, Masashi; Matsuda, Masami; Maruyama, Hiromi; Yamanaka, Tsuneyasu.

    1997-01-01

    The structure of a spent fuel storage rack is determined by the material, thickness, size of square cylindrical tubes (the gap between spent fuel assemblies and the square cylindrical tubes) and pitch of the arrangement (the gap between each of the square cylindrical tubes). In the present invention, the thickness and the pitch of the arrangement of the square tubes are optimized while evaluating subcriticality. Namely, when the sum of the thickness of the water gap at the outer side (the pitch of arrangement of the cylindrical tubes) and the thickness of the cylindrical tubes is made constant, the storage rack is formed by determining the thickness of the cylindrical tubes which is smaller than the optimum value among the combination of the thickness of the water gap at the outer side and that of the cylindrical tube under the effective multiplication factor to be performed. Then, the weight of the rack can be reduced, and the burden of the load on the bottom of the pool can be reduced. Further, the amount of the constitutional materials of the rack itself can be reduced thereby capable of reducing the cost for the materials of the rack. (T.M.)

  20. Magnetic energy storage

    International Nuclear Information System (INIS)

    Rogers, J.D.

    1980-01-01

    The fusion program embraces low loss superconductor strand development with integration into cables capable of carrying 50 kA in pulsed mode at high fields. This evolvement has been paralleled with pulsed energy storage coil development and testing from tens of kJ at low fields to a 20 MJ prototype tokamak induction coil at 7.5 T. Energy transfer times have ranged from 0.7 ms to several seconds. Electric utility magnetic storage for prospective application is for diurnal load leveling with massive systems to store 10 GWh at 1.8 K in a dewar structure supported on bedrock underground. An immediate utility application is a 30 MJ system to be used to damp power oscillations on the Bonneville Power Administration electric transmission lines. An off-shoot of this last work is a new program for electric utility VAR control with the potential for use to suppress subsynchronous resonance. This paper presents work in progress, work planned, and recently completed unusual work

  1. Underground storage tank program

    International Nuclear Information System (INIS)

    Lewis, M.W.

    1994-01-01

    Underground storage tanks, UST'S, have become a major component of the Louisville District's Environmental Support Program. The District's Geotechnical and Environmental Engineering Branch has spear-headed an innovative effort to streamline the time, effort and expense for removal, replacement, upgrade and associated cleanup of USTs at military and civil work installations. This program, called Yank-A-Tank, creates generic state-wide contracts for removal, remediation, installation and upgrade of storage tanks for which individual delivery orders are written under the basic contract. The idea is to create a ''JOC type'' contract containing all the components of work necessary to remove, reinstall or upgrade an underground or above ground tank. The contract documents contain a set of generic specifications and unit price books in addition to the standard ''boiler plate'' information. Each contract requires conformance to the specific regulations for the state in which it is issued. The contractor's bid consists of a bid factor which in the multiplier used with the prices in the unit price book. The solicitation is issued as a Request for Proposal (RPP) which allows the government to select a contractor based on technical qualification an well as bid factor. Once the basic contract is awarded individual delivery orders addressing specific areas of work are scoped, negotiated and awarded an modifications to the original contract. The delivery orders utilize the prepriced components and the contractor's factor to determine the value of the work

  2. Physalis peruviana seed storage

    Directory of Open Access Journals (Sweden)

    Cíntia L. M. de Souza

    2016-03-01

    Full Text Available ABSTRACT Physalis peruviana belongs to Solanaceae family and has a high nutritional and nutraceutical potential. The production is intended for fruit consumption and the propagation is mainly by seeds. This study aimed to evaluate the influence of priming on the kinetics of germination of P. peruviana seeds stored at different temperatures. The seeds were stored at 5 and 25 °C in a chamber saturated with zinc chloride solution and in liquid nitrogen (-196 °C. Every 4 months, the seeds were removed from storage for evaluation of germination and moisture content in the laboratory and emergence and development of seedlings in greenhouse. During the last evaluation at 16 months, the seeds under the same conditions were subjected to salt stress. The moisture content varied during the storage period, but was always higher for seeds kept at -196 ºC. These seeds kept high germination percentage in water until 16 months, regardless of the tested temperature; however, in salt solution the germination percentage was significantly reduced.

  3. Bioética da proteção e tratamento de doenças genéticas raras no Brasil: o caso das doenças de depósito lisossomal Principle of protection and treatment of rare genetic diseases in Brazil: the case of lysosomal storage disorders

    Directory of Open Access Journals (Sweden)

    Raquel Boy

    2009-06-01

    Full Text Available Este trabalho tem como objetivo discutir a moralidade do financiamento público das drogas órfãs, de altíssimo custo, para o tratamento de doenças genéticas raras, utilizando as ferramentas da Bioética, em especial o princípio da proteção, aplicável a indivíduos e populações vulneradas. Com base neste princípio, e considerando o contexto normativo constituído pelo Sistema Único de Saúde (SUS, argumenta-se sobre a obrigação moral do Estado de prover políticas públicas que assistam ao indivíduo portador de uma doença genética - como a de depósito lisossômico - e que pode, portanto, ser considerado, "vulnerado", bem como são sugeridas medidas que possam implementar e dar sustentabilidade a tais políticas com ênfase em questões de alocação de recursos, focalização e equanimidade.This study aimed to discuss the morality of public funding for highly expensive orphan drugs for treatment of rare genetic diseases, using tools from bioethics, especially the principle of protection, applicable to vulnerable individuals and populations. Based on this principle, and considering the provisions of the Unified National Health System (SUS, the article argues for the state's moral obligation to provide public policies to ensure care for individuals with genetic diseases like lysosomal storage disorders, who can thus be viewed as "injured", besides suggesting measures to implement and ensure the sustainability of policies with an emphasis on resource allocation, targeting, and equity.

  4. Nanoparticles for heat transfer and thermal energy storage

    Science.gov (United States)

    Singh, Dileep; Cingarapu, Sreeram; Timofeeva, Elena V.; Moravek, Michael

    2015-07-14

    An article of manufacture and method of preparation thereof. The article of manufacture and method of making the article includes an eutectic salt solution suspensions and a plurality of nanocrystalline phase change material particles having a coating disposed thereon and the particles capable of undergoing the phase change which provides increase in thermal energy storage. In addition, other articles of manufacture can include a nanofluid additive comprised of nanometer-sized particles consisting of copper decorated graphene particles that provide advanced thermal conductivity to heat transfer fluids.

  5. Photodimerization in dipeptides for high capacity optical digital storage

    DEFF Research Database (Denmark)

    Ramanujam, P.S.; Berg, R.H.

    2004-01-01

    We have developed peptide materials with chromophores that undergo cycloaddition, suitable for terabit optical digital storage in a 5.25 in. disc. The rationale behind this design is that the length and rigidity of the backbone can be adjusted to facilitate the formation of a photodimer without...... large physical movements of the chromophores on exposure to UV light. Initially strongly absorbing films transmit up to 50% of light on irradiation at dimerizing wavelengths. This property can be utilized to record grey levels. An intensity-dependent transmission behavior has been observed that may...

  6. Cortisol levels and sleep patterns in infants with orofacial clefts undergoing surgery

    Directory of Open Access Journals (Sweden)

    Mueller AA

    2014-10-01

    Full Text Available Andreas A Mueller,1,2 Nadeem Kalak,3 Katja Schwenzer-Zimmerer,1,2 Edith Holsboer-Trachsler,3 Serge Brand3,4 1Craniomaxillofacial Surgery, University of Basel and University Hospital of Basel, Basel, Switzerland; 2Hightech Research Center of Craniomaxillofacial Surgery, University of Basel, Basel, Switzerland; 3Psychiatric Clinics of the University of Basel, Center for Affective, Stress, and Sleep Disorders, Basel, Switzerland; 4Department of Sport and Health Science, Division of Sport Science, University of Basel, Basel, Switzerland Background: Traumatic events during early infancy might damage infants’ psychobiological functioning, such as sleep and cortisol secretion. Infants born with orofacial clefts (OFCs undergo functional, anatomical, and aesthetic surgery. The aim of the present study was to determine whether infants with OFC and undergoing OFC surgery show deteriorated sleep and cortisol secretion compared with healthy controls and with their presurgery status.Methods: A total of 27 infants with OFC (mean age: 22 weeks and 30 healthy controls (mean age: 23 weeks took part in the study. For infants with OFC, sleep actigraphy was performed and saliva cortisol was analyzed 5 days before, during, and 5 days after surgery. For controls, sleep and saliva cortisol were assessed similarly, except for the period taken up with surgery.Results: Compared with healthy controls, infants with OFC undergoing OFC surgery did not differ in sleep and cortisol secretion. Their sleep and cortisol secretion did deteriorate during the perisurgical period but recovered 5 days postsurgery. Conclusion: In infants with OFC undergoing corrective surgery, the pattern of results for sleep and cortisol suggests that OFC surgery does not seem to constitute a traumatic event with long-term consequences. Keywords: cortisol, sleep, orofacial cleft, surgery, infants

  7. Gas hydrate cool storage system

    Science.gov (United States)

    Ternes, M.P.; Kedl, R.J.

    1984-09-12

    The invention presented relates to the development of a process utilizing a gas hydrate as a cool storage medium for alleviating electric load demands during peak usage periods. Several objectives of the invention are mentioned concerning the formation of the gas hydrate as storage material in a thermal energy storage system within a heat pump cycle system. The gas hydrate was formed using a refrigerant in water and an example with R-12 refrigerant is included. (BCS)

  8. The circular RFQ storage ring

    International Nuclear Information System (INIS)

    Ruggiero, A.G.

    1998-01-01

    This paper presents a novel idea of storage ring for the accumulation of intense beams of light and heavy ions at low energy. The new concept is a natural development of the combined features used in a conventional storage ring and an ion trap, and is basically a linear RFQ bend on itself. In summary the advantages are: smaller beam dimensions, higher beam intensity, and a more compact storage device

  9. The Circular RFQ Storage Ring

    International Nuclear Information System (INIS)

    Ruggiero, A. G.

    1999-01-01

    This paper presents a novel idea of storage ring for the accumulation of intense beams of light and heavy ions at low energy. The new concept is a natural development of the combined features of conventional storage rings and ion traps, and is basically a linear RFQ bent on itself. The advantages are: smaller beam dimensions, higher beam intensity, and a more compact storage device

  10. The Petascale Data Storage Institute

    Energy Technology Data Exchange (ETDEWEB)

    Gibson, Garth [Carnegie Mellon Univ., Pittsburgh, PA (United States); Long, Darrell [The Regents of the University of California, Santa Cruz, CA (United States); Honeyman, Peter [Univ. of Michigan, Ann Arbor, MI (United States); Grider, Gary [Los Alamos National Lab. (LANL), Los Alamos, NM (United States); Kramer, William [National Energy Research Scientific Computing Center, Berkeley, CA (United States); Shalf, John [National Energy Research Scientific Computing Center, Berkeley, CA (United States); Roth, Philip [Oak Ridge National Lab. (ORNL), Oak Ridge, TN (United States); Felix, Evan [Pacific Northwest National Lab. (PNNL), Richland, WA (United States); Ward, Lee [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States)

    2013-07-01

    Petascale computing infrastructures for scientific discovery make petascale demands on information storage capacity, performance, concurrency, reliability, availability, and manageability.The Petascale Data Storage Institute focuses on the data storage problems found in petascale scientific computing environments, with special attention to community issues such as interoperability, community buy-in, and shared tools.The Petascale Data Storage Institute is a collaboration between researchers at Carnegie Mellon University, National Energy Research Scientific Computing Center, Pacific Northwest National Laboratory, Oak Ridge National Laboratory, Sandia National Laboratory, Los Alamos National Laboratory, University of Michigan, and the University of California at Santa Cruz.

  11. HTGR spent fuel storage study

    International Nuclear Information System (INIS)

    Burgoyne, R.M.; Holder, N.D.

    1979-04-01

    This report documents a study of alternate methods of storing high-temperature gas-cooled reactor (HTGR) spent fuel. General requirements and design considerations are defined for a storage facility integral to a fuel recycle plant. Requirements for stand-alone storage are briefly considered. Three alternate water-cooled storage conceptual designs (plug well, portable well, and monolith) are considered and compared to a previous air-cooled design. A concept using portable storage wells in racks appears to be the most favorable, subject to seismic analysis and economic evaluation verification

  12. Advanced materials for energy storage.

    Science.gov (United States)

    Liu, Chang; Li, Feng; Ma, Lai-Peng; Cheng, Hui-Ming

    2010-02-23

    Popularization of portable electronics and electric vehicles worldwide stimulates the development of energy storage devices, such as batteries and supercapacitors, toward higher power density and energy density, which significantly depends upon the advancement of new materials used in these devices. Moreover, energy storage materials play a key role in efficient, clean, and versatile use of energy, and are crucial for the exploitation of renewable energy. Therefore, energy storage materials cover a wide range of materials and have been receiving intensive attention from research and development to industrialization. In this Review, firstly a general introduction is given to several typical energy storage systems, including thermal, mechanical, electromagnetic, hydrogen, and electrochemical energy storage. Then the current status of high-performance hydrogen storage materials for on-board applications and electrochemical energy storage materials for lithium-ion batteries and supercapacitors is introduced in detail. The strategies for developing these advanced energy storage materials, including nanostructuring, nano-/microcombination, hybridization, pore-structure control, configuration design, surface modification, and composition optimization, are discussed. Finally, the future trends and prospects in the development of advanced energy storage materials are highlighted.

  13. Advanced materials for energy storage

    Energy Technology Data Exchange (ETDEWEB)

    Liu, Chang; Li, Feng; Ma, Lai-Peng; Cheng, Hui-Ming [Shenyang National Laboratory for Materials Science Institute of Metal Research, Chinese Academy of Sciences 72 Wenhua Road, Shenyang 110016 (China)

    2010-02-23

    Popularization of portable electronics and electric vehicles worldwide stimulates the development of energy storage devices, such as batteries and supercapacitors, toward higher power density and energy density, which significantly depends upon the advancement of new materials used in these devices. Moreover, energy storage materials play a key role in efficient, clean, and versatile use of energy, and are crucial for the exploitation of renewable energy. Therefore, energy storage materials cover a wide range of materials and have been receiving intensive attention from research and development to industrialization. In this review, firstly a general introduction is given to several typical energy storage systems, including thermal, mechanical, electromagnetic, hydrogen, and electrochemical energy storage. Then the current status of high-performance hydrogen storage materials for on-board applications and electrochemical energy storage materials for lithium-ion batteries and supercapacitors is introduced in detail. The strategies for developing these advanced energy storage materials, including nanostructuring, nano-/microcombination, hybridization, pore-structure control, configuration design, surface modification, and composition optimization, are discussed. Finally, the future trends and prospects in the development of advanced energy storage materials are highlighted. (Abstract Copyright [2010], Wiley Periodicals, Inc.)

  14. The Fermilab data storage infrastructure

    International Nuclear Information System (INIS)

    Jon A Bakken et al.

    2003-01-01

    Fermilab, in collaboration with the DESY laboratory in Hamburg, Germany, has created a petabyte scale data storage infrastructure to meet the requirements of experiments to store and access large data sets. The Fermilab data storage infrastructure consists of the following major storage and data transfer components: Enstore mass storage system, DCache distributed data cache, ftp and Grid ftp for primarily external data transfers. This infrastructure provides a data throughput sufficient for transferring data from experiments' data acquisition systems. It also allows access to data in the Grid framework

  15. Conduct disorders

    NARCIS (Netherlands)

    Buitelaar, J.K.; Smeets, K.C.; Herpers, P.; Scheepers, F.; Glennon, J.; Rommelse, N.N.J.

    2013-01-01

    Conduct disorder (CD) is a frequently occurring psychiatric disorder characterized by a persistent pattern of aggressive and non-aggressive rule breaking antisocial behaviours that lead to considerable burden for the patients themselves, their family and society. This review paper updates diagnostic

  16. Personality disorders

    NARCIS (Netherlands)

    van den Bosch, L.M.C.; Verheul, R.; Verster, J.C.; Brady, K.; Galanter, M.; Conrod, P.

    2012-01-01

    Subject of this chapter is the often found combination of personality disorders and ­substance abuse disorders. The serious nature of this comorbidity is shown through the discussion of prevalence and epidemiological data. Literature shows that the comorbidity, hampering the diagnostic process, is

  17. Personality disorder

    DEFF Research Database (Denmark)

    Tyrer, Peter; Mulder, Roger; Crawford, Mike

    2010-01-01

    and to society, and interferes, usually negatively, with progress in the treatment of other mental disorders. We now have evidence that personality disorder, as currently classified, affects around 6% of the world population, and the differences between countries show no consistent variation. We are also getting......Personality disorder is now being accepted as an important condition in mainstream psychiatry across the world. Although it often remains unrecognized in ordinary practice, research studies have shown it is common, creates considerable morbidity, is associated with high costs to services...... increasing evidence that some treatments, mainly psychological, are of value in this group of disorders. What is now needed is a new classification that is of greater value to clinicians, and the WPA Section on Personality Disorders is currently undertaking this task....

  18. Gambling disorders.

    Science.gov (United States)

    Hodgins, David C; Stea, Jonathan N; Grant, Jon E

    2011-11-26

    Gambling disorders, including pathological gambling and problem gambling, have received increased attention from clinicians and researchers over the past three decades since gambling opportunities have expanded around the world. This Seminar reviews prevalence, causes and associated features, screening and diagnosis, and treatment approaches. Gambling disorders affect 0·2-5·3% of adults worldwide, although measurement and prevalence varies according to the screening instruments and methods used, and availability and accessibility of gambling opportunities. Several distinct treatment approaches have been favourably evaluated, such as cognitive behavioural and brief treatment models and pharmacological interventions. Although promising, family therapy and support from Gamblers Anonymous are less well empirically supported. Gambling disorders are highly comorbid with other mental health and substance use disorders, and a further understanding is needed of both the causes and treatment implications of this disorder. Copyright © 2011 Elsevier Ltd. All rights reserved.

  19. In vitro storage of synthetic seeds: Effect of different storage ...

    African Journals Online (AJOL)

    In vitro derived shoots of olive cv. Moraiolo were employed in synthetic seeds preparation by alginate encapsulation, and then stored in artificial endosperm solution at cold (4°C) and room storage (21 ± 2°C) conditions in interaction with different storage intervals of 0, 15, 30, 45 and 60 days to evaluate the comparative ...

  20. Effects of storage conditions and storage period on nutritional and ...

    African Journals Online (AJOL)

    In this work, the effects of storage conditions and storage period on the nutritional and other qualities of stored yam tubers were investigated. ... environment, signs of deterioration of the tuber such as sprouting, weight loss and rotting and some ...

  1. Storage envelopes or sleeves

    International Nuclear Information System (INIS)

    Freshwater, J.R.; Wagman, P.I.

    1980-01-01

    A storage envelope or sleeve particularly for processed X-ray films is described. It consists of front and back panels joined together at a hinge line and connected along the intermediate sides by connecting flaps. An inner pocket is formed from a third flap which is folded to lie against the inner face of the back panel. The panels may have additional score lines parallel to the closed sides of the envelope and the inner pocket so that the envelope and the inner pocket can accommodate bulky contents. The free edge of the pocket is inset from the open side of the envelope, and finger cut-outs may be provided to facilitate access to the contents of the envelope and the pocket. (author)

  2. Reversible hydrogen storage materials

    Science.gov (United States)

    Ritter, James A [Lexington, SC; Wang, Tao [Columbia, SC; Ebner, Armin D [Lexington, SC; Holland, Charles E [Cayce, SC

    2012-04-10

    In accordance with the present disclosure, a process for synthesis of a complex hydride material for hydrogen storage is provided. The process includes mixing a borohydride with at least one additive agent and at least one catalyst and heating the mixture at a temperature of less than about 600.degree. C. and a pressure of H.sub.2 gas to form a complex hydride material. The complex hydride material comprises MAl.sub.xB.sub.yH.sub.z, wherein M is an alkali metal or group IIA metal, Al is the element aluminum, x is any number from 0 to 1, B is the element boron, y is a number from 0 to 13, and z is a number from 4 to 57 with the additive agent and catalyst still being present. The complex hydride material is capable of cyclic dehydrogenation and rehydrogenation and has a hydrogen capacity of at least about 4 weight percent.

  3. Inductive energy storage commutator

    International Nuclear Information System (INIS)

    Gavrilov, I.M.

    1987-01-01

    An inductive energy storage commutator is described. The value of commutated current is up to 800 A, the voltage amplitude in the load is up to 50 kV, the working frequency is equal to 1-50 Hz, the commutated power is up to 40 MW. The commutating device comprises of the first stage commutator having two in-series connected modules of the BTSV - 800/235 high-voltage thyristor unit, the second stage commutator containing three GMI-43A parallel connected powerful pulsed triodes, a commutating capacitor, an induction coil, two supplementary high-voltage thyristor keys (20 in-series connected thyristors T2-300 (13 class)), load, control pulse shapers, thyristor keys, power supply

  4. Radioactive gas storage device

    International Nuclear Information System (INIS)

    Sano, Yuji.

    1988-01-01

    Purpose: To easily and reliably detect the consumption of a sputtered cathode in a radioactive gas storage device using ion injection method. Constitution: Inert gases are sealed to the inside of a cathode. As the device is operated, the cathode is consumed and, if it is scraped to some extent, inert gases in the cathode gases are blown out to increase the inner pressure of the device. The pressure elevation is detected by a pressure detector connected with a gas introduction pipe or discharge pipe. Further, since the discharge current in the inside is increased along with the elevation of the pressure, it is possible to detect the increase of the electrical current. In this way, the consumption of the cathode can be recognized by detecting the elevation in the pressure or increase in the current. (Ikeda, J.)

  5. Terrestrial Water Storage

    Science.gov (United States)

    Rodell, M.; Chambers, D. P.; Famiglietti, J. S.

    2015-01-01

    During 2014 dryness continued in the Northern Hemisphere and relative wetness continued in the Southern Hemisphere (Fig. 2.21; Plate 2.1g). These largely canceled out such that the global land surface began and ended the year with a terrestrial water storage (TWS) anomaly slightly below 0 cm (equivalent height of water; Fig. 2.22). TWS is the sum of groundwater, soil moisture, surface water, snow, and ice. Groundwater responds more slowly to meteorological phenomena than the other components because the overlying soil acts as a low pass filter, but often it has a larger range of variability on multiannual timescales (Rodell and Famiglietti 2001; Alley et al. 2002).In situ groundwater data are only archived and made and Tanzania. The rest of the continent experienced mixed to dry conditions. Significant reductions in TWS in Greenland, Antarctica, and southern coastal Alaska reflect ongoing ice sheet and glacier ablation, not groundwater depletion.

  6. Autism spectrum disorder - childhood disintegrative disorder

    Science.gov (United States)

    ... part of the larger developmental disorder category of autism spectrum disorder . ... American Psychiatric Association. Autism spectrum disorder. ... VA: American Psychiatric Publishing: 2013;50-59. Raviola GJ, ...

  7. Refractory Hyperlactatemia with Organ Insufficiency in Lipid Storage Myopathy.

    Science.gov (United States)

    Xu, Yuanda; Zhou, Li; Liang, Weibo; He, Weiqun; Liu, Xiaoqing; Liang, Xiuling; Zhong, Nanshan; Li, Yimin

    2015-08-01

    Lipid storage myopathy is a metabolic disorder characterized by abnormal lipid accumulation in muscle fibers and progressive muscle weakness. Here, we report the case of a 17-year-old woman with progressive muscle weakness, refractory hyperlactatemia, and multiple organ insufficiency. Severe pneumonia was the initial diagnosis. After anti-infective treatment, fluid resuscitation, and mechanical ventilation, the patient's symptoms improved but hyperlactatemia and muscle weakness persisted. She was empirically treated with carnitine. Biochemical tests, electromyography, and muscle biopsy confirmed lipid storage myopathy. After 7 weeks of treatment, the patient resumed normal daily life. An empirical treatment with carnitine may be beneficial for patients before an accurate diagnosis of lipid storage myopathy is made.

  8. Spent fuel storage - dry storage options and issues

    International Nuclear Information System (INIS)

    Akins, M.J.

    2007-01-01

    The increase in the number of nuclear energy power generation facilities will require the ability to store the spent nuclear fuel for a long period until the host countries develop reprocessing or disposal options. Plants have storage pools which are closely associated with the operating units. These are excellent for short term storage, but require active maintenance and operations support which are not desirable for the long term. Over the past 25 years, dry storage options have been developed and implemented throughout the world. In recent years, protection against terrorist attack has become an increasing source of design objectives for these facilities, as well as the main nuclear plant. This paper explores the current design options of dry storage cask systems and examines some of the current design issues for above ground , in-ground, or below-ground storage of spent fuel in dry casks. (author)

  9. 303-K Storage Facility closure plan. Revision 2

    Energy Technology Data Exchange (ETDEWEB)

    1993-12-15

    Recyclable scrap uranium with zircaloy-2 and copper silicon alloy, uranium-titanium alloy, beryllium/zircaloy-2 alloy, and zircaloy-2 chips and fines were secured in concrete billets (7.5-gallon containers) in the 303-K Storage Facility, located in the 300 Area. The beryllium/zircaloy-2 alloy and zircaloy-2 chips and fines are designated as mixed waste with the characteristic of ignitability. The concretion process reduced the ignitability of the fines and chips for safe storage and shipment. This process has been discontinued and the 303-K Storage Facility is now undergoing closure as defined in the Resource Conservation and Recovery Act (RCRA) of 1976 and the Washington Administrative Code (WAC) Dangerous Waste Regulations, WAC 173-303-040. This closure plan presents a description of the 303-K Storage Facility, the history of materials and waste managed, and the procedures that will be followed to close the 303-K Storage Facility. The 303-K Storage Facility is located within the 300-FF-3 (source) and 300-FF-5 (groundwater) operable units, as designated in the Hanford Federal Facility Agreement and Consent Order (Tri-Party Agreement) (Ecology et al. 1992). Contamination in the operable units 300-FF-3 and 300-FF-5 is scheduled to be addressed through the Comprehensive Environmental Response, Compensation, and Liability Act (CERCLA) of 1980 remedial action process. Therefore, all soil remedial action at the 304 Facility will be conducted as part of the CERCLA remedial action of operable units 300-FF-3 and 300-FF-5.

  10. A novel transgenic mouse model of lysosomal storage disorder

    OpenAIRE

    Ortiz-Miranda, Sonia; Ji, Rui; Jurczyk, Agata; Aryee, Ken-Edwin; Mo, Shunyan; Fletcher, Terry; Shaffer, Scott A.; Greiner, Dale L.; Bortell, Rita; Gregg, Ronald G.; Cheng, Alan; Hennings, Leah J.; Rittenhouse, Ann R.

    2016-01-01

    We provide an explanation for striking pathology found in a subset of genetically engineered mice homozygous for a rat CaVβ2a transgene (Tg+/+). Multiple transgene (Tg) copies inserted into chromosome 19; at this same site a large deletion occurred, ablating cholesterol 25-hydroxylase and partially deleting lysosomal acid lipase and CD95. Their loss of function can account for lipid build up and immune system hypertrophy, which defines this phenotype and serendipitously provides a novel model...

  11. Licensing of spent fuel dry storage and consolidated rod storage

    International Nuclear Information System (INIS)

    Bailey, W.J.

    1990-02-01

    The results of this study, performed by Pacific Northwest Laboratory (PNL) and sponsored by the US Department of Energy (DOE), respond to the nuclear industry's recommendation that a report be prepared that collects and describes the licensing issues (and their resolutions) that confront a new applicant requesting approval from the US Nuclear Regulatory Commission (NRC) for dry storage of spent fuel or for large-scale storage of consolidated spent fuel rods in pools. The issues are identified in comments, questions, and requests from the NRC during its review of applicants' submittals. Included in the report are discussions of (1) the 18 topical reports on cask and module designs for dry storage fuel that have been submitted to the NRC, (2) the three license applications for dry storage of spent fuel at independent spent fuel storage installations (ISFSIs) that have been submitted to the NRC, and (3) the three applications (one of which was later withdrawn) for large-scale storage of consolidated fuel rods in existing spent fuel storage pools at reactors that were submitted tot he NRC. For each of the applications submitted, examples of some of the issues (and suggestions for their resolutions) are described. The issues and their resolutions are also covered in detail in an example in each of the three subject areas: (1) the application for the CASTOR V/21 dry spent fuel storage cask, (2) the application for the ISFSI for dry storage of spent fuel at Surry, and (3) the application for full-scale wet storage of consolidated spent fuel at Millstone-2. The conclusions in the report include examples of major issues that applicants have encountered. Recommendations for future applicants to follow are listed. 401 refs., 26 tabs

  12. Coagulation profile in patients undergoing video-assisted thoracoscopic lobectomy

    DEFF Research Database (Denmark)

    Christensen, Thomas Decker; Vad, Henrik; Pedersen, Søren

    2017-01-01

    -, and the first two days postoperatively by standard coagulation blood test, thromboelastometry (ROTEM®) and thrombin generation. Results: Patients undergoing potential curative surgery for lung cancer were not hypercoagulable preoperatively. There was no statistically significant difference in the majority......Background: Knowledge about the impact of Low-Molecular-Weight Heparin (LMWH) on the coagulation system in patients undergoing minimal invasive lung cancer surgery is sparse. The aim of this study was to assess the effect of LMWH on the coagulation system in patients undergoing Video......-Assisted Thoracoscopic Surgery (VATS) lobectomy for primary lung cancer. Methods: Sixty-three patients diagnosed with primary lung cancer undergoing VATS lobectomy were randomized to either subcutaneous injection with dalteparin (Fragmin®) 5000 IE once daily or no intervention. Coagulation was assessed pre-, peri...

  13. Experiense with remineraling means in patients undergoing orthodontic treatment

    Directory of Open Access Journals (Sweden)

    Stepanova Ye.A.

    2011-03-01

    Full Text Available In patients undergoing orthodontic treatment using bracket-technology a high risk of caries development. The algorithm of preventive interventions for the prevention of hair demineralization of enamel of the teeth

  14. Preoperative B-type natriuretic peptides in patients undergoing ...

    African Journals Online (AJOL)

    Southern African Journal of Anaesthesia and Analgesia ... Preoperative B-type natriuretic peptides in patients undergoing noncardiac surgery: a cumulative ... Future investigation should focus on the clinical implications of these data and the ...

  15. Do patients fear undergoing general anesthesia for oral surgery?

    Science.gov (United States)

    Elmore, Jasmine R; Priest, James H; Laskin, Daniel M

    2014-01-01

    Many patients undergoing major surgery have more fear of the general anesthesia than the procedure. This appears to be reversed with oral surgery. Therefore, patients need to be as well informed about this aspect as the surgical operation.

  16. CNS-directed gene therapy for lysosomal storage diseases

    OpenAIRE

    Sands, Mark S; Haskins, Mark E

    2008-01-01

    Lysosomal storage diseases (LSDs) are a group of inherited metabolic disorders usually caused by deficient activity of a single lysosomal enzyme. As most lysosomal enzymes are ubiquitously expressed, a deficiency in a single enzyme can affect multiple organ systems, including the central nervous system (CNS). At least 75% of all LSDs have a significant CNS component. Approaches such as bone marrow transplantation (BMT) or enzyme replacement therapy (ERT) can effectively treat the systemic dis...

  17. Preoperative autologous plateletpheresis in patients undergoing open heart surgery.

    OpenAIRE

    Tomar Akhlesh; Tempe Deepak; Banerjee A; Hegde R; Cooper A; Khanna S

    2003-01-01

    Blood conservation is an important aspect of care provided to the patients undergoing cardiac operations with cardiopulmonary bypass (CPB). It is even more important in patients with anticipated prolonged CPB, redo cardiac surgery, patients having negative blood group and in patients undergoing emergency cardiac surgery. In prolonged CPB the blood is subjected to more destruction of important coagulation factors, in redo surgery the separation of adhesions leads to increased bleeding and diff...

  18. [Dissociative disorders and affective disorders].

    Science.gov (United States)

    Montant, J; Adida, M; Belzeaux, R; Cermolacce, M; Pringuey, D; Da Fonseca, D; Azorin, J-M

    2014-12-01

    The phenomenology of dissociative disorders may be complex and sometimes confusing. We describe here two cases who were initially misdiagnosed. The first case concerned a 61 year-old woman, who was initially diagnosed as an isolated dissociative fugue and was actually suffering from severe major depressive episode. The second case concerned a 55 year-old man, who was suffering from type I bipolar disorder and polyvascular disease, and was initially diagnosed as dissociative fugue in a mooddestabilization context, while it was finally a stroke. Yet dissociative disorders as affective disorder comorbidity are relatively unknown. We made a review on this topic. Dissociative disorders are often studied through psycho-trauma issues. Litterature is rare on affective illness comorbid with dissociative disorders, but highlight the link between bipolar and dissociative disorders. The later comorbidity often refers to an early onset subtype with also comorbid panic and depersonalization-derealization disorder. Besides, unipolar patients suffering from dissociative symptoms have more often cyclothymic affective temperament. Despite the limits of such studies dissociative symptoms-BD association seems to correspond to a clinical reality and further works on this topic may be warranted. Copyright © 2014 L’Encéphale. Published by Elsevier Masson SAS.. All rights reserved.

  19. Lysosomal Disorders Drive Susceptibility to Tuberculosis by Compromising Macrophage Migration

    Science.gov (United States)

    Berg, Russell D.; Levitte, Steven; O’Sullivan, Mary P.; O’Leary, Seónadh M.; Cambier, C.J.; Cameron, James; Takaki, Kevin K.; Moens, Cecilia B.; Tobin, David M.; Keane, Joseph; Ramakrishnan, Lalita

    2016-01-01

    Summary A zebrafish genetic screen for determinants of susceptibility to Mycobacterium marinum identified a hypersusceptible mutant deficient in lysosomal cysteine cathepsins that manifests hallmarks of human lysosomal storage diseases. Under homeostatic conditions, mutant macrophages accumulate undigested lysosomal material, which disrupts endocytic recycling and impairs their migration to, and thus engulfment of, dying cells. This causes a buildup of unengulfed cell debris. During mycobacterial infection, macrophages with lysosomal storage cannot migrate toward infected macrophages undergoing apoptosis in the tuberculous granuloma. The unengulfed apoptotic macrophages undergo secondary necrosis, causing granuloma breakdown and increased mycobacterial growth. Macrophage lysosomal storage similarly impairs migration to newly infecting mycobacteria. This phenotype is recapitulated in human smokers, who are at increased risk for tuberculosis. A majority of their alveolar macrophages exhibit lysosomal accumulations of tobacco smoke particulates and do not migrate to Mycobacterium tuberculosis. The incapacitation of highly microbicidal first-responding macrophages may contribute to smokers’ susceptibility to tuberculosis. PMID:27015311

  20. Meeting Disorders.

    Science.gov (United States)

    Yager, Joel; Katzman, Jeffrey W

    2017-12-01

    Although meetings are central to organizational work, considerable time devoted to meetings in Academic Health Centers appears to be unproductively spent. The primary purposes of this article are to delineate and describe Meeting Disorders, pathological processes resulting in these inefficient and ineffective scenarios, and Meeting Fatigue Disorder (MFD), a clinical syndrome. The paper also offers preliminary approaches to remedies. The authors integrate observations made during tens of thousands of hours in administrative meetings in academic medical settings with information in the literature regarding the nature, causes and potential interventions for dysfunctional groups and meetings. Meeting Disorders, resulting from distinct pathologies of leadership and organization, constitute prevalent subgroups of the bureaucrapathologies, pathological conditions caused by dysfunctional bureaucratic processes that generate excesses of wasted time, effort, and other resources. These disorders also generate frustration and demoralization among participants, contributing to professional burnout. Meeting Fatigue Disorder (MFD) is a subjective condition that develops in individuals who overdose on these experiences and may reflect one manifestation of burnout. Meeting disorders and Meeting Fatigue Disorder occur commonly in bureaucratic life. Resources and potential remedies are available to help ameliorate their more deleterious effects.

  1. Status of US storage efforts

    International Nuclear Information System (INIS)

    Leasburg, R.H.

    1984-01-01

    Tasks involved in the implementation of the Nuclear Waste Policy Act are discussed. The need for speedy action on applications to deal with spent fuel storage problems is stressed. The problems faced by the Virginia Electric and Power Company, where full core discharge capability at the 1600-megawatt Surry power station is expected to be reached in early 1986, are reviewed. It is pointed out that although the Nuclear Waste Policy Act does not apply in this case, the problems illustrate the situation that may be faced after the Act is implemented. Problems involved in intro-utility transhipments and dry cask storage of spent fuel from Surry, including transportation ordinances at state and local levels and approval for the use of dry casks for storage, are reported. The suggestion that dry casks be used for interim storage and eventual transport to monitored retrievable storage facilities or permanent storage sites is considered. It is pointed out that data from a proposed 3-utility demonstration program of dry cask storage of consolidated fuels and the storage of fuels in air should give information applicable to the timely implementation of the Nuclear Waste Policy Act

  2. Tribology of magnetic storage systems

    Science.gov (United States)

    Bhushan, Bharat

    1992-01-01

    The construction and the materials used in different magnetic storage devices are defined. The theories of friction and adhesion, interface temperatures, wear, and solid-liquid lubrication relevant to magnetic storage systems are presented. Experimental data are presented wherever possible to support the relevant theories advanced.

  3. Glycogen Storage Disease Type IV

    DEFF Research Database (Denmark)

    Bendroth-Asmussen, Lisa; Aksglaede, Lise; Gernow, Anne B

    2016-01-01

    molecular genetic analyses confirmed glycogen storage disease Type IV with the finding of compound heterozygosity for 2 mutations (c.691+2T>C and c.1570C>T, p.R524X) in the GBE1 gene. We conclude that glycogen storage disease Type IV can cause early miscarriage and that diagnosis can initially be made...

  4. Spent fuel storage facility, Kalpakkam

    International Nuclear Information System (INIS)

    Shreekumar, B.; Anthony, S.

    2017-01-01

    Spent Fuel Storage Facility (SFSF), Kalpakkam is designed to store spent fuel arising from PHWRs. Spent fuel is transported in AERB qualified/authorized shipping cask by NPCIL to SFSF by road or rail route. The spent fuel storage facility at Kalpakkam was hot commissioned in December 2006. All systems, structures and components (SSCs) related to safety are designed to meet the operational requirements

  5. Nanostructured materials for hydrogen storage

    Science.gov (United States)

    Williamson, Andrew J.; Reboredo, Fernando A.

    2007-12-04

    A system for hydrogen storage comprising a porous nano-structured material with hydrogen absorbed on the surfaces of the porous nano-structured material. The system of hydrogen storage comprises absorbing hydrogen on the surfaces of a porous nano-structured semiconductor material.

  6. Hydrogen storage in carbon nanostruc

    NARCIS (Netherlands)

    Hirscher, M.; Becher, M.; Haluska, M.; Quintel, A.; Skakalova, V.; Choi, M.; Dettlaff-Weglikowska, U.; Roth, S.; Stepanek, I.; Bernier, P.; Leonhardt, A.; Fink, J.

    2002-01-01

    The paper gives a critical review of the literature on hydrogen storage in carbon nanostructures. Furthermore, the hydrogen storage of graphite, graphite nanofibers (GNFs), and single-walled carbon nanotubes (SWNTs) was measured by thermal desorption spectroscopy (TDS). The samples were ball milled

  7. Study of mined storage caverns

    International Nuclear Information System (INIS)

    1975-06-01

    All available information concerning 75 mined storage caverns as well as the investigative techniques utilized to determine the feasibility of a cavern site is presented. Potential new storage caverns may be for products such as high or low vapor pressure petroleum products, other chemical products, aid industrial or nuclear waste

  8. Dry spent fuel storage licensing

    International Nuclear Information System (INIS)

    Sturz, F.C.

    1995-01-01

    In the US, at-reactor-site dry spent fuel storage in independent spent fuel storage installations (ISFSI) has become the principal option for utilities needing storage capacity outside of the reactor spent fuel pools. Delays in the geologic repository operational date at or beyond 2010, and the increasing uncertainty of the US Department of Energy's (DOE) being able to site and license a Monitored Retrievable Storage (MRS) facility by 1998 make at-reactor-site dry storage of spent nuclear fuel increasingly desirable to utilities and DOE to meet the need for additional spent fuel storage capacity until disposal, in a repository, is available. The past year has been another busy year for dry spent fuel storage licensing. The licensing staff has been reviewing 7 applications and 12 amendment requests, as well as participating in inspection-related activities. The authors have licensed, on a site-specific basis, a variety of dry technologies (cask, module, and vault). By using certified designs, site-specific licensing is no longer required. Another new cask has been certified. They have received one new application for cask certification and two amendments to a certified cask design. As they stand on the brink of receiving multiple applications from DOE for the MPC, they are preparing to meet the needs of this national program. With the range of technical and licensing options available to utilities, the authors believe that utilities can meet their need for additional spent fuel storage capacity for essentially all reactor sites through the next decade

  9. Block storage subsystem performance analysis

    CERN Multimedia

    CERN. Geneva

    2016-01-01

    You feel that your service is slow because of the storage subsystem? But there are too many abstraction layers between your software and the raw block device for you to debug all this pile... Let's dive on the platters and check out how the block storage sees your I/Os! We can even figure out what those patterns are meaning.

  10. Southern company energy storage study :

    Energy Technology Data Exchange (ETDEWEB)

    Ellison, James; Bhatnagar, Dhruv; Black, Clifton; Jenkins, Kip

    2013-03-01

    This study evaluates the business case for additional bulk electric energy storage in the Southern Company service territory for the year 2020. The model was used to examine how system operations are likely to change as additional storage is added. The storage resources were allowed to provide energy time shift, regulation reserve, and spinning reserve services. Several storage facilities, including pumped hydroelectric systems, flywheels, and bulk-scale batteries, were considered. These scenarios were tested against a range of sensitivities: three different natural gas price assumptions, a 15% decrease in coal-fired generation capacity, and a high renewable penetration (10% of total generation from wind energy). Only in the elevated natural gas price sensitivities did some of the additional bulk-scale storage projects appear justifiable on the basis of projected production cost savings. Enabling existing peak shaving hydroelectric plants to provide regulation and spinning reserve, however, is likely to provide savings that justify the project cost even at anticipated natural gas price levels. Transmission and distribution applications of storage were not examined in this study. Allowing new storage facilities to serve both bulk grid and transmission/distribution-level needs may provide for increased benefit streams, and thus make a stronger business case for additional storage.

  11. Spent fuel storage and isolation

    International Nuclear Information System (INIS)

    Bensky, M.S.; Kurzeka, W.J.; Bauer, A.A.; Carr, J.A.; Matthews, S.C.

    1979-02-01

    The principal spent fuel activities conducted within the commercial waste and spent fuel within the Commercial Waste and Spent Fuel Packaging Program are: simulated near-surface (drywell) storage demonstrations at Hanford and the Nevada Test Site; surface (sealed storage cask) and drywell demonstrations at the Nevada Test Site; and spent fuel receiving and packaging facility conceptual design. These investigations are described

  12. Storage arrangements for nuclear fuel

    International Nuclear Information System (INIS)

    Ealing, C.J.

    1985-01-01

    A storage arrangement for nuclear fuel has a plurality of storage tubes connected by individual pipes to manifolds which are connected, in turn, to an exhaust system for maintaining the tubes at sub-atmospheric pressure, and means for producing a flow of a cooling fluid, such as air, over the exterior surfaces of the tubes. (author)

  13. Electrochemistry and Storage Panel Report

    Science.gov (United States)

    Stedman, J. K.; Halpert, G.

    1984-01-01

    Design and performance requirements for electrochemical power storage systems are discussed and some of the approaches towards satisfying these constraints are described. Geosynchronous and low Earth orbit applications, radar type load constraints, and high voltage systems requirements are addressed. In addition, flywheel energy storage is discussed.

  14. Androgens (dehydroepiandrosterone or testosterone) for women undergoing assisted reproduction.

    Science.gov (United States)

    Nagels, Helen E; Rishworth, Josephine R; Siristatidis, Charalampos S; Kroon, Ben

    2015-11-26

    Infertility is a condition affecting 10% to 15% of couples of reproductive age. It is generally defined as "the failure to achieve a clinical pregnancy after 12 months or more of regular unprotected sexual intercourse". The treatment of infertility may involve manipulation of gametes or of the embryos themselves. These techniques are together known as assisted reproductive technology (ART). Practitioners are constantly seeking alternative or adjunct treatments, or both, in the hope that they may improve the outcome of assisted reproductive techniques. This Cochrane review focusses on the adjunct use of synthetic versions of two naturally-produced hormones, dehydroepiandrosterone (DHEA) and testosterone (T), in assisted reproduction.DHEA and its derivative testosterone are steroid hormones proposed to increase conception rates by positively affecting follicular response to gonadotrophin stimulation, leading to greater oocyte yields and, in turn, increased chance of pregnancy. To assess the effectiveness and safety of DHEA and testosterone as pre- or co-treatments in subfertile women undergoing assisted reproduction. We searched the following electronic databases, trial registers and websites up to 12 March 2015: the Cochrane Central Register of Controlled Trials (CENTRAL), the Menstrual Disorders and Subfertility Group (MDSG) Specialised Register, MEDLINE, EMBASE, PsycINFO, CINAHL, electronic trial registers for ongoing and registered trials, citation indexes, conference abstracts in the Web of Science, PubMed and OpenSIGLE. We also carried out handsearches. There were no language restrictions. We included randomised controlled trials (RCTs) comparing DHEA or testosterone as an adjunct treatment to any other active intervention, placebo, or no treatment in women undergoing assisted reproduction. Two review authors independently selected studies, extracted relevant data and assessed them for risk of bias. We pooled studies using fixed-effect models. We calculated

  15. Different types of intermediate storages

    International Nuclear Information System (INIS)

    Spilker, H.

    1982-01-01

    Spent nuclear fuel elements require a period of ''cooling'' after withdrawal from a nuclear reactor. This is done in water storage pools located at the reactor facility. The minimum storage time prior to subsequent operations with the spent fuel is recognized as six to twelve months. After the initial storage period for cooling, the subsequent storage time in water pools could vary from a few years to several decades. Up to 1980, about 15.700 MTHM of spent LWR and HWR fuel have been discharged from nuclear stations. Since only a small fraction thereof has been already reprocessed. The spent fuel assemblies are stored at the reactor-side (AR) in reactor basins in normal or compact racks and away from reactor (AFR) in water pool storages awaiting either reprocessing or final disposal depending on the fuel cycle concept chosen by the individual countries. (orig.)

  16. Anthology of dry storage solutions

    Energy Technology Data Exchange (ETDEWEB)

    Allimann, Nathalie; Otton, Camille [AREVA, Paris (France)

    2012-03-15

    Around 35,000 PWR, BWR or Veer used fuel elements with various enrichment value up to 5%, various cooling time down to 2 years and various burn-ups up to 60,000 Mwd/tU are currently stored in AREVA dry storage solutions. These solutions are delivered in the United States, in Japan and in many European countries like Belgium, Switzerland, Italy, Armenia and Germany. With more than 1000 dry storage solutions delivered all over the world AREVA is the leader on this market. Dealing with dry storage is not an easy task. Products have to be flexible, to be adapted to customer needs and to the national regulations which may stipulate very strict tests such as airplane crash or simulation of earthquake. To develop a dry storage solution for a foreign country means to deal with its national competent authorities. All the national competent authorities do not have the same requirements. Storage conditions may also be different.

  17. Anthology of dry storage solutions

    International Nuclear Information System (INIS)

    Allimann, Nathalie; Otton, Camille

    2012-01-01

    Around 35,000 PWR, BWR or Veer used fuel elements with various enrichment value up to 5%, various cooling time down to 2 years and various burn-ups up to 60,000 Mwd/tU are currently stored in AREVA dry storage solutions. These solutions are delivered in the United States, in Japan and in many European countries like Belgium, Switzerland, Italy, Armenia and Germany. With more than 1000 dry storage solutions delivered all over the world AREVA is the leader on this market. Dealing with dry storage is not an easy task. Products have to be flexible, to be adapted to customer needs and to the national regulations which may stipulate very strict tests such as airplane crash or simulation of earthquake. To develop a dry storage solution for a foreign country means to deal with its national competent authorities. All the national competent authorities do not have the same requirements. Storage conditions may also be different

  18. Hydrogen isotope storage behavior of Zr1-xTixCo alloys

    International Nuclear Information System (INIS)

    Jat, Ram Avtar; Pati, Subhasis; Parida, S.C.; Agarwal, Renu; Mukerjee, S.K.

    2016-01-01

    Tritium storage properties similar to uranium make ZrCo as a suitable candidate material for storage, supply and recovery of hydrogen isotopes in various tritium facilities. Beside non-radioactive, nonpyrophoric at room temperature and higher storage capacity (H/f.u. up to 3, f.u. = ZrCo), it has been reported that upon repeated hydriding-dehydriding cycles, ZrCo undergoes dis-proportionation as per the reaction; ZrCo + H 2 ↔ ZrH 2 + ZrCo 2 . The present study is aimed to investigate the effect of Ti content on the hydrogen storage behavior of Zr 1-x Ti x Co alloys and the hydrogen isotope effect

  19. A review of pumped energy storage schemes

    International Nuclear Information System (INIS)

    Unsworth, G.N.

    1975-07-01

    The comparative advantages and costs of pumped water storage, steam accumulators, storage of boiler feedwater , and compressed air storage in caverns are described. Boiler feedwater storage in caverns and pumped water storage are most economical. All systems are costly enough to justify developing reactors with load following capabilities. (E.C.B.)

  20. Locally Minimum Storage Regenerating Codes in Distributed Cloud Storage Systems

    Institute of Scientific and Technical Information of China (English)

    Jing Wang; Wei Luo; Wei Liang; Xiangyang Liu; Xiaodai Dong

    2017-01-01

    In distributed cloud storage sys-tems, inevitably there exist multiple node fail-ures at the same time. The existing methods of regenerating codes, including minimum storage regenerating (MSR) codes and mini-mum bandwidth regenerating (MBR) codes, are mainly to repair one single or several failed nodes, unable to meet the repair need of distributed cloud storage systems. In this paper, we present locally minimum storage re-generating (LMSR) codes to recover multiple failed nodes at the same time. Specifically, the nodes in distributed cloud storage systems are divided into multiple local groups, and in each local group (4, 2) or (5, 3) MSR codes are constructed. Moreover, the grouping method of storage nodes and the repairing process of failed nodes in local groups are studied. The-oretical analysis shows that LMSR codes can achieve the same storage overhead as MSR codes. Furthermore, we verify by means of simulation that, compared with MSR codes, LMSR codes can reduce the repair bandwidth and disk I/O overhead effectively.

  1. Association of Oxidative Stress with Psychiatric Disorders.

    Science.gov (United States)

    Hassan, Waseem; Noreen, Hamsa; Castro-Gomes, Vitor; Mohammadzai, Imdadullah; da Rocha, Joao Batista Teixeira; Landeira-Fernandez, J

    2016-01-01

    When concentrations of both reactive oxygen species and reactive nitrogen species exceed the antioxidative capability of an organism, the cells undergo oxidative impairment. Impairments in membrane integrity and lipid and protein oxidation, protein mutilation, DNA damage, and neuronal dysfunction are some of the fundamental consequences of oxidative stress. The purpose of this work was to review the associations between oxidative stress and psychological disorders. The search terms were the following: "oxidative stress and affective disorders," "free radicals and neurodegenerative disorders," "oxidative stress and psychological disorders," "oxidative stress, free radicals, and psychiatric disorders," and "association of oxidative stress." These search terms were used in conjunction with each of the diagnostic categories of the American Psychiatric Association's Diagnostic and Statistical Manual of Mental Disorders and World Health Organization's International Statistical Classification of Diseases and Related Health Problems. Genetic, pharmacological, biochemical, and preclinical therapeutic studies, case reports, and clinical trials were selected to explore the molecular aspects of psychological disorders that are associated with oxidative stress. We identified a broad spectrum of 83 degenerative syndromes and psychiatric disorders that were associated with oxidative stress. The multi-dimensional information identified herein supports the role of oxidative stress in various psychiatric disorders. We discuss the results from the perspective of developing novel therapeutic interventions.

  2. Conduct disorder

    Science.gov (United States)

    ... develop problems with drug abuse and the law. Depression and bipolar disorder may develop in the teen years and early adulthood. Suicide and violence toward others are also possible complications.

  3. Sleep Disorders

    Science.gov (United States)

    ... the day, even if you have had enough sleep? You might have a sleep disorder. The most common kinds are Insomnia - a hard time falling or staying asleep Sleep apnea - breathing interruptions during sleep Restless legs syndrome - ...

  4. Eating Disorders

    Science.gov (United States)

    ... to control them. Avoidant/Restrictive Food Intake Disorder (ARFID) ARFID is a new term that some people think ... eating issues can also cause it. People with ARFID don't have anorexia or bulimia, but they ...

  5. Neurocutaneous Disorders.

    Science.gov (United States)

    Rosser, Tena

    2018-02-01

    This article presents an up-to-date summary of the genetic etiology, diagnostic criteria, clinical features, and current management recommendations for the most common neurocutaneous disorders encountered in clinical adult and pediatric neurology practices. The phakomatoses are a phenotypically and genetically diverse group of multisystem disorders that primarily affect the skin and central nervous system. A greater understanding of the genetic and biological underpinnings of numerous neurocutaneous disorders has led to better clinical characterization, more refined diagnostic criteria, and improved treatments in neurofibromatosis type 1, Legius syndrome, neurofibromatosis type 2, Noonan syndrome with multiple lentigines, tuberous sclerosis complex, Sturge-Weber syndrome, and incontinentia pigmenti. Neurologists require a basic knowledge of and familiarity with a wide variety of neurocutaneous disorders because of the frequent involvement of the central and peripheral nervous systems. A simple routine skin examination can often open a broad differential diagnosis and lead to improved patient care.

  6. Factitious Disorder

    Science.gov (United States)

    ... support their claims. Factitious disorder signs and symptoms may include: Clever and convincing medical or psychological problems Extensive knowledge of medical terms and diseases Vague or inconsistent symptoms Conditions that get worse for no apparent ...

  7. Neuromuscular Disorders

    Science.gov (United States)

    ... lead to twitching, cramps, aches and pains, and joint and movement problems. Sometimes it also affects heart function and your ability to breathe. Examples of neuromuscular disorders include Amyotrophic lateral sclerosis Multiple sclerosis Myasthenia ...

  8. Conduct Disorder

    Science.gov (United States)

    ... objections runs away from home often truant from school Children who exhibit these behaviors should receive a comprehensive evaluation by an experience mental health professional. Many children with a conduct disorder may ...

  9. Sleep Disorders

    DEFF Research Database (Denmark)

    Rahbek Kornum, Birgitte; Mignot, Emmanuel

    2014-01-01

    mediates circadian regulation of sleep. Misalignment with the rhythm of the sun results in circadian disorders and jet lag. The molecular basis of homeostatic sleep regulation is mostly unknown. A network of mutually inhibitory brain nuclei regulates sleep states and sleep-wake transitions. Abnormalities...... in these networks create sleep disorders, including rapid eye movement sleep behavior disorder, sleep walking, and narcolepsy. Physiological changes associated with sleep can be imbalanced, resulting in excess movements such as periodic leg movements during sleep or abnormal breathing in obstructive sleep apneas....... As every organ in the body is affected by sleep directly or indirectly, sleep and sleep-associated disorders are frequent and only now starting to be understood....

  10. TMJ Disorders

    Science.gov (United States)

    ... Aching pain in and around your ear Difficulty chewing or pain while chewing Aching facial pain Locking of the joint, making ... disorder. When to see a doctor Seek medical attention if you have persistent pain or tenderness in ...

  11. Autoimmune disorders

    Science.gov (United States)

    ... exact cause of autoimmune disorders is unknown. One theory is that some microorganisms (such as bacteria or ... M. is also a founding member of Hi-Ethics and subscribes to the principles of the Health ...

  12. Thermochemical Heat Storage: from Reaction Storage Density to System Storage Density

    NARCIS (Netherlands)

    Jong, A.J. de; Vliet, L.D. van; Hoegaerts, C.L.G.; Roelands, C.P.M.; Cuypers, R.

    2016-01-01

    Long-term and compact storage of solar energy is crucial for the eventual transition to a 100% renewable energy economy. For this, thermochemical materials provide a promising solution. The compactness of a long-term storage system is determined by the thermochemical reaction, operating conditions,

  13. Genetic autonomic disorders.

    Science.gov (United States)

    Axelrod, Felicia B

    2013-03-01

    Genetic disorders affecting the autonomic nervous system can result in abnormal development of the nervous system or they can be caused by neurotransmitter imbalance, an ion-channel disturbance or by storage of deleterious material. The symptoms indicating autonomic dysfunction, however, will depend upon whether the genetic lesion has disrupted peripheral or central autonomic centers or both. Because the autonomic nervous system is pervasive and affects every organ system in the body, autonomic dysfunction will result in impaired homeostasis and symptoms will vary. The possibility of genetic confirmation by molecular testing for specific diagnosis is increasing but treatments tend to remain only supportive and directed toward particular symptoms. Copyright © 2013 Elsevier Inc. All rights reserved.

  14. Eating disorders

    OpenAIRE

    Kontić Olga; Vasiljević Nadja; Trišović Marija; Jorga Jagoda; Lakić Aneta; Jašović-Gašić Miroslava

    2012-01-01

    Eating disorders are considered chronic diseases of civilization. The most studied and well known are anorexia and bulimia nervosa. Anorexia is considered one of the most common psychiatric problems of girls in puberty and adolescence. Due to high mortality and morbidity as well as the increasing expansion of these diseases, it is clear why the amount of research on these diseases is growing worldwide. Eating disorders lead to numerous medical complications, mostly due to late diagnosis...

  15. Submersible energy storage apparatus

    International Nuclear Information System (INIS)

    Mccartney, J.F.; Rowe, R.A.

    1980-01-01

    A submersible energy storage apparatus for an electrical power source is provided which includes an electrolysis unit feed water gas collection assembly and a fuel cell. The electrolysis unit feed water gas collection assembly includes a hydrogen container and an oxygen container wherein each container has a gas outlet and is capable of containing feed water as well as hydrogen and oxygen gases respectively. An electrolysis cell is provided which has a hydrogen outlet, an oxygen outlet and a feed water inlet. The hydrogen outlet is located in the hydrogen container, the oxygen outlet is located in the oxygen container, and the feed water inlet is located in one of the containers. Each of the containers has an opening to the submersible environment so as to be pressure responsive thereto. A barrier device is provided in association with the opening in each container for isolating the feed water in the container from water in the submersible environment. The fuel cell is operatively connected to the hydrogen and oxygen containers, and the electrical power source is operatively connected to the electrolysis cell. With this arrangement the electrolysis cell is capable of utilizing power from the power source during low electrical energy demand, and the fuel cell is capable of utilizing the hydrogen and oxygen gases for generating electricity during high demand periods

  16. Tritium Storage Material

    International Nuclear Information System (INIS)

    Cowgill, Donald F.; Luo, Weifang; Smugeresky, John E.; Robinson, David B.; Fares, Stephen James; Ong, Markus D.; Arslan, Ilke; Tran, Kim L.; McCarty, Kevin F.; Sartor, George B.; Stewart, Kenneth D.; Clift, W. Miles

    2008-01-01

    Nano-structured palladium is examined as a tritium storage material with the potential to release beta-decay-generated helium at the generation rate, thereby mitigating the aging effects produced by enlarging He bubbles. Helium retention in proposed structures is modeled by adapting the Sandia Bubble Evolution model to nano-dimensional material. The model shows that even with ligament dimensions of 6-12 nm, elevated temperatures will be required for low He retention. Two nanomaterial synthesis pathways were explored: de-alloying and surfactant templating. For de-alloying, PdAg alloys with piranha etchants appeared likely to generate the desired morphology with some additional development effort. Nano-structured 50 nm Pd particles with 2-3 nm pores were successfully produced by surfactant templating using PdCl salts and an oligo(ethylene oxide) hexadecyl ether surfactant. Tests were performed on this material to investigate processes for removing residual pore fluids and to examine the thermal stability of pores. A tritium manifold was fabricated to measure the early He release behavior of this and Pd black material and is installed in the Tritium Science Station glove box at LLNL. Pressure-composition isotherms and particle sizes of a commercial Pd black were measured.

  17. Disk storage at CERN

    CERN Document Server

    Mascetti, L; Chan, B; Espinal, X; Fiorot, A; Labrador, H Gonz; Iven, J; Lamanna, M; Presti, G Lo; Mościcki, JT; Peters, AJ; Ponce, S; Rousseau, H; van der Ster, D

    2015-01-01

    CERN IT DSS operates the main storage resources for data taking and physics analysis mainly via three system: AFS, CASTOR and EOS. The total usable space available on disk for users is about 100 PB (with relative ratios 1:20:120). EOS actively uses the two CERN Tier0 centres (Meyrin and Wigner) with 50:50 ratio. IT DSS also provide sizeable on-demand resources for IT services most notably OpenStack and NFS-based clients: this is provided by a Ceph infrastructure (3 PB) and few proprietary servers (NetApp). We will describe our operational experience and recent changes to these systems with special emphasis to the present usages for LHC data taking, the convergence to commodity hardware (nodes with 200-TB each with optional SSD) shared across all services. We also describe our experience in coupling commodity and home-grown solution (e.g. CERNBox integration in EOS, Ceph disk pools for AFS, CASTOR and NFS) and finally the future evolution of these systems for WLCG and beyond.

  18. Disk storage at CERN

    Science.gov (United States)

    Mascetti, L.; Cano, E.; Chan, B.; Espinal, X.; Fiorot, A.; González Labrador, H.; Iven, J.; Lamanna, M.; Lo Presti, G.; Mościcki, JT; Peters, AJ; Ponce, S.; Rousseau, H.; van der Ster, D.

    2015-12-01

    CERN IT DSS operates the main storage resources for data taking and physics analysis mainly via three system: AFS, CASTOR and EOS. The total usable space available on disk for users is about 100 PB (with relative ratios 1:20:120). EOS actively uses the two CERN Tier0 centres (Meyrin and Wigner) with 50:50 ratio. IT DSS also provide sizeable on-demand resources for IT services most notably OpenStack and NFS-based clients: this is provided by a Ceph infrastructure (3 PB) and few proprietary servers (NetApp). We will describe our operational experience and recent changes to these systems with special emphasis to the present usages for LHC data taking, the convergence to commodity hardware (nodes with 200-TB each with optional SSD) shared across all services. We also describe our experience in coupling commodity and home-grown solution (e.g. CERNBox integration in EOS, Ceph disk pools for AFS, CASTOR and NFS) and finally the future evolution of these systems for WLCG and beyond.

  19. Storage of sunflower seeds

    Directory of Open Access Journals (Sweden)

    Denise de Castro Lima

    Full Text Available The sunflower is among the top five crops in the world for the production of edible vegetable oil. The species displays rustic behavior, with an excellent edaphic and climatic adaptability index, being able to be cultivated throughout Brazil. Seed quality is the key to increasing production and productivity in the sunflower. The objective of this work was to monitor the viability of sunflower seeds with a view to their conservation when stored in different environments and packaging. The seeds were packed in paper bags, multilayered paper, black polyethylene and PET bottles; and stored for a period of twelve months in the following environments: dry cold room (10 ºC and 55% RH, the ambient conditions of Fortaleza, Ceará, Brazil (30-32 ºC and 75% RH, refrigerator (4 ºC and 38-43% RH and freezer (-20 ºC. Every three months, the water content of the seeds was determined and germination, accelerated ageing, speed of emergence index, and seedling dry weight were evaluated. The experimental design was completely randomized, in a scheme of split-lots, with four replications. It can be concluded that the natural environment is not suitable for the storage of sunflower seeds. Sunflower seeds remain viable for 12 months when stored in a dry cold room, refrigerator or freezer, irrespective of the type of packaging used.

  20. Nuclear materials management storage study

    International Nuclear Information System (INIS)

    Becker, G.W. Jr.

    1994-02-01

    The Office of Weapons and Materials Planning (DP-27) requested the Planning Support Group (PSG) at the Savannah River Site to help coordinate a Departmental complex-wide nuclear materials storage study. This study will support the development of management strategies and plans until Defense Programs' Complex 21 is operational by DOE organizations that have direct interest/concerns about or responsibilities for nuclear material storage. They include the Materials Planning Division (DP-273) of DP-27, the Office of the Deputy Assistant Secretary for Facilities (DP-60), the Office of Weapons Complex Reconfiguration (DP-40), and other program areas, including Environmental Restoration and Waste Management (EM). To facilitate data collection, a questionnaire was developed and issued to nuclear materials custodian sites soliciting information on nuclear materials characteristics, storage plans, issues, etc. Sites were asked to functionally group materials identified in DOE Order 5660.1A (Management of Nuclear Materials) based on common physical and chemical characteristics and common material management strategies and to relate these groupings to Nuclear Materials Management Safeguards and Security (NMMSS) records. A database was constructed using 843 storage records from 70 responding sites. The database and an initial report summarizing storage issues were issued to participating Field Offices and DP-27 for comment. This report presents the background for the Storage Study and an initial, unclassified summary of storage issues and concerns identified by the sites

  1. Interim and final storage casks

    International Nuclear Information System (INIS)

    Stumpfrock, L.; Kockelmann, H.

    2012-01-01

    The disposal of radioactive waste is a huge social challenge in Germany and all over the world. As is well known the search for a site for a final repository for high-level waste in Germany is not complete. Therefore, interim storage facilities for radioactive waste were built at plant sites in Germany. The waste is stored in these storage facilities in appropriate storage and transport casks until the transport in a final repository can be carried out. Licensing of the storage and transport casks aimed for use in the public space is done according to the traffic laws and for handling in the storage facility according to nuclear law. Taking into account the activity of the waste to be stored, different containers are in use, so that experience is available from the licensing and operation in interim storage facilities. The large volume of radioactive waste to be disposed of after the shut-down of power generation in nuclear power stations makes it necessary for large quantities of licensed storage and transport casks to be provided soon.

  2. Opportunities in independent gas storage

    International Nuclear Information System (INIS)

    Daniel, R.

    1999-01-01

    The range of business opportunities currently available for the midstream oil and gas business were discussed with particular focus on storage opportunities. Alberta Energy Co. (AEC) Ltd.'s two midstream business units include AEC Pipelines and Gas Processing, and AEC Storage and Hub Services. These two businesses provide the company with good investment returns, stable cash flow, and some significant strategic synergies with their exploration and production businesses. In 1988, the AECO C Hub in southeastern Alberta was created as an outgrowth of AEC's gas production operations on the Suffield block, where they had depleted gas reservoirs with high rock quality suitable for high deliverability storage. With the AECO C Hub, AEC was able to offer firm storage contracts of from 1 to 20 years, and to introduce short term interruptible parking and lending services, title exchange, a spot price index for greater price discovery, and an electronic nomination system. AEC is currently completing construction of their second commercial storage facility, the Wild Goose project, in northern California. D ebottlenecking' the Western Canada supply basin should provide additional opportunities for further expansion not only for AEC but also for other midstream service providers. Opportunities are especially available in the areas of new storage facilities to serve location-specific needs, replacement of declining storage capacity, replacement of retiring facilities, technological optimization of existing facilities, more flexible, higher deliverability facilities and commercial optimization of existing facilities. A map of the hubs and market centres of North America are included. 5 figs

  3. Lithium ion storage between graphenes

    Directory of Open Access Journals (Sweden)

    Chan Yue

    2011-01-01

    Full Text Available Abstract In this article, we investigate the storage of lithium ions between two parallel graphene sheets using the continuous approximation and the 6-12 Lennard-Jones potential. The continuous approximation assumes that the carbon atoms can be replaced by a uniform distribution across the surface of the graphene sheets so that the total interaction potential can be approximated by performing surface integrations. The number of ion layers determines the major storage characteristics of the battery, and our results show three distinct ionic configurations, namely single, double, and triple ion forming layers between graphenes. The number densities of lithium ions between the two graphenes are estimated from existing semi-empirical molecular orbital calculations, and the graphene sheets giving rise to the triple ion layers admit the largest storage capacity at all temperatures, followed by a marginal decrease of storage capacity for the case of double ion layers. These two configurations exceed the maximum theoretical storage capacity of graphite. Further, on taking into account the charge-discharge property, the double ion layers are the most preferable choice for enhanced lithium storage. Although the single ion layer provides the least charge storage, it turns out to be the most stable configuration at all temperatures. One application of the present study is for the design of future high energy density alkali batteries using graphene sheets as anodes for which an analytical formulation might greatly facilitate rapid computational results.

  4. Safety considerations for compressed hydrogen storage systems

    International Nuclear Information System (INIS)

    Gleason, D.

    2006-01-01

    An overview of the safety considerations for various hydrogen storage options, including stationary, vehicle storage, and mobile refueling technologies. Indications of some of the challenges facing the industry as the demand for hydrogen fuel storage systems increases. (author)

  5. Ultra-High Density Spectral Storage Materials

    National Research Council Canada - National Science Library

    Hasan, Zameer U

    2002-01-01

    .... Being atomic scale storage, spectral storage has the potential of providing orders of magnitude denser memories than present day memories that depend on the hulk properties of the storage medium...

  6. The SERI solar energy storage program

    Science.gov (United States)

    Copeland, R. J.; Wright, J. D.; Wyman, C. E.

    1980-01-01

    In support of the DOE thermal and chemical energy storage program, the solar energy storage program (SERI) provides research on advanced technologies, systems analyses, and assessments of thermal energy storage for solar applications in support of the Thermal and Chemical Energy Storage Program of the DOE Division of Energy Storage Systems. Currently, research is in progress on direct contact latent heat storage and thermochemical energy storage and transport. Systems analyses are being performed of thermal energy storage for solar thermal applications, and surveys and assessments are being prepared of thermal energy storage in solar applications. A ranking methodology for comparing thermal storage systems (performance and cost) is presented. Research in latent heat storage and thermochemical storage and transport is reported.

  7. Freezing and refrigerated storage in fisheries

    National Research Council Canada - National Science Library

    Johnston, W. A

    1994-01-01

    ...; the factors affecting cold storage conditions, etc. In addition, the publication describes the methods used to calculate cold storage refrigeration loads as well as the costs of freezing and cold storage...

  8. Oppositional Defiant Disorder (ODD)

    Science.gov (United States)

    ... Antisocial behavior Impulse control problems Substance use disorder Suicide Many children and teens with ODD also have other mental health disorders, such as: Attention-deficit/hyperactivity disorder (ADHD) Conduct disorder Depression Anxiety Learning and communication disorders Treating these other ...

  9. [Molecular and clinical characterization of Colombian patients suffering from type III glycogen storage disease].

    Science.gov (United States)

    Mantilla, Carolina; Toro, Mónica; Sepúlveda, María Elsy; Insuasty, Margarita; Di Filippo, Diana; López, Juan Álvaro; Baquero, Carolina; Navas, María Cristina; Arias, Andrés Augusto

    2018-05-01

    Type III glycogen storage disease (GSD III) is an autosomal recessive disorder in which a mutation in the AGL gene causes deficiency of the glycogen debranching enzyme. The disease is characterized by fasting hypoglycemia, hepatomegaly and progressive myopathy. Molecular analyses of AGL have indicated heterogeneity depending on ethnic groups. The full spectrum of AGL mutations in Colombia remains unclear. To describe the clinical and molecular characteristics of ten Colombian patients diagnosed with GSD III. We recruited ten Colombian children with a clinical and biochemical diagnosis of GSD III to undergo genetic testing. The full coding exons and the relevant exon-intron boundaries of the AGL underwent Sanger sequencing to identify mutation. All patients had the classic phenotype of the GSD III. Genetic analysis revealed a mutation p.Arg910X in two patients. One patient had the mutation p.Glu1072AspfsX36, and one case showed a compound heterozygosity with p.Arg910X and p.Glu1072AspfsX36 mutations. We also detected the deletion of AGL gene 3, 4, 5, and 6 exons in three patients. The in silico studies predicted that these defects are pathogenic. No mutations were detected in the amplified regions in three patients. We found mutations and deletions that explain the clinical phenotype of GSD III patients. This is the first report with a description of the clinical phenotype and the spectrum of AGL mutations in Colombian patients. This is important to provide appropriate prognosis and genetic counseling to the patient and their relatives.

  10. ICI optical data storage tape: An archival mass storage media

    Science.gov (United States)

    Ruddick, Andrew J.

    1993-01-01

    At the 1991 Conference on Mass Storage Systems and Technologies, ICI Imagedata presented a paper which introduced ICI Optical Data Storage Tape. This paper placed specific emphasis on the media characteristics and initial data was presented which illustrated the archival stability of the media. More exhaustive analysis that was carried out on the chemical stability of the media is covered. Equally important, it also addresses archive management issues associated with, for example, the benefits of reduced rewind requirements to accommodate tape relaxation effects that result from careful tribology control in ICI Optical Tape media. ICI Optical Tape media was designed to meet the most demanding requirements of archival mass storage. It is envisaged that the volumetric data capacity, long term stability and low maintenance characteristics demonstrated will have major benefits in increasing reliability and reducing the costs associated with archival storage of large data volumes.

  11. Postquench prethermalization in a disordered quantum fluid of light

    Science.gov (United States)

    Larré, Pierre-Élie; Delande, Dominique; Cherroret, Nicolas

    2018-04-01

    We study the coherence of a disordered and interacting quantum light field after propagation along a nonlinear optical fiber. Disorder is generated by a cross-phase modulation with a randomized auxiliary classical light field, while interactions are induced by self-phase modulation. When penetrating the fiber from free space, the incoming quantum light undergoes a disorder and interaction quench. By calculating the coherence function of the transmitted quantum light, we show that the decoherence induced by the quench spreads in a light-cone fashion in the nonequilibrium many-body quantum system, leaving the latter prethermalize with peculiar features originating from disorder.

  12. Assessing Cardiovascular Health Using Life′s Simple 7 in a Chinese Population Undergoing Stroke Prevention

    Directory of Open Access Journals (Sweden)

    Qiong Yang

    2015-01-01

    Conclusions: Few Chinese patients undergoing stroke prevention had optimal CVH (determined using LS7. Additionally, fewer patients undergoing secondary prevention had optimal CVH than those undergoing primary prevention. In particular, physical activity and diet status in this population require improvement.

  13. Polarized particles in storage rings

    International Nuclear Information System (INIS)

    Derbenev, Ya.S.; Kondratenko, A.M.; Serednyakov, S.I.; Skrinskij, A.N.; Tumajkin, G.M.; Shatunov, Yu.M.

    1977-01-01

    Experiments with polarized beams on the VEPP-2M and SPEAK storage rings are described. Possible methods of producing polarized particle beams in storage rings as well as method of polarization monitoring are counted. Considered are the processes of radiation polarization of electrons and positrons. It is shown, that to preserve radiation polarization the introduction of regions with a strong sign-variable magnetic field is recommended. Methods of polarization measurement are counted. It is suggested for high energies to use dependence of synchrotron radiation power on transverse polarization of electrons and positrons. Examples of using polarizability of colliding beams in storage rings are presented

  14. Grand Challenges facing Storage Systems

    CERN Multimedia

    CERN. Geneva

    2004-01-01

    In this talk, we will discuss the future of storage systems. In particular, we will focus on several big challenges which we are facing in storage, such as being able to build, manage and backup really massive storage systems, being able to find information of interest, being able to do long-term archival of data, and so on. We also present ideas and research being done to address these challenges, and provide a perspective on how we expect these challenges to be resolved as we go forward.

  15. Carbon material for hydrogen storage

    Science.gov (United States)

    Bourlinos, Athanasios; Steriotis, Theodore; Stubos, Athanasios; Miller, Michael A

    2016-09-13

    The present invention relates to carbon based materials that are employed for hydrogen storage applications. The material may be described as the pyrolysis product of a molecular precursor such as a cyclic quinone compound. The pyrolysis product may then be combined with selected transition metal atoms which may be in nanoparticulate form, where the metals may be dispersed on the material surface. Such product may then provide for the reversible storage of hydrogen. The metallic nanoparticles may also be combined with a second metal as an alloy to further improve hydrogen storage performance.

  16. Troubleshooting vSphere storage

    CERN Document Server

    Preston, Mike

    2013-01-01

    This is a step-by-step example-oriented tutorial aimed at showing the reader how to troubleshoot a variety of vSphere storage problems, and providing the reader with solutions that can be completed with minimal effort and time in order to limit damage to work.If you are a vSphere administrator, this is the book for you. This book will provide you with 'need to know' information about the various storage transports that ESXi utilizes, the tools and techniques we can use to identify problems, and the fundamental knowledge and steps to take to troubleshoot storage-related issues. Prior knowledge

  17. Energy storage for sustainable microgrid

    CERN Document Server

    Gao, David Wenzhong

    2015-01-01

    Energy Storage for Sustainable Microgrid addresses the issues related to modelling, operation and control, steady-state and dynamic analysis of microgrids with ESS. This book discusses major electricity storage technologies in depth along with their efficiency, lifetime cycles, environmental benefits and capacity, so that readers can envisage which type of storage technology is best for a particular microgrid application. This book offers solutions to numerous difficulties such as choosing the right ESS for the particular microgrid application, proper sizing of ESS for microgrid, as well as

  18. Monitored Retrievable Storage conceptual system study: metal storage casks

    International Nuclear Information System (INIS)

    Unterzuber, R.; Cross, T.E.; Krasicki, B.R.

    1983-08-01

    A description of the metal cask storage facility concept is presented with the operations required to handle the spent fuel or high-level wastes and transuranic wastes. A generic Receiving and Handling Facility, provided by PNL, has been used for this study. Modifications to the storage delivery side of the handling facility, necessary to couple the Receiving and Handling Facility with the storage facility, are described. The equipment and support facilities needed for the storage facility are also described. Two separate storage facilities are presented herein: one for all spent fuel storage, and one for storage of high-level waste (HLW) and transuranic waste (TRU). Each facility is described for the capacities and rates defined by PNL in the Concept Technical Performance Criteria and Base Assumptions (see Table 1.3-1). Estimates of costs and time-distributions of expenditures have been developed to construct, operate, and decommission the conceptual MRS facilities in mid-1983 dollars, for the base cases given using the cost categories and percentages provided by PNL. Cost estimates and time-distributions of expenditures have also been developed to expand the facility throughput rate from 1800 MTU to 3000 MTU, and to expand the facility storage capacity from 15,000 MTU to 72,00 MTU. The life cycle cost of the facility for the bounding cases of all spent fuel and all HLW and TRU, using the time-distributions of costs developed above and assuming a two percent per year discount rate, are also presented. 3 references, 16 figures, 18 tables

  19. Identification of a large intronic transposal insertion in SLC17A5 causing sialic acid storage disease

    NARCIS (Netherlands)

    Tarailo-Graovac, M. (Maja); Drögemöller, B.I. (Britt I.); Wasserman, W.W. (Wyeth W.); C.J. Ross; A.M.W. van den Ouweland (Ans); N. Darin (Niklas); Kollberg, G. (Gittan); Van Karnebeek, C.D.M. (Clara D. M.); Blomqvist, M. (Maria)

    2017-01-01

    textabstractBackground: Sialic acid storage diseases are neurodegenerative disorders characterized by accumulation of sialic acid in the lysosome. These disorders are caused by mutations in SLC17A5, the gene encoding sialin, a sialic acid transporter located in the lysosomal membrane. The most

  20. Biochemical Storage Lesions Occurring in Nonirradiated and Irradiated Red Blood Cells: A Brief Review

    Directory of Open Access Journals (Sweden)

    F. Adams

    2015-01-01

    Full Text Available Red blood cells undergo a series of biochemical fluctuations during 35–42-day storage period at 1°C to 6°C. The sodium/potassium pump is immobilised causing a decrease in intracellular potassium with an increase in cytoplasmic sodium levels, glucose levels decline, and acidosis occurs as a result of low pH levels. The frailty of stored erythrocytes triggers the formation of haemoglobin-containing microparticles and the release of cell-free haemoglobin which may add to transfusion difficulties. Lipid peroxidation, oxidative stress to band 3 structures, and other morphological and structural molecular changes also occur leading to spheroechinocytes and osmotic fragility. These changes that transpire in the red cells during the storage period are referred to as “storage lesions.” It is well documented that gamma irradiation exacerbates storage lesions and the reports of increased potassium levels leading to adverse reactions observed in neonates and infants have been of particular concern. There are, however, remarkably few systematic studies comparing the in vitro storage lesions of irradiated and nonirradiated red cell concentrates and it has been suggested that the impact of storage lesions on leucocyte reduced red blood cell concentrate (RBCC is incomplete. The review examines storage lesions in red blood cells and their adverse effects in reference to blood transfusion.

  1. Tic disorders.

    Science.gov (United States)

    Martino, Davide; Mink, Jonathan W

    2013-10-01

    Primary tic disorders are complex, multifactorial disorders in which tics are accompanied by other sensory features and an array of comorbid behavioral disorders. Secondary tics are proportionally much less frequent, but their etiology is diverse. This review aims to guide clinicians in the recognition of the phenomenology, pathophysiology, and treatment of these disorders. Advances include greater phenomenologic insights, particularly of nonmotor (sensory) features; increased knowledge of disease mechanisms, particularly coming from neuropsychological, functional imaging, pathologic, and animal model studies; growing evidence on the efficacy of alpha-2 agonists and the newer generation of dopamine-modulating agents; and recent strides in the evaluation of cognitive-behavioral therapy and deep brain stimulation surgery. The correct diagnostic approach to tic disorders requires accurate historical gathering, a thorough neurologic examination, and detailed definition of the patient's psychopathologic profile. Treatment should always begin with individualized psychoeducational strategies. Although pharmacologic treatments remain beneficial for most patients, cognitive-behavioral treatments have thus far shown promising efficacy. Deep brain stimulation surgery should still be limited to adult patients refractory to pharmacotherapy and cognitive-behavioral therapy.

  2. Type I Glycogen Storage Disease

    Science.gov (United States)

    ... Legacy Society Make Gifts of Stock Donate Your Car Personal Fundraising Partnership & Support Share Your Story Spread the Word Give While You Shop Contact Us Donate Now Glycogen Storage Disease Type ...

  3. Autumn study on storage rings

    CERN Multimedia

    1974-01-01

    The first two weeks of October have seen storage ring people from accelerator Laboratories throughout the world at CERN to study the fundamental problems of very high energy protonproton colliding beam machines.

  4. Solid-State Hydrogen Storage

    Data.gov (United States)

    National Aeronautics and Space Administration — This project will develop a method for converting metals to metal hydrides at low pressures for hydrogen storage systems with high efficiency with respect to volume...

  5. Cryogenic Propellant Storage and Transfer

    Data.gov (United States)

    National Aeronautics and Space Administration — Space Flight Demonstration development has been canceled in favor of a ground test bed development for of passive/active cryogenic propellant storage, transfer, and...

  6. Type I Glycogen Storage Disease

    Science.gov (United States)

    ... the most common form of glycogen storage disease, accounting for 25% of all cases. It is an ... Links Videos Webinars About ALF OVERVIEW Programs About Liver Disease Ask the Experts People ALF ...

  7. Storage Tank Legionella and Community

    Data.gov (United States)

    U.S. Environmental Protection Agency — Storage Tank Legionella and Community. This dataset is associated with the following publication: Qin, K., I. Struewing, J. Santodomingo, D. Lytle, and J. Lu....

  8. ALICE bags data storage accolades

    CERN Multimedia

    2007-01-01

    ComputerWorld has recognized CERN with an award for the 'Best Practices in Storage' for ALICE's data acquisition system, in the category of 'Systems Implementation'. The award was presented to the ALICE DAQ team on 18 April at a ceremony in San Diego, CA. (Top) ALICE physicist Ulrich Fuchs. (Bottom) Three of the five storage racks for the ALICE Data Acquisition system (Photo Antonio Saba). Between 16 and19 April, one thousand people from data storage networks around the world gathered to attend the biannual Storage Networking World Conference. Twenty-five companies and organizations were celebrated as finalists, and five of those were given honorary awards-among them CERN, which tied for first place in the category of Systems Implementation for the success of the ALICE Data Acquisition System. CERN was one of five finalists in this category, which recognizes the winning facility for 'the successful design, implementation and management of an interoperable environment'. 'Successful' could include documentati...

  9. Complex Hydrides for Hydrogen Storage

    Energy Technology Data Exchange (ETDEWEB)

    Slattery, Darlene; Hampton, Michael

    2003-03-10

    This report describes research into the use of complex hydrides for hydrogen storage. The synthesis of a number of alanates, (AIH4) compounds, was investigated. Both wet chemical and mechano-chemical methods were studied.

  10. Old radioactive waste storage sites

    International Nuclear Information System (INIS)

    2008-01-01

    After a recall of the regulatory context for the management of old sites used for the storage of radioactive wastes with respect with their activity, the concerned products, the disposal or storage type, this document describes AREVA's involvement in the radioactive waste management process in France. Then, for the different kinds of sites (currently operated sites having radioactive waste storage, storage sites for uranium mineral processing residues), it indicates their location and name, their regulatory status and their control authority, the reference documents. It briefly presents the investigation on the long term impact of uranium mineral processing residues on health and environment, evokes some aspects of public information transparency, and presents the activities of an expertise group on old uranium mines. The examples of the sites of Bellezane (uranium mineral processing residues) and COMURHEX Malvesi (assessment of underground and surface water quality at the vicinity of this installation) are given in appendix

  11. Storage facility for radioactive wastes

    International Nuclear Information System (INIS)

    Okada, Kyo

    1998-01-01

    Canisters containing high level radioactive wastes are sealed in overpacks in a receiving building constructed on the ground. A plurality of storage pits are formed in a layered manner vertically in multi-stages in deep underground just beneath the receiving building, for example underground of about 1000m from the ground surface. Each of the storage pits is in communication with a shaft which vertically communicates the receiving building and the storage pits, and is extended plainly in a horizontal direction from the shaft. The storage pit comprises an overpack receiving chamber, a main gallery and a plurality of galleries. A plurality of holes for burying the overpacks are formed on the bottom of the galleries in the longitudinal direction of the galleries. A plurality of overpack-positioning devices which run in the main gallery and the galleries by remote operation are disposed in the main gallery and the galleries. (I.N.)

  12. Photovoltaic power systems energy storage

    International Nuclear Information System (INIS)

    Buldini, P.L.

    1991-01-01

    Basically, the solar photovoltaic power system consists of: Array of solar panels; Charge/voltage stabilizer; Blocking diode and Storage device. The storage device is a very important part of the system due to the necessity to harmonize the inevitable time shift between energy supply and demand. As energy storage, different devices can be utilized, such as hydropumping, air or other gas compression, flywheel, superconducting magnet, hydrogen generation and so on, but actually secondary (rechargeable) electrochemical cells appear to be the best storage device, due to the direct use for recharge of the d.c. current provided by the solar panels, without any intermediate step of energy transformation and its consequent loss of efficiency

  13. DSM-5: proposed changes to depressive disorders.

    Science.gov (United States)

    Wakefield, Jerome C

    2012-03-01

    The Diagnostic and Statistical Manual of Mental Disorders (DSM) is currently undergoing a revision that will lead to a fifth edition in 2013. Proposed changes for DSM-5 include the creation of several new categories of depressive disorder. Some nosologists have expressed concern that the proposed changes could yield many 'false-positive diagnoses' in which normal distress is mislabeled as a mental disorder. Such confusion of normal distress and mental disorder undermines the interpretability of clinical trials and etiological research, causes inefficient allocation of resources, and incurs risks of unnecessary treatment. To evaluate these concerns, I critically examine five proposed DSM-5 expansions in the scope of depressive and grief disorders: (1) a new mixed anxiety/depression category; (2) a new premenstrual dysphoric disorder category; (3) elimination of the major depression bereavement exclusion; (4) elimination of the adjustment disorder bereavement exclusion, thus allowing the diagnosis of subsyndromal depressive symptoms during bereavement as adjustment disorders; and (5) a new category of adjustment disorder related to bereavement for diagnosing pathological non-depressive grief. I examine each proposal's face validity and conceptual coherence as well as empirical support where relevant, with special attention to potential implications for false-positive diagnoses. I conclude that mixed anxiety/depression and premenstrual dysphoric disorder are needed categories, but are too broadly drawn and will yield substantial false positives; that the elimination of the bereavement exclusion is not supported by the evidence; and that the proposed elimination of the adjustment-disorder bereavement exclusion, as well as the new category of grief-related adjustment disorder, are inconsistent with recent grief research, which suggests that these proposals would massively pathologize normal grief responses.

  14. AB Levitator and Electricity Storage

    OpenAIRE

    Bolonkin, Alexander

    2007-01-01

    The author researched this new idea - support of flight by any aerial vehicles at significant altitude solely by the magnetic field of the planet. It is shown that current technology allows humans to create a light propulsion (AB engine) which does not depend on air, water or ground terrain. Simultaniosly, this revolutionary thruster is a device for the storage of electricity which is extracted and is replenished (during braking) from/into the storage with 100 percent efficiency. The relative...

  15. Compressed air energy storage system

    Science.gov (United States)

    Ahrens, Frederick W.; Kartsounes, George T.

    1981-01-01

    An internal combustion reciprocating engine is operable as a compressor during slack demand periods utilizing excess power from a power grid to charge air into an air storage reservoir and as an expander during peak demand periods to feed power into the power grid utilizing air obtained from the air storage reservoir together with combustible fuel. Preferably the internal combustion reciprocating engine is operated at high pressure and a low pressure turbine and compressor are also employed for air compression and power generation.

  16. Superconductive energy storage magnet study

    International Nuclear Information System (INIS)

    Rhee, S.W.

    1982-01-01

    Among many methods of energy storages the superconducting energy storage has been considered as the most promising method. Many related technical problems are still unsolved. One of the problems is the magnetizing and demagnetizing loss of superconducting coil. This loss is mainly because of hysteresis of pinning force. In this paper the hysteresis loss is calculated and field dependence of the a.c. losses is explained. The ratio of loss and stored energy is also calculated. (Author)

  17. Hydrogen Storage In Nanostructured Materials

    OpenAIRE

    Assfour, Bassem

    2011-01-01

    Hydrogen is an appealing energy carrier for clean energy use. However, storage of hydrogen is still the main bottleneck for the realization of an energy economy based on hydrogen. Many materials with outstanding properties have been synthesized with the aim to store enough amount of hydrogen under ambient conditions. Such efforts need guidance from material science, which includes predictive theoretical tools. Carbon nanotubes were considered as promising candidates for hydrogen storag...

  18. Temporary storage area characterization report

    International Nuclear Information System (INIS)

    1990-01-01

    The preferred alternative identified in the Remedial Investigation/Feasibility Study (RI/FS) for the Weldon Spring Quarry Bulk Wastes is to remove the wastes from the quarry and transport them by truck to temporary storage facility at the chemical plant site. To support the RI/FS, this report provides data to characterize the temporary storage area (TSA) site and to ensure the suitability of the proposed location. 31 refs., 14 figs., 7 tabs

  19. Effects of hand massage on anxiety in patients undergoing ophthalmology surgery using local anesthesia

    Directory of Open Access Journals (Sweden)

    Jafar Rafiei Kiasari

    2012-08-01

    Full Text Available Introduction: Anxiety is a common disorder in patients before surgery. Inappropriately managed anxiety can cause psychological and physiological reactions and will affect the process of surgery and recovery. Therefore, this study examined the effects of hand mas-sage on anxiety in patients undergoing ophthalmology surgery using local anesthesia. Methods: In this interventional study, 52 patients who were supposed to undergo oph-thalmology surgery using local anesthesia were studied. Patients were randomly as-signed to two groups of intervention, who received hand massage before surgery (n = 27 and control (n = 25. Massaging lasted for 5 minutes (2.5 minutes on each hand before surgery. Stroking and scrubbing methods were performed by 2 trained research-ers. Anxiety level, blood pressure, heart rate, and respiratory rate were measured before and after the intervention in both groups. Anxiety was evaluated using Spielberger State-Trait Anxiety Inventory. Data was analyzed by chi-square, independent samples t-test, and paired t-test. Results: There were no significant differences in mean anxiety, systolic blood pressure, diastolic blood pressure, heart rate, and respiratory rate between the two groups before the intervention (p > 0.05. However, there was a significant differenc in the mean stress level between the two groups after the intervention (p 0.05. Conclusion: Our findings suggested that 5 minutes of hand massage before ophthalmology surgery (under local anesthesia could reduce anxiety. Therefore, this method can be used to increase patient comfort and reduce anxiety before surgical interventions.

  20. Effects of music therapy under general anesthesia in patients undergoing abdominal surgery.

    Science.gov (United States)

    Kahloul, Mohamed; Mhamdi, Salah; Nakhli, Mohamed Said; Sfeyhi, Ahmed Nadhir; Azzaza, Mohamed; Chaouch, Ajmi; Naija, Walid

    2017-12-01

    Music therapy, an innovative approach that has proven effectiveness in many medical conditions, seems beneficial also in managing surgical patients. The aim of this study is to evaluate its effects, under general anesthesia, on perioperative patient satisfaction, stress, pain, and awareness. This is a prospective, randomized, double-blind study conducted in the operating theatre of visceral surgery at Sahloul Teaching Hospital over a period of 4 months. Patients aged more than 18 undergoing a scheduled surgery under general anesthesia were included. Patients undergoing urgent surgery or presenting hearing or cognitive disorders were excluded. Before induction, patients wore headphones linked to an MP3 player. They were randomly allocated into 2 groups: Group M (with music during surgery) and group C (without music). Hemodynamic parameters, quality of arousal, pain experienced, patient's satisfaction, and awareness incidence during anesthesia were recorded. One hundred and forty patients were included and allocated into 2 groups that were comparable in demographic characteristics, surgical intervention type and anesthesia duration. Comparison of these two groups regarding the hemodynamic profile found more stability in group M for systolic arterial blood pressure. A calm recovery was more often noted in group M (77.1% versus 44%, p Music therapy is a non-pharmacological, inexpensive, and non-invasive technique that can significantly enhance patient satisfaction and decrease patients' embarrassing experiences related to perioperative stress, pain, and awareness.

  1. Survey of relationship between spiritual health and mental health in patients undergoing methadone maintenance treatment (MMT

    Directory of Open Access Journals (Sweden)

    abolhassan naghibi

    2015-12-01

    Full Text Available Introduction and goal: Religiosity and spirituality decreasesof the impact of life stress on the tendency to substance use. Everyday addedto the number of people who believe that spirituality is the way to treat neuroses and mental problems. This study aimed to determine the relationship between spiritual health and mental health in patients undergoing to methadone maintenance treatment (MMT dependent on the private and government sector in Sari. Method: This study was cross- sectional study. The target populations of this study were 123 women and men undergoing to methadone maintenance treatment (MMT. The data collected by spiritual and mental health questionnaire and were analyzed using two-sample t-test and spearman correlationin theSPSS (18 software. Findings: The grade average of spiritual health was 43/29 and mental health was 41/26.The results showed that a significant correlation between spiritual health with mental health. The highest correlation was between spiritual healthwith the social function and the lowestcorrelation was with physical problems. There was no significant relationshipbetween of marital status, number of children, sex and spiritual health. Conclusion: According to positive and significant role spiritual health in mental health, so, strengthen the spiritual dimension can to promote mental health and reduce mental disorders and the tendency to addiction.

  2. Risk of disseminated intravascular coagulation in patients undergoing US-guided transperineal prostatic biopsy

    International Nuclear Information System (INIS)

    Stella, M.S.; Comparato, D.; Camici, M.; Evangelisti, L.; Gaudio, V.; De Negri, F.; Talarico, L.; Giusti, C.; Morelli, G.

    1991-01-01

    Disseminated intravascular coagulation (DIC) is a severe life-threatening acute bleeding disorder. Traumatized tissues, tumors, necrotic tissues, or bacterial endotoxines release similar material in the blood to the tissutal factors activating the coagulation cascade. This preliminary study was aimed at verifying the risk of DIV in patients undergoing US-guided transperineal prostatic biopsy with Chiba and Tru-Cut needles. To evaluate the activation degree of coagulation factors in the circulation, the authors measured the concentrations of urinary fibrin degradation products in 10 patients undergoing US-guided transperineal prostatic biopsy, both before and after biopsy, every second hour, for 24 hours. Every tube of urine sample contained soya bean trypsin inhibitor and bovine thrombin to prevent any further fibrin degradation during incubation period for the possible presence of blood in urine samples. The results showed that 7/10 patients had marked increase in urinary fibrin degradation product levels (up to 800 XXXX%), with a 3-phase trend: early peak after 2-6 hours, middle peak after 6-14 hours, and late peak after 18-24 hours, which proved the activation of the coagulation cascade

  3. Factors Associated with Post-Surgical Delirium in Patients Undergoing Open Heart Surgery

    Directory of Open Access Journals (Sweden)

    Yadollah Jannati

    2014-09-01

    Full Text Available Objective: The objective of the present study is to determine the incidence of delirium and the associated factors in patients undergoing open heart surgery. Methods: This is an Analytic-descriptive study conducted on 404 patients undergoing elective open heart surgery in Fatemeh Zahra Heart Center, Sari, over the period of 6 months from July to December 2011. Sampling was achieved in a nonrandomized targeted manner and delirium was assessed using NeeCham questionnaire. A trained nurse evaluated the patients for delirium and completed the risk factor checklist on days 1 to 5 after surgery. Data analyses were accomplished using survival analysis (Kaplan-Meier and Cox regression on SPSS software version 15. Results: We found that variables, including ventilation time, increased drainage during the first 24 hours, the need for re-operation in the first 24 hours, dysrhythmias, use of inotropic agents, increased use of analgesics, increased arterial carbon dioxide, lack of visitors, and use of physical restrainers were associated with the development of delirium. In addition, we found a delirium incidence of 29%. Conclusion: Diagnosis of cognitive disorders is of utmost value; therefore, further studies are required to clarify the risk factors because controlling them will help prevent delirium.

  4. Evaluation of the lung function in cancer patients undergoing to chemotherapy

    Directory of Open Access Journals (Sweden)

    Caroline Heemann Vione

    2016-10-01

    Full Text Available Background and Objective: Some neoplastic agents used in cancer treatment cause pulmonary toxicity and other important adverse effects, therefore, the present study aimed to evaluate the presence of obstructive lung disease (OLD, restrictive lung disease (RLD or mixed in patients 2 with cancer undergoing chemotherapy. Method: This is a cross-sectional and descriptive study that evaluated patients diagnosed with cancer and undergoing chemotherapy using Doxorubicin, Bleomycin, Vinblastine, Dacarbazine, Cyclophosphamide, Fluorouracil and Vincristine, being excluded those diagnosed with lung cancer. Realized pulmonary function test byspirometric analysis (EasyOne®, Switzerland in patients who started chemotherapy for over 30 days, it was evaluated the Forced Vital Capacity (FVC, forced expiratory volume in one second FVC (FEV1, forced expiratory flow between 25-75% of FVC (FEF25-75 % and the FEV1 / FVC Relation before the chemotherapy session. Results: Sample (n = 18 composed of cancer patients with average age of 49.28±9.90 years and 26.49±5.67 kg/m2 on which 44.4% had normal spirometry standard, 27.8% had OLD, 16.7% presented RLD and 11.1% presented mixed respiratory disorder. Conclusion: Thechemotherapy resulted in obstructive pulmonary disease, restrictive and mixedin patients with leukemia, breast cancer and Hodgkin's lymphoma not specific evaluated.

  5. Development of spent fuel dry storage technology

    International Nuclear Information System (INIS)

    Maruoka, Kunio; Matsunaga, Kenichi; Kunishima, Shigeru

    2000-01-01

    The spent fuels are the recycle fuel resources, and it is very important to store the spent fuels in safety. There are two types of the spent fuel interim storage system. One is wet storage system and another is dry storage system. In this study, the dry storage technology, dual purpose metal cask storage and canister storage, has been developed. For the dual purpose metal cask storage, boronated aluminum basket cell, rational cask body shape and shaping process have been developed, and new type dual purpose metal cask has been designed. For the canister storage, new type concrete cask and high density vault storage technology have been developed. The results of this study will be useful for the spent fuel interim storage. Safety and economical spent fuel interim storage will be realized in the near future. (author)

  6. Digested disorder

    Science.gov (United States)

    DeForte, Shelly; Reddy, Krishna D; Uversky, Vladimir N

    2013-01-01

    The current literature on intrinsically disordered proteins is overwhelming. To keep interested readers up to speed with this literature, we continue a “Digested Disorder” project and represent a series of reader’s digest type articles objectively representing the research papers and reviews on intrinsically disordered proteins. The only 2 criteria for inclusion in this digest are the publication date (a paper should be published within the covered time frame) and topic (a paper should be dedicated to any aspect of protein intrinsic disorder). The current digest issue covers papers published during the period of April, May, and June of 2013. The papers are grouped hierarchically by topics they cover, and for each of the included paper a short description is given on its major findings. PMID:28516028

  7. Digested disorder

    Science.gov (United States)

    Reddy, Krishna D; DeForte, Shelly; Uversky, Vladimir N

    2014-01-01

    The current literature on intrinsically disordered proteins grows fast. To keep interested readers up to speed with this literature, we continue a “Digested Disorder” project and represent a new issue of reader’s digest of the research papers and reviews on intrinsically disordered proteins. The only 2 criteria for inclusion in this digest are the publication date (a paper should be published within the covered time frame) and topic (a paper should be dedicated to any aspect of protein intrinsic disorder). The current digest issue covers papers published during the third quarter of 2013; i.e., during the period of June, July, and September of 2013. Similar to previous issues, the papers are grouped hierarchically by topics they cover, and for each of the included paper a short description is given on its major findings. PMID:28232877

  8. Storage of Human Breast Milk

    Directory of Open Access Journals (Sweden)

    Gamze Can

    2007-10-01

    Full Text Available Storage of human breast milk by freezing or refrigeration of milk has been recommended especially at some social circumstances of most mothers who are regularly separated from their infants because of work. The greatest fear that has hindered the prospects of in - vitro storage of breast milk for any considerable period of time is the possibility of bacterial contamination and growth of infectious pathogens in the stored milk, there by rendering them unsafe for human consumption. The storage container can influence the cell content of milk, as the cells adhere to the walls of a glass container but not to polyethylene or polypropylene containers. Bacteriological examination of refrigerated milks has proven their safety for human consumption for even up to 72 h. For a storage over longer periods up to 1 month, freezing at - 20 0C could be recommended, but the most preferred method, especially for longer storage would be fresh freezing at - 70 0C, if affordable or available. The nutrient value of human milk is essentially unchanged, but the immunological properties are reduced by various storage techniques. Boiling and microwave radiation have not been recommended. [TAF Prev Med Bull 2007; 6(5.000: 375-379

  9. Geological storage of CO2

    International Nuclear Information System (INIS)

    Czernichowski-Lauriol, I.

    2005-01-01

    The industrial storage of CO 2 is comprised of three steps: - capture of CO 2 where it is produced (power plants, cement plants, etc.); - transport (pipe lines or boats); - storage, mainly underground, called geological sequestration... Three types of reservoirs are considered: - salted deep aquifers - they offer the biggest storage capacity; - exhausted oil and gas fields; - non-exploited deep coal mine streams. The two latter storage types may allow the recovery of sellable products, which partially or totally offsets the storage costs. This process is largely used in the petroleum industry to improve the productivity of an oil field, and is called FOR (Enhanced Oil Recovery). A similar process is applied in the coal mining industry to recover the imprisoned gas, and is called ECBM (Enhanced Coal Bed methane). Two storage operations have been initiated in Norway and in Canada, as well as research programmes in Europe, North America, Australia and Japan. International organisations to stimulate this technology have been created such as the 'Carbon Sequestration Leadership Forum' and 'the Intergovernmental Group for Climate Change'. This technology will be taken into account in the instruments provided by the Tokyo Protocol. (author)

  10. NV energy electricity storage valuation :

    Energy Technology Data Exchange (ETDEWEB)

    Ellison, James F.; Bhatnagar, Dhruv; Samaan, Nader; Jin, Chunlian

    2013-06-01

    This study examines how grid-level electricity storage may benefit the operations of NV Energy, and assesses whether those benefits are likely to justify the cost of the storage system. To determine the impact of grid-level storage, an hourly production cost model of the Nevada Balancing Authority ("BA") as projected for 2020 was created. Storage was found to add value primarily through the provision of regulating reserve. Certain storage resources were found likely to be cost-effective even without considering their capacity value, as long as their effectiveness in providing regulating reserve was taken into account. Giving fast resources credit for their ability to provide regulating reserve is reasonable, given the adoption of FERC Order 755 ("Pay-for-performance"). Using a traditional five-minute test to determine how much a resource can contribute to regulating reserve does not adequately value fast-ramping resources, as the regulating reserve these resources can provide is constrained by their installed capacity. While an approximation was made to consider the additional value provided by a fast-ramping resource, a more precise valuation requires an alternate regulating reserve methodology. Developing and modeling a new regulating reserve methodology for NV Energy was beyond the scope of this study, as was assessing the incremental value of distributed storage.

  11. Optimal design and application of a compound cold storage system combining seasonal ice storage and chilled water storage

    NARCIS (Netherlands)

    Yan, C.; Shi, W.; Li, X.; Zhao, Y.

    2016-01-01

    Seasonal cold storage using natural cold sources for cooling is a sustainable cooling technique. However, this technique suffers from limitations such as large storage space and poor reliability. Combining seasonal storage with short-term storage might be a promising solution while it is not

  12. A concept of an electricity storage system with 50 MWh storage capacity

    Directory of Open Access Journals (Sweden)

    Józef Paska

    2012-06-01

    Full Text Available Electricity storage devices can be divided into indirect storage technology devices (involving electricity conversion into another form of energy, and direct storage (in an electric or magnetic fi eld. Electricity storage technologies include: pumped-storage power plants, BES Battery Energy Storage, CAES Compressed Air Energy Storage, Supercapacitors, FES Flywheel Energy Storage, SMES Superconducting Magnetic Energy Storage, FC Fuel Cells reverse or operated in systems with electrolysers and hydrogen storage. These technologies have diff erent technical characteristics and economic parameters that determine their usability. This paper presents two concepts of an electricity storage tank with a storage capacity of at least 50 MWh, using the BES battery energy storage and CAES compressed air energy storage technologies.

  13. Globalization and eating disorder risk: Peer influence, perceived social norms, and adolescent disordered eating in Fiji

    Science.gov (United States)

    Gerbasi, Margaret E.; Richards, Lauren K.; Thomas, Jennifer J.; Agnew-Blais, Jessica C.; Thompson-Brenner, Heather; Gilman, Stephen E.; Becker, Anne E.

    2014-01-01

    Objective The increasing global health burden imposed by eating disorders warrants close examination of social exposures associated with globalization that potentially elevate risk during the critical developmental period of adolescence in low- and middle-income countries (LMICs). The study aim was to investigate the association of peer influence and perceived social norms with adolescent eating pathology in Fiji, a LMIC undergoing rapid social change. Method We measured peer influence on eating concerns (with the Inventory of Peer Influence on Eating Concerns; IPIEC), perceived peer norms associated with disordered eating and body concerns, perceived community cultural norms, and individual cultural orientations in a representative sample of school-going ethnic Fijian adolescent girls (n=523). We then developed a multivariable linear regression model to examine their relation to eating pathology (measured by the Eating Disorder Examination-Questionnaire; EDE-Q). Results We found independent and statistically significant associations between both IPIEC scores and our proxy for perceived social norms specific to disordered eating (both p disordered eating may elevate risk for disordered eating in Fiji, during the critical developmental period of adolescence. Replication and extension of these research findings in other populations undergoing rapid social transition—and where globalization is also influencing local social norms—may enrich etiologic models and inform strategies to mitigate risk. PMID:25139374

  14. Globalization and eating disorder risk: peer influence, perceived social norms, and adolescent disordered eating in Fiji.

    Science.gov (United States)

    Gerbasi, Margaret E; Richards, Lauren K; Thomas, Jennifer J; Agnew-Blais, Jessica C; Thompson-Brenner, Heather; Gilman, Stephen E; Becker, Anne E

    2014-11-01

    The increasing global health burden imposed by eating disorders warrants close examination of social exposures associated with globalization that potentially elevate risk during the critical developmental period of adolescence in low- and middle-income countries (LMICs). The study aim was to investigate the association of peer influence and perceived social norms with adolescent eating pathology in Fiji, a LMIC undergoing rapid social change. We measured peer influence on eating concerns (with the Inventory of Peer Influence on Eating Concerns; IPIEC), perceived peer norms associated with disordered eating and body concerns, perceived community cultural norms, and individual cultural orientations in a representative sample of school-going ethnic Fijian adolescent girls (n = 523). We then developed a multivariable linear regression model to examine their relation to eating pathology (measured by the Eating Disorder Examination-Questionnaire; EDE-Q). We found independent and statistically significant associations between both IPIEC scores and our proxy for perceived social norms specific to disordered eating (both p peer influence as well as perceived social norms relevant to disordered eating may elevate risk for disordered eating in Fiji, during the critical developmental period of adolescence. Replication and extension of these research findings in other populations undergoing rapid social transition--and where globalization is also influencing local social norms--may enrich etiologic models and inform strategies to mitigate risk. © 2014 Wiley Periodicals, Inc.

  15. Inherited Disorders of Bilirubin Clearance

    Science.gov (United States)

    Memon, Naureen; Weinberger, Barry I; Hegyi, Thomas; Aleksunes, Lauren M

    2016-01-01

    Inherited disorders of hyperbilirubinemia may be caused by increased bilirubin production or decreased bilirubin clearance. Reduced hepatic bilirubin clearance can be due to defective 1) unconjugated bilirubin uptake and intrahepatic storage, 2) conjugation of glucuronic acid to bilirubin (e.g. Gilbert syndrome, Crigler-Najjar syndrome, Lucey-Driscoll syndrome, breast milk jaundice), 3) bilirubin excretion into bile (Dubin-Johnson syndrome), or 4) conjugated bilirubin re-uptake (Rotor syndrome). In this review, the molecular mechanisms and clinical manifestations of these conditions are described, as well as current approaches to diagnosis and therapy. PMID:26595536

  16. Movement disorders

    International Nuclear Information System (INIS)

    Leenders, K.L.

    1986-01-01

    This thesis describes the measurement of brain-tissue functions in patients with movement disorders using positron emission tomography (PET). This scanning technique is a method for direct in vivo quantitation of the regional tissue content of positron emitting radionuclides in brain (or other organs) in an essentially non-invasive way. Ch. 2 outlines some general features of PET and describes the scanner which has been used for the studies in this thesis. Also the tracer methodology, as applied to data investigations of movement disorders, are discussed. Ch. 3 contains the results of the PET investigations which were performed in the study of movement disorders. The results are presented in the form of 12 papers. The main goals of these studies were the understanding of the pathophysiology of Parkinson's disease, Huntington's chorea, Steele-Richardson-Olzewski syndrome and special case reports. Ch. 4 summarizes the results of these publications and Ch. 5 concludes the main part of this thesis with a general discussion of movement disorders in relation to PET investigations. 697 refs.; 60 figs.; 31 tabs

  17. Penis Disorders

    Science.gov (United States)

    Problems with the penis can cause pain and affect a man's sexual function and fertility. Penis disorders include Erectile dysfunction - inability to get or ... not go away Peyronie's disease - bending of the penis during an erection due to a hard lump ...

  18. Bipolar Disorder

    Science.gov (United States)

    ... one or other traumatic event Drug or alcohol abuse Complications Left untreated, bipolar disorder can result in serious problems that affect every area of your life, such as: Problems related to drug and alcohol use Suicide or suicide attempts Legal or financial problems Damaged ...

  19. Hoarding disorder

    Science.gov (United States)

    ... a reminder of happier times or representing beloved people or pets They feel safer when surrounded by the things ... that are part of hoarding disorder. Hoarding animals People who hoard animals may collect dozens or even hundreds of pets. Animals may be confined inside or outside. Because ...

  20. Anorectal Disorders

    Science.gov (United States)

    Rao, Satish S. C.; Bharucha, Adil E.; Chiarioni, Giuseppe; Felt-Bersma, Richelle; Knowles, Charles; Malcolm, Allison; Wald, Arnold

    2016-01-01

    This report defines criteria and reviews the epidemiology, pathophysiology, and management of the following common anorectal disorders: fecal incontinence (FI), functional anorectal pain, and functional defecation disorders. FI is defined as the recurrent uncontrolled passage of fecal material for at least 3 months. The clinical features of FI are useful for guiding diagnostic testing and therapy. Anorectal manometry and imaging are useful for evaluating anal and pelvic floor structure and function. Education, antidiarrheals, and biofeedback therapy are the mainstay of management; surgery may be useful in refractory cases. Functional anorectal pain syndromes are defined by clinical features and categorized into 3 subtypes. In proctalgia fugax, the pain is typically fleeting and lasts for seconds to minutes. In levator ani syndrome and unspecified anorectal pain, the pain lasts more than 30 minutes, but in levator ani syndrome there is puborectalis tenderness. Functional defecation disorders are defined by ≥2 symptoms of chronic constipation or irritable bowel syndrome with constipation, and with ≥2 features of impaired evacuation, that is, abnormal evacuation pattern on manometry, abnormal balloon expulsion test, or impaired rectal evacuation by imaging. It includes 2 subtypes: dyssynergic defecation and inadequate defecatory propulsion. Pelvic floor biofeedback therapy is effective for treating levator ani syndrome and defecatory disorders. PMID:27144630