WorldWideScience

Sample records for storage disorders undergoing

  1. 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...

  2. 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…

  3. 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).

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

    Directory of Open Access Journals (Sweden)

    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.

  5. 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.

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

    Science.gov (United States)

    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.

  7. 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

  8. 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...

  9. Autophagy, lipophagy and lysosomal lipid storage disorders.

    Science.gov (United States)

    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.

  10. 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.

  11. 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

  12. 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.

  13. 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

  14. 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

  15. 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.

  16. 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

  17. 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.

  18. 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.

  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

    2016-08-01

    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.

  20. 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

  1. 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.

  2. 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.

  3. 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.

  4. 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...

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

    Directory of Open Access Journals (Sweden)

    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.

  6. 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.

  7. 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

  8. 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

  9. 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

  10. 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.

  11. 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.

  12. 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.

  13. 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.

  14. '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.

  15. 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...

  16. 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

  17. 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

  18. 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

  19. 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.

  20. 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.

  1. 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...

  2. 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.

  3. 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...

  4. 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)

  5. 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.

  6. 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...

  7. 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.

  8. 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.

  9. 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

  10. 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.

  11. 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,…

  12. 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.

  13. 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.

  14. 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

  15. 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

  16. '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.

  17. 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

  18. 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).

  19. 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

  20. 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

  1. 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...

  2. 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.

  3. 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.

  4. 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.

  5. 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 ...

  6. 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...

  7. 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...

  8. 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)

  9. 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.

  10. 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

  11. Size effects on rhodium nanoparticles related to hydrogen-storage capability.

    Science.gov (United States)

    Song, Chulho; Yang, Anli; Sakata, Osami; Kumara, L S R; Hiroi, Satoshi; Cui, Yi-Tao; Kusada, Kohei; Kobayashi, Hirokazu; Kitagawa, Hiroshi

    2018-06-06

    To unveil the origin of the hydrogen-storage properties of rhodium nanoparticles (Rh NPs), we investigated the electronic and crystal structures of the Rh NPs using various synchrotron based X-ray techniques. Electronic structure studies revealed that the hydrogen-storage capability of Rh NPs could be attributed to their more unoccupied d-DOSs than that of the bulk near the Fermi level. Crystal structure studies indicated that lattice distortion and mean-square displacement increase while coordination number decreases with decreasing particle size and the hydrogen-absorption capability of Rh NPs improves to a greater extent with increased structural disorder in the local structure than with that in the mean structure. The smallest Rh NPs, having the largest structural disorder/increased vacancy spaces and the smallest coordination number, exhibited excellent hydrogen-storage capacity. Finally, from the bond-orientational order analysis, we confirmed that the localized disordering is distributed more over the surface part than the core part and hydrogen can be trapped on the surface part of Rh NPs which increases with a decrease in NP diameter.

  12. 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.

  13. 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.

  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. 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

  16. 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:

  17. 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.

  18. 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

  19. 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.

  20. 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.

  1. 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

  2. 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.

  3. 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.

  4. 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.

  5. 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

  6. 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.

  7. 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.

  8. ALTERNATIVE FOR REDUCING PHYSIOLOGICAL DISORDERS IN ‘BARTLETT’ PEARS

    Directory of Open Access Journals (Sweden)

    MOISES ZUCOLOTO

    2016-01-01

    Full Text Available ABSTRACT ‘Bartlett’ pears from different harvest dates were assessed regarding to cold storage potential and reduction of physiological disorder incidence. Three harvests, the first (HD1, second (HD2, and third (HD3, were carried out at weekly intervals. The pears were assessed after the harvest, with no exposition to the temperature conditioning, after 20, 40, 60, 80, 100 and 120 days at 0 ± 1 °C and 90 ± 5% RH and after three and six days at room temperature (20 ± 1 °C. Fruit from the early harvest (HD1 showed the smallest incidence of physiological disorder during both cold and room temperature storage. The disorder symptoms became apparent in HD1 fruit after 20 days at cold storage followed by three days at 20 °C, whereas HD2 and HD3 fruit showed the symptoms before being kept in a cold room. ‘Bartlett’ pears harvested at 70.75 N flesh firmness can be stored at 0 ± 1 °C for up to 40 days and preferably commercialized within three days, when they reach the firmness for eating. The extension of cold storage as well as the trade period can result in higher physiological disorder incidence and loss of sensorial quality.

  9. 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.

  10. 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.

  11. Storage time of transfused blood and disease recurrence after colorectal cancer surgery

    DEFF Research Database (Denmark)

    Mynster, T; Nielsen, Hans Jørgen

    2001-01-01

    of the transfused blood. Therefore, we studied the relationship between blood storage time and the development of disease recurrence and long-term survival after colorectal cancer surgery. METHODS: Preoperative and postoperative data were prospectively recorded in 740 patients undergoing elective resection...... for primary colorectal cancer. None of the patients received preoperative or postoperative chemotherapy or radiation therapy. Endpoints were overall survival and disease recurrence in the subgroup of patients operated on with curative intention who also survived the first 30 days after operation. Storage......BACKGROUND: Perioperative blood transfusion and subsequent development of postoperative infectious complications may lead to poor prognosis of patients with colorectal cancer. It has been suggested that the development of postoperative infectious complications may be related to the storage time...

  12. 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...

  13. Hydrogen storage behavior of ZrCo1-xNix alloys

    International Nuclear Information System (INIS)

    Jat, Ram Avtar; Parida, S.C.; Agarwal, Renu; Kulkarni, S.G.

    2012-01-01

    Intermetallic compound ZrCo is proposed as a candidate material for storage, supply and recovery of hydrogen isotopes in International Thermonuclear Experimental Reactor (ITER) Storage and Delivery System (SDS). However, it has been reported that upon repeated hydriding-dehydriding cycles, ZrCo undergoes disproportionation as per the reaction; 2ZrCo + H 2 ↔ ZrH 2 + ZrCO 2 . This results in reduction in hydrogen storage capacity of ZrCo, which is not a desirable property for SDS. Konishi et al. reported that the disproportionation reaction can be suppressed by decreasing the desorption temperature. It is anticipated that suitable ternary alloying of ZrCo can elevated the hydrogen equilibrium pressure and hence decrease the desorption temperature for supply of 100 kPa of hydrogen. In this study, we have investigated the effect of Ni content on the hydrogenation behavior of ZrCo 1-x Ni x alloys

  14. 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 ...

  15. 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.

  16. Near-surface storage facilities for vitrified high-level wastes

    International Nuclear Information System (INIS)

    Kondrat'ev, A.N.; Kulichenko, V.V.; Kryukov, I.I.; Krylova, N.V.; Paramoshkin, V.I.; Strakhov, M.V.

    1980-01-01

    Concurrently with the development of methods for solidifying liquid radioactive wastes, reliable and safe methods for the storage and disposal of solidified wastes are being devised in the USSR and other countries. One of the main factors affecting the choice of storage conditions for solidified wastes originating from the vitrification of high-level liquid wastes from fuel reprocessing plants is the problem of removing the heat produced by radioactive decay. In order to prevent the temperature of solidified wastes from exceeding the maximum permissible level for the material concerned, it is necessary to limit either the capacity of waste containers or the specific heat release of the wastes themselves. In order that disposal of high-level wastes in geological formations should be reliable and economic, solidified wastes undergo interim storage in near-surface storage facilities with engineered cooling systems. The paper demonstrates the relative influences of specific heat release, of the maximum permissible storage temperature for vitrified wastes and of the methods chosen for cooling wastes in order for the dimensions of waste containers to be reduced to the extent required. The effect of concentrating wastes to a given level in the vitrification process on the cost of storage in different types of storage facility is also examined. Calculations were performed for the amount of vitrified wastes produced by a reprocessing plant with a capacity of five tonnes of uranium per 24 hours. Fuel elements from reactors of the water-cooled, water-moderated type are sent for reprocessing after having been held for about two years. The dimensions of the storage facility are calculated on the assumption that it will take five years to fill

  17. 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.

  18. 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.

  19. 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.

  20. 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.

  1. 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.

  2. 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.

  3. 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

  4. Prevalence and characteristics of avoidant/restrictive food intake disorder in a cohort of young patients in day treatment for eating disorders

    OpenAIRE

    Nicely, Terri A; Lane-Loney, Susan; Masciulli, Emily; Hollenbeak, Christopher S; Ornstein, Rollyn M

    2014-01-01

    Background Avoidant/Restrictive Food Intake Disorder (ARFID) is a “new” diagnosis in the recently published DSM-5, but there is very little literature on patients with ARFID. Our objectives were to determine the prevalence of ARFID in children and adolescents undergoing day treatment for an eating disorder, and to compare ARFID patients to other eating disorder patients in the same cohort. Methods A retrospective chart review of 7-17 year olds admitted to a day program for younger patients wi...

  5. 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...

  6. 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.

  7. 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....

  8. Dietary Management of the Ketogenic Glycogen Storage Diseases

    Directory of Open Access Journals (Sweden)

    Kaustuv Bhattacharya MBBS, MRCPCH, FRACP, MD

    2016-08-01

    Full Text Available The glycogen storage diseases (GSDs comprise a group of rare inherited disorders of glycogen metabolism. The hepatic glycogenolytic forms of these disorders are typically associated with hypoglycemia and hepatomegaly. For GSD I, secondary metabolic disturbances include fasting hyperlactatemia, hyperuricemia, and hyperlipidemia. Glycogen storage disease III is caused by reduced activity of the debrancher enzyme, GSD VI by phosphorylase, and GSD IX by phosphorylase kinase. It has often been reported that the non-GSD I group of disorders have a benign course. However, myopathy, cardiomyopathy, and cirrhosis have been reported significant clinical morbidities associated with GSD III and IX in particular. There have been a range of reports indicating high-protein diets, high-fat diets, medium chain triglyceride (MCT, modified Atkins diet, and therapeutic ketones as rescuing severe phenotypes of GSD III in particular. The etiology of these severe phenotypes has not been defined. Cases presented in this report indicate potential harm from excessive simple sugar use in GSD IX C. Review of the literature indicates that most interventions have reduced the glycemic load and provide alternate substrates for energy in rescue situations. Prevention of complications is most likely to occur with a mixed balanced low glycemic index diet potentially with relative increases in protein.

  9. 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.

  10. The Storage of Power Development and Research Reactor Fuel at Sellafield

    International Nuclear Information System (INIS)

    Standring, P.N.; Callaghan, A.H.C.

    2009-01-01

    Sellafield Limited has extensive experience of building and operating spent nuclear fuel storage facilities on the Sellafield site. Since the first operation in 1952, a total of six storage facilities have been built in support of reprocessing spent fuel. Currently, four of these facilities are operational and two are undergoing decommissioning activities. Whilst the routine spent fuel operations are primarily associated with managing Magnox, Advanced Gas Reactor and LWR fuel from power generation reactors, management services to other fuel types are offered. Examples of these services include the storage of British naval training reactor fuel; the reprocessing of two skips of aluminium clad uranium metal fuel from Swedish AB SVAFO and the management of fuel from the UK Power Development Programme. The current paper provides an account of the management of the UK's Power Development Programme fuel stored on the Sellafield site. The fuel has been pond stored for up to 42 years and periodic inspection during this time has revealed no significant deterioration of the fuel, particularly that which has been containerised during its storage period. The paper also outlines some of the issues associated with the recovery and transfer of long stored fuel and assessment of the fuel storage can longevity if the material is not reprocessed. (author)

  11. 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

  12. [Prevalence of depression and body dysmorphic disorder in patients before functional rhinosurgery].

    Science.gov (United States)

    Bender, M; Rustige, L; Lindemann, J

    2014-11-01

    Psychiatric disorders are known to influence the result of many surgical procedures. Body dysmorphic disorder (BDD) is found in many patients undergoing plastic surgery. The prevalence before functional rhinosurgery has not been examined so far. The aim of this study was to determine the prevalence of depression and BDD before functional rhinosurgery. 201 patients were prospectively examined with a questionnaire before functional rhinosurgery. Beck-Depression-Index was used to rate depression, the PISA body dsymorphic symptom scale was used to evaluate the likelihood that a patient suffers from body dysmoprhic disorder. 186 patients returned a complete questionnaire. In 33.9% showed a mild or strong indication for a body dysmorphic disorder. Patients who were planned to undergo septorhinoplasty had a significantly higher scores in the PISA-scale compared to patients before septoplasty. 1.7% patients were depressive without a significant difference between the planned surgical procedure. Many patients before functional septorhinoplasty show signs of body dysmorphic disorder. Whether this influences the subjective clinical outcome needs to be evaluated in further studies. © Georg Thieme Verlag KG Stuttgart · New York.

  13. 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.

  14. 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.

  15. 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

  16. Chain Stretching and Order-Disorder Transitions in Block Copolymer Monolayers and Multilayers

    Science.gov (United States)

    Kramer, Edward J.; Mishra, Vindhya; Stein, Gila E.; Sohn, Karen E.; Hur, Sumi; Fredrickson, Glenn H.; Cochran, Eric W.

    2009-03-01

    Both monolayers of block copolymer cylinders and spheres undergo order to disorder transitions (ODT) at temperatures well below those of the bulk. Monolayers of PS-b-P2VP cylinders undergo a ``nematic'' to ``isotropic'' transition at temperatures about 20 K below the bulk ODT while monolayers of PS-b-P2VP with P2VP spheres undergo a 2D crystal to hexatic transition at least 10 K below the bulk ODT. Bilayers of each structure disorder at temperatures well above that of the monolayers. While one is tempted to attribute all of the difference to the fact that ordered monolayers are quasi 2 dimensional while bilayers are not, an alternative explanation exists. In the cylinder monolayer the corona PS chains must stretch to fill a nearly square cross-section domain rather than a hexagonal one in the bulk, while the corona PS chains in a sphere monolayer must stretch to fill a hexagonal prism rather than an octahedron in the bulk. The more non-uniform stretching of the chains in the monolayer should increase its free energy and decrease its order-disorder temperature.

  17. 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.

  18. What happens to anxiety disorders in later life?

    Directory of Open Access Journals (Sweden)

    Byrne Gerard JA

    2002-01-01

    Full Text Available Anxiety disorders decline in prevalence with advancing age but remain more common than depressive disorders. They are often of late-onset and there is frequent comorbidity with depressive disorders and physical illness. While anxiety disorders in older people are likely to respond to the same non-pharmacological interventions that have been shown to work in younger people, there is currently little formal evidence of this. Although there is some evidence that the non-benzodiazepine anxiolytic medication, buspirone, is effective against late life anxiety symptoms, clinical trials in older people with rigorously diagnosed anxiety disorders are needed. An anxiety scale with demonstrated reliability and validity in older people is needed for screening for pathological anxiety and for measuring change in older patients undergoing treatment for anxiety disorders.

  19. 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.

  20. 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

  1. 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...

  2. Radical covalent organic frameworks: a general strategy to immobilize open-accessible polyradicals for high-performance capacitive energy storage.

    Science.gov (United States)

    Xu, Fei; Xu, Hong; Chen, Xiong; Wu, Dingcai; Wu, Yang; Liu, Hao; Gu, Cheng; Fu, Ruowen; Jiang, Donglin

    2015-06-01

    Ordered π-columns and open nanochannels found in covalent organic frameworks (COFs) could render them able to store electric energy. However, the synthetic difficulty in achieving redox-active skeletons has thus far restricted their potential for energy storage. A general strategy is presented for converting a conventional COF into an outstanding platform for energy storage through post-synthetic functionalization with organic radicals. The radical frameworks with openly accessible polyradicals immobilized on the pore walls undergo rapid and reversible redox reactions, leading to capacitive energy storage with high capacitance, high-rate kinetics, and robust cycle stability. The results suggest that channel-wall functional engineering with redox-active species will be a facile and versatile strategy to explore COFs for energy storage. © 2015 WILEY-VCH Verlag GmbH & Co. KGaA, Weinheim.

  3. Storage time of transfused blood and disease recurrence after colorectal cancer surgery

    DEFF Research Database (Denmark)

    Mynster, T; Nielsen, Hans Jørgen

    2001-01-01

    of the transfused blood. Therefore, we studied the relationship between blood storage time and the development of disease recurrence and long-term survival after colorectal cancer surgery. METHODS: Preoperative and postoperative data were prospectively recorded in 740 patients undergoing elective resection......BACKGROUND: Perioperative blood transfusion and subsequent development of postoperative infectious complications may lead to poor prognosis of patients with colorectal cancer. It has been suggested that the development of postoperative infectious complications may be related to the storage time...... transfused patients (P = 0.004). The survival of patients receiving blood exclusively stored blood stored > or = 21 days, survival was 3.7 years (P = 0.12). Among patients with curative resection (n = 532), the hazard ratio of disease recurrence was 1.5 (95...

  4. Critical Phenomena of the Disorder Driven Localization-Delocalization Transition

    International Nuclear Information System (INIS)

    Marc Ruehlaender

    2001-01-01

    Metal-to-insulator transitions are generally linked to two phenomena: electron-electron correlations and disorder. Although real systems are usually responding to a mixture of both, they can be classified as undergoing a Mott-transition, if the former process dominates, or an Anderson-transition, if the latter dominates. High-T c superconductors, e.g., are a candidate for the first class. Materials in which disorder drives the metal-to-insulator transition include doped semiconductors and amorphous materials. After briefly reviewing the previous research on transport in disordered materials and the disorder-induced metal-to-insulator transition, a summary of the model and the methods used in subsequent chapters is given

  5. 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.

  6. 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

  7. 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.

  8. Evaluation of Physico-biochemichal Characteristics of Fruits in New Varieties of Noushin and Shahin Mandarins During Storage

    Directory of Open Access Journals (Sweden)

    Javad Fatahi Moghadam

    2018-03-01

    Full Text Available Introduction: According to a breeding program that was carried out in Citrus and Subtropical Fruits Research Center, Noushin (C. reticulata cv Clementine × C. sinensis cv. Salustiana and Shahin (C. reticulata cv Clementine × C. sinensis cv. Hamlin mandarins were released by using crossing method in 20-year program. In general, mandarins do not have the ability of being kept in common or cold storage for long time compared to oranges. The main problem is the change of fruit taste during storage, therefore, it is an attracted subject for researchers. Furthermore, storage temperature plays an important role in the quality of the taste of mandarins. The new released mandarins, which hve not been yet studied completely for their storability, need to be evaluated. Therefore, the aim of this study was to investigate fruit physico-chemical and sensory characteristics of Noushin and Shahin varieties in common and cold storages for two years. Materials and Methods: In this study, fruits of Noshin and Shahin mandarins were harvested at seasonal harvesting time and then placed in could storage (5 oC and 85% RH and common storage (7-10 oC and 60-70 %RH based on completely randomized design with three replications for 60 days every year. Fruits on days 0 (at harvesting time, 20, 40 and 60 of storage were sampled. Various physico-chemical and sensory characteristics were evaluated including iuice percentage, weight loss, peel color indices (L*, a*, b*, hue angle, chroma and CCI, total soluble solid (TSS, titratable acidity (TA, technological index (TI, skin disorder index (SDI, pH, electrical conductivity (EC, total phenolic, ascorbic acid, antioxidant capacity and sensory parameters during experiment. Statistical analysis of the data was performed using statistical software MSTAT-C. Analysis of variance combined in a randomized complete design (two years with three replications for each variety. Results and Discussion: The results showed that the amount of

  9. Opposed-phase MR imaging of lipid storage myopathy in a case of Chanarin-Dorfman disease

    International Nuclear Information System (INIS)

    Gaeta, Michele; Celona, Antonio; Racchiusa, Sergio; Mazziotti, Silvio; Minutoli, Fabio; Toscano, Antonio; Musumeci, Olimpia

    2008-01-01

    Chanarin-Dorfman disease (CDD) is a rare genetic disorder characterized by ichthyosis, myopathy, central nervous system disturbances, and intracellular lipid storage in muscle fibers, hepatocytes, and granulocytes. We describe skeletal muscle magnetic resonance imaging findings in a case of CDD, outlining the potential role of GE T1-weighted opposed-phase sequence (chemical shift imaging) in the evaluation of lipid storage myopathies. (orig.)

  10. Opposed-phase MR imaging of lipid storage myopathy in a case of Chanarin-Dorfman disease

    Energy Technology Data Exchange (ETDEWEB)

    Gaeta, Michele; Celona, Antonio; Racchiusa, Sergio; Mazziotti, Silvio [University of Messina, Department of Radiological Sciences, Messina (Italy); Minutoli, Fabio [University of Messina, Department of Radiological Sciences, Messina (Italy); A.O.U. ' ' Policlinico G. Martino' ' , Dipartimento di Scienze Radiologiche, Messina (Italy); Toscano, Antonio; Musumeci, Olimpia [University of Messina, Department of Neurosciences, Psychiatry and Anaesthesiology, Messina (Italy)

    2008-11-15

    Chanarin-Dorfman disease (CDD) is a rare genetic disorder characterized by ichthyosis, myopathy, central nervous system disturbances, and intracellular lipid storage in muscle fibers, hepatocytes, and granulocytes. We describe skeletal muscle magnetic resonance imaging findings in a case of CDD, outlining the potential role of GE T1-weighted opposed-phase sequence (chemical shift imaging) in the evaluation of lipid storage myopathies. (orig.)

  11. 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

  12. 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.

  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. Disorders of muscle lipid metabolism: diagnostic and therapeutic challenges.

    Science.gov (United States)

    Laforêt, Pascal; Vianey-Saban, Christine

    2010-11-01

    Disorders of muscle lipid metabolism may involve intramyocellular triglyceride degradation, carnitine uptake, long-chain fatty acids mitochondrial transport, or fatty acid β-oxidation. Three main diseases leading to permanent muscle weakness are associated with severe increased muscle lipid content (lipid storage myopathies): primary carnitine deficiency, neutral lipid storage disease and multiple acyl-CoA dehydrogenase deficiency. A moderate lipidosis may be observed in fatty acid oxidation disorders revealed by rhabdomyolysis episodes such as carnitine palmitoyl transferase II, very-long-chain acyl-CoA dehydrogenase, mitochondrial trifunctional protein deficiencies, and in recently described phosphatidic acid phosphatase deficiency. Respiratory chain disorders and congenital myasthenic syndromes may also be misdiagnosed as fatty acid oxidation disorders due to the presence of secondary muscle lipidosis. The main biochemical tests giving clues for the diagnosis of these various disorders are measurements of blood carnitine and acylcarnitines, urinary organic acid profile, and search for intracytoplasmic lipid on peripheral blood smear (Jordan's anomaly). Genetic analysis orientated by the results of biochemical investigation allows establishing a firm diagnosis. Primary carnitine deficiency and multiple acyl-CoA dehydrogenase deficiency may be treated after supplementation with carnitine, riboflavine and coenzyme Q10. New therapeutic approaches for fatty acid oxidation disorders are currently developed, based on pharmacological treatment with bezafibrate, and specific diets enriched in medium-chain triglycerides or triheptanoin. Copyright © 2010 Elsevier B.V. All rights reserved.

  15. Engineering and planning for reactor 105-C interim safe storage project subcontract no. 0100C-SC-G0001 conceptual design report. Volume 1

    International Nuclear Information System (INIS)

    1996-04-01

    The 105-C Reactor, one of eight surplus production reactors at the Hanford Site, has been proposed by the U.S. Department of Energy, Richland, Operations Office to be the first large-scale technology demonstration project in the decontamination and decommissioning (D ampersand D) focus area as part of the project for dismantlement and interim safe storage. The 105-C Reactor will be placed in an interim safe storage condition, then undergo the decontamination and decommissioning phase. After D ampersand D, the reactor will be placed in long- term safe storage. This report provides the conceptual design for these activities

  16. Disorder-induced transitions in resonantly driven Floquet topological insulators

    Science.gov (United States)

    Titum, Paraj; Lindner, Netanel H.; Refael, Gil

    2017-08-01

    We investigate the effects of disorder in Floquet topological insulators (FTIs) occurring in semiconductor quantum wells. Such FTIs are induced by resonantly driving a transition between the valence and conduction bands. We show that when disorder is added, the topological nature of such FTIs persists as long as there is a mobility gap at the resonant quasienergy. For strong enough disorder, this gap closes and all the states become localized as the system undergoes a transition to a trivial insulator. Interestingly, the effects of disorder are not necessarily adverse: we show that in the same quantum well, disorder can also induce a transition from a trivial to a topological system, thereby establishing a Floquet topological Anderson insulator (FTAI). We identify the conditions on the driving field necessary for observing such a transition.

  17. Fulminant lipid storage myopathy due to multiple acyl-coenzyme a dehydrogenase deficiency.

    Science.gov (United States)

    Whitaker, Charles H; Felice, Kevin J; Silvers, David; Wu, Qian

    2015-08-01

    The lipid storage myopathies, primary carnitine deficiency, neutral lipid storage disease, and multiple acyl coenzyme A dehydrogenase deficiency (MADD), are progressive disorders that cause permanent weakness. These disorders of fatty acid metabolism and intracellular triglyceride degradation cause marked fat deposition and damage to muscle cells. We describe a rapidly progressive myopathy in a previously healthy 33-year-old woman. Over 4 months, she developed a proximal and axial myopathy associated with diffuse myalgia and dysphagia, ultimately leading to respiratory failure and death. Muscle biopsy showed massive accumulation of lipid. Plasma acylcarnitine and urine organic acid analysis was consistent with MADD. This was confirmed by molecular genetic testing, which revealed 2 pathogenic mutations in the ETFDH gene. This report illustrates a late-onset case of MADD and reviews the differential diagnosis and evaluation of patients with proximal myopathy and excessive accumulation of lipid on muscle biopsy. © 2014 Wiley Periodicals, Inc.

  18. 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…

  19. 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)

  20. Extensive colonization of apples by smut anamorphs causes a new posthavest disorder

    NARCIS (Netherlands)

    Boekhout, T.; Gildemacher, P.R.; Theelen, B.; Müller, W.H.; Heijne, B.; Lutz, M.

    2006-01-01

    Colonization of apples by ballistoconidium-forming fungi causes a new disorder, here named 'white haze'. White haze may occur in mild form in the field, but only becomes problematic after Ultra-Low Oxygen storage, and, therefore, may be considered as a postharvest disorder. All isolates, obtained

  1. 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...

  2. Generalized glycogen storage and cardiomegaly in a knockout mouse model of Pompe disease

    NARCIS (Netherlands)

    A.G.A. Bijvoet (Agnes); A.T. van der Ploeg (Ans); E.H. van de Kamp; M.A. Kroos (Marian); J.-H. Ding (Jia-Huan); B.Z. Yang (Bing); P. Visser (Pim); C.E. Bakker (Cathy); M.Ph. Verbeet (Martin); B.A. Oostra (Ben); A.J.J. Reuser (Arnold)

    1998-01-01

    textabstractGlycogen storage disease type II (GSDII; Pompe disease), caused by inherited deficiency of acid alpha-glucosidase, is a lysosomal disorder affecting heart and skeletal muscles. A mouse model of this disease was obtained by targeted disruption of the

  3. Different Storage Conditions Influence Biocompatibility and Physicochemical Properties of Iron Oxide Nanoparticles

    Directory of Open Access Journals (Sweden)

    Jan Zaloga

    2015-04-01

    Full Text Available Superparamagnetic iron oxide nanoparticles (SPIONs have attracted increasing attention in many biomedical fields. In magnetic drug targeting SPIONs are injected into a tumour supplying artery and accumulated inside the tumour with a magnet. The effectiveness of this therapy is thus dependent on magnetic properties, stability and biocompatibility of the particles. A good knowledge of the effect of storage conditions on those parameters is of utmost importance for the translation of the therapy concept into the clinic and for reproducibility in preclinical studies. Here, core shell SPIONs with a hybrid coating consisting of lauric acid and albumin were stored at different temperatures from 4 to 45 °C over twelve weeks and periodically tested for their physicochemical properties over time. Surprisingly, even at the highest storage temperature we did not observe denaturation of the protein or colloidal instability. However, the saturation magnetisation decreased by maximally 28.8% with clear correlation to time and storage temperature. Furthermore, the biocompatibility was clearly affected, as cellular uptake of the SPIONs into human T-lymphoma cells was crucially dependent on the storage conditions. Taken together, the results show that the particle properties undergo significant changes over time depending on the way they are stored.

  4. Antioxidative Activity of Colostrum and Human Milk: Effects of Pasteurization and Storage.

    Science.gov (United States)

    Marinković, Vesna; Ranković-Janevski, Milica; Spasić, Snežana; Nikolić-Kokić, Aleksandra; Lugonja, Nikoleta; Djurović, Dijana; Miletić, Srdjan; Vrvić, Miroslav M; Spasojević, Ivan

    2016-06-01

    Milk banks collect, pasteurize, and freeze/store human milk. The processing may alter redox properties of milk, but the effects have not been fully examined. We collected 10 mature milk and 10 colostrum samples and applied a battery of biochemical assays and electron paramagnetic resonance spectroscopy to inspect changes that milk undergoes with pasteurization and 30 days storage at -20°C. Pasteurization and storage of raw milk did not affect total nonenzymatic antioxidative capacity, but specific components and features were altered. Urate radical and ascorbyl radical emerge as products of exposure of milk to hydroxyl radical-generating system. Processing shifted the load of antioxidative activity from ascorbate to urate and lowered the capacity of milk to diminish hydroxyl radical. Pasteurization caused a significant drop in the activity of 2 major antioxidative enzymes-superoxide dismutase and glutathione peroxidase, whereas freezing/storage of raw milk affected only superoxide dismutase. Colostrum showed drastically higher total nonenzymatic antioxidative capacity, hydroxyl radical scavenging ability, and glutathione reductase activity compared with mature milk. Pasteurization and storage affect nonenzymatic and enzymatic antioxidative agents in human milk. It appears that nonenzymatic antioxidative systems in colostrum and milk are different. The effects of processing may be partially compensated by fortification/spiking with ascorbate before use.

  5. Demonstration of Isothermal Compressed Air Energy Storage to Support Renewable Energy Production

    Energy Technology Data Exchange (ETDEWEB)

    Bollinger, Benjamin [Sustainx, Incorporated, Seabrook, NH (United States)

    2015-01-02

    This project develops and demonstrates a megawatt (MW)-scale Energy Storage System that employs compressed air as the storage medium. An isothermal compressed air energy storage (ICAESTM) system rated for 1 MW or more will be demonstrated in a full-scale prototype unit. Breakthrough cost-effectiveness will be achieved through the use of proprietary methods for isothermal gas cycling and staged gas expansion implemented using industrially mature, readily-available components.The ICAES approach uses an electrically driven mechanical system to raise air to high pressure for storage in low-cost pressure vessels, pipeline, or lined-rock cavern (LRC). This air is later expanded through the same mechanical system to drive the electric motor as a generator. The approach incorporates two key efficiency-enhancing innovations: (1) isothermal (constant temperature) gas cycling, which is achieved by mixing liquid with air (via spray or foam) to exchange heat with air undergoing compression or expansion; and (2) a novel, staged gas-expansion scheme that allows the drivetrain to operate at constant power while still allowing the stored gas to work over its entire pressure range. The ICAES system will be scalable, non-toxic, and cost-effective, making it suitable for firming renewables and for other grid applications.

  6. Symptomatic lipid storage in carriers for the PNPLA2 gene.

    Science.gov (United States)

    Janssen, Mirian C H; van Engelen, Baziel; Kapusta, Livia; Lammens, Martin; van Dijk, Martin; Fischer, Judith; van der Graaf, Marinette; Wevers, Ron A; Fahrleitner, Manuela; Zimmermann, Robert; Morava, Eva

    2013-08-01

    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 associated cardiac disease, myopathy and frequent infections, but without ichthyosis. Here we describe the clinical and biochemical characteristics of a long surviving patient and report on four carrier family members with diverse clinical involvement. Interestingly, heterozygous patients show neutral lipid storage in muscle and in the keratocytes of the skin, Jordans' bodies, mild myopathy and frequent infections. Biochemical analysis of fibroblasts obtained from patients revealed increased triglyceride storage and reduced lipid droplet-associated triglyceride hydrolase activity. Together, our data implicate that the wild-type allele cannot fully compensate for the mutated dysfunctional allele of PNPLA2 leading to triglyceride accumulation in muscle and mild myopathy in PNPLA2 mutation carriers. The presence of neutral lipid droplets in the skin in PNPLA2 mutation carriers strengthens the link between NLSD and other neutral lipid storage diseases with ichthyosis.

  7. 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

  8. Research highlights of partial neuromuscular disorders

    Directory of Open Access Journals (Sweden)

    Cheng ZHANG

    2014-05-01

    Full Text Available In order to understand the latest progression on neuromuscular disorders for clinicians, this review screened and systemized the papers on neuromuscular disorders which were collected by PubMed from January 2013 to February 2014. This review also introduced the clinical diagnosis and treatment hightlights on glycogen storage disease type Ⅱ (GSD Ⅱ, Duchenne muscular dystrophy (DMD, amyotrophic lateral sclerosis (ALS and spinal muscular atrophy (SMA. The important references will be useful for clinicians. doi: 10.3969/j.issn.1672-6731.2014.05.004

  9. Type IIIb glycogen storage disease associated with end-stage cirrhosis and hepatocellular carcinoma

    NARCIS (Netherlands)

    Haagsma, EB; Smit, GPA; NiezenKoning, KE; Gouw, ASH; Meerman, L; Slooff, MJH

    Type III glycogen storage disease (GSD) is a disorder of carbohydrate metabolism caused by a deficiency of debranching enzyme. Different subtypes with different clinical pictures have been recognized. During childhood and early adulthood, the symptoms generally regress, and normal adulthood appears

  10. Condensation with two constraints and disorder

    Science.gov (United States)

    Barré, J.; Mangeolle, L.

    2018-04-01

    We consider a set of positive random variables obeying two additive constraints, a linear and a quadratic one; these constraints mimic the conservation laws of a dynamical system. In the simplest setting, without disorder, it is known that such a system may undergo a ‘condensation’ transition, whereby one random variable becomes much larger than the others; this transition has been related to the spontaneous appearance of non linear localized excitations in certain nonlinear chains, called breathers. Motivated by the study of breathers in a disordered discrete nonlinear Schrödinger equation, we study different instances of this problem in presence of a quenched disorder. Unless the disorder is too strong, the phase diagram looks like the one without disorder, with a transition separating a fluid phase, where all variables have the same order of magnitude, and a condensed phase, where one variable is much larger than the others. We then show that the condensed phase exhibits various degrees of ‘intermediate symmetry breaking’: the site hosting the condensate is chosen neither uniformly at random, nor is it fixed by the disorder realization. Throughout the article, our heuristic arguments are complemented with direct Monte Carlo simulations.

  11. Detection of airborne psychrotrophic bacteria and fungi in food storage refrigerators

    Directory of Open Access Journals (Sweden)

    Sema Sandikci Altunatmaz

    2012-12-01

    Full Text Available The purpose of this study was to determine the microbiological air quality (psychrotrophic bacteria and airborne fungi and distribution of fungi in different types of ready-to-eat (RTE food-storage refrigerators (n=48 at selected retail stores in the city of Edirne, Turkey. Refrigerators were categorized according to the type of RTE food-storage: meat products, vegetables, desserts, or a mix of food types. Microbiological quality of air samples was evaluated by using a Mas-100 Eco Air Sampler. Four refrigerators (all containing meat products, 8.3% produced air samples with undetectable microorganisms. The highest detected mean value of airborne psychrotrophic bacteria and fungi was 82.3 CFU/m³ and 54.6 CFU/m³, respectively and were found in mixed-food refrigerators. The dominant airborne fungal genera found were Penicillium (29.0%, Aspergillus (12.0%, Mucor (9%, Cladosporium (8%, Botyrtis (7%, and Acremonium (6%. By definition, RTE food does not undergo a final treatment to ensure its safety prior to consumption. Therefore, ensuring a clean storage environment for these foods is important to prevent food-borne disease and other health risks.

  12. [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.

  13. Schizophrenia, depression, and sleep disorders: Their traditional oriental medicine equivalents

    NARCIS (Netherlands)

    Bosch, M.P.C.; Rover, P. de; Staudte, H.; Lim, S.; Noort, M.W.M.L. van den

    2015-01-01

    Psychiatric disorders can be described and treated from both a Western (allopathic) and an Eastern perspective, which should be taken into account when conducting research. Patients with schizophrenia or depression are likely to be undergoing Western treatment when they are referred to an

  14. 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.

  15. 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.

  16. Prevention of Lysosomal Storage Diseases and Derivation of Mutant Stem Cell Lines by Preimplantation Genetic Diagnosis

    Science.gov (United States)

    Altarescu, Gheona; Beeri, Rachel; Eiges, Rachel; Epsztejn-Litman, Silvina; Eldar-Geva, Talia; Elstein, Deborah; Zimran, Ari; Margalioth, Ehud J.; Levy-Lahad, Ephrat; Renbaum, Paul

    2012-01-01

    Preimplantation genetic diagnosis (PGD) allows birth of unaffected children for couples at risk for a genetic disorder. We present the strategy and outcome of PGD for four lysosomal storage disorders (LSD): Tay-Sachs disease (TSD), Gaucher disease (GD), Fabry disease (FD), and Hunter syndrome (HS), and subsequent development of stem cell lines. For each disease, we developed a family-specific fluorescent multiplex single-cell PCR protocol that included the familial mutation and informative markers surrounding the mutation. Embryo biopsy and PGD analysis were performed on either oocytes (polar bodies one and two) or on single blastomeres from a six-cell embryo. We treated twenty families carrying mutations in these lysosomal storage disorders, including 3 couples requiring simultaneous analysis for two disorders (TSD/GD, TSD/balanced Robertsonian translocation 45XYder(21;14), and HS/oculocutaneus albinism). These analyses led to an overall pregnancy rate/embryo transfer of 38% and the birth of 20 unaffected children from 17 families. We have found that PGD for lysosomal disorders is a safe and effective method to prevent birth of affected children. In addition, by using mutant embryos for the derivation of stem cell lines, we have successfully established GD and HS hESC lines for use as valuable models in LSD research. PMID:23320174

  17. 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.

  18. 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.

  19. 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...

  20. 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

  1. In-vivo staging of pathology in REM sleep behaviour disorder

    DEFF Research Database (Denmark)

    Knudsen, Karoline; Fedorova, Tatyana D.; Hansen, Allan K.

    2018-01-01

    originating in the locus coeruleus, and 18F-dihydroxyphenylalanine (DOPA) PET to assess nigrostriatal dopamine storage capacity. For each imaging modality, we compared patients with idiopathic REM sleep behaviour disorder with previously published reference data of controls without neurological disorders...... or cognitive impairment and with symptomatic patients with Parkinson's disease. We assessed imaging data using one-way ANOVA corrected for multiple comparisons. Findings: Between June 3, 2016, and Dec 19, 2017, we recruited 22 consecutive patients with idiopathic REM sleep behaviour disorder to the study...... REM sleep behaviour disorder (pequal to that in diagnosed Parkinson's disease. These patients also showed noradrenergic...

  2. 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.

  3. An inhibition of p38 mitogen activated protein kinase delays the platelet storage lesion.

    Directory of Open Access Journals (Sweden)

    Andrey Skripchenko

    Full Text Available BACKGROUND AND OBJECTIVES: Platelets during storage undergo diverse alterations collectively known as the platelet storage lesion, including metabolic, morphological, functional and structural changes. Some changes correlate with activation of p38 mitogen activated protein kinase (p38 MAPK. Another MAPK, extracellular signal-related kinase (ERK, is involved in PLT activation. The aim of this study was to compare the properties of platelets stored in plasma in the presence or absence of p38 and ERK MAPK inhibitors. MATERIALS AND METHODS: A single Trima apheresis platelet unit (n = 12 was aliquoted into five CLX storage bags. Two aliquots were continuously agitated with or without MAPK inhibitors. Two aliquots were subjected to 48 hours of interruption of agitation with or without MAPK inhibitors. One aliquot contained the same amount of solvent vehicle used to deliver the inhibitor. Platelets were stored at 20-24°C for 7 days and sampled on Days 1, 4, and 7 for 18 in vitro parameters. RESULTS: Inhibition of p38 MAPK by VX-702 leads to better maintenance of all platelet in vitro storage parameters including platelet mitochondrial function. Accelerated by interruption of agitation, the platelet storage lesion of units stored with VX-702 was diminished to that of platelets stored with continuous agitation. Inhibition of ERK MAPK did not ameliorate decrements in any in vitro platelet properties. CONCLUSION: Signaling through p38 MAPK, but not ERK, is associated with platelet deterioration during storage.

  4. Effect of storage and LEO cycling on manufacturing technology IPV nickel-hydrogen cells

    Science.gov (United States)

    Smithrick, John J.

    1987-01-01

    Yardney Manufacturing Technology (MANTECH) 50 A-hr space weight individual pressure vessel nickel-hydrogen cells were evaluated. This consisted of investigating: the effect of storage and charge/discharge cycling on cell performance. For the storage test the cells were precharged with hydrogen, by the manufacturer, to a pressure of 14.5 psia. After undergoing activation and acceptance tests, the cells were discharged at C/10 rate (5A) to 0.1 V or less. The terminals were then shorted. The cells were shipped to NASA Lewis Research Center where they were stored at room temperature in the shorted condition for 1 year. After storage, the acceptance tests were repeated at NASA Lewis. A comparison of test results indicate no significant degradation in electrical performance due to 1 year storage. For the cycle life test the regime was a 90 minute low earth orbit at deep depths of discharge (80 and 60 percent). At the 80 percent DOD the three cells failed on the average at cycle 741. Failure for this test was defined to occur when the cell voltage degraded to 1 V prior to completion of the 35 min discharge. The DOD was reduced to 60 percent. The cycle life test was continued.

  5. 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 ...

  6. Assessment of changes in plasma hemoglobin and potassium levels in red cell units during processing and storage.

    Science.gov (United States)

    Saini, Nishant; Basu, Sabita; Kaur, Ravneet; Kaur, Jasbinder

    2015-06-01

    Red cell units undergo changes during storage and processing. The study was planned to assess plasma potassium, plasma hemoglobin, percentage hemolysis during storage and to determine the effects of outdoor blood collection and processing on those parameters. Blood collection in three types of blood storage bags was done - single CPDA bag (40 outdoor and 40 in-house collection), triple CPD + SAGM bag (40 in-house collection) and quadruple CPD + SAGM bag with integral leukoreduction filter (40 in-house collection). All bags were sampled on day 0 (day of collection), day 1 (after processing), day 7, day 14 and day 28 for measurement of percentage hemolysis and potassium levels in the plasma of bag contents. There was significant increase in percentage hemolysis, plasma hemoglobin and plasma potassium level in all the groups during storage (p levels during the storage of red blood cells. Blood collection can be safely undertaken in outdoor blood donation camps even in hot summer months in monitored blood transport boxes. SAGM additive solution decreases the red cell hemolysis and allows extended storage of red cells. Prestorage leukoreduction decreases the red cell hemolysis and improves the quality of blood. Copyright © 2015 Elsevier Ltd. All rights reserved.

  7. A Review on Disorder-Driven Metal-Insulator Transition in Crystalline Vacancy-Rich GeSbTe Phase-Change Materials.

    Science.gov (United States)

    Wang, Jiang-Jing; Xu, Ya-Zhi; Mazzarello, Riccardo; Wuttig, Matthias; Zhang, Wei

    2017-07-27

    Metal-insulator transition (MIT) is one of the most essential topics in condensed matter physics and materials science. The accompanied drastic change in electrical resistance can be exploited in electronic devices, such as data storage and memory technology. It is generally accepted that the underlying mechanism of most MITs is an interplay of electron correlation effects (Mott type) and disorder effects (Anderson type), and to disentangle the two effects is difficult. Recent progress on the crystalline Ge₁Sb₂Te₄ (GST) compound provides compelling evidence for a disorder-driven MIT. In this work, we discuss the presence of strong disorder in GST, and elucidate its effects on electron localization and transport properties. We also show how the degree of disorder in GST can be reduced via thermal annealing, triggering a disorder-driven metal-insulator transition. The resistance switching by disorder tuning in crystalline GST may enable novel multilevel data storage devices.

  8. 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...

  9. Use of storage substance in garlic propagules irradiated of gamma rays

    International Nuclear Information System (INIS)

    Perez Talavera, S.; Lopez Cervantes, M.; Perez Lezcano, A.

    1997-01-01

    Gamma radioinduced effects on different growth indicators and the destination of storage substances of cloves were investigated in garlic cv. Martinez. To this purpose, the doses interval of its radiosensitivity curve was considered. Within the Gr 10-20 (growth reduction) interval there was no damage or if was small in growth indicators and also leaves width was stimulated, enabling to each leave areas similar to those of testing plants. In addition significant increases of storage compounds use in cloves were observed. This result may be interpreted as an additional energetic consumption to repair radiation damage. Behaviour starting at Gr 30 may be critical because plants undergo severe damage in most of the analyzed indicators principally those that depend of leaves area. This result. from a physiological point of view, complement the recommendation of selecting doses in Gr 10-20 interval for the radio mutagenesis works, recommendation made in base of small lethality and sterility, and high percentage of useful mutation reported for this doses interval

  10. 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.

  11. Loss of control eating and eating disorders in adolescents before bariatric surgery.

    Science.gov (United States)

    Utzinger, Linsey M; Gowey, Marissa A; Zeller, Meg; Jenkins, Todd M; Engel, Scott G; Rofey, Dana L; Inge, Thomas H; Mitchell, James E

    2016-10-01

    This study assessed loss of control (LOC) eating and eating disorders (EDs) in adolescents undergoing bariatric surgery for severe obesity. Preoperative baseline data from the Teen Longitudinal Assessment of Bariatric Surgery (Teen-LABS) multisite observational study (n = 242; median BMI = 51 kg/m 2 ; mean age= 17; 76% female adolescents; 72% Caucasian) included anthropometric and self-report questionnaires, including the Questionnaire of Eating and Weight Patterns-Revised (QEWP-R), the Night Eating Questionnaire (NEQ), the Beck Depression Inventory (BDI-II), and the Impact of Weight on Quality of Life-Kids (IWQOL-Kids) RESULTS: LOC eating (27%) was common and ED diagnoses included binge-eating disorder (7%), night eating syndrome (5%), and bulimia nervosa (1%). Compared to those without LOC eating, those with LOC eating reported greater depressive symptomatology and greater impairment in weight-related quality of life. Before undergoing bariatric surgery, adolescents with severe obesity present with problematic disordered eating behaviors and meet diagnostic criteria for EDs. LOC eating, in particular, was associated with several negative psychosocial factors. Findings highlight targets for assessment and intervention in adolescents before bariatric surgery. © 2016 Wiley Periodicals, Inc.(Int J Eat Disord 2016; 49:947-952). © 2016 Wiley Periodicals, Inc.

  12. 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.

  13. 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

  14. Differentiation of norm and pathology during selective biochemical skreening of lysosomal storage diseases with increased excretion of oligosaccharides

    Directory of Open Access Journals (Sweden)

    N. Y. Mytsyk

    2015-06-01

    Full Text Available Oligosaccharides are a class of polymeric carbohydrates, which are constituents of a glycoside portion of glycoprotein and glycolipid molecules. The lysosomal hydrolase dysfunction due to lysosomal storage disorders results in partial or complete failure of degradation of some glycoproteins and glycolipids, causing the accumulation of specific undegraded substrates in the lysosomes of cells, the formation of the great number of oligosaccharide chains and their increased excretion with urine. Our work was aimed at detailed study of the specificities of interpreting the results of thin-layer chromatography (TLC of urine oligosaccharides in healthy persons of different age groups with the purpose of further application of these data while differentiating the norm and pathology in the course of primary selective screening of lysosomal storage disorders. The results obtained demonstrated that TLC plates for the majority of healthy persons had insignificant excretion of a number of oligosaccharides (from monosaccharides to hexasaccharides with Rlac > 0.15, which can be characterized as physiological oligosacchariduria, conditioned by the metabolism specificities in lysosomes. Therefore while interpreting the urine samples of patients with the suspected lysosomal storage disorder it is diagnostically reasonable to examine the TLC plates for the presence of both oligosaccharide groups, absent in the samples of healthy persons, and all the fractions with Rlac < 0.15.

  15. 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.

  16. A Review on Disorder-Driven Metal–Insulator Transition in Crystalline Vacancy-Rich GeSbTe Phase-Change Materials

    Science.gov (United States)

    Wang, Jiang-Jing; Xu, Ya-Zhi; Mazzarello, Riccardo; Wuttig, Matthias; Zhang, Wei

    2017-01-01

    Metal–insulator transition (MIT) is one of the most essential topics in condensed matter physics and materials science. The accompanied drastic change in electrical resistance can be exploited in electronic devices, such as data storage and memory technology. It is generally accepted that the underlying mechanism of most MITs is an interplay of electron correlation effects (Mott type) and disorder effects (Anderson type), and to disentangle the two effects is difficult. Recent progress on the crystalline Ge1Sb2Te4 (GST) compound provides compelling evidence for a disorder-driven MIT. In this work, we discuss the presence of strong disorder in GST, and elucidate its effects on electron localization and transport properties. We also show how the degree of disorder in GST can be reduced via thermal annealing, triggering a disorder-driven metal–insulator transition. The resistance switching by disorder tuning in crystalline GST may enable novel multilevel data storage devices. PMID:28773222

  17. Role of Disorder in Enhancing Lithium-Ion Battery Performance

    DEFF Research Database (Denmark)

    Yue, Yuanzheng; He, W.

    and type of disorder, material performances can be significantly enhanced. Disorder can be tuned by doping, calcination, redox reaction, composition tuning, and so on. Recently we have fabricated a cathode material for lithium ion battery by introducing heterostructure and disorder into the material...... material exhibits the extremely high reversible lithium ion capacity and extraordinary rate capability with high cycling stability at high discharge current. In this presentation we demonstrate that the disorder plays a decisive role in achieving those exceptional electrochemical performances. We describe...... how the disorder affects the migration of both lithium ions and electrons. It is found that both the modified glassy surface and the heterogeneous superlattice structure greatly contribute to the extremely high discharge/charge rates owing to the enhanced storage capacity of lithium ions and ultrafast...

  18. 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.

  19. The relationship between working memory and episodic memory disorders in transient global amnesia.

    Science.gov (United States)

    Quinette, Peggy; Guillery-Girard, Bérengère; Noël, Audrey; de la Sayette, Vincent; Viader, Fausto; Desgranges, Béatrice; Eustache, Francis

    2006-01-01

    In a previous study, we investigated the relationship between the disorders of both episodic memory and working memory in the acute phase of transient global amnesia (TGA). Since executive functions were spared, another dysfunction may be responsible for the binding and maintenance of multimodal informations and contribute to the encoding disorders observed in some patients [Quinette, P., Guillery, B., Desgranges, B., de la Sayette, V., Viader, F., & Eustache, F. (2003). Working memory and executive functions in transient global amnesia. Brain, 126, 1917-1934.]. The aim of this present study was to assess the functions of binding and maintenance of multimodal information during TGA and explore their involvement in episodic memory disorders. We therefore conducted a more thorough investigation of working memory in 16 new patients during the acute phase of TGA using two tasks designed to assess the binding process and both dimensions of the maintenance, namely the active storage and the memory load ability. We also investigated the nature of the episodic memory impairment in distinguishing between the performance of patients with preferential encoding deficits and those of patients with preferential storage disorders on the episodic memory task. This distinction was closely related to the severity of amnesia, i.e. an encoding disorder was observed rather in the early phase of TGA. The results showed that while the functions of binding and maintenance of multimodal information were intact in patients with storage disorders, they were impaired in the case of encoding deficits. These results are interpreted in the recent framework of episodic buffer proposed by Baddeley [Baddeley, A. D. (2000). The episodic buffer: A new component of working memory? Trends in Cognitive Sciences, 4, 417-423] that represents an interface between working memory and episodic memory.

  20. Gene expression profiling in equine polysaccharide storage myopathy revealed inflammation, glycogenesis inhibition, hypoxia and mitochondrial dysfunctions

    Directory of Open Access Journals (Sweden)

    Benech Philippe

    2009-08-01

    Full Text Available Abstract Background Several cases of myopathies have been observed in the horse Norman Cob breed. Muscle histology examinations revealed that some families suffer from a polysaccharide storage myopathy (PSSM. It is assumed that a gene expression signature related to PSSM should be observed at the transcriptional level because the glycogen storage disease could also be linked to other dysfunctions in gene regulation. Thus, the functional genomic approach could be conducted in order to provide new knowledge about the metabolic disorders related to PSSM. We propose exploring the PSSM muscle fiber metabolic disorders by measuring gene expression in relationship with the histological phenotype. Results Genotypying analysis of GYS1 mutation revealed 2 homozygous (AA and 5 heterozygous (GA PSSM horses. In the PSSM muscles, histological data revealed PAS positive amylase resistant abnormal polysaccharides, inflammation, necrosis, and lipomatosis and active regeneration of fibers. Ultrastructural evaluation revealed a decrease of mitochondrial number and structural disorders. Extensive accumulation of an abnormal polysaccharide displaced and partially replaced mitochondria and myofibrils. The severity of the disease was higher in the two homozygous PSSM horses. Gene expression analysis revealed 129 genes significantly modulated (p Conclusion The main disorders observed in PSSM muscles could be related to mitochondrial dysfunctions, glycogenesis inhibition and the chronic hypoxia of the PSSM muscles.

  1. CDP-diacylglycerol synthetase coordinates cell growth and fat storage through phosphatidylinositol metabolism and the insulin pathway.

    Directory of Open Access Journals (Sweden)

    Yuan Liu

    2014-03-01

    Full Text Available During development, animals usually undergo a rapid growth phase followed by a homeostatic stage when growth has ceased. The increase in cell size and number during the growth phase requires a large amount of lipids; while in the static state, excess lipids are usually stored in adipose tissues in preparation for nutrient-limited conditions. How cells coordinate growth and fat storage is not fully understood. Through a genetic screen we identified Drosophila melanogaster CDP-diacylglycerol synthetase (CDS/CdsA, which diverts phosphatidic acid from triacylglycerol synthesis to phosphatidylinositol (PI synthesis and coordinates cell growth and fat storage. Loss of CdsA function causes significant accumulation of neutral lipids in many tissues along with reduced cell/organ size. These phenotypes can be traced back to reduced PI levels and, subsequently, low insulin pathway activity. Overexpressing CdsA rescues the fat storage and cell growth phenotypes of insulin pathway mutants, suggesting that CdsA coordinates cell/tissue growth and lipid storage through the insulin pathway. We also revealed that a DAG-to-PE route mediated by the choline/ethanolamine phosphotransferase Bbc may contribute to the growth of fat cells in CdsA RNAi.

  2. 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

  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. 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.

  5. Proposal of a dry storage installation in Angra NPP for spent nuclear fuel

    International Nuclear Information System (INIS)

    Romanato, Luiz S.; Rzyski, Barbara M.

    2009-01-01

    When nuclear fuel is removed from a power reactor core after the depletion of efficiency in generating energy is called Spent Nuclear Fuel (SNF). After its withdrawal from the reactor core, SNF is temporarily stored in pools usually at the same site of the reactor. During this time, short-living radioactive elements and generated heat undergo decay until levels that allow removing the SNF from the pool and sending it for reprocessing or a temporary storage whether any of its final destinations has not yet been defined. It can be loaded in casks and disposed during years in a dry storage installations until be sent to a reprocessing plant or deep repositories. Before any decision, reprocessing or disposal, the SNF needs to be safely and efficiently isolated in one of many types of storages that exist around the world. Worldwide, the amount of SNF increases annually and in the next years this amount will be higher as a consequence of new Nuclear Power Plants (NPP) construction. In Brazil, that is about to construct the Angra 3 nuclear power reactor, a project about the final destination of the SNF is not yet ready. This paper presents a proposal for a dry storage installation in the Angra NPP site since it can be an initial solution for the Brazilian's SNF, until a final decision is taken. (author)

  6. TECHNOLOGY OF FRESH HERBS STORAGE USING HYDROGEL AND ANTIOXIDANT COMPOSITION

    Directory of Open Access Journals (Sweden)

    Olesia PRISS

    2017-12-01

    Full Text Available There is a stable consumer demand for fresh culinary herbs. Also, the greenery contains a large number of valuable phytonutrients. Despite high efficiency and increasing annual production of fresh herbs, the problem of preserving their quality in the post-harvest period remains unresolved. Because of the high specific surface area of evaporation, in the green crops droop quickly, they lose their marketable quality, and, as a result, the level of profitability of greenery production in general is being reduced. It is necessary to use new effective approaches to leafy greens storage in order to reduce product losses during transportation and storage. For example, agrarian hydrogel can be used for storage of greenery. Hydrogel is an acrylic potassium polymer that is non-toxic and has a high environmental standard. The hydrogel granules can absorb up to 250 times more moisture than their weight. We propose the following procedure as the method of greenery preservation: the greens are packed in bundles and put in sticks in polyethylene bags with a fastener, pre-filled with hydrogel solutions. The storage temperature is maintained optimally for each species of fresh herbs, the relative humidity is 95 ± 3%. Usage of the proposed method allows obtaining environment-friendly products, preserving their high biological value and increasing the shelf life. The accumulation of peroxide products, which cause physiological disorders, is inhibited as the result of such storage. The use of hydrogel reduces the natural loss of mass by 10% as compared with the control. Duration of greenery storage increases by 30 days.

  7. A "coca-cola" shape: cultural change, body image, and eating disorders in San Andrés, Belize.

    Science.gov (United States)

    Anderson-Fye, Eileen P

    2004-12-01

    Eating disorders have been associated with developing nations undergoing rapid social transition, including participation in a global market economy and heavy media exposure. San Andrés, Belize, a community with many risk factors associated with the cross-cultural development of eating disorders, has shown remarkable resistance to previously documented patterns, despite a local focus on female beauty. Drawing on longitudinal person-centered ethnography with adolescent girls, this article examines why this community appears exceptional in light of the literature. First, community beauty and body image ideals and practices are explicated. Then, a protective ethnopsychology is proposed as a key mediating factor of the rapid socio-cultural change among young women. Finally, possible nascent cases of eating disordered behavior are discussed in light of their unique phenomenology: that is, having to do more with economic opportunity in the tourism industry and less with personal distress or desire for thinness. Close, meaning-centered examination of eating and body image practices may aid understanding and prevention of eating disorders among adolescents undergoing rapid social change in situations of globalization and immigration.

  8. 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.

  9. 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.

  10. 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

  11. Glassy transition in a disordered model for the RNA secondary structure

    International Nuclear Information System (INIS)

    Pagnani, A.; Parisi, G.; Ricci-Tersenghi, F.

    2000-04-01

    We numerically study a disordered model for the RNA secondary structure and we find that it undergoes a phase transition, with a breaking of the replica symmetry in the low temperature region (like in spin glasses). Our results are based on the exact evaluation of the partition function. (author)

  12. 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

  13. Optimizing and Quantifying CO2 Storage Resource in Saline Formations and Hydrocarbon Reservoirs

    Energy Technology Data Exchange (ETDEWEB)

    Bosshart, Nicholas W. [Univ. of North Dakota, Grand Folks, ND (United States). Energy & Environmental Research Center; Ayash, Scott C. [Univ. of North Dakota, Grand Folks, ND (United States). Energy & Environmental Research Center; Azzolina, Nicholas A. [Univ. of North Dakota, Grand Folks, ND (United States). Energy & Environmental Research Center; Peck, Wesley D. [Univ. of North Dakota, Grand Folks, ND (United States). Energy & Environmental Research Center; Gorecki, Charles D. [Univ. of North Dakota, Grand Folks, ND (United States). Energy & Environmental Research Center; Ge, Jun [Univ. of North Dakota, Grand Folks, ND (United States). Energy & Environmental Research Center; Jiang, Tao [Univ. of North Dakota, Grand Folks, ND (United States). Energy & Environmental Research Center; Burton-Kelly, Matthew E. [Univ. of North Dakota, Grand Folks, ND (United States). Energy & Environmental Research Center; Anderson, Parker W. [Univ. of North Dakota, Grand Folks, ND (United States). Energy & Environmental Research Center; Dotzenrod, Neil W. [Univ. of North Dakota, Grand Folks, ND (United States). Energy & Environmental Research Center; Gorz, Andrew J. [Univ. of North Dakota, Grand Folks, ND (United States). Energy & Environmental Research Center

    2017-06-30

    In an effort to reduce carbon dioxide (CO2) emissions from large stationary sources, carbon capture and storage (CCS) is being investigated as one approach. This work assesses CO2 storage resource estimation methods for deep saline formations (DSFs) and hydrocarbon reservoirs undergoing CO2 enhanced oil recovery (EOR). Project activities were conducted using geologic modeling and simulation to investigate CO2 storage efficiency. CO2 storage rates and efficiencies in DSFs classified by interpreted depositional environment were evaluated at the regional scale over a 100-year time frame. A focus was placed on developing results applicable to future widespread commercial-scale CO2 storage operations in which an array of injection wells may be used to optimize storage in saline formations. The results of this work suggest future investigations of prospective storage resource in closed or semiclosed formations need not have a detailed understanding of the depositional environment of the reservoir to generate meaningful estimates. However, the results of this work also illustrate the relative importance of depositional environment, formation depth, structural geometry, and boundary conditions on the rate of CO2 storage in these types of systems. CO2 EOR occupies an important place in the realm of geologic storage of CO2, as it is likely to be the primary means of geologic CO2 storage during the early stages of commercial implementation, given the lack of a national policy and the viability of the current business case. This work estimates CO2 storage efficiency factors using a unique industry database of CO2 EOR sites and 18 different reservoir simulation models capturing fluvial clastic and shallow shelf carbonate depositional environments for reservoir depths of 1219 and 2438 meters (4000 and 8000 feet) and 7.6-, 20-, and 64-meter (25-, 66

  14. Technical study of a thermally dense long term interim storage facility

    International Nuclear Information System (INIS)

    Le Duigou, A.; Badie, M.; Duret, B.; Bricard, A.

    2001-01-01

    The COFRE concept is aimed at the surface and thermal densification of the interim storage facility for irradiated fuels. The facility provides the biological shielding. A conditioning cell is used to load and retrieve the fuel assemblies. The facility container is the second containment barrier. The high power levels are managed by an auxiliary cooling system whose original feature is the passive use of a water evaporation-condensation cycle in a sealed circuit. The removable evaporator abuts the container. The air cooled condenser is placed outside the facility. Contact resistance and heat pipe mode were successfully modelled and are undergoing experimental validation on the THERESE and REBECA loops. (author)

  15. 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.

  16. Features of the differential diagnosis of persons with gender identity disorders

    Directory of Open Access Journals (Sweden)

    Z.D. Novikova

    2013-10-01

    Full Text Available We presented a study of the features of gender identity in people undergoing gender, psychological and psychiatric examination to address the issue of gender reassignment. We analyze the specifics of gender identity, levels of masculinity and femininity, the similarities and differentiation within four nosological groups, which include persons with gender identity disorders (GID with transsexualism, personality disorders, diseases of the schizophrenia spectrum, and with organic mental disorders. We address the question of the differential diagnosis in the process of psychological screening of people with transsexualism and other types of GID. The analytical description of the four algorithms and their comparison are psychologically specific, qualitative research, almost impossible using statistical method of data processing. The data presented may be useful to specialists involved in the study of persons with gender identity disorders

  17. 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.

  18. Colonoscopic Diagnostic Findings in Patients Undergoing Colonoscopy In Qom Hazrat-e-Masoome Hospital During 2007-2008

    Directory of Open Access Journals (Sweden)

    M.R Ghadir

    2012-05-01

    Full Text Available

    Background and Objectives: In recent years there have been noticeable changes in diagnosis and treatment of colon disorders by colonoscopy and direct vision. Along with its international development, this useful equipment is being used in Iran to treat various disorders. It should be mentioned that there are no exact statistics of these disorders to date. This study was done with aim of evaluating the diagnostic findings in patients undergoing colonoscopy in Qom during 2007-2008.

     

    Methods: This descriptive-cross sectional study was done on 500 patients having referred to colonoscopy ward of Hazrate-e-Masoome Hospital in Qom. After colonoscopy, patient data were entered into a special questionnaire and then pathologic findings were added to it. The data were taken for statistical analysis.

     

    Results: Out of 500 patients undergoing colonoscopy 279 were male (55.8% and 221 female (44.2%. In all groups and both sexes the most common reason for carrying out colonoscopy was abdominal pain (46.6% rectorrhagia (41%. As regards diagnosis, a total of 199 cases (39.8% of all 500 colonoscopies had normal colonoscopy,124 cases (24.8% had hemorrhoid, 64 cases (12.8% had polyp, 55 cases (11% had inflammatory bowel diseases (IBD, 30 cases (6% had tumor, 17 cases(3.2% had diverticulosis and 12 cases (2.4% had solitary rectal ulcer. There was a significant relationship between abdominal pain and tumor, polyp and diverticulosis. (p<0.001 There was also a significant relationship between age and the aforementioned disorders. (p<0.001

     

    Conclusion: Based on the findings of this study, the prevalence of cancer and IBD is higher in men

  19. Configurational and constitutional information storage: multiple dynamics in systems based on pyridyl and acyl hydrazones.

    Science.gov (United States)

    Chaur, Manuel N; Collado, Daniel; Lehn, Jean-Marie

    2011-01-03

    The C=N group of hydrazones can undergo E/Z isomerization both photochemically and thermally, allowing the generation of a closed process that can be tuned by either of these two physical stimuli. On the other hand, hydrazine-exchange reactions enable a constitutional change in a given hydrazone. The two classes of processes: 1) configurational (physically stimulated) and 2) constitutional (chemically stimulated) give access to short-term and long-term information storage, respectively. Such transformations are reported herein for two hydrazones (bis-pyridyl hydrazone and 2-pyridinecarboxaldehyde phenylhydrazone) that undergo a closed, chemically or physically driven process, and, in addition, can be locked or unlocked at will by metal-ion coordination or removal. These features also extend to acyl hydrazones derived from 2-pyridinecarboxaldehyde. Similarly to the terpydine-like hydrazones, such acyl hydrazones can undergo both constitutional and configurational changes, as well as metal-ion coordination. All these types of hydrazones represent dynamic systems capable of acting as multiple state molecular devices, in which the presence of coordination sites furthermore allows the metal ion-controlled locking and unlocking of the interconversion of the different states. Copyright © 2011 WILEY-VCH Verlag GmbH & Co. KGaA, Weinheim.

  20. Psychological assessment tool for patients diagnosed with absolute uterine factor infertility and planning to undergo uterine transplantation.

    Science.gov (United States)

    Saso, S; Bracewell-Milnes, T; Ismail, L; Hamed, A H; Thum, M-Y; Ghaem-Maghami, S; Del Priore, G; Smith, J R

    2014-08-01

    Uterine transplantation (UTn) has been proposed as a treatment option for women diagnosed with absolute uterine factor infertility (AUFI) and who are willing to bear their own child. AUFI renders a woman 'unconditionally infertile'. For AUFI women in general, UTn may offer a way to re-discover their own femininity through the restoration of fertility. Thus, when faced with a patient who may undergo UTn, the 'holistic approach' takes on an extra meaning. This is because the psychological element is two-sided for these patients. On one side lies the psychology of infertility, and on the other and equally important, is the substantially higher prevalence of psychiatric disorders in transplant candidates and recipients than in the general population. However, the psychology of a potential recipient of a uterine graft in order to bring about fertility has not been adequately explored or reviewed scientifically. We have presented here an outline of the areas which should be included in a psychological assessment for patients wishing to undergo UTn.

  1. 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,

  2. Parent Expectations Mediate Outcomes for Young Adults with Autism Spectrum Disorder

    Science.gov (United States)

    Kirby, Anne V.

    2016-01-01

    Understanding the complex relationships among factors that may predict the outcomes of young adults with autism spectrum disorder (ASD) is of utmost importance given the increasing population undergoing and anticipating the transition to adulthood. With a sample of youth with ASD (n = 1170) from the National Longitudinal Transition Study-2,…

  3. "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

  4. 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

  5. Inherited disorders of brain neurotransmitters: pathogenesis and diagnostic approach.

    Science.gov (United States)

    Szymańska, Krystyna; Kuśmierska, Katarzyna; Demkow, Urszula

    2015-01-01

    Neurotransmitters (NTs) play a central role in the efficient communication between neurons necessary for normal functioning of the nervous system. NTs can be divided into two groups: small molecule NTs and larger neuropeptide NTs. Inherited disorders of NTs result from a primary disturbance of NTs metabolism or transport. This group of disorders requires sophisticated diagnostic procedures. In this review we discuss disturbances in the metabolism of tetrahydrobiopterin, biogenic amines, γ-aminobutyric acid, foliate, pyridoxine-dependent enzymes, and also the glycine-dependent encephalopathy. We point to pathologic alterations of proteins involved in synaptic neurotransmission that may cause neurological and psychiatric symptoms. We postulate that synaptic receptors and transporter proteins for neurotransmitters should be investigated in unresolved cases. Patients with inherited neurotransmitters disorders present various clinical presentations such as mental retardation, refractory seizures, pyramidal and extrapyramidal syndromes, impaired locomotor patterns, and progressive encephalopathy. Every patient with suspected inherited neurotransmitter disorder should undergo a structured interview and a careful examination including neurological, biochemical, and imaging.

  6. Postharvest storage quality of grapefruit placed in polyethylene bags with or without micro-perforation

    Science.gov (United States)

    Washed ‘Marsh’ white grapefruit were placed in polyethylene (PE) bags (1 mil) with or without micro-perforation holes (representing 0.002% of the bag surface) and evaluated for juice quality, firmness, and the development of decay and disorders during storage for 30, 60, or 90 days at 50oF. Each tre...

  7. Intraoperative Diagnosis of Anderson-Fabry Disease in Patients With Obstructive Hypertrophic Cardiomyopathy Undergoing Surgical Myectomy.

    Science.gov (United States)

    Cecchi, Franco; Iascone, Maria; Maurizi, Niccolò; Pezzoli, Laura; Binaco, Irene; Biagini, Elena; Fibbi, Maria Laura; Olivotto, Iacopo; Pieruzzi, Federico; Fruntelata, Ana; Dorobantu, Lucian; Rapezzi, Claudio; Ferrazzi, Paolo

    2017-10-01

    Diagnostic screening for Anderson-Fabry cardiomyopathy (AFC) is performed in the presence of specific clinical red flags in patients with hypertrophic cardiomyopathy (HCM) older than 25 years. However, left ventricular outflow tract obstruction (LVOTO) has been traditionally considered an exclusion criteria for AFC. To examine a series of patients diagnosed with HCM and severe basal LVOTO undergoing myectomy in whom the diagnosis of AFC was suspected by the cardiac surgeon intraoperatively and confirmed by histological and genetic examinations. This retrospective analysis of patients undergoing surgical septal reduction strategies was conducted in 3 European tertiary referral centers for HCM from July 2013 to December 2016. Patients with a clinical diagnosis of obstructive HCM referred for surgical management of LVOTO were observed for at least 18 months after the procedure (mean [SD] follow-up, 33 [14] months). Etiology of patients with HCM who underwent surgical myectomy. From 2013, 235 consecutive patients with a clinical diagnosis of HCM underwent septal myectomy. The cardiac surgeon suspected a storage disease in 3 patients (1.3%) while inspecting their heart samples extracted from myectomy. The mean (SD) age at diagnosis for these 3 patients was 42 (4) years; all were male. None of the 3 patients presented with extracardiac features suggestive of AFC. All patients showed asymmetrical left ventricular hypertrophy, with maximal left ventricular thickness in the basal septum (19-31 mm), severe basal LVOTO (70-120 mm Hg), and left atrial dilatation (44-57 mm). Only 1 patient presented with late gadolinium enhancement on cardiovascular magnetic resonance at the right ventricle insertion site. The mean (SD) age at surgical procedure was 63 (5) years. On tactile sensation, the surgeon felt a spongy consistency of the surgical samples, different from the usual stony-elastic consistency typical of classic HCM, and this prompted histological examinations. Histology

  8. Mental health treatment after major surgery among Vietnam-era Veterans with posttraumatic stress disorder.

    Science.gov (United States)

    Tsan, Jack Y; Stock, Eileen M; Greenawalt, David S; Zeber, John E; Copeland, Laurel A

    2016-07-01

    The purpose of this study was to examine mental health treatment use among Vietnam Veterans with posttraumatic stress disorder and determine whether undergoing major surgery interrupted mental health treatment or increased the risk of psychiatric hospitalization. Using retrospective data from Veterans Health Administration's electronic medical record system, a total of 3320 Vietnam-era surgery patients with preoperative posttraumatic stress disorder were identified and matched 1:4 with non-surgical patients with posttraumatic stress disorder. The receipt of surgery was associated with a decline in overall mental health treatment and posttraumatic stress disorder-specific treatment 1 month following surgery but not during any subsequent month thereafter. Additionally, surgery was not associated with psychiatric admission. © The Author(s) 2014.

  9. Retrievable surface storage: interim storage of solidified high-level waste

    International Nuclear Information System (INIS)

    LaRiviere, J.R.; Nelson, D.C.

    1976-01-01

    Studies have been conducted on retrievable-surface-storage concepts for the interim storage of solidified high-level wastes. These studies have been reviewed by the Panel on Engineered Storage, convened by the Committee on Radioactive Waste Management of the National Research Council-National Academy of Sciences. The Panel has concluded that ''retrievable surface storage is an acceptable interim stage in a comprehensive system for managing high-level radioactive wastes.'' The scaled storage cask concept, which was recommended by the Panel on Engineered Storage, consists of placing a canister of waste inside a carbon-steel cask, which in turn is placed inside a thick concrete cylinder. The waste is cooled by natural convection air flow through an annulus between the cask and the inner wall of the concrete cylinder. The complete assembly is placed above ground in an outdoor storage area

  10. 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

  11. Thought-action fusion across anxiety disorder diagnoses: specificity and treatment effects.

    Science.gov (United States)

    Thompson-Hollands, Johanna; Farchione, Todd J; Barlow, David H

    2013-05-01

    Thought-action fusion (TAF) is a cognitive error that has been frequently investigated within the context of obsessive-compulsive disorder (OCD). However, evidence suggests that this error may also be present in disorders other than OCD, indicating that TAF is related to higher order factors rather than a specific diagnosis. We explored TAF in a sample of patients with mixed diagnoses undergoing treatment with a transdiagnostic CBT protocol. Elevated TAF levels at baseline were not specific to patients with OCD. However, the presence of any generalized anxiety disorder (GAD) diagnosis was unexpectedly the strongest predictor of likelihood TAF. Likelihood TAF, a particular component of TAF, was reduced after transdiagnostic treatment, and this reduction was not affected by the presence of a GAD diagnosis. Results indicate that TAF is responsive to treatment and should be assessed and, perhaps, treated in disorders beyond OCD.

  12. 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)

  13. Level-statistics in Disordered Systems: A single parametric scaling and Connection to Brownian Ensembles

    OpenAIRE

    Shukla, Pragya

    2004-01-01

    We find that the statistics of levels undergoing metal-insulator transition in systems with multi-parametric Gaussian disorders and non-interacting electrons behaves in a way similar to that of the single parametric Brownian ensembles \\cite{dy}. The latter appear during a Poisson $\\to$ Wigner-Dyson transition, driven by a random perturbation. The analogy provides the analytical evidence for the single parameter scaling of the level-correlations in disordered systems as well as a tool to obtai...

  14. Differential diagnosis of Mendelian and mitochondrial disorders in patients with suspected multiple sclerosis

    Science.gov (United States)

    Katz Sand, Ilana B.; Honce, Justin M.; Lublin, Fred D.

    2015-01-01

    Several single gene disorders share clinical and radiologic characteristics with multiple sclerosis and have the potential to be overlooked in the differential diagnostic evaluation of both adult and paediatric patients with multiple sclerosis. This group includes lysosomal storage disorders, various mitochondrial diseases, other neurometabolic disorders, and several other miscellaneous disorders. Recognition of a single-gene disorder as causal for a patient’s ‘multiple sclerosis-like’ phenotype is critically important for accurate direction of patient management, and evokes broader genetic counselling implications for affected families. Here we review single gene disorders that have the potential to mimic multiple sclerosis, provide an overview of clinical and investigational characteristics of each disorder, and present guidelines for when clinicians should suspect an underlying heritable disorder that requires diagnostic confirmation in a patient with a definite or probable diagnosis of multiple sclerosis. PMID:25636970

  15. 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.

  16. Method of storing the fuel storage pot in a fuel storage tank for away-from-reactor-storage

    International Nuclear Information System (INIS)

    Ishiguro, Jun-ichi.

    1980-01-01

    Purpose: To prevent the contact of sodium in the away-from-reactor-storage fuel storage tank with sodium in a fuel storage pool having radioactivity ana always retain clean state therein. Method: Sodium is filled in a container body of the away-from-reactor-storage fuel storage tank, and a conduit, a cycling pump, and cooling means are disposed to form a sodium coolant cycling loop. The fuel storage pool is so stored in the container body that the heat of the pool is projected from the liquid surface of the sodium in the container. Therefore, the sodium in the container is isolated from the sodium in the pool containing strong radioactivity to prevent contact of the former sodium from the latter sodium. (Sekiya, K.)

  17. 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.

  18. Altered memory and affective instability in prisoners assessed for dangerous and severe personality disorder.

    Science.gov (United States)

    Kirkpatrick, Tim; Joyce, Eileen; Milton, John; Duggan, Conor; Tyrer, Peter; Rogers, Robert D

    2007-05-01

    Previous studies of borderline personality disorder report neuropsychological impairments in several domains, including memory. No studies have compared memory functioning in high-risk prisoners with borderline personality disorder with similar prisoners with other personality disorders. To explore mnemonic impairments in prisoners undergoing personality assessment as part of the dangerous and severe personality disorder initiative or detained in a medium secure facility. We investigated memory function in 18 prisoners with borderline personality disorder and 18 prisoners with other personality disorders. Prisoners with borderline personality disorder exhibited a pattern of multi-modal impairments in the immediate and delayed recall of verbal and visual information, with some association with affective instability. These deficits were not associated with the severity of personality disturbance. These data suggest that memory deficits have some specificity in relation to the constituent traits of borderline personality disorder and indicate that neuropsychological assessment may be a source of useful adjunctive information for distinguishing between the cognitive and psychological difficulties of individual prisoners.

  19. 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)

  20. Intrathecal enzyme replacement therapy reduces lysosomal storage in the brain and meninges of the canine model of MPS I.

    Science.gov (United States)

    Kakkis, E; McEntee, M; Vogler, C; Le, S; Levy, B; Belichenko, P; Mobley, W; Dickson, P; Hanson, S; Passage, M

    2004-01-01

    Enzyme replacement therapy (ERT) has been developed for several lysosomal storage disorders, including mucopolysaccharidosis I (MPS I), and is effective at reducing lysosomal storage in many tissues and in ameliorating clinical disease. However, intravenous ERT does not adequately treat storage disease in the central nervous system (CNS), presumably due to effects of the blood-brain barrier on enzyme distribution. To circumvent this barrier, we studied whether intrathecal (IT) recombinant human alpha-L-iduronidase (rhIDU) could penetrate and treat the brain and meninges. An initial dose-response study showed that doses of 0.46-4.14 mg of IT rhIDU successfully penetrated the brain of normal dogs and reached tissue levels 5.6 to 18.9-fold normal overall and 2.7 to 5.9-fold normal in deep brain sections lacking CSF contact. To assess the efficacy and safety in treating lysosomal storage disease, four weekly doses of approximately 1 mg of IT rhIDU were administered to MPS I-affected dogs resulting in a mean 23- and 300-fold normal levels of iduronidase in total brain and meninges, respectively. Quantitative glycosaminoglycan (GAG) analysis showed that the IT treatment reduced mean total brain GAG to normal levels and achieved a 57% reduction in meningeal GAG levels accompanied by histologic improvement in lysosomal storage in all cell types. The dogs did develop a dose-dependent immune response against the recombinant human protein and a meningeal lymphocytic/plasmacytic infiltrate. The IT route of ERT administration may be an effective way to treat the CNS disease in MPS I and could be applicable to other lysosomal storage disorders.

  1. Sleep disorders in pediatric chronic kidney disease patients.

    Science.gov (United States)

    Stabouli, Stella; Papadimitriou, Eleni; Printza, Nikoleta; Dotis, John; Papachristou, Fotios

    2016-08-01

    The prevalence of sleep disorders during childhood has been estimated to range from 25 to 43 %. The aim of this review is to determine the prevalence of sleep disorders and possible associations with chronic kidney disease (CKD)-related factors and health-related quality of life (HRQOL) in children with CKD. An electronic systematic literature search for sleep disorders in children with CKD in Pubmed, Embase and the Cochrane Library Databases identified seven relevant articles for review, all of which reported an increased prevalence of sleep disorders in children with CKD. Five studies included children with CKD undergoing dialysis, and two studies included only non-dialysis patients. In all studies the presence of sleep disturbances was assessed by questionnaires; only one study compared the results of a validated questionnaire with laboratory-based polysomnography. The prevalence of any sleep disorder ranged from 77 to 85 % in dialysis patients, to 32-50 % in transplanted patients and 40-50 % in non-dialysis patients. The most commonly studied disorder was restless legs syndrome, which presented at a prevalence of 10-35 %. Three studies showed significant associations between presence of sleep disorders and HRQOL. We found consistent evidence of an increased prevalence of sleep disturbances in children with CKD, and these seemed to play a critical role in HRQOL.

  2. Glycogen storage disease type I: clinical and laboratory profile

    Directory of Open Access Journals (Sweden)

    Berenice L. Santos

    2014-12-01

    Full Text Available OBJECTIVES: To characterize the clinical, laboratory, and anthropometric profile of a sample of Brazilian patients with glycogen storage disease type I managed at an outpatient referral clinic for inborn errors of metabolism. METHODS: This was a cross-sectional outpatient study based on a convenience sampling strategy. Data on diagnosis, management, anthropometric parameters, and follow-up were assessed. RESULTS: Twenty-one patients were included (median age 10 years, range 1-25 years, all using uncooked cornstarch therapy. Median age at diagnosis was 7 months (range, 1-132 months, and 19 patients underwent liver biopsy for diagnostic confirmation. Overweight, short stature, hepatomegaly, and liver nodules were present in 16 of 21, four of 21, nine of 14, and three of 14 patients, respectively. A correlation was found between height-for-age and BMI-for-age Z-scores (r = 0.561; p = 0.008. CONCLUSIONS: Diagnosis of glycogen storage disease type I is delayed in Brazil. Most patients undergo liver biopsy for diagnostic confirmation, even though the combination of a characteristic clinical presentation and molecular methods can provide a definitive diagnosis in a less invasive manner. Obesity is a side effect of cornstarch therapy, and appears to be associated with growth in these patients.

  3. Revertant mosaicism in a human skin fragility disorder results from slipped mispairing and mitotic recombination

    NARCIS (Netherlands)

    Kiritsi, Dimitra; He, Yinghong; Pasmooij, Anna M. G.; Onder, Meltem; Happle, Rudolf; Jonkman, Marcel F.; Bruckner-Tuderman, Leena; Has, Cristina

    Spontaneous gene repair, also called revertant mosaicism, has been documented in several genetic disorders involving organs that undergo self-regeneration, including the skin. Genetic reversion may occur through different mechanisms, and in a single individual, the mutation can be repaired in

  4. Cholesteryl ester storage disease: a rare and possibly treatable cause of premature vascular disease and cirrhosis.

    Science.gov (United States)

    Reynolds, Tim

    2013-11-01

    Cholesteryl ester storage disease (CESD) is an autosomal recessive lysosomal storage disorder caused by a variety of mutations of the LIPA gene. These cause reduced activity of lysosomal acid lipase, which results in accumulation of cholesteryl esters in lysosomes. If enzyme activity is very low/absent, presentation is in infancy with failure to thrive, malabsorption, hepatosplenomegaly and rapid early death (Wolman disease). With higher but still low enzyme activity, presentation is later in life with hepatic fibrosis, dyslipidaemia and early atherosclerosis.Identification of this rare disorder is difficult as it is essential to assay leucocyte acid phosphatase activity. An assay using specific inhibitors has now been developed that facilitates measurement in dried blood spots. Treatment of CESD has until now been limited to management of the dyslipidaemia, but this does not influence the liver effects. A new enzyme replacement therapy (Sebelipase) has now been developed that could change treatment options for the future.

  5. 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.

  6. 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

  7. Psychological, behavioral, and weight-related aspects of patients undergoing reoperative bariatric surgery after gastric band: comparison with primary surgery patients.

    Science.gov (United States)

    Conceição, Eva; Pinto-Bastos, Ana; de Lourdes, Marta; Brandão, Isabel; Teixeira, Cristina; Machado, Paulo P P

    2018-02-15

    Patients experiencing insufficient weight loss or weight regain in their first bariatric surgery may represent a high-risk group with greater problematic eating and general psychopathology levels, which may compromise the success of a reoperative procedure. This study seeks to describe and compare disordered eating-related psychological and behavioral features of primary and reoperative surgery candidates after gastric band. Hospital center and university in Portugal. The baseline (preoperative) data from a longitudinal observational study are presented. Patients were interviewed by trained psychologists to identify binge-eating episodes and grazing and responded to a set of self-report measures: disordered eating, grazing, negative urgency, depression, anxiety, and stress. Two hundred twenty-five patients were undergoing primary surgery, and 166 were undergoing reoperative (REOP group) surgery. The groups did not differ in body mass index preoperatively, but the REOP group had greater weight suppression (t 387 = -5.35, P = .001), higher highest (t 387 = -3.40, P = .001) and lower lowest body mass index (t 381 = 2.22, P = .03). The main reasons for reoperative surgery were weight regain/poor weight loss (42.8%) or medical complications (32.5%). REOP patients with objective binge eating reported a higher frequency of these episodes (t 47 = 2.15, P = .04). No significant difference was found for the self-report measures assessed (only shape concern was higher for REOP group, F 1,216 = 8.30, Psurgeries, the differences in binge eating and weight-related variables may associate with postoperative difficulties. The link between binge eating, weight suppression, and weight gain found in other samples suggests that patients undergoing reoperative surgery may be at increased risk for poor weight outcomes. Copyright © 2018 American Society for Bariatric Surgery. Published by Elsevier Inc. All rights reserved.

  8. Two-dimensional mapping of underdosed areas using radiochromic film for patients undergoing total skin electron beam radiotherapy

    International Nuclear Information System (INIS)

    Gamble, Lisa M.; Farrell, Thomas J.; Jones, Glenn W.; Hayward, Joseph E.

    2005-01-01

    Purpose: To demonstrate the viability of radiochromic film as an in vivo, two-dimensional dosimeter for the measurement of underdosed areas in patients undergoing total skin electron beam (TSEB) radiotherapy. The results were compared with thermoluminescent dosimeter measurements. Methods and Materials: Dosimetry results are reported for an inframammary fold of 2 patients treated using a modified version of the Stanford six-position (i.e., six-field and dual-beam) TSEB technique. The results are presented as contour plots of film optical density and percentage of dose. A linear dose profile measured from film was compared with the thermoluminescent dosimeter measurements. Results: The results showed that the percentage doses as measured by film are in good agreement with those measured by the thermoluminescent dosimeters. The isodose contour plots provided by film can be used as a two-dimensional dose map for a patient when determining the size of the supplemental patch fields. Conclusion: Radiochromic film is a viable dosimetry tool that the radiation oncologist can use to understand the surface dose heterogeneity better across complex concave regions of skin to help establish more appropriate margins to patch underdosed areas. Film could be used for patients undergoing TSEB for disorders such as mycosis fungoides or undergoing TSEB or regional skin electron beam for widespread skin metastases from breast cancer and other malignancies

  9. Prevalence and characteristics of avoidant/restrictive food intake disorder in a cohort of young patients in day treatment for eating disorders.

    Science.gov (United States)

    Nicely, Terri A; Lane-Loney, Susan; Masciulli, Emily; Hollenbeak, Christopher S; Ornstein, Rollyn M

    2014-01-01

    Avoidant/Restrictive Food Intake Disorder (ARFID) is a "new" diagnosis in the recently published DSM-5, but there is very little literature on patients with ARFID. Our objectives were to determine the prevalence of ARFID in children and adolescents undergoing day treatment for an eating disorder, and to compare ARFID patients to other eating disorder patients in the same cohort. A retrospective chart review of 7-17 year olds admitted to a day program for younger patients with eating disorders between 2008 and 2012 was performed. Patients with ARFID were compared to those with anorexia nervosa, bulimia nervosa, and other specified feeding or eating disorder/unspecified feeding or eating disorder with respect to demographics, anthropometrics, clinical symptoms, and psychometric testing, using Chi-square, ANOVA, and post-hoc analysis. 39/173 (22.5%) patients met ARFID criteria. The ARFID group was younger than the non-ARFID group and had a greater proportion of males. Similar degrees of weight loss and malnutrition were found between groups. Patients with ARFID reported greater fears of vomiting and/or choking and food texture issues than those with other eating disorders, as well as greater dependency on nutritional supplements at intake. Children's Eating Attitudes Test scores were lower for children with than without ARFID. A higher comorbidity of anxiety disorders, pervasive developmental disorder, and learning disorders, and a lower comorbidity of depression, were found in those with ARFID. This study demonstrates that there are significant demographic and clinical characteristics that differentiate children with ARFID from those with other eating disorders in a day treatment program, and helps substantiate the recognition of ARFID as a distinct eating disorder diagnosis in the DSM-5.

  10. 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

  11. 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

  12. Disorder-induced localization in crystalline phase-change materials.

    Science.gov (United States)

    Siegrist, T; Jost, P; Volker, H; Woda, M; Merkelbach, P; Schlockermann, C; Wuttig, M

    2011-03-01

    Localization of charge carriers in crystalline solids has been the subject of numerous investigations over more than half a century. Materials that show a metal-insulator transition without a structural change are therefore of interest. Mechanisms leading to metal-insulator transition include electron correlation (Mott transition) or disorder (Anderson localization), but a clear distinction is difficult. Here we report on a metal-insulator transition on increasing annealing temperature for a group of crystalline phase-change materials, where the metal-insulator transition is due to strong disorder usually associated only with amorphous solids. With pronounced disorder but weak electron correlation, these phase-change materials form an unparalleled quantum state of matter. Their universal electronic behaviour seems to be at the origin of the remarkable reproducibility of the resistance switching that is crucial to their applications in non-volatile-memory devices. Controlling the degree of disorder in crystalline phase-change materials might enable multilevel resistance states in upcoming storage devices.

  13. Predicting personality disorder functioning styles by the Chinese Adjective Descriptors of Personality: a preliminary trial in healthy people and personality disorder patients.

    Science.gov (United States)

    Fan, Hongying; Zhu, Qisha; Ma, Guorong; Shen, Chanchan; Zhang, Bingren; Wang, Wei

    2016-08-30

    Cultural and personality factors might contribute to the clinical differences of psychiatric patients all over the world including China. One cultural oriented Chinese Adjective Descriptors of Personality (CADP) designed to measure normal personality traits, might be specifically associated with different personality disorder functioning styles. We therefore have invited 201 healthy volunteers and 67 personality disorder patients to undergo CADP, the Parker Personality Measure (PERM), and the Plutchik-van Praag Depression Inventory (PVP) tests. Patients scored significantly higher on PVP scale and all 11 PERM personality disorder functioning styles, as well as CADP Emotional and Unsocial traits. The PVP was significantly correlated with some CADP traits and PERM styles in both groups. In healthy volunteers, only one CADP trait, Unsocial, prominently predicted 11 PERM styles. By contrast in patients, CADP Intelligent predicted the PERM Narcissistic and Passive-Aggressive styles; CADP Emotional the PERM Paranoid, Borderline, and Histrionic styles; CADP Conscientious the PERM Obsessive-Compulsive style; CADP Unsocial the PERM Schizotypal, Antisocial, Narcissistic, Avoidant, Dependent, and Passive-Aggressive styles; CADP Agreeable the PERM Antisocial style. As a preliminary study, our results demonstrated that, in personality disorder patients, all five CADP traits were specifically associated with almost all 11 personality disorder functioning styles, indicating that CADP might be used as an aid to diagnose personality disorders in China.

  14. The DAPP-SF as a screener for personality disorder in a forensic setting.

    Science.gov (United States)

    Spaans, Marleen; Rinne, Thomas; de Beurs, Edwin; Spinhoven, Philip

    2015-01-01

    Studies on the Dimensional Assessment of Personality Pathology-Short Form (DAPP-SF) have shown its ability to identify treatment-seeking patients with personality disorders. This study focuses on its screening potential for personality disorder in 89 criminal suspects (77 men, 12 women; M age = 37.0 years) undergoing residential pretrial psychological assessments in a high-security setting. It was expected that Structured Interview for DSM-IV Personality (SIDP-IV) criteria met for personality disorder(s) would be associated with higher DAPP-SF scores. A floor effect was found in DAPP-SF scores: The forensic population reported less personality pathology than the general population. Only moderate associations between DAPP-SF and SIDP-IV outcome were found. Receiver operating characteristic analysis showed that some DAPP-SF subscales did not exceed chance level in their ability to screen for personality disorders. It is concluded that the DAPP-SF has limited usefulness as a screener for personality disorders in a forensic pretrial setting. Alternative forensic screening instruments are presented.

  15. High prevalence of body dysmorphic disorder symptoms in patients seeking rhinoplasty.

    Science.gov (United States)

    Picavet, Valerie A; Prokopakis, Emmanuel P; Gabriëls, Lutgardis; Jorissen, Mark; Hellings, Peter W

    2011-08-01

    Nasal aesthetic deformities may be associated with significant body image dissatisfaction. The only diagnostic category in the current list of psychiatric disorders that directly addresses these concerns is body dysmorphic disorder. This large-scale study determined the prevalence of body dysmorphic disorder and its symptoms in patients seeking rhinoplasty and evaluated the clinical profile of these patients. Two hundred twenty-six patients were given questionnaires including demographic characteristics, visual analogue scales for nasal shape, the Yale-Brown Obsessive Compulsive Scale modified for body dysmorphic disorder to assess severity of symptoms, a generic quality-of-life questionnaire, and the Derriford Appearance Scale 59, to assess appearance-related disruption of everyday living. Independent observers scored the nasal shape. Thirty-three percent of patients showed at least moderate symptoms of body dysmorphic disorder. Aesthetic goals (p dysmorphic disorder scores correlated inversely with the subjective nasal scoring (n = 210, p dysmorphic disorder symptoms significantly reduced the generic quality of life (n = 160, p dysmorphic disorder symptoms in an aesthetic rhinoplasty population is high. Patients undergoing revision rhinoplasty and with psychiatric history are particularly at risk. Body dysmorphic disorder symptoms significantly reduce the quality of life and cause significant appearance-related disruption of everyday living. Risk, III.

  16. Thermal energy storage devices, systems, and thermal energy storage device monitoring methods

    Science.gov (United States)

    Tugurlan, Maria; Tuffner, Francis K; Chassin, David P.

    2016-09-13

    Thermal energy storage devices, systems, and thermal energy storage device monitoring methods are described. According to one aspect, a thermal energy storage device includes a reservoir configured to hold a thermal energy storage medium, a temperature control system configured to adjust a temperature of the thermal energy storage medium, and a state observation system configured to provide information regarding an energy state of the thermal energy storage device at a plurality of different moments in time.

  17. SECON - A tool for estimation of storage costs and storage project revenue

    International Nuclear Information System (INIS)

    Hall, O.

    1997-01-01

    The SECON model Storage ECONomics is useful for gas suppliers, storage operators, gas distributors and consumers when investigating new storage possibilities. SECON has been used within the Sydkraft group to compare cost for different types of storage and to identify the market niche for lined rock cavern (LRC) storage. In the model cost for the different storage types, salt caverns, LNG, and LRC can be compared. By using input according to market needs each storage type can be validated for a specific service e.g. peak shaving, seasonal storage or balancing. The project revenue can also be calculated. SECON includes three models for income calculation; US storage service, Trading and Avoided Supply Contract Costs. The income models calculates annual turnover, pay of time, net present value, internal rate of return and max. liquidity shortfall for the project. The SECON will facilitate sensitivity analysis both regarding cost for different services and different storage types and on the income side by using different scenarios. At the poster session SECON will be presented live and the delegates will have the opportunity to test the model. (au)

  18. 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)

  19. 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

  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. Predicting storage-dependent damage to red blood cells using nitrite oxidation kinetics, peroxiredoxin-2 oxidation, and hemoglobin and free heme measurements.

    Science.gov (United States)

    Oh, Joo-Yeun; Stapley, Ryan; Harper, Victoria; Marques, Marisa B; Patel, Rakesh P

    2015-12-01

    Storage-dependent damage to red blood cells (RBCs) varies significantly. Identifying RBC units that will undergo higher levels of hemolysis during storage may allow for more efficient inventory management decision-making. Oxidative-stress mediates storage-dependent damage to RBCs and will depend on the oxidant:antioxidant balance. We reasoned that this balance or redox tone will serve as a determinant of how a given RBC unit stores and that its assessment in "young" RBCs will predict storage-dependent hemolysis. RBCs were sampled from bags and segments stored for 7 to 42 days. Redox tone was assessed by nitrite oxidation kinetics and peroxiredoxin-2 (Prx-2) oxidation. In parallel, hemolysis was assessed by measuring cell-free hemoglobin (Hb) and free heme (hemin). Correlation analyses were performed to determine if Day 7 measurements predicted either the level of hemolysis at Day 35 or the increase in hemolysis during storage. Higher Day 7 Prx-2 oxidation was associated with higher Day 35 Prx-2 oxidation, suggesting that early assessment of this variable may identify RBCs that will incur the most oxidative damage during storage. RBCs that oxidized nitrite faster on Day 7 were associated with the greatest levels of storage-dependent hemolysis and increases in Prx-2 oxidation. An inverse relationship between storage-dependent changes in oxyhemoglobin and free heme was observed underscoring an unappreciated reciprocity between these molecular species. Moreover, free heme was higher in the bag compared to paired segments, with opposite trends observed for free Hb. Measurement of Prx-2 oxidation and nitrite oxidation kinetics early during RBC storage may predict storage-dependent damage to RBC including hemolysis-dependent formation of free Hb and heme. © 2015 AABB.

  2. Depression as an independent predictor of postoperative delirium in spine deformity patients undergoing elective spine surgery.

    Science.gov (United States)

    Elsamadicy, Aladine A; Adogwa, Owoicho; Lydon, Emily; Sergesketter, Amanda; Kaakati, Rayan; Mehta, Ankit I; Vasquez, Raul A; Cheng, Joseph; Bagley, Carlos A; Karikari, Isaac O

    2017-08-01

    OBJECTIVE Depression is the most prevalent affective disorder in the US, and patients with spinal deformity are at increased risk. Postoperative delirium has been associated with inferior surgical outcomes, including morbidity and mortality. The relationship between depression and postoperative delirium in patients undergoing spine surgery is relatively unknown. The aim of this study was to determine if depression is an independent risk factor for the development of postoperative delirium in patients undergoing decompression and fusion for deformity. METHODS The medical records of 923 adult patients (age ≥ 18 years) undergoing elective spine surgery at a single major academic institution from 2005 through 2015 were reviewed. Of these patients, 255 (27.6%) patients had been diagnosed with depression by a board-certified psychiatrist and constituted the Depression group; the remaining 668 patients constituted the No-Depression group. Patient demographics, comorbidities, and intra- and postoperative complication rates were collected for each patient and compared between groups. The primary outcome investigated in this study was rate of postoperative delirium, according to DSM-V criteria, during initial hospital stay after surgery. The association between depression and postoperative delirium rate was assessed via multivariate logistic regression analysis. RESULTS Patient demographics and comorbidities other than depression were similar in the 2 groups. In the Depression group, 85.1% of the patients were taking an antidepressant prior to surgery. There were no significant between-group differences in intraoperative variables and rates of complications other than delirium. Postoperative complication rates were also similar between the cohorts, including rates of urinary tract infection, fever, deep and superficial surgical site infection, pulmonary embolism, deep vein thrombosis, urinary retention, and proportion of patients transferred to the intensive care unit. In

  3. The management of perioperative nutrition in patients with end stage liver disease undergoing liver transplantation.

    Science.gov (United States)

    Zhang, Qi-Kun; Wang, Meng-Long

    2015-10-01

    Malnutrition is found in almost 100% of patients with end stage liver disease (ESLD) awaiting transplantation and malnutrition before transplantation leads to higher rates of post-transplant complications and worse graft survival outcomes. Reasons for protein energy malnutrition include several metabolic alterations such as inadequate intake, malabsorption, and overloaded expenditure. And also, stress from surgery, gastrointestinal reperfusion injury, immunosuppressive therapy and corticosteriods use lead to delayed bowl function recovery and disorder of nutrients absorption. In the pretransplant phase, nutritional goals include optimization of nutritional status and treatment of nutrition-related symptoms induced by hepatic decompensation. During the acute post-transplant phase, adequate nutrition is required to help support metabolic demands, replenish lost stores, prevent infection, arrive at a new immunologic balance, and promote overall recovery. In a word, it is extremely important to identify and correct nutritional deficiencies in this population and provide an adequate nutritional support during all phases of liver transplantation (LT). This study review focuses on prevalence, nutrition support, evaluation, and management of perioperative nutrition disorder in patients with ESLD undergoing LT.

  4. Spectrum Of Inherited Metabolic Disorders In Pakistani Children Presenting At A Tertiary Care Centre

    International Nuclear Information System (INIS)

    Cheema, H. A.; Malik, H. S.; Parkash, A.; Fayyaz, Z.

    2016-01-01

    Objective: To determine the frequency, presentation and outcome of various inherited metabolic diseases in children presenting in a tertiary care hospital, Lahore, Pakistan. Study Design: An observational study. Place and Duration of Study: Gastroenterology, Hepatology and Nutrition Department of The Children Hospital and Institute of Child Health, Lahore, from January 2011 to October 2014. Methodology: All children aged < 14 years with high suspicion of a metabolic disorder were inducted. Routine and radiological investigation were carried out at the study place. Comprehensive diagnostic testing of particular metabolic disorder was sent abroad. Those with a specific metabolic disorder were included in the study while those with normal metabolic work-up were excluded. All data was collected on preformed proforma. Result: A total of 239 patients were enrolled. Nineteen different types of inherited metabolic disorders were diagnosed in 180 patients; age ranged from 8 days to 14 years. Consanguinity was positive in 175 (97 percentage) among the parents of the affected children, with previously affected siblings in 64 (35.5 percentage). The most frequent disorders were inherited disorders of carbohydrate metabolism (92, 51 percentage), lipid storage disease (59, 32.7 percentage), organic acidemia and energy defects (18, 10 percentage), amino acid disorder (6, 3.3 percentage), and miscellaneous (4, 2.2 percentage). Fifty-eight (32.2 percentage) presented with acute metabolic crisis, 28 (15.5 percentage) patients presented with early onset liver failure, and 24 (13.3 percentage) with mental retardation. Out of these, 16 (8.8 percentage) expired. Conclusion: Glycogen storage disorders being the commonest followed by Gaucher disease and Galactosemia. The associated complications resulted in high morbidity and mortality. (author)

  5. 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

  6. 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

  7. 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

  8. 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

  9. Use of nuclear explosions to create gas condensate storage in the USSR. LLL Treaty Verification Program

    International Nuclear Information System (INIS)

    Borg, I.Y.

    1982-01-01

    The Soviet Union has described industrial use of nuclear explosions to produce underground hydrocarbon storage. To examples are in the giant Orenburg gas condensate field. There is good reason to believe that three additional cavities were created in bedded salt in the yet to be fully developed giant Astrakhan gas condensate field in the region of the lower Volga. Although contrary to usual western practice, the cavities are believed to be used to store H 2 S-rich, unstable gas condensate prior to processing in the main gas plants located tens of kilometers from the producing fields. Detonations at Orenburg and Astrakhan preceded plant construction. The use of nuclear explosions at several sites to create underground storage of highly corrosive liquid hydrocarbons suggests that the Soviets consider this time and cost effective. The possible benefits from such a plan include degasification and stabilization of the condensate before final processing, providing storage of condensate during periods of abnormally high natural gas production or during periods when condensate but not gas processing facilities are undergoing maintenance. Judging from information provided by Soviet specialists, the individual cavities have a maximum capacity on the order of 50,000 m 3

  10. Corrosion in systems for storage and transportation of petroleum products and biofuels identification, monitoring and solutions

    CERN Document Server

    Groysman, Alec

    2014-01-01

    This book treats corrosion as it occurs and affects processes in real-world situations, and thus points the way to practical solutions. Topics described include the conditions in which petroleum products are corrosive to metals; corrosion mechanisms of petroleum products; which parts of storage tanks containing crude oils and petroleum products undergo corrosion; dependence of corrosion in tanks on type of petroleum products; aggressiveness of petroleum products to polymeric material; how microorganisms take part in corrosion of tanks and pipes containing petroleum products; which corrosion monitoring methods are used in systems for storage and transportation of petroleum products; what corrosion control measures should be chosen; how to choose coatings for inner and outer surfaces of tanks containing petroleum products; and how different additives (oxygenates, aromatic solvents) to petroleum products and biofuels influence metallic and polymeric materials. The book is of interest to corrosion engineers, mat...

  11. Feasibility study of D-mannitol as phase change material for thermal storage

    Directory of Open Access Journals (Sweden)

    Rocío Bayón

    2017-05-01

    Full Text Available The feasibility of D-mannitol as PCM for latent heat storage has been studied by keeping it melted at 180 ºC in air for up to 16 days. During this period of time, down to 80% initial mass was lost and sample appearance changed to a dark-brown-sticky paste. The strong mass decrease implies that not only water but also carbon containing volatile species are produced. These results indicate that D-mannitol undergoes thermal degradation which can be associated to caramelization processes similar to the ones occurring in sugars. Such processes involve complex reactions like dehydrations, condensations and polymerizations, which lead to a great variety of volatile and non-volatile products. Various characterization techniques have been applied in order to study both kinetics and reaction products. From the kinetic point of view thermal degradation of D-mannitol proceeds much faster and at lower temperatures than predicted by the models found in the literature. In relation to the degradation products, the analyses with liquid chromatography indicate the formation of three compounds although their stoichiometry could not be fully elucidated with the characterization techniques applied in this work. However it seems that some of the products are polymeric species that contain carbonyl groups and that are the responsible for the sample browning, the strong absorption in the 200 nm–500 nm range, the two bands at 1730 cm–1–1645 cm–1 observed in infrared spectra and also for the occurrence of a glass transition. Therefore this study proves that D-mannitol undergoes strong and fast degradation at temperatures close to its melting point which should be enough reason for avoiding its use as PCM in any latent heat storage application unless it is somehow stabilized.

  12. Reversible deep storage: reversibility options for storage in deep geological formations

    International Nuclear Information System (INIS)

    2009-01-01

    This report describes the definition approach to reversibility conditions, presents the main characteristics of high-activity and intermediate-activity long-lived wastes, describes the storage in deep geological formations (safety functions, general description of the storage centre), discusses the design options for the different types of wastes (container, storage module, handling processes, phenomenological analysis, monitoring arrangements) and the decision process in support reversibility (steering of the storage process, progressive development and step-by-step closing), and reports and discusses the researches concerning the memory of the storage site

  13. 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.

  14. Functional Neuroimaging of Avoidance Habits in Obsessive-Compulsive Disorder

    OpenAIRE

    Gillan, Claire Marie; Apergis-Schoute, Annemieke Margaretha; Morein-Zamir, Sharon; Urcelay, Gonzalo P; Sule, Akeem; Fineberg, Naomi A; Sahakian, Barbara Jacquelyn; Robbins, Trevor William

    2015-01-01

    Objective: The goal of this study was to determine the neural correlates of excessive habit formation in obsessive-compulsive disorder (OCD). We aimed to (i) test for neurobiological convergence with the known pathophysiology of OCD and (ii) infer, based on abnormalities in brain activation, whether these habits arise from dysfunction in the goal-directed or habit system. Method: Thirty-seven OCD patients and 33 controls learned to avoid shocks while undergoing a functional Magnetic Resona...

  15. Improvement of metabolic disorders by an EP2 receptor agonist via restoration of the subcutaneous adipose tissue in pulmonary emphysema.

    Science.gov (United States)

    Tsuji, Takao; Yamaguchi, Kazuhiro; Kikuchi, Ryota; Nakamura, Hiroyuki; Misaka, Ryoichi; Nagai, Atsushi; Aoshiba, Kazutetsu

    2017-05-01

    Chronic obstructive pulmonary disease (COPD) is often associated with co-morbidities. Metabolic disorders like hyperlipidemia and diabetes occur also in underweight COPD patients, although the mechanism is uncertain. Subcutaneous adipose tissue (SAT) plays an important role in energy homeostasis, since restricted capacity to increase fat cell number with increase in fat cell size occurring instead, is associated with lipotoxicity and metabolic disorders. The aim of this study is to show the protective role of SAT for the metabolic disorders in pulmonary emphysema of a murine model. We found ectopic fat accumulation and impaired glucose homeostasis with wasting of SAT in a murine model of elastase-induced pulmonary emphysema (EIE mice) reared on a high-fat diet. ONO-AE1-259, a selective E-prostanoid (EP) 2 receptor agonist, improved angiogenesis and subsequently adipogenesis, and finally improved ectopic fat accumulation and glucose homeostasis with restoration of the capacity for storage of surplus energy in SAT. These results suggest that metabolic disorders like hyperlipidemia and diabetes occured in underweight COPD is partially due to the less capacity for storage of surplus energy in SAT, though the precise mechanism is uncertained. Our data pave the way for the development of therapeutic interventions for metabolic disorders in emphysema patients, e.g., use of pro-angiogenic agents targeting the capacity for storage of surplus energy in the subcutaneous adipose tissue. Copyright © 2017 Elsevier Inc. All rights reserved.

  16. Gaucher disease: a model disorder for biomarker discovery

    DEFF Research Database (Denmark)

    Boot, Rolf G; van Breemen, Mariëlle J; Wegdam, Wouter

    2009-01-01

    Gaucher disease is an inherited lysosomal storage disorder, characterized by massive accumulation of glucosylceramide-laden macrophages in the spleen, liver and bone marrow as a consequence of deficient activity of glucocerebrosidase. Gaucher disease has been the playground to develop new therape...... in clinical management of Gaucher patients are discussed. Moreover, the use of several modern proteomic technologies for the identification of Gaucher biomarkers is reviewed....

  17. Quantum hologram of macroscopically entangled light via the mechanism of diffuse light storage

    International Nuclear Information System (INIS)

    Gerasimov, L V; Sokolov, I M; Kupriyanov, D V; Havey, M D

    2012-01-01

    In this paper, we consider a quantum memory scheme for light diffusely propagating through a spatially disordered atomic gas. A unique characteristic is enhanced trapping of the signal light pulse by quantum multiple scattering, which can be naturally integrated with the mechanism of stimulated Raman conversion into a long-lived spin coherence. Then, the quantum state of the light can be mapped onto the disordered atomic spin subsystem and can be stored in it for a relatively long time. The proposed memory scheme can be applicable for storage of the macroscopic analogue of the Ψ (−) Bell state and the prepared entangled atomic state performs its quantum hologram, which suggests the possibility of further quantum information processing. (paper)

  18. Endoplasmic Reticulum (ER Stress and Endocrine Disorders

    Directory of Open Access Journals (Sweden)

    Daisuke Ariyasu

    2017-02-01

    Full Text Available The endoplasmic reticulum (ER is the organelle where secretory and membrane proteins are synthesized and folded. Unfolded proteins that are retained within the ER can cause ER stress. Eukaryotic cells have a defense system called the “unfolded protein response” (UPR, which protects cells from ER stress. Cells undergo apoptosis when ER stress exceeds the capacity of the UPR, which has been revealed to cause human diseases. Although neurodegenerative diseases are well-known ER stress-related diseases, it has been discovered that endocrine diseases are also related to ER stress. In this review, we focus on ER stress-related human endocrine disorders. In addition to diabetes mellitus, which is well characterized, several relatively rare genetic disorders such as familial neurohypophyseal diabetes insipidus (FNDI, Wolfram syndrome, and isolated growth hormone deficiency type II (IGHD2 are discussed in this article.

  19. Endoplasmic Reticulum (ER) Stress and Endocrine Disorders

    Science.gov (United States)

    Ariyasu, Daisuke; Yoshida, Hiderou; Hasegawa, Yukihiro

    2017-01-01

    The endoplasmic reticulum (ER) is the organelle where secretory and membrane proteins are synthesized and folded. Unfolded proteins that are retained within the ER can cause ER stress. Eukaryotic cells have a defense system called the “unfolded protein response” (UPR), which protects cells from ER stress. Cells undergo apoptosis when ER stress exceeds the capacity of the UPR, which has been revealed to cause human diseases. Although neurodegenerative diseases are well-known ER stress-related diseases, it has been discovered that endocrine diseases are also related to ER stress. In this review, we focus on ER stress-related human endocrine disorders. In addition to diabetes mellitus, which is well characterized, several relatively rare genetic disorders such as familial neurohypophyseal diabetes insipidus (FNDI), Wolfram syndrome, and isolated growth hormone deficiency type II (IGHD2) are discussed in this article. PMID:28208663

  20. 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.

  1. 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.

  2. 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

  3. [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.

  4. 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

  5. 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

  6. 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

  7. Smoking and the Developing Brain : Altered White Matter Microstructure in Attention-Deficit/Hyperactivity Disorder and Healthy Controls

    NARCIS (Netherlands)

    van Ewijk, Hanneke; Groenman, Annabeth P.; Zwiers, Marcel P.; Heslenfeld, Dirk J.; Faraone, Stephen V.; Hartman, Catharina A.; Luman, Marjolein; Greven, Corina U.; Hoekstra, Pieter J.; Franke, Barbara; Buitelaar, Jan; Oosterlaan, Jaap

    Brain white matter (WM) tracts, playing a vital role in the communication between brain regions, undergo important maturational changes during adolescence and young adulthood, a critical period for the development of nicotine dependence. Attention-deficit/hyperactivity disorder (ADHD) is associated

  8. 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

  9. 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...

  10. Dorfman-Chanarin syndrome: a rare neutral lipid storage disease.

    Science.gov (United States)

    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 of the peripheral smear revealed lipid vacuoles in the leucocytes consistent with Jordan's anomaly, which was confirmed by transmission electron microscopy. Liver biopsy revealed micronodular cirrhosis with macrovesicular steatosis while skin biopsy showed ichthyosis vulgaris. Dorfman-Chanarin syndrome was diagnosed on the basis of clinical and laboratory criteria with certain unreported manifestations. Dietary modifications were instituted and followed up after 1 year with promising results. This emphasizes the importance of neonatal screening for lipid vacuolations in peripheral blood in all cases of congenital ichthyosis.

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

    Directory of Open Access Journals (Sweden)

    Mitra Souvik

    2010-10-01

    Full Text Available 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 of the peripheral smear revealed lipid vacuoles in the leucocytes consistent with Jordan′s anomaly, which was confirmed by transmission electron microscopy. Liver biopsy revealed micronodular cirrhosis with macrovesicular steatosis while skin biopsy showed ichthyosis vulgaris. Dorfman-Chanarin syndrome was diagnosed on the basis of clinical and laboratory criteria with certain unreported manifestations. Dietary modifications were instituted and followed up after 1 year with promising results. This emphasizes the importance of neonatal screening for lipid vacuolations in peripheral blood in all cases of congenital ichthyosis.

  12. 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.)

  13. 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)

  14. Group schema therapy for eating disorders: study protocol.

    Science.gov (United States)

    Calvert, Fiona; Smith, Evelyn; Brockman, Rob; Simpson, Susan

    2018-01-01

    The treatment of eating disorders is a difficult endeavor, with only a relatively small proportion of clients responding to and completing standard cognitive behavioural therapy (CBT). Given the prevalence of co-morbidity and complex personality traits in this population, Schema Therapy has been identified as a potentially viable treatment option. A case series of Group Schema Therapy for Eating Disorders (ST-E-g) yielded positive findings and the study protocol outlined in this article aims to extend upon these preliminary findings to evaluate group Schema Therapy for eating disorders in a larger sample ( n  = 40). Participants undergo a two-hour assessment where they complete a number of standard questionnaires and their diagnostic status is ascertained using the Eating Disorder Examination. Participants then commence treatment, which consists of 25 weekly group sessions lasting for 1.5 h and four individual sessions. Each group consists of five to eight participants and is facilitated by two therapists, at least one of who is a registered psychologist trained on schema therapy. The primary outcome in this study is eating disorder symptom severity. Secondary outcomes include: cognitive schemas, self-objectification, general quality of life, self-compassion, schema mode presentations, and Personality Disorder features. Participants complete psychological measures and questionnaires at pre, post, six-month and 1-year follow-up. This study will expand upon preliminary research into the efficacy of group Schema Therapy for individuals with eating disorders. If group Schema Therapy is shown to reduce eating disorder symptoms, it will hold considerable promise as an intervention option for a group of disorders that is typically difficult to treat. ACTRN12615001323516. Registered: 2/12/2015 (retrospectively registered, still recruiting).

  15. 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.

  16. Association between REM sleep behaviour disorder and impulse control disorder in patients with Parkinson's disease.

    Science.gov (United States)

    Bellosta Diago, E; Lopez Del Val, L J; Santos Lasaosa, S; López Garcia, E; Viloria Alebesque, A

    2017-10-01

    The relationship between impulse control disorder (ICD) and REM sleep behaviour disorder (RBD) has not yet been clarified, and the literature reports contradictory results. Our purpose is to analyse the association between these 2 disorders and their presence in patients under dopaminergic treatment. A total of 73 patients diagnosed with Parkinson's disease and treated with a single dopamine agonist were included in the study after undergoing clinical assessment and completing the single-question screen for REM sleep behaviour disorder and the short version of the questionnaire for impulsive-compulsive behaviours in Parkinson's disease. Mean age was 68.88 ± 7.758 years. Twenty-six patients (35.6%) were classified as probable-RBD. This group showed a significant association with ICD (P=.001) and had a higher prevalence of non-tremor akinetic rigid syndrome and longer duration of treatment with levodopa and dopamine agonists than the group without probable-RBD. We found a significant correlation between the use of oral dopamine agonists and ICD. Likewise, patients treated with oral dopamine agonists demonstrated a greater tendency toward presenting probable-RBD than patients taking dopamine agonists by other routes; the difference was non-significant. The present study confirms the association between RBD and a higher risk of developing symptoms of ICD in Parkinson's disease. Copyright © 2016 Sociedad Española de Neurología. Publicado por Elsevier España, S.L.U. All rights reserved.

  17. Thermal energy storage apparatus, controllers and thermal energy storage control methods

    Science.gov (United States)

    Hammerstrom, Donald J.

    2016-05-03

    Thermal energy storage apparatus, controllers and thermal energy storage control methods are described. According to one aspect, a thermal energy storage apparatus controller includes processing circuitry configured to access first information which is indicative of surpluses and deficiencies of electrical energy upon an electrical power system at a plurality of moments in time, access second information which is indicative of temperature of a thermal energy storage medium at a plurality of moments in time, and use the first and second information to control an amount of electrical energy which is utilized by a heating element to heat the thermal energy storage medium at a plurality of moments in time.

  18. Psychiatric comorbidity of gender identity disorders: a survey among Dutch psychiatrists.

    Science.gov (United States)

    à Campo, Joost; Nijman, Henk; Merckelbach, H; Evers, Catharine

    2003-07-01

    In the Netherlands, it is considered good medical practice to offer patients with gender identity disorder the option to undergo hormonal and surgical sex reassignment therapy. A liberalization of treatment guidelines now allows for such treatment to be started at puberty or prepuberty. The question arises as to what extent gender identity disorder can be reliably distinguished from a cross-gender identification that is secondary to other psychiatric disorders. The authors sent survey questionnaires to 382 board-certified Dutch psychiatrists regarding their experiences with diagnosing and treating patients with gender identity disorder. One hundred eighty-six psychiatrists responded to the survey. These respondents reported on 584 patients with cross-gender identification. In 225 patients (39%), gender identity disorder was regarded as the primary diagnosis. For the remaining 359 patients (61%), cross-gender identification was comorbid with other psychiatric disorders. In 270 (75%) of these 359 patients, cross-gender identification was interpreted as an epiphenomenon of other psychiatric illnesses, notably personality, mood, dissociative, and psychotic disorders. These data suggest that there is little consensus, at least among Dutch psychiatrists, about diagnostic features of gender identity disorder or about the minimum age at which sex reassignment therapy is a safe option. Therapy options proposed to patients with gender identity disorder appear to depend on personal preferences of psychiatrists. These results underline the need for more specific diagnostic rules in this area.

  19. Prevalence and impact of alcohol and other drug use disorders on sedation and mechanical ventilation: a retrospective study

    Directory of Open Access Journals (Sweden)

    Jenvey Wendy I

    2007-03-01

    Full Text Available Abstract Background Experience suggests that patients with alcohol and other drug use disorders (AOD are commonly cared for in our intensive care units (ICU's and require more sedation. We sought to determine the impact of AOD on sedation requirement and mechanical ventilation (MV duration. Methods Retrospective review of randomly selected records of adult patients undergoing MV in the medical ICU. Diagnoses of AOD were identified using strict criteria in Diagnostic and Statistical Manual of Mental Disorders, and through review of medical records and toxicology results. Results Of the 70 MV patients reviewed, 27 had AOD (39%. Implicated substances were alcohol in 22 patients, cocaine in 5, heroin in 2, opioids in 2, marijuana in 2. There was no difference between AOD and non-AOD patients in age, race, or reason for MV, but patients with AOD were more likely to be male (21 versus 15, p Conclusion The prevalence of AOD among medical ICU patients undergoing MV is high. Patients with AOD receive higher doses of sedation than their non-AOD counterparts to achieve similar RASS scores but do not undergo longer duration of MV.

  20. 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

  1. 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

  2. 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.)

  3. Organ specific proteomic dissection of Selaginella bryopteris undergoing dehydration and rehydration

    Directory of Open Access Journals (Sweden)

    Farah eDeeba

    2016-04-01

    Full Text Available To explore molecular mechanisms underlying the physiological response of Selaginella bryopteris, a comprehensive proteome analysis was carried out in roots and fronds undergoing dehydration and rehydration. Plants were dehydrated for 7 days followed by 2 h and 24 h of rehydration. In roots out of 59 identified spots, 58 protein spots were found to be up-regulated during dehydration stress. The identified proteins were related to signaling, stress and defense, protein and nucleotide metabolism, carbohydrate and energy metabolism, storage and epigenetic control. Most of these proteins remained up-regulated on first rehydration, suggesting their role in recovery phase also. Among the 90 identified proteins in fronds, about 49% proteins were up-regulated during dehydration stress. Large number of ROS scavenging proteins was enhanced on dehydration. Many other proteins involved in energy, protein turnover and nucleotide metabolism, epigenetic control were also highly upregulated. Many photosynthesis related proteins were upregulated during stress. This would have helped plant to recover rapidly on rehydration. This study provides a comprehensive picture of different cellular responses elucidated by the proteome changes during dehydration and rehydration in roots and fronds as expected from a well-choreographed response from a resurrection plant.

  4. Functional neuroanatomy of disorders of consciousness.

    Science.gov (United States)

    Di Perri, Carol; Stender, Johan; Laureys, Steven; Gosseries, Olivia

    2014-01-01

    Our understanding of the mechanisms of loss and recovery of consciousness, following severe brain injury or during anesthesia, is changing rapidly. Recent neuroimaging studies have shown that patients with chronic disorders of consciousness and subjects undergoing general anesthesia present a complex dysfunctionality in the architecture of brain connectivity. At present, the global hallmark of impaired consciousness appears to be a multifaceted dysfunctional connectivity pattern with both within-network loss of connectivity in a widespread frontoparietal network and between-network hyperconnectivity involving other regions such as the insula and ventral tegmental area. Despite ongoing efforts, the mechanisms underlying the emergence of consciousness after severe brain injury are not thoroughly understood. Important questions remain unanswered: What triggers the connectivity impairment leading to disorders of consciousness? Why do some patients recover from coma, while others with apparently similar brain injuries do not? Understanding these mechanisms could lead to a better comprehension of brain function and, hopefully, lead to new therapeutic strategies in this challenging patient population. © 2013.

  5. 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.

  6. An intelligent data model for the storage of structured grids

    Science.gov (United States)

    Clyne, John; Norton, Alan

    2013-04-01

    With support from the U.S. National Science Foundation we have developed, and currently maintain, VAPOR: a geosciences-focused, open source visual data analysis package. VAPOR enables highly interactive exploration, as well as qualitative and quantitative analysis of high-resolution simulation outputs using only a commodity, desktop computer. The enabling technology behind VAPOR's ability to interact with a data set, whose size would overwhelm all but the largest analysis computing resources, is a progressive data access file format, called the VAPOR Data Collection (VDC). The VDC is based on the discrete wavelet transform and their information compaction properties. Prior to analysis, raw data undergo a wavelet transform, concentrating the information content into a fraction of the coefficients. The coefficients are then sorted by their information content (magnitude) into a small number of bins. Data are reconstructed by applying an inverse wavelet transform. If all of the coefficient bins are used during reconstruction the process is lossless (up to floating point round-off). If only a subset of the bins are used, an approximation of the original data is produced. A crucial point here is that the principal benefit to reconstruction from a subset of wavelet coefficients is a reduction in I/O. Further, if smaller coefficients are simply discarded, or perhaps stored on more capacious tertiary storage, secondary storage requirements (e.g. disk) can be reduced as well. In practice, these reductions in I/O or storage can be on the order of tens or even hundreds. This talk will briefly describe the VAPOR Data Collection, and will present real world success stories from the geosciences that illustrate how progressive data access enables highly interactive exploration of Big Data.

  7. Utilizing cloud storage architecture for long-pulse fusion experiment data storage

    Energy Technology Data Exchange (ETDEWEB)

    Zhang, Ming; Liu, Qiang [State Key Laboratory of Advanced Electromagnetic Engineering and Technology, Wuhan, Hubei (China); School of Electrical and Electronic Engineering, Huazhong University of Science and Technology, Wuhan, Hubei (China); Zheng, Wei, E-mail: zhenghaku@gmail.com [State Key Laboratory of Advanced Electromagnetic Engineering and Technology, Wuhan, Hubei (China); School of Electrical and Electronic Engineering, Huazhong University of Science and Technology, Wuhan, Hubei (China); Wan, Kuanhong; Hu, Feiran; Yu, Kexun [State Key Laboratory of Advanced Electromagnetic Engineering and Technology, Wuhan, Hubei (China); School of Electrical and Electronic Engineering, Huazhong University of Science and Technology, Wuhan, Hubei (China)

    2016-11-15

    Scientific data storage plays a significant role in research facility. The explosion of data in recent years was always going to make data access, acquiring and management more difficult especially in fusion research field. For future long-pulse experiment like ITER, the extremely large data will be generated continuously for a long time, putting much pressure on both the write performance and the scalability. And traditional database has some defects such as inconvenience of management, hard to scale architecture. Hence a new data storage system is very essential. J-TEXTDB is a data storage and management system based on an application cluster and a storage cluster. J-TEXTDB is designed for big data storage and access, aiming at improving read–write speed, optimizing data system structure. The application cluster of J-TEXTDB is used to provide data manage functions and handles data read and write operations from the users. The storage cluster is used to provide the storage services. Both clusters are composed with general servers. By simply adding server to the cluster can improve the read–write performance, the storage space and redundancy, making whole data system highly scalable and available. In this paper, we propose a data system architecture and data model to manage data more efficient. Benchmarks of J-TEXTDB performance including read and write operations are given.

  8. Utilizing cloud storage architecture for long-pulse fusion experiment data storage

    International Nuclear Information System (INIS)

    Zhang, Ming; Liu, Qiang; Zheng, Wei; Wan, Kuanhong; Hu, Feiran; Yu, Kexun

    2016-01-01

    Scientific data storage plays a significant role in research facility. The explosion of data in recent years was always going to make data access, acquiring and management more difficult especially in fusion research field. For future long-pulse experiment like ITER, the extremely large data will be generated continuously for a long time, putting much pressure on both the write performance and the scalability. And traditional database has some defects such as inconvenience of management, hard to scale architecture. Hence a new data storage system is very essential. J-TEXTDB is a data storage and management system based on an application cluster and a storage cluster. J-TEXTDB is designed for big data storage and access, aiming at improving read–write speed, optimizing data system structure. The application cluster of J-TEXTDB is used to provide data manage functions and handles data read and write operations from the users. The storage cluster is used to provide the storage services. Both clusters are composed with general servers. By simply adding server to the cluster can improve the read–write performance, the storage space and redundancy, making whole data system highly scalable and available. In this paper, we propose a data system architecture and data model to manage data more efficient. Benchmarks of J-TEXTDB performance including read and write operations are given.

  9. Convergence of Artificial Protein Polymers and Intrinsically Disordered Proteins.

    Science.gov (United States)

    Dzuricky, Michael; Roberts, Stefan; Chilkoti, Ashutosh

    2018-05-01

    A flurry of research in recent years has revealed the molecular origins of many membraneless organelles to be the liquid phase separation of intrinsically disordered proteins (IDPs). Consequently, protein disorder has emerged as an important driver of intracellular compartmentalization by providing specialized microenvironments chemically distinct from the surrounding medium. Though the importance of protein disorder and its relationship to intracellular phase behavior are clear, a detailed understanding of how such phase behavior can be predicted and controlled remains elusive. While research in IDPs has largely focused on the implications of structural disorder on cellular function and disease, another field, that of artificial protein polymers, has focused on the de novo design of protein polymers with controllable material properties. A subset of these polymers, specifically those derived from structural proteins such as elastin and resilin, are also disordered sequences that undergo liquid-liquid phase separation. This phase separation has been used in a variety of biomedical applications, and researchers studying these polymers have developed methods to precisely characterize and tune their phase behavior. Despite their disparate origins, both fields are complementary as they study the phase behavior of intrinsically disordered polypeptides. This Perspective hopes to stimulate collaborative efforts by highlighting the similarities between these two fields and by providing examples of how such collaboration could be mutually beneficial.

  10. Optimal routing in an automated storage/retrieval system with dedicated storage

    NARCIS (Netherlands)

    Berg, van den J.P.; Gademann, A.J.R.M.

    1999-01-01

    We address the sequencing of requests in an automated storage/retrieval system with dedicated storage. We consider the block sequencing approach, where a set of storage and retrieval requests is given beforehand and no new requests come in during operation. The objective for this static problem is

  11. Investigation and management of the hepatic glycogen storage diseases.

    Science.gov (United States)

    Bhattacharya, Kaustuv

    2015-07-01

    The glycogen storage diseases (GSD) comprise a group of disorders that involve the disruption of metabolism of glycogen. Glycogen is stored in various organs including skeletal muscle, the kidneys and liver. The liver stores glycogen to supply the rest of the body with glucose when required. Therefore, disruption of this process can lead to hypoglycaemia. If glycogen is not broken down effectively, this can lead to hepatomegaly. Glycogen synthase deficiency leads to impaired glycogen synthesis and consequently the liver is small. Glycogen brancher deficiency can lead to abnormal glycogen being stored in the liver leading to a quite different disorder of progressive liver dysfunction. Understanding the physiology of GSD I, III, VI and IX guides dietary treatments and the provision of appropriate amounts and types of carbohydrates. There has been recent re-emergence in the literature of the use of ketones in therapy, either in the form of the salt D,L-3-hydroxybutyrate or medium chain triglyceride (MCT). High protein diets have also been advocated. Alternative waxy maize based starches seem to show promising early data of efficacy. There are many complications of each of these disorders and they need to be prospectively surveyed and managed. Liver and kidney transplantation is still indicated in severe refractory disease.

  12. 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

  13. Efficient Supercapacitor Energy Storage Using Conjugated Microporous Polymer Networks Synthesized from Buchwald-Hartwig Coupling.

    Science.gov (United States)

    Liao, Yaozu; Wang, Haige; Zhu, Meifang; Thomas, Arne

    2018-03-01

    Supercapacitors have received increasing interest as energy storage devices due to their rapid charge-discharge rates, high power densities, and high durability. In this work, novel conjugated microporous polymer (CMP) networks are presented for supercapacitor energy storage, namely 3D polyaminoanthraquinone (PAQ) networks synthesized via Buchwald-Hartwig coupling between 2,6-diaminoanthraquinone and aryl bromides. PAQs exhibit surface areas up to 600 m 2 g -1 , good dispersibility in polar solvents, and can be processed to flexible electrodes. The PAQs exhibit a three-electrode specific capacitance of 576 F g -1 in 0.5 m H 2 SO 4 at a current of 1 A g -1 retaining 80-85% capacitances and nearly 100% Coulombic efficiencies (95-98%) upon 6000 cycles at a current density of 2 A g -1 . Asymmetric two-electrode supercapacitors assembled by PAQs show a capacitance of 168 F g -1 of total electrode materials, an energy density of 60 Wh kg -1 at a power density of 1300 W kg -1 , and a wide working potential window (0-1.6 V). The asymmetric supercapacitors show Coulombic efficiencies up to 97% and can retain 95.5% of initial capacitance undergo 2000 cycles. This work thus presents novel promising CMP networks for charge energy storage. © 2018 WILEY-VCH Verlag GmbH & Co. KGaA, Weinheim.

  14. Using animal models to study post-partum psychiatric disorders.

    Science.gov (United States)

    Perani, C V; Slattery, D A

    2014-10-01

    The post-partum period represents a time during which all maternal organisms undergo substantial plasticity in a wide variety of systems in order to ensure the well-being of the offspring. Although this time is generally associated with increased calmness and decreased stress responses, for a substantial subset of mothers, this period represents a time of particular risk for the onset of psychiatric disorders. Thus, post-partum anxiety, depression and, to a lesser extent, psychosis may develop, and not only affect the well-being of the mother but also place at risk the long-term health of the infant. Although the risk factors for these disorders, as well as normal peripartum-associated adaptations, are well known, the underlying aetiology of post-partum psychiatric disorders remains poorly understood. However, there have been a number of attempts to model these disorders in basic research, which aim to reveal their underlying mechanisms. In the following review, we first discuss known peripartum adaptations and then describe post-partum mood and anxiety disorders, including their risk factors, prevalence and symptoms. Thereafter, we discuss the animal models that have been designed in order to study them and what they have revealed about their aetiology to date. Overall, these studies show that it is feasible to study such complex disorders in animal models, but that more needs to be done in order to increase our knowledge of these severe and debilitating mood and anxiety disorders. © 2014 The British Pharmacological Society.

  15. 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.

  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. Spent fuel storage requirements: the need for away-from-reactor storage

    International Nuclear Information System (INIS)

    1980-01-01

    The analyses of on-site storage capabilities of domestic utilities and estimates of timing and magnitude of away-from-reactor (AFR) storage requirements were presented in the report DOE/ET-0075 entitled Spent Fuel Storage Requirements: The Need For Away-From-Reactor Storage published in February 1979 by the US Department of Energy. Since utility plans and requirements continue to change with time, a need exists to update the AFR requirements estimates as appropriate. This short report updates the results presented in DOE/ET-0075 to reflect recent data on reactor operations and spent fuel storage. In addition to the updates of cases representing the range of AFR requirements in DOE/ET-0075, new cases of interest reflecting utility and regulatory trends are presented

  18. A mouse model for fucosidosis recapitulates storage pathology and neurological features of the milder form of the human disease

    DEFF Research Database (Denmark)

    Wolf, Heike; Damme, Markus; Stroobants, Stijn

    2016-01-01

    Fucosidosis is a rare lysosomal storage disorder caused by the inherited deficiency of the lysosomal hydrolase α-L-fucosidase, which leads to an impaired degradation of fucosylated glycoconjugates. Here we report the generation of a fucosidosis mouse model, in which the gene for lysosomal α-L-fuc...

  19. 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.

  20. 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

  1. Storage of platelets: effects associated with high platelet content in platelet storage containers.

    Science.gov (United States)

    Gulliksson, Hans; Sandgren, Per; Sjödin, Agneta; Hultenby, Kjell

    2012-04-01

    A major problem associated with platelet storage containers is that some platelet units show a dramatic fall in pH, especially above certain platelet contents. The aim of this study was a detailed investigation of the different in vitro effects occurring when the maximum storage capacity of a platelet container is exceeded as compared to normal storage. Buffy coats were combined in large-volume containers to create primary pools to be split into two equal aliquots for the preparation of platelets (450-520×10(9) platelets/unit) in SSP+ for 7-day storage in two containers (test and reference) with different platelet storage capacity (n=8). Exceeding the maximum storage capacity of the test platelet storage container resulted in immediate negative effects on platelet metabolism and energy supply, but also delayed effects on platelet function, activation and disintegration. Our study gives a very clear indication of the effects in different phases associated with exceeding the maximum storage capacity of platelet containers but throw little additional light on the mechanism initiating those negative effects. The problem appears to be complex and further studies in different media using different storage containers will be needed to understand the mechanisms involved.

  2. 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

  3. 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.

  4. Post-transplant Lymphoproliferative Disorder Arising from Renal Allograft Parenchyma: A Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Park, Byung Kwan; Kim, Chan Kyo; Kwon, Ghee Young [Samsung Medical Center, Sungkyunkwan University College of Medicine, Seoul (Korea, Republic of)

    2010-06-15

    Post-transplant lymphoproliferative disorder (PTLD) is a rare but serious complication that occurs in patients undergoing kidney transplantation. PTLD usually manifests as a renal hilar mass comprised of histologically B-lymphocytes. We report our experience of managing a patient with PTLD arising from renal parenchyma. Ultrasonographic and MR imaging features of this unusual PTLD suggested differentiated renal cell carcinoma arising from the renal allograft

  5. 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.)

  6. 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.

  7. Structural Disorder within Paramyxoviral Nucleoproteins and Phosphoproteins in Their Free and Bound Forms: From Predictions to Experimental Assessment

    Directory of Open Access Journals (Sweden)

    Johnny Habchi

    2015-07-01

    Full Text Available We herein review available computational and experimental data pointing to the abundance of structural disorder within the nucleoprotein (N and phosphoprotein (P from three paramyxoviruses, namely the measles (MeV, Nipah (NiV and Hendra (HeV viruses. We provide a detailed molecular description of the mechanisms governing the disorder-to-order transition that the intrinsically disordered C-terminal domain (NTAIL of their N proteins undergoes upon binding to the C-terminal X domain (PXD of the homologous P proteins. We also show that NTAIL–PXD complexes are “fuzzy”, i.e., they possess a significant residual disorder, and discuss the possible functional significance of this fuzziness. Finally, we emphasize the relevance of N–P interactions involving intrinsically disordered proteins as promising targets for new antiviral approaches, and end up summarizing the general functional advantages of disorder for viruses.

  8. Storage and distribution/Linear programming for storage operations

    Energy Technology Data Exchange (ETDEWEB)

    Coleman, D

    1978-07-15

    The techniques of linear programing to solve storage problems as applied in a tank farm tie-in with refinery throughput operation include: (1) the time-phased model which works on storage and refinery operations input parameters, e.g., production, distribution, cracking, etc., and is capable of representing product stockpiling in slack periods to meet future peak demands, and investigating alternative strategies such as exchange deals and purchase and leasing of additional storage, and (2) the Monte Carlo simulation method, which inputs parameters, e.g., arrival of crude products at refinery, tankage size, likely demand for products, etc., as probability distributions rather than single values, and is capable of showing the average utilization of facilities, potential bottlenecks, investment required to achieve an increase in utilization, and to enable the user to predict total investment, cash flow, and profit emanating from the original financing decision. The increasing use of computer techniques to solve refinery and storage problems is attributed to potential savings resulting from more effective planning, reduced computer costs, ease of access and more usable software. Diagrams.

  9. [Cerebrotendinous xanthomatosis is a rare disorder, which requires a specific treatment].

    Science.gov (United States)

    Blaabjerg, Morten; Marjanovic, Dragan

    2013-01-28

    Cerebrotendinous xanthomatosis (CTX) is a rare, but treatable lipid storage disorder caused by mutation in the CYP27A1 gene. The disorder results in deposition of cholestanol in various tissues. The classical CTX phenotype includes diarrhoea, juvenile cataract, xanthoma and progressive neurological symptoms. Studies have shown that progression of symptoms can be halted or even reversed, if treatment with chenodeoxycholic acid is initiated early. The diagnosis of CTX is often delayed due to lack of awareness of the disease. We describe the history, clinical features, biochemical, genetic and magnetic resonance imaging findings of the first reported case of CTX in Denmark.

  10. The Effectiveness of Group Spiritual Intervention on Self-esteem and Happiness among Men Undergoing Methadone Maintenance Treatment.

    Science.gov (United States)

    Jalali, Amir; Behrouzi, Mahvash Kashkouli; Salari, Nader; Bazrafshan, Mohammad-Rafi; Rahmati, Mahmoud

    2018-05-10

    Drug dependence or substance use disorder not only affects a person's life but also brings a lot of challenges for families and communities and imposes heavy burdens on them. There are various therapies in the domain of addiction whose main purposes are to reduce or to cut down substance abuse. This study aimed to determine the effectiveness of group spiritual intervention on self-esteem and happiness among male clients undergoing methadone maintenance treatment. This study was an intervention study in which 60 clients affected with substance abuse and undergoing methadone maintenance treatment were recruited. The study samples were selected through convenience sampling method and then divided randomly into two groups of 30 individuals: intervention and control. The intervention group attended group spiritual interventions for 10 sessions. Self-esteem and happiness among the study participants were also measured through Coopersmith Self-Esteem Inventory and Oxford Happiness Questionnaire before and after the intervention. The results showed a significant difference between both intervention and control groups in terms of self-esteem and happiness (P˂0.05); so that the participants in the intervention group demonstrated a significant improvement in their self-esteem and happiness. It was concluded that group spiritual intervention as a useful method could be effective in enhancing self-esteem and happiness among addicted individuals undergoing methadone maintenance treatment. The given treatment could be also used as a complementary therapy beside methadone maintenance treatment to reduce the likelihood of people returning to substance abuse. Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.org.

  11. Dissociative trance disorder: clinical and Rorschach findings in ten persons reporting demon possession and treated by exorcism.

    Science.gov (United States)

    Ferracuti, S; Sacco, R; Lazzari, R

    1996-06-01

    Although dissociative trance disorders, especially possession disorder, are probably more common than is usually though, precise clinical data are lacking. Ten persons undergoing exorcisms for devil trance possession state were studied with the Dissociative Disorders Diagnostic Schedule and the Rorschach test. These persons had many traits in common with dissociative identity disorder patients. They were overwhelmed by paranormal experiences. Despite claiming possession by a demon, most of them managed to maintain normal social functioning. Rorschach findings showed that these persons had a complex personality organization: Some of them displayed a tendency to oversimplify stimulus perception whereas others seemed more committed to psychological complexity. Most had severe impairment of reality testing, and 6 of the participants had an extratensive coping stile. In this group of persons reporting demon possession, dissociative trance disorder seems to be a distinct clinical manifestation of a dissociative continuum, sharing some features with dissociative identity disorder.

  12. A saposin deficiency model in Drosophila: Lysosomal storage, progressive neurodegeneration and sensory physiological decline.

    Science.gov (United States)

    Hindle, Samantha J; Hebbar, Sarita; Schwudke, Dominik; Elliott, Christopher J H; Sweeney, Sean T

    2017-02-01

    Saposin deficiency is a childhood neurodegenerative lysosomal storage disorder (LSD) that can cause premature death within three months of life. Saposins are activator proteins that promote the function of lysosomal hydrolases that mediate the degradation of sphingolipids. There are four saposin proteins in humans, which are encoded by the prosaposin gene. Mutations causing an absence or impaired function of individual saposins or the whole prosaposin gene lead to distinct LSDs due to the storage of different classes of sphingolipids. The pathological events leading to neuronal dysfunction induced by lysosomal storage of sphingolipids are as yet poorly defined. We have generated and characterised a Drosophila model of saposin deficiency that shows striking similarities to the human diseases. Drosophila saposin-related (dSap-r) mutants show a reduced longevity, progressive neurodegeneration, lysosomal storage, dramatic swelling of neuronal soma, perturbations in sphingolipid catabolism, and sensory physiological deterioration. Our data suggests a genetic interaction with a calcium exchanger (Calx) pointing to a possible calcium homeostasis deficit in dSap-r mutants. Together these findings support the use of dSap-r mutants in advancing our understanding of the cellular pathology implicated in saposin deficiency and related LSDs. Copyright © 2016 The Authors. Published by Elsevier Inc. All rights reserved.

  13. Integrity of spent CANDU fuel during and following dry storage

    International Nuclear Information System (INIS)

    Villagran, J.E.

    2004-01-01

    This report examines the issue of CANDU fuel integrity at the back end of the fuel cycle and outlines a program designed to provide assurance that used CANDU fuel will retain its integrity over an extended period. In specific terms, the program is intended to provide assurance that during and following extended dry storage the fuel will remain fit to undergo, without loss of integrity, the handling, packaging and transportation operations that might be necessary until it is placed in disposal containers. The first step in the development of the program was a review of the available technical information on phenomena relevant to fuel integrity. The major conclusions from that review were the following: Under normal storage conditions it is unlikely that the spent fuel will suffer significant degradation during a one-hundred year period and it should be possible to retrieve, repackage and transport the fuel as required, using methods and systems similar to those used today. However, to provide increased confidence regarding the above conclusion, investigations should be conducted in areas where there is higher uncertainty in the prediction of fuel condition and on some degradation processes to which the fuel appears to present higher vulnerability. The proposed program includes, among other tasks, irradiated fuel tests, analytical studies on the most relevant fuel degradation processes and the development of a long-term fuel verification program. (Author)

  14. The miRNA Profile of Platelets Stored in a Blood Bank and Its Relation to Cellular Damage from Storage.

    Directory of Open Access Journals (Sweden)

    Thaís Brilhante Pontes

    Full Text Available Millions of blood products are transfused each year, and many lives are directly affected by transfusion. Platelet concentrate (PC is one of the main products derived from blood. Even under good storage conditions, PC is likely to suffer cell damage. The shape of platelets changes after 5 to 7 days of storage at 22°C. Taking into consideration that some platelet proteins undergo changes in their shape and functionality during PC storage. Sixteen PC bags were collected and each PC bag tube was cut into six equal pieces to perform experiments with platelets from six different days of storage. Thus, on the first day of storage, 1/6 of the tube was used for miRNA extraction, and the remaining 5/6 was stored under the same conditions until extraction of miRNAs on each the following five days. Samples were sequenced on an Illumina Platform to demonstrate the most highly expressed miRNAs. Three miRNAs, mir127, mir191 and mir320a were validated by real-time quantitative PCR (RQ-PCR in 100 PC bags tubes. Our method suggests, the use of the miRNAs mir127 and mir320a as biomarkers to assess the "validity period" of PC bags stored in blood banks for long periods. Thus, bags can be tested on the 5th day of storage for the relative expression levels of mir127 and mir320a. Thus, we highlight candidate miRNAs as biomarkers of storage damage that can be used as tools to evaluate the quality of stored PC. The use of miRNAs as biomarkers of damage is unprecedented and will contribute to improved quality of blood products for transfusions.

  15. 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

  16. 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.

  17. 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.

  18. Measuring influenza RNA quantity after prolonged storage or multiple freeze/thaw cycles.

    Science.gov (United States)

    Granados, Andrea; Petrich, Astrid; McGeer, Allison; Gubbay, Jonathan B

    2017-09-01

    In this study, we aim to determine what effects prolonged storage and repeated freeze/thaw cycles have on the stability of influenza A(H1N1)pdm09 (influenza A/H1N1)RNA. Cloned influenza A/H1N1 RNA transcripts were serially diluted from 8.0-1.0 log 10 copies/μl. RT-qPCR was used to measure RNA loss in transcripts stored at -80°C, -20°C, 4°C and 25°C for up to 84days or transcripts undergoing a total of 10 freeze/thaw cycles. Viral load was measured in clinical specimens stored at-80°C for three years (n=89 influenza A RNA extracts; n=35 primary specimens) and in 10 clinical specimens from the 2015/2016 influenza season that underwent 7 freeze/thaw cycles. RNA stored at -80°C, -20°C, 4°C and 25°C is stable for up to 56, 56, 21, and 7days respectively or up to 9 freeze/thaw cycles when stored at -80°C. There is no difference in viral load in clinical specimens that have been stored for up to three years at -80°C if they are re-extracted. Similarly, clinical specimens undergoing up to 7 freeze/thaw cycles are stable if they are re-extracted between cycles. Influenza specimens can be stored for up to three years at -80°C or undergo up to 7 freeze/thaw cycles without loss of RNA quantity if re-extracted. Copyright © 2017 Elsevier B.V. All rights reserved.

  19. 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.

  20. Maximizing the energy storage performance of phase change thermal storage systems

    Energy Technology Data Exchange (ETDEWEB)

    Amin, N.A.M.; Bruno, F.; Belusko, M. [South Australia Univ., Mawson Lakes, South Australia (Australia). Inst. for Sustainable Systems and Technologies

    2009-07-01

    The demand for electricity in South Australia is highly influenced by the need for refrigeration and air-conditioning. An extensive literature review has been conducted on the use of phase change materials (PCMs) in thermal storage systems. PCMs use latent heat at the solid-liquid phase transition point to store thermal energy. They are considered to be useful as a thermal energy storage (TES) material because they can provide much higher energy storage densities compared to conventional sensible thermal storage materials. This paper reviewed the main disadvantages of using PCMs for energy storage, such as low heat transfer, super cooling and system design issues. Other issues with PCMs include incongruence and corrosion of heat exchanger surfaces. The authors suggested that in order to address these problems, future research should focus on maximizing heat transfer by optimizing the configuration of the encapsulation through a parametric analysis using a PCM numerical model. The effective conductivity in encapsulated PCMs in a latent heat thermal energy storage (LHTES) system can also be increased by using conductors in the encapsulation that have high thermal conductivity. 47 refs., 1 tab., 1 fig.

  1. 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.

  2. 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.

  3. Saline Cavern Adiabatic Compressed Air Energy Storage Using Sand as Heat Storage Material

    Directory of Open Access Journals (Sweden)

    Martin Haemmerle

    2017-03-01

    Full Text Available Adiabatic compressed air energy storage systems offer large energy storage capacities and power outputs beyond 100MWel. Salt production in Austria produces large caverns which are able to hold pressure up to 100 bar, thus providing low cost pressurized air storage reservoirs for adiabatic compressed air energy storage plants. In this paper the results of a feasibility study is presented, which was financed by the Austrian Research Promotion Agency, with the objective to determine the adiabatic compressed air energy storage potential of Austria’s salt caverns. The study contains designs of realisable plants with capacities between 10 and 50 MWel, applying a high temperature energy storage system currently developed at the Institute for Energy Systems and Thermodynamics in Vienna. It could be shown that the overall storage potential of Austria’s salt caverns exceeds a total of 4GWhel in the year 2030 and, assuming an adequate performance of the heat exchanger, that a 10MWel adiabatic compressed air energy storage plant in Upper Austria is currently feasible using state of the art thermal turbomachinery which is able to provide a compressor discharge temperature of 400 °C.

  4. Modular vault dry storage system for interim storage of irradiated fuel

    International Nuclear Information System (INIS)

    Cundill, B.R.; Ealing, C.J.; Agarwal, B.K.

    1988-01-01

    The Foster Wheeler Energy Application (FWEA) Modular Vault Dry Store (MVDS) is a dry storage concept for the storage of all types of irradiated reactor fuel. For applications in the US, FWEA submitted an MVDS Topical Report to the US NRC during 1986. Following NRC approval of the MVDS Topical Report concept for unconsolidated LWR fuel, US utilities have available a new, compact, economic and flexible system for the storage of irradiated fuel at the reactor site for time periods of at least 20 years (the period of the first license). The MVDS concept jointly developed by FWEA and GEC in the U.K., has other applications for large central away from reactor storage facilities such as a Monitorable Retrievable Storage (MRS) installation. This paper describes the licensed MVDS design, aspects of performance are discussed and capital costs compared with alternative concepts. Alternative configurations of MVDS are outlined

  5. 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.)

  6. 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.

  7. Data on the changes of the mussels׳ metabolic profile under different cold storage conditions

    Directory of Open Access Journals (Sweden)

    Violetta Aru

    2016-06-01

    Full Text Available One of the main problems of seafood marketing is the ease with which fish and shellfish undergo deterioration after death. 1H NMR spectroscopy and microbiological analysis were applied to get in depth insight into the effects of cold storage (4 °C and 0 °C on the spoilage of the mussel Mytilus galloprovincialis. This data article provides information on the average distribution of the microbial loads in mussels׳ specimens and on the acquisition, processing, and multivariate analysis of the 1H NMR spectra from the hydrosoluble phase of stored mussels. This data article is referred to the research article entitled “Metabolomics analysis of shucked mussels’ freshness” (Aru et al., 2016 [1].

  8. Preliminary assessment of alternative dry storage methods for the storage of commercial spent nuclear fuel

    International Nuclear Information System (INIS)

    1981-11-01

    This report presents the results of an assessment of the (1) state of technology, (2) licensability, (3) implementation schedule, and (4) costs of alternative dry methods for storage of spent fuel at a reactor location when used to supplement reactor pool storage facilities. The methods of storage that were considered included storage in casks, drywells, concrete silos and air-cooled vaults. The impact of disassembly of spent fuel and storage of consolidated fuel rods was also determined. The economic assessments were made based on the current projected storage requirements of Virginia Electric and Power Company's Surry Station for the period 1985 to 2009, which has two operating pressurized water reactors (824 MWe each). It was estimated that the unit cost for storage of spent fuel in casks would amount to $117/kgU and that such costs for storage in drywells would amount to $137/kgU. However, based on the overall assessment it was concluded both storage methods were equal in merit. Modular methods of storage were generally found to be more economic than those requiring all or most of the facilities to be constructed prior to commencement of storage operations

  9. Apparatus for the storage of transport- and storage-containers containing radioactive fuel elements

    International Nuclear Information System (INIS)

    Vox, A.

    1983-01-01

    The invention concerns an apparatus for the storage of transport and storage containers containing radioactive fuel elements. For each transport or storage container there is a separate silo-type container of steel, concrete, prestressed concrete or suchlike breakproof and fireproof material, to be placed in the open, that can be opened for removal and placing of the transport or storage container respectively. (orig.) [de

  10. Some wind-energy storage options

    Energy Technology Data Exchange (ETDEWEB)

    Eldridge, F R; Ljungstroem, O [ed.

    1976-01-01

    Systems capable of storing energy generated from the wind can be categorized in terms of electrochemical energy storage systems, thermal energy storage systems, kinetic energy systems, and potential energy systems. Recent surveys of energy storage systems have evaluated some of these available storage technologies in terms of the minimum economic sizes for utility applications, estimated capital costs of these units, expected life, dispersed storage capabilities, and estimated turn-around efficiencies of the units. These are summarized for various types of energy storage options.

  11. Hydrogen-based electrochemical energy storage

    Science.gov (United States)

    Simpson, Lin Jay

    2013-08-06

    An energy storage device (100) providing high storage densities via hydrogen storage. The device (100) includes a counter electrode (110), a storage electrode (130), and an ion conducting membrane (120) positioned between the counter electrode (110) and the storage electrode (130). The counter electrode (110) is formed of one or more materials with an affinity for hydrogen and includes an exchange matrix for elements/materials selected from the non-noble materials that have an affinity for hydrogen. The storage electrode (130) is loaded with hydrogen such as atomic or mono-hydrogen that is adsorbed by a hydrogen storage material such that the hydrogen (132, 134) may be stored with low chemical bonding. The hydrogen storage material is typically formed of a lightweight material such as carbon or boron with a network of passage-ways or intercalants for storing and conducting mono-hydrogen, protons, or the like. The hydrogen storage material may store at least ten percent by weight hydrogen (132, 134) at ambient temperature and pressure.

  12. Effect of substrate storage conditions on the stability of “Smart” films used for mammalian cell applications

    Energy Technology Data Exchange (ETDEWEB)

    Bluestein, Blake M.; Reed, Jamie A.; Canavan, Heather E., E-mail: Canavan@unm.edu

    2017-01-15

    Highlights: • pNIPAM can be deposited onto a surface using plasma polymerization or spin coating. • Storage conditions can affect both surfaces although thermoresponse is maintained. • spNIPAM surfaces delaminate over time, regardless of storage conditions. • Delamination will affect cell attachment/detachment, resulting in limited attachment. • ppNIPAM surfaces are more stable, regardless of storage conditions. - Abstract: When poly(N-isopropyl acrylamide) (pNIPAM) is tethered to a surface, it can induce the spontaneous release of a sheet of mammalian cells. The release of cells is a result of the reversible phase transition the polymer undergoes at its lower critical solution temperature (LCST). Many techniques are used for the deposition of pNIPAM onto cell culture substrates. Previously, we compared two methods of deposition (plasma polymerization, and co-deposition with a sol-gel). We proved that although both were technically appropriate for obtaining thermoresponsive pNIPAM films, the surfaces that were co-deposited with a sol-gel caused some disruption in cell activity. The variation of cell behavior could be due to the delamination of pNIPAM films leaching toxic chemicals into solution. In this work, we assessed the stability of these pNIPAM films by manipulating the storage conditions and analyzing the surface chemistry using X-ray photoelectron spectroscopy (XPS) and contact angle measurements over the amount of time required to obtain confluent cell sheets. From XPS, we demonstrated that ppNIPAM (plasma polymerized NIPAM) films remains stable across all storage conditions while sol-gel deposition show large deviations after 48 h of storage. Cell response of the deposited films was assessed by investigating the cytotoxicity and biocompatibility. The 37 °C and high humidity storage affects sol-gel deposited films, inhibiting normal cell growth and proper thermoresponse of the film. Surface chemistry, thermoresponse and cell growth remained

  13. Metastatic melanoma misdiagnosed as a temporomandibular disorder: a case report and review of the literature.

    Science.gov (United States)

    Singh, Samir; Desai, Bhavik; Laskin, Daniel

    2014-10-01

    Malignancies in the head and neck region are difficult to diagnose because of their deep location and presence of symptoms mimicking those of temporomandibular disorders or other orofacial pain disorders. A 75-year-old woman reported experiencing right-sided jaw pain, temporal discomfort and paresthesia. She had undergone conservative therapy for temporomandibular joint disorder, which was unsuccessful. A magnetic resonance image of the midface revealed a mass on the base of the tongue along with possible metastatic lesions to the brain. Further investigation of the lesions revealed them to be metastatic melanoma. Patients with atypical symptoms of facial pain, including neurological signs, should undergo further investigation with advanced imaging to determine the source of the symptoms, which could include neoplasms.

  14. 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.

  15. 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

  16. AN ASSOCIATION OF THE CLINICAL MANIFESTATIONS OF NSAID GASTROPATHY WITH UPPER GASTROINTESTINAL MOTOR DISORDERS IN PATIENTS WITH RHEUMATOID ARTHRITIS

    Directory of Open Access Journals (Sweden)

    D Abdulganiyeva

    2011-01-01

    Conclusion. The upper GI motor disorders found in the RA patients taking NSAIDs may play an important role in the development of clinical manifestations of NSAID gastropathy and, probably, a certain group of patients must undergo medical correction of the symptoms related to dysmotility.

  17. Issues related to the transport of a transportable storage cask after storage

    International Nuclear Information System (INIS)

    McConnell, P.; Brimhall, J.L.; Creer, J.M.; Gilbert, E.R.; Sanders, T.L.; Jones, R.H.

    1991-01-01

    An evaluation was performed to assess whether the reliability of a transportable storage cask system and the risks associated with its use are comparable to those associated with existing transport cask systems and, if they are not, determine how the transportable storage cask system can be made as reliable as existing systems. Reliability and failure mode analyses of both transport-only casks and transportable storage casks and implementation options are compared. Current knowledge regarding the potential effects of a long-term dry storage environment on spent fuel and cask materials is reviewed. A summary assessment of the consideration for deploying a transportable storage cask (TSC) system with emphasis on preliminary design, validation and operational recommendations for TSC implementations is presented. The analyses conclude that a transportable storage cask can likely be shipped upopened by applying a combination of design considerations and operational constraints, including environmental monitoring and pretransport assessments of functional reliability of the cask. A proper mix of these constraints should yield risk parity with any existing transport cask

  18. 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.

  19. Quantification of free sialic acid in urine by HPLC-electrospray tandem mass spectrometry: A tool for the diagnosis of sialic acid storage disease

    NARCIS (Netherlands)

    Valianpour, Fredoen; Abeling, Nicolaas G. G. M.; Duran, Marinus; Huijmans, Jan G. M.; Kulik, Willem

    2004-01-01

    Background: Sialic acid storage diseases (SSDs) are severe autosomal recessive neurodegenerative disorders caused by a transport defect across the lysosomal membrane, which leads to accumulation of sialic acid in tissues, fibroblasts, and urine. Defective free sialic acid transport can be

  20. Dry storage systems using casks for long term storage in an AFR and repository

    International Nuclear Information System (INIS)

    Einfeld, K.; Popp, F.W.

    1986-01-01

    In conclusion it can be stated that two basic routes with respect to spent fuel storage casks are feasible. One is the Multiple Transport Cask, which with certain modifications can be upgraded to meet the criteria for intermediate storage. Its status is characterized by the licensing of several types of Castor Casks for an intermediate storage period of 30 years in the AFR Storage Facility of DWK at Gorleben in the FRG. The other one is the Final Disposal (Repository) Cask, which can be made suitable for long term storage before a final decision with respect to a repository application is taken. The licensing procedure for a Pilot Conditioning Facility with the Pollux Cask System as reference case will be initiated by DWK in the near future. Under the assumption that in addition to the present Multiple Transport/Storage Casks a license for a Final disposal Cask with respect to long term storage is available, the relative merits of different cask storage systems would have to be evaluated

  1. 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.

  2. 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).

  3. 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.

  4. 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 ...

  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. 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. "Shell Shock": An Entity that Predated Combat-related Posttraumatic Stress Disorder

    Directory of Open Access Journals (Sweden)

    Ragul Ganesh

    2015-01-01

    Full Text Available In the last century, numerous soldiers had been diagnosed with various post combat disorders. The terminology that has been utilized to describe such patients include combat fatigue, combat stress reaction, soldier′s heart, effort syndrome, non-ulcer dyspepsia, effects of agent orange and gulf war syndrome. The initial description of such post combat disorder was probably ′shell shock′ which came into vogue during the World War I. The soldiers, undergoing unyielding artillery bombardment, in the trenches suffered persistent symptoms of headache, behavioural changes and memory impairment, and was designated at the time as ′shell shock′. Myers and Mott, independently studied numerous soldiers to elucidate the features and aetiology of the entity. An attempt was made to restrict the usage of the term, but the psychological distress of the soldiers persisted to be addressed in some manner or the other, culminating in the genesis of Post Traumatic Stress Disorder.

  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. Lipid storage myopathy with clinical markers of Marfan syndrome: A rare association

    Directory of Open Access Journals (Sweden)

    Subasree Ramakrishnan

    2012-01-01

    Full Text Available Disorders of lipid metabolism can cause variable clinical presentations, often involving skeletal muscle, alone or together with other tissues. A 19-year-old boy presented with a 2-year history of muscle pain, cramps, exercise intolerance and progressive weakness of proximal lower limbs. Examination revealed skeletal markers of Marfan syndrome in the form of increased arm span compared with height, Kyphoscoliois, moderate pectus excavatum, high arched palate and wrist sign. He also had mild neck flexor weakness and proximal lower limb weakness with areflexia. Pathologic findings revealed lipid-laden fine vacuoles in the muscle fibers. Possibility of carnitine deficiency myopathy was considered and the patient was started on carnitine and Co Q. The patient made remarkable clinical improvement over the next 2 months. This case is reported for rarity of the association of clinical markers of Marfan syndrome and lipid storage myopathy and sparse literature on lipid storage myopathy in the Indian context.

  10. Light affects the chloroplast ultrastructure and post-storage photosynthetic performance of watermelon (Citrullus lanatus) plug seedlings.

    Science.gov (United States)

    Duan, Qingqing; Jiang, Wu; Ding, Ming; Lin, Ye; Huang, Danfeng

    2014-01-01

    Watermelon [Citrullus lanatus (Thunb.) Matsum. and Nakai] plug seedlings were stored at 15°C in the light at a photosynthetic photon flux density of 15 µmol·m(-2)·s(-1) or in darkness for 6 days, to evaluate their chloroplast ultrastructure, and associated photosynthetic characteristics. Storage in the dark caused swelling, disordered granal arrangement, and starch grain disappearance in the chloroplasts. In contrast, the chloroplasts stored in the light were relatively normal. As a result, the light-stored seedlings had a significantly higher chlorophyll content, Fv/Fm, and Pn than did dark-stored seedlings. Regardless of whether the seedlings were stored in light or darkness, the Gs and Ls of the seedlings significantly decreased, while the Ci obviously increased when the Pn decreased after 6 days of storage. This result suggests that the decreased Pn is not solely a stomatal effect, as the effects on the chloroplasts contributed to this photosynthetic inhibition. Six days after transplanting, seedlings that were stored in the light or darkness for 2 or 4 days showed complete recovery of chloroplast ultrastructure, chlorophyll content, Fv/Fm, Gs and Pn. When the storage period increased to 6 days, the dark-stored seedlings had a significantly lower Fv/Fm and Pn than the light-stored and control seedlings 6 days after transplanting, which was mainly ascribed to incomplete recovery of chloroplast ultrastructure. Furthermore, the light-stored seedlings exhibited a significantly higher shoot dry weight during storage and a higher percentage dry weight increase after transplanting than the dark-stored seedlings. These effects were enhanced by prolonged storage (4 to 6 days). This study demonstrated that dim light during storage is beneficial for maintaining chloroplast ultrastructure as well as photosynthetic efficiency in watermelon seedlings, thus contributing to the rapid recovery of post-storage photosynthetic performance, which ensures the transplant quality

  11. Incidence of postoperative delirium in patients undergoing total knee arthroplasty-an Asian perspective.

    Science.gov (United States)

    Huang, Juncheng; Bin Abd Razak, Hamid Rahmatullah; Yeo, Seng Jin

    2017-08-01

    Very little is known in the literature with regards to the incidence of postoperative delirium following total knee arthroplasty (TKA) in Asians and the associated surgical factors. We conducted a retrospective study on incidence of postoperative delirium following TKA in Asians. One thousand sixteen knees of 954 consecutive patients who underwent TKA by the senior author of this study in the year 2006 were included in this review. All written and electronic medical records for patients were screened. Delirium was recorded to be present based on clinical entry onto the patients' inpatient hospital notes and a diagnosis made by psychiatrist based on the Diagnostic and Statistical Manual of Mental Disorders, 4th Edition (DSM-IV). All statistical analysis was performed using SPSS v.18.0 (IBM Corp., Armonk, NY, USA). The incidence of postoperative delirium in our patients undergoing TKA was 0.59%. Six patients had a confirmed diagnosis of delirium postoperatively. We noted an association between a higher mean age (P<0.0001), a lower body mass index (P<0.0001), Chinese ethnicity (P=0.002), male gender (P=0.002) and chronic opioid use (P<0.0001) with incidence of post-operative delirium. We also noted a higher proportion of post-operative DVT (P<0.0001) and wound infection (P<0.0001) in the delirium group. The incidence of postoperative delirium in Asians undergoing TKA in our institution is very low at 0.59%. Advanced age, lower body mass index, Chinese ethnicity, male gender and preoperative chronic opioid use may be associated with developing postoperative delirium.

  12. 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.)

  13. 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.

  14. Spent LWR fuel-storage costs

    International Nuclear Information System (INIS)

    Clark, H.J.

    1981-01-01

    Expanded use of existing storage basins is clearly the most economic solution to the spent fuel storage problem. The use of high-density racks followed by fuel disassembly and rod storage is an order of magnitude cheaper than building new facilities adjacent to the reactor. The choice of a new storage facility is not as obvious; however, if the timing of expenditures and risk allowance are to be considered, then modular concepts such as silos, drywells, and storage casks may cost less than water basins and air-cooled vaults. A comparison of the costs of the various storage techniques without allowances for timing or risk is shown. The impact of allowances for discounting and early resumption of reprocessing is also shown. Economics is not the only issue to be considered in selecting a storage facility. The licensing, environmental impact, timing, and social responses must also be considered. Each utility must assess all of these issues for their particular reactors before the best storage solution can be selected

  15. 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.

  16. Molecular diagnostics of neurodegenerative disorders

    Directory of Open Access Journals (Sweden)

    Megha eAgrawal

    2015-09-01

    Full Text Available Molecular diagnostics provide a powerful method to detect and diagnose various neurological diseases such as Alzheimer’s and Parkinson’s disease. The confirmation of such diagnosis allows early detection and subsequent medical counseling that help specific patients to undergo clinically important drug trials. This provides a medical pathway to have better insight of neurogenesis and eventual cure of the neurodegenerative diseases. In this short review, we present recent advances in molecular diagnostics especially biomarkers and imaging spectroscopy for neurological diseases. We describe advances made in Alzheimer’s disease, Parkinson’s disease, Amyotrophic lateral sclerosis and Huntington’s disease, and finally present a perspective on the future directions to provide a framework for further developments and refinements of molecular diagnostics to combat neurodegenerative disorders.

  17. Molecular diagnostics of neurodegenerative disorders.

    Science.gov (United States)

    Agrawal, Megha; Biswas, Abhijit

    2015-01-01

    Molecular diagnostics provide a powerful method to detect and diagnose various neurological diseases such as Alzheimer's and Parkinson's disease. The confirmation of such diagnosis allows early detection and subsequent medical counseling that help specific patients to undergo clinically important drug trials. This provides a medical pathway to have better insight of neurogenesis and eventual cure of the neurodegenerative diseases. In this short review, we present recent advances in molecular diagnostics especially biomarkers and imaging spectroscopy for neurological diseases. We describe advances made in Alzheimer's disease (AD), Parkinson's disease (PD), Amyotrophic lateral sclerosis (ALS) and Huntington's disease (HD), and finally present a perspective on the future directions to provide a framework for further developments and refinements of molecular diagnostics to combat neurodegenerative disorders.

  18. Behavior of spent nuclear fuel and storage system components in dry interim storage.

    Energy Technology Data Exchange (ETDEWEB)

    Johnson, A.B. Jr.; Gilbert, E.R.; Guenther, R.J.

    1982-08-01

    Irradiated nuclear fuel has been handled under dry conditions since the early days of nuclear reactor operation, and use of dry storage facilities for extended management of irradiated fuel began in 1964. Irradiated fuel is currently being stored dry in four types of facilities: dry wells, vaults, silos, and metal casks. Essentially all types of irradiated nuclear fuel are currently stored under dry conditions. Gas-cooled reactor (GCR) and liquid metal fast breeder reactor (LMFBR) fuels are stored in vaults and dry wells. Certain types of fuel are being stored in licensed dry storage facilities: Magnox fuel in vaults in the United Kingdom and organic-cooled reactor (OCR) fuel in silos in Canada. Dry storage demonstrations are under way for Zircaloy-clad fuel from boiling water reactors BWR's, pressurized heavy-water reactors (PHWRs), and pressurized water reactors (PWRs) in all four types of dry storage facilities. The demonstrations and related hot cell and laboratory tests are directed toward expanding the data base and establishing a licensing basis for dry storage of water reactor fuel. This report reviews the scope of dry interim storage technology, the performance of fuel and facility materials, the status of programs in several countries to license dry storage of water reactor fuel, and the characteristics of water reactor fuel that relate to dry storage conditions.

  19. Behavior of spent nuclear fuel and storage-system components in dry interim storage

    International Nuclear Information System (INIS)

    Johnson, A.B. Jr.; Gilbert, E.R.; Guenther, R.J.

    1982-08-01

    Irradiated nuclear fuel has been handled under dry conditions since the early days of nuclear reactor operation, and use of dry storage facilities for extended management of irradiated fuel began in 1964. Irradiated fuel is currently being stored dry in four types of facilities: dry wells, vaults, silos, and metal casks. Essentially all types of irradiated nuclear fuel are currently stored under dry conditions. Gas-cooled reactor (GCR) and liquid metal fast breeder reactor (LMFBR) fuels are stored in vaults and dry wells. Certain types of fuel are being stored in licensed dry storage facilities: Magnox fuel in vaults in the United Kingdom and organic-cooled reactor (OCR) fuel in silos in Canada. Dry storage demonstrations are under way for Zircaloy-clad fuel from boiling water reactors BWR's, pressurized heavy-water reactors (PHWRs), and pressurized water reactors (PWRs) in all four types of dry storage facilities. The demonstrations and related hot cell and laboratory tests are directed toward expanding the data base and establishing a licensing basis for dry storage of water reactor fuel. This report reviews the scope of dry interim storage technology, the performance of fuel and facility materials, the status of programs in several countries to license dry storage of water reactor fuel, and the characteristics of water reactor fuel that relate to dry storage conditions

  20. EEG markers of reduced visual short-term memory capacity in adult attention deficit/hyperactivity disorder

    DEFF Research Database (Denmark)

    Wiegand, Iris Michaela; Kilian, Beate; Hennig-Fast, Kristina

    2015-01-01

    Attention deficit hyperactivity disorder (ADHD) persists frequently into adulthood. The disease is associated with difficulties in many cognitive tasks, which are assumed to be caused by neurobiologically-based basal dysfunctions. A reduction in visual working memory storage capacity has recently...... been claimed a testable endophenotype of ADHD. This study aimed at identifying brain abnormalities underlying this deficit by combining parameter-based assessment with electrophysiology. We compared unmedicated adult ADHD patients and demographically matched, healthy controls. We found reduced storage...... capacity in the patient group and delineated neural correlates of the deficit by analyzing ERP amplitudes according to (1) differences between patients and controls and (2) individual’s performance level of storage capacity K: First, the contralateral delay activity (CDA) was higher for individuals...

  1. 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...

  2. Economic analysis of using above ground gas storage devices for compressed air energy storage system

    Science.gov (United States)

    Liu, Jinchao; Zhang, Xinjing; Xu, Yujie; Chen, Zongyan; Chen, Haisheng; Tan, Chunqing

    2014-12-01

    Above ground gas storage devices for compressed air energy storage (CAES) have three types: air storage tanks, gas cylinders, and gas storage pipelines. A cost model of these gas storage devices is established on the basis of whole life cycle cost (LCC) analysis. The optimum parameters of the three types are determined by calculating the theoretical metallic raw material consumption of these three devices and considering the difficulties in manufacture and the influence of gas storage device number. The LCCs of the three types are comprehensively analyzed and compared. The result reveal that the cost of the gas storage pipeline type is lower than that of the other two types. This study may serve as a reference for designing large-scale CAES systems.

  3. Large-scale electrophysiology: acquisition, compression, encryption, and storage of big data.

    Science.gov (United States)

    Brinkmann, Benjamin H; Bower, Mark R; Stengel, Keith A; Worrell, Gregory A; Stead, Matt

    2009-05-30

    The use of large-scale electrophysiology to obtain high spatiotemporal resolution brain recordings (>100 channels) capable of probing the range of neural activity from local field potential oscillations to single-neuron action potentials presents new challenges for data acquisition, storage, and analysis. Our group is currently performing continuous, long-term electrophysiological recordings in human subjects undergoing evaluation for epilepsy surgery using hybrid intracranial electrodes composed of up to 320 micro- and clinical macroelectrode arrays. DC-capable amplifiers, sampling at 32kHz per channel with 18-bits of A/D resolution are capable of resolving extracellular voltages spanning single-neuron action potentials, high frequency oscillations, and high amplitude ultra-slow activity, but this approach generates 3 terabytes of data per day (at 4 bytes per sample) using current data formats. Data compression can provide several practical benefits, but only if data can be compressed and appended to files in real-time in a format that allows random access to data segments of varying size. Here we describe a state-of-the-art, scalable, electrophysiology platform designed for acquisition, compression, encryption, and storage of large-scale data. Data are stored in a file format that incorporates lossless data compression using range-encoded differences, a 32-bit cyclically redundant checksum to ensure data integrity, and 128-bit encryption for protection of patient information.

  4. International long-term interim storage for spent fuel. An independent storage service investor model

    International Nuclear Information System (INIS)

    Leister, P.

    1999-01-01

    Thinking globally the obvious world-wide demands for large storage capacities for spent fuel within the next decades and the newly arising demands for long-term interim storage of spent fuel urges to respond by international interim storage facilities of high capacity. Low cost storage can be achieved only by arranging the storage facility underground in a suitable host rock formation and by selecting the geographical are by an international competition under those countries, who are willing to offer their land. The investor and operator of an international storage facility selected and realised by a competition on the free market as well as the country where the storage is built are both bound by two different kinds of contacts. The main contract is between the offering country/region and the independent operator. The independent operator has in addition a series of contracts with various utilities, which are interested to have their spent fuel stored for a longer period

  5. Carbon dioxide storage. EU legal framework for carbon capture and storage

    International Nuclear Information System (INIS)

    Heller, W.

    2008-01-01

    In the correct opinion of the EU Commission, fossil fuels are going to remain the most important energy source worldwide also in the decades to come. The intention of the EU to reduce by 50% the 1990 level of greenhouse gas emission by 2050 can become reality, in the light of worldwide developments, only if the energy potential of coal can be tapped without multiplying emissions. The EU therefore initiated measures to make carbon capture and storage a standard technology in new fossil fired power plants. The CCS technology is to be demonstrated so as to make it available commercially for plant renewal after 2020 (CCS = Carbon Capture and Storage). To outline the future legal framework in the European Union, the EU Commission on January 23, 2008 presented the proposal of a Directive on Geologic Storage of Carbon Dioxide (CO 2 ). That proposal mainly focuses on the storage of CO 2 and the removal of obstacles in the way of CO 2 storage. The capture and pipeline transport of CO 2 are taken into account in the appropriate amendments to existing directives. (orig.)

  6. 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.)

  7. 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...

  8. Performance of high-resolution position-sensitive detectors developed for storage-ring decay experiments

    International Nuclear Information System (INIS)

    Yamaguchi, T.; Suzaki, F.; Izumikawa, T.; Miyazawa, S.; Morimoto, K.; Suzuki, T.; Tokanai, F.; Furuki, H.; Ichihashi, N.; Ichikawa, C.; Kitagawa, A.; Kuboki, T.; Momota, S.; Nagae, D.; Nagashima, M.; Nakamura, Y.; Nishikiori, R.; Niwa, T.; Ohtsubo, T.; Ozawa, A.

    2013-01-01

    Highlights: • Position-sensitive detectors were developed for storage-ring decay spectroscopy. • Fiber scintillation and silicon strip detectors were tested with heavy ion beams. • A new fiber scintillation detector showed an excellent position resolution. • Position and energy detection by silicon strip detectors enable full identification. -- Abstract: As next generation spectroscopic tools, heavy-ion cooler storage rings will be a unique application of highly charged RI beam experiments. Decay spectroscopy of highly charged rare isotopes provides us important information relevant to the stellar conditions, such as for the s- and r-process nucleosynthesis. In-ring decay products of highly charged RI will be momentum-analyzed and reach a position-sensitive detector set-up located outside of the storage orbit. To realize such in-ring decay experiments, we have developed and tested two types of high-resolution position-sensitive detectors: silicon strips and scintillating fibers. The beam test experiments resulted in excellent position resolutions for both detectors, which will be available for future storage-ring experiments

  9. Beyond peak reservoir storage? A global estimate of declining water storage capacity in large reservoirs

    NARCIS (Netherlands)

    Wisser, D.; Frolking, S.; Hagen, Stephen; Bierkens, M.F.P.|info:eu-repo/dai/nl/125022794

    2013-01-01

    Water storage is an important way to cope with temporal variation in water supply anddemand. The storage capacity and the lifetime of water storage reservoirs can besignificantly reduced by the inflow of sediments. A global, spatially explicit assessment ofreservoir storage loss in conjunction with

  10. CFD research on runaway transient of pumped storage power station caused by pumping power failure

    International Nuclear Information System (INIS)

    Zhang, L G; Zhou, D Q

    2013-01-01

    To study runaway transient of pumped storage power station caused by pumping power failure, three dimensional unsteady numerical simulations were executed on geometrical model of the whole flow system. Through numerical calculation, the changeable flow configuration and variation law of some parameters such as unit rotate speed,flow rate and static pressure of measurement points were obtained and compared with experimental data. Numerical results show that runaway speed agrees well with experimental date and its error was 3.7%. The unit undergoes pump condition, brake condition, turbine condition and runaway condition with flow characteristic changing violently. In runaway condition, static pressure in passage pulses very strongly which frequency is related to runaway speed

  11. 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)

  12. 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

  13. Working memory deficits in high-functioning adolescents with autism spectrum disorders: neurpsychological and neuroimaging correlates

    NARCIS (Netherlands)

    Barendse, E.M.; Hendriks, M.P.H.; Jansen, J.F.A.; Backes, W.H.; Hofman, P.A.M.; Thoonen, G.; Kessels, R.P.; Aldenkamp, A.P.

    2013-01-01

    Working memory is a temporary storage system under attentional control. It is believed to play a central role in online processing of complex cognitive information and may also play a role in social cognition and interpersonal interactions. Adolescents with a disorder on the autism spectrum display

  14. Working memory deficits in high-functioning adolescents with autism spectrum disorders: neuropsychological and neuroimaging correlates

    NARCIS (Netherlands)

    Barendse, E.M.; Hendriks, M.P.H.; Jansen, J.F.A.; Backes, W.H.; Hofman, P.A.M.; Thoonen, G.H.J.; Kessels, R.P.C.; Aldenkamp, A.P.

    2013-01-01

    Working memory is a temporary storage system under attentional control. It is believed to play a central role in online processing of complex cognitive information and may also play a role in social cognition and interpersonal interactions. Adolescents with a disorder on the autism spectrum display

  15. Considerations for Disposition of Dry Cask Storage System Materials at End of Storage System Life

    International Nuclear Information System (INIS)

    Howard, Rob; Van den Akker, Bret

    2014-01-01

    Dry cask storage systems are deployed at nuclear power plants for used nuclear fuel (UNF) storage when spent fuel pools reach their storage capacity and/or the plants are decommissioned. An important waste and materials disposition consideration arising from the increasing use of these systems is the management of the dry cask storage systems' materials after the UNF proceeds to disposition. Thermal analyses of repository design concepts currently under consideration internationally indicate that waste package sizes for the geologic media under consideration may be significantly smaller than the canisters being used for on-site dry storage by the nuclear utilities. Therefore, at some point along the UNF disposition pathway, there could be a need to repackage fuel assemblies already loaded into the dry storage canisters currently in use. In the United States, there are already over 1650 of these dry storage canisters deployed and approximately 200 canisters per year are being loaded at the current fleet of commercial nuclear power plants. There is about 10 cubic meters of material from each dry storage canister system that will need to be dispositioned. The concrete horizontal storage modules or vertical storage overpacks will need to be reused, re-purposed, recycled, or disposed of in some manner. The empty metal storage canister/cask would also have to be cleaned, and decontaminated for possible reuse or recycling or disposed of, likely as low-level radioactive waste. These material disposition options can have impacts of the overall used fuel management system costs. This paper will identify and explore some of the technical and interface considerations associated with managing the dry cask storage system materials. (authors)

  16. Rock cavern storage of spent fuel

    Energy Technology Data Exchange (ETDEWEB)

    Cho, Won Jin; Kim, Kyung Soo [Korea Atomic Energy Research Institute, Daejeon (Korea, Republic of); Kwon, Sang Ki [Inha University, Incheon (Korea, Republic of)

    2015-12-15

    The rock cavern storage for spent fuel has been assessed to apply in Korea with reviewing the state of the art of the technologies for surface storage and rock cavern storage of spent fuel. The technical feasibility and economic aspects of the rock cavern storage of spent fuel were also analyzed. A considerable area of flat land isolated from the exterior are needed to meet the requirement for the site of the surface storage facilities. It may, however, not be easy to secure such areas in the mountainous region of Korea. Instead, the spent fuel storage facilities constructed in the rock cavern moderate their demands for the suitable site. As a result, the rock cavern storage is a promising alternative for the storage of spent fuel in the aspect of natural and social environments. The rock cavern storage of spent fuel has several advantages compared with the surface storage, and there is no significant difference on the viewpoint of economy between the two alternatives. In addition, no great technical difficulties are present to apply the rock cavern storage technologies to the storage of domestic spent fuel.

  17. Advantages of dry hardened cask storage over wet storage for spent nuclear fuel

    Energy Technology Data Exchange (ETDEWEB)

    Romanato, Luiz Sergio, E-mail: romanato@ctmsp.mar.mil.b [Centro Tecnologico da Marinha em Sao Paulo (CTMSP), Sao Paulo, SP (Brazil). Dept. da Qualidade

    2011-07-01

    Pools are generally used to store and maintain spent nuclear fuel assemblies for cooling, after removed from reactors. After three to five years stored in the pools, spent fuel can be reprocessed or sent to a final disposition in a geological repository and handled as radioactive waste or sent to another site waiting for future solution. Spent fuel can be stored in dry or wet installations, depending on the method adopted by the nuclear plant. If this storage were exclusively wet, at the installation decommissioning in the future, another solution for storage will need to be found. Today, after a preliminary cooling, the spent fuel assemblies can be removed from the pool and sent to dry hardened storage installations. This kind of storage does not need complex radiation monitoring and it is safer than wet storage. Brazil has two nuclear reactors in operation, a third reactor is under construction and they use wet spent fuel storage . Dry hardened casks use metal or both metal and concrete for radiation shielding and they are safe, especially during an earthquake. An earthquake struck Japan on March 11, 2011 damaging Fukushima Daiichi nuclear power plant. The occurrence of earthquakes in Brazil is very small but dry casks can resist to other events, including terrorist acts, better than pools. This paper shows the advantages of dry hardened cask storage in comparison with the wet storage (water pools) for spent nuclear fuel. (author)

  18. Advantages of dry hardened cask storage over wet storage for spent nuclear fuel

    International Nuclear Information System (INIS)

    Romanato, Luiz Sergio

    2011-01-01

    Pools are generally used to store and maintain spent nuclear fuel assemblies for cooling, after removed from reactors. After three to five years stored in the pools, spent fuel can be reprocessed or sent to a final disposition in a geological repository and handled as radioactive waste or sent to another site waiting for future solution. Spent fuel can be stored in dry or wet installations, depending on the method adopted by the nuclear plant. If this storage were exclusively wet, at the installation decommissioning in the future, another solution for storage will need to be found. Today, after a preliminary cooling, the spent fuel assemblies can be removed from the pool and sent to dry hardened storage installations. This kind of storage does not need complex radiation monitoring and it is safer than wet storage. Brazil has two nuclear reactors in operation, a third reactor is under construction and they use wet spent fuel storage . Dry hardened casks use metal or both metal and concrete for radiation shielding and they are safe, especially during an earthquake. An earthquake struck Japan on March 11, 2011 damaging Fukushima Daiichi nuclear power plant. The occurrence of earthquakes in Brazil is very small but dry casks can resist to other events, including terrorist acts, better than pools. This paper shows the advantages of dry hardened cask storage in comparison with the wet storage (water pools) for spent nuclear fuel. (author)

  19. Aripiprazole plus topiramate in opioid-dependent patients with schizoaffective disorder: an 8-week, open-label, uncontrolled, preliminary study.

    Science.gov (United States)

    Bruno, Antonio; Romeo, Vincenzo M; Pandolfo, Gianluca; Scimeca, Giuseppe; Zoccali, Rocco A; Muscatello, Maria Rosaria A

    2014-01-01

    The aims of this study were to evaluate a combination of aripiprazole and topiramate in the treatment of opioid-dependent patients with schizoaffective disorder undergoing methadone maintenance therapy (MMT) and, further, to taper off patients from methadone treatment. Twenty patients who met DSM-IV (Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition) criteria for opioid dependence and schizoaffective disorder receiving MMT (80 mg/day) were given aripiprazole (10 mg/day) plus topiramate (up to 200 mg/day) for 8 weeks. A methadone dose reduction of 3 mg/day until suspension at week 4 was established. Aripiprazole plus topiramate was effective in reducing clinical symptoms, and a rapid tapering off of MMT was achieved. Combining aripiprazole and topiramate may be effective in patients with a dual diagnosis of opioid dependency and schizoaffective disorder.

  20. Interim storage of radioactive waste packages

    International Nuclear Information System (INIS)

    1998-01-01

    This report covers all the principal aspects of production and interim storage of radioactive waste packages. The latest design solutions of waste storage facilities and the operational experiences of developed countries are described and evaluated in order to assist developing Member States in decision making and design and construction of their own storage facilities. This report is applicable to any category of radioactive waste package prepared for interim storage, including conditioned spent fuel, high level waste and sealed radiation sources. This report addresses the following issues: safety principles and requirements for storage of waste packages; treatment and conditioning methods for the main categories of radioactive waste; examples of existing interim storage facilities for LILW, spent fuel and high level waste; operational experience of Member States in waste storage operations including control of storage conditions, surveillance of waste packages and observation of the behaviour of waste packages during storage; retrieval of waste packages from storage facilities; technical and administrative measures that will ensure optimal performance of waste packages subject to various periods of interim storage

  1. Electric energy storage - Overview of technologies

    International Nuclear Information System (INIS)

    Boye, Henri

    2013-01-01

    Energy storage is a challenging and costly process, as electricity can only be stored by conversion into other forms of energy (e.g. potential, thermal, chemical or magnetic energy). The grids must be precisely balanced in real time and it must be made sure that the cost of electricity is the lowest possible. Storage of electricity has many advantages, in centralized mass storages used for the management of the transmission network, or in decentralized storages of smaller dimensions. This article presents an overview of the storage technologies: mechanical storage in hydroelectric and pumped storage power stations, compressed air energy storage (CAES), flywheels accumulating kinetic energy, electrochemical batteries with various technologies, traditional lead acid batteries, lithium ion, sodium sulfur (NaS) and others, including vehicle to grid, sensible heat thermal storage, superconducting magnetic energy storage (SMES), super-capacitors, conversion into hydrogen... The different technologies are compared in terms of cost and level of maturity. The development of intermittent renewable energies will result in a growing need for mechanisms to regulate energy flow and innovative energy storage solutions seem well positioned to develop. (author)

  2. Common management issues in pediatric patients with mild bleeding disorders.

    Science.gov (United States)

    O'Brien, Sarah H

    2012-10-01

    Type 1 von Willebrand disease and mild platelet function defects are among the most common disorders seen by pediatric hematologists. The management and prevention of bleeding in these patients can be challenging, as there are limited published data to guide clinical practice, and a complete lack of randomized clinical trials. Desmopressin (DDAVP) and antifibrinolytics are the mainstays of treatment in these patients, yet the optimal dosing and timing of these agents to prevent or resolve bleeding, while minimizing adverse side effects, is sometimes unclear. DDAVP-induced hyponatremia is a particularly under-recognized complication in children with bleeding disorders who undergo surgery. Clinicians need to be aware of local measures that are equally important in treating problems such as epistaxis and surgical bleeding. This review will discuss the published literature and provide practical suggestions regarding four common management issues in the care of children and adolescents with mild bleeding disorders: epistaxis, heavy menstrual bleeding, dental extractions, and tonsillectomy. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

  3. Energy storage systems cost update : a study for the DOE Energy Storage Systems Program.

    Energy Technology Data Exchange (ETDEWEB)

    Schoenung, Susan M. (Longitude 122 West, Menlo Park, CA)

    2011-04-01

    This paper reports the methodology for calculating present worth of system and operating costs for a number of energy storage technologies for representative electric utility applications. The values are an update from earlier reports, categorized by application use parameters. This work presents an update of energy storage system costs assessed previously and separately by the U.S. Department of Energy (DOE) Energy Storage Systems Program. The primary objective of the series of studies has been to express electricity storage benefits and costs using consistent assumptions, so that helpful benefit/cost comparisons can be made. Costs of energy storage systems depend not only on the type of technology, but also on the planned operation and especially the hours of storage needed. Calculating the present worth of life-cycle costs makes it possible to compare benefit values estimated on the same basis.

  4. 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

  5. Chemical-clathrate hybrid hydrogen storage: storage in both guest and host.

    Science.gov (United States)

    Strobel, Timothy A; Kim, Yongkwan; Andrews, Gary S; Ferrell, Jack R; Koh, Carolyn A; Herring, Andrew M; Sloan, E Dendy

    2008-11-12

    Hydrogen storage from two independent sources of the same material represents a novel approach to the hydrogen storage problem, yielding storage capacities greater than either of the individual constituents. Here we report a novel hydrogen storage scheme in which recoverable hydrogen is stored molecularly within clathrate cavities as well as chemically in the clathrate host material. X-ray diffraction and Raman spectroscopic measurements confirm the formation of beta-hydroquinone (beta-HQ) clathrate with molecular hydrogen. Hydrogen within the beta-HQ clathrate vibrates at considerably lower frequency than hydrogen in the free gaseous phase and rotates nondegenerately with splitting comparable to the rotational constant. Compared with water-based clathrate hydrate phases, the beta-HQ+H2 clathrate shows remarkable stability over a range of p-T conditions. Subsequent to clathrate decomposition, the host HQ was used to directly power a PEM fuel cell. With one H2 molecule per cavity, 0.61 wt % hydrogen may be stored in the beta-HQ clathrate cavities. When this amount is combined with complete dehydrogenation of the host hydroxyl hydrogens, the maximum hydrogen storage capacity increases nearly 300% to 2.43 wt %.

  6. 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.

  7. 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

  8. 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

  9. Prevalence of Body Dysmorphic Disorder Among Patients Seeking Breast Reconstruction.

    Science.gov (United States)

    Metcalfe, Drew B; Duggal, Claire S; Gabriel, Allen; Nahabedian, Maurice Y; Carlson, Grant W; Losken, Albert

    2014-07-01

    Body dysmorphic disorder (BDD) is characterized by a preoccupation with a slight or imagined defect in physical appearance. It has significant implications for patients who desire breast reconstruction, because patient satisfaction with the aesthetic outcome is a substantial contributor to the success of the procedure. The authors estimated the prevalence of BDD among women seeking breast reconstruction by surveying patients with the previously validated Dysmorphic Concerns Questionnaire (DCQ). One hundred eighty-eight women who presented for immediate or delayed breast reconstruction completed the DCQ anonymously, during initial consultation with a plastic surgeon. Two groups of respondents were identified: those who desired immediate reconstruction and those who planned to undergo delayed reconstruction. The prevalence of BDD among breast reconstruction patients was compared between the 2 groups, and the overall prevalence was compared with published rates for the general public. Body dysmorphic disorder was significantly more prevalent in breast reconstruction patients than in the general population (17% vs 2%; P < .001). It also was much more common among patients who planned to undergo delayed (vs immediate) reconstruction (34% vs 13%; P = .004). Relative to the general public, significantly more women who sought breast reconstruction were diagnosed as having BDD. Awareness of the potential for BDD will enable clinicians to better understand their patients' perspectives and discuss realistic expectations at the initial consultation. Future studies are warranted to examine the implications of BDD on patient satisfaction with reconstructive surgery. 3. © 2014 The American Society for Aesthetic Plastic Surgery, Inc.

  10. Economics of Energy Storage. An analysis of the administrative consequences of electricity storage

    International Nuclear Information System (INIS)

    Wals, A.F.; Hendriks, R.H.

    2004-03-01

    This report discusses the administrative aspects connected to the introduction of electricity storage in the energy system. First, the macro-economic aspects of utilizing storage facilities are discussed, and the possible benefits of storage in the electricity system are summarized. Next, the discussion focuses on the administrative aspects. In particular, the regulation system of the Dutch electricity market is reviewed, paying particular attention to the market design in connection with Distribution Network Operators. A number of relevant aspects are discussed, such as the incentives for the Operators to optimize network performance, as well as the means available to the Operators to stimulate third parties to do so. Finally, the perspectives for storage operators to enter directly on the different power markets are treated. Generally, one can conclude that the administrative aspects for storage facilities leave room for improvement

  11. The Impact of Mood and Anxiety Disorders on Incident Hypertension at One Year

    Directory of Open Access Journals (Sweden)

    Simon L. Bacon

    2014-01-01

    Full Text Available Background. Studies assessing the association between psychological factors and hypertension have been equivocal, which may reflect limitations in the assessment of psychological factors. Purpose. To assess the relationship between mood and anxiety disorders, measured using a psychiatric interview, and 1-year incident hypertension. Methods. 197 nonhypertensive individuals undergoing exercise stress testing at baseline provided follow-up data at 1 year. Baseline assessments included a structure psychiatric interview (PRIME-MD, physician diagnosis of hypertension, and measured blood pressure. At follow-up, hypertension status was assessed via self-reported physician diagnosis. Results. Having an anxiety disorder was associated with a 4-fold increase in the risk of developing hypertension (adjusted OR = 4.14, 95% CIs = 1.18–14.56. In contrast, having a mood disorder was not associated with incident hypertension (adjusted OR = 1.21, 95% CIs = 0.24–5.86. Conclusions. There are potential mechanisms which could explain our differential mood and anxiety findings. The impact of screening and treatment of anxiety disorders on hypertension needs to be explored.

  12. 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

  13. Physicochemical changes of 'Phulae' pineapple fruit treated with short-term anoxia during ambient storage.

    Science.gov (United States)

    Techavuthiporn, Chairat; Boonyaritthongchai, Panida; Supabvanich, Suriyan

    2017-08-01

    The effects of short-term anoxia exposure for 16h on physicochemical changes of 'Phulae' pineapple fruit stored at ambient temperature (25±2°C) were investigated. The respiratory rate of the fruit was induced by the anoxia treatment. However, it retarded the increase in moisture loss and maintained both flesh and pulp colour by inhibiting polyphenol oxidase (PPO) activity of the both tissues. The anoxia exposure delayed the increase in total sugar content and enhanced total ascorbic acid content during storage. The half-cut pineapple fruit showed that the anoxia exposure completely inhibited internal transparency of the flesh tissue adjacent to core during the storage. In conclusion, the short-term anoxia exposure for 16h maintained postharvest quality, retarded physiological disorder and enhanced nutritional values of the pineapple fruit stored at ambient temperature (25±2°C). Copyright © 2017 Elsevier Ltd. All rights reserved.

  14. Altered Development of White Matter in Youth at High Familial Risk for Bipolar Disorder: A Diffusion Tensor Imaging Study

    Science.gov (United States)

    Versace, Amelia; Ladouceur, Cecile D.; Romero, Soledad; Birmaher, Boris; Axelson, David A.; Kupfer, David J.; Phillips, Mary L.

    2010-01-01

    Objective: To study white matter (WM) development in youth at high familial risk for bipolar disorder (BD). WM alterations are reported in youth and adults with BD. WM undergoes important maturational changes in adolescence. Age-related changes in WM microstructure using diffusion tensor imaging with tract-based spatial statistics in healthy…

  15. 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

  16. Developing semi-analytical solution for multiple-zone transient storage model with spatially non-uniform storage

    Science.gov (United States)

    Deng, Baoqing; Si, Yinbing; Wang, Jia

    2017-12-01

    Transient storages may vary along the stream due to stream hydraulic conditions and the characteristics of storage. Analytical solutions of transient storage models in literature didn't cover the spatially non-uniform storage. A novel integral transform strategy is presented that simultaneously performs integral transforms to the concentrations in the stream and in storage zones by using the single set of eigenfunctions derived from the advection-diffusion equation of the stream. The semi-analytical solution of the multiple-zone transient storage model with the spatially non-uniform storage is obtained by applying the generalized integral transform technique to all partial differential equations in the multiple-zone transient storage model. The derived semi-analytical solution is validated against the field data in literature. Good agreement between the computed data and the field data is obtained. Some illustrative examples are formulated to demonstrate the applications of the present solution. It is shown that solute transport can be greatly affected by the variation of mass exchange coefficient and the ratio of cross-sectional areas. When the ratio of cross-sectional areas is big or the mass exchange coefficient is small, more reaches are recommended to calibrate the parameter.

  17. Towards solar energy storage in the photochromic dihydroazulene-vinylheptafulvene system.

    Science.gov (United States)

    Cacciarini, Martina; Skov, Anders B; Jevric, Martyn; Hansen, Anne S; Elm, Jonas; Kjaergaard, Henrik G; Mikkelsen, Kurt V; Brøndsted Nielsen, Mogens

    2015-05-11

    One key challenge in the field of exploitation of solar energy is to store the energy and make it available on demand. One possibility is to use photochromic molecules that undergo light-induced isomerization to metastable isomers. Here we present efforts to develop solar thermal energy storage systems based on the dihydroazulene (DHA)/vinylheptafulvene (VHF) photo/thermoswitch. New DHA derivatives with one electron-withdrawing cyano group at position 1 and one or two phenyl substituents in the five-membered ring were prepared by using different synthetic routes. In particular, a diastereoselective reductive removal of one cyano group from DHAs incorporating two cyano groups at position 1 turned out to be most effective. Quantum chemical calculations reveal that the structural modifications provide two benefits relative to DHAs with two cyano groups at position 1: 1) The DHA-VHF energy difference is increased (i.e., higher energy capacity of metastable VHF isomer); 2) the Gibbs free energy of activation is increased for the energy-releasing VHF to DHA back-reaction. In fact, experimentally, these new derivatives were so reluctant to undergo the back-reaction at room temperature that they practically behaved as DHA to VHF one-way switches. Although lifetimes of years are at first attractive, which offers the ultimate control of energy release, for a real device it must of course be possible to trigger the back-reaction, which calls for further iterations in the future. © 2015 WILEY-VCH Verlag GmbH & Co. KGaA, Weinheim.

  18. Costing of spent nuclear fuel storage

    International Nuclear Information System (INIS)

    2009-01-01

    This report deals with economic analysis and cost estimation, based on exploration of relevant issues, including a survey of analytical tools for assessment and updated information on the market and financial issues associated with spent fuel storage. The development of new storage technologies and changes in some of the circumstances affecting the costs of spent fuel storage are also incorporated. This report aims to provide comprehensive information on spent fuel storage costs to engineers and nuclear professionals as well as other stakeholders in the nuclear industry. This report is meant to provide informative guidance on economic aspects involved in selecting a spent fuel storage system, including basic methods of analysis and cost data for project evaluation and comparison of storage options, together with financial and business aspects associated with spent fuel storage. After the review of technical options for spent fuel storage in Section 2, cost categories and components involved in the lifecycle of a storage facility are identified in Section 3 and factors affecting costs of spent fuel storage are then reviewed in the Section 4. Methods for cost estimation and analysis are introduced in Section 5, and other financial and business aspects associated with spent fuel storage are discussed in Section 6.

  19. 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

  20. 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...

  1. 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 ...

  2. 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

  3. Solar applications analysis for energy storage

    Science.gov (United States)

    Blanchard, T.

    1980-01-01

    The role of energy storage as it relates to solar energy systems is considered. Storage technologies to support solar energy applications, the status of storage technologies, requirements and specifications for storage technologies, and the adequacy of the current storage research and development program to meet these requirements are among the factors discussed. Emphasis is placed on identification of where the greatest potential exists for energy storage in support of those solar energy systems which could have a significant impact on the U.S. energy mix.

  4. Yield, utilization, storage and ultimate storage of depleted uranium

    International Nuclear Information System (INIS)

    Aumueller, L.; Hermann, J.

    1977-11-01

    More than 80% of the uranium leaving uranium enrichment plants is depleted to a residual content of about 0,25% U 235. Due to the present ineconomical further depletion to the technically possible residual content of 0,1% U 235, the so-called 'tails' are first of all stored. The quantity of stored depleted uranium in the FRG should be about 100.000 t by the year 2000. It represents a strategic reserve for future energy supply regardless of profitableness. The study analysis the conceivable possible uses for the tails quantity considered. These are, besides further depletion whose profitableness is considered, also the use as breeder material in breeder reactors and the use in the non-nuclear field. The main part of the study deals with the various storage possibilities of the depleted uranium in oxidic or fluoride form. A comparison of costs of alternative storage concepts showed a clear advantage for the storage of UF 6 in 48 inch containers already in use. The conceivable accidents in storing are analyzed and measures to reduce the consequences are discussed. Finally, the problems of ultimate storage for the remaining waste after further depletion or use are investigated and the costs arising here are also estimated. (RB) [de

  5. Inherent lipid metabolic dysfunction in glycogen storage disease IIIa.

    Science.gov (United States)

    Li, Xin-Hua; Gong, Qi-Ming; Ling, Yun; Huang, Chong; Yu, De-Min; Gu, Lei-Lei; Liao, Xiang-Wei; Zhang, Dong-Hua; Hu, Xi-Qi; Han, Yue; Kong, Xiao-Fei; Zhang, Xin-Xin

    2014-12-05

    We studied two patients from a nonconsanguineous family with life-long abnormal liver function, hepatomegaly and abnormal fatty acid profiles. Abnormal liver function, hypoglycemia and muscle weakness are observed in various genetic diseases, including medium-chain acyl-CoA dehydrogenase (MCAD) deficiency and glycogen storage diseases. The proband showed increased free fatty acids, mainly C8 and C10, resembling fatty acid oxidation disorder. However, no mutation was found in ACADM and ACADL gene. Sequencing of theamylo-alpha-1, 6-glucosidase, 4-alpha-glucanotransferase (AGL) gene showed that both patients were compound heterozygotes for c.118C > T (p.Gln40X) and c.753_756 del CAGA (p.Asp251Glufsx29), whereas their parents were each heterozygous for one of these mutations. The AGL protein was undetectable in EBV-B cells from the two patients. Transcriptome analysis demonstrated a significant different pattern of gene expression in both of patients’ cells, including genes involving in the PPAR signaling pathway, fatty acid biosynthesis, lipid synthesis and visceral fat deposition and metabolic syndrome. This unique gene expression pattern is probably due to the absence of AGL, which potentially accounts for the observed clinical phenotypes of hyperlipidemia and hepatocyte steatosis in glycogen storage disease type IIIa.

  6. Photoswitchable Dihydroazulene Macrocycles for Solar Energy Storage: The Effects of Ring Strain.

    Science.gov (United States)

    Vlasceanu, Alexandru; Frandsen, Benjamin N; Skov, Anders B; Hansen, Anne Schou; Rasmussen, Mads Georg; Kjaergaard, Henrik G; Mikkelsen, Kurt V; Nielsen, Mogens Brøndsted

    2017-10-06

    Efficient energy storage and release are two major challenges of solar energy harvesting technologies. The development of molecular solar thermal systems presents one approach to address these issues by tuning the isomerization reactions of photo/thermoswitches. Here we show that the incorporation of photoswitches into macrocyclic structures is a particularly attractive solution for increasing the storage time. We present the synthesis and properties of a series of macrocycles incorporating two dihydroazulene (DHA) photoswitching subunits, bridged by linkers of varying chain length. Independent of ring size, all macrocycles exhibit stepwise, light-induced, ring-opening reactions (DHA-DHA to DHA-VHF to VHF-VHF; VHF = vinylheptafulvene) with the first DHA undergoing isomerization with a similar efficiency as the uncyclized parent system while the second (DHA-VHF to VHF-VHF) is significantly slower. The energy-releasing, VHF-to-DHA, ring closures also occur in a stepwise manner and are systematically found to proceed slower in the more strained (smaller) cycles, but in all cases with a remarkably slow conversion of the second VHF to DHA. We managed to increase the half-life of the second VHF-to-DHA conversion from 65 to 202 h at room temperature by simply decreasing the ring size. A computational study reveals the smallest macrocycle to have the most energetic VHF-VHF state and hence highest energy density.

  7. Storage fee analysis for a retrievable surface storage facility

    International Nuclear Information System (INIS)

    Field, B.B.; Rosnick, C.K.

    1973-12-01

    Conceptual design studies are in progress for a Water Basin Concept (WBC) and an alternative Sealed Storage Cask Concept (SSCC) of a Retrievable Surface Storage Facility (RSSF) intended as a Federal government facility for storing high-level radioactive wastes until a permanent disposal method is established. The RSSF will be a man-made facility with a design life of at least 100 y, and will have capacity to store all of the high-level waste from the reprocessing of nuclear power plant spent fuels generated by the industry through the year 2000. This report is a basic version of ARH-2746, ''Retrievable Surface Storage Facility, Water Basin Concept, User Charge Analysis.'' It is concerned with the issue of establishing a fee to cover the cost of storing nuclear wastes both in the RSSF and at the subsequent disposal facility. (U.S.)

  8. Cytochrome oxidase as an indicator of ice storage and frozen storage

    DEFF Research Database (Denmark)

    Godiksen, Helene; Jessen, Flemming

    2001-01-01

    in different cods was 21%, and the coefficient of variation of different analyses on the same homogenate was 5%. It was shown that ice storage of muscle samples before they were frozen and thawed resulted in a major freezing-induced activation of cytochrome oxidase activity. The enzyme may therefore be used...... as an indicator of frozen fish to determine if the fish has been stored on ice before freezing. Cytochrome oxidase activity showed also potential as an indicator of frozen storage, as it was possible to distinguish between the frozen storage temperatures -9, -20, and -40 degreesC....

  9. 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.

  10. 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.

  11. 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.

  12. 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.

  13. 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.

  14. 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

  15. 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...

  16. 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.

  17. 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

  18. 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

  19. 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

  20. Nonopioid substance use disorders and opioid dose predict therapeutic opioid addiction.

    Science.gov (United States)

    Huffman, Kelly L; Shella, Elizabeth R; Sweis, Giries; Griffith, Sandra D; Scheman, Judith; Covington, Edward C

    2015-02-01

    Limited research examines the risk of therapeutic opioid addiction (TOA) in patients with chronic noncancer pain. This study examined TOA among 199 patients undergoing long-term opioid therapy at the time of admission to a pain rehabilitation program. It was hypothesized that nonopioid substance use disorders and opioid dosage would predict TOA. Daily mean opioid dose was 132.85 mg ± 175.39. Patients with nonopioid substance use disorders had 28 times the odds (odds ratio [OR] = 28.58; 95% confidence interval [CI] = 10.86, 75.27) of having TOA. Each 50-mg increase in opioid dose nearly doubled the odds of TOA (OR = 1.73; 95% CI = 1.29, 2.32). A 100-mg increase was associated with a 3-fold increase in odds (OR = 3.00; 95% CI = 1.67, 5.41). Receiver operating characteristic analysis revealed that opioid dose was a moderately accurate predictor (area under the curve = .75; 95% CI = .68, .82) of TOA. The sensitivity (.70) and specificity (.68) of opioid dose in predicting TOA was maximized at 76.10 mg; in addition, 46.00 mg yielded 80% sensitivity in identifying TOA. These results underscore the importance of obtaining a substance use history prior to prescribing and suggest a low screening threshold for TOA in patients who use opioids in the absence of improvement in pain or functional impairment. This article examines TOA in patients with chronic noncancer pain undergoing long-term opioid therapy. Results suggest that patients should be screened for nonopioid substance use disorders prior to prescribing. In the absence of improvement in pain or function, there is a low threshold (∼50 mg daily opioid dose) for addiction screening. Copyright © 2015 American Pain Society. Published by Elsevier Inc. All rights reserved.

  1. Investigating the motives of patients with knee OA undergoing a TKR

    DEFF Research Database (Denmark)

    Traumer, Line; Sørensen, Erik Elgaard; Kusk, Kathrine Hoffmann

    2018-01-01

    , a qualitatively assessment of what actually affects the patient's decision to undergo TKR would be important. Therefore, the purpose of this study was to investigate the motives of patients with knee OA choosing to undergo TKR and to explore the factors considered important during their decision?making process...... was a legitimate argument among patients. The prospect of avoiding taking high doses of pain?relieving medication and hearing positive experiences of others who had undergone TKR motivated patients to undergo TKR. However, negative experiences of others were neglected. Conclusions Patients' decision?making process...... revealed that the interaction between doctor and patient emerged as a factor highly influential on patients' decision?making process with patients choosing the treatment option suggested by the doctor. Using x?rays, revealing no cartilage between the bones, as a reason for choosing to undergo TKR...

  2. Coping strategies used by hospitalized children with cancer undergoing chemotherapy.

    Science.gov (United States)

    Sposito, Amanda Mota Pacciulio; Silva-Rodrigues, Fernanda Machado; Sparapani, Valéria de Cássia; Pfeifer, Luzia Iara; de Lima, Regina Aparecida Garcia; Nascimento, Lucila Castanheira

    2015-03-01

    To analyze coping strategies used by children with cancer undergoing chemotherapy during hospitalization. This was an exploratory study to analyze qualitative data using an inductive thematic analysis. Semistructured interviews using puppets were conducted with 10 children with cancer, between 7 and 12 years old, who were hospitalized and undergoing chemotherapy. The coping strategies to deal with chemotherapy were: understanding the need for chemotherapy; finding relief for the chemotherapy's side effects and pain; seeking pleasure in nourishment; engaging in entertaining activities and having fun; keeping the hope of cure alive; and finding support in religion. Children with cancer undergoing chemotherapy need to cope with hospitalizations, pain, medication side effects, idle time, and uncertainty regarding the success of treatment. These challenges motivated children to develop their own coping strategies, which were effective while undergoing chemotherapy. By gaining knowledge and further understanding about valid coping strategies during chemotherapy treatment, health professionals can mobilize personal and material resources from the children, health teams, and institutions aiming to potentiate the use of these strategies to make treatments the least traumatic. © 2015 Sigma Theta Tau International.

  3. Increased de novo lipogenesis and delayed conversion of large VLDL into intermediate density lipoprotein particles contribute to Hyperlipidemia in glycogen storage disease type 1a

    NARCIS (Netherlands)

    Bandsma, Robert H. J.; Prinsen, Berthil H.; Van Der Velden, Monique De Sain; Rake, Jan-Peter; Boer, Theo; Smit, G. Peter A.; Reijngoud, Dirk-Jan; Kuipers, Folkert

    Glycogen storage disease type 1a (GSD-1a) is a metabolic disorder characterized by fasting-induced hypoglycemia, hepatic steatosis, and hyperlipidemia. The mechanisms underlying the lipid abnormalities are largely unknown. To investigate these mechanisms seven GSD-1a patients and four healthy

  4. 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)

  5. Comparison of Dry Gas Seasonal Storage with CO2 Storage and Re-Use Potential

    OpenAIRE

    Killerud, Marie

    2013-01-01

    To make large-scale CO2 storage economic, many groups have proposed using CO2in EOR projects to create value for CO2 storage. However, CO2 EOR projectsgenerally require a large and variable supply of CO2 and consequently may requiretemporary storage of CO2 in geological formations. In order to store CO2 atoffshore sites as a source for CO2 EOR projects, the CO2 needs to be extractedfrom a storage site to a certain extent. Alternatively, CO2 EOR projects maybe developed alongside saline aquife...

  6. 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.

  7. 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)

  8. Long-term plutonium storage: Design concepts

    International Nuclear Information System (INIS)

    Wilkey, D.D.; Wood, W.T.; Guenther, C.D.

    1994-01-01

    An important part of the Department of Energy (DOE) Weapons Complex Reconfiguration (WCR) Program is the development of facilities for long-term storage of plutonium. The WCR design goals are to provide storage for metals, oxides, pits, and fuel-grade plutonium, including material being held as part of the Strategic Reserve and excess material. Major activities associated with plutonium storage are sorting the plutonium inventory, material handling and storage support, shipping and receiving, and surveillance of material in storage for both safety evaluations and safeguards and security. A variety of methods for plutonium storage have been used, both within the DOE weapons complex and by external organizations. This paper discusses the advantages and disadvantages of proposed storage concepts based upon functional criteria. The concepts discussed include floor wells, vertical and horizontal sleeves, warehouse storage on vertical racks, and modular storage units. Issues/factors considered in determining a preferred design include operational efficiency, maintenance and repair, environmental impact, radiation and criticality safety, safeguards and security, heat removal, waste minimization, international inspection requirements, and construction and operational costs

  9. Comparison of bispectral index and entropy monitoring in patients undergoing internalisation of deep brain stimulators

    Directory of Open Access Journals (Sweden)

    Suparna Bharadwaj

    2016-01-01

    Full Text Available Introduction: Depth of anaesthesia (DOA monitors are shown to reduce the intra-operative dose of anaesthetic agents, provide haemodynamic stability and shorten emergence times. Electroencephalography (EEG based DOA monitors such as bispectral index (BIS and entropy have been calibrated and validated in healthy subjects. Hence the clinical effectiveness of these monitors may be affected when monitoring patients with neurological disorders (e.g., epilepsy, dystonia, dementia and Parkinson's disease. The aim of this study was to determine whether BIS and entropy correlate with each other and with clinical indices of DOA in patients with movement disorders under general anaesthesia (GA. Materials and Methods: We conducted a prospective, observational study in patients with movement disorders undergoing internalization of deep brain stimulators. All patients received standard GA with age-adjusted mean alveolar concentration (aaMAC of an inhalational agent between 0.7 and 1.1. BIS and entropy sensors were applied on the patient's left forehead. Data collected included clinical parameters and EEG-based DOA indices. Correlation analysis was performed between entropy, BIS and the clinical indices of DOA. Bland Altman analysis was performed to determine the agreement between BIS and entropy. Results: Thirty patients were studied (mean age was 58.4 ± 11 years, male: female 18:12 and weight 79.2 ± 17 kg. Indications for deep brain stimulation were Parkinson's disease (n = 25, essential tremors (n = 2 and dystonia (n = 3. There was a very strong positive correlation between BIS and response entropy (RE (r = 0.932 and BIS and state entropy (SE (r = 0.950 and a strong negative correlation among aaMAC and BIS, RE and SE with r values of −0.686, −0.788 and −0.732, respectively. However, there was no correlation between BIS, RE, SE and haemodynamic values. Conclusion: Our study showed that BIS and entropy perform well in patients with movement disorders

  10. Order-disorder antiferroelectric phase transition in a hybrid inorganic-organic framework with the perovskite architecture.

    Science.gov (United States)

    Jain, Prashant; Dalal, Naresh S; Toby, Brian H; Kroto, Harold W; Cheetham, Anthony K

    2008-08-13

    [(CH3)2NH2]Zn(HCOO)3, 1, adopts a structure that is analogous to that of a traditional perovskite, ABX3, with A = [(CH3)2NH2], B = Zn, and X = HCOO. The hydrogen atoms of the dimethyl ammonium cation, which hydrogen bond to oxygen atoms of the formate framework, are disordered at room temperature. X-ray powder diffraction, dielectric constant, and specific heat data show that 1 undergoes an order-disorder phase transition on cooling below 156 K. We present evidence that this is a classical paraelectric to antiferroelectric phase transition that is driven by ordering of the hydrogen atoms. This sort of electrical ordering associated with order-disorder phase transition is unprecedented in hybrid frameworks and opens up an exciting new direction in rational synthetic strategies to create extended hybrid networks for applications in ferroic-related fields.

  11. Radioactive waste interim storage in Germany

    International Nuclear Information System (INIS)

    2015-12-01

    The short summary on the radioactive waste interim storage in Germany covers the following issues: importance of interim storage in the frame of radioactive waste management, responsibilities and regulations, waste forms, storage containers, transport of vitrified high-level radioactive wastes from the reprocessing plants, central interim storage facilities (Gorleben, Ahaus, Nord/Lubmin), local interim storage facilities at nuclear power plant sites, federal state collecting facilities, safety, radiation exposure in Germany.

  12. Synthesis of long live storage studies surface storage of MA-VL wastes

    International Nuclear Information System (INIS)

    Hollender, F.; Jourdain, F.; Piault, E.; Blanchet, Y.; Avakian, G.; Goger, F.; Caillaud, J.; Devictor, N.; Bary, B.; Moitier, C.; Breton, E.; Ranc, G.; Gaillard, J.P.; Lagrave, H.

    2004-01-01

    This document is realized in the framework of the axis 3 of the law of 1991 on the radioactive wastes management. It presents a long time surface storage installation of medium activity long life wastes. The long time of the installation would reach 300 years at the maximum. The feasibility is demonstrated and the design choices are presented and justified. The specific points of the long time storage installation, which are different from a classical industrial storage installation, are also discussed. (A.L.B.)

  13. The prospects for dry fuel storage

    International Nuclear Information System (INIS)

    Harris, G.G.; Elliott, D.

    1994-01-01

    Dry storage of spent nuclear fuels is one method of dealing with radioactive waste. This article reports from a one day seminar on future prospects for dry fuel storage held in November 1993. Dry storage in an inert gas or air environment in vaults or casks, is an alternative to wet storage in water-filled ponds. Both wet and dry storage form part of the Interim Storage option for radioactive waste materials, and form alternatives to reprocessing or direct disposal in a deep repository. It has become clear that a large market for dry fuel storage will exist in the future. It will therefore be necessary to ensure that the various technical, safety, commercial, legislative and political constraints associated with it can be met effectively. (UK)

  14. Research on high-performance mass storage system

    International Nuclear Information System (INIS)

    Cheng Yaodong; Wang Lu; Huang Qiulan; Zheng Wei

    2010-01-01

    With the enlargement of scientific experiments, more and more data will be produced, which brings great challenge to storage system. Large storage capacity and high data access performance are both important to Mass storage system. This paper firstly reviews some kinds of popular storage systems including network storage system, SAN-based sharing system, WAN File system, object-based parallel file system, hierarchical storage system and cloud storage systems. Then some key technologies are presented. Finally, this paper takes BES storage system as an example and introduces its requirements, architecture and operation results. (authors)

  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. What is the real significance and management of major thyroid disorders in bipolar patients?

    Science.gov (United States)

    Sierra, Pilar; Cámara, Rosa; Tobella, Helena; Livianos, Lorenzo

    2014-01-01

    Thyroid disfunction affects negatively emotional stability and worsens the clinical course of bipolar affective disorder. The main stabilizer used in this illness, lithium carbonate has numerous effects on the physiology of the thyroid, with the most significant being the inhibition of thyroid hormone release that may occur at therapeutic levels. These dysfunctions have also been reported most frequently in bipolar patients not undergoing treatment with lithium, and was not completely explained by the effects of this drug. Apart from the numerous medical complications and mood disturbances, the cognitive or perceptual system may also be affected. In fact, the presence of thyroid disease increases the rates of obsessive compulsive disorder, phobias, panic disorder, major depressive disorder, cyclothymia, or bipolar disorder. In severe cases of hypothyroidism, the clinical symptoms and signs can be similar to a melancholic depression or dementia. It is therefore important to know well all these possible complications in daily clinical practice. This review will cover the main thyroid dysfunctions present in bipolar patients, whether ot not produced by treatment with lithium carbonate, and will provide a series of recommendations for clinical management. Copyright © 2013 SEP y SEPB. Published by Elsevier España. All rights reserved.

  17. The Contribution of Optical Coherence Tomography in Neuromyelitis Optica Spectrum Disorders

    Science.gov (United States)

    Mateo, Javier; Esteban, Olivia; Martínez, Mireya; Grzybowski, Andrzej; Ascaso, Francisco Javier

    2017-01-01

    Neuromyelitis optica spectrum disorders (NMOSD) comprises a group of central nervous system disorders of inflammatory autoimmune origin that mainly affect the optic nerves and the spinal cord and can cause severe visual and general disability. The clinical signs are similar to those of multiple sclerosis (MS), with the result that it is often difficult to differentiate between the two, thus leading to misdiagnosis. As the treatment and prognosis of NMOSD and MS are different, it is important to make an accurate and early diagnosis of NMOSD. Optical coherence tomography (OCT) is a non-invasive technique that enables a quantitative study of the changes that the optic nerve and the macula undergo in several neurodegenerative diseases. Many studies have shown that some of these changes, such as retinal nerve fiber layer thinning or microcystic macular edema, can be related to alterations in the brain due to neurodegenerative disorders. The purpose of this mini-review is to show how OCT can be useful for the diagnosis of NMOSD and follow-up of affected patients, as well as for the differential diagnosis with MS. PMID:29085325

  18. Live Cell Imaging During Germination Reveals Dynamic Tubular Structures Derived from Protein Storage Vacuoles of Barley Aleurone Cells

    Directory of Open Access Journals (Sweden)

    Verena Ibl

    2014-09-01

    Full Text Available The germination of cereal seeds is a rapid developmental process in which the endomembrane system undergoes a series of dynamic morphological changes to mobilize storage compounds. The changing ultrastructure of protein storage vacuoles (PSVs in the cells of the aleurone layer has been investigated in the past, but generally this involved inferences drawn from static pictures representing different developmental stages. We used live cell imaging in transgenic barley plants expressing a TIP3-GFP fusion protein as a fluorescent PSV marker to follow in real time the spatially and temporally regulated remodeling and reshaping of PSVs during germination. During late-stage germination, we observed thin, tubular structures extending from PSVs in an actin-dependent manner. No extensions were detected following the disruption of actin microfilaments, while microtubules did not appear to be involved in the process. The previously-undetected tubular PSV structures were characterized by complex movements, fusion events and a dynamic morphology. Their function during germination remains unknown, but might be related to the transport of solutes and metabolites.

  19. Development of Seasonal Storage in Denmark

    DEFF Research Database (Denmark)

    Heller, Alfred

    2000-01-01

    National survey on seasonal (thermal, large-scale) storage activities in Denmark. A storage programme under the Danish Energy Agency. Programme background, objectives, activities, projects and results.Technologies presented: Pit water storage, gravel water storage with pipe heat exchangers, lining...... materials for pit and lid designs....

  20. Abundance of intrinsic structural disorder in the histone H1 subtypes.

    Science.gov (United States)

    Kowalski, Andrzej

    2015-12-01

    The intrinsically disordered proteins consist of partially structured regions linked to the unstructured stretches, which consequently form the transient and dynamic conformational ensembles. They undergo disorder to order transition upon binding their partners. Intrinsic disorder is attributed to histones H1, perceived as assemblers of chromatin structure and the regulators of DNA and proteins activity. In this work, the comparison of intrinsic disorder abundance in the histone H1 subtypes was performed both by the analysis of their amino acid composition and by the prediction of disordered stretches, as well as by identifying molecular recognition features (MoRFs) and ANCHOR protein binding regions (APBR) that are responsible for recognition and binding. Both human and model organisms-animals, plants, fungi and protists-have H1 histone subtypes with the properties typical of disordered state. They possess a significantly higher content of hydrophilic and charged amino acid residues, arranged in the long regions, covering over half of the whole amino acid residues in chain. Almost complete disorder corresponds to histone H1 terminal domains, including MoRFs and ANCHOR. Those motifs were also identified in a more ordered histone H1 globular domain. Compared to the control (globular and fibrous) proteins, H1 histones demonstrate the increased folding rate and a higher proportion of low-complexity segments. The results of this work indicate that intrinsic disorder is an inherent structural property of histone H1 subtypes and it is essential for establishing a protein conformation which defines functional outcomes affecting on DNA- and/or partner protein-dependent cell processes. Copyright © 2015 Elsevier Ltd. All rights reserved.

  1. 40 CFR 280.220 - Ownership of an underground storage tank or underground storage tank system or facility or...

    Science.gov (United States)

    2010-07-01

    ... tank or underground storage tank system or facility or property on which an underground storage tank or underground storage tank system is located. 280.220 Section 280.220 Protection of Environment ENVIRONMENTAL... underground storage tank or underground storage tank system or facility or property on which an underground...

  2. Comparative proteomics of oxalate downregulated tomatoes points towards cross talk of signal components and metabolic consequences during post-harvest storage

    Directory of Open Access Journals (Sweden)

    Kanika Narula

    2016-08-01

    Full Text Available Fruits of angiosperms evolved intricate regulatory machinery for sensorial attributes and storage quality after harvesting. Organic acid composition of storage organs forms the molecular and biochemical basis of organoleptic and nutritional qualities with metabolic specialization. Of these, oxalic acid (OA, determines the post-harvest quality in fruits. Tomato (Solanum lycopersicum fruit have distinctive feature to undergo a shift from heterotrophic metabolism to carbon assimilation partitioning during storage. We have earlier shown that decarboxylative degradation of OA by FvOXDC leads to acid homeostasis besides increased fungal tolerance in E8.2-OXDC tomato. Here, we elucidate the metabolic consequences of oxalate down-regulation and molecular mechanisms that determine organoleptic features, signaling and hormonal regulation in E8.2-OXDC fruit during post-harvest storage. A comparative proteomics approach has been applied between wild-type and E8.2-OXDC tomato in temporal manner. The MS/MS analyses led to the identification of 32 and 39 differentially abundant proteins associated with primary and secondary metabolism, assimilation, biogenesis, and development in wild-type and E8.2-OXDC tomatoes, respectively. Next, we interrogated the proteome data using correlation network analysis that identified significant functional hubs pointing toward storage related coinciding processes through a common mechanism of function and modulation. Furthermore, physiochemical analyses exhibited reduced oxalic acid content with concomitant increase in citric acid, lycopene and marginal decrease in malic acid in E8.2-OXDC fruit. Nevertheless, E8.2-OXDC fruit maintained an optimal pH and a steady state acid pool. These might contribute to reorganization of pectin constituent, reduced membrane leakage and improved fruit firmness in E8.2-OXDC fruit with that of wild-type tomato during storage. Collectively, our study provides insights into kinetically controlled

  3. 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...

  4. 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.

  5. Chemical Changes In Shortfin Scad (Decapterus Macrosoma) at Chilled (4 degree Celcius) and Frozen (-18 degree Celcius) Storage

    International Nuclear Information System (INIS)

    Fazilah Fazilin Juhari; Norizzah Abd Rashid; Seng, C.C.; Anida Yusoff; Emilia Azrina Mohd Bakri

    2016-01-01

    The aim of this study was to determine the chemical changes in muscle tissue of shortfin scad during storage at chilled (4 degree Celcius) and frozen (-18 degree Celcius) conditions for 18 days. The chemical changes were monitored every three days for Thiobarbituric acid (TBA), Peroxide value (PV), Total Volatile Base Nitrogen (TVBN) and Trimethylamine (TMA) content. Results show that there was a significant difference (p<0.05) in peroxide and TBA values between chilled and frozen shortfin scad starting from day 3. The highest PV values occured in chilled and frozen shortfin scad at day 12 (1.57 mEq/ kg and 1.13 mEq/ kg, respectively), and then decreased due to decomposition of hydroperoxides to secondary products such as aldehydes, alcohols and ketones. In contrast, TBA reached the highest values at day 15 for both chilled and frozen shortfin scad. For TVBN content, only the chilled sample shows significant increased (p<0.05) with storage time. The TVBN values declined significantly (p<0.05) for frozen shortfin scad. The TMA values for both chilled and frozen shortfin scad increased during storage. However, the TMA values increased at a faster rate in chilled compared to frozen shortfin scad. Based on the PV, TBA, TVBN and TMA values, chilled shortfin scad undergoes spoilage at a faster rate compared to the frozen shortfin scad. (author)

  6. Thermodynamic analysis of a compressed carbon dioxide energy storage system using two saline aquifers at different depths as storage reservoirs

    International Nuclear Information System (INIS)

    Liu, Hui; He, Qing; Borgia, Andrea; Pan, Lehua; Oldenburg, Curtis M.

    2016-01-01

    Highlights: • A compressed CO_2 energy storage system using two storage reservoirs is presented. • Compressed CO_2 energy storage density is higher than that of CAES. • The effects of storage reservoir pressure on the system performance are studied. - Abstract: Compressed air energy storage (CAES) is one of the leading large-scale energy storage technologies. However, low thermal efficiency and low energy storage density restrict its application. To improve the energy storage density, we propose a two-reservoir compressed CO_2 energy storage system. We present here thermodynamic and parametric analyses of the performance of an idealized two-reservoir CO_2 energy storage system under supercritical and transcritical conditions using a steady-state mathematical model. Results show that the transcritical compressed CO_2 energy storage system has higher round-trip efficiency and exergy efficiency, and larger energy storage density than the supercritical compressed CO_2 energy storage. However, the configuration of supercritical compressed CO_2 energy storage is simpler, and the energy storage densities of the two systems are both higher than that of CAES, which is advantageous in terms of storage volume for a given power rating.

  7. Electricity storage by gas pumping. An introduction to thermodynamic storage processes

    International Nuclear Information System (INIS)

    Ruer, Jacques

    2013-01-01

    To date, Pumped Hydro Storage (PHS) is practically the only technology used to store large quantities of electricity. There are however other ways to achieve the same goal. There are not yet well known, because the interest for large scale storage is quite new A complete family of storage technologies can be defined as 'Thermodynamic Storage Systems'. Their only common factor is that a gas is pumped and expanded in the process. If the gas is air taken from the atmosphere and discharged to it, the system is said 'an open system'. This is already developed in the form of Compressed Air Energy Storage (CAES). Different embodiments are possible, following the way the heat gene - rated during the compression stage is conserved. The compressed air is generally stored in underground caverns created in deep salt formations. 2 installations are presently operating and many projects are envisaged. if the gas circulates in closed loop within the plant, the system is said 'a closed system' In this case, the energy is stored as heat and/or cold at different temperature levels. A great variety of technologies can be imagined and are under development, using different gases (e.g. argon, CO 2 ) and different temperature ranges. PHS and CAES require specific sites for water reservoirs or underground caverns. The dosed systems can be installed basically anywhere. (author)

  8. 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.

  9. Data Centre Infrastructure & Data Storage @ Facebook

    CERN Multimedia

    CERN. Geneva; Garson, Matt; Kauffman, Mike

    2018-01-01

    Several speakers from the Facebook company will present their take on the infrastructure of their Data Center and Storage facilities, as follows: 10:00 - Facebook Data Center Infrastructure, by Delfina Eberly, Mike Kauffman and Veerendra Mulay Insight into how Facebook thinks about data center design, including electrical and cooling systems, and the technology and tooling used to manage data centers. 11:00 - Storage at Facebook, by Matt Garson An overview of Facebook infrastructure, focusing on different storage systems, in particular photo/video storage and storage for data analytics. About the speakers Mike Kauffman, Director, Data Center Site Engineering Delfina Eberly, Infrastructure, Site Services Matt Garson, Storage at Facebook Veerendra Mulay, Infrastructure

  10. Integrating new Storage Technologies into EOS

    Science.gov (United States)

    Peters, Andreas J.; van der Ster, Dan C.; Rocha, Joaquim; Lensing, Paul

    2015-12-01

    The EOS[1] storage software was designed to cover CERN disk-only storage use cases in the medium-term trading scalability against latency. To cover and prepare for long-term requirements the CERN IT data and storage services group (DSS) is actively conducting R&D and open source contributions to experiment with a next generation storage software based on CEPH[3] and ethernet enabled disk drives. CEPH provides a scale-out object storage system RADOS and additionally various optional high-level services like S3 gateway, RADOS block devices and a POSIX compliant file system CephFS. The acquisition of CEPH by Redhat underlines the promising role of CEPH as the open source storage platform of the future. CERN IT is running a CEPH service in the context of OpenStack on a moderate scale of 1 PB replicated storage. Building a 100+PB storage system based on CEPH will require software and hardware tuning. It is of capital importance to demonstrate the feasibility and possibly iron out bottlenecks and blocking issues beforehand. The main idea behind this R&D is to leverage and contribute to existing building blocks in the CEPH storage stack and implement a few CERN specific requirements in a thin, customisable storage layer. A second research topic is the integration of ethernet enabled disks. This paper introduces various ongoing open source developments, their status and applicability.

  11. Transparent caesium bromide storage-phosphors for radiation imaging

    Energy Technology Data Exchange (ETDEWEB)

    Winch, Nicola M. [School of Chemical and Physical Sciences, Victoria University of Wellington, Wellington (New Zealand); Edgar, Andrew [School of Chemical and Physical Sciences, Victoria University of Wellington, Wellington (New Zealand); MacDiarmid Institute for Advanced Materials and Nanotechnology, Victoria University of Wellington, Wellington (New Zealand)

    2012-12-15

    Semi-transparent discs of polycrystalline CsBr:Eu{sup 2+} storage phosphor for X-ray imaging have been produced using a room temperature milling/pressing technique, and have been studied using infrared, and photoluminescence spectroscopies, together with scanning electron microscopy. The pressed discs are very sensitive to water vapour, and scanning electron microscopy shows that material undergoes a complete recrystallization following exposure to humid air for a few minutes through a room temperature hydration/crystallization process. Discs prepared by a simple mixture of CsBr and EuBr{sub 2} show photostimulated luminescence (PSL) at 440 nm without further processing, and the conversion efficiency is sufficient that X-ray imaging can be demonstrated. Discs which have been prepared by first sintering at 600 C, then pressed, and finally hydrated at room temperature show very strong PSL with a conversion efficiency of {proportional_to}36 pJ mR{sup -1} mm{sup -3}. A model is proposed for the generation of the active PSL centres in CsBr by the milling/hydration process. (Copyright copyright 2012 WILEY-VCH Verlag GmbH and Co. KGaA, Weinheim)

  12. Fetal ascites and oligohydramnios: prenatal diagnosis of a sialic acid storage disease (index case).

    Science.gov (United States)

    Poulain, P; Odent, S; Maire, I; Milon, J; Proudhon, J F; Jouan, H; Le Marec, B

    1995-09-01

    In a 20-year-old primiparous patient, a routine ultrasound scan performed at 28 weeks revealed fetal ascites, bilateral talipes, and oligohydramnios. This woman, married to possibly her first cousin, was at risk for an autosomal recessive disease, a metabolic disorder. At 29 weeks, an amniotic fluid biochemical study revealed the presence of an abnormal band of free sialic acid, leading to a diagnosis of a congenital form of sialic acid storage disease. Termination of pregnancy was performed at 30 weeks. Measurement of free sialic acid in cultured fetal skin fibroblasts confirmed the diagnosis.

  13. Energy storage. A challenge for energy transition

    International Nuclear Information System (INIS)

    Bart, Jean-Baptiste; Nekrasov, Andre; Pastor, Emmanuel; Benefice, Emmanuel; Brincourt, Thierry; Brisse, Annabelle; Cagnac, Albannie; Delille, Gauthier; Hinchliffe, Timothee; Lancel, Gilles; Jeandel, Elodie; Lefebvre, Thierry; Loevenbruck, Philippe; Penneau, Jean-Francois; Soler, Robert; Stevens, Philippe; Radvanyi, Etienne; Torcheux, Laurent

    2017-06-01

    Written by several EDF R and D engineers, this book aims at presenting an overview of knowledge and know-how of EDF R and D in the field of energy storage, and at presenting the different technologies and their application to electric power systems. After a description of the context related to a necessary energy transition, the authors present the numerous storage technologies. They distinguish direct storage of power (pumped storage water stations, compressed air energy storage, flywheels, the various electrochemical batteries, metal-air batteries, redox flow batteries, superconductors), thermal storage (power to heat, heat to power) and hydrogen storage (storage under different forms), and propose an overview of the situation of standardisation of storage technologies. In the next part, they give an overview of the main services provided by storage to the electric power system: production optimisation, frequency adjustment, grid constraint resolution, local smoothing of PV and wind production, supply continuity. The last part discusses perspectives regarding the role of tomorrow's storage in the field of electrical mobility, for emerging markets, and with respect to different scenarios

  14. 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.

  15. 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

  16. 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

  17. 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.

  18. 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)

  19. Economics of spent LWR fuel storage

    International Nuclear Information System (INIS)

    Clark, H.J.

    1980-01-01

    A low cost option for spent fuel inventories would be to ship excess fuel from the overburdened reactor to another reactor in the utility's system that has available space. The only cost would be for cask leasing and shipping. Three other alternatives all require considerable capital expenditures: reracking, new at-reactor (AR) storage facilities, and away-from-reactor (AFR) storage facilities. Fuel storage requirements will be met best by transfer of fuel or by re-racking existing reactor basins whenever these options are available. These alternatives represent not only the lowest cost storage options but also the most timely. Fuel can be shipped to other storage pools for about $10/kg depending on the distance, while costs for reracking range from $18 to 25/kg depending on the approach. These alternatives are recognized to face environmental and regulatory obstacles. However, such obstacles should be less severe than similar issues that would be encountered with AR or AFR basin storage. When storage requirements cannot be met by the first two options, the next least costly alternative for most utilities will be use of a Federal AFR. Storage cost of about $137/kg at an AFR are less costly than charges of up to $350/kg that could be incurred by the use of AR basins. AR basins are practical only when a utility requires storage capacity to accommodate annual additions of 100 MT or more of spent fuel. The large reactor complexes discharging this much feul are not currently those that require relief from fuel storage problems. A recent development in Germany may offer an AR alternative of dry storage in transportation/storage casks at a cost of $200/kg; however, this method has not yet been accepted and licensed for use in the US

  20. SLUDGE TREATMENT PROJECT PHASE 1 SLUDGE STORAGE OPTIONS. ASSESSMENT OF T PLANT VERSUS ALTERNATE STORAGE FACILITY

    International Nuclear Information System (INIS)

    Rutherford, W.W.; Geuther, W.J.; Strankman, M.R.; Conrad, E.A.; Rhoadarmer, D.D.; Black, D.M.; Pottmeyer, J.A.

    2009-01-01

    The CH2M HILL Plateau Remediation Company (CHPRC) has recommended to the U.S. Department of Energy (DOE) a two phase approach for removal and storage (Phase 1) and treatment and packaging for offsite shipment (Phase 2) of the sludge currently stored within the 105-K West Basin. This two phased strategy enables early removal of sludge from the 105-K West Basin by 2015, allowing remediation of historical unplanned releases of waste and closure of the 100-K Area. In Phase 1, the sludge currently stored in the Engineered Containers and Settler Tanks within the 105-K West Basin will be transferred into sludge transport and storage containers (STSCs). The STSCs will be transported to an interim storage facility. In Phase 2, sludge will be processed (treated) to meet shipping and disposal requirements and the sludge will be packaged for final disposal at a geologic repository. The purpose of this study is to evaluate two alternatives for interim Phase 1 storage of K Basin sludge. The cost, schedule, and risks for sludge storage at a newly-constructed Alternate Storage Facility (ASF) are compared to those at T Plant, which has been used previously for sludge storage. Based on the results of the assessment, T Plant is recommended for Phase 1 interim storage of sludge. Key elements that support this recommendation are the following: (1) T Plant has a proven process for storing sludge; (2) T Plant storage can be implemented at a lower incremental cost than the ASF; and (3) T Plant storage has a more favorable schedule profile, which provides more float, than the ASF. Underpinning the recommendation of T Plant for sludge storage is the assumption that T Plant has a durable, extended mission independent of the K Basin sludge interim storage mission. If this assumption cannot be validated and the operating costs of T Plant are borne by the Sludge Treatment Project, the conclusions and recommendations of this study would change. The following decision-making strategy, which is

  1. SLUDGE TREATMENT PROJECT PHASE 1 SLUDGE STORAGE OPTIONS ASSESSMENT OF T PLANT VERSUS ALTERNATE STORAGE FACILITY

    Energy Technology Data Exchange (ETDEWEB)

    RUTHERFORD WW; GEUTHER WJ; STRANKMAN MR; CONRAD EA; RHOADARMER DD; BLACK DM; POTTMEYER JA

    2009-04-29

    The CH2M HILL Plateau Remediation Company (CHPRC) has recommended to the U.S. Department of Energy (DOE) a two phase approach for removal and storage (Phase 1) and treatment and packaging for offsite shipment (Phase 2) of the sludge currently stored within the 105-K West Basin. This two phased strategy enables early removal of sludge from the 105-K West Basin by 2015, allowing remediation of historical unplanned releases of waste and closure of the 100-K Area. In Phase 1, the sludge currently stored in the Engineered Containers and Settler Tanks within the 105-K West Basin will be transferred into sludge transport and storage containers (STSCs). The STSCs will be transported to an interim storage facility. In Phase 2, sludge will be processed (treated) to meet shipping and disposal requirements and the sludge will be packaged for final disposal at a geologic repository. The purpose of this study is to evaluate two alternatives for interim Phase 1 storage of K Basin sludge. The cost, schedule, and risks for sludge storage at a newly-constructed Alternate Storage Facility (ASF) are compared to those at T Plant, which has been used previously for sludge storage. Based on the results of the assessment, T Plant is recommended for Phase 1 interim storage of sludge. Key elements that support this recommendation are the following: (1) T Plant has a proven process for storing sludge; (2) T Plant storage can be implemented at a lower incremental cost than the ASF; and (3) T Plant storage has a more favorable schedule profile, which provides more float, than the ASF. Underpinning the recommendation of T Plant for sludge storage is the assumption that T Plant has a durable, extended mission independent of the K Basin sludge interim storage mission. If this assumption cannot be validated and the operating costs of T Plant are borne by the Sludge Treatment Project, the conclusions and recommendations of this study would change. The following decision-making strategy, which is

  2. Integrating new Storage Technologies into EOS

    CERN Document Server

    Peters, Andreas J; Rocha, Joaquim; Lensing, Paul

    2015-01-01

    The EOS[1] storage software was designed to cover CERN disk-only storage use cases in the medium-term trading scalability against latency. To cover and prepare for long-term requirements the CERN IT data and storage services group (DSS) is actively conducting R&D; and open source contributions to experiment with a next generation storage software based on CEPH[3] and ethernet enabled disk drives. CEPH provides a scale-out object storage system RADOS and additionally various optional high-level services like S3 gateway, RADOS block devices and a POSIX compliant file system CephFS. The acquisition of CEPH by Redhat underlines the promising role of CEPH as the open source storage platform of the future. CERN IT is running a CEPH service in the context of OpenStack on a moderate scale of 1 PB replicated storage. Building a 100+PB storage system based on CEPH will require software and hardware tuning. It is of capital importance to demonstrate the feasibility and possibly iron out bottlenecks and blocking issu...

  3. Criticality impacts on LWR fuel storage efficiency

    International Nuclear Information System (INIS)

    Napolitano, D.

    1992-01-01

    This presentation discusses the criticality impacts throughout storage of fuel onsite including new fuel storage, spent fuel storage, consolidation, and dry storage. The general principles for criticality safety are also be discussed. There is first an introduction which explains today's situation for criticality safety concerns. This is followed by a discussion of criticality safety Regulatory Guides, safety limits and fundamental principles. Design objectives for criticality safety in the 1990's include higher burnups, longer cycles, and higher enrichments which impact the criticality safety design. Criticality safety for new fuel storage, spent fuel storage, fuel consolidation, and dry storage are followed by conclusions. Today's situation is one in which the US does not reprocess, and does not have an operating MRS facility or repository. High density fuel storage rack designs of the 1980s, are filling up. Dry cask storage systems for spent fuel storage are being utilized. Enrichments continue to increase PWR fuel assemblies with enrichments of 4.5 to 5.0 weight percent U-235 and BWR fuel assemblies with enrichments of 3.25 to 3.5 weight percent U-235 are common. Criticality concerns affect the capacity and the economics of light water reactor (LWR) fuel storage arrays by dictating the spacing of fuel assemblies in a storage system, or the use of poisons or exotic materials in the storage system design

  4. Energy storage: a review of recent literature

    International Nuclear Information System (INIS)

    Tatone, O.S.

    1981-12-01

    Recent literature on the technological and economic status of reversible energy storage has been reviewed. A broad range of research and development activities have been pursued between 1975 and the present. Most of this work has concentrated on improving technical and economic performance of previously known storage technologies. Hydraulic pumped storage with both reservoirs above ground and compressed air storage (1 plant) are the only methods that have been adopted by electric utilities. The need for electrical energy storage in Canada has not been acute because of the large proportion of hydraulic generation which incorporates some storge and, in most cases, can readily be used for load-following. Residential heat storage in ceramic room heaters has been used in Europe for several years. For Canadian climatic and market conditions larger, central heating units would be required. Residential heat storage depends upon utilities offering time-of-use rates and none in Canada do so at present. Most seasonal storage concepts depend upon storage of low-grade heat for district heating. The cost of energy storage is highly dependent upon annual energy throughput and hence favours smaller capacity systems operating on frequent charge/discharge cycles over long-term storage. Capital costs of energy storage methods from the literature, expressed in constant dollars, are compared graphically and tentative investment costs are presented for several storage methods

  5. Energy storage label : a method for comparing storage systems over all ranges

    NARCIS (Netherlands)

    Pierie, Frank; van Someren, Christian

    2015-01-01

    This report describes the creation and use of a database for energy storage technologies which was developed in conjunction with Netbeheer Nederland and the Hanze University of Applied Sciences. This database can be used to make comparisons between a selection of storage technologies and will

  6. Translational Chemistry Meets Gluten-Related Disorders.

    Science.gov (United States)

    Lammers, Karen M; Herrera, Maria G; Dodero, Veronica I

    2018-03-01

    Gluten-related disorders are a complex group of diseases that involve the activation of the immune system triggered by the ingestion of gluten. Among these, celiac disease, with a prevalence of 1 %, is the most investigated, but recently, a new pathology, named nonceliac gluten sensitivity, was reported with a general prevalence of 7 %. Finally, there other less-prevalent gluten-related diseases such as wheat allergy, gluten ataxia, and dermatitis herpetiformis (with an overall prevalence of less than 0.1 %). As mentioned, the common molecular trigger is gluten, a complex mixture of storage proteins present in wheat, barley, and a variety of oats that are not fully degraded by humans. The most-studied protein related to disease is gliadin, present in wheat, which possesses in its sequence many pathological fragments. Despite a lot of effort to treat these disorders, the only effective method is a long-life gluten-free diet. This Review summarizes the actual knowledge of gluten-related disorders from a translational chemistry point of view. We discuss what is currently known from the literature about the interaction of gluten with the gut and the critical host responses it evokes and, finally, connect them to our current and novel molecular understanding of the supramolecular organization of gliadin and the 33-mer gliadin peptide fragment under physiological conditions.

  7. A mouse model for fucosidosis recapitulates storage pathology and neurological features of the milder form of the human disease

    Directory of Open Access Journals (Sweden)

    Heike Wolf

    2016-09-01

    Full Text Available Fucosidosis is a rare lysosomal storage disorder caused by the inherited deficiency of the lysosomal hydrolase α-L-fucosidase, which leads to an impaired degradation of fucosylated glycoconjugates. Here, we report the generation of a fucosidosis mouse model, in which the gene for lysosomal α-L-fucosidase (Fuca1 was disrupted by gene targeting. Homozygous knockout mice completely lack α-L-fucosidase activity in all tested organs leading to highly elevated amounts of the core-fucosylated glycoasparagine Fuc(α1,6-GlcNAc(β1-N-Asn and, to a lesser extent, other fucosylated glycoasparagines, which all were also partially excreted in urine. Lysosomal storage pathology was observed in many visceral organs, such as in the liver, kidney, spleen and bladder, as well as in the central nervous system (CNS. On the cellular level, storage was characterized by membrane-limited cytoplasmic vacuoles primarily containing water-soluble storage material. In the CNS, cellular alterations included enlargement of the lysosomal compartment in various cell types, accumulation of secondary storage material and neuroinflammation, as well as a progressive loss of Purkinje cells combined with astrogliosis leading to psychomotor and memory deficits. Our results demonstrate that this new fucosidosis mouse model resembles the human disease and thus will help to unravel underlying pathological processes. Moreover, this model could be utilized to establish diagnostic and therapeutic strategies for fucosidosis.

  8. 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 ...

  9. Efficiency of Compressed Air Energy Storage

    DEFF Research Database (Denmark)

    Elmegaard, Brian; Brix, Wiebke

    2011-01-01

    The simplest type of a Compressed Air Energy Storage (CAES) facility would be an adiabatic process consisting only of a compressor, a storage and a turbine, compressing air into a container when storing and expanding when producing. This type of CAES would be adiabatic and would if the machines...... were reversible have a storage efficiency of 100%. However, due to the specific capacity of the storage and the construction materials the air is cooled during and after compression in practice, making the CAES process diabatic. The cooling involves exergy losses and thus lowers the efficiency...... of the storage significantly. The efficiency of CAES as an electricity storage may be defined in several ways, we discuss these and find that the exergetic efficiency of compression, storage and production together determine the efficiency of CAES. In the paper we find that the efficiency of the practical CAES...

  10. 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

  11. 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

  12. 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

  13. Bioinformatics analysis of disordered proteins in prokaryotes

    Directory of Open Access Journals (Sweden)

    Malkov Saša N

    2011-03-01

    Full Text Available Abstract Background A significant number of proteins have been shown to be intrinsically disordered, meaning that they lack a fixed 3 D structure or contain regions that do not posses a well defined 3 D structure. It has also been proven that a protein's disorder content is related to its function. We have performed an exhaustive analysis and comparison of the disorder content of proteins from prokaryotic organisms (i.e., superkingdoms Archaea and Bacteria with respect to functional categories they belong to, i.e., Clusters of Orthologous Groups of proteins (COGs and groups of COGs-Cellular processes (Cp, Information storage and processing (Isp, Metabolism (Me and Poorly characterized (Pc. We also analyzed the disorder content of proteins with respect to various genomic, metabolic and ecological characteristics of the organism they belong to. We used correlations and association rule mining in order to identify the most confident associations between specific modalities of the characteristics considered and disorder content. Results Bacteria are shown to have a somewhat higher level of protein disorder than archaea, except for proteins in the Me functional group. It is demonstrated that the Isp and Cp functional groups in particular (L-repair function and N-cell motility and secretion COGs of proteins in specific possess the highest disorder content, while Me proteins, in general, posses the lowest. Disorder fractions have been confirmed to have the lowest level for the so-called order-promoting amino acids and the highest level for the so-called disorder promoters. For each pair of organism characteristics, specific modalities are identified with the maximum disorder proteins in the corresponding organisms, e.g., high genome size-high GC content organisms, facultative anaerobic-low GC content organisms, aerobic-high genome size organisms, etc. Maximum disorder in archaea is observed for high GC content-low genome size organisms, high GC content

  14. 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.)

  15. 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

  16. Compartmentalized storage tank for electrochemical cell system

    Science.gov (United States)

    Piecuch, Benjamin Michael (Inventor); Dalton, Luke Thomas (Inventor)

    2010-01-01

    A compartmentalized storage tank is disclosed. The compartmentalized storage tank includes a housing, a first fluid storage section disposed within the housing, a second fluid storage section disposed within the housing, the first and second fluid storage sections being separated by a movable divider, and a constant force spring. The constant force spring is disposed between the housing and the movable divider to exert a constant force on the movable divider to cause a pressure P1 in the first fluid storage section to be greater than a pressure P2 in the second fluid storage section, thereby defining a pressure differential.

  17. 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...

  18. 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

  19. 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

  20. 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

  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. Nonlinear Modeling and Application of PI Control on Pre-cooling Session of a Carbon Dioxide Storage Tank at Normal Temperature and Pressure

    Energy Technology Data Exchange (ETDEWEB)

    Lim, Yu Kyung; Lee, Seok Goo; Dan, Seungkyu; Lee, Jong Min [Seoul National University, Seoul (Korea, Republic of); Ko, Min Su [Samsung Heavy Industries, Geoje (Korea, Republic of)

    2014-10-15

    Storage tanks of Carbon dioxide (CO{sub 2}) carriers utilized for the purpose of carbon capture and storage (CCS) into subsea strata have to undergo a pre-cooling session before beginning to load cryogenic liquid cargos in order to prevent physical and thermal deterioration of tanks which may result from cryogenic CO{sub 2} contacting tank walls directly. In this study we propose dynamic model to calculate the tank inflow of CO{sub 2} gas injected for precooling process and its dynamic simulation results under proportional-integral control algorithm. We selected two cases in which each of them had one controlled variable (CV) as either the tank pressure or the tank temperature and discussed the results of that decision-making on the pre-cooling process. As a result we demonstrated that the controlling instability arising from nonlinearity and singularity of the mathematical model could be avoided by choosing tank pressure as CV instead of tank temperature.

  3. 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

  4. Analysis on applicable error-correcting code strength of storage class memory and NAND flash in hybrid storage

    Science.gov (United States)

    Matsui, Chihiro; Kinoshita, Reika; Takeuchi, Ken

    2018-04-01

    A hybrid of storage class memory (SCM) and NAND flash is a promising technology for high performance storage. Error correction is inevitable on SCM and NAND flash because their bit error rate (BER) increases with write/erase (W/E) cycles, data retention, and program/read disturb. In addition, scaling and multi-level cell technologies increase BER. However, error-correcting code (ECC) degrades storage performance because of extra memory reading and encoding/decoding time. Therefore, applicable ECC strength of SCM and NAND flash is evaluated independently by fixing ECC strength of one memory in the hybrid storage. As a result, weak BCH ECC with small correctable bit is recommended for the hybrid storage with large SCM capacity because SCM is accessed frequently. In contrast, strong and long-latency LDPC ECC can be applied to NAND flash in the hybrid storage with large SCM capacity because large-capacity SCM improves the storage performance.

  5. 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...

  6. Electricity storage - A challenge for energy transition

    International Nuclear Information System (INIS)

    Bart, Jean-Baptiste; Nekrasov, Andre; Pastor, Emmanuel; Benefice, Emmanuel; Brincourt, Thierry; Cagnac, Albannie; Brisse, Annabelle; Jeandel, Elodie; Lefebvre, Thierry; Penneau, Jean-Francois; Radvanyi, Etienne; Delille, Gautier; Hinchliffe, Timothee; Lancel, Gilles; Loevenbruck, Philippe; Soler, Robert; Stevens, Philippe; Torcheux, Laurent

    2017-01-01

    After a presentation of the energetic context and of its issues, this collective publication proposes presentations of various electricity storage technologies with a distinction between direct storage, thermal storage and hydrogen storage. As far as direct storage is concerned, the following options are described: pumped energy transfer stations or PETS, compressed air energy storage or CAES, flywheels, various types of electrochemical batteries (lead, alkaline, sodium, lithium), metal air batteries, redox flow batteries, and super-capacitors. Thermal storage comprises power-to-heat and heat-to-power technologies. Hydrogen can be stored under different forms (compressed gas, liquid), in saline underground cavities, or by using water electrolysis and fuel cells. The authors propose an overview of the different services provided by energy storage to the electricity system, and discuss the main perspectives and challenges for tomorrow's storage (electric mobility, integration of renewable energies, electrification of isolated areas, scenarios of development)

  7. 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.

  8. High Density Digital Data Storage System

    Science.gov (United States)

    Wright, Kenneth D., II; Gray, David L.; Rowland, Wayne D.

    1991-01-01

    The High Density Digital Data Storage System was designed to provide a cost effective means for storing real-time data from the field-deployable digital acoustic measurement system. However, the high density data storage system is a standalone system that could provide a storage solution for many other real time data acquisition applications. The storage system has inputs for up to 20 channels of 16-bit digital data. The high density tape recorders presently being used in the storage system are capable of storing over 5 gigabytes of data at overall transfer rates of 500 kilobytes per second. However, through the use of data compression techniques the system storage capacity and transfer rate can be doubled. Two tape recorders have been incorporated into the storage system to produce a backup tape of data in real-time. An analog output is provided for each data channel as a means of monitoring the data as it is being recorded.

  9. 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.

  10. 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)

  11. Transitions in genetic toggle switches driven by dynamic disorder in rate coefficients

    International Nuclear Information System (INIS)

    Chen, Hang; Thill, Peter; Cao, Jianshu

    2016-01-01

    In biochemical systems, intrinsic noise may drive the system switch from one stable state to another. We investigate how kinetic switching between stable states in a bistable network is influenced by dynamic disorder, i.e., fluctuations in the rate coefficients. Using the geometric minimum action method, we first investigate the optimal transition paths and the corresponding minimum actions based on a genetic toggle switch model in which reaction coefficients draw from a discrete probability distribution. For the continuous probability distribution of the rate coefficient, we then consider two models of dynamic disorder in which reaction coefficients undergo different stochastic processes with the same stationary distribution. In one, the kinetic parameters follow a discrete Markov process and in the other they follow continuous Langevin dynamics. We find that regulation of the parameters modulating the dynamic disorder, as has been demonstrated to occur through allosteric control in bistable networks in the immune system, can be crucial in shaping the statistics of optimal transition paths, transition probabilities, and the stationary probability distribution of the network.

  12. Transitions in genetic toggle switches driven by dynamic disorder in rate coefficients

    Energy Technology Data Exchange (ETDEWEB)

    Chen, Hang, E-mail: hangchen@mit.edu; Thill, Peter; Cao, Jianshu [Department of Chemistry, Massachusetts Institute of Technology, Cambridge, Massachusetts 02139 (United States)

    2016-05-07

    In biochemical systems, intrinsic noise may drive the system switch from one stable state to another. We investigate how kinetic switching between stable states in a bistable network is influenced by dynamic disorder, i.e., fluctuations in the rate coefficients. Using the geometric minimum action method, we first investigate the optimal transition paths and the corresponding minimum actions based on a genetic toggle switch model in which reaction coefficients draw from a discrete probability distribution. For the continuous probability distribution of the rate coefficient, we then consider two models of dynamic disorder in which reaction coefficients undergo different stochastic processes with the same stationary distribution. In one, the kinetic parameters follow a discrete Markov process and in the other they follow continuous Langevin dynamics. We find that regulation of the parameters modulating the dynamic disorder, as has been demonstrated to occur through allosteric control in bistable networks in the immune system, can be crucial in shaping the statistics of optimal transition paths, transition probabilities, and the stationary probability distribution of the network.

  13. Globally distributed software defined storage (proposal)

    Science.gov (United States)

    Shevel, A.; Khoruzhnikov, S.; Grudinin, V.; Sadov, O.; Kairkanov, A.

    2017-10-01

    The volume of the coming data in HEP is growing. The volume of the data to be held for a long time is growing as well. Large volume of data - big data - is distributed around the planet. The methods, approaches how to organize and manage the globally distributed data storage are required. The distributed storage has several examples for personal needs like own-cloud.org, pydio.com, seafile.com, sparkleshare.org. For enterprise-level there is a number of systems: SWIFT - distributed storage systems (part of Openstack), CEPH and the like which are mostly object storage. When several data center’s resources are integrated, the organization of data links becomes very important issue especially if several parallel data links between data centers are used. The situation in data centers and in data links may vary each hour. All that means each part of distributed data storage has to be able to rearrange usage of data links and storage servers in each data center. In addition, for each customer of distributed storage different requirements could appear. The above topics are planned to be discussed in data storage proposal.

  14. Technical study gas storage. Final report

    International Nuclear Information System (INIS)

    Borowka, J.; Moeller, A.; Zander, W.; Koischwitz, M.A.

    2001-01-01

    This study will answer the following questions: (a) For what uses was the storage facility designed and for what use is it currently applied? Provide an overview of the technical data per gas storage facility: for instance, what is its capacity, volume, start-up time, etc.; (b) How often has this facility been used during the past 10 years? With what purpose was the facility brought into operation at the time? How much gas was supplied at the time from the storage facility?; (c) Given the characteristics and the use of the storage facility during the past 10 years and projected gas consumption in the future, how will the storage facility be used in the future?; (d) Are there other uses for which the gas storage facility can be deployed, or can a single facility be deployed for numerous uses? What are the technical possibilities in such cases? Questions (a) and (b) are answered separately for every storage facility. Questions (c) and (d) in a single chapter each (Chapter 2 and 3). An overview of the relevant storage data relating to current use, use in the last 10 years and use in future is given in the Annex

  15. The storage capacity of cocoa seeds (Theobroma cacao L.) through giving Polyethylene Glycol (PEG) in the various of storage container

    Science.gov (United States)

    Lahay, R. R.; Misrun, S.; Sipayung, R.

    2018-02-01

    Cocoa is plant which it’s seed character is recalcitrant. Giving PEG and using various of storage containers was hoped to increase storage capacity of cocoa seeds as long as period of saving. The reseach was aimed to identify the storage capacity of cocoa seeds through giving PEG in the various of storage containers. Research took place in Hataram Jawa II, Kabupaten Simalungun, Propinsi Sumatera Utara, Indonesia. The method of this research is spit-split plot design with 3 replication. Storage period was put on main plot which was consisted of 4 level, PEG concentration was put on sub plot, consisted of 4 level and storage container was put on the sub sub plot consisted of 3 types. The results showed that until 4 days at storage with 45 % PEG concentration at all storage container, percentage of seed germination at storage can be decreased to be 2.90 %, and can be defensed until 16 days with 45 % PEG concentration at perforated plastic storage container. Percentage of molded seeds and seed moisture content were increased with added period of storage but seed moisture content was increased until 12 days at storage and was decreased at 16 days in storage.

  16. Sampling and decontamination plan for the Transuranic Storage Area--1/-R container storage unit

    International Nuclear Information System (INIS)

    Barry, G.A.

    1992-11-01

    This document describes the sampling and decontamination of the Transuranic Storage Area (TSA)-l/-R container storage area and the earthen-covered portion of the TSA-2 container storage unit at the Radioactive Waste Management Complex. Stored containers from the earthen-covered asphalt pads will be retrieved from the TSA-l/-R and TSA-2 container storage units. Container retrieval will be conducted under the TSA retrieval enclosure, a fabricated steel building to be constructed over the earthen-covered pad to provide containment and weather protection. Following container retrieval, the TSA retrieval enclosure will be decontaminated to remove radioactive and hazardous contamination. The underlying soils will be sampled and analyzed to determine whether any contaminated soils require removal

  17. 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

  18. The Effect of Sperm Concentration and Storage Vessel on Quercetin-Supplemented Rabbit Semen During Chilled Storage.

    Science.gov (United States)

    Johinke, D; de Graaf, S P; Bathgate, R

    2015-08-01

    Extending the shelf life of chilled rabbit spermatozoa is vital for the expansion of the farmed rabbit industry. This study evaluated the relationship between sperm concentration and packaging on in vitro quality of chilled rabbit semen over 96 h. Semen was collected from adult bucks (n = 4) and pooled at 37°C following evaluation. Pooled ejaculates were diluted with a Tris-based extender supplemented with 100 μm quercetin to a concentration of 15, 30 or 60 × 10(6)  spermatozoa/ml, packaged into plastic tubes or 0.5-ml straws and stored at 15°C. Sperm quality was assessed by computer-assisted sperm Analysis [total motility (tMOT)] and flow cytometry [viability, acrosome integrity, H2 O2 production, plasma membrane disorder, apoptosis and DNA fragmentation index (DFI)] at 0, 48, 72 and 96 h. From 48 h, concentrations of 30 and 60 × 10(6)  spermatozoa/ml reported the highest tMOT, irrespective of storage vessel (p straws reduced oxidative stress and improved plasma membrane stability. The %DFI, mean DFI and SD-DFI were increased in spermatozoa stored in tubes compared with straws (p < 0.05). Although the use of low sperm concentrations in artificial insemination doses would facilitate greater dispersion of genetically superior rabbit bucks, dilution to 15 × 10(6)  spermatozoa/ml had a detrimental impact on motility. As such, chilled storage at 30 × 10(6)  spermatozoa/ml may provide a suitable balance between motility and H2 O2 production to best maintain overall sperm function and should be evaluated in a large-scale AI trial. © 2015 Blackwell Verlag GmbH.

  19. Electrochemical Energy Storage Technical Team Roadmap

    Energy Technology Data Exchange (ETDEWEB)

    None

    2013-06-01

    This U.S. DRIVE electrochemical energy storage roadmap describes ongoing and planned efforts to develop electrochemical energy storage technologies for plug-in electric vehicles (PEVs). The Energy Storage activity comprises a number of research areas (including advanced materials research, cell level research, battery development, and enabling R&D which includes analysis, testing and other activities) for advanced energy storage technologies (batteries and ultra-capacitors).

  20. Licensing of spent fuel dry storage and consolidated rod storage: A Review of Issues and Experiences

    Energy Technology Data Exchange (ETDEWEB)

    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.